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A 40-year-old male was referred to oral surgery speciality at HCF Dental Centre, Sydney for opinion and treatment of a chronic ulcer. The patient stated that the lesion had first occurred 6 months back which had increased in size and giving him more discomfort. He was apparently fit and well and not on any medications.\nClinical examination revealed a large ulcer in the right buccal mucosa adjacent to tooth number 18. The ulcer was about 4 and 5 cm with the grayish pink base and indurated margins []. Tooth number 18 was buccally inclined and difficult to clean. There were no palpebal lymph nodes. The patient did not recall any trauma to cheek in the past.\nHe visited a dentist initially about 6 months back with the symptom of pain and discomfort in that region to be looked at. The patient was diagnosed as temporomandibular joint pain on the right side and was advised symptomatic treatment. There was no mention of any ulcer which might have been missed during the first visit. Subsequently, the area did not get better and he visited again to be checked after 5 months. Then, he was diagnosed as a chronic ulcer and got referred to oral surgery.\nConsidering chronicity of the ulcer and its indurated margin it was decided to have an excisional/incisional biopsy and the removal of tooth number 18 which could have been a causative factor for trauma. The patient decided to seek the second opinion where he was advised the same line of treatment.\nHe decided to have a biopsy and removal of the tooth under general anesthetic. After removal of tooth number 18, the exposure of ulcer was good and an excisional biopsy was performed with safety margins. The area was sutured primarily. The postoperative period was uneventful and the wound healed within 3 weeks without any complications []. A follow-up of a year showed no recurrence or abnormalities.\nMicroscopic examination revealed the extent of ulceration, and all sections presented similar histologic features. The surrounding epithelium was intact and well differentiated though slightly hyperplasic. The cellular infiltrate within the submucosal tissue consisted predominantly of eosinophils with a few scattered lymphocytes, plasma cells, and lymphocytes [].\nThe inflammatory infiltrate included numerous mast cells, particularly in the corium adjacent to the hyperplastic epithelium. The infiltrated tissue was well vascularized. No atypical cells or signs of granulomatous infiltration were seen []. A follow-up of a year showed no recurrence or abnormalities [].
A 43-year-old female patient had come with the complaint of pain and swelling in the left angle of mandible which was progressively increasing in size since the last 4 months. Pain was continuous and was aggravated while having food and while opening the mouth. Patient had been operated 15 years back for swelling at the same site, records for which were not available.\nOn inspection, a swelling was seen in the left angle of the mandible measuring around 7 cm × 5 cm in diameter. The skin over the swelling was puckered with a healed scar which was probably due to the previous surgery [].\nOn palpation, there was a firm to hard tender swelling in the left angle of mandible extending from left first molar to the third molar region which was fixed to underlying bone. The involved teeth showed no signs of mobility or resorption. There was no paresthesia in the area supplied by inferior alveolar nerve. The mucosa over the swelling was normal and showed no secondary changes.\nOPG showed scattered calcification superimposed over the left third molar and ramus region on the left side []. CT scan showed a well-encapsulated juxtacortical mass in left angle of mandible with areas of calcification [Figures and ]. There was erosion of cortical bone in the third molar and the anterior ramus region with some intramedullary extension. A well-defined swelling overlying the left mandibular body with calcified/ ossified areas was seen in the soft tissue.\nIncisional biopsy was done intraorally and the extrabony component of the mass was removed. The extraosseous mass was well encapsulated but fixed to the underlying bone which was eroded in the third molar region where the mass was found infiltrating into bone and was not separable. The section of specimen showed multiple irregular calcified masses some of them measuring almost 2 cm × 3 cm in diameter.\nH and E-stained section showed highly cellular areas of connective tissue in which lace-like pattern of neoplastic osteoid tissue was seen. There was no demarcation between tumor osteoid and rest of connective tissue cells. Osteoid tissue was surrounded by abnormally plump osteoblast and immature bone. Few osteoblasts exhibited abnormal mitosis. Rest of the connective tissue showed plump, pleormorphic, and haphazardly arranged cells with vesicular nuclei []. Moderate vascularity and areas of hemorrhage were also seen. Overall picture was suggestive of a low-grade osteosarcoma involving the mandible.\nThe patient was subjected to a whole body examination and relevant radiographs were done to rule out primaries elsewhere. The radiographs were normal and it was confirmed as primary osteosarcoma of the mandible.\nWithin 2 weeks of the biopsy, the patient developed trismus and increasing pain in the same region. The skin over the lesion was fixed and nonpinchable. Repeat CT scan showed involvement of masseter muscle along with the skin.\nPatient underwent a hemimandibulectomy with wide excision of the tumor mass with involved skin. Involved skin paddle was marked with a circular incision which was connected anteriorly to the left commisure of the lip. It was then extended along the paramedian line all along the neck to the clavicle where it was extended horizontally. Blunt dissection was done through layers. Lower border of mandible was exposed. The tumor mass along with the overlying skin and involved soft tissue and hemimandible on that side from canine to condyle was excised []. Adequate clearance was achieved on all aspects and was confirmed with fresh frozen section. A pectoralis major myocutaneous flap was raised and used to line the intraoral defect, while the overlying skin defect was covered with cervical rotation flap []. Postoperative period was uneventful and patient after regular follow-up for 1 year was symptom free with no signs of local recurrence or distant metastasis [].
A 75-year-old man was scheduled for elective coronary artery bypass grafting (CABG) due to coronary artery disease. His past medical history was notable for chronic atrial fibrillation and he had had a history of stable coronary artery disease for five years but he complained of angina at rest during the last two months before admission to our institution. His medications included aspirin, beta blockers, and nitrates. However, this patient was not receiving any anticoagulation. On admission to the hospital the patient underwent a transthoracic echocardiography that showed mild left ventricular systolic dysfunction with ejection fraction of 40% to 45% and mild dilation of both atria. The presence of interatrial septal aneurysm was not noticed. Coronary angiography revealed severe stenosis of the proximal left anterior descending artery. The EuroSCORE Logistic was estimated at 4.36%. A transesophageal echocardiography was performed after induction of anesthesia and tracheal intubation, and revealed a large echogenic mass in the right atrium (). The mass was at first thought to be a large myxoma or probably thrombus attached to the atrial septum. Continuing with TEE examination, a visible deficiency was observed in the huge mass which was attached to the atrial septum. This was possibly due to a detachment of part of the thrombus (Figures and ). The extraction of the echogenic parts towards the left atrium raised the suspicion of an atrial septal aneurysm which was completely filled with thrombi. In the following echo recordings, these thrombi were not visible any more (). A huge atrial septal aneurysm was revealed, after bolus injection of agitated saline in the right jugular vein with automated contrast () and having used provocation manoeuvres including ventilation with PEEP (15 cm H2O) and high tidal volume, a patent foramen ovale was established. Afterwards, during cannulation for cardiopulmonary bypass the patient became haemodynamically unstable and was urgently introduced into extracorporeal circulation. After institution of cardiopulmonary bypass (CBP) the aneurysm was initially resected and then CABG was successfully performed. Although the administration of inotropic agents had already started, the first attempt at weaning off was unsuccessful, CBP was restarted and intra-aortic balloon pumping (IABP) was instituted. In spite of repeatedly attempting and manipulating, this subject never achieved a successful separation from CBP. The fatal outcome was possibly due to multiple embolic events which occurred after induction of anaesthesia and mainly during cannulation.
The patient was a 76-year-old man who came to the office in 2014 looking for possible treatments of his fractured central incisors. Nothing was found relevant about his medical condition. The patient shows a high risk for caries and also eccentric bruxism. He has partial edentulism in the superior left quadrant and multiple decay and fractured teeth. The initial approach was conservative aiming to keep the upper front by means of composite fillings (Figures and ). Then, the posterior superior quadrants needed to be restored with implants.\nThree years after, in 2017, the patient came back to the office referring pain of endodontic origin in the upper left canine. New and secondary subgingival caries were found in the six front teeth. The conservative prognosis was considered poor due to the subgingival depth and extent of decay presented by the lesions from canine to canine. After having discussed the treatment options, especially the surgical lengthening of the front teeth or the orthodontic extrusion, the patient decides to replace the residual teeth with a new implant-supported bridge similar to the recently performed prostheses of the posterior areas that were judged by him as a highly satisfactory treatment. The patient preferred not to involve these restorations in the present anterior treatment and limited it to place only two implants in the lateral incisors' positions ().\nThe treatment was carried out in a staged approach. Briefly, first, we extracted the lateral incisors, using the SST, and placed two immediate implants. The four residual teeth were then prepared to be used as abutments of a temporary bridge for the purpose of maintaining the aesthetics and function of the patient during the early osseointegration period. In a further step, the four remaining teeth were also extracted using the SST, and the initial provisional bridge was replaced by the second provisional screwed on the uncovered implants. Only one out of the four abutment teeth used for the temporization of root canal treatment was needed due to a periapical infection.\nWhen placing the two immediate implants into the alveolus of the lateral incisors, a section of the buccal part of the root (about the two middle thirds) was left in place and no biomaterial was used at all. An impression of the implants was taken to have the second temporary bridge available in the second surgery. Healing abutments were then attached with the proper height for the soft tissue to cover them but at the same time facilitating the uncovering. Finally, a temporary acrylic bridge was cemented onto the four abutment teeth 13-11 and 21-23 ().\nThree months later, the implants were uncovered, the four abutment teeth were extracted, again with the SST—partial extraction of the roots—but this time no more implants were placed in these sockets. The first provisional cemented onto the teeth was then replaced by a second acrylic bridge screwed onto the implants though temporary abutments ().\nThe partial extraction of the canines, aiming to leave a buccal slice of the root, was so hard to perform, and further instrumentation would lead to the socket destruction that a decision was intraoperatively made and a greater portion of the root, including the apex, was finally left. As the locations of the canines did not involve the implant sites, any potential complication could be addressed efficiently.\nOne month later, the prosthodontic phase was undertaken. Little if any differences in the buccal tissue volume and no noticeable aesthetic impact could be found after the multiple extractions (). The desired position of the incisal border was determined by various try-ins, and five months after implant surgery, the definitive prosthesis was placed. The final clinical aspect can be appreciated in the pictures (Figures and ).
A 28-year-old male patient reported to the department of oral and maxillofacial surgery at people's dental academy complaining of asymmetry of the face and scar in the mandibular angle region on the left side since 3 years. The patient gave a history that he had tripped and fallen down 3 years back and he had a laceration on the left side of the face that developed in to a scar. The patient's chief complaint was left side facial growth without pain. The patient had no history of systemic diseases. Extraoral examination showed an obvious unilateral swelling centered over the mandibular angle. Palpation indicated that the swollen tissue was normal in tone and nontender. Mandibular movements were in the normal range. When the patient was asked to clench, the swelling became more prominent and firm. The patient said that he uses the left side of the jaw more while chewing food. There was no history of temporomandibular joint clicking, and no family history of masseter hypertrophy. Physical examination revealed that the patient had unilateral masseter muscle bulging, with a prominent mandibular angle at the lower border. Intraoral examination revealed distoangularly impacted 38 and 48. OPG showed a prominent mandibular angle. Data from clinical and radiographic examination led to the diagnosis of unilateral masseter muscle hypertrophy (Figures , , , , , and ). Nonsurgical options such as botox therapy and the advantages and disadvantages of both surgery and botox treatment were discussed with the patient. The patient opted for surgical option as he wanted to get rid of the scar immediately and we told him that we can do the correction of masseter hypertrophy and scar revision through one incisional approach only. A combined reduction of the mandibular angle and shaving of the masseter muscle was planned. The surgery was done under general anesthesia with nasotracheal intubation. Xylocaine 2% with adrenalin was infiltrated in the angle of the mandible. An elliptical incision was placed around the hypertrophic scar and the scar was removed (). The marginal mandibular nerve was identified and protected. Debulking of the masseter muscle was performed as the patient was very worried about the asymmetry of the face (). The muscle was incised approximately 5 mm above the mandibular basilar. The entire ascending portion of the masseter muscle was detached, and a vertical internal muscle band equivalent to two third of the thickness of the muscle was resected. After the muscle was resected, the remaining external third was sutured to its site of origin onto the muscle stump inserted in the mandibular basilar. The bony deformity was trimmed and removed in the angle of the mandible with surgical bur (). Sharp margins were trimmed with a bone file. The shaved masseter muscle and the resected excess mandibular angle was sent to oral pathology department in 10% formalin (Figures and ). Primary closure was done with 5-0 prolene suture (). After 1 week, the prolene sutures were removed and the wound healed uneventfully.
A 40-yrs-old male came to the orthopaedic OPD holding his left arm with the right one and the arm and shoulder were covered with a cloth. When the cloth was removed, there showed a shockingly huge mass in the left arm and shoulder with veins prominent all over it and a small wound over its posterior aspect. The tumour was pressing into the chest wall on the left side, but without causing any dyspnoea. Vascular status was normal distal to the tumour mass, but there was complete motor deficit with no power in muscles on forearm and hand. On taking history, it was found that, patient had come 3 yrs. Back with a small swelling in left upper arm and was admitted for the excision of the mass, but the patient absconded due to fear of surgery and started receiving treatment from some bone setters. Present situation developed over a period of 3 years. Patent came back to the hospital as he could no longer manage with the tumor due to the large size and weight of the growth and also due to social queries directed at his condition.\nOn measurement, tumour was 32 cms long, 28Cms wide and the circumference was 87Cms (). X-Ray of the area showed complete destruction of upper half of the humerus with central calcification of the tumour mass (). CT Angiography showed extensive destruction of the osseous tissue with calcification and there appeared involvement of lateral aspect of scapula also (). Blood flow in major vessels was normal without undue hyper vascularity in the tumour area & the tumour mass was pushing the thoracic cage inwards. A radiographic skeletal survey and bone scan did not reveal any other lesion. Biopsy of the tumour mass confirmed the diagnosis of Chondrosarcoma. It was decided to get rid of the bulky mass from the body, so as to make the patient comfortable and to clear the surrounding area of any suspected tumour mass. Forequarter amputation with adequate margins was performed.\nSurgical Procedure (): The patient was placed in a right lateral decubitus position to expose the thorax with the left upper extremity free. Anterior approach was used. An elliptical incision was made with the superior apex over the clavicle on one end and inferior apex was made at the mid axillary line and continued posterio superiorly over the scapula to join the superior apex. The clavicle was divided at the proximal third and carefully elevated to expose the subclavian artery, vein and brachial plexus (,). The subclavian artery was ligated and cut first, then the subclavian vein was tied. Branches of the brachial plexus were divided proximally. Next, the chest wall attachments of the pectoralis major and minor were divided. Posteriorly, fasciocutaneous skin flaps were developed and all periscapular muscles were released (). This allowed the shoulder girdle to be freed and the whole extremity to fall away from the trunk along with the scapula (). The anterior and posterior skin flaps were used to close the defect primarily over a corrugated drain ). Cautery was used extensively for cutting and patient required two blood transfusions. Removed limb weighed 16 Kgs along with the tumor () At three years follow up the patient had no complains and had rejoined his work at a government office ()
A 59-year-old woman had a minor fall with injury to the right leg. She was diagnosed as having stress fracture of the proximal tibia [] and a plaster cast was applied above the knee at a different institution. She was evaluated 1 month later with no evidence of union. The patient underwent surgery in the form of dual plating []. On the 3rd day postoperative, compartment syndrome was diagnosed. During fasicotomy, necrosis of the entire anterior compartment was noted and tibialis anterior and a part of the peroneus musculature were debrided and excised. The fasicotomy wound got infected with the spread of infection to the knee joint, which drained through a large wound on the medial side. Wound cleaning and repeated debridements were performed for 2 weeks with parenteral antibiotics with no improvement in the status of the wound. Finally, the patient was advised amputation. The patient did not consent for amputation and came to our center for further management. At the time of presentation, the wound was covered with necrotic tissue and slough and middle 4/5 of the tibia bone exposed with blackening at the lower end []. Active pus was discharging from the knee joint through the medial wound []. The knee joint had painful movements of only 10°–30°, whereas the ankle had 20° of equinus deformity. The patient was a known case of hypertension and was on medication since last 8 years. There was no history of diabetes or any other medical or surgical illness. Biochemical investigations showed hemoglobin of 10.2 mg% with rest of the parameters within normal limits. A swab from the wound cultured Staphylococcus aureus. The patient was taken in for the first debridement. Three holes were drilled in the tibia to verify bone viability. All the 3 sites had active bleeding from the bone. A thorough debridement of the wound was done with removal of all the dead and necrotic tissue. Postsurgery the entire lower leg was wetted with sterile distilled water and covered with a sterile plastic bag, which has outlet and inlet valves. First, air was sucked out of the bag and then ozone mixture at a dose of 70 μg was infused into the bag till the bag was full with the gas. After the entire dose was infused, the ozone machine was switched off and the bag was left in place for the next 30 min []. These sittings were given 2 times a day. Antibiotics in the form of ceftriaxone 1 g and amikacin 500 mg twice daily and metronidazole 100 mL thrice daily were given. Autohemotransfusion of ozone was done once daily by taking out 50 cc of the patient's blood and ozonizing it with 70 μg of ozone and then re-injecting it into the patient. Routine wound cleaning and dressing were performed daily. This protocol was followed for the next 4 days. On day 5, the wound was granulating and red with minimal necrosed tissue; however, the knee wound was still having some slough. A decision to provide biological cover to the exposed tibia bone was taken and a split thickness skin grafting was done on 5th day postadmission []. Postoperatively, further 5 sittings of local ozone were given till the knee wound became healthy and started granulating at the base. The tibia skin graft healed well with no signs of graft rejection. At this time, the knee wound had healthy granulation tissue. The skin graft was well accepted over the surrounding soft tissues, the graft over the bone and implant was removed on the 15th day from admission and the tibia was fixed with intramedullary interlocked nail and a latissimus dorsi pedicle flap []. The flap was taken up well and the knee wound healed by secondary intention. Knee and ankle mobilization were started on the 3rd day postsurgery. The follow-up examination had the flap healing well and bone showing progressive signs of union. No further reactivation of infection was noted. The bone healed in 4 months with the patient starting to walk toe-touch weight bearing by 2 months, partial weight bearing 4 months, and full weight bearing 6 months. At 20 months followup the patient is walking full weight bearing and using an anti–foot-drop splint. The knee has a range of movement of 10°–100° []. The patient is able to perform her daily activities and is able to walk 200 m distance at a stretch.
We were provided with a right kidney from a 55-year-old male donation after brain death donor who had died as a result of an intracerebral haemorrhage. The kidney had a single renal artery with aortic patch and a single ureter but there were dual renal veins. The main hilar renal vein had a diameter of 12 mm, the lower polar vein had a diameter of 8 mm and the veins were separated by a distance of 4 cm. The veins had been divided at the level of the inferior vena cava; a caval tube had not been provided.\nThe recipient was a 47-year-old female with a body mass index of 28.5 m2/kg and end-stage renal failure secondary to chronic pyelonephritis. The kidney was implanted into the right iliac fossa. The main hilar renal vein was anastomosed end to side to the external iliac vein using 5/0 polypropylene and the renal arterial patch was anastomosed end to side to the external iliac artery using 5/0 polypropylene. The lower polar vein was controlled with a fine atraumatic vascular clamp and not anastomosed initially. On release of the arterial and venous clamps, the kidney appeared globally well perfused and some urine production was observed. Nonetheless, both the main hilar and lower polar veins were distended and felt congested. On-table Doppler ultrasound was performed by members of the surgical team. This confirmed that the kidney was globally perfused but the waveforms showed reversal of flow in diastole throughout the kidney. The external iliac vein was clamped inferior to the upper renal vein anastomosis and at the inguinal ligament and the lower polar vein was then anastomosed end to side to the external iliac vein using 5/0 prolene (). On clamp release the kidney’s appearance improved immediately and both renal veins became soft and easily compressible. A repeat intraoperative Doppler ultrasound examination demonstrated excellent perfusion of the whole kidney and a triphasic waveform with forward flow in diastole. These findings were confirmed in the recovery room by an experienced radiologist. The cold ischaemic time was 12 h 18 min and the anastomosis time was initially 32 min with a further 10 min for the lower polar venous anastomosis. The kidney had initial function and at 12 month follow-up the recipient’s serum creatinine was 103 μmol/l.
A 25-year-old male patient was admitted to our department, presenting progressive pain and numbness with a rapidly growing mass located in the upper part of the right arm for 2 months in 2005. At the time of the hospital admission, he had completely lost his occupational capacity of the arm (100% unable to work). Physical examination revealed a palpable mass on the upper part of his right arm with a size 13 × 12 cm. Radiographic examination demonstrated a large mass over 10 cm in diameter on the proximal humerus, with massive cortical erosion, extension of the tumor into soft tissues and indistinctness of the surface of the tumor (). A diagnosis of chondrosarcoma was made in view of the radiologic findings in combination with the histology by the needle biopsy. The specimen was histologically classified as grade II chondrosarcoma. The patient had a 20-year history of a painless and palpable bony mass on this location and had gradually increased in size. He first sought treatment at age 16 because he had a distal radius fracture. At that period of time, the plain radiographs demonstrated multiple cartilaginous dysplasia or multiple enchondromatosis (Ollier’s disease) in the humerus with most evidently involving the upper part of the humerus (), and the lower part of the radius () of the right upper limb. Soft tissue of hemangiomas was not detected. The patient had no family history of skeletal disorders and no other medical history of note.\nA limb-salvage strategy with treatment of the large segmental defects following resection of the tumor and a prosthetic replacement of the proximal humerus was designed for the patient. The informed consent was obtained from the patient prior to the operation. The tumor resection prosthesis was applied in the same session following the tumor resection (). The tumor tissues were excised with maximum possible surgical safety margins. Frozen section biopsy was conducted during the surgery to ensure a safe surgical margin. Then the humeral head prosthesis (Waldemar LINK GmbH & Co., Germany) tailored for the patient was implanted, and the shaft preparation with cementing the prosthesis (, arrow indicated) was designed for supporting the reattachment of muscles and/or tendons to the prosthesis. This strategy was designed to get better active function, because inadequate reattachment of muscles and/or tendons to prosthesis cause considerable loss of function and loss of joint stability [, ]. No adjuvant chemotherapy and radiotherapy for the patient were given. Postoperative physiotherapy was applied to this patient. After surgery, the patient was to start active motion of the hand, wrist and elbow at 1 day even with mild tolerable pain. The patient could start to swing the shoulder joint at 4 weeks after surgery. Lifting and abduction of the shoulder joint were gradually started at 6 weeks after surgery. Early full weight bearing was started at 8 weeks postoperatively. Three months after surgery, the patient was almost 100% back to work and could perform all activities of daily life.\nDuring the 8-year follow-up, clinical and radiologic examinations were done at the periodic controls. There are no signs of local recurrence and/or remote metastasis so far. The therapeutic results have been satisfied with a good functional recovery of the treated limb, enabling the patient to return to the pre-disease daily living and occupational activities. The overall functional outcomes were assessed by the musculoskeletal tumor society (MSTS) scoring system. The patient had the overall score 24 in the last examinations of the follow-up, and the ranges of motion of the shoulder joint are shown in .
A girl aged seven years and eight months was referred to our observation because of left peripheral facial palsy, causing the inability to close the left eye and dropping of the corner of the mouth. A first peripheral facial nerve palsy occurred when she was three years and one month old, with complete regression after corticosteroid treatment. At the age of three years and nine months, she was diagnosed with pure red cell hypoplasia, manifested as severe anaemia (haemoglobin: 3.00 g/dL; red blood cells: 1,000,000/mm3) with an extreme lack of erythroid precursors in the bone marrow, but high growth of them in culture, probably caused by anti-EPO antibodies. The detection of anti-EPO antibodies, however, is not routinely performed in a clinical setting. The autoimmune hypothesis was postulated on empirical bases, since haemoglobin levels did not increase after recombinant human EPO administration, but normalized after corticosteroid therapy, and the addition of autologous serum to the erythroid precursor culture inhibited EPO growth. When she was 4 years and 4 months old, the patient presented with a second episode of left facial palsy, combined with the acute onset of a strength deficit on the left side of the body. Mingazzini I and II were positive for the left limbs. The imaging assessment showed a haemorrhagic stroke corresponding to the anterior portion of the right putamen and of the external capsule with perilesional oedema, involving the anterior limb of the internal capsule. Blood pressure measurements performed during the hospitalisation revealed high diastolic blood pressure values. These findings suggest a central rather than peripheral involvement of the facial nerve. Three weeks after their beginning, the symptoms had completely regressed. At the age of five years and six months, a third episode of left peripheral facial palsy occurred. Brain magnetic resonance imaging (MRI) was repeated, showing gliotic evolution of the previous haemorrhagic insult without new lesions. The patient was treated with corticosteroids, with a good regression of symptoms. On the last episode, the patient had initially visited a first level emergency room, where laboratory tests, as well as ophthalmologic, neurologic, and otoscopic examinations and a head computed tomography (CT) scan performed were normal. When admitted to our department, the neurologic examination showed complete peripheral left facial palsy (House–Brackmann grade V). Physical examination showed the presence of a furrowed tongue as a synchronous anomaly. No active herpetic mucosal and skin lesions were found. The patient was started on a tapering dose of prednisone for 30 days and vitamin B group supplementation was added. The clinical course was favourable. Three months after, at last follow up, neurological impairment had clearly improved. Facial palsy gradually resolved after the third week of treatment.
A 44-year old male was referred following investigation for chest pain and dyspnoea. He had no pre-existing co-morbidities. Physical examination revealed feeble femoral pulses and he was found to be hypertensive with marked differences between the upper and lower limbs (systolic blood pressure upper limb 190mmmHg, lower limb 75 mmHg, with an ankle brachial index (ABI) of 0.39). Electrocardiogram revealed evidence of severe left ventricular hypertrophy. This was confirmed with echocardiography which also demonstrated a tricuspid aortic valve with significant aortic regurgitation in the presence of an aortic root aneurysm of approximately 9 cm. Left ventricular function was preserved. Computerised tomography angiography (CTA) was performed to evaluate the aortic pathology in further detail (Fig. ). The scan noted an aortic root aneurysm (8.8 cm), in addition to the presence of severe aortic coarctation, with subtotal occlusion and a lumen less than 6 mm in size. The coarctation was just distal to the left subclavian artery, at the aortic isthmus. There was clear evidence of collateral circulation to the descending thoracic aorta via the subclavian and intercostal arteries. Coronary angiography confirmed a right dominant coronary system with no significant coronary disease.\nA multidisciplinary team meeting took place and a consensus was agreed to proceed with a two staged hybrid approach, with the first phase involving an endovascular approach to stent the coarctation, followed by a second stage to perform the surgical repair of the aortic root aneurysm. The first stage to stent the coarctation was unsuccessful via the femoral approach, as the guidewire could not cross the coarctation. Assessment through angiography via the left brachial artery showed complete obstruction at the aortic isthmus. The decision was then made to proceed to a single stage surgical approach to treat both lesions.\nAfter induction of anaesthesia, arterial lines were placed in the left radial and left femoral artery. A right infraclavicular incision and a right groin incision was made this was to establish peripheral arterial cannulation access to the right axillary and right femoral artery. An 8 mm dacron graft was anastomosed to each vessel for indirect cannulation. Median sternotomy was performed to access the mediastinum and expose the heart and aorta. Following heparinisation cardiopulmonary bypass (CPB) was established with venous return from bi-caval cannulation. The body temperature was cooled to 25 degrees Celsius. The right superior pulmonary vein was used for venting. Once the cross clamp was applied, complete cardiac arrest was achieved using Custodiol 25 ml/kg crystalloid cardioplegia via a retrograde cannula through the coronary sinus. A further top up of cardioplegia was given once the aorta was opened through direct cannulation of the coronary ostia.\nThe aortic root, valve and ascending aorta were excised. The coronary ostia were fashioned as buttons from the native aortic root. The coronary ostia were noted to be significantly displaced, with distorted anatomy due to the patient’s disease process. Therefore, 8 mm dacron grafts were attached end-to-end to each ostia, with view to performing the modified Cabrol technique later following replacement of the root. The heart was then retracted in a cephalad position to access the posterior pericardium. A vertical incision was made to expose the descending thoracic aorta (DTA). An end to side anastomosis was formed with a 20 mm dacron graft to the DTA (Fig. ). This graft was then routed posterior to the inferior vena cava (IVC) and anterior to the right inferior pulmonary vein (RIPV), adjacent to the right atrium (RA). Root replacement was then performed with a 25 mm biological valved-conduit, as this was favoured by the patient over a mechanical prosthesis, despite the risk of a difficult redo procedure in the future. The 8 mm dacron grafts attached to the coronary ostia were anastmosed to the root conduit as neo coronary ostia. The distal part of the valved-conduit was anastomosed to the proximal arch under selective antegrade cerebral perfusion (SACP). Finally, an end to side anastomosis was fashioned between the 20 mm extra-cardiac graft (attached to the descending thoracic aorta) and the ascending portion of the valved-conduit. Valve-sparing root replacement was not considered in this patient due to the grossly abnormal aortic anatomy.\nFollowing rewarming and deairing the patient was successfully weaned off CPB. The bypass time was 160 min, the cross-clamp time was 120 min, and the SACP time was 40 min. Haemostasis was achieved and thereafter a routine closure of all incision sites. The patient remained in ICU for less than 48 h, and made excellent progress on the ward. Minimal anti-hypertensives were required and the patient was discharged on 8th day post operatively neurologically intact and independent. At 3 months follow up the patient underwent a repeat CTA scan which showed complete patency in the extra-anatomical graft and resolution of the collateral arterial network (Fig. ).
Case 1 – A 20-year-old male patient with a history of gunshot injury to his face happened a week ago was admitted to our clinic in March 2017 with the complaint of restricted mouth opening. On examination, vital signs were stable, left maxillary, the infraorbital lesion on the maxillary bone, consistent with the bullet entry hole, and a skin burn measuring 1x1 cm were observed. Mouth opening was restricted and widespread tenderness and oedema were detected by palpation on the left temporomandibular joint and left maxillary region. The functions of the facial nerve of the patient were intact. The patient’s brain and maxillofacial tomography showed multiple segmental fracture lines in the anterior and lateral walls of the maxillary sinus, in the zygomatic process, in the mandible and the pterygoid process.\nDiffuse air images and areas of hematoma were observed in the adjacent soft tissue. At the same time, a metallic foreign body image with a diameter of 1 cm was seen in the subcutaneous fatty tissues in the posterior neighborhood of the mandible on the left side (). The patient was evaluated by neurosurgery for cranial pathologies, and any pathological condition was not observed. To evaluate the patient as for orbital pathologies, consultation from the Department of Ophthalmology was requested and left orbital floor fracture and minimal bleeding in the left intraorbital region were observed by the consultant ophthalmologist, and application of cold compresses and conservative approach were recommended. To remove the foreign body in the left parotid region, the patient underwent exploration of the left parotid region under general anesthesia. The foreign body was palpated in parotid tissue after application of modified Blair incision.\nAfter identification of the main truncus of the facial nerve, its branches were followed and preserved. The bullet was not associated with the facial nerve, and it was confined to the superficial lobe (). After the bullet was removed from the superficial lobe, the drain was placed, and any perioperative complications were not observed. In the postoperative period, facial nerve functions were intact and the patient was discharged on the 4th day.
This is the case of a fifty-year-old man who was hospitalized after being involved in a car accident in which he reported a concussive head trauma. Except for the trauma he did not have a significant history of illness. The patient was subjected to a brain computed tomography (CT) scan which showed the presence of a combined right supra-subtentorial hematoma (Figure ). Clinical evaluation of the patient did not reveal any neurological signs except for a mild headache. Because of the site of the hematoma, an involvement of the transverse sinus was suspected. As a consequence a brain magnetic resonance imaging (MRI) with arterial and venous reconstruction was performed. The MRI confirmed the presence of the hematoma involving the supratentorial and the subtentorial compartment. Furthermore the venous study did not show any appreciable signal of blood flow within the right transverse sinus. This radiological finding was likely to be due to the occlusion of the sinus (Figures and ). Because of the absence of neurological signs as well as the patency of the contralateral dural sinuses system, a conservative management was adopted. A CT scan performed 48 h after the accident showed a slight increase in the size of the hematoma (Figure ). As a consequence, administration of LMWH was delayed. By the 10th post-traumatic day two more brain CT scan had been performed which had shown the progressive decrease in the size of the hematoma (Figure ). This reduction encouraged us to begin the administration of LMWH. On 15th post-traumatic day the patient was discharged at home. During the subsequent 23 d the patient did not experience any symptoms related to the trauma. On 24th post-traumatic day, he began to complain of mild headache, vertigo and nausea. Since the symptoms were not responsive to oral analgesics and antiemetic drugs, the patient came back to the Emergency Department of our Hospital. A new brain CT scan was performed. It showed a further reduction of the size of both hematomas. Given the clinical history, a new brain MRIs can with venous angiographic reconstruction was performed. The new MRI confirmed the further decrease in the size of the epidural hematomas. Angiographic reconstructions of the dural sinuses showed that, although characterized by a less intensity in comparison with the contralateral sinus, the blood flow signal within the previously occluded transverse sinus was now visible. These radiological findings were likely to be due to the partial recanalization of the sinus (Figure ). Symptoms progressively disappeared and after a brief period of hospitalization the patient was discharged at home.
A 76-year-old male patient came to our hospital with complaints of pain, swelling, and difficulty in flexing and weight-bearing in the left knee. The patient had a history of fall from bike 15 days back and took treatment at the local hospital for the same in the form of above knee slab and was referred to our hospital. There was no history of any head, chest, and abdominal injury and the patient was not a known case of hypertension and diabetes. The patient had a history of coronary artery bypass graft surgery 6 years back and is on tablet Ecosprin since then. On local examination, tenderness was present over the lateral tibial condyle, skin condition was good, and range of motion was restricted. X-ray of the left knee was taken, which was suggestive of the left tibia lateral condyle split fracture (Schatzker type 1) with severe osteoarthritis of the left knee ( and ). The patient was managed initially with above knee slab and limb elevation. As the patient was having osteoarthritis with fracture, we planned for primary TKR with stemmed tibial component. Surgery was postponed for 15 days because of patient medical fitness issues.\nAfter getting the fitness for surgery (40 days after the trauma), the patient was operated in supine position through medial parapatellar approach using tourniquet. After the femur preparation, tibial preparation was started with removal of fibrous tissue at the fracture site, anatomical reduction of fracture, and maintaining the reduction with reduction forceps till the completion of the tibial preparation to prevent the displacement of fracture fragment. Cemented TKR was done using Zimmer implant of femoral component size E, tibial component of size 4 with stem extension of 12 mm diameter × 155 mm length and articular surface height of 10 mm ( and ). As the fracture was properly reduced to anatomical position and there was only fracture split, no depression, the augmentation was not required. Wound was closed in layers and the patient was given long knee brace postoperatively. Partial weight-bearing and knee range of motion were started after 3 days and full weight-bearing was started after 3 weeks. Eight months post-operative X-rays ( and ) showed healing of lateral condyle fracture and without any loosening of implants. The patient is having good knee range of movements and no pain on weight-bearing at present ( and ).\nAt present 1.2 years follow-up, the patient is comfortable with no pain and difficulty in walking. Functional outcome is good as per Oxford knee score. Shorter follow-up period is the limitation of this case report.
Our patient is a 27-year old female that denied any past medical and surgical history. Her family and drug history were also unremarkable. The patient presented with a 7-month history of progressively enlarging mass on her left ring finger. The patient mentioned that the mass appeared suddenly with no history of trauma and that she was concerned about the potential malignant nature of the mass. The patient also mentioned that she underwent incision and drainage of that mass 2 months after its appearance with no improvement and provided no detailed surgical or pathological reports, which was the reason for her delayed presentation. Upon her assessment, the mass was located over the ulnar side of the proximal phalanx of left ring finger with extensive involvement of the 4th web space. The overlying skin coverage was ulcerative with no active signs of infection. Range of motion of the involved digit was limited, however neurovascular examination was normal. ().\nRadiological evaluation of the involved hand showed a soft tissue swelling with no evidence of bone involvement (). Further magnetic resonant (MRI) evaluation showed a mass on the volar aspect of the ring finger encasing about 50% of the flexor tendons of that digit with low signal intensity on T1 and high signal intensity on T2 evaluation with strong enhancement in post contrast evaluation. Assessment of neurovascular structures showed partial abutment of the radial sided bundle together with complete encirclement of the ulnar sided neurovascular bundle. The surrounding bone was free of any masses and associated mass effect.\nThe patient was taken to the OR for exploration and mass excision by the senior author. Possible risks associated with such intervention were explained. Intra-operatively, bruner type incision was designed together with island of skin involved in the mass. Exploration revealed extensive subcutaneous mass with fibro-fatty consistency with extensive fascia like extension to the surrounding soft tissue. The mass was encircling the ulnar neurovascular bundle with mass abutment over the radial bundle as seen in pre-op assessment. The mass was dissected freely from its attachment to those bundles preserving both radial and ulnar structures. The mass was then excised en-bloc having a dimension of 3.5 × 4x2.5 cm (). Histological assessment showed a lesion with fasciitis like features, myofibroblastic proliferation and scattered foci of osteoid formation that was positive for Alpha-Smooth Muscle Actin (ASMA 1A4) immune staining and no evidence of malignancy (). The resected margins were however, positive for residual lesion with difficulty in obtaining negative margins due to the extensive nature of the mass. Post-operatively, the patient had an un-eventual course. She was informed about the need for close follow-ups for both clinical and/or radiological signs of lesion recurrence, pending early surgical intervention (see ).
A 53-year-old woman was referred to the department of ophthalmology with a palpable mass at the left medial canthal region. She complained of a six month history of tearing and a two month history of occasional bloody discharge from the left eye as well as left medial canthal region swelling. The swelling was not associated with pain or visual disturbance. The patient had no previous history of regional surgery or trauma or excessive sun exposure. There was no history of a cutaneous primary melanoma.\nThe physical examination revealed a cystic, localized, non tender palpable mass at the left medial canthus consistent with an enlargement of the lacrimal sac. Computed tomography scanning of the paranasal sinus showed a soft tissue mass in the left lacrimal sac, attached to the medial orbital wall (). However, neither orbital cavity involvement nor bony destruction was detected. In addition, infiltration of the surrounding soft tissue was not detected, and the mass did not extend into the nasolacrimal duct. There was no associated cervical lymphadenopathy. The patient was taken to the operating room for a scheduled dacryocystectomy. A brown-pigmented soft tissue containing cystic mass filled the lacrimal sac and did not protrude through the nasolacrimal duct. The mass was removed from the nasal and lacrimal bone and dissected from the medial canthal tendon. The cystic mass was thought to be benign and therefore only a dacryocystectomy was performed.\nBiopsy and histological examination of the surgical specimen showed solid growth of malignant cells characterized by production of melanin pigments and hyperchromatic nuclei with prominent nucleoli (). The tumor cells showed strong immunoreactivity to melanoma specific antibody, HBM-45 in the immunohistochemical examination, consistent with a diagnosis of malignant melanoma (); whereas the immunoreactivity to the S 100 protein was negative. This histological examination confirmed the diagnosis of malignant melanoma and the resection margins were not free of tumor. A complete metastatic workup including computed tomography scanning of the head and neck, chest, abdomen and pelvis failed to demonstrate lymphadenopathy or other evidence of metastasis. A general physical examination and routine laboratory tests were all within normal limits. The patient underwent 6000 rad of adjuvant irradiation therapy at the left lacrimal region for four weeks after surgery. During the two months follow up after surgery and radiation therapy, the patient had no evidence of local recurrence; however, since then she has been lost to further follow up.
A 36-year-old male, with a previous history of ACL reconstruction six years prior, was admitted due to painful swelling of the knee associated with recurring knee instability. A hamstring graft was used for subsequent ACL reconstruction, and it was performed outside our institution by a senior and experienced surgeon. Patients could walk normally in about 6 months, although heavy sports activity has not yet been achieved. The patient remained without symptoms for the next six years. He began to experience pain and swelling over his knee after a six-year period. There was no history of trauma. There was also stiffness of the knee. The patient had difficulty moving his knee. The patient had a previous history of hyperuricemia without symptoms and did not take medication routinely.\nThe physical examination revealed a painful, swollen knee without sinus discharge and limitations in knee flexion-extension that ranged from 30 to 80 degrees (). Special tests such as the Lachman test as well as the pivot shift test could not be done properly due to the limitation of the knee range of motion. In addition, the left contralateral knee shows similar manifestation clinically with tenderness, mild swelling, stiffness and limited range of motion.\nThe radiographic finding shows early arthritic changes in the knee. This kind of plain x-ray of the knee resembles a picture of the degenerative condition of joints (). Besides, the MRI shows effusion, and there is a suggested solid mass on the posterior part of the femoral notch with suspected arthrofibrosis. The previous graft is nowhere to be seen in . Laboratory results prior to the surgery revealed an increase in urate acid level in the serum of about 11.6 mg/dL. Synovial fluid analysis showed an increase in urate crystals as well as polymorphonuclear neutrophils (PMN) count ().\nAfter explanation and education regarding the problem, informed consent was taken and arthroscopic debridement was obtained and some tissue samples were obtained for pathological analysis. During the surgery, surprisingly, an abundant amount of gouty crystal was found almost on the entire surface of the joint cartilage and synovial tissue (). The ACL graft was no longer found and disappeared (). A tissue sample was obtained for pathologic anatomy, and revealed pieces of tissue with a needle-shaped structure under a light microscope, respectively. Tophus was seen intraarticularly and numerous urate crystals came out from it. At the time, ACL revision surgery was not obtained with consideration of waiting until optimum condition.
This is a case of a 25 years old Malay girl with learning disability and no significant past medical history, who started noticing a sacral mass since August 2015. The mass was painless and gradually increasing in size. The family members of this patient brought her to a traditional healer. They did not seek any medical treatment until late 2017. By this time, the mass over the sacrum was extremely large. Family members claimed the mass was preventing the patient from lying down flat supine. The patient was also unable to ambulate for the past 2 years. Hence, she was bedbound most of the time. It was difficult for her to sit on the wheelchair. She also felt tired to move because the mass was quite heavy. The family members claimed when the patient was lying down flat, she had to flex her hips and knees to achieve a more comfortable position. In addition, she often slept either in prone position or in supine with multiple pillows below her body. The mother also claimed over the last 2 months, the patient’s body had been getting thinner despite her physical weight was increasing due to the increase in size of the sacral mass. The patient had been passing stool and urine in pampers. There was no past medical history and no family history of cancer. Socially, the patient lived with her mother and siblings. The mother was the main care taker. Her father passed away 10 years ago because of heart attack. The patient previously attended a special needs school, but she stopped going to school since 2015 after developing the sacral mass.\nThis patient was managed in the Southern Region referral centre for Orthopaedic Oncology in Malaysia. On clinical examination in the Orthopaedic Oncology ward, the patient appeared cachexic, she had slightly pale conjunctiva, but she was not dysmorphic. Vital signs were Blood Pressure 142/90, Pulse Rate 98 beats per minute and Temperature 37 degrees Celsius. There was a large mass 40 cm × 30 cm × 20 cm over the sacrum. The mass was firm to hard in consistency and involved both buttocks and the gluteal fold (Fig. ). Dilated veins were noted under the skin overlying the sacral mass. Neurological exam of bilateral lower limb was normal. However, there was generalized wasting of all muscles over the bilateral lower limb. Anal tone was intact.\nLaboratory investigations taken were unremarkable. Computed Tomography of the Pelvis showed a large destructive sacrococcygeal mass measuring 43 cm × 38 cm × 27 cm with extension into the presacral space resulting in anterior displacement of the rectum, urinary bladder and uterus and posterior extension into the dorsal soft tissue with involvement of the gluteus, piriformis, and left erector spinae muscles (Figs. and ). Superior margin of the sacral bone involvement was up to S2. The mass was predominantly of fluid density with internal enhancing septation and calcifications which suggested primary chordoma more likely (Figs. and ). Magnetic Resonance Imaging done showed similar findings. Skeletal Survey Radiograph did not show any distant metastasis. A Trucut biopsy of the mass was done. Histopathological analysis showed tumour cells with “physaliphorous cells” positive for pancytokeratin, EMA, Vimentin and S-100 immunohistochemistry stainings with minimal mitotic figures and mild nuclear pleomorphism (Fig. ). Brachyury immunohistochemistry staining was not available in our centre. However, the clinical history, morphology of tumour on microscopy and immunohistochemistry staining available were consistent sacral chordoma.\nThe diagnosis of Sacral Chordoma was confirmed. Multidisciplinary team discussion done among Orthopaedic Oncology, General Surgery, Obstetrics and Gynaecology, Blood Bank, Anaesthetic and Plastic Surgery teams. A family conference was done. The family’s aim was for removal of the sacral mass to allow the patient lie supine on bed and sit on the wheelchair.\nSubsequently, the patient undergone Wide Resection and En Bloc Sacrectomy. The Posterior-Only Approach was used with a “Mercedes Star” 3 limbed incision. Duration of surgery was 8 h. The patient was supported with blood products transfusion during surgery. Intraoperatively, the sacral tumour had eroded the sacral bone from S2 to S5 (Figs. , and ). Sacrectomy was done at the level of S2. Sacral nerve roots S2-S5 were all infiltrated by the mass and therefore were unable to be preserved. The mass and surrounding gluteal muscles invaded by the tumour were also all resected. All resection margins were less than 1 mm from the tumour. Primary closure was done without any distant or local flap as per consultation with Plastic Surgery team. The tumour weight was 25 kg (Figs. , and ). Post operatively, the patient was monitored in Intensive Care Unit for 3 days. The patient developed neurogenic bowel and bladder post sacrectomy requiring enema and long-term urinary catheter. In addition, the post-operative course was complicated by wound breakdown and surgical site infection requiring wound debridement. Dressing was done as per local protocol until wound bed granulating well. Split Skin Graft was done about 3 months post wide resection once the tissue culture results were free of significant infection.\nThe patient also required extensive rehabilitation for transfer, ambulation and bowel and bladder care. Rehabilitation was difficult because the patient had learning disability and she had been habitually keeping her hips and knees flexed because of the sacral tumour for the past 2 years. During the last review 5 months post operatively, patient was able to sit on the wheelchair comfortably. The surgical wound was healing well with good uptake of the Split Skin Graft (Fig. ).
A 56-year-old lady presented with a 6 month history of rectal bleeding, passage of mucus and a change in bowel habit to more frequent stools. She had no abdominal pains and her weight was maintained. The patient had been born with bladder exstrophy and had multiple surgeries culminating in a cystectomy with ileal conduit formation at 5 years of age. She had suffered with recurrent urinary tract infections for most of her childhood and adult life but was otherwise well with no other major co-morbidities or risk factors for colorectal malignancy and had no family history of colorectal disease.\nColonoscopy revealed two large sessile polyps in the sigmoid colon in close proximity to each other and adjacent to a diverticulum-like structure (Fig. ). Each polyp was approximately 3 cm in size and both exhibited a type IV pit pattern with areas of irregularity suggestive of focally advanced disease. Histological examination confirmed both polyps were adenomas comprising both low and high grade dysplasia, without submucosal invasion (Fig. ). On closer inspection the mucosa around the diverticulum was also atypical but not adenomatous. The remainder of the colonoscopy was unremarkable with no other evidence of diverticular disease or polyps elsewhere.\nEndoscopic resection was considered as a therapeutic option however in view of the above characteristics as well as difficult endoscopic access surgery was preferred. Furthermore, radiological imaging had initially raised the possibility of invasive disease in view of sigmoid thickening. The patient underwent high anterior resection and an open approach was chosen because of suspected intra-abdominal adhesions following extensive pelvic surgery. An end colostomy was formed at the patient’s pre-operative request. At laparotomy the right fallopian tube was adherent to the sigmoid colon and adjacent to this a blind ending tube was noted to emerge from the anti-mesenteric border of the colon. This was marked for pathological identification.\nOur patient went on to have an uneventful recovery and her quality of life following surgery was good. Her wish to have a permanent colostomy stemmed from the fact that she had always suffered from an erratic bowel habit and that she was already knowledgeable with regards to stoma care in view of her pre-existing ileal conduit.
A 40-year-old Caucasian lady of UK origin was seen in our Early Pregnancy Unit at 8 weeks gestation to confirm site of pregnancy as she had a previous ectopic pregnancy. This was a spontaneous conception with no previous fertility treatment. She did not use any contraception in the interim.\nThree years earlier she was diagnosed with a right tubal ectopic pregnancy which was treated by a laparoscopic salpingectomy. Prior to that she had two normal pregnancies and deliveries. She did not have any risk factors for an ectopic pregnancy at that time. The left fallopian tube looked normal at the time of the laparoscopy.\nShe was completely asymptomatic at the initial consultation. A transvaginal scan showed an irregular gestational sac of approximately 8 weeks with a yolk sac but no fetal pole (Figure ). There was a corpus luteal cyst noted in the left ovary with a small amount of free fluid in the Pouch of Douglas. The right ovary was normal with no obvious adnexal masses seen. A rescan was scheduled in 10 days time to check for viability of the pregnancy.\nHowever six days later the patient presented as an emergency with acute left sided abdomino-pelvic pain and generally feeling unwell. She had no vaginal bleeding. On examination she was cold, clammy and hypotensive. Abdominal examination was suggestive of an acute abdomen with severe tenderness, guarding and rigidity. Clinical differential diagnosis at that stage was a possible bleed from the corpus luteal cyst which was seen in the initial scan or an ectopic pregnancy.\nA transvaginal scan done by the same senior sonographer showed the intrauterine gestational sac similar to the previous scan few days earlier. In addition however there was a left adnexal mass with a gestational sac and a fetal pole suggestive of an ectopic pregnancy (Figure ). There was a faint fetal heart seen in the fetal pole. The amount of free fluid in the pelvis was significantly more as compared to her previous scan.\nShe was counseled and consented for an operative laparoscopy and ERPC (evacuation of retained products of conception).\nAt laparoscopy (Figure ) there was approximately a litre of hemoperitoneum. There was a 3-4 cm left tubal isthmic ectopic pregnancy seen. Both the ovaries appeared normal with an absent right fallopian tube.\nA total left salpingectomy (Figure ) was performed laparoscopically followed by and ERPC. A serum beta hCG taken just before surgery was 21846 iu.\nThe patient made an unremarkable recovery from the surgery and was discharged the following day. Both the tissue specimens were sent separately and the histology from each of the specimens confirmed chorionic villi suggestive of a heterotopic pregnancy.
A thirty-one-year old female patient reported to the Department of Oral and Maxillofacial Surgery with a swelling in the floor of the mouth for the past two months. The swelling was initially small and gradually reached the present size over two months. On extraoral examination, a diffuse swelling in the left submandibular region was present. On intraoral examination, a single well-defined encapsulated swelling was present in the left side of floor of the mouth (). On palpation, the swelling was tender, firm, and nonfluctuant and the swelling extends anteroposteriorly from the midline of the floor of the mouth to the second molar region in the left side of the mandible (). Patient was having altered sensations in the left lateral border of tongue and the lingual mucoperiosteum of the left side of mandible which suggested that the mass related to the lingual nerve. FNAC was found negative as the swelling was firm.\nAxial computed tomogram of head and neck showed a dense mass in the left side of floor of the mouth region measuring about 33 mm × 23 mm with a dilated lingual nerve found within the mass (Figures and ).\nUnder general anaesthesia, a mucosal incision was placed over the mass in the left side of the floor of the mouth, the tumor was exposed and dissected safely from the lingual nerve, and excision was carried out by ligating the lingual nerve on the distal and mesial end of the swelling (). The encapsulated tumor was then excised completely from the lingual nerve and the lingual nerve repair was done by perineural suturing. Macroscopic examination of the excised specimen reveals a well-encapsulated mass and the cut surface of the specimen measures about 3.5 cm × 4 cm × 3 cm showing an area of cystic degeneration and solid areas (Figures and ). Histopathological examination showed fibrous capsule enclosing proliferation of Schwann cells with characteristic Antoni A and Antoni B types of tissue with areas of cystic and myxoid degeneration, suggestive of schwannoma (Figures and ). Patient was then followed up postoperatively for six months without any recurrence and complications.
A girl aged seven years and eight months was referred to our observation because of left peripheral facial palsy, causing the inability to close the left eye and dropping of the corner of the mouth. A first peripheral facial nerve palsy occurred when she was three years and one month old, with complete regression after corticosteroid treatment. At the age of three years and nine months, she was diagnosed with pure red cell hypoplasia, manifested as severe anaemia (haemoglobin: 3.00 g/dL; red blood cells: 1,000,000/mm3) with an extreme lack of erythroid precursors in the bone marrow, but high growth of them in culture, probably caused by anti-EPO antibodies. The detection of anti-EPO antibodies, however, is not routinely performed in a clinical setting. The autoimmune hypothesis was postulated on empirical bases, since haemoglobin levels did not increase after recombinant human EPO administration, but normalized after corticosteroid therapy, and the addition of autologous serum to the erythroid precursor culture inhibited EPO growth. When she was 4 years and 4 months old, the patient presented with a second episode of left facial palsy, combined with the acute onset of a strength deficit on the left side of the body. Mingazzini I and II were positive for the left limbs. The imaging assessment showed a haemorrhagic stroke corresponding to the anterior portion of the right putamen and of the external capsule with perilesional oedema, involving the anterior limb of the internal capsule. Blood pressure measurements performed during the hospitalisation revealed high diastolic blood pressure values. These findings suggest a central rather than peripheral involvement of the facial nerve. Three weeks after their beginning, the symptoms had completely regressed. At the age of five years and six months, a third episode of left peripheral facial palsy occurred. Brain magnetic resonance imaging (MRI) was repeated, showing gliotic evolution of the previous haemorrhagic insult without new lesions. The patient was treated with corticosteroids, with a good regression of symptoms. On the last episode, the patient had initially visited a first level emergency room, where laboratory tests, as well as ophthalmologic, neurologic, and otoscopic examinations and a head computed tomography (CT) scan performed were normal. When admitted to our department, the neurologic examination showed complete peripheral left facial palsy (House–Brackmann grade V). Physical examination showed the presence of a furrowed tongue as a synchronous anomaly. No active herpetic mucosal and skin lesions were found. The patient was started on a tapering dose of prednisone for 30 days and vitamin B group supplementation was added. The clinical course was favourable. Three months after, at last follow up, neurological impairment had clearly improved. Facial palsy gradually resolved after the third week of treatment.
A six and half year old boy referred to us from another center had complaints of an insidious onset of pain in his right forearm, which initially was less intense. The parents ignored it initially thinking it might be due to his excessive playful nature and cycling. They had a delay of three and half months to consult a doctor in their town when the child complained of an increasing pain. In the meantime, there appeared some swelling in his forearm, which was gradually progressing. A X-ray was done () which revealed an expansile lytic lesion in the proximal half of the radius. He was initially kept under observation and was then referred to a higher center. A repeat X-ray was done, which revealed an expansile lytic lesion involving the proximal half of the radius. On comparing the previous X-rays (), it was seen that the lesion had significantly increased in size with thinning of the cortex rendering it difficult to decide the treatment options. As per the protocol, an MRI was done (–) which revealed low to intermediate signal intensity with a rim of low signal intensity with internal septa. A bone biopsy was done which after radiological correlation was confirmed to be a case of aneurysmal bone cyst.\nThe tumor was expansile with papery thin cortices and hence was not amenable to curettage. It was decided to do wide resection of the tumor. Reconstruction of the radius following tumor resection can be done with different techniques. First to use child’s own fibula may be considered as vascularized or nonvascularized pattern. Second, the cadaveric allograft. Third, an allogenic allograft from the mother. The parents were informed regarding all the options for reconstructing the radius following excision of the tumor. We were a bit concerned with regard to the diameter of child’s own fibula to be used with the radius because of the possible size mismatch as well as the surgical trauma at two sites. The parents were reluctant to use the allograft stored in the bone bank and hence opted for the mother’s fibula. A written consent was taken from both the parents. Two operating tables were used with two surgical teams. The mother’s surgery was commenced after exposing the tumor in the child and establishing length. Both the surgeries were conducted under tourniquet to reduce blood loss.\nThe radius was exposed using the Henry’s approach with around 15 cm long longitudinal incision along the line joining the lateral side of biceps tendon to the radial styloid process. After incising the deep fascia, a plan was developed between the brachioradialis and flexor carpi radialis. Proximally a plane was developed between the pronator teres and brachioradialis. The superficial radial nerve, radial artery, and poster interosseous nerve all were secured. Periosteum was elevated and was tagged. The exposed tumor length was found to be 11 cm long. The tumor was completely excised ().\nThe mother was screened before the surgery and was found to be free of any diseases. It was decided to harvest 12 cm of fibula. A linear incision was made just posterior to the fibula. A plane was developed between the peroneal and the soleus muscles. The muscles as well as the interosseous membrane attached to the fibula were stripped off. The required length of the fibula was taken out (). The harvested fibula was cleared of the medullary contents. The graft was fashioned to fit into the bone gap and small cuts were made over the fibular cortex to facilitate the ingrowth (). It was fixed with an intramedullary K-wire (). The periosteal covering was sutured back over the fibular graft. No cancellous graft was used in the procedure. The limb was kept in the above elbow Plaster of Paris (POP) slab in immediate postoperative period (). The patient was followed up first at 2 weeks for suture removal. Twelve weeks later following the surgery, there were signs of incorporation of the graft () but some resorption of bone was noticed along the shaft of graft. The patient was started on weekly dose of bisphosphonate therapy (35 mg of alendronate). The cast was removed and a forearm brace was applied. Mobilization of the elbow and wrist was started along with strengthening of the forearm muscles. The roentgenograph at six months () showed significant decrease in bone resorption. The K-wire was removed and the brace discontinued. The bisphosphonate was continued for further three months and stopped. At the latest follow-up of two years (), the graft was found to be fully incorporated and the child regained full range of motion and strength of elbow.
A diabetic 90 years old woman with cough and diffuse abdominal pain was admitted into our hospital with an initial diagnosis of sepsis. Blood sample investigation revealed mild leucocytosis with PRC augmentation and normal AST, ALT and bilirubin levels. Preoperative ultrasound revealed thickened gallbladder wall suggestive of acute cholecystitis. She has had previous sigmoid resection for diverticulitis and ERCP for choledocholithiasis without any mention of biliary abnormality. She was scheduled for a laparoscopic cholecystectomy during which the incidental intraoperative finding of a left sided gallbladder was made. Standard trocar positions were used (A) without any variation of technique, simply following the Strasberg criteria for safe cholecystectomy []. Having seen the anatomical variation (B), a careful dissection of Calot’s triangle was carried out, the cystic duct and artery were identified and isolated, and subsequent complete dissection of the gallbladder from the liver was done (C). This manoeuvre allowed us to demonstrate a right sided positioning of the cystic duct. Furthermore, a safe clipping and transection of the cystic duct and artery was achieved (D), and the gallbladder was extracted in an endobag. The postoperative course was complicated by an aggravation of diabetes and basal pneumonia which were controlled postoperatively in the intensive unit. The patient was discharged on postoperative day 16, in good condition. Retrospective analysis of the clinical case was done and a previous CT scan was reevaluated. Even with the knowledge of a left sided gallbladder, the CT scan was unable to demonstrate its attachment to the left side of the liver. This was probably due to the presence of a hypertrophic left lobe mimicking only a contiguity with the gallbladder, which itself appeared to be in a physiological position (). A vascular and biliary reconstruction was then evaluated with evidence of agenesis of the right anterior sector of the liver () with the absence of the right hepatic duct and an intrahepatic variation with insertion of the right inferior sectorial duct into the left common duct () as type A3 of Huang Classification [].
A 57 year old White British woman with CKD stage five was referred to the regional joint renal genetics clinic after her sister was noted to have a family history of renal disease during work up as a potential live kidney donor. The patient had been under nephrological follow up since adolescence, when she was incidentally discovered to have mild renal impairment (Fig. ). An intravenous pyelogram performed at the time showed that both kidneys were small with decreased cortical thickness and the most likely diagnosis was felt to be chronic pyelonephritis. Ultrasound scanning confirmed small kidneys with no overt renal cysts observed; urinalysis was consistently bland. She had also been investigated for symptoms of marked tiredness as a teenager and was found to be anaemic with a haemoglobin value of approximately 90 g/L. This was felt to be out of keeping with her level of kidney function at the time and remained unexplained despite extensive investigation. She developed gout in her twenties and her serum uric acid level was measured at 550 μmol/L; treatment with allopurinol was initiated. She remained under renal follow up and her kidney function continued to slowly deteriorate. Her haemoglobin level remained stable at around 100 g/L after initial treatment with iron and later an erythropoiesis-stimulating agent (ESA). When she was aged 37 years, one of the nephrology team noted that her mother had recently commenced haemodialysis. As both mother and daughter had presented with slowly progressive CKD, bland urinary sediment and small kidneys on renal imaging, the most likely diagnosis was now felt to be ADTKD.\nA detailed family history revealed that the patient’s maternal grandmother had been told she had small kidneys but no further clinical details were available (Fig. ). The patient’s mother was identified as having mild CKD and hypertension at the age of 65 years during a routine health screen; renal ultrasound scanning at the time showed two small kidneys. Her renal function continued to decline and she commenced haemodialysis at the age of 70 years, before passing away 5 years later due to infective endocarditis. The patient’s siblings were all reported as being healthy.\nBy the time the patient was reviewed in the renal genetics clinic, her estimated glomerular filtration rate had fallen to 12 and she was hoping to undergo imminent pre-emptive live renal transplantation with her sister as the donor. Given her clinical diagnosis of ADTKD, hyperuricaemia and history of young-onset anaemia, urgent next generation sequencing was performed using the Illumina TruSight One sequencing kit and the data was analysed to search for pathogenic variants in HNF1B (NM_000458.2), REN (NM_000537.3) and UMOD (NM_003361.2). The full coding regions (+/−five base pairs) of all three genes were sequenced to a depth of 20 fold or more, with analytical sensitivity of 98.3%–100% (95% confidence intervals). This identified a heterozygous and likely pathogenic variant in the REN gene: c.49 T > C, p.(Trp17Arg); no abnormalities were detected in HNF1B or UMOD (Fig. ). Although this sequence change has not previously been reported in the published literature, missense pathogenic mutations in the same region of REN have been described and associated with a similar phenotype. [, , ] The c.49 T > C variant alters a highly conserved nucleotide and weakly conserved amino acid in the aspartic peptidase domain of renin. Although in silico analyses are inconsistent and classify the variant from tolerated to possibly damaging, it has not been reported in the Exome Aggregation Consortium, Exome Sequencing Project or 1000 Genomes Project and is therefore very rare. [–] Functional studies have shown that a mutation in the adjacent amino acid, p.(Leu16Arg), prevents prorenin and renin biosynthesis and secretion. [] Therefore, it was considered highly likely that the REN: c.49 T > C, p.(Trp17Arg) variant was the cause of disease in this patient. Unpublished observations presented by Vincent Morinière and colleagues at the 43rd Annual Meeting and Scientific Exposition of the American Society of Nephrology in 2010 include discovery of the same p.(Trp17Arg) REN mutation in an individual with chronic tubulointerstitial nephritis and hyperuricaemia with a family history of both tubulointerstitial kidney disease and gout, providing further evidence for the pathogenicity of this variant.\nThe patient’s sister commenced work up as a live kidney donor aged 53 years. She was normotensive with bland urinalysis and a serum creatinine of 72 μmol/L. Her serum urate level was also within normal limits at 250 μmol/L. Renal ultrasound scanning suggested a slight size discrepancy between the right and left kidneys of approximately 1.6 cm; she went on to have magnetic resonance angiography of the kidney, which showed no evidence of narrowing or dilatation of the renal arteries. Urgent REN genetic testing excluded the c.49 T > C pathogenic variant identified in her sister (Fig. ). This allowed pre-emptive live donor transplantation with a 0:0:0 HLA mismatch to take place shortly afterwards. The patient’s serum creatinine was 124 μmol/L one month post-transplant and 139 μmol/L when last checked; her haemoglobin levels have remained approximately 100 g/L despite stopping treatment with an ESA at the time of the transplant.
A girl aged seven years and eight months was referred to our observation because of left peripheral facial palsy, causing the inability to close the left eye and dropping of the corner of the mouth. A first peripheral facial nerve palsy occurred when she was three years and one month old, with complete regression after corticosteroid treatment. At the age of three years and nine months, she was diagnosed with pure red cell hypoplasia, manifested as severe anaemia (haemoglobin: 3.00 g/dL; red blood cells: 1,000,000/mm3) with an extreme lack of erythroid precursors in the bone marrow, but high growth of them in culture, probably caused by anti-EPO antibodies. The detection of anti-EPO antibodies, however, is not routinely performed in a clinical setting. The autoimmune hypothesis was postulated on empirical bases, since haemoglobin levels did not increase after recombinant human EPO administration, but normalized after corticosteroid therapy, and the addition of autologous serum to the erythroid precursor culture inhibited EPO growth. When she was 4 years and 4 months old, the patient presented with a second episode of left facial palsy, combined with the acute onset of a strength deficit on the left side of the body. Mingazzini I and II were positive for the left limbs. The imaging assessment showed a haemorrhagic stroke corresponding to the anterior portion of the right putamen and of the external capsule with perilesional oedema, involving the anterior limb of the internal capsule. Blood pressure measurements performed during the hospitalisation revealed high diastolic blood pressure values. These findings suggest a central rather than peripheral involvement of the facial nerve. Three weeks after their beginning, the symptoms had completely regressed. At the age of five years and six months, a third episode of left peripheral facial palsy occurred. Brain magnetic resonance imaging (MRI) was repeated, showing gliotic evolution of the previous haemorrhagic insult without new lesions. The patient was treated with corticosteroids, with a good regression of symptoms. On the last episode, the patient had initially visited a first level emergency room, where laboratory tests, as well as ophthalmologic, neurologic, and otoscopic examinations and a head computed tomography (CT) scan performed were normal. When admitted to our department, the neurologic examination showed complete peripheral left facial palsy (House–Brackmann grade V). Physical examination showed the presence of a furrowed tongue as a synchronous anomaly. No active herpetic mucosal and skin lesions were found. The patient was started on a tapering dose of prednisone for 30 days and vitamin B group supplementation was added. The clinical course was favourable. Three months after, at last follow up, neurological impairment had clearly improved. Facial palsy gradually resolved after the third week of treatment.
We are presenting a case of a 15-year-old male from a rural area living on a farm and raising livestock. He presented with six months history of easy fatigability, shortness of breath, and dull right upper abdominal pain. Physical examination revealed that the patient had tachycardia at rest and hepatomegaly and no other significant findings. Chest X-ray (CXR) showed clear lung fields and mildly enlarged heart. Ultrasound examination of the abdomen revealed few cysts in the liver without any associated lymphadenopathy in the coeliac group of lymph nodes. As part of the workup, the patient had a CT scan of the abdomen, chest, and brain. The CT showed one cyst in the heart, four cysts in the liver, but no cysts in the lungs or the brain (Figures -).\nThe cystic nature of the lesions and their locations in multiple organs made us think of the HC as the pathology. The serology test for HC using the ELISA test came positive, which helped to confirm the diagnosis. The patient was scheduled for urgent open-heart surgery, fearing the risk of potential rupture of the cyst and the grave consequences which come with that. The patient underwent conventional median sternotomy and aortocaval cannulation with aortic cross-clamp and cardioplegic heart arrest. CT images helped to locate the cardia cyst and its relation to the cardiac chambers. The location of the cyst was confirmed by direct examination of the heart when it was arrested. The cyst was totally located intramurally in the interventricular septum and expanding down mostly to the left side of the diaphragmatic surface of the heart and partly crossing intramurally to the diaphragmatic surface of the right ventricle making a visible and palpable soft cystic bulge. The myocardium overlying the cyst on the left side of the posterior descending coronary artery on the diaphragmatic surface of the heart was fibrosed, making part of the ectocyst wall. This was the most epicardially prominent part of the cyst and was chosen as the site of aspiration and later incision to evacuate and remove the cyst. Initially, a 50 mL syringe was used to aspirate the cyst and make it less tense to avoid rupturing it during incision (Figure ).\nAround 200 mL of clear fluid was aspirated, and the bulge disappeared. After the evacuation of most of the fluid, a small surgical incision of the fibrotic ectocyst was performed from the same site of aspiration (Figure ).\nA surgical discard sucker tip was introduced via the incision, and some residual fluid was aspirated. The incision was enlarged to admit forceps to take the cyst out. There was a residual cavity left behind intramurally caused by the cyst. The residual cavity was inspected to make sure that no part of the cyst was left behind, and the wall was intact, and no communication with cardiac chambers was noticed as no blood was ever seen to collect. The cyst and the fluid were sent to the histopathological and cytological exam (Figure ).\nThe cavity left draining to the base of the heart without closure of the surgical incision, hoping that the normal filling of the ventricular chambers will eventually lead to the collapse of the residual cavity and prevent any future collection. The patient weaned successfully from the heart and lung machine, and a mediastinal drain was left on the diaphragmatic surface of the heart and another on the anterior surface of the heart. The patient was then discharged to the cardiac intensive care unit (CICU). After a few hours the patient was extubated from the ventilator without any pharmacological support. After 24 hours, CXR and echocardiography were repeated, and both were normal. The patient was discharged home after six days very stable and was referred to general surgery to deal with the liver cysts in the future.
A 47-year-old woman in good general health, with no other co-morbidities presented with a long history of chronic right fifth toe pain for 30 years. She reported a history of injuring her right foot when playing sport for 30 years. She when landing recalled that she had twisted her right foot; moreover, the foot was not well treated at that time of the injury. The only treatment for the fifth toe was with the temporary bandage to reduce the deformity. Then, the PIP joint of the fifth toe was redislocated for several times in 6 months after the first injury and the same treatment was attempted each time. After that, the fifth toe gradually became permanently swollen and painful on movement. When the patient presented in the out patient clinic with chronic right fifth toe pain and swelling, (Figure ), the toe was displaced in the dorsolateral plane. The plain radiograph showed a simple dislocation of PIP joint in the dorsolateral direction. The articular cartilage of middle phalanx impinged on the lateral condyle of proximal phalanx. There was no indication of definite joint destruction, erosion or unhealed fracture (Figure ). The distal segment of the toes was unstable because of the chronic and persistent joint subluxation.\nThe decision was made to attempt the correction of the chronic dislocation by open reduction, as the problem had been present for many years and all previous attempts at closed reduction had been non-successful. The dislocated PIP joint was exposed via a dorsal incision, revealing that the joint capsule and attenuated lateral collateral ligament were interposed within the PIP joint space, and the articular cartilage at lateral condyle of proximal phalanx of the fifth toe became eroded (Figure ). The joint capsule and the lateral collateral ligament were dissected away from the joint space. The tight plantar plate was released from plantar lateral aspect of the joint and the corrected position of the PIP joint was maintained by inserting a longitudinal Kirschner wire. The lateral collateral ligament was repaired anatomically. The tightened joint capsule, and the completed skin closure were done, thereby permitting soft tissue correction of the deformity (Figure ). The sutures were removed in 2 weeks after surgery and the PIP joint correction was kept up by the retention of the in-situ Kirschner wire for 4 weeks. The wound was completely healed. The patient was able to resume normal footwear within 2 months after surgery.\nFor the 12-month post-surgery, the PIP joint of the fifth toe remained stable, painless and aesthetically pleasing (Figure ). The patient was able to normally walk, and the range of motion of the PIP joint was within normal limits. Plain radiographs confirmed joint congruity and no signs of arthritic deterioration (Figure ).\nDislocation of the PIP joint of the toe is an uncommon injury of the forefoot. The mechanism of injury usually occurs from hyperdorsiflexion or sudden application of a dorsolateral force causing traumatic abduction of the PIP joint of the toe. This is a common injury to both the great and lesser toes [-,,]. The dislocation causes instablility of the PIP joint because the soft tissues that normally ensure joint stability and congruency such as medial and lateral collateral ligaments, the fibrocartilagenous plantar plate (usually from the proximal attachment), the joint capsule and digital long and short toe extensor and flexor tendons are disrupted, compromised and injured during the forceful joint dislocation. Traumatic dislocation of the PIP joint of a toe may be successfully treated with closed reduction and adhesive neighbor strapping to adjacent toes []. The complex dislocation as described in this case, involving a chronic irreducible joint subluxation, or a reducible but unstable dislocation requires the surgical intervention to correct the disrupted soft tissues stabilizers, such as the medial collateral ligament, the joint capsule or the plantar plate in order to achieve joint stability.\nClosed reduction is usually successful in correction of IP joint dislocation in hallucal or lesser toes. The digital deformity is initially accentuated. The longitudinal traction, allowing the joint to resume this deformity was applied and then released, so it corrected the anatomical relationship []. Stientra and Denner described a modification of this technique, where the dislocated joint was dorsiflexed to exaggerate the deformity. The dorsiflexion was maintained while the affected IP joint was further dorsiflexed and then plantarflexed to relocate in the anatomical joint position, usually with an audible ‘pop’ []. All cases should be treated initially by closed reduction, but the closed maneuver failed to achieve joint correction. The open reduction was another treatment choice [,,,]. In the case reported, only correction by closed reduction did not achieve a stable joint due to the displacement of the joint capsule, the laxity of the collateral ligament, and in part by the articular erosion of the lateral condyle of the proximal phalanx. Yang reported that approximately 50% of patients could be successfully treated by closed reduction [].\nOpen reduction is best accomplished through a dorsal approach [-]. Temporary pin fixation is only indicated when the reduced joint is very unstable. Ultimate joint stability is improved when structures causing the buttonhole effect (i.e., the injury involving with the long extensor tendon, the distal end of the phalanx becoming “buttonholed” between the outer bands of the tendon) are removed. If the capsule, collateral ligaments, tendons or plantar plate are injured or attenuated, they should be repaired to achieve a neutral and anatomical position [,,,,]. In our case, open reduction was performed via dorsal approach after failure of closed method. The attenuated lateral collateral ligament found to be entrapped within the joint was released. This finding was different from the previous publication in that medial collateral ligament and flexor digitorum longus tendon became entrapped in the joint. The plantar plate that contracted on the plantar lateral aspect of the joint from prolong period of subluxation and dislocation was one of the important structures causing the irreducible or reducible instability of the joint. It was released to restore joint stability. The dorsal capsule and lateral collateral ligament were repaired to achieve the stability. Temporary pin fixation was performed to enhance the joint stability during the surrounding tissue healing promoted. The pin was removed after 4 weeks to achieve stability and painless PIP joint. Radiographic examination following closed reduction is strongly recommended [], as chronic irreducible dislocation of the lesser toes could result from the lack of definitive radiographic evaluation since closed reduction with inadequate immobilization was insufficient.
This is a case of a 25 years old Malay girl with learning disability and no significant past medical history, who started noticing a sacral mass since August 2015. The mass was painless and gradually increasing in size. The family members of this patient brought her to a traditional healer. They did not seek any medical treatment until late 2017. By this time, the mass over the sacrum was extremely large. Family members claimed the mass was preventing the patient from lying down flat supine. The patient was also unable to ambulate for the past 2 years. Hence, she was bedbound most of the time. It was difficult for her to sit on the wheelchair. She also felt tired to move because the mass was quite heavy. The family members claimed when the patient was lying down flat, she had to flex her hips and knees to achieve a more comfortable position. In addition, she often slept either in prone position or in supine with multiple pillows below her body. The mother also claimed over the last 2 months, the patient’s body had been getting thinner despite her physical weight was increasing due to the increase in size of the sacral mass. The patient had been passing stool and urine in pampers. There was no past medical history and no family history of cancer. Socially, the patient lived with her mother and siblings. The mother was the main care taker. Her father passed away 10 years ago because of heart attack. The patient previously attended a special needs school, but she stopped going to school since 2015 after developing the sacral mass.\nThis patient was managed in the Southern Region referral centre for Orthopaedic Oncology in Malaysia. On clinical examination in the Orthopaedic Oncology ward, the patient appeared cachexic, she had slightly pale conjunctiva, but she was not dysmorphic. Vital signs were Blood Pressure 142/90, Pulse Rate 98 beats per minute and Temperature 37 degrees Celsius. There was a large mass 40 cm × 30 cm × 20 cm over the sacrum. The mass was firm to hard in consistency and involved both buttocks and the gluteal fold (Fig. ). Dilated veins were noted under the skin overlying the sacral mass. Neurological exam of bilateral lower limb was normal. However, there was generalized wasting of all muscles over the bilateral lower limb. Anal tone was intact.\nLaboratory investigations taken were unremarkable. Computed Tomography of the Pelvis showed a large destructive sacrococcygeal mass measuring 43 cm × 38 cm × 27 cm with extension into the presacral space resulting in anterior displacement of the rectum, urinary bladder and uterus and posterior extension into the dorsal soft tissue with involvement of the gluteus, piriformis, and left erector spinae muscles (Figs. and ). Superior margin of the sacral bone involvement was up to S2. The mass was predominantly of fluid density with internal enhancing septation and calcifications which suggested primary chordoma more likely (Figs. and ). Magnetic Resonance Imaging done showed similar findings. Skeletal Survey Radiograph did not show any distant metastasis. A Trucut biopsy of the mass was done. Histopathological analysis showed tumour cells with “physaliphorous cells” positive for pancytokeratin, EMA, Vimentin and S-100 immunohistochemistry stainings with minimal mitotic figures and mild nuclear pleomorphism (Fig. ). Brachyury immunohistochemistry staining was not available in our centre. However, the clinical history, morphology of tumour on microscopy and immunohistochemistry staining available were consistent sacral chordoma.\nThe diagnosis of Sacral Chordoma was confirmed. Multidisciplinary team discussion done among Orthopaedic Oncology, General Surgery, Obstetrics and Gynaecology, Blood Bank, Anaesthetic and Plastic Surgery teams. A family conference was done. The family’s aim was for removal of the sacral mass to allow the patient lie supine on bed and sit on the wheelchair.\nSubsequently, the patient undergone Wide Resection and En Bloc Sacrectomy. The Posterior-Only Approach was used with a “Mercedes Star” 3 limbed incision. Duration of surgery was 8 h. The patient was supported with blood products transfusion during surgery. Intraoperatively, the sacral tumour had eroded the sacral bone from S2 to S5 (Figs. , and ). Sacrectomy was done at the level of S2. Sacral nerve roots S2-S5 were all infiltrated by the mass and therefore were unable to be preserved. The mass and surrounding gluteal muscles invaded by the tumour were also all resected. All resection margins were less than 1 mm from the tumour. Primary closure was done without any distant or local flap as per consultation with Plastic Surgery team. The tumour weight was 25 kg (Figs. , and ). Post operatively, the patient was monitored in Intensive Care Unit for 3 days. The patient developed neurogenic bowel and bladder post sacrectomy requiring enema and long-term urinary catheter. In addition, the post-operative course was complicated by wound breakdown and surgical site infection requiring wound debridement. Dressing was done as per local protocol until wound bed granulating well. Split Skin Graft was done about 3 months post wide resection once the tissue culture results were free of significant infection.\nThe patient also required extensive rehabilitation for transfer, ambulation and bowel and bladder care. Rehabilitation was difficult because the patient had learning disability and she had been habitually keeping her hips and knees flexed because of the sacral tumour for the past 2 years. During the last review 5 months post operatively, patient was able to sit on the wheelchair comfortably. The surgical wound was healing well with good uptake of the Split Skin Graft (Fig. ).
A 56-year-old lady presented with a 6 month history of rectal bleeding, passage of mucus and a change in bowel habit to more frequent stools. She had no abdominal pains and her weight was maintained. The patient had been born with bladder exstrophy and had multiple surgeries culminating in a cystectomy with ileal conduit formation at 5 years of age. She had suffered with recurrent urinary tract infections for most of her childhood and adult life but was otherwise well with no other major co-morbidities or risk factors for colorectal malignancy and had no family history of colorectal disease.\nColonoscopy revealed two large sessile polyps in the sigmoid colon in close proximity to each other and adjacent to a diverticulum-like structure (Fig. ). Each polyp was approximately 3 cm in size and both exhibited a type IV pit pattern with areas of irregularity suggestive of focally advanced disease. Histological examination confirmed both polyps were adenomas comprising both low and high grade dysplasia, without submucosal invasion (Fig. ). On closer inspection the mucosa around the diverticulum was also atypical but not adenomatous. The remainder of the colonoscopy was unremarkable with no other evidence of diverticular disease or polyps elsewhere.\nEndoscopic resection was considered as a therapeutic option however in view of the above characteristics as well as difficult endoscopic access surgery was preferred. Furthermore, radiological imaging had initially raised the possibility of invasive disease in view of sigmoid thickening. The patient underwent high anterior resection and an open approach was chosen because of suspected intra-abdominal adhesions following extensive pelvic surgery. An end colostomy was formed at the patient’s pre-operative request. At laparotomy the right fallopian tube was adherent to the sigmoid colon and adjacent to this a blind ending tube was noted to emerge from the anti-mesenteric border of the colon. This was marked for pathological identification.\nOur patient went on to have an uneventful recovery and her quality of life following surgery was good. Her wish to have a permanent colostomy stemmed from the fact that she had always suffered from an erratic bowel habit and that she was already knowledgeable with regards to stoma care in view of her pre-existing ileal conduit.
A 58-year-old female with a past medical history of hypertension that was well controlled with medications and type 2 diabetes mellitus who recently lost her husband and father within a span of 2 weeks and was in a lot of stress was found by the police in a confused state at a general store, which resulted in the emergency medical services being called. On arrival of the emergency medical services, the patient became obtunded and was brought to the emergency room. Her last known contact with any family member was the night before in the evening with her niece over a phone conversation. She was maintaining her airways and saturating well throughout her course. Imaging with a head computed tomography (CT) and head and neck CT angiography (CTA) was done, both of which showed no evidence of acute stroke or any aneurysms. The patient was found to be in a hypertensive emergency with a blood pressure of 230/110 mm Hg. The patient was admitted to the neuro intensive care unit for further monitoring and treatment. Her metabolic panel was normal, and she had normal blood sugars. Straight catheterization, a urinalysis, and a urine drug screen were done. The urine drug screen came back negative.\nOn examination, the patient was obtunded with a minimal response on deep sternal rub and on painful stimulus. Neurological examination revealed a positive gag reflex with a fixed and dilated left pupil at 7 mm with a normal nonreactive right pupil. The patient also had lateral deviation of the left eye with no nystagmus appreciated. Based on the lateral deviation of the left eye and clinical presentation, a magnetic resonance imaging (MRI) of the head was done. The MRI showed increased FLAIR (fluid-attenuated inversion recovery) signal within the periventricular white matter consistent with paramedian thalamic nuclei without midbrain involvement and acute bilateral ischemic infarction consistent with the AOP occlusion was diagnosed (-).\nBecause of her hypertensive emergency and more than 24 hours of documented stroke diagnosis since she was known at her baseline, the patient was not a candidate for tissue plasminogen activating factor (tPA). The neurosurgery recommendation was conservative management. The patient was kept in the neuro intensive care unit and got a 2-dimensional echocardiogram with bubble study done the next day, which was negative. Telemetry did not show any abnormal cardiac rhythms. The patient continued to improve. Within the first 48 hours, the patient was confused but awake and following minimal commands. She continued to show improvement and passed a speech and swallow test. Eventually, the patient was discharged to subacute rehab with neurological follow-ups and was given dual antiplatelet therapy with Aspirin and Plavix as per recommendations of neurosurgery with reevaluation for the need of dual antiplatelet in 4 weeks.
A 29-year-old man was admitted to emergency room 30 hours after ingestion of about 1 liter of homemade alcohol. The clinical picture began with frequent vomiting, blurred vision and back pain. Initial examination demonstrated an agitated and confused patient with respiratory distress. The respiratory rate was 26 per minute, pulse rate of 82 per minute, blood pressure of 130/80 mm Hg and axillary temperature of 36.5 C and laboratory tests as presented in .\nIn clinical examination both pupils were dilated and the response to light was slow. The results of examination of the heart, lungs were normal and the findings of chest x-ray were unremarkable. There was no cyanosis or edema.\nThe patient was intubated in emergency room and mechanical ventilation was started. The PH was 7.017, gavage of ethanol, infusion of sodium bicarbonate was started and folic acid, pantoprazole was administered in accordance with intravenous fluids. In quantitative test of methanol assessment, the serum methanol level was estimated at the level of 50 mg/dl. The victim underwent two courses of hemodialysis. Over the course of poisoning, the level of Glasgow Coma Scale (GCS) deteriorated and on the seventh admission day the status of GCS remained at the level of B-C. The pupils were not reactive. The patients was required to be under ventilator in intensive care unit (ICU). The results of the laboratory tests at this time are presented in .\nTwo units of packed cells were transfused and a brain CT scanning showed large and symmetric brain edema as well as brain hematoma in subcortical brain over frontal and occipital lobes bilaterally as well as in putamen and right capsule areas. (). Repeated clinical examinations, x-rays of the heart and lungs as well as the results of laboratory tests were normal and several attempts of weaning patients from the ventilator were unsuccessful and so a tracheostomy as well as percutaneous endoscopic gastrostomy was performed on the 18th day of hospitalization. Combined enteral and parenteral nutrition was continued according to current treatment criteria (). The status of consciousness improved over the hospitalization period and the patient could be detached from the ventilator on the 79th day of hospitalization with GCS level of 9-10.\nFollow-up examinations were continued for six months. At the latest examination the general condition of patient improved a little as compared during hospitalization time and was poor because of visual and neurological complications. The patient had urinary and fecal incontinence. The visual capacity of patient was limited to recognizing only a shaking hand and the potential of motor system was limited to slight movement of the hands and feet. The latest brain CT scanning demonstrated symmetrical brain atrophy of both frontal lobes. The lateral ventricles were dilate d, there were hypodensities in external capsule, left internal capsule and bilateral caudate areas ().
An 84-year old male presented with shortness of breath during ordinary daily physical activity {New York Heart Association (NYHA) functional classification class III}. He also experienced syncope 2 months prior to admission to our hospital and intermittent chest pain for 2 weeks leading up to admission. The patient had a past medical history of hypertension and chronic obstructive pulmonary disease. He had been diagnosed with moderate AS 4 years ago and followed up regularly with checkups in the outpatient clinic, and the patient had been prescribed irbesartan, amlodipine, hydrochlorothiazide, atorvastatin. On admission his blood pressure and pulse rate were 150/70 mm Hg and 78/min, while body weight and height were 53 kg and 172 cm. The 12-lead electrocardiogram (ECG) on admission showed sinus rhythm with intermittent premature atrial complexes and findings suggestive of left ventricular hypertrophy with strain signs on lateral leads. Cardiomegaly was noted on the chest X-ray. Transthoracic and transesophageal echocardiography revealed severe degenerative change of aortic valve with heavy calcification (). The aortic valve area was 0.76 cm2 with a mean systolic pressure gradient of 43 mm Hg and peak systolic pressure gradient of 79 mm Hg compatible with severe AS. The aorta diameter was 21 mm at the aortic annulus, 32 mm at the sinus of valsalva, and 36 mm in the ascending aorta. Left ventricular end diastolic diameter was 52 mm and ejection fraction was 67%. There was mild pulmonary hypertension with an estimated right ventricular systolic pressure of 38 mm Hg. Coronary angiography showed mild coronary artery disease with 50% stenosis at the mid left anterior descending segment. Computed tomography showed diffuse atherosclerotic change of aorta and both external iliac arteries with calcification. The minimal lumen diameter of right and left external iliac arteries was 6 mm and 7 mm, respectively. A pulmonary function test revealed mild obstructive airway disease. The logistic Euroscore was calculated to be 36.1%.\nThe TAVI procedure was performed under general anesthesia. The vascular access for CoreValve delivery catheter was obtained at the right common femoral artery (CFA) with standard percutaneous access techniques. An 18 Fr introducer sheath was inserted through the right CFA after preparing percutaneous closure using two pre-loaded suture devices (Perclose Proglide, Abbott Vascular, Abbott Park, IL, USA). A pig tail catheter was inserted through the left CFA and positioned at the aortic root for the aortography during the procedure. A temporary pacemaker was placed in the right ventricle via the left femoral vein. A 0.035 inch Amplatz Super Stiff wire (Boston Scientific, Natick, MA, USA) was inserted into the left ventricle through the 18 Fr sheath. Balloon dilation of the stenotic aortic valve was performed with a balloon (diameter 25 mm, length 40 mm, Z-med, NuMED Inc., Hopkinton, NY, USA) under rapid pacing using a temporary pacemaker. Then, a 29 mm CoreValve was deployed at the aortic annulus under angiographic guidance (). An immediate post-procedural aortogram showed good position of the CoreValve with mild aortic regurgitation (). Post-procedural transthoracic echocardiography demonstrated a well-functioning CoreValve with a mean systolic pressure gradient of 7 mm Hg and peak systolic pressure gradient of 16 mm Hg (). The right ventricular systolic pressure decreased from 38 to 31 mm Hg, accompanied by moderate paravalvular aortic regurgitation (grade II/IV). After the procedure, the patient was observed in an intensive care unit for 72 hours with continuous ECG monitoring and temporary pacemaker back-up. No significant conduction abnormalities were observed except for transient nonspecific intraventricular conduction delay with incomplete left posterior hemifascicular block. The patient's symptoms subsequently improved from NYHA class III to class I and was discharged from the hospital at day 8 post procedure without any significant complication. A 3-month follow-up echocardiography revealed no significant interval change from the immediate post procedure echocardiographic findings. The patient remained free from any symptom or any major cardiovascular event for a total follow-up period of 6 months.
A 66-year-old man, who had been undergoing hemodialysis for years, was referred for the creation of a proper long-term dialysis access. He had multiple AVFs in both upper extremities that had failed previously and was currently on dialysis through a left femoral vein double-lumen catheter. He had a right lung empyema a month ago and was still undergoing drainage and appropriate antibiotic treatment. Because of the history of empyema, which increases the risk of infection in AVG, we decided to implant an AVF in the upper extremity again. Ultrasound mapping was performed for the patient in the operating room without tourniquet application to examine the basilic and brachial veins in both limbs. The basilic vein was thrombosed previously, but in the left upper limb, the brachial veins were open and the medial vein was 3 mm and joined by the axillary vein in the higher part of the upper arm. The paired brachial vein diameter was also 3 mm. Therefore, we planned the surgery to transpose the left medial brachial vein. BrVT was performed under local anesthesia with sedation with three skip incisions in the medial arm. Initially, a 4-cm horizontal skin incision was made distally in the arm to explore the brachial artery and vein. Then, the lateral branches were found and separated along the brachial vein. The connections between the two brachial veins were divided by simple sutures. The main difficulty with the restricted incision was to obtain proper exposure and this was resolved by cutting the brachial vein in the cubital part and changing its direction in different views. In the second stage, another 4-cm incision was made horizontally in the middle of the arm. The lateral branches of the brachial vein were carefully explored and separated from the lateral incisions at intervals between skin incisions. Finally, by incision of the skin across the hairline in the axillary area, the brachial vein was explored and released from the elbow level. The vein was transposed anteriorly in a superficial tunnel and re-anastomosed higher up on the brachial artery by using a monofilament polypropylene 7.0 continuous end-to-side suture (). Mild edema occurred after the surgery, but no complications were observed 3 weeks postoperatively. After 45 days of maturation, the diameter of the vein reached 7 mm and hemodialysis was initiated successfully through the new autologous vascular access.
A 25-year-old man was admitted to the emergency department after falling off his bicycle. He was hit by the bicycle's handle bar into the left side of the abdomen. The clinical examination revealed a fit young man who had only a scratch (abrasion) on the abdominal wall left of the umbilicus. Hemodynamically he was normal and alert and referred to intense pain at the abrasion site. The abdomen was soft, without signs of peritoneal irritation while sensitivity was only at the point of the abrasion. Laboratory tests, including white blood cells, liver enzymes, and amylase, were normal. The abdominal X-ray in upright (standing) position did not reveal the presence of free intraperitoneal air. Abdominal ultrasound did not reveal any solid abdominal organ injury nor the presence of free intraperitoneal fluid. The pain was attributed to hematoma of the rectus abdominis muscle pricey below the abrasion which was revealed by ultrasound.\nThe pain of the young man was gradually worsening and was mostly described diffusely throughout the whole abdomen. The abdomen remained soft and tender without signs of peritoneal irritation. We proceeded to the administration of per os contrast material and CT abdomen. The CT scan revealed the presence of sufficient amount of free intraperitoneal contrast but did not allow the clarification of the exact point of escape. The free contrast material was detected at the left side of the abdomen, at the area of the descending colon but mainly in the pelvis (). The intraluminal contrast had even reached up the level of the transverse colon.\nThe patient was taken to the operating room for exploratory laparotomy immediately after the CT scan (4 hours after the arrival at the emergency department and about 4.5 hours after the time of injury). Intraoperatively, fecal peritonitis due to isolated rupture of jejunum 30 cm from the ligament of Treitz was revealed. The rupture was at the center of an extensive jejunum serous layer fissure giving the picture of “blow out” perforation. At the jejunal mesentery there was minimal bruising. Multiple washings of the peritoneal cavity were performed with normal saline, and the restoration of jejunal injury was done with partial resection and end-to-end anastomosis ().\nThe postoperative course of the patient was uncomplicated, and he was discharged on the 6th postoperative day. 9 days later he returned with clinical and radiological signs of ileus that led us to the operating room 48 hours later due to deterioration of his clinical picture. The cause of the ileum was adhesions, so we proceeded to lysis of adhesions. The anastomosis was normal without leakage. Five days later the patient was discharged, and a year later he is still free of symptoms.
A 76-year-old woman who was suffering from persistent atrial fibrillation, atrial hypertension, and chronic obstructive lung disease and who had previously been in treatment for ovarian cancer, colon cancer, and breast cancer was hospitalized due to an INR (international normalized ratio) above 9.0. At the time of hospitalization, she was in treatment with verapamil 120 mg daily and warfarin for persistent atrial fibrillation. A complete list of medicine at the time of hospitalization is available in Table .\nThe patient had recently had a gastroscopy revealing a fungal infection and a high level of gastric acid. Her family physician therefore initiated a treatment with a short course of fluconazole and pantoprazole. After 2 days of treatment with fluconazole, her physician measured an INR of 5.6. After a control visit 2 days later, the INR had increased to eight and the patient was hospitalized.\nAt the time of hospitalization, her heart rate was 96 bpm. During the evening on the third day of hospitalization, an electrocardiogram (ECG) showed atrial fibrillation and a junior physician prescribed Selo-zok® (metoprolol), 50 mg slow-release tablet. According to the latest guideline from the European Society of Cardiology, a patient in need of acute rhythm control can have digoxin added to the treatment with verapamil, if the patient has a left ventricular ejection fraction of above 40 and the heart rate is above 110 bpm. The patient was not known with previous heart failure or reduced ventricular ejection fraction, and a suspicion of heart failure was not mentioned in the hospital records at this time. The next morning an experienced doctor discontinued metoprolol during the morning rounds, as she was aware of a potential interaction between metoprolol and verapamil. The patient was well and had no signs of acute illnesses. Only one tablet of metoprolol 50 mg had been administered to the patient. After lunch on the same day, the patient developed bradycardia and hypotension and infusion with isoprenaline was initiated (see Table for details). An interaction between verapamil and metoprolol was suspected. The condition progressed and despite of isoprenaline, atropine, and external pacing, her blood pressure was immeasurable and her heart rate decreased (Figure ).\nShe was transferred to another hospital for the insertion of a temporary pace wire. On arrival at the second hospital, the patient was completely unresponsive, she had been intubated in the ambulance and her pulse had decreased to ten beats per minute. Blood analysis showed serious metabolic acidosis. Further treatment was considered futile, and the patient was declared dead at 19.30 on the third day of hospitalization. A medicolegal autopsy was performed, and a standard forensic toxicological analysis was performed on blood withdrawn from the femoral vein at autopsy shortly after her death, see Table for the blood concentrations of drugs. The autopsy showed right atrial dilatation, but otherwise normal right and left ventricles, normal heart valves, and no signs of acute coronary syndrome, and the liver was normal.
Our patient is a 27-year old female that denied any past medical and surgical history. Her family and drug history were also unremarkable. The patient presented with a 7-month history of progressively enlarging mass on her left ring finger. The patient mentioned that the mass appeared suddenly with no history of trauma and that she was concerned about the potential malignant nature of the mass. The patient also mentioned that she underwent incision and drainage of that mass 2 months after its appearance with no improvement and provided no detailed surgical or pathological reports, which was the reason for her delayed presentation. Upon her assessment, the mass was located over the ulnar side of the proximal phalanx of left ring finger with extensive involvement of the 4th web space. The overlying skin coverage was ulcerative with no active signs of infection. Range of motion of the involved digit was limited, however neurovascular examination was normal. ().\nRadiological evaluation of the involved hand showed a soft tissue swelling with no evidence of bone involvement (). Further magnetic resonant (MRI) evaluation showed a mass on the volar aspect of the ring finger encasing about 50% of the flexor tendons of that digit with low signal intensity on T1 and high signal intensity on T2 evaluation with strong enhancement in post contrast evaluation. Assessment of neurovascular structures showed partial abutment of the radial sided bundle together with complete encirclement of the ulnar sided neurovascular bundle. The surrounding bone was free of any masses and associated mass effect.\nThe patient was taken to the OR for exploration and mass excision by the senior author. Possible risks associated with such intervention were explained. Intra-operatively, bruner type incision was designed together with island of skin involved in the mass. Exploration revealed extensive subcutaneous mass with fibro-fatty consistency with extensive fascia like extension to the surrounding soft tissue. The mass was encircling the ulnar neurovascular bundle with mass abutment over the radial bundle as seen in pre-op assessment. The mass was dissected freely from its attachment to those bundles preserving both radial and ulnar structures. The mass was then excised en-bloc having a dimension of 3.5 × 4x2.5 cm (). Histological assessment showed a lesion with fasciitis like features, myofibroblastic proliferation and scattered foci of osteoid formation that was positive for Alpha-Smooth Muscle Actin (ASMA 1A4) immune staining and no evidence of malignancy (). The resected margins were however, positive for residual lesion with difficulty in obtaining negative margins due to the extensive nature of the mass. Post-operatively, the patient had an un-eventual course. She was informed about the need for close follow-ups for both clinical and/or radiological signs of lesion recurrence, pending early surgical intervention (see ).
The 72 year old male patient was referred to our clinic with the complaint of bilateral axillary masses and numbness in the right hand. In his medical history, he had hypertension for 4 years but no history of trauma. A physical examination showed the presence of palpable pulsatile masses in his both axillae (12 cm in size in the right axilla and 8 cm in size in the left axilla) (Fig. ). His neurological and systemic examinations were within normal limits. An upper-extremity ultrasound examination revealed an aneurysm in a 12-cm segment of the right axillary artery, reaching 67 × 45 mm in dimension. There was a 27-mm thick thrombus at the edge of the aneurysm lumen. Adjacent to the aneurysm, there was a 48 × 40 mm hyperechogenic mass not related to the aneurysm. There was also an aneurysm reaching 40 × 43 mm in dimension in a 9-cm segment of the left axillary artery, with a 17-mm thick thrombus. A Doppler ultrasonic examination revealed a thrombus surrounding the lumen and a turbulent, pulsatile flow in the center. The aneurysmal segment was continuous with the brachial artery. There was no blood flow in the region adjacent to the right axillary artery (Figs. and ). On CT angiography, giant fusiform aneurysms were detected, measuring 140 × 77 mm in the right axillary artery and 93 × 45 mm in the left axillary artery. There were thrombi in both aneurysms, allowing the flow in the lumen. There was also an extension to the brachial artery and contour irregularities at the distal end of the right-side aneurysm. These latter findings were proposed to be due to a spontaneously healed rupture. A physical examination of the eyes, bones, skin and joints revealed no signs of collagen tissue disease, nor was it detected through laboratory examinations. Because the patient was symptomatic and had a high risk of aneurysm rupture, endovascular closure was initially planned. However, appropriate size stents could not be found for aneurysms so large and tortuous. Besides, the long-term success of the stenting procedure could not be guaranteed due to the large sizes and high mobility of the aneurysms, so the patient was referred for surgery.
R.F. is a healthy appearing 55 year old man who had gross hematuria February 2016. His past medical history is negative with the exception of a 15 pack/year history of cigarette smoking. He had a normal CT scan with the exception of a bladder tumor located in the posterior wall of the bladder. He underwent an uneventful transurethral resection of a papillary bladder tumor in March 2016 at another medical center.\nHe received a single postoperative dose of intravesical mitomycin C 40 mg in 40 ml in the recovery room.\nThe pathology report indicated a high grade (grade 3) urothelial carcinoma which invaded the lamina propria. There was muscularis propria present in the specimen and there was no invasion beyond the lamina propria.\nThe patient did not receive additional adjuvant therapy. He began to have marked urinary frequency and dysuria shortly after the TUR BT.\nI initially saw him in December 2016. He still had marked lower urinary tract symptoms. He voids every 1–2 hours – day and night. Anticholinergic medications were tried without improvement. He has not had a urinary tract infection.\nA flexible cystoscopy in December 2016 revealed a 4–5 cm denuded area on the posterior wall of the bladder with calcifications on the surface. There was no obvious tumor in the bladder. A voided and bladder wash urine for cytology indicated atypical cells.\nAlthough an office cystoscopy in March 2017 was unchanged a urine cytology was reported as high grade urothelial cancer. Cystoscopy and bladder biopsies were therefore performed under anesthesia. The same denuded area was observed on the posterior wall. The rest of the bladder appeared normal as did the prostatic urethra. Cold cup bladder biopsies did not indicate cancer. Urine cytology was again consistent with high grade cancer.\nI performed a flexible cystoscopy and obtained urine for cytology in October 2017. The abnormal appearing concretions were again observed on the denuded urothelium on the posterior wall. The remainder of the bladder was normal. The cytology was again positive for high grade cancer.\nThe patient’s voiding symptoms persist and are a major quality of life problem.\nA recent CT scan of the abdomen and pelvis is perfectly normal with the exception of a line of calcification at the posterior bladder wall consistent with the endoscopic findings.\nWhat should one do? Possibilities include a partial cystectomy, total cystectomy or cystoprostatectomy and neobladder, or continued monitoring.
A previously healthy 33 year old woman was admitted to the acute medical care unit with a two day history of feeling lethargic and unwell. She was a non smoker and did not drink alcohol. She had developed severe continuous pain in the right upper limb with no specific aggravating or relieving factors and associated with severe weakness. She had had three episodes of vomiting and diarrhoea and had been treated by her general practitioner with paracetamol for flu-like symptoms for three days. On the morning of the day of admission she had noticed that her lips and tongue were swollen and she felt short of breath. She was given intramuscular adrenaline by the ambulance staff for a threatened airway and was transferred to the acute medical unit for further evaluation and management.\nOn admission she had pulse rate of 110/min, respiratory rate of 22/min and was febrile with a temperature of 38.9 deg c. She was agitated, restless and incontinent. Three hours later she started to develop swelling of the face. The facial skin was intact and there was no erythema, rash or blisters. Examination of the oral cavity revealed an oedematous tongue, pharynx and buccal mucosa with no detectable septic focus. There was no neck stiffness, no localising neurological signs and her GCS was 14. There was no local rise of temperature and soft tissue palpation of the head and neck was normal with no localised tenderness and no palpable lymph nodes or surgical emphysema. Air entry was equal on both sides but there were diffusely scattered rhonchi. Cardiovascular system examination was normal. Abdominal examination revealed a localised tenderness in the left iliac fossa. Pelvic and perineal examination by the gynaecologist was normal. Right upper limb examination was negative for any soft tissue or skeletal injuries. There were no signs of cellulitis, erythema or blisters. Palpation of the soft tissues was non tender, with no signs of grimacing or withdrawl and consistency was soft with no signs of compartment syndrome. Active movements at shoulder, elbow and wrist joints were within normal range and painless. All peripheral pulsations were normal with satisfactory capillary circulation.\nThe laboratory parameters following admission have been summarised in table . The C -reactive protein [CRP], urea, creatinine and leucocyte count were elevated. Arterial blood gas analysis was consistent with metabolic acidosis. Chest X-ray and CT scan of the abdomen were normal. Blood sample for Polymerised Chain Reaction [PCR] test for meningococcal antigen was negative. Lumbar puncture examination was normal. Synacthen stimulation test revealed a high baseline reading which was suggestive of maximal stimulation. A provisional diagnosis of meningococcal septicaemia was made and on microbiological advice treatment was commenced with a third generation cephalosporin and teicoplanin by intravenous route. Vital parameters were supported with intravenous fluids and oxygen therapy by mask. Intake output chart was strictly maintained.\nEight hours later her GCS dropped to 7 and in view of her threatened airway, she was transferred to the intensive care unit. She was further resuscitated, intubated, and ventilated. 'Sepsis care bundle' was commenced [] which included optimisation of arterial pressure, haematocrit, central venous pressure and mixed venous saturation [], low dose hydrocortisone administration following the synacthen test [], intensive insulin therapy [], and low tidal volume ventilation[]. Renal replacement therapy by veno-venous filtration was begun because of the worsening acidosis. Recombinant human protein C was not given, as the criteria for its administration were not met [].\nOver the next 18 hours a non-blanching erythematous rash appeared over the right upper limb, which progressed rapidly to a blister over the medial aspect of the arm with gross swelling. There was no local rise of temperature and the consistency was soft with satisfactory capillary circulation [Fig ]. A diagnosis of necrotising fasciitis was considered. X-ray of the arm revealed no bony injury and no gas was evident in the subcutaneous tissues. The patient was hypotensive, clinically unstable and required constant monitoring and support. MRI was not available over the weekend and it was decided to proceed further on clinical assessment alone. Emergency surgery including the option of an amputation or a disarticulation was considered.\nOn microbiological advice, the antibiotics were changed to imipenem to cover both staphylococcal and streptococcal infection and clindamycin for its improved soft tissue penetration and ability to halt toxin production. Repeat blood samples revealed an elevated white cell count and CRP and normal coagulation parameters. Creatinine kinase was high and urine was negative for myoglobin. Initial blood cultures were negative and a repeat blood culture at 48 hours following admission was positive for alpha haemolytic (viridans) streptococcus mitis. The sepsis care bundle was continued, and the clinical and biochemical picture were reviewed frequently by senior orthopaedic, and intensive care staff.\nThe patient improved within the next 20 hours and she was weaned from renal replacement therapy, ventilation and was extubated. She was transferred to the ward after 24 hours for further management. Oral clindamycin was continued for a further 2 weeks until her blood parameters were optimised. The skin lesions disappeared over the next three weeks with full recovery of her upper limb function [Fig ].
Patient B is a 76-year-old white male who presented with microscopic hematuria in 1986 on a routine urinalysis and a diagnosis of left RCC was made by imaging. He then underwent nephrectomy for a 7 × 6 × 5.5 cm tumor extending though the fascia into the perinephric fat. He did well until March of 2006 when he had a routine chest X-ray that showed new lung nodules and a CT scan was ordered. A large retroperitoneal mass posterior to the stomach measuring 10 × 9 × 8 cm was also found along with bilateral lung nodules, with the largest lung lesion measuring 2 cm. Biopsy of the retroperitoneal mass confirmed RCC. MRI of the brain and bone scan was negative. He was seen for the first time at our institution in April of 2006. He was determined to be a candidate for high-dose IL-2 (600,000 IU/kg). After 8 weeks, he did show some response, but was only able to tolerate half of cycle 2 and he declined further IL-2. He was then started on sorafenib and had a minor response at all disease sites until CT scan in January 2007 after 24 weeks showed progression. He was then switched to sunitinib 50 mg daily for 4 weeks of a 6 weeks cycles, and after 12 weeks on therapy had a significant response which he enjoyed until week 54 when CT scanning showed marked disease progression. Sunitinib was discontinued and in January of 2008 he was started on temsirolimus 25 mg IV weekly. He required a 20% dose reduction for thrombocytopenia and oral ulcers. He remained on temsirolimus until April of 2008 when he developed symptomatic mTOR interstitial pneumonitis, so temsirolimus was discontinued. His disease continued to progress off therapy and in October of 2008, bevacizumab 10 mg/m2 given every 2 weeks was started as fifth line therapy. After 24 weeks of treatment the patient was found to have a pulmonary embolus. Lovenox was started and bevacizumab was continued without incident. At the time of submission, the patient's disease has remained stable after 72 weeks of bevacizumab. He has continued to work full time as a maintenance man.
The 72 year old male patient was referred to our clinic with the complaint of bilateral axillary masses and numbness in the right hand. In his medical history, he had hypertension for 4 years but no history of trauma. A physical examination showed the presence of palpable pulsatile masses in his both axillae (12 cm in size in the right axilla and 8 cm in size in the left axilla) (Fig. ). His neurological and systemic examinations were within normal limits. An upper-extremity ultrasound examination revealed an aneurysm in a 12-cm segment of the right axillary artery, reaching 67 × 45 mm in dimension. There was a 27-mm thick thrombus at the edge of the aneurysm lumen. Adjacent to the aneurysm, there was a 48 × 40 mm hyperechogenic mass not related to the aneurysm. There was also an aneurysm reaching 40 × 43 mm in dimension in a 9-cm segment of the left axillary artery, with a 17-mm thick thrombus. A Doppler ultrasonic examination revealed a thrombus surrounding the lumen and a turbulent, pulsatile flow in the center. The aneurysmal segment was continuous with the brachial artery. There was no blood flow in the region adjacent to the right axillary artery (Figs. and ). On CT angiography, giant fusiform aneurysms were detected, measuring 140 × 77 mm in the right axillary artery and 93 × 45 mm in the left axillary artery. There were thrombi in both aneurysms, allowing the flow in the lumen. There was also an extension to the brachial artery and contour irregularities at the distal end of the right-side aneurysm. These latter findings were proposed to be due to a spontaneously healed rupture. A physical examination of the eyes, bones, skin and joints revealed no signs of collagen tissue disease, nor was it detected through laboratory examinations. Because the patient was symptomatic and had a high risk of aneurysm rupture, endovascular closure was initially planned. However, appropriate size stents could not be found for aneurysms so large and tortuous. Besides, the long-term success of the stenting procedure could not be guaranteed due to the large sizes and high mobility of the aneurysms, so the patient was referred for surgery.
A 37-years-old female patient presented with complaints of pain in the left arm since three months followed by weakness a month later. The patient had dull boring pain around the left shoulder which radiated on lateral aspect of left arm. The pain progressed over 15 days to involve lateral forearm as well as the thumb and the index finger. This pain did not interfere in her daily activities and would respond partly to analgesics. After about a month, she noticed acute onset weakness in raising her left arm above the shoulder. The weakness progressed over a day to involve the entire left limb and she was unable to flex her elbow or grip objects. The patient also had decreased perception of hot and cold as well as texture of clothes. The patient had noticed that her left shoulder appeared thinner than the right and had history of occasional fasciculations in that region. There was no history of weakness or sensory loss in lower limbs or bladder bowel complaints. The patient had no history of fever, cough with expectoration or hemoptysis, trauma, weight loss, diabetes mellitus or hypertension. The patient did not complain of arthralgia, arthritis, rash or oral ulcers. She denied recent vaccination or unsafe sexual practices. With these above complaints, she consulted neurologist and was subjected to nerve conduction studies and electromyography and was diagnosed to have idiopathic left upper trunk brachial plexopathy. The patient was started on oral steroids, pain subsided transiently and the weakness did not respond to treatment. After about a month's therapy, she started noticing similar complaints in the right arm and at that time she was referred to our center for further evaluation and management. When the patient was presented in our outpatient department, she had developed painful weakness in both upper limbs (left > right) along with paraesthesia in both arms.\nOn examination, she was conscious, oriented and had normal higher mental functions. Cranial nerve examination was normal. Wasting was noted around the left shoulder girdle and left arm. The patient had hypotonia in both upper limbs. The power was grade 2 at shoulder joint, grade 3 at elbow joint, grade 4 at wrist extension and grade 5 at wrist flexion in the right upper limb as per Medical Research Council (MRC) grade. On the left side, power was MRC grade 2/5 at shoulder joint, 2/5 at elbow joint, 3/5 at wrist extension, 4/5 at wrist flexion and hand grip weakness was noted. The patient had normal power in both lower limbs. Deep tendon jerks in bilateral biceps, triceps, supinator were absent, ankle and knee jerk were normally elicitable. Bilateral plantar reflexes were flexor and abdominal reflex were present in all 4 quadrants. Sensory examination revealed about 30% loss in pin prick sensation in left arm in C5-C7 dermatome. Joint position and vibration sensation were impaired in the upper as well as lower limbs. The patient had positive Romberg's sign. There were no cerebellar signs, thickened nerves, stigma of tuberculosis or syphilis. No lymphadenopathy or spine abnormality was noted.\nComplete hemogram, fasting blood sugar, renal and liver function tests were within normal range. Erythrocyte sedimentation rate was 24 mm at the end of one hour by Westergren method. C-reactive protein and anti- nuclear antibodies were negative. Anti-neutrophilic antibody (cANCA and pANCA), extractable nuclear antigens (ENA), serum were negative and cerebrospinal fluid (CSF) angiotensin converting enzyme (ACE) level was within normal limits. Microscopy of CSF revealed 10 cells, all of which were lymphocytes. CSF biochemistry showed protein 48 mg/dl and CSF sugar 67 mg/dl (corresponding blood sugar: 98 mg/dl). CSF for oligoclonal bands was positive. The CSF virology assessment for HSV PCR and IgM antibodies against herpes simplex, cytomegalovirus, Japanese encephalitis, varicella zoster virus, dengue and Epstein Barr virus was negative. Enzyme linked immunoassay (ELISA) for human immunodeficiency virus I and II was negative. Hepatitis B surface antigen (HBsAg) and anti- HCV antibody were also negative. Polymerase chain reaction for M. tuberculosis was negative. Cancer antigen 125 (CA-125) level was 45.39 U/ml (normal value below 35 U/ml). Onconeural antibodies could not be performed. The repeat nerve conduction studies revealed left sided pan-plexopathy and right upper trunk plexopathy. Electromyography was suggestive of denervation and showed fibrillation, fasciculations and large amplitude motor unit action potentials.\nMagnetic resonance imaging (MRI) of cervical spine showed mild swelling of the cord along with hyperintense signals within the spinal cord on T2 weighted images. In addition, hyperintensities of multiple radicles were noted bilaterally on T2 fat suppression images []. Incidentally, MRI revealed an ill defined, heterogeneously enhancing lesion (4 × 3 × 3 cm3) in the left upper lung []. Computed tomography (CT) thorax showed well defined heterogeneously enhancing soft tissue attenuation lesion with speculated margin in apico posterior segment of left upper lobe []. Rest of the lung parenchyma was normal and no significant mediastinal lymphadenopathy was noted. CT guided fine needle aspiration cytology revealed malignant epithelial cells lying in clusters in a hemorrhagic background [].\nThe patient received intravenous methylprednisolone 1 gm/day for 5 days followed by oral prednisolone 1 mg/kg/day for one month. After the confirmation of the lung malignancy on follow up imaging, she was referred to oncology and was advised chemotherapy followed by radiotherapy.\nMRI cervical spine [] done after one month of steroid therapy showed disappearance of the cord hyperintensity but the mass lesion had increased significantly in the size (6 × 4 × 6 cm3). The arm pain had responded but weakness persisted. The patient is currently receiving chemotherapy and radiotherapy.
A diabetic 90 years old woman with cough and diffuse abdominal pain was admitted into our hospital with an initial diagnosis of sepsis. Blood sample investigation revealed mild leucocytosis with PRC augmentation and normal AST, ALT and bilirubin levels. Preoperative ultrasound revealed thickened gallbladder wall suggestive of acute cholecystitis. She has had previous sigmoid resection for diverticulitis and ERCP for choledocholithiasis without any mention of biliary abnormality. She was scheduled for a laparoscopic cholecystectomy during which the incidental intraoperative finding of a left sided gallbladder was made. Standard trocar positions were used (A) without any variation of technique, simply following the Strasberg criteria for safe cholecystectomy []. Having seen the anatomical variation (B), a careful dissection of Calot’s triangle was carried out, the cystic duct and artery were identified and isolated, and subsequent complete dissection of the gallbladder from the liver was done (C). This manoeuvre allowed us to demonstrate a right sided positioning of the cystic duct. Furthermore, a safe clipping and transection of the cystic duct and artery was achieved (D), and the gallbladder was extracted in an endobag. The postoperative course was complicated by an aggravation of diabetes and basal pneumonia which were controlled postoperatively in the intensive unit. The patient was discharged on postoperative day 16, in good condition. Retrospective analysis of the clinical case was done and a previous CT scan was reevaluated. Even with the knowledge of a left sided gallbladder, the CT scan was unable to demonstrate its attachment to the left side of the liver. This was probably due to the presence of a hypertrophic left lobe mimicking only a contiguity with the gallbladder, which itself appeared to be in a physiological position (). A vascular and biliary reconstruction was then evaluated with evidence of agenesis of the right anterior sector of the liver () with the absence of the right hepatic duct and an intrahepatic variation with insertion of the right inferior sectorial duct into the left common duct () as type A3 of Huang Classification [].
A 78-year-old Caucasian woman with no known past medical history presented to the emergency department with complaints of bilateral lower extremity edema and erythema with a clear serous discharge. The patient first noticed some small blisters on her left lower extremity which subsequently opened with some clear serous drainage. She also experienced progressively worsening swelling first of the left lower extremity and then her right lower extremity which prompted her to come into the hospital. Except for a cough, the patient had no other symptoms.\nPreliminary evaluation at the emergency department indicated the patient had cardiomegaly and bilateral pleural effusions with a BNP of 1260. She also had atrial fibrillation with rapid ventricular response. The patient was not complaining of any chest pain, fevers, chills, nausea, vomiting or diarrhea. Her chest x-ray showed evidence of cardiac enlargement with interstitial densities and bilateral air-space processes possibly associated with asymmetric pulmonary edema. Large bilateral pleural effusions were noted, with the left greater than the right side. Incidentally, some evidence indicating a compression fracture of indeterminate age involving the upper lumbar/lower dorsal spine was observed.\nThe patient had no known medical history. In fact, she had not been seen by a physician for the last 15 years. Besides taking aspirin occasionally, she was not on any medications. She did not use alcohol, tobacco or illicit drugs. Physical examination did not reveal any specific concerns other than bilateral lower extremity edema.\nCT angiogram of the chest showed large bilateral pleural effusions with bilateral lower lobe enhancing volume loss and worse on the left. Cardiomegaly was noted and there was no evidence for a pulmonary embolus. In addition, there was no evidence of deep venous thrombosis in the venous Doppler studies of her lower extremities bilaterally.\nEchocardiogram indicated the left ventricle with some mild hypertrophy. Systolic function was normal with an ejection fraction estimated at about 60%. The right ventricle was mildly enlarged and mildly hypokinetic. The left atrium showed marked enlargement and the right atrium was moderately enlarged as well. The aortic valve was mildly sclerotic and trileaflet. The pulmonic valve was unremarkable. The mitral valve showed flail motion of the posterior leaflet and there was a small mobile echo seen in the left atrium which strongly indicated endocarditis as the cause for pulmonary edema. Doppler examination showed severe mitral regurgitation that tracked underneath the anterior leaflet along the interatrial septum. There was mild aortic insufficiency. The inferior vena cava was dilated indicating elevated right atrial pressure and the pulmonary systolic pressure was estimated at about 50 mmHg.\nGram positive cocci in diploids, tetrads, and clusters were isolated from three sets of blood cultures. The organism was catalase positive and produced a negative coagulase reaction by SlidexTM (BioMérieux, NC). The Vitek 2 GP card (BioMérieux, MO) identified the bacterium as Staphylococcus warneri. It produced acid from trehalose and lacked phosphatase enzyme, two key characteristics that differentiate this species from S. epidermidis - one of the most recognized CoNS [].\nThe patient was initially treated with Unasyn, 3 grams IV every 6 hours for presumed bilateral lower extremity cellulitis. After the diagnosis of bacterial endocarditis was made and supported by the echo-cardiographic results, the patient was started on nafcillin with gentamicin following consultation with the infectious diseases specialist. In addition, congestive heart failure was treated initially with lasix IV and subsequently with lasix orally. Atrial fibrillation was rate controlled with diltiazem. Gentamicin was later discontinued due to acute renal failure, and the patient was discharged with a PICC line for treatment with 2 grams Nafcillin intravenously every 4 hours for a total of 6 weeks. Repeat blood cultures were negative for 48 hours prior to discharge.
A 51 year-old woman with a past medical history of well controlled asthma, allergic rhinitis and atopic dermatitis was referred to our university clinic complaining of chronic urticaria for the previous three years. She experienced almost daily episodes of hives that would last less than a day and were intensely pruritic. Prior to presentation in our clinic, she was treated by several physicians with various combinations of high dose first and second generation antihistamines without success. Montelukast offered no benefit when added to treatment with antihistamines. However, relief was obtainable with oral prednisone (20 mg/day) or cyclosporine (200 mg daily in divided doses). A biopsy was obtained which confirmed the diagnosis of true urticaria and ruled out urticarial vasculitis. During the course of her work up, a number of laboratory tests were ordered and were unrevealing as to the potential etiology of her hives (Table ). She noted no association of symptoms with food or medications. Despite frequent monitoring, she was fearful of the potential toxic effects from cyclosporine after she experienced a transient decrease in renal function of unknown significance. Additionally, she was intolerant of prednisone when used at times in place of cyclosporine due weight gain and psychosis. In an effort to find a more tolerable and effective alternative, treatment was initiated with omalizumab 300 mg every two weeks. This dose was chosen based upon the severity of her symptoms and previous successful outcomes []. After the first treatment with omalizumab, the patient noted significant improvement. Over the course of the subsequent 2 weeks, she was able to wean cyclosporine down to 25 mg daily without experiencing an urticarial flare. This was the lowest tolerable dose required to prevent flares until the eighth treatment visit at which time she was able to completely withdraw from cyclosporine use. At the start of omalizumab treatment, she would experience only a generalized sensation of pruritus without visible lesions. This symptom was controlled initially with the addition of diphenhydramine 25 mg every 8 hours, and later with this medication used only on an as needed basis. Interestingly, a tolerance to the sedative effects of diphenhydramine developed after a few days of scheduled treatment as has been suggested by other authors []. During the course of the first 28 weeks of therapy she experienced only three episodes of hives that were easily managed. After this initial successful time period, her treatments were spaced to every three weeks with no further symptoms or complications. There were no immediate or late phase hypersensitivity reactions experienced by the patient during treatment with omalizumab.\nPrior to treatment with omalizumab and after consent was obtained, peripheral blood was obtained from the patient and from a control subject with no history of urticaria or allergic disease. After collection, samples were placed on ice, processed within three hours on the same day of collection and analyzed using dual staining flow cytometry to measure baseline expression of FcεRI on the surface of blood basophils (Figure ). Additionally, expression of FcεRI was measured prior to each subsequent treatment over a 52 week period (Figure ). For these experiments, FITC anti-FcεRI and PE anti-CD 123 antibodies (eBioscience, San Diego, CA), along with isotype controls, were added to whole blood. The sample was then treated with BD FACS Lysing Solution (BD Biosciences, San Jose, CA) to lyse the red blood cells. After a series of centrifugation and washing steps with staining buffer (1:10 dilution of PBS and 10% FBS), the cells were fixed with 2% paraformaldehyde and analyzed by flow cytometry.\nWhen compared with the control subject, our patient displayed a five fold greater expression of FcεRI prior to treatment with omalizumab. After the first 14 days of treatment, there was an approximate 80% decrease in the expression of the high affinity IgE receptor that was maintained throughout the duration of treatment. This level of decrease is similar to previous published reports []. While mast cells represent the effector cell implicated in chronic urticaria, these experiments utilized antibodies for two surface markers found on the surface of basophils. It has been previously shown that treatment with omalizumab results in a reduction in free IgE and a decrease in FcεRI on blood basophils []. Previous studies have also reported that after treatment with omalizumab skin mast cells demonstrate a phenotypic shift and a reduction of surface FcεRI, albeit at a slower rate than is seen with blood basophils []. The patient in this study experienced significant improvement after the first treatment, though it was 14 weeks until she was able to completely withdraw from cyclosporine use altogether. This may be due to a slower response for achieving a decrease in mast cell numbers, mast cell function and/or mediator release. Indeed, regulation of mast cell survival is thought to be mediated in part by IgE-FcεRI dependent pathways []. While further studies are needed to fully understand the mechanism of efficacy with this new treatment modality, our study points to the importance of decreased FcεRI expression in this process.
This is a case report of a 57-year old female patient with a history of left nephrectomy due to clear cell RCC in December 2008. The patient was in favorable prognostic categories according to Motzer et al. The patient had no family history of malignant neoplasm and no history of chronic diseases. Due to metastatic disease (metastases in lungs), interferon immunotherapy was used as a first line therapy. The best response to treatment was partial regression according to RECIST criteria (version 1.0). After 11 months immunotherapy was stopped due to the disease progression – enlargement of existing lesions. In April 2010 the patient was qualified to 2nd line therapy with a tyrosine kinase inhibitor (sorafenib). The patient received a total of 8 chemotherapy courses. The response to treatment was stable disease according to RECIST criteria. In December 2010, due to the disease progression – appearance of new focal lesions in the liver, the patient was qualified to 3rd line therapy using a selective mTOR inhibitor (everolimus). Marked toxicity was observed during the therapy – anemia grade 3 according to CTC AE that required transfusion of packed red blood cells. The treatment was stopped after 13 courses in January 2012 due to the disease progression – appearance of new lesions in lungs, left adrenal gland and the skeletal system. The patient underwent palliative radiotherapy on the tumor site, 11th rib in January 2012, and then in June 2012 on central nervous system (CNS) area due to focal lesions located in the right parietal and temporal lobes and in the right cerebellar hemisphere. In May 2012 the patient started a 4th line therapy, axitinib at a dose of 5 mg twice daily. Partial response to treatment was achieved. The treatment was stopped in November 2012 due to the disease progression. The total time to progression was 37.5 months (). The patient died in December 2012. The total survival time from the disease diagnosis was 45 months.\nThe treatment was carried out in accordance with NCCN (National Comprehensive Cancer Network) recommendations and with polish National Health Service.
The male infant was born at 38 weeks of gestation to a 32 year old primiparous mother. He developed respiratory distress within an hour of birth and was transferred to the neonatal intensive care nursery for treatment with supplemental oxygen. The infant was fed colostrum until resolution of respiratory symptoms on day two, when breastfeeding was initiated with use of a nipple shield to aid attachment. Three hourly breastfeeds were supplemented with infant formula or expressed breast milk (EBM) and he was exclusively fed breast milk from day four. The parents were informed of the infant’s clinical features of Down syndrome on the night of his birth and diagnosis was confirmed by genetic testing on day six. Associated health complications including congenital cardiac anomalies, thyroid disease and gastrointestinal malformations such as Hirschsprung’s disease were not identified [].\nThe family was discharged home with instructions to breastfeed and supplement with EBM every 3 h. At 3 weeks the mother was not confident of adequate milk transfer at the breast and concerned about continued use of a nipple shield to maintain attachment at the breast. She felt that her milk supply was reducing and sought help from an international board certified lactation consultant. After measurement of 24 h milk production, informed written consent was provided to participate in a longitudinal study of infant sucking dynamics, milk production and transfer. Studies were conducted at 4, 10, 14, 19 and 24 weeks.\nAt 4 weeks the infant was breastfeeding with a nipple shield and supplemented with EBM after most feeds. The infant was sleepy during both breast and bottle feeding. Chin support and dancer hand techniques [] had been trialled to aid feeding but were not found to be helpful. Simultaneous breast expression was performed twice daily using a hospital grade electric breast pump. The 24 h milk profile confirmed low supply ie. < 600 mL/24 h [] so expression frequency was increased. Nipple shield use was discontinued at 8 weeks due to a maternal sensation of nipple tightness.\nAs the mother was not confident of adequate milk transfer at the breast, an electronic baby scale was loaned so that the family could use test weights to guide supplementary feed volumes. Expressed breast milk supplements were given until 5 months. The mother aimed to feed the infant 800 mL/24 h. Using this strategy the infant maintained a pattern of weight gain considered adequate for male infants with Down syndrome (Fig. ) []. The mother reported that test weighing was useful.\nAt the first visit, weak lip closure and compression without the application of intraoral vacuum was noted during digital examination. Sucking, rooting, gag and tongue thrust reflexes were present. The infant became fatigued during breastfeeding, but maintained an adequate seal and no swallowing difficulties were observed at all monitored breastfeeds. The mother reported that other than rare episodes of gagging, she did not observe any difficulties with swallowing. A speech therapist assessed the infant at 6 months and advised there were no signs of oral phase or pharyngeal phase dysphagia so family foods could be introduced.\nThe infant was regularly reviewed by a paediatrician and was treated for concurrent episodes of otitis media and urinary tract infection at 3 months. He was otherwise well, maintained adequate weight gain and had no clinical signs associated with aspiration and so was not investigated for this.\nAt 24 weeks the dyad was exclusively breastfeeding. The mother was confident of her infant’s milk intake and enjoyed her breastfeeding experience. She now felt certain of achieving her initial goal of breastfeeding for 12 months. Twice daily breast expression continued for the purpose of storing EBM for later use. First foods were gradually introduced from 6 months and breastfeeding continued until mutual weaning at 19 months.
A 9-yr-old boy presented with pain on his left distal thigh for 6 months without any trauma history (date of initial consultation: 2009-12-31). On physical examination, no palpable mass, no tenderness was identified and the range of motion was full. A plain radiograph was taken () and it showed moth eaten both osteolytic and osteosclerotic lesions in the left distal femoral metaphysis. But there was no periosteal reaction or no cortical destruction. Magnetic resonance imaging (MRI) showed T1 low and T2 heterogenous signal intensity intramedullary lesion in the metaphysis which had not crossed the growth plate and no definite cortical destruction was also identified (). A radiologist reported that this case was consistent with an osteosarcoma but the possibility of benign lesion could not be excluded. A whole body bone scan () showed mild increased uptake in the left distal femur. Complete blood count, erythrocyte sedimentation rate, C-reactive protein level and blood chemistry were all within normal limits. An incisional biopsy was performed in the belief of possible malignancy. Pathologic specimen () showed that the lesion revealed many irregular shaped and sclerotic mature and immature bony trabeculae with moderately cellular stroma composed of relatively uniform spindle cells and cartilaginous components without significant cellular atypia. A "diagnosis" was not easily made because of conflicting debates for the diagnosis were issued from the conventional osteosarcoma to low grade central osteosarcoma or benign intramedullary bone forming lesion after case conference discussion and consultations with foreign pathologic specialists. We informed all of the findings to his parents and decided to have a close observation without any further invasive procedures or surgical treatments, because the boy had a just minimal symptom of intermittent pain and the parents agreed.\nThirty-six months after initial visit, radiologic findings from the plain radiographs () and MRI findings () showed no significant interval changes of extent and morphology. A whole body bone scan showed mild decreased uptake in comparison to the initial scan (). Now he is 12-yr-old and clinically he is pain free and has no restriction of any activities until now. There is no sign of growth disturbance. He will be followed annually.
The patient was a 56-year-old woman with recurrent abdominal pain as the main complaint and who had been on maintenance hemodialysis of ESRF for 26 years. She had the operations of total arch replacement 8 years ago and a Bentall 7 years ago for acute Stanford type A aortic dissection. Although dissections still remained on the descending thoracic and abdominal aorta from the previous two operations, the diameter of the aorta remained stable without its enlargement. Therefore, she underwent conservative treatments.\nHer initial abdominal pain with ischemic colitis occurred 1 year ago, and colonoscopy was performed. By the diagnosis of the colonoscopy, we found a white coating in the vicinity of the Bauhin valve and localized distorted ulcers in the ascending colon. However, mucosal necrosis was not found (Fig. ). Since her symptoms were slight and temporary, she remained on conservative treatments at that time. Her abdominal pain became worse; melena occurred often during the course of her conservative treatments, and these symptoms occurred when blood pressure became low under hemodialysis. Then, she was referred to our hospital for further evaluation of her recurrent ischemic colitis. Computed tomography (CT) scans showed chronic B-AD, which extended from the distal anastomotic part of the total arch graft to both common iliac arteries. The distal re-entry size was 26 mm. The expanded false lumen compressed the true lumen of most of the aorta, including the origin of the superior mesenteric artery (SMA) (Fig. ).\nBased on these clinical findings, we considered that this recurrent ischemic colitis was a result of diminished blood flow to the SMA due to the compressed true lumen combined with hypotension due to hemodialysis. We then determined that therapeutic enlargement of the true lumen would improve blood flow to the SMA and reduce the risk of ischemic colitis. For treatment, we decided to perform TEVAR because open surgical repair can be risky for the patient considering the history of previous Bentall operation and comorbid ESRF. Because the patient’s quality of life was adversely affected by recurrent ischemic colitis, she agreed to undergo the procedure we suggested, and informed consent was obtained. The operation was performed under general anesthesia. An incision was made in the right groin to expose the right femoral artery. A 6Fr sheath introducer was inserted percutaneously from the left common femoral artery for aortography. A 8Fr sheath introducer was inserted from the right exposed common femoral artery, and we delivered a guidewire and catheter with contrast radiography to pass into the compressed true lumen. The proximal entry tear in the patient was covered by a Zenith TX2 stent graft (diameter, 32–28 mm; mean length, 200 mm; Cook Medical, Bloomington, IN), supplemented by a noncovered aortic stent (TXD; diameter, 36 mm; length, 164 mm; Cook Medical) around both renal arteries, the SMA, and the celiac artery (CA). The TX2 was deployed just distal to the left subclavian branch to seal the entry tear (Fig. a). The noncovered aortic stent (TXD) was deployed from above the CA to under both renal arteries to expand the true lumen and to improve blood flow of the CA and SMA (Fig. b). The operation time was 2 h and 33 min, and blood loss was 150 cc. The patient tolerated these procedures well and was transferred to the postoperative care unit in a hemodynamically stable condition. Her bowel condition improved just after the operation.\nSeven days after the operation, CT showed improved blood flow in the true lumen, CA, and SMA, and the false lumen tended to be thrombosed (Fig. ). She was discharged 10 days after the operation without any postoperative complications. She then underwent hemodialysis three times per week after the operation, but her abdominal pain following hypotension due to hemodialysis had completely disappeared. She remained well for 6 months after the operation.\nWe experienced successful TEVAR for complicated chronic B-AD. The patient had ischemic complications 8 years after the conservative treatments for chronic B-AD. This clinical course is rare because it took a long period of 8 years for ischemic colitis to appear after the occurrence of B-AD []. Furthermore, the occurrence of ischemic colitis on this patient was not easily recognized solely through the history of hemodialysis-dependent ESRF. Ischemic colitis is a well-known complication of ESRF [], and abdominal pain after hemodialysis can then often occur because of hypovolemia. Preoperatively, in our patient, we observed that ischemic colitis under hemodialysis was coincidentally worsened by chronic B-AD. Interestingly, however, the abdominal ischemic symptoms disappeared completely after TEVAR. This clinical course suggested that the ischemic colitis was mainly caused by chronic B-AD and the relative hypovolemia following hemodialysis which had worsened the ischemic condition. Therefore, physicians should carefully evaluate the effect of stable chronic B-AD on ischemic colitis considering ischemic colitis may take a long period of time to appear after chronic B-AD and diagnosis of ischemic colitis is difficult.\nWith regard to treatment of B-AD, TEVAR is less invasive and an effective treatment compared with open surgery []. Fattori et al. demonstrated that operative mortality and complications were 33.9 and 40 % in open surgery compared with 10.6 and 20 % in TEVAR, respectively []. Hogendoorn et al. also reported that the 30-day mortality rate was 17.5 % in open surgery and 10.2 % in TEVAR []. In addition, TEVAR has better aorta-specific survival and delayed disease progression than conservative treatment []. Currently, TEVAR is a good treatment option for B-AD and is expected to be effective, even for complicated cases.\nIn the present case, the purpose of surgical treatment was to expand the true lumen and improve blood flow into the SMA. No aortic dilatation or rapid expansion was found in our case. We considered that our patient had many risks for surgical treatment because of her comorbidities. Therefore, we performed TEVAR with the TX2 in the proximal site to seal the entry tear and the TXD was deployed at the SMA to expand the true lumen and improve blood flow in the SMA. We did not expect that TEVAR above the CA could demonstrate the proper radial force to expand the true lumen below the CA because the dissection had occurred 8 years ago and the vessel wall could be highly sclerotic. In our case, organ ischemia was severe enough that an improvement of SMA blood flow was required. With regard to the distal site, the TXD which was composed of a noncovered aortic stent contributed to maintaining the opening of the origin of the SMA. At the same time, use of a bare metal stent allowed the remaining flow to the false lumen. In general, such endoleak is a risk for progression of aortic dilatation. TEVAR sometimes causes retrograde aortic dissection, which often requires immediate open surgical repair []. Therefore, covering the true lumen and extending into the thoracoabdominal aorta with a noncovered aortic stent to diminish retrograde endoleak could be important. In addition, flow to the artery of Adamkiewicz could contribute to prevent acute vertebral ischemia.\nIn our patient, TEVAR supplemented with a noncovered aortic stent was successful as the initial procedure. However, the long-term outcome is also important. The purpose of TEVAR for chronic B-AD includes not only improving prognosis but also preventing future surgical intervention of aortic dilatation []. Physicians need to be aware of other complications of TEVAR, such as stent-graft collapse, stent-graft migration, stent-graft torsion, aortoesophageal fistula, mobile thrombus within the stent-graft lumen, aneurysm formation, and late rupture. These complications warrant the importance of close and lifelong imaging follow-up of these patients []. Therefore, after TEVAR for B-AD, patients should be carefully followed up to identify any complications.
A 79-year-old male presented to a district emergency department having been sent in by his GP for review of a left leg ulcer. His history included type 2 diabetes mellitus. In spite of living independently at home, he was noted to have dementia with significant cognitive impairment.\nThe patient was a vague historian and was unable to give sufficient details regarding the genesis or duration of the wound. He stated the ulcer might have been present for two weeks and had occurred subsequent to a fall. No further information regarding the cause of the ulcer could be elicited. His GP had treated the ulcer unsuccessfully with a one-week course of antibiotics prior to presentation. On examination, the patient was noted to be malnourished and cachectic with significant lower limb oedema. Mini Mental State Examination on review was 12/30. Circumferential ulceration of the left lower leg was noted (). The wound was approximately 2 cm wide and extended through the fascia throughout the wound. There was a large burden of tenacious slough, obscuring the base of the wound. The presence of larval infestation was identified. The wound was dressed, antibiotics were commenced, and the patient was transferred to a tertiary referral hospital for review and management by the vascular surgery and geriatric medicine teams.\nThe patient was reviewed in the emergency department of the tertiary hospital where the wound was inspected before transfer to the ward. A plain radiograph of the area of concern noted a soft tissue defect with no bony abnormality. On review of the wound by the admitting team, the peculiar morphology of the ulcer was noted. The uniform circumferential nature of the wound resembled that of a ligature type injury. Additional questioning however revealed no further clues to elucidate the causative mechanism. The patient denied any process that could adequately explain the circular nature of the lesion. A management plan of elevation, compression, antibiotics, and a dressing regime with the aim of eventual grafting was commenced.\nThe wound was debrided on the ward, removing the bulk of the tenacious slough covering the wound bed and revealing a clean wound with the base visible. This cleaning demonstrated a thin, tan coloured structure running transversely through the wound. On closer inspection, this was found to be an elastic band, that was constricting the leg down to the level of the fascia and was clearly the aetiological factor precipitating the wound. This had not been previously visible due to the thick layer of slough enveloping the wound. The band was transected and removed (Figures and ). Further discussion with the patient gave no further insight into how, when, or why the band was placed.
We present a case of a 53-year-old female who presented with a six-week history of painful subcutaneous masses. Various soft tissue nodules had been present for multiple years; however, during the last few weeks prior to the initial evaluation, she developed an alarming increased number of widespread soft-tissue masses with significant tenderness in comparison. Her daily activities are often interrupted due to the menacing symptoms, causing significant attrition to her quality of life. Multiple attempts of over-the-counter trials of pain medications have failed to provide the patent with any relief and prescription pain medications are but a bandage for her illness. The patient has a significant past medical history including obesity (BMI 35), chronic atrial fibrillation, chronic obstructive pulmonary disease (COPD), diabetes, hypertension, and sick sinus syndrome status post atrial pacemaker placement.\nDuring the initial presentation, ultrasound examination revealed hyperechoic foci in the subcutaneous tissues that were circumscribed without any Doppler flow, consistent with lipomas. The patient then underwent excision of multiple back nodules to further characterize the lesions, revealing a gross pathology report of adipose tissue with nodularity. An interesting point to note is that the rate at which new palpable nodules developed was intriguing. She developed four new identifiable lesions in various parts of the body in the short interval between her office visit and the date of the operation. Histopathology reports one of four specimens demonstrating portions of mature adipose tissue consistent with lipoma, and three of four specimens with mature adipocytes and branching network of small vessels consistent with angiolipoma.\nShe was seen in the postoperative period with a continued manifestation of these subcutaneous nodules throughout her body. The pain along the sites of excised lesions had improved but she continues to have significant tenderness along the areas of new masses. The patient's case was further reviewed and labeled with the diagnosis of Dercum’s disease - type 2 []. The patient again was seen in the surgery clinic at our institution with subsequent referral to endocrinology for further evaluation and to the pain clinic for multimodality treatment. In the interim, she continues to develop new painful collections of fatty tissue masses.
This is a case of a 25 years old Malay girl with learning disability and no significant past medical history, who started noticing a sacral mass since August 2015. The mass was painless and gradually increasing in size. The family members of this patient brought her to a traditional healer. They did not seek any medical treatment until late 2017. By this time, the mass over the sacrum was extremely large. Family members claimed the mass was preventing the patient from lying down flat supine. The patient was also unable to ambulate for the past 2 years. Hence, she was bedbound most of the time. It was difficult for her to sit on the wheelchair. She also felt tired to move because the mass was quite heavy. The family members claimed when the patient was lying down flat, she had to flex her hips and knees to achieve a more comfortable position. In addition, she often slept either in prone position or in supine with multiple pillows below her body. The mother also claimed over the last 2 months, the patient’s body had been getting thinner despite her physical weight was increasing due to the increase in size of the sacral mass. The patient had been passing stool and urine in pampers. There was no past medical history and no family history of cancer. Socially, the patient lived with her mother and siblings. The mother was the main care taker. Her father passed away 10 years ago because of heart attack. The patient previously attended a special needs school, but she stopped going to school since 2015 after developing the sacral mass.\nThis patient was managed in the Southern Region referral centre for Orthopaedic Oncology in Malaysia. On clinical examination in the Orthopaedic Oncology ward, the patient appeared cachexic, she had slightly pale conjunctiva, but she was not dysmorphic. Vital signs were Blood Pressure 142/90, Pulse Rate 98 beats per minute and Temperature 37 degrees Celsius. There was a large mass 40 cm × 30 cm × 20 cm over the sacrum. The mass was firm to hard in consistency and involved both buttocks and the gluteal fold (Fig. ). Dilated veins were noted under the skin overlying the sacral mass. Neurological exam of bilateral lower limb was normal. However, there was generalized wasting of all muscles over the bilateral lower limb. Anal tone was intact.\nLaboratory investigations taken were unremarkable. Computed Tomography of the Pelvis showed a large destructive sacrococcygeal mass measuring 43 cm × 38 cm × 27 cm with extension into the presacral space resulting in anterior displacement of the rectum, urinary bladder and uterus and posterior extension into the dorsal soft tissue with involvement of the gluteus, piriformis, and left erector spinae muscles (Figs. and ). Superior margin of the sacral bone involvement was up to S2. The mass was predominantly of fluid density with internal enhancing septation and calcifications which suggested primary chordoma more likely (Figs. and ). Magnetic Resonance Imaging done showed similar findings. Skeletal Survey Radiograph did not show any distant metastasis. A Trucut biopsy of the mass was done. Histopathological analysis showed tumour cells with “physaliphorous cells” positive for pancytokeratin, EMA, Vimentin and S-100 immunohistochemistry stainings with minimal mitotic figures and mild nuclear pleomorphism (Fig. ). Brachyury immunohistochemistry staining was not available in our centre. However, the clinical history, morphology of tumour on microscopy and immunohistochemistry staining available were consistent sacral chordoma.\nThe diagnosis of Sacral Chordoma was confirmed. Multidisciplinary team discussion done among Orthopaedic Oncology, General Surgery, Obstetrics and Gynaecology, Blood Bank, Anaesthetic and Plastic Surgery teams. A family conference was done. The family’s aim was for removal of the sacral mass to allow the patient lie supine on bed and sit on the wheelchair.\nSubsequently, the patient undergone Wide Resection and En Bloc Sacrectomy. The Posterior-Only Approach was used with a “Mercedes Star” 3 limbed incision. Duration of surgery was 8 h. The patient was supported with blood products transfusion during surgery. Intraoperatively, the sacral tumour had eroded the sacral bone from S2 to S5 (Figs. , and ). Sacrectomy was done at the level of S2. Sacral nerve roots S2-S5 were all infiltrated by the mass and therefore were unable to be preserved. The mass and surrounding gluteal muscles invaded by the tumour were also all resected. All resection margins were less than 1 mm from the tumour. Primary closure was done without any distant or local flap as per consultation with Plastic Surgery team. The tumour weight was 25 kg (Figs. , and ). Post operatively, the patient was monitored in Intensive Care Unit for 3 days. The patient developed neurogenic bowel and bladder post sacrectomy requiring enema and long-term urinary catheter. In addition, the post-operative course was complicated by wound breakdown and surgical site infection requiring wound debridement. Dressing was done as per local protocol until wound bed granulating well. Split Skin Graft was done about 3 months post wide resection once the tissue culture results were free of significant infection.\nThe patient also required extensive rehabilitation for transfer, ambulation and bowel and bladder care. Rehabilitation was difficult because the patient had learning disability and she had been habitually keeping her hips and knees flexed because of the sacral tumour for the past 2 years. During the last review 5 months post operatively, patient was able to sit on the wheelchair comfortably. The surgical wound was healing well with good uptake of the Split Skin Graft (Fig. ).
A 56-year-old male patient presented to head and neck clinic with an ulcerative lesion in the right temporal area of the scalp since the last 3 years that gradually increased in size and associated with mild pain. He denied any history of other skin lesion or swelling in the neck. He has no history of smoking, alcohol consumption, or sun exposure. On physical examination, the lesion was an ulcerative measuring approximately 3 × 3 cm. There was no palpable cervical lymphadenopathy. No pre- or postauricular lymphadenopathy. No other suspicious skin lesions of the face or the neck were found. The rest of the examination was unremarkable. Head and neck computerized tomography (CT scan) did not show any evidence of pathologic lymph nodes or masses. Staging with CT scan of the chest, abdomen, and pelvis was also negative for metastatic disease. The patient underwent complete local excision and split-thickness skin graft. Histopathology assessment revealed skin tissue infiltrated by neoplastic growth composed of tubules, cords, and nests of malignant cells. The extensive perineural invasion was seen. There was no lymphovascular invasion. The resection margins were clear. The pathology confirmed an apocrine cutaneous carcinoma [see ]. The case was reviewed in the head and neck tumor board and the decision of no adjuvant treatment. 1.5 years later, the patient presented to the combined head and neck oncology clinic with right neck lymphadenopathy for the last few weeks. On physical examination, there was around 2 × 2 cm mass in the right posterior neck looks like a lymph node. CT scan of the head and neck revealed 1.8 × 1.5 cm right neck lymph node at the level Va with central necrosis [see ]. FNA biopsy was taken and histopathology reported malignant cells to consist with metastatic of cutaneous malignancy. The patient underwent right modified radical neck dissection and the histopathology reported the diagnosis of metastatic cutaneous apocrine carcinoma. The case again discussed in the tumor board and the plan was to give adjuvant radiation therapy. No chemotherapy was given. During his follow-up for the last few years, the patient was from the disease on the clinical and radiological evaluation.
A 40-year old female patient reported with severe pain and burning sensation in her right buccal mucosa. Intra-oral examination revealed a white to blackish elevated lesion on the right buccal mucosa in the second and third molar region measuring about 4x5 cm in size. An extra-oral swelling was also evident, which appeared normal in color but was tender on palpation. During elicitation of detailed history from the patient, it was revealed that patient was tobacco chewer since last 20 years and had leukoplakia patch on the right buccal mucosa. An incisional biopsy was advised for the leukoplakia at a private dental hospital. However, immediately after the local infiltration into the buccal mucosa, the patient complained of severe pain and burning sensation at the site of injection. On investigation of the local anesthetic bottle, it was found that 2 to 3 cc of 10% formalin was injected accidently, as both solutions were stored in similar containers. Formalin container was placed on the operating table for fixation and transport of the biopsy sample. After recording the case history, administration of 2 mL dexamethasone injection, in and around the lesion, was one of the immediate measures taken to reduce inflammation. The extra-oral swelling was noted to increase progressively for the first 24 h, though pain reduced over a period of time. The patient was kept under strict supervision and monitored for any adverse cardiac or pulmonary event for 48 h. Medications prescribed included injection dexamethasone 8 mg twice a day (tapering doses: 8 mg once a day for 2 days and 4 mg once a day for 2 days), injection diclofenac sodium thrice a day to reduce pain, injection taxim 1 gm twice a day for 5 days, injection metronidazole 400 mg thrice a day for 5 days and oral rinses with 2% betadine solution to avoid development of any local infection. Patient was subject to liver and renal function tests and the findings were reported to be within the normal ranges.\nAfter a period of 48 h of observation, the patient was discharged at her request against the medical advice. A follow-up visit was scheduled after 7 days for evaluation of the intra-oral site of injection. In the follow-up visit, necrotic tissue at the site of injection measuring approximately 2x3 cm in size was noted (). Once again the patient was admitted and removal of the necrotic tissue and reconstruction of the defect was planned under general anesthesia. The necrotized tissue, which was of 4 to 5 cm penetration depth, was excised and the defect was reconstructed with bovine origin sterile commercially available collagen membrane (Kollagen, Eucare Pharmacuticals Private Limited, Chennai, India) ( and C). Patient was kept on Ryle’s tube for feeding to avoid any secondary infection at the site of defect. In the subsequent follow-up visit after 2 weeks, the oral tissues appeared healthy with no post-operative scar formation ().
The patient was a 76-year-old man who came to the office in 2014 looking for possible treatments of his fractured central incisors. Nothing was found relevant about his medical condition. The patient shows a high risk for caries and also eccentric bruxism. He has partial edentulism in the superior left quadrant and multiple decay and fractured teeth. The initial approach was conservative aiming to keep the upper front by means of composite fillings (Figures and ). Then, the posterior superior quadrants needed to be restored with implants.\nThree years after, in 2017, the patient came back to the office referring pain of endodontic origin in the upper left canine. New and secondary subgingival caries were found in the six front teeth. The conservative prognosis was considered poor due to the subgingival depth and extent of decay presented by the lesions from canine to canine. After having discussed the treatment options, especially the surgical lengthening of the front teeth or the orthodontic extrusion, the patient decides to replace the residual teeth with a new implant-supported bridge similar to the recently performed prostheses of the posterior areas that were judged by him as a highly satisfactory treatment. The patient preferred not to involve these restorations in the present anterior treatment and limited it to place only two implants in the lateral incisors' positions ().\nThe treatment was carried out in a staged approach. Briefly, first, we extracted the lateral incisors, using the SST, and placed two immediate implants. The four residual teeth were then prepared to be used as abutments of a temporary bridge for the purpose of maintaining the aesthetics and function of the patient during the early osseointegration period. In a further step, the four remaining teeth were also extracted using the SST, and the initial provisional bridge was replaced by the second provisional screwed on the uncovered implants. Only one out of the four abutment teeth used for the temporization of root canal treatment was needed due to a periapical infection.\nWhen placing the two immediate implants into the alveolus of the lateral incisors, a section of the buccal part of the root (about the two middle thirds) was left in place and no biomaterial was used at all. An impression of the implants was taken to have the second temporary bridge available in the second surgery. Healing abutments were then attached with the proper height for the soft tissue to cover them but at the same time facilitating the uncovering. Finally, a temporary acrylic bridge was cemented onto the four abutment teeth 13-11 and 21-23 ().\nThree months later, the implants were uncovered, the four abutment teeth were extracted, again with the SST—partial extraction of the roots—but this time no more implants were placed in these sockets. The first provisional cemented onto the teeth was then replaced by a second acrylic bridge screwed onto the implants though temporary abutments ().\nThe partial extraction of the canines, aiming to leave a buccal slice of the root, was so hard to perform, and further instrumentation would lead to the socket destruction that a decision was intraoperatively made and a greater portion of the root, including the apex, was finally left. As the locations of the canines did not involve the implant sites, any potential complication could be addressed efficiently.\nOne month later, the prosthodontic phase was undertaken. Little if any differences in the buccal tissue volume and no noticeable aesthetic impact could be found after the multiple extractions (). The desired position of the incisal border was determined by various try-ins, and five months after implant surgery, the definitive prosthesis was placed. The final clinical aspect can be appreciated in the pictures (Figures and ).
A 57-year-old male patient reported to our department of oral medicine and radiology with a complaint of painful ulcer on the left side of the palate and unhealed socket for 2 months. History of the present illness revealed pain and discharge from the palate for 2 months. Pain was insidious in onset, dull, gnawing, intermittent in nature, lasts for hours, and relived on its own. Pain was associated with discharge. There was no history of paresthesia or anesthesia in the region of area of interest. Past medical history revealed that the patient was a known diabetic for 5 years and not on any medications. Past dental history revealed that the patient reported to a local dentist 2 months ago with tender swelling of gums and loosening of teeth. The condition was diagnosed as advanced periodontal disease with severe bone loss and periodontal abscess involving the left maxillary canine. The patient had undergone localized oral prophylaxis followed by extraction of the involved tooth under local anesthesia and oral prophylaxis was later completed. He was advised to maintain good oral hygiene and replacement of missing tooth once the socket heals. Personal history revealed that the patient was a smoker and smoked 2–3 bundles of bidi for 35 years. General examination revealed that he had poor built and nourishment with the presence of pallor and signs of anemia. The patient had submandibular lymphadenopathy without any evidence of tenderness or swelling extraorally. Intraoral examination revealed missing left maxillary canine, generalized signs of severe periodontitis, and exposed necrotic maxillary bone in the palatal area with a necrotizing ulcer. The ulcer extends mediolaterally from the crest of alveolar bone at canine region toward the midline of hard palate and anteroposteriorly from palatal gingival region of the left maxillary central incisor to the left maxillary second molar. The ulcer was covered by brownish yellow slough, deprived of soft tissues. The ulcer measured around 3 cm × 5 cm in size and had denuded bone in its floor with irregular margins and sloping edges. The slough was scrapable with evidence of bleeding and the surrounding mucosa was indurated. Soft-tissue margins around the necrotic bone appeared normal with no signs of inflammation or purulent discharge []. Based on clinical findings and history, a provisional diagnosis of deep fungal infection and a differential diagnosis of chronic nonhealing ulcer, mucormycosis, and midline lethal granuloma were made.\nThe history of extraction, uncontrolled diabetic status and nonhealing wound with wide extensions, and osteomyelitic type of destruction of the left maxilla in the absence of systemic signs and symptoms of bacterial osteomyelitis prompted us to investigate further. The patient was later subjected to the following biochemical and blood investigations such as hemoglobin, which was 10.2 gm%, and fasting blood sugar, which was 183 mg/dl. Glycosylated hemoglobin value was found to be 8.2% and was suggestive of diabetes mellitus. Cytological smears were prepared from the lesion which revealed numerous fungal hyphae intermixed with bacterial colonies []. Biopsy under local anesthesia was planned and specimen was obtained from the palatal mucosa and, on histopathological examination, necrosed bony trabeculae showed empty bone lacunae []. However, deeper sections in histopathological examination on lower and higher magnification (×10 and × 40, respectively) revealed cellular connective tissue stroma with large nonseptate (aseptate), with randomly spaced branches and nonparallel sides and broad fungal hyphae along with few areas of necrosis and focal areas of mixed inflammatory cell infiltrate []. Periodic acid–Schiff (PAS) and Grocott's silver methenamine (GSM) staining further showed broad, thick-walled infrequent nonseptate with randomly spaced branches and nonparallel sides indicative of mucormycosis [Figures and ]. Based on histopathological findings, a final diagnosis of mucormycosis associated with candidal osteomyelitis of the palate was made.\nFirst and 2nd weeks: Local surgical debridement was planned and done under local anesthesia. The patient was advised for mouth rinse with 2% diluted hydrogen peroxide and Betadine® mouthwash (povidone-iodine USP 1.0% w/v) 3 times a day after meals for 15 days. Medical treatment included use of antibacterial drugs such as amoxicillin 500 mg and potassium clavulanate 125 mg, 2 times a day after meals for 5 days. Anti-inflammatory drug such as aceclofenac 100 mg in combination with paracetamol 325 mg, 3 times a day (6–8 hourly), was also given for 5 days. The patient was recalled after 15 days. He was also referred to a general physician for evaluation of diabetes and treatment of the same.\nThird–10th weeks []: The patient was advised to use clotrimazole 1% w/w mouth paint, 3–4 times a day, for local/topical application and ketoconazole 200 mg 1 tablet daily for 7 weeks for systemic use. He was also advised to take supportive therapy for 2 months which included 5 mg folic acid tablet once daily and iron therapy available as “Syrup Haem Up” 30 ml daily before meals. The patient was followed up after 8 weeks.\nEleventh–13th weeks: Surgical debridement was repeated and the same treatment was continued for 3 more weeks.\nFourteenth–17th weeks: A remarkable healed alveolar mucosa was observed in the regions of 23 and 24 with regeneration of palatal mucosa in the posterior part of the denuded bone and area of denuded bone reduced up to 50% []. The patient was again recalled after 4 weeks.\nEighteenth week: Significant healing of denuded mucosa and bone was seen and the lesion healed up to 80% with no fresh complaints []. The patient was advised to continue the same treatment for the next 2 weeks so that the total duration of treatment becomes 20 weeks (5 months). Unfortunately, the patient was lost to follow-up after this visit.
A 32-year-old multiparous woman was admitted at 37 2/7 weeks for induction of labor for abnormal antenatal testing. She had a history of a prior cesarean delivery for arrest of active labor at 7 cm and after extensive counseling chose to undergo a TOLAC. The initial cervical exam was 2 cm dilation and 50% effacement. Her labor was induced with oxytocin and amniotomy was performed at 5 cm dilation with clear fluid noted. She progressed from 2 cm to 5 cm over the course of 10 hours; interval time to progression to 6 cm was 3.5 hours. The patient then began to have a moderate amount of vaginal bleeding with associated minimal fetal heart rate variability and variable decelerations that did not improve with intrauterine resuscitation. She was taken to the operating room for repeat cesarean delivery due to concern for a uterine rupture and/or a placental abruption. Upon entry into the abdomen, there was no uterine rupture or dehiscence, nor was there evidence of a significant placental abruption. She delivered a male infant with a weight of 2659 g and Apgar scores of 9 and 9 at 1 and 5 minutes, respectively; arterial cord pH was 7.26. No extension of the hysterotomy was noted following delivery of the infant. Following repair of the hysterotomy, the bladder was noted to be distended and edematous, despite the presence of a patent Foley catheter. This finding prompted a vaginal exam to assist in evaluating the integrity of the lower uterine segment behind the bladder due to concern for an occult uterine rupture or dehiscence. The vaginal exam revealed a spontaneous detachment of the anterior rim of the cervix from approximately 9 to 3 o'clock. The avulsed portion of cervix appeared necrotic () and there was no bleeding noted at the site of cervical detachment. The cesarean delivery was completed, and an attempt was made to repair the site of the detachment vaginally, at which time the entirety of the cervix completely detached. No excessive bleeding was noted and placement of compression sutures at the site of detachment helped ensure hemostasis. Her recovery and postpartum course were otherwise unremarkable, and she was discharged on postoperative day #3. The patient was examined at a six-week postpartum visit. No cervix was seen on speculum exam and on digital exam the cervix was flush with the vaginal vault. The pathology report for the detached cervix showed diffuse hemorrhage and vascular congestion; there was no pathologic evidence of placental abruption.
The 72 year old male patient was referred to our clinic with the complaint of bilateral axillary masses and numbness in the right hand. In his medical history, he had hypertension for 4 years but no history of trauma. A physical examination showed the presence of palpable pulsatile masses in his both axillae (12 cm in size in the right axilla and 8 cm in size in the left axilla) (Fig. ). His neurological and systemic examinations were within normal limits. An upper-extremity ultrasound examination revealed an aneurysm in a 12-cm segment of the right axillary artery, reaching 67 × 45 mm in dimension. There was a 27-mm thick thrombus at the edge of the aneurysm lumen. Adjacent to the aneurysm, there was a 48 × 40 mm hyperechogenic mass not related to the aneurysm. There was also an aneurysm reaching 40 × 43 mm in dimension in a 9-cm segment of the left axillary artery, with a 17-mm thick thrombus. A Doppler ultrasonic examination revealed a thrombus surrounding the lumen and a turbulent, pulsatile flow in the center. The aneurysmal segment was continuous with the brachial artery. There was no blood flow in the region adjacent to the right axillary artery (Figs. and ). On CT angiography, giant fusiform aneurysms were detected, measuring 140 × 77 mm in the right axillary artery and 93 × 45 mm in the left axillary artery. There were thrombi in both aneurysms, allowing the flow in the lumen. There was also an extension to the brachial artery and contour irregularities at the distal end of the right-side aneurysm. These latter findings were proposed to be due to a spontaneously healed rupture. A physical examination of the eyes, bones, skin and joints revealed no signs of collagen tissue disease, nor was it detected through laboratory examinations. Because the patient was symptomatic and had a high risk of aneurysm rupture, endovascular closure was initially planned. However, appropriate size stents could not be found for aneurysms so large and tortuous. Besides, the long-term success of the stenting procedure could not be guaranteed due to the large sizes and high mobility of the aneurysms, so the patient was referred for surgery.
This 36-year-old man presented with a three-month history of headaches which he attributed to sinusitis. He took several courses of antibiotics which did not relieve his symptoms. He began to gradually develop fullness and a lump in the midline of his forehead over the frontal sinus region. Sinus plain radiographs demonstrated ‘sinusitis’ for which he was referred to an otolaryngologist who arranged a paranasal sinus CT scan. This scan demonstrated a bone destructive lesion of the frontal sinus. A further MRI scan confirmed the presence of a frontal sinus tumour without intracranial extension or extension into the ethmoid bone. The tumour was limited to the lower two-thirds and anterior wall of the frontal sinus with obstructive changes in the superior part of the right frontal sinus. Fine needle aspiration biopsy of the lesion revealed a squamous cell carcinoma. He was otherwise fit and well barring mild hyperlipidemia. He has no family history of sinus cancer and did not take any medications. He did not smoke but drank alcohol two to three times a week.\nOn examination there was a poorly-defined smooth swelling approximately 4 cm in width on the forehead in the midline just about the eyebrows. There was normal skin sensation on the forehead bilaterally and no cranial nerve abnormalities. No other palpable masses were present in the head and neck. Otolaryngological examination was normal. There were no other abnormalities detected on general examination. The diagnosis of primary squamous cell carcinoma of the frontal sinus was discussed with the patient along with the possible treatment options. It was agreed that the optimal treatment option would be surgical resection of the primary tumor followed by postoperative radiochemotherapy.\nIt was agreed with the patient that the optimal treatment option would be surgical resection of the primary tumour followed by postoperative radiochemotherapy. The procedure was done conjointly between the head and neck and neurosurgery teams. This case offered a surgically challenging frontal craniotomy with resection of the frontal sinus and reconstruction of the frontal bone.\nThe initial access and exposure was attained via a bicoronal incision was fashioned taking care to spare pericranium. Pericranium was dissected posteriorly and then retracted anteriorly with the flap. The tumour extended proximally 6 cm from nasion and the anterior scalp flaps were raised up to the level of the supraorbital ridge. The galeal pericranial were left on the tumour, which was dissected down just inferior to the nasion on the nasal bridge. The flap overlying the tumour was raised superficial to the galea whereas at all other points, the flaps were raised deep to the pericranium. Having opened this two fish hook retractors were laced and the resection stage of the operation could be begun.\nThe area to be excised was marked out using 1:1 scalp films that were previously obtained. The craniectomy was started using a Mida-Rex saw around the frontal sinus where the resection was marked out. This began at the top of the frontal sinus overlying the frontal bone to ensure a wide margin around the tumour and was brought down until the supraorbital region. The tumour was circumferentially drilled in the frontal sinus taking care not to enter the tumour with the M8 bur on Midas-Rex drill. This was drilled into the frontal sinus bilaterally and the posterior wall frontal sinus was likewise taken with M8 bur on Midas-Rex drill.\nDura was identified and was dissected from superior to inferior down to the base of skull. A small dural tear was encountered, which was sewn with 4-U Surgilon stitched in a water-tight fashion. These bone cuts were then extended over the supraorbital region and the glabella and towards the nasal bone. This was done on both sides. On the left side, the bone cuts were made further inferiorly to include the suraorbital ridge and in the process, the Periorbital fat was exposed and the globe retracted downwards to prevent any injury. All this was done using a 2-mm burr. We then switched to a power saw to cut through the nasal bone in midline.\nOnce this was done, an osteotome was used to carefully chisel out the bone cuts and the entire specimen was removed en bloc and sent for routine histological examination. We then subsequently excised the frontal sinus mucosa on either side and sent that for routine histological examination as well as the mucosa of the nasofrontal ducts on either side. Once this was done, several small dural tears that were noted were repaired primarily using Nurolon, and the final reconstruction stage of the operation began.\nA bone flap was subsequently harvested from the right parietal region and fashioned into shape to fit into the frontal sinus defect. A galeal pericranial flap was raised/harvested from the scalp flap and this was divided in midline to get two long strips, the left side was sutured down to the sinus mucosa to completely separate the nasal cavity from the bone flap itself. This was sutured all around the sinus mucosa circumferentially using 4-0 Surgilon stitches and at points required drill holes to be drilled into the frontal sinus bone and this was sutured in place.\nThe bone plate, a 6 × 5 cm graft, was taken from the right side calvarium taking care to stay off the midline and anterior to the motor strip. This was taken using a craniotome followed by the B1 bit on Midas-Rex drill. This was contoured to fit the defect in the frontal sinus and was placed using the Neuroprobe miniplate system. The right leaf of the galeal and pericranial flap was then laid over the bone flap and sutured in place as well.\nPorex graft was also cut 6 × 5 cm and contoured to fit the defect where the skull had been resected for donor graft harvest. The wound was copiously irrigated with saline and bacitracin. A Relia-Vac drain was laced in the epidural space and exited through separate stab wound. The wound was closed in layers and the galea was closed with inverted 2-0 Vicryl and staples were used for skin. Xeroform and clean dry dressing were applied. The patient tolerated the procedure well. In the post-anaesthetic care unit, the patient had no neurological deficit and was easily arousable.
A 65-year-old Japanese woman was referred by her local dentist to our hospital for retrieval of a fragment of Peeso reamer that had broken during a root canal enlargement and that was embedded in the canal of her right mandibular first premolar, extending beyond the apex (tooth number 44). As it was not possible to remove the mandibular foreign body from the root canal at the referral dentist, she was admitted to get it removed using a surgical approach. During the intraoral clinical examination, there was an access cavity filled with a temporary filling material and the tooth was sensitive to pressure. There was tenderness in the apical portion of the tooth. A panoramic radiograph obtained at the initial visit after the patient referral revealed a well-defined fractured instrument lying in the mandible beyond the apical foramen of her right mandibular premolar tooth. Moreover, computed tomography (CT) images revealed that the fractured segment was located within the mandibular bone (Fig. ). A diagnosis of migration of a broken endodontic instrument beyond the apical foramen into the mandible was made, and surgery was considered necessary to eliminate the symptoms. The decision was made to proceed with the surgery because of increasing pain.\nSurgical intervention was required to find the apical root cutting-edge lesion and the broken dental instrument through a small bony window and to remove it from the oral cavity by pushing up in order to reliably preserve the tooth for restorative treatment. Therefore, we decided to use three-dimensional navigation-guided surgery for its minimally invasive nature and high surgical accuracy. As it is difficult to synchronize with the preoperative imaging data during surgery due to the mobile nature of the mandible, we used a customized interocclusal splint for repeatable mandibular positioning while enabling surgical access. As part of the CT imaging preparation for navigation, a customized interocclusal splint was fabricated by pressing acrylic resin into a dental mold at the first visit. First, the created bimaxillary upper and lower jaw splints were adhered using resin at a stable position of the mandible, that is, the position in which the mandible is slightly opened from the central occlusion. A maxillofacial CT was obtained using the customized interocclusal splint to maintain the mandible in a repeatable position, which would be vital for the accuracy required for retrieving the small foreign body (Fig. ).\nThe imaging data were obtained in a Digital Imaging and Communication in Medicine format and transferred to a Medtronic StealthStation S7 workstation with Synergy Fusion Cranial 2.2.6 software (Medtronic Navigation Inc., Louisville, CO, United States). Our patient was taken into the operating room where her customized interocclusal splint was reinserted. A Patient Tracker EM was affixed to her forehead to act as a reference array to track the navigation probe. To perform patient-to-CT data registration, the instrumentation navigation probe was used to trace the reference array, soft tissue landmarks of the face, and hard tissue points (for example, tooth cusps and incisal edges) (Fig. ). After data registration was complete, continuous three-dimensional tracking of the navigation probe was available to the surgeon in real time. This was possible because of the identical position of the mandible during the CT scan and in the operating room due to the use of the interocclusal splint. In this case, the splint was not sterilized but chemically disinfected with benzalkonium chloride as it did not directly contact the surgical site.\nUsing the three-dimensional location of the navigation probe with respect to the broken instrument fragment, a location was selected that best approximated the most anterior extent of the fragment in conjunction with the navigation probe (Fig. ). An approximately 15-mm vestibular incision was made in this location, subperiosteal reflection was performed, and the foreign body location was confirmed using a careful navigation system (Fig. ). A 3-mm bony window was prepared through the buccal cortex that corresponded to the root apex of the right premolar. The instrument was carefully visualized and extruded from the apical to the tooth crown side and was then removed using mosquito forceps through the medullary cavity of the crown side of the tooth (Fig. ). A postoperative radiograph was obtained to confirm the complete removal of the fractured segment. Our patient was discharged later that day. Follow-up after the prosthetic treatment was uneventful; her clinical course was good.
Patient A is a 64 year old white female who presented with a large right breast mass. The mass had been present for at least 2 years, and the breast was swollen, streaked with grey and blue, and mildly tender. There was no personal or family history of breast cancer. Her past medical history was significant for a hysterectomy and oophorectomy at 46 years of age. Her first menstrual period was at age 13, and she had never been pregnant.\nPhysical exam revealed a well-nourished female with an obvious mass of the right breast. The mass measured 36 × 30 cm at the time of presentation. The skin of the breast was blue at the apex of the mass, and the nipple was massively enlarged and excoriated (Fig ). On initial presentation, there was no evidence of skin breakdown, but by the time of surgery, the patient had experienced loss of skin integrity. The contralateral breast was of normal size, with no significant masses on palpation. There was no palpable adenopathy in either of the axillary basins.\nFine needle aspiration was performed during the initial visit and revealed highly atypical cells suspicious of a malignant neoplasm. Core tissue biopsy showed mixed epithelial-stromal proliferation suggestive of a phyllodes tumor. CT scans of the chest, abdomen, and head showed no evidence of distant metastasis but did suggest invasion of the tumor into the chest wall. For this reason, initial surgical management only involved tumor resection, and breast reconstruction was deferred.\nRight simple mastectomy was performed. Superior and inferior skin flaps were designed to allow skin approximation and closure after removal of the large tumor. These flaps included skin directly overlying the tumor that appeared normal (Fig ). The superior flap was raised to the level of the clavicle. Dissection revealed that the blood supply to the tumor was derived largely from collateral vessels arising from the skin. These vessels were large and friable, yet easily managed using standard techniques. The inferior flap was then raised, demonstrating tumor that was partly adherent to the inferior aspect of the pectoralis major muscle. No blood supply originated from the muscle. A portion of the pectoralis major muscle was excised with the tumor, and no invasion of deeper chest wall structures was noted. No lymphadenopathy was appreciated; therefore, an axillary lymph node dissection was not pursued. Two #19 Blake drains were placed beneath the superior and inferior flaps, followed by approximation of the flaps and skin closure (Fig ).\nThe pathologic findings of this procedure were consistent with benign phyllodes tumor. The tumor measured 30.0 × 25.0 × 20.0 cm ex vivo. Microscopic sections demonstrated large, simple ducts surrounded by a uniform, bland stroma (Fig ). The margin of resection was negative for the tumor with a tumor-free zone that ranged from 0.3 to 1.0 cm (Fig ). The Ki67 proliferation index of the tumor from patient A was 5 and 13 for the epithelial and stromal component, respectively (Fig ). The epithelial component Ki67 index from this specimen is typical of adenomas, whereas the index of 13 for the stromal component is consistent with benign yet brisk stromal proliferation. Mitoses were less than 3 per 10 high power field. Three axillary lymph nodes were present within the mastectomy specimen, and none of these showed evidence of malignancy. The patient is currently over 7 years post-surgery and has shown no evidence of local recurrence or distant metastasis.
A 56-year-old lady presented with a 6 month history of rectal bleeding, passage of mucus and a change in bowel habit to more frequent stools. She had no abdominal pains and her weight was maintained. The patient had been born with bladder exstrophy and had multiple surgeries culminating in a cystectomy with ileal conduit formation at 5 years of age. She had suffered with recurrent urinary tract infections for most of her childhood and adult life but was otherwise well with no other major co-morbidities or risk factors for colorectal malignancy and had no family history of colorectal disease.\nColonoscopy revealed two large sessile polyps in the sigmoid colon in close proximity to each other and adjacent to a diverticulum-like structure (Fig. ). Each polyp was approximately 3 cm in size and both exhibited a type IV pit pattern with areas of irregularity suggestive of focally advanced disease. Histological examination confirmed both polyps were adenomas comprising both low and high grade dysplasia, without submucosal invasion (Fig. ). On closer inspection the mucosa around the diverticulum was also atypical but not adenomatous. The remainder of the colonoscopy was unremarkable with no other evidence of diverticular disease or polyps elsewhere.\nEndoscopic resection was considered as a therapeutic option however in view of the above characteristics as well as difficult endoscopic access surgery was preferred. Furthermore, radiological imaging had initially raised the possibility of invasive disease in view of sigmoid thickening. The patient underwent high anterior resection and an open approach was chosen because of suspected intra-abdominal adhesions following extensive pelvic surgery. An end colostomy was formed at the patient’s pre-operative request. At laparotomy the right fallopian tube was adherent to the sigmoid colon and adjacent to this a blind ending tube was noted to emerge from the anti-mesenteric border of the colon. This was marked for pathological identification.\nOur patient went on to have an uneventful recovery and her quality of life following surgery was good. Her wish to have a permanent colostomy stemmed from the fact that she had always suffered from an erratic bowel habit and that she was already knowledgeable with regards to stoma care in view of her pre-existing ileal conduit.
A 28-year-old woman presented with a pregnancy wish. She had a history of subvalvular membranous aortic stenosis. At 7 and at 14 years of age surgical resection of the membrane was performed. As a young adult, she developed recurrent severe subvalvular stenosis which became symptomatic. When she was 21 years old, the membrane was radically resected through a Morrow procedure and the left ventricular (LV) outflow tract was widened with a Konno procedure. After this operation she was symptom-free and needed no medication. Her echocardiogram demonstrated septal akinesia with an LV diastolic diameter of 57 mm at the basal level but a normal midventricular diameter of 52 mm. LV ejection fraction was 50%. The aortic valve was abnormal with grade I regurgitation and valvular aortic stenosis with peak and mean gradients of 27 and 15 mmHg. It was concluded that she had a slightly elevated risk of heart failure and arrhythmias during pregnancy because of her mild aortic valve dysfunction and local wall motion abnormality of the LV. She was graded as pregnancy risk WHO class II [–] (low-moderate risk). She became pregnant in 2007 and was followed in the cardiac service at 20, 26 and 34 weeks. Her LV diameters and ejection fraction as well as the aortic stenosis and regurgitation were stable. She developed dyspnoea on exertion during the second trimester without signs of heart failure, which was attributed to the pregnancy in combination with moderate overweight. During the last weeks of pregnancy she started to gain weight, a total of 8 kg in 2 weeks time, and she felt more dyspnoeic. The patient, a medical doctor herself, suggested that she had fluid retention due to heart failure, but the junior gynaecologist assured her that this weight gain was normal and the cardiologist was not consulted. Delivery was induced at 39 weeks and a healthy daughter was delivered by secondary caesarean section. Two months after delivery she was admitted because of bradycardia due to 2nd degree AV block and a DDDR pacemaker was inserted. Unfortunately, at the first echocardiogram after delivery her LV diameters were significantly larger than pre-pregnancy, 65 and 58 mm at basal and midventricular levels, respectively, with preserved ejection fraction of 50%. She remained in NYHA class II post-pregnancy. It was considered that she may have had an unrecognised period of heart failure in the weeks before delivery which was a likely explanation for the LV dilatation. Alternatively, ventricular pacing may have caused the dilatation post-pregnancy.\nIt can be concluded that the care of this woman was suboptimal in the last weeks of her pregnancy. Her pregnancy risk was adequately assessed as low to moderate, therefore routine cardiac supervision was not planned after the 34th week. However, the cardiologist should have been consulted both when she complained of excessive weight gain and increasing dyspnoea and when the delivery started. We can only speculate if this would have made any difference in outcome. Nevertheless, if she had been diagnosed with heart failure, medication and/or early delivery might have prevented damage. In the CEMACH (Confidential Enquiry on Maternal Death) report [], it appeared that substandard care was an important contributing factor to the death of many mothers. Substandard care included insufficient communication between team members and inappropriate delegation of tasks to junior doctors. Though outcome was more favourable than in the CEMACH report, these factors may have also played a role in our case. Adequate organisation of care with skilled multidisciplinary teams working according to standard operating procedures is important to prevent unnecessary complications.
In March 2012, a 37-year-old, previously healthy woman started with essential right trigeminal neuralgia. Though pharmacological treatment was started, the patient did not improve. After one year of treatment, she presented with skin lesions on the face, along the areas innervated by the ipsilateral V cranial nerve. The patient was exhaustively studied, including an assessment by multiple specialists. Herpes and other infectious and dermatological diseases were ruled out by performing a biopsy of the skin and a pathology assessment. The lesions were increasing in size and were located in areas innervated by the three branches of right cranial nerve V. The lesions were painless and related to the longest period of painful symptoms (Figure ). In July 2013, she underwent a Fogarty rhizotomy with no improvement. Subsequently, in January 2014, she underwent right suboccipital decompression with the Janetta microsurgical technique due to the clinical suspicion of vascular compression, without improvement. She continued to present very severe paroxysmal pain crises in the three branches of the right trigeminal daily, which did not respond to any type of treatment and continued with skin lesions. Although the patient did not report repeated manipulations in the innervation zone of the trigeminal nerve, a bandage was used on the face, seeking an improvement in the skin lesions, but the improvement was not as expected. The patient lost weight and when we first evaluated her, she was in a very altered psychological situation, due to both the pain and the expression of the disease on her face. Assessing the case and analyzing all the previous treatments in the context of neuralgia, it was decided to conduct radiosurgery using the CyberKnife technique in order to control pain.\nRadiosurgical treatment\nThe patient is informed about the radiosurgical procedure and signs the consent. A reinforced immobilization thermoplastic mask is made, which has a greater thickness than the typical mask, to increase its rigidity and further decrease the possibility of head movement in order to achieve greater precision. Planning neuroimaging is performed: a simple CT with 1.2 mm fine cuts with the immobilization mask on and a simple MRI sequence T1, T2, and fast imaging employing steady-state acquisition (FIESTA) with fine cuts (Figure ). Subsequently, images are merged in Multiplan software (Microsoft Corporation, Redmond, Washington). A volume of the trigeminal nerve was outlined, starting 3 mm apart from the brainstem and covering 5 mm of the trigeminal nerve, exceeding 1 mm on each side of the nerve. It was prescribed a dose of 60 Gy to an isodose of 80% of the outlined volume, provided that at least 50% of the volume received 70 Gy and seeking a maximum dose of 85 Gy. Isocentric reverse planning was performed with a brainstem constraint of V10 <0.5 cc and Dmax of 12 Gy (Figure ). After radiosurgery treatment, the patient's neuropathic pain improved, decreasing on the analogous pain scale from 10 to 5 in the second week of treatment. Additionally, her skin lesions improved (Figures -).
An 8 year old Caucasian boy presented acutely with generalized weakness, shortness of breath on minimal exertion, marked dysarthria and difficulty swallowing with nasal regurgitation of fluids. His symptoms has progressed rapidly over a few weeks. Clinically his symptoms were felt to be consistent with MG. He was admitted to the pediatric ward and reviewed by the anesthetic service. He was maintained under close surveillance but a decision was made to hold off invasive ventilation. He was commenced on ChE-I, oral prednisolone and IVIG. He made good progress and was discharged home with a plan for a further course of IVIG in 4 weeks in his local hospital due the severity of his initial symptoms, and lag time for steroids to take effect. He was seen in clinic 12 months later and at this time was on maximum alternate day steroids. He was also receiving 4-weekly IVIG infusions at his local hospital. Both him and his parents reported a dramatic response to the IVIG but felt the effect wore off after about 3 weeks and his symptoms particularly fatigue became “as bad as ever.” On examination he had no weakness. Prior to his diagnosis he was said to be an outgoing boy and very involved in sports. His parents now reported he was refusing to go to school most days and no longer engaging in any extra-curricular activities. They felt the slightest thing could have him in tears. A decision was made to assess him neurologically at the time of his next infusion. While on the ward strength was noted to be normal and on further questioning he said that he kept reliving his initial hospital admission and felt that the “only reason he didn't die was because of the special protein drip.” He was felt to have evidence of post-traumatic stress disorder and appropriate psychological supports were put in place. IVIG was withheld and a gradual improvement was seen.\nThis case highlights the importance of the multidisciplinary team in managing young patients with JMG. Psychological issues need to be addressed early and the necessary supports put in place. It is not uncommon for young patients to report fatigue rather than true muscle weakness, and this is often a manifestation of an underlying mood disorder rather than their MG. Careful assessment needs to be carried out in all patients prior to using IVIG to ensure that it is being used in the appropriate setting.
A 48 year old woman who was diagnosed with a huge intraabdominal cystic mass was transferred to our department. She had been diagnosed with breast cancer two months earlier and had undergone partial mastectomy with sentinel lymph node biopsy. After surgery, adjuvant chemotherapy of three cycles was administered. She had recently developed intermittent abdominal pain. Under physical examination, a diffuse soft mass was palpable with mild tenderness, but the margin of the mass was not clear. Abdominal computed tomography (CT) for evaluating the abdominal pain and mass revealed a giant multi-lobulated mass, measuring 26×12 cm in size, which was adjacent to the lesser curvature of the stomach (). However, the origin of this mass was not determined by CT. The gastrofiberscopic findings revealed a large bulging lesion with intact overlying mucosa on the posterior wall and lesser curvature side of the stomach (). All blood laboratory data values were within normal ranges. Based on the physical examination and the imaging study, we decided to perform exploratory laparotomy after obtaining informed consent from the patient.\nAfter the upper midline incision, a large multi-cystic mass originating from the lesser omentum of the stomach was detected. A portion of the mass adhered to the omentum; this adhesion was easily dissected, but the lesser omentum, including the right and left gastric vessels and the vagus nerves, were fully involved in the mass. The mass appeared to invade the lesser curvature of the gastric antrum. We decided to perform distal gastrectomy with mass excision for curative resection.\nFirst, the gastrohepatic and gastrocolic ligaments were dissected to mobilize the stomach and the mass, and the right gastric vessels were resected. The right gastroepiploic vessels were resected, and the first portion of the duodenum was resected. The posterior wall of the stomach was exposed, and the left gastric vessels were subsequently resected. Because the mass involved the trunk of both vagus nerves near the abdominal esophagogastric junction, truncal vagotomy and dissection of the lesser omentum from the upper portion of the lesser curvature were performed. Finally, the distal portion of the stomach was resected, and the mass with the distal stomach was extracted without any lymphatic leakage. The gastroduodenostomy was performed with a 29 mm circular stapler.\nThe specimen was 22×10×5 cm in size, and its cross-section presented as a multilobular cyst (). The tumor was slightly attached to the lesser curvature of the stomach, but the mucosa of the stomach was intact (). In the microscopical finding, tumor was composed with the multiple cysts which were various sizes, and lined with the endothelial cells ().\nThe patient had no complications during the postoperative period, and oral intake began on postoperative day 3. She was discharged from the hospital on the seventh day after surgery. Additional adjuvant treatment for breast cancer was started again, and the patient had no complaints related to the gastrointestinal tract 6 months after surgery.
A 36-year-old premenopausal woman presented with a painful mass in the outer quadrants of the left breast that had grown rapidly for two months. The woman had no significant medical records and no family history of breast cancer. Her menstrual cycles were regular and she had three children. The initial physical examination revealed a well circumscribed elastic mass measuring about 30 mm not adherent to the underlying tissues or to the skin and no skin or nipple retraction was visible. Besides, the clinical examination did not reveal ipsilateral, axillary, or supraclavicular palpable lymph nodes. Nipple discharge was not evident. Contralateral breast and axilla were normal. The left breast ultrasound showed a cyst measuring 26 mm with irregular and hypoechogenic vegetation growing on the inner wall. A mammographic exam was not performed mostly due to the cystic appearance of the mass. Besides that there are no characteristic findings on mammography specific for this kind of tumours. The patient was submitted to fine needle aspiration cytology (FNAC) of the cyst and of the inner vegetation (). The fluid obtained was yellowish and translucent and the size of the mass got reduced after the procedure. The smears were stained with Papanicolaou and May-Grünwald Giemsa stains. Microscopic evaluation revealed some foam cells and epithelial cells without atypia suggesting a benign cystic lesion. The biopsy was not performed since it was a cystic lesion, and, besides the inner vegetation, the whole ultrasonographic features and the FNAC results suggested a benign condition. One month later the breast ultrasound exam showed the persistence of the cyst, with increased volume, measuring about 44 mm maintaining the inner vegetation of 6 × 27 mm, which was irregular in shape and had continuity with the cyst wall (). A surgery was performed because of the cyst rapid growth and ultrasound characteristics. At the time of hospitalization, one month after the second breast ultrasound, the physical examination revealed a painful large mass with 90 mm in the outer quadrants of the left breast. A breast tumorectomy was carried out. Macroscopically the surgical resection specimen, measuring 80 × 55 × 40 mm, showed a cyst. The inner surface of the cyst was mostly irregular with whitish vegetation. On histological examination a cystic lesion was seen, focally with atypical epithelial lining, with extensive ulceration. On the wall of the cyst there was a neoplastic infiltrative carcinoma with a solid pattern, high grade, with areas of squamous differentiation. Therefore, pure squamous cell carcinoma of the breast, grade 3, was diagnosed (Figures and ). It was difficult to establish the real size of the carcinoma because there was an intralesional section. The R classification was not given by the pathologist. The immunostaining for cytokeratin 34 beta E12 and p63 antibody was positive. Vimentin antibody and estrogen receptors were only positive in less than 5% of the cells. There was no reactivity for progesterone receptors or for HER2. The bone scan and thoracic-abdominal-pelvic computed tomography were negative as far as metastatic disease was concerned. The patient underwent surgery for a simple mastectomy with sentinel node biopsy. The decision was made taking into account the size of the tumour, the uncertainties about the residual tumour, and the preference of the patient. The histological study of the specimen revealed residual SCC reaching the deep margin with sentinel lymph node negativity regarding metastasis. The patient received 6 cycles of adjuvant chemotherapy (3 cycles of docetaxel and carboplatin followed by 3 cycles of fluorouracil, epirubicin, and cyclophosphamide). Adjuvant radiotherapy was performed to the chest wall in a standard regimen. Follow-up time is now four years and there is no evidence of disease. Cancer antigen 15.3 is negative. Imagiologic features of the left chest wall and right breast and axilla are normal.
A 74-year-old woman was admitted to the hospital for sudden onset of left eyelid ptosis for 1 day with no obvious inducement. The patient had a history of hypertension, but she did not regularly measure blood pressure and take oral antihypertensive drugs. Physical examination revealed that she had clear consciousness and normal speech, but the left eyelid drooped. The left pupil diameter was 5 mm and light reflex was absent. The left eyeball could not move, and the right eye examinations were normal. The limb muscle strength and muscle tension were normal. CTA examination showed bilateral internal carotid artery aneurysms. After admission, the whole cerebral angiography was performed under local anesthesia and the procedure was smooth. The imaging diagnosis was bilateral carotid artery communicating aneurysm combined with severe stenosis of the origin of left carotid artery. The size of the left aneurysm was about 8 mm × 3 mm and the neck was about 3 mm, and the right aneurysm was about 3.7mm × 3.8 mm and the neck was about 3.8 mm (Fig. ). After the operation, when the patient was ready to be lifted down the bed after the angiography, she suddenly became unconscious and could not open her eyes. We found bilateral pupil dilation with a diameter of 5 mm, absence of the light reflex, and there was no reaction to the stabbing pain in the limbs. The patient's emergency head computed tomography (CT) examination showed subarachnoid hemorrhage (Fig. ). The doctor immediately gave the patient intravenous injection of 20% mannitol solution 250 mL. About 15 minutes later, the patient regained consciousness, questions could be answered, and the limbs could move autonomously. We considered the possibility of rupture of the left posterior communicating artery aneurysm. Because of her older age and poor physical general condition of the body, the main worry about the patient was that she might show a poor tolerance to operation and anesthesia. Thus, the patient received conservative treatment.\nOn the third day after operation, the patient's condition gradually improved. Under the general anesthesia, left carotid artery stenosis stent implantation and left posterior communicating artery aneurysm stent assisted coil embolization were performed successfully (Fig. ). On the second day after embolization, the patient was in a coma with grade 0 muscle strength on the right limb, positive on the right babinski, and occasional voluntary movement on the left limb. Head CT showed subarachnoid hemorrhage with hydrocephalus (Fig. A). Day after surgery, emergency external drainage was performed, and the postoperative CT scan showed cerebral infarction in the left temporal lobe (Fig. B). Postoperative 250 mL of normal saline + 30 mL of edaravone injection was injected twice a day, 50 mL of normal saline + 24 mg of nimodipine injection was pumped continuously, and 250 mL of normal saline + 40 mg of monosialotetrahexosylganglioside sodium injection were injected once a day. The patient's condition was stable. A month later, the patient underwent ventriculoperitoneal shunt. The operation was successful and the postoperative condition improved. Six months later, the patient visited our hospital for a follow-up, and she was clear-headed, aphasia, right limb hemiplegia with muscle strength grade II, left side autonomous activities, and the GOS score was 2 points. Head CT showed the ventricles were normal (Fig. ).
A 12-year-old boy presented with painful swelling on his left thigh after being kicked by his friend. Plain radiography revealed a concentric cystic lesion with cortical thinning and ballooning in the mid-portion of the femoral shaft accompanied by a pathologic fracture (). The cystic lesion was extensile without a periosteal reaction. The patient underwent curettage of the cystic bone lesion and autogenous bone grafting, followed by open reduction and internal fixation for the pathologic fracture (). Intraoperative biopsy revealed a unicameral bone cyst () and, therefore, it was decided to continue close observation of this case without any further intervention. Two years after surgery, plate removal was performed based on radiographic evidence of complete healing of the cystic bone lesion (). Although 1.5 cm femoral shortening was initially noted after surgery, the leg length discrepancy appeared to have increased with age. Therefore, the patient was scheduled for annual follow-up until skeletal maturity. At the age of 19 years, no recurrence of the cyst was seen on plain radiographs of the femur (), and the final leg length discrepancy was 3.5 cm (). The patient complained of abnormal gait and posture resulting from the 3.5 cm discrepancy, for which lengthening of the shortened femur was planned in the near future.\nAt the age of 20 years, 8 years after the initial surgery, the patient complained of pain in the left thigh. A review of his plain radiographs revealed an impending pathologic fracture caused by a recurrent simple bone cyst in the left femur (). Magnetic resonance imaging (MRI) scan demonstrated a 6.6 cm × 3 cm × 3 cm-sized, well-defined cystic lesion in the left distal femur (). Comparison of the radiographs and MRI findings revealed the presence of a linear pathologic fracture accompanied by a periosteal reaction. For treatment of the fracture, curettage and autogenous bone grafting combined with internal fixation using an intramedullary nail () were performed. One year after the second surgery, the lesion was stabilized despite cortical thinning and ballooning of the femur (). Therefore, lengthening of the left femur was carried out with an application of the Ilizarov apparatus over the intramedullary nail to treat the cystic lesion and limb length discrepancy simultaneously (). Corticotomy was conducted at the level of the proximal femur in order to avoid stress on the cystic lesion. The femur was lengthened by 3.4 cm, and the external fixation index was 11.7 day/cm. Subsequent follow-up was conducted on a regular basis. For 20 months after the lengthening with the Ilizarov apparatus, no evidence of recurrence was observed (). Intramedullary nail removal was done 30 months after the Ilizarov procedure (). At 1 year after the hardware removal, there was no radiographic evidence of recurrence of the bone cyst and limb length discrepancy ().
The 8-year-old boy first presented in January 2012, having been referred by the family dentist, to the Department of Orthodontics at the Medical Center of the University of Göttingen for a routine orthodontic control and evaluation of treatment need (). The clinical examination of the asymptomatic patient showed no extra- or intraoral pathological findings. The medical history of the boy included a mild pulmonary valve stenosis and a secundum atrial septal defect with a left-right shunt. He showed a good physical and cardiac fitness and a normal nutritional status. The family history was positive for maxillofacial anomalies: the boy's older sister had been previously diagnosed with a dysplastic fibroma, a rare benign fibrovascular defect in the mandible, and a resection of the affected area in the mandible had been performed. His father and paternal grandmother had a positive history of odontogenic cysts as well as basal cell carcinomas, although the family history of OKCs was negative. The radiological examination showed three suspicious hypomineralisations visible as radiolucencies in the panoramic radiograph associated with the retained teeth 13 and 23 and the ectopic tooth 27 (). The young patient was referred to the Department of Maxillofacial Surgery for a surgical examination of the radiologic anomalies.\nThe operation was performed under general anesthesia. The suspected pathological area around teeth 23 and 13 showed no visible intraoperative pathological signs. A bone and soft tissue biopsy for histological examination was taken. In the area of tooth 27, a well-marked membrane was revealed, filled with a viscid fluid and fully enclosing the dental crown. The clinical aspect was consistent with a follicular cyst. During the radical cystectomy, tooth 27 was removed due to massive attachment loss. The histopathological biopsy showed a fibroosseous lesion in the area of teeth 13 and 23. The biopsy from region 27 showed an odontogenic connective tissue cyst wall with intramural odontogenic cell islands. On request of the surgeon, samples were sent for further diagnosis to the Bone Tumor Reference Center of the Swiss Society of Pathology at the University Hospital in Basel, Switzerland. The initial histological diagnosis was corrected to an OKC of the parakeratin variant. Microscopically, the cyst shows a squamous epithelium. The basal cells are palisading, with hyperchromatic nuclei (HE staining, 5x magnification) (). Due to the high recurrence of OKCs, a radiological control interval of 6 months was indicated (). Furthermore, orthodontic treatment was initiated.\nIn August 2014, during a regular radiological control, a new radiolucency was detected, associated with the retained and displaced teeth 47 and 48 (). The surgical removal of the cystic lesion and tooth 47 was performed under general anesthesia. The pathological finding was consistent with an OKC.\nThe regular control examinations were interrupted by missed appointments, so the next evaluation took place one year later, in October 2015 (). New radiolucencies were detected in the panoramic radiograph associated with the retained teeth 18, 17, 37, 38, and 48 as well as an evident enlargement of the radiolucency around the crown of tooth 13. A cone beam computer tomography scan was performed and showed well-defined radiolucent areas, associated with the retained teeth. Details of the surgical enucleation of the cysts with the extraction of teeth 18, 17, 13, 37, 38, and 48 are shown below. The postoperative radiological examination is depicted in . Clinical and radiological examinations were then performed every 6 months.\nWe describe the surgical enucleation of the cystic lesions using the example of the third operation (2015). This was performed under general anesthesia; the affected regions were exposed after lifting a mucoperiosteal flap. After a careful removal of a thin bone cortex, the cystic capsule was found () and separated from the bone with an obtuse instrument. The aim was to leave no epithelial remnants on the trabecular bone. All four lesions were associated with a retained tooth, which was only loosely anchored in the alveolar bone. Due to the high recurrence rate of the cystic lesions in this particular case, all affected teeth were extracted. In the area of the mandible, the use of Carnoy's solution was not indicated because of the exposure of the lower alveolar nerve (). Due to their large size, the cystic cavities were filled with a collagen graft, which stabilized the formation of a coagulum. No reconstruction with iliac crest bone or allogenic bone grafts was attempted. Subsequently, the mucoperiosteal flap was reverted back to its original position and fixed by sutures.\nBy October 2016 and August 2017, bone remodeling of the affected area had been detected and no new lesions were observed (Figures and ).\nDue to the recurrence and the appearance of new lesions, Gorlin-Goltz syndrome was suspected in the patient. After genetic counselling at the Institute of Human Genetics of the University Medical Center of Göttingen, molecular genetic analysis of the genes PTCH1 and PTCH2 was performed in 2015. Sanger sequencing revealed the heterozygous germline variant c.2779_2793del (p.Ser927_Val931del) in the PTCH1 gene. This variant leads to an “in-frame” deletion of 5 amino acids between amino acid positions 927 and 931 of the protein. This variant is listed neither in the Human Gene Mutation Database (HGMD) nor in the Leiden Open Variation Database (LOVD). However, a pathogenic effect of the variant seemed likely as many pathogenic variants have already been described in this region of the PTCH1 gene, even several in-frame deletions [, ]. Since the boy's father had shown similar symptoms (odontogenic cysts, basal cell carcinomas) that could be in line with a Gorlin-Goltz syndrome, he, too, was tested for the PTCH1 variant and resulted to be carrier of the variant.\nIn summary, clinical and molecular data together with the positive segregation analysis led to the classification of the variant as “probably pathogenic” and being responsible for Gorlin-Goltz syndrome in the patient and his father. The importance of talking precautions (e.g., sun protection due to the high risk of basal cell carcinomas) and regular medical surveillance (e.g., regular orthodontic care and annual dermatologic examinations) was emphasized.
A 38-year-old male presented with pain in his right thigh. The patient had a past history of treatment for a bone tumor of the right proximal femur. At 20 years of age, the patient had experienced right thigh pain and consulted a doctor at the local hospital. The patient was noted to have a bone tumor of the right femoral bone without distant metastasis and underwent surgical curettage of the tumor. The specimen revealed a malignant small round cell neoplasm with regular nuclear contours, finely dispersed chromatin and scanty cytoplasm without prominent nucleoli (). The patient received one cycle of postoperative chemotherapy that consisted of vincristine and cyclophosphamide and local irradiation with a total dose of 40 Gy. Two years after the initial operation (at 22 years of age), the patient developed severe pain in his right thigh after braking hard while driving. The radiographs taken at the time showed a fracture of the femur where the bone tumor had been located. Open reduction and internal fixation (ORIF) using intramedullary nails was performed. One year after the ORIF, the patient stubbed his toe and could not walk due to severe right thigh pain. Radiographs showed an undisplaced femoral re-fracture. He was conservatively treated and subsequent roentgenograms demonstrated bone union. His subsequent postoperative course had been uneventful for 15 years.\nAt 38 years of age, the patient was referred with right thigh pain that had persisted for several months. Radiographs showed a lytic lesion from the trochanteric area to the proximal diaphysis (). Magnetic resonance images showed an intramedullary lesion with an extraskeletal mass () and further metastatic work-up was negative. As local recurrence was suspected, an open biopsy from the intertrochanteric lesion was performed. Microscopic findings revealed a malignant small round cell neoplasm with regular nuclear contours and finely dispersed chromatin without prominent nucleoli (). The microscopic findings were similar to those obtained at first surgery, performed at 20 years of age. Immunohistochemical and histochemical staining showed that the neoplasm was positive for glycogen (PAS), neuron-specific enolase (NSE), MIC-2 and S-100 protein, and negative for α-SMA, CD34, CD56, chromogranin A, neurofilament and vimentin. The MIB-1 index was <10%. Although molecular biological investigations did not reveal the presence of any characteristic fusion genes, including EWS-FLI1, EWS-ERG, EWS-FEV, EWS-ETV1 and EWS-E1AF, a final diagnosis of a local recurrence of ESFT was made. Based on the diagnosis of ESFT, the patient underwent chemotherapy consisting of vincristine, doxorubicin, cyclophosphamide and ifosfamide () as well as wide resection of the tumor, combined with reconstruction using a prosthesis. The microscopic findings of the specimen from the widely resected proximal femur revealed a minimum response to the preoperative chemotherapy (necrotic rate <10%). The patient has been disease-free for the past 9 years following this surgery.
A 62-year-old otherwise healthy Caucasian male complained of some minor nonspecific respiratory symptoms for a few days. These complaints evolved into a more worrisome pulmonary disease characterized by cough and progressive respiratory distress 2 days prior to the hospitalization. Chest CT scan on admission to an outside hospital showed diffuse bilateral alveolar and interstitial lung infiltrates. A diagnosis of atypical pneumonia and possible myocarditis was made and the patient was given antibiotic treatment and was discharged two weeks later. However, dyspnea persisted and he was admitted to our hospital for further workup. On admission, patient was found to have fever, acute hypoxic respiratory failure, pleural effusion, pericardial effusion, and irregular heartbeats with bouts of ventricular fibrillation. Upon hospitalization, additional diagnoses were made including acute renal failure, as well as possible liver disease marked by an elevation of liver function tests. A poorly defined pancreatic mass was noted on the CT scan. Head CT without contrast was performed which showed minimal mucosal thickening in the left maxillary sinus. There was opacification of several left ethmoid air cells with fluid levels in the left sphenoid and left maxillary sinuses. A nasoenteric tube was in place in nasal cavity. There was no mass lesion, erosion, or bone destruction. These finding were most consistent with mild inflammatory changes ().\nSepsis was suspected and he was given antibiotics without improvement of his clinical condition. His condition rapidly deteriorated with progressive dyspnea and pulmonary infiltrates which by X-ray examination involved all five lobes (). Extensive microbiological studies were performed with no positive bacteriologic, viral, or fungal isolates. The only positive test was a mildly increased DNA load of Epstein-Barr virus (EBV) in blood with 0.5 copies per microliter detected by a quantitative PCR assay. EBV capsid IgG and nuclear antigen/antibody were positive. Peripheral blood smear showed mild normocytic anemia with moderate thrombocytopenia. White cells count was within the normal range with absolute lymphopenia and no atypical lymphocytes or lymphoma cells.\nAs his condition deteriorated, he developed signs of cardiac tamponade. Pericardial effusion was drained and sent to the pathology laboratory. He developed uncontrollable ventricular fibrillation with subsequent cardiac arrest, which could not be reversed by resuscitation. At the same time, patient hemoglobin and platelet count decreased to 6.9 mg/dL and 22,000/μL. His fibrinogen was decreased (178 mg/dL), ferritin was markedly elevated (1763 ng/Ml), and lactate dehydrogenase was elevated (628 U/L). Neutrophil count and triglyceride were within normal range. Hemophagocytic lymphohistiocytosis (HLH) was suspected. However, the final diagnosis of HLH was never made since the patient's condition deteriorated quickly and he died in a cardiopulmonary failure.\nThe pericardial fluid sent for cytological analysis one day before his death contained numerous markedly atypical lymphoid cells () which were positive for Epstein-Barr virus (EBV) as detected by in situ hybridization (). Immunohistochemical stains performed on the cell block showed that the atypical cells were positive for CD45, CD2, and CD3 () and negative for CD4, CD8, CD56 (), CD138, pan-cytokeratin, MOC-31, Ber-EP4, chromogranin, synaptophysin, calretinin, S-100, and HHV8. Ki-67 (MIB-1) showed an increased proliferation index of approximately 20% reflecting the number of EBV-positive atypical cells. Flow cytometry confirmed that the lymphoma cells were CD2 positive. There was cytoplasmic CD3 positivity, but there was no surface CD3 expression. Bone marrow aspiration and core biopsy revealed a normocellular marrow for age (30–40% cellularity) with active trilineage hematopoiesis and left shifted granulopoiesis but no evidence of lymphoma. Cytogenetic analysis did not reveal any clonal chromosomal abnormalities.\nAt autopsy, the decedent was found to have cardiomegaly (weight 650 grams) () with four-chamber dilatation and serous pericardial effusion (100 mL). Patchy whitish discoloration was found on the surface of epicardium and endocardium, and the myocardium had a mottled appearance on cross section (). The right and left lungs were heavy and weighed 1000 and 940 grams, respectively. The pleural surfaces were smooth, glistening, and unremarkable and the cut surface revealed patchy consolidation of pulmonary parenchyma with red and grey discoloration. There was 500 mL of straw colored pleural fluid in each of the pleural cavities. The pancreas was enlarged and the cut surface showed scattered areas of fat necrosis with hemorrhage. There was 500 mL of opaque fluid present in the abdominal cavity. The spleen was enlarged, weighed 330 grams, and had a smooth intact capsule covering red-purple semiautolytic parenchyma. The splenic white pulp was grossly unremarkable. Other organs were grossly unremarkable. Under microscopic examination, the myocardial tissue was found to be diffusely infiltrated by atypical lymphoid cells (). These cells had abundant pale cytoplasm, irregular nuclear contours, granular chromatin, and inconspicuous nucleoli (). In situ hybridization performed on the cardiac tissue showed that the atypical lymphoid cells were strongly positive for EBV. Immunohistochemical stains performed on the same tissue showed that the lymphoid cells were positive for CD3 (cytoplasmic stain) () and granzyme B () and negative for CD5, CD10, CD20, CD30, CD34, CD43, and CD56. Cells infiltrating the lung parenchyma in all five lobes (Figures and ) had the same immunohistochemical features. Diffuse infiltrates of identical neoplastic cells were found in the pancreatic () and gastric tissue ().
A 28-year-old Caucasian woman presented to our Emergency Unit with a short history of progressive shortness of breath and a swollen right arm. She had previously been in good health with no medical history of note but was just recovering from a severe upper respiratory tract infection, which had caused her to be unwell in bed for several days. Aside from the oral contraceptive pill she took no regular medication and had no family medical history of note.\nInitial investigations with a Doppler ultrasound of her right arm and axilla demonstrated a deep vein thrombosis in the right subclavian vein with extension into the proximal internal jugular vein. A computed tomography scan confirmed the presence of the right axillary vein thrombus and the pulmonary embolus and a large right pleural effusion as shown in Figure . Despite these findings, the patient was hemodynamically stable and after review by the vascular surgery team the decision was made to manage with anticoagulation rather than thrombolysis. The initial imaging also demonstrated enlarged lymph nodes, without the normal fatty hilum, in the mediastinum and right supraclavicular fossa. The imaging of the patient’s abdomen and pelvis demonstrated a mixed echogenicity 13.5cm left adnexal mass situated between the sacrum and the anterior abdominal wall shown in Figure .\nThe tumor markers performed at presentation demonstrated normal values for both hCG and AFP whereas the CA-125 level was moderately elevated at 368U/mL (normal range 0 to 35). The initial management centered on the treatment of the pulmonary embolus with anticoagulation and the insertion of a prophylactic inferior vena cava filter. The differential diagnoses at this stage included: epithelial ovarian cancer, an ovarian germ cell tumor, an ovarian sex cord-stromal tumor or an atypical Meigs syndrome.\nAfter a week of anticoagulation treatment, a laparotomy was performed which demonstrated a large left ovarian mass with ascites but no other abnormal findings. The ovarian mass was removed along with a biopsy of the omentum and a fine needle aspirate of the supraclavicular lymph nodes was performed. Postoperatively the patient made an excellent recovery and was well enough to be discharged home 6 days later.\nOn pathological examination the ovarian mass measured 14×8.5×6.5cm and weighed 470g. The outer surface was predominantly smooth with a focus of roughened area, suggesting a site of adhesion to adjacent structures. The cut surface was solid, tan in color and focally hemorrhagic. Histological examination, as shown in Figure , demonstrated a spindle cell tumor of variable cellularity with both hypercellular and hypocellular areas. The tumor cells were arranged in sheets and intersecting fascicles with the intervening stroma containing short collagen bundles and areas of necrosis and focal hemorrhage. The cells had oval to spindle nuclei with fine chromatin and inconspicuous nucleoli. No notable cytological atypia was seen. The mitotic activity was variable with most parts showing no mitotic figures while focally mitoses were seen with up to two mitotic figures per 10 high-power fields (HPF).\nOn immunostaining the tumor cells expressed inhibin, calretenin (focally), progesterone receptor (weak expression in some cells) but were negative for estrogen receptor, CD34, HMB-45, S-100 protein, h-caldesmon, MNF116, epithelial membrane antigen, CAM 5.2 and cytokeratin 5/6. The morphology and immunoprofile were those of cellular fibroma. The omental tissue showed only mesothelial hyperplasia, and no tumor deposits and the ascitic fluid showed no malignant cells. The biopsy from the enlarged supraclavicular lymph nodes showed prominent interstitial reticulum cells, but no evidence of malignancy.\nThe pathological findings, coupled with the rapid spontaneous resolution of the ascites, pleural effusion and lymphadenopathy postoperatively, confirmed the diagnosis of Meigs syndrome, combined with reactive lymphadenopathy.\nThe patient was referred to the hematology team for investigation of any clotting tendency. Fortunately, the results of all investigations including Factor V, lupus and anti-cardiolipin screens were negative and routine anticoagulation was continued for 1 year and then discontinued. After 5 years of follow up the patient remains in good health and has recently had a successful pregnancy with a healthy baby girl delivered without complications.
The patient was a 10-year-old girl, recently diagnosed with leukemia and hospitalized for treatment. She required central venous (CV) line placement for chemotherapy. Other than her diagnosed leukemia, she had no other significant medical history.\nAfter the procedure was explained thoroughly to the patient and her parents, a consent form was completed by her parents, and she was transferred to the CV line room. The patient was very alert and cooperative. Based on the local protocols, all patients requiring CV line placement are transferred to a room dedicated for CV line placement (the intravenous access room), which is located in the operating room. Standard monitoring including an electrocardiogram (ECG), noninvasive blood pressure, and pulse-oximetry were initiated. A 20-gauge cannula was inserted into the vein on the dorsum of the patient's left hand.\nThe right internal jugular vein was selected for CV cannulation.\nPropofol was used for sedation, and after adequate sedation, a single-lumen 14-gauge catheter was inserted in the right internal jugular vein using ultrasound sonography under sterile conditions by an experienced anesthesiologist. No problem was encountered during the procedure, and after blood was aspirated, the catheter was fixed at 13 cm. Normal saline infusion was initiated through the CV. Central venous waveforms were not used for catheter position confirmation.\nA chest radiograph was immediately arranged to confirm the catheter position.\nOn chest radiograph, the catheter could be seen looping back and going upward at the junction of the right internal jugular vein and the right subclavian vein (). The team decided to use ultrasonography to verify that the catheter was in the jugular vein and had not punctured the dorsal wall of the vein. During scanning of the right internal jugular vein, only a single lumen of the catheter could be seen until we scanned the bottom third of the jugular vein ().\nWe decided to pull back the catheter under the guidance of ultrasonography until only one lumen could be visualized and then pass a guidewire over the catheter to reposition the catheter. While doing so and after only one lumen was visualized following extraction of the central venous line under the guidance of ultrasonography, passage of the guidewire was attempted, but resistance was encountered. Therefore, we decided to completely remove the catheter and reinsert another catheter.\nWhen the catheter was removed, it was observed that the catheter had bent at the distal end ().\nSubsequently, we placed another CVC through the internal jugular vein without any complications. The position of the CVC was confirmed by radiograph.
Case 2: A 20 year-old male was admitted to our hospital on 15th February 2005 with a history of intravenous injection of mercury taken by him and his friend as an aphrodisiac. The patient presented with fever, tachycardia, and swelling of the right hand (which was the primary site of injection) extending up to the forearm [Figures –]. The patient was haemodynamically stable. An attempt had been made for incision and drainage in the emergency room because it was diagnosed as a injection abscess. An X-ray of the hand revealed a radio-opaque shadow overlying the fourth metacarpal, third and fourth intermetacarpal space extending over the carpus, and the wrist joint up to about 1 cm over the distal end of the radius and ulna dorsal to the skeleton. A diagnosis of extravasation of injected mercury was made. After basic investigations, the patient was shifted to the operation theatre where a thorough debridement was performed under general anaesthesia and a large amount of mercury was removed physically from the dorsum of the hand. Some of the mercury could only be removed along with excision of the surrounding soft tissue; the removal of mercury was confirmed by radiography. The wound was left open so that any mercury left behind could find a passage to come out. On the following day, the patient went home against medical advice but came back on the 7th of March with a history of another subcutaneous injection of elemental mercury over the precordium. Once again, an attempt had been made to drain an abscess in another hospital. The patient refused any further surgical procedures although the radiograph showed the deposition of mercury in the subcutaneous plane in the chest wall. The patient turned up in October 2005, with a swelling over the precordium with surrounding inflammation []. This time, he was willing for surgery and the mercury was removed along with surrounding tissue en masse and the wound healed uneventfully.\nOn both occasions, the patient was not willing to be subjected to any investigations apart from a basic presurgical profile, and hence, serum levels of mercury could not be determined.\nThese two patients happened (the authors have used past tense because one of them viz. Case 1 is dead—he went home against medical advice and is known to have died in another hospital due to unknown causes. He was himself an assistant to a Unani physician and was repeatedly taking injections of multiple unknown drugs) to be close friends and apparently had taken these injections on the advice of patient no: 1 (in case report 1) as an aphrodisiac. Patient no: 2, being less adventurous, stopped after one injection and turned up at the hospital for treatment.
An 80-year-old female patient underwent total hip replacement for primary osteoarthritis of left hip through minimally invasive Watson jones approach (). During the surgery uncemented press fit acetabular cup and uncemented femoral stem were used. We did not use any screw for acetabular cup fixation. Immediate postoperative period was uneventful and patient was discharged from the hospital 6 days after surgery. As per our hospital protocol we use low molecular weight heparin for 6 weeks for thromboprophylaxis in total hip replacement patients.\nAfter 3 months of surgery, patient came back with the complaint of pain in the left inguinal region and development of ecchymosis over the left inguinal region. Patient was readmitted to the hospital for observation and further investigation. Next morning patient started complaining of weakness of left lower limb and numbness over medial aspect of thigh and knee. On physical examination, the patient was found to have grade 3/5 motor function of the quadriceps muscle, as well as decreased light touch and pinprick sensation over the femoral nerve distribution area. Patient did not have any spine tenderness.\nBlood investigations showed mild anemia with haemoglobin level of 9.0 g/dL. There were no abnormal findings of bleeding tendency or disorders of hemostasis and she had not been treated with any anticoagulant drugs. The anteroposterior and lateral radiographs of the pelvis and left hip showed no abnormality except previously performed total hip replacement, with no displacement or dislocation of the prosthesis noted (). A radiograph of lower spine was normal. MRI of lower spine was normal. Computerized tomography (CT) examination of pelvis showed 8 × 10 cm mass around the left lesser trochanter extending in thigh in vastus intermedius muscle (Figures and ). So the case was diagnosed as femoral nerve palsy due to delayed hematoma development in iliopsoas region. We have tried to aspirate the hematoma under sterile precautions but only 6 mL of occult blood comes out. CT guided aspiration of hematoma was refused by radiologist as hematoma was organized. Surgical decompression was planned after opinion of vascular surgeon.\nIncision was given through direct anterior approach. A hematoma was found in the lesser trochanter region and in front of joint. The femoral nerve was found stretched by the hematoma. Femoral nerve and artery were identified and secured with mersilene tape. The organized hematoma around 150 gms was removed. Fresh bleeding starts once the hematoma was removed, because the ruptured pseudoaneurysm was occluded only by tamponade effect of the hematoma. Bleeding vessel was identified as branch of profunda femoris artery and sutured. A drain was kept in situ and closure was done. Postoperative culture reports of hematoma were sterile. The inguinal pain was relieved completely after the surgery. Patient was started walking with knee brace. The power of the quadriceps femoris recovered gradually.
An 81-year-old British Caucasian male presented to the physicians with a cough, haemoptysis and an irregularly irregular pulse rhythm. A CT chest and upper abdomen demonstrated consolidation of the right lung base and he was treated for his pneumonia and atrial fibrillation. Shortly after admission he developed abdominal pain, diarrhoea and a low-grade pyrexia. His CT scan was reviewed and it was noted that there was a small amount of intra-abdominal fluid with several retroperitoneal lymph nodes. The right kidney had an atypical area of calcification within its upper pole and he was subsequently referred to the urologists for further evaluation (). As his condition failed to improve he underwent a further CT scan that demonstrated a large right psoas abscess (). This was drained under CT guidance and he was eventually discharged with a urology outpatient follow up as it was thought his renal problem was the cause of the abscess. Upon review the patient was symptomatically better, a diagnosis of an old calcified cyst in the right kidney was made and he was discharged to his GP.\nEight months later, after a permanent pacemaker had been fitted, an interval USS showed no residual psoas abscess. However, one month subsequently he was admitted under the general surgeons with a 3-week history of mild back pain, diarrhoea and rigors. He also had a discharging abscess in his right lumbar region which contained gas bubbles. A CT scan revealed sigmoid diverticulosis () with a recurrence of the extensive right-sided retroperitoneal abscess and a tract connecting it to the sigmoid colon ().\nAs an interim measure whilst being optimised for surgery, the abscess was drained with a 14F pigtail drain. At laparotomy the sigmoid colon was found frozen in the pelvis with perforated sigmoid diverticulosis noted to be the cause of the right sided retroperitoneal abscess. A sigmoid colectomy and primary anastomosis was performed and he was treated post-operatively with antibiotics. He made a slow but uneventful recovery and was discharged fit enough to undergo an open cholecystectomy for acute cholecystitis 16 months later.
A 27-year-old Japanese woman in the 26th week of gestation (gravida 1, para 0) was referred to the Obstetric Outpatient Clinic at Niigata University Medical and Dental Hospital for close examination of a fetal cardiac tumor. The patient had no complications, no genetic diseases, and had conceived spontaneously. She had undergone prenatal management and care at the hospital near her residence. Ultrasonographic examinations performed during the 25th week of gestation by the referring hospital demonstrated a fetal cardiac tumor. Ultrasonographic examinations performed at the first visit revealed a single, homogeneous tumor 14 mm in diameter in the intracardic space (). The tumor was believed to emerge from the ventricular septum and to occupy the left ventricle. A significant pericardial effusion was noted. The ventricular and atrial rates were 140 beats per minute and no atrioventricular block was observed. Other cardiac abnormalities were not detected, and the velocity of blood flow in the aorta and pulmonary arteries was within the normal range. The fetus was diagnosed with an intracardic tumor, possibly cardiac rhabdomyoma. Pedigree analyses revealed no family history of tuberous sclerosis.\nThe patient was managed in the outpatient clinic at our hospital. Serial ultrasonographic examinations were performed and fetal cardiac function was investigated. The size of the fetal cardiac tumor gradually increased (), although the fetal cardiac function was revealed to be within the normal range. The patient was hospitalized with the onset of spontaneous labor pain in the 39th week of gestation, and a female infant weighing 2716 g was delivered with Apgar scores of nine and ten at one and five minutes after delivery, respectively. The patient’s puerperal course was uneventful, and she was discharged from our hospital at the sixth day postpartum.\nAfter delivery, examination by a pediatric cardiologist confirmed that the infant had a cardiac tumor and the cardiac function of the infant was diagnosed as normal. There were multiple tumors; the largest tumor with a diameter of 26 mm was in the left ventricle and tumors with diameters of 5 to 15 mm were in the right ventricle, on the aortic and tricuspid valves, and at the apex. There was no evidence of outflow tract obstruction or hemodynamic compromise. The infant’s postpartum weight gain was observed to be normal. Computed tomography (CT) of the head revealed multiple calcified intracranial lesions (), which are symptomatic of tuberous sclerosis. Therefore, the infant was diagnosed with tuberous sclerosis. No surgical treatment was performed as there were no indications. The infant was discharged and has been managed at the outpatient pediatric clinic at our hospital.
This is a case of a 68-year-old female presented to the clinic for a 2-month history of recurrent left ankle pain, associated with swelling and edema. The patient had a history of thyroid follicular adenocarcinoma which was treated surgically with total thyroidectomy and postoperative iodine treatment 25 years prior to presentation. At 12 years post thyroidectomy, she was diagnosed with a calcaneal mass of the same pathology and diagnosed with delayed distant thyroid carcinoma metastasis to the calcaneus. 10 years after calcaneal mass excision, the patient was diagnosed with a proximal tibial mass that turned out to be also delayed metastasis of the same pathology.\nAt the first presentation 15 years ago, she initially presented complaining of mild edema of the left ankle with intermittent pain upon daily activity. Physical exam showed full range of motion of the ankle joint with intact motor power and intact neurovascular status. There were no cutaneous lesions.\nPlain radiographs of the left ankle showed a 3-centimeter oval lytic lesion in the anterior aspect of the calcaneum ().\nAn MRI of the left ankle showed a 3.2 cm well-defined benign-looking lytic lesion of the calcaneal neck reaching the cortex which appeared to be mildly irregular with mild degenerative disease of the posterior subtalar joint consistent with an intraosseous ganglion cyst of the calcaneus ().\nMedical treatment with NSAIDs and paracetamol was initiated, along with partial weight bearing and relative rest with no improvement at follow-up at 4 weeks. Surgical intervention was decided due to the persistent pain. The cyst was resected with a margin of surrounding fibro-osseous tissue and the bone grafted. Histopathological evaluation revealed a metastatic carcinoma of the thyroid gland. Immunostaining showed that the cells expressed cytokeratin, cytokeratin 7, and thyroglobulin, all of which confirm the diagnosis ().\nFor further confirmation, the recently excised cyst slides were compared to the pathology slides of the thyroid excision undertaken 12 years prior to the calcaneal presentation and were found out to be of the same pathology ().\nThe patient had a smooth postoperative hospital stay and clinical recovery from pain before discharge; postoperative follow-up showed necrosis of the upper part of the wound which healed by secondary intention.\nThe patient had a complete bone metastasis workup; chest X-ray showed a right upper lobe nodule for which an FNA biopsy under CT scan was done demonstrating the same pathology as for the calcaneus. A bone scan was ordered showing no definitive sign suggesting metastasis with no specific abnormality of the manubrium-sterni joint and the right proximal metaphysis of the right tibia which was nonspecific for distant metastasis according to the nuclear radiologist. After the discussion with the oncologist, decision for radioactive iodine therapy was made.\nAt 3-month postoperative follow-up, while the patient was undergoing chemotherapy, she recomplained of ankle pain upon ambulation, associated with edema. The patient had 5/5 motor strength, no numbness, and no sign of infection. A left ankle X-ray and MRI were ordered () to rule out any recurrence at the surgical site. The new investigations showed oval-shaped lytic lesion of 2.8 cm, and MRI showed increase in size of the calcaneal mass without pathologic fracture.\nThe patient was treated symptomatically; a follow-up MRI at 8 months showed postoperative enhancement in the surgical bed suggestive of viable tissue, and at 10 months, a follow-up MRI showed that there was near total healing of the calcaneal region.\nAfter 10 years post calcaneal mass excision, keeping in mind that the patient was symptom-free with respect to her ankle since the surgery, the patient presented to the clinic complaining of recurrent right knee pain. Investigation showed right proximal tibial metastasis with invasion of the patellar tendon. Surgical resection of the metastasis with repair of the patellar tendon was performed, and pathology was also consistent with thyroid follicular cell carcinoma (Figures and ).\nThe oncologist was consulted, and the decision for treatment with teroglobulin as well as adjuvant radiation therapy was made.
A 20-year-old male was transferred to our tertiary level one trauma center from an outlying community-based hospital following a noncontact basketball injury. Earlier that evening, the patient had been playing basketball at a local church when he jumped for a rebound and experienced severe pain in the left hip and thigh upon landing. He was unable to bear weight. At that time, he was taken to a local emergency department where he underwent immediate evaluation. Following the initial evaluation and review of radiographs, it was determined that the patient had sustained a subtrochanteric periprosthetic femur fracture. At that time, he was transferred to our facility for definitive management.\nAfter further investigation and questioning upon arrival at our facility, the patient reported a 5-6 month history of increasing left thigh pain prior to this injury. He had never received any medical care for the new onset thigh pain and attributed it to overuse. New radiographs obtained at our facility demonstrated a transverse periprosthetic femur fracture at the distal aspect of the previously placed dynamic hip screw. Nine years prior at the age of 11, the patient had been involved in a motor vehicle accident sustaining a subtrochanteric femur fracture which was addressed with reduction and placement of a dynamic hip screw (DHS). The surgery was noted to be performed flawlessly with no complications noted, but little is known about the details of the postoperative course as it was done at an outside facility.\nDespite discussions with the index surgeon to eventually remove the hardware, his case was never scheduled and the hardware was retained into skeletal maturity. Close inspection of imaging at the time of repeat injury showed evidence of a potential stress riser at the distal DHS/bone junction, with a significant amount of bony overgrowth of the DHS (). Additionally, there was evidence of stress shielding with significant cortical thickening involving the entire lateral cortex seen on radiographs taken prior to injury ().\nThe patient was taken to the operating room for explant, fracture reduction, and insertion of a cephalomedullary nail. An extensile approach was necessary to completely expose the retained hardware. Gross intraoperative inspection showed extensive bony overgrowth of the implant (). Osteotomies were utilized to remove the bony overgrowth to extract the hardware (Figures and ). After successful removal of the previous sliding hip screw, the fracture was exposed, reduced, and a long reamed cephalomedullary nail was placed with distal interlocking screws without difficulty (Figures –). Postoperatively, his pain was initially controlled with IV pain medication and he was quickly transitioned to orals only. He was immediately weightbearing as tolerated with the aid of a walker. He progressed quickly with PT, working with them once a day. He was able to walk 120 feet with PT on postoperative day 2 with the use of a walker. He was placed on Lovenox for DVT prophylaxis until he was fully weightbearing using no assistance. He was discharged home on postoperative day #2. He presented to the emergency department two weeks postoperatively complaining of bloody drainage from the incision. The dressing was removed by the ED physician who noted no drainage and was unable to express any blood. The patient was otherwise feeling well. His pain had been controlled, and he denied fevers, chills, nausea, or vomiting. A new dressing was placed and he was discharged home with follow-up with the orthopedic surgeon who performed the case. In outpatient follow-up, the patient continued with Lovenox for DVT prophylaxis as well as physical therapy until he was weightbearing as tolerated without the use of aids. At subsequent postoperative follow-up visits, radiographic imaging revealed complete union of the previous fracture with an abundant amount of bridging callus (Figures and ). The patient reported no other postoperative complications.
A 49-year-old Caucasian woman was referred to a general surgeon by her primary care physician for a right nipple lesion. She reports no past medical history, and social history is notable for tobacco use. The patient stated that she first noticed a small, yellow, and fleshy bump on her right nipple approximately two years prior. She became concerned due to its rapid growth in the last year. Four months prior, she visited her primary care provider who performed a shave biopsy of the lesion. Initial pathology results suggested squamous cell carcinoma, but this was thought to be discordant with the clinical picture. At consultation, she reported that her right breast had also started feeling hot and tender for two weeks duration. On examination, her right breast was erythematous and rigid with a 2.2 cm lesion consuming the right nipple. Her nipple also drained yellow pus. The patient was placed on a course of antibiotics due to concerns for an abscess.\nAn MRI was ordered to investigate possible underlying breast malignancy. Results of the MRI showed no solid mass underlying the nipple. A wedge biopsy of the nipple was performed to confirm the initial shave biopsy pathology (). Pathology showed verrucous histologic features with chronic inflammation and underlying abscess with concerns for a possible cutaneous malignancy. Specifically, there was marked papillomatosis with hypergranulomatosis between the papillae as well as a lack of granules at the papillary surfaces. Immunohistochemical testing was also positive for HPV L1 capsid protein. Because the initial shave biopsy showed SCC with positive margins, this clinical picture suggested that an inadequate sample was taken during the wedge biopsy. After consultation of literature, excision with wide margins was determined to be appropriate for removal of the lesion.\nThe patient underwent a wide excision of the right nipple-areola complex for removal of the growth. A 6.2 cm x 3.2 cm skin ellipse was excised encompassing the 2.2 cm nipple lesion. On final histologic examination, the lesion was found to be a large tumor with verrucous features, hyperkeratosis, dyskeratosis, and nests of keratin pearl formation (). Two areas showed microinvasion of the dermis with loss of the basal lamina. There were also significant acute and chronic inflammatory responses in the deep dermis representing an underlying abscess. These findings are congruent with the diagnosis of a well-differentiated squamous cell carcinoma with arising within verruca vulgaris. All margins were found to be clear of tumor cells. The post-op course was uneventful. She continues to follow up to monitor potential recurrence of the growth.
She was a 69-year-old found to be suffering from Alzheimer's disease. Relatives noted she was discussing her family matters with her own image. When asked, she declared the image as an old friend and she had no problems with identification of others in the mirror. While seeing her children directly, she declared that they were trying to cheat her by putting on the appearance of her children but could recognize them when they spoke to her from the next room or when their reflected images where shown. When she was told the images of her children and the people in front belonged to each other, she insisted that ‘those people’ did not carry the emotions and the mannerisms that were so familiar to her. She vehemently denied that the people in her front were her own children rather became quite defensive and said that “I am sure that they are not my children at all”. (The patient identified the reflected images of her children and by their voice and did not recognize when they came in front of them. The children were seated parallel to the patient in front of the mirror and only the same mirror (45 cm × 45 cm) was used. The patient did feel her old friend next to the image of the children. We did not make her speak to the children in this context when she was asked to identify the reflected image. She felt that the reflected image had all the characteristics of her children. When the children were in the different room, her ability to recognize was inferred by the way she responded to their oral communication from the next room. They were enquiring about general matters such as “How she's doing? What about her food? and their personal matters with reference to routine agricultural activities in the house. To these questions, she relied quite coherently and relevantly without showing any features of disinterest or suspicion but with a sense of authority and experience, which was reconfirmed by her relatives who were quite familiar with her similar premorbid conversational manner. She was started on anti-cholinesterases and after 12 months, the aforementioned symptom did not persist, but there was global deterioration and patient passed away due to coronary artery disease after two and half years.
A 19-year-old male patient with trauma was admitted to our hospital when he was in a shock state due to a motor vehicle accident. The patient had been resuscitated initially by infusion of crystalloids at the emergency department and intra-abdominal complications had been ruled out by physical exam and ultrasound studies.\nAt the beginning of emergent operation due to bilateral epidural hematoma, the patient was tachycardic with acceptable blood pressure (110/65 mmHg). Premedication with 2 Mcg/Kg of fentanyl was implemented. Induction of anesthesia was carried out by sevoflurane via an endotracheal tube previously fixed at the emergency department and appropriate dose of cisatracurium was used for muscle relaxation.\nAfter an hour of cranial decompressive surgery, both large intravenous lines became malfunctioning. For this reason, preparation of another large bore peripheral intravenous line was planned yet multiple trials of our expert colleagues failed and the medical team was forced to plan for establishment of a centrally inserted intravenous catheter. Surgical conditions and patient dressing forced the selection of the right femoral vein for this purpose. The patient had become hemodynamically unstable at that time and because of that, femoral artery pulsation wasn’t palpable in the anatomically expected site. Next, insertion of an introducer catheter next to the weak pulsation of femoral artery and a little below the inguinal ligament was decided. After appropriate preparation, central vein catheterization was performed uneventfully by a single trial and appropriateness of placement was confirmed by aspiration of all three lumens of the catheter and visualization of retrograde flow of liquid due to gravity and pressure difference laws. The catheter was then fixed to the skin and the entire area was dressed appropriately.\nDuring the surgery, infusion of 2 units of packed RBCs and 2 liters of crystalloid were done by the means of a central line. Infusion of norepinephrine was considered too because of diminished blood pressure right before thinking about central catheterization following a large amount of blood lost in the surgical field.\nAt the end of surgery, the patient was transferred to the intensive care unit while he was intubated and had a stable hemodynamic status.\nOne day after decompressive craniotomy, laparotomy was planned for the patient because of abdominal distension and ultrasound findings of the presence of a large amount of fluid in the abdominal cavity. It was found that misplacement of the femoralcatheter in the abdominal cavity was the main reason for accumulation of fluid. The femoral catheter was then extracted and patient care was continued at the ICU for the following days.\nThe patient had a right inguinal hernia simultaneously as the surgeon denoted following the second surgery. She also mentioned that the fluid in the abdominal cavity was largely in retroperitoneal space and fortunately there wasn’t any evidence of intestinal perforation. As she explained, retroperitoneal blood was in both sides and after removal of the catheter, bleeding of iliac vein perforation site was evident for a short time.
A 29-year-old man with a known case of hypothyroidism and HCV was in remission. He was referred to the hospital because of a huge abdominal mass on his lower left side that had been there for 7 years. It was associated with discomfort and heaviness, progressively increased in size, yielded no pain, no other swelling, no history of trauma or surgeries, and no constitutional symptoms. Therefore, otherwise, the patient was healthy.\nThe patient's BMI was 29.53. The mass measured about 15 × 13 cm in the left lower abdomen (). There were no signs of infection or skin abnormalities; the mass was soft and mobile, tethered to the skin, and was not pulsatile or tender. There were no abdominal hernias.\nBlood work was within normal limits. An MRI abdomen with contrast was taken and showed subcutaneous fat tissue with no worrisome underlying mass or abdominal enhancement to suggest liposarcoma. There was no definitive evidence of an abdominal wall hernia. The visualized portion of the pelvic organs and bones appeared to be grossly normal. The patient was admitted to the surgical ward for surgical excision of the mass and provided consent for the surgery.\nThe procedure was carried out under general anesthesia. The patient was placed in a supine position on the operating table, and the abdomen was prepared and draped in the standard sterile manner. A lower abdominal incision 8 cm from the root of the penis extended to the level of the anterior superior iliac spine bilaterally was made. The abdominal flap was undermined to a level just above the rectus fascia. Once the umbilicus was reached, it was freed from the raised abdominal flap circumstantially and the flap was raised to the level of the costal margin (). The mass was found to be diffused and arising from the subcutaneous tissue with no definite capsule. The mass was included and completely resected along with the excess abdominal skin. Hemostasis was secured, as two suction drains were inserted and brought out through the pubic area. Finally, the skin was approximated and closed in three layers using 0 vicryl for the Scarpas fascia, 2.0 vicryl for the deep dermal layer, and 3.0 monocryl for the subcuticular layer (). The wound was covered with a single layer of Dermabond and an abdominal binder was applied. The weight of the excised mass was about 2 kilograms ().\nThe specimen showed lipoma in the left side of the abdomen, which consisted of an oriented fibrofatty tissue measuring 20 × 20 × 10 cm and elliptical skin measuring 22 × 14 cm.\nThe patient had an uneventful hospital course and was discharged on the first postoperative day in a stable condition.\nThe patient was seen in the clinic 2 days after discharge, 7 days later, and 2 months after surgery (). The drains were removed once the drainage output was less than 30 mL in 24 hours. No complications were encountered.
A 78-year-old male patient with coronary artery disease status post coronary stent placement was found to have a lung nodule on the chest radiograph at that time. The patient underwent a computed tomography (CT) scan, and bronchoscopy, and was found to have a 9 mm fatty endobronchial lesion in the bronchus intermedius above the middle lobe with 2 cm extraluminal fatty lesion into the right hilum. An endobronchial ultrasound with biopsy of the mass was performed, which showed benign bronchial epithelial cells. Since endobronchial resection of the mass would lead to a large defect in the right bronchus intermedius, the decision was made to perform robotic-assisted resection of the lesion (Video ).\nWe used the Da Vinci Xi robot to perform resection of the endobronchial lesion and hilar mass with right lower lobe superior segmentectomy to remove the lesion. The patient had a “five on a dice” port placement for the operation [, ]. First, we performed the right lower lobe superior segmentectomy to obtain adequate exposure of the hilar mass. We mobilized the superior segmental branch of pulmonary artery and superior segmental branch of the right lower lobe going to the inferior pulmonary vein and divided them with the vascular robot stapler. We divided the superior segmental branch of right lower lobe bronchus with the robot blue load stapler. We used indocyanine green angiography to define the borders of the superior segment of the right lower lobe, which was divided using the robot blue load stapler. This provided access to the hilar fatty tumor, which allowed for removal of the hilar mass and subsequent resection of endobronchial lesion with scissors. The frozen section on both lesions was negative for malignancy. We confirmed complete resection with intraoperative bronchoscopy that also showed a large opening in the airway.\nIn order to reconstruct the airway, we placed two 3-0 vicryl stay sutures at the proximal and distal ends of the airway and placed the suture through the posterior ports to pull the airway posteriorly away from the main pulmonary artery. We closed the opening with 4-0 PDS (polydioxanone) in an interrupted fashion eight times. This provided good closure of the opening. We performed a bronchoscopy that showed no abnormalities and the air leak test demonstrated no appreciable air leaks.\nThe patient went home on postoperative day 3 without any complications. The final pathology report was lipomatous hamartoma.
A 25-year-old African American married female with a past medical history of a previous pregnancy that resulted in a missed abortion, depression, bilateral MCA stroke, sickle cell trait, and mitral valve congenital defect replaced with St. Jude mechanical valve in 2001 presented at 11 weeks pregnant for management of high-risk pregnancy. The patient was diagnosed at birth with a mitral valve defect which was surgically repaired at age 2. However, at the age of 12, her native mitral valve was replaced with a St. Jude Master Series 27 mm mitral valve prosthesis. After replacement with mechanical mitral valve, she was prescribed oral warfarin therapy for anticoagulation. Her anticoagulation was managed by her cardiologist at an outside facility with a target INR of 2.5 to 3.5. The patient admitted to being compliant with her warfarin therapy. The patient only noted one prior ischemic event, a TIA at 12 years old, which was attributed to a missed medication dose. She said her mother forgot to give her the medication prior to the TIA. Her INRs were checked weekly although the patient did not have any lab work to present at the time of presentation. Approximately seven months prior to presentation, the patient became pregnant for her first time. She was switched from warfarin to Lovenox therapy to avoid teratogenic effects of warfarin to the fetus. Lovenox dosage was 60 mg (1 mg per kg dosing) administered twice daily, with antifactor Xa monitored by her cardiologist. The patient admitted to strict compliance to this anticoagulation regimen without any missed dosages. During her 8th week of pregnancy, the patient presented to the emergency department with a sudden onset of difficulty speaking and bilateral facial/upper extremity weakness. She was diagnosed with bilateral MCA infarcts and not deemed a candidate for intervention due to her pregnancy. During her admission for the stroke, the patient suffered a missed abortion. She was eventually discharged to a rehabilitation center for poststroke care and regained full motor and speech function. Approximately 3 months after her initial stroke, the patient became pregnant again. With this subsequent pregnancy, she was advised by her cardiologist to switch from warfarin therapy to Lovenox as she had done in the previous pregnancy. However, the patient refused given concern over another stroke even though she was aware of teratogenic risks to the unborn child. Although she was on a therapeutic dose of warfarin with regular INR checks, the patient had noted intermittent tingling around her mouth and numbness in her fingers during her first trimester, findings consistent with a TIA.\nShe was admitted to the hospital under the OBGYN service with consultation to cardiology for recommendations on anticoagulation. It was noted that she was on a daily dose of 10 mg warfarin, and her INR was therapeutic at 3.0 at the time of hospital admission. In anticipation of possible procedures, her warfarin was discontinued, and the patient was placed on a heparin infusion with goal activated partial thromboplastin time (aPTT) two times greater than the control. Electrocardiogram showed normal sinus rhythm. Given concern for valve thrombosis, transthoracic echo was ordered for further evaluation of mechanical valve. The transthoracic echo showed decreased posterior leaflet motion of the mechanical valve. As a result, transesophageal echo was performed which revealed normal valve motion and no evidence of thrombus. Normal atria and ventricle sizes were noted with left ventricular ejection fraction at 65–70%. Hematology was consulted for further workup for thrombophilia. Workup was unremarkable except she was noted to be heterozygous for MTHFR C677T gene. Homocysteine was within normal limits. Upon discharge, the patient was again advised on the need to switch to Lovenox therapy for anticoagulation; however, the patient refused and requested to be placed back on warfarin. Warfarin was restarted at 10 mg per day, and heparin drip was continued until therapeutic INR could be reached. The patient was discharged with follow-up with outside providers for INR checks and continued prenatal care. Since the patient did not follow up at our facility, it is unknown if she was able to deliver to term without any ischemic events or any effects to the fetus.
A 50-year-old male with a past medical history of chronic cocaine abuse was found unresponsive in the field. He was given multiple doses of naloxone in the field without any improvement in mental status. On presentation in the emergency department (ED), the patient was found unresponsive to questions and unable to follow commands but withdrawing to painful stimuli, with a Glasgow Coma Score of 8, and tachypneic. Vital signs were significant for a respiratory rate of 38 breaths per minute and oxygen saturation of 90% on 6 liters nasal cannula. Physical exam appreciated no tracheal deviation and no jugular venous distention but exhibited an uneven rise and fall of his chest on the right side compared to the left. On cardiopulmonary auscultation, absent breath sounds of the right lung fields were noted with normal cardiac heart sounds. The patient was emergently intubated for airway protection in the ED, and further evaluation was initiated. Immediate imaging with portable chest X-ray was obtained which showed extensive lucency in the right hemithorax suggestive of large pneumothorax or an air-filled cyst (). A CT scan of the chest was performed which suggested extensive pneumothorax of the right lung without a mediastinal shift ().\nFor this reason, an emergent 24 French chest tube was placed in the right 6th intercostal space but failed to expand the lung. In response, the chest tube was then exchanged for a 32 French tube under the assumption that a larger size may be required, but it too failed to expand the lung. There was no air drainage from either tube placed suggesting lack of a pneumothorax. He was stabilized and admitted to the intensive care unit for further management. A few hours after admission, he underwent bronchoscopy with a right chest VATS which revealed a giant bulla encasing the entire right pleural cavity with its origin in the right upper lobe beginning at the apex. The bulla was extracted, and pleurodesis was performed by mechanical and chemical means. After the procedure, the patient's respiratory status significantly improved. His partial pressure of oxygen on arterial blood gas was 105 on a fraction of inspired oxygen of 40% and positive end-expiratory pressure of 5. He had excellent ventilatory weaning parameters and was finally extubated to nasal cannula on day 6 of hospitalization. He was transferred to the general medical floors the following day and discharged without supplemental oxygen requirement in a stable condition on day 8. shows post-VATS chest X-ray at discharge. The patient was evaluated for various causes of GBE while inpatient. Alpha-1 antitrypsin levels were within normal limits, and HIV was not detected. Evaluation of malignancies and cystic lung diseases yielded negative results. Urine drug screen was positive for cocaine, and the patient endorsed no tobacco smoking history and a long history of intravenous cocaine use as well as intranasal prior to discharge. On outpatient follow-up 2 weeks after discharge, the patient remained symptom-free and in good health.
A 44-year-old male patient was admitted to our emergency department due to a seizure attack while he was undergoing brain magnetic resonance imaging (MRI) scan at a local hospital. He presented a history of a sudden onset of severe headache three weeks prior to his visit to our institution. Except the surgical history of cholecystectomy in 2014, he had neither a history of epilepsy nor medical diseases. He was an alert patient without neurologic deficit on his first arrival. The initial brain computed tomography (CT) scan demonstrated a 3.5 × 3.2 cm sized oval shape mass filled with a thrombus. A midline shift to the left of 3 mm and a slightly decreased size of right lateral ventricle were observed. The brain CT scan with contrast showed a mass-like lesion with a heterogeneous enhancement. It was identified to be a large tortuous vessel connected to an aneurysmal sac arising from the inferior trunk of right middle cerebral artery (MCA) according to the brain CT angiography (). The brain MRI revealed a mass of heterogenic signal intensity suggesting different stages of thrombus formation within the aneurysmal sac (). The patient consequently underwent a distracted subtraction angiography (DSA). We observed a contrast pooling in the inferior trunk of right MCA and a dilated outflow, which was drained to the distal MCA branches ().\nUpon his hospital admission, he had no further seizure events, but, his symptoms of headache and dizziness remained the same consistently. Subsequently, a BOT was carried out to assess the patency of collateral cerebral circulation. After the proximal occlusion of the aneurysm by Sceptor C balloon (Microvention, Tustin CA, USA), the following parameters were intensively monitored for half an hour: the patient's somatosensory evoked potential (SSEP); cognitive and language function; and motor and sensory changes. The patient did not present any neurologic symptoms during the test ().\nBased on the BOT results, an endovascular embolization was planned. 100 mg of aspirin and 75 mg of clopidogrel were given to the patient for seven days as a premedication method. On the day of coil embolization, a proximal artery occlusion by a dual catheter technique was planned. Firstly, we placed two catheters in the proximal artery of right MCA inferior trunk. Then, coiling was performed with two catheters in turn (). After coil embolization, the aneurysmal sac was completely occluded with its distal outflow. The patient was alert with no neurological symptoms immediately after the embolization. However, the next day after coiling, he was deteriorated to a drowsy mental status with dyscalculia. Diffusion weighted imaging of brain MRI was performed immediately in the acute setting of suspected stroke. A focal area of restricted diffusion involving right temporo-parietal lobe was observed (). The treatment protocol with mannitol and hypertonic saline was added while maintaining the oral medication with aspirin and clopidogrel. On the post-operative day 22, the patient's mental status improved and was noted as being alert even though he still exhibited mild Gerstmann syndrome. Two weeks after his hospital discharge, the follow-up brain CT scan showed a resolved mass effect and a decreased size of a low density lesion ().
A 42 year old female presented with a progressively enlarging painless swelling in the anterior part of the neck since 1 year. The only complaint was that of disfigurement. There was no other significant history. On examination, a single swelling was present in anterior neck, 13 × 8 cm in size, irregular in shape, extending vertically from thyroid cartilage above to supra sternal notch below and between two sternomastoid muscles. It was firm in consistency, moved freely with deglutition and had ill defined lower margin. There was no cervical lymphadenopathy. Examination of respiratory, cardiovascular, nervous systems and abdomen were normal. Thyroid function test was within normal range and FNAC (fine needle aspiration cytology) of the thyroid swelling revealed papillary carcinoma. Ultrasound of the neck documented a single mass in anterior neck, 12 × 8 cms in size with complex cystic and solid components without any associated cervical lymphadenopathy. The patient was planned for total thyroidectomy. Intra operative findings included slightly enlarged left lobe of thyroid (4 × 3 × 3 cm) with normal sized right lobe (3 × 3 × 2 cm). Another swelling (thymus) about 8 × 4 × 3 cm in size was found incidentally, adjacent to but separate from the thyroid at its lower margin and extending upto suprasternal notch. The thyroid and the thymus were only connected by a fibrous band (figure ). Total thyroidectomy with thymectomy was performed. Post operative period was uneventful. The histopathological examination of the thyroid specimen revealed papillary carcinoma (figures and ) and the sections from attached mass (thymus) revealed multiple cysts with its tissue replaced by metastatic papillary carcinoma of thyroid (figures and ). Both tumors were reactive to thyroglobulin, keratin and CD3 confirming papillary carcinoma of thyroid with metastasis to ectopic cervical thymus. Radioiodine scan was done on follow up which did not detect any residual or any other metastatic disease. The patient is on regular follow up and is presently doing well.\nPapillary carcinoma thyroid is the most common type of thyroid malignancy seen in the population especially females. This tumor usually has a good prognosis. It spreads via lymphatics and is commonly associated with enlarged cervical nodes. Bones and lungs are the usual sites of distant metastasis [], however only one case of thymic metastasis has been reported till date [].\nAs the thymus is an essential organ that controls the cellular immunity function, it has been considered almost impossible that a tumor could metastasize to the thymus. Although tumors almost never metastasize to the thymus, precise observation of the thymic structure has revealed that the thymus is not absolutely safe from tumor metastasis. The parenchyma of the thymus has a blood thymus barrier, which prevents the thymus from making direct contact with antigens or cancerous cells, thereby seemingly excluding the occurrence of cancer metastasis. However, the septum of the thymus is comprised of interlobular connective tissue with blood vessels, lymphoducts and nerves, which theoretically does not exclude the possibility of metastasis. Blood-thymus barrier is not as robust in the medulla of the organ as it is in the cortex. Also, it is not that blood-organ barrier can always prevent metastasis. Brain, eye and testis also have a blood-organ barrier and metastases in these organs have also been reported. Therefore, when the structure of the thymus is precisely analyzed, a remote possibility of the thymic metastasis from tumors is imaginable [].\nEmbryologically, the thymus originates from the third pair of branchial pouches high in the neck during early foetal life and reaches its final destination in the mediastinum after a process of progressive decent. Rarely thymus fails to decent and appears as a remnant, implant, or accessory nodules any were along the cervical pathway, the most commonest site being at the level of thyroid gland []. Adult cases of ectopic thymus are exceedingly rare due to age related involution and replacement by fibro-adipose tissue. Ectopic thymus tissue like its normal counterpart may also undergo transformation to thymic hyperplasia or even thymic neoplasia [].\nThere are very few reports of thymic metastasis including those from breast [], prostate [], testis [] and thyroid cancers [,]. Our case represents a rarest case of papillary carcinoma thyroid with metastasis to the ectopic cervical thymus, which was found incidentally during thyroid surgery and was confirmed by histopathology and immunohistochemistry. The metastatic spread from thyroid cancer to ectopic thymus is presumed to be of haematogenous origin in absence of radiological or histological evidence of any local or nodal spread.
A 42-year-old man with end-stage renal disease due to a combination of diabetes mellitus and cardiovascular disease presented to the emergency room with shortness of breath. Due to his lack of health-care insurance and ineligibility for funding to receive outpatient dialysis, he presented several times to the ED for intermittent dialysis where he was admitted and dialyzed if his symptoms met criteria for emergent dialysis. He survived this way for many years. On this presentation for emergent dialysis, he suffered a cardiac event requiring intubation for a cardiac cath. It was during extubation that the clinician noted marked the prominence of the palate and mucosal ulceration. Once stable from the procedure, further history from the patient was elicited. The patient noted that the bulky hard mass on his palate had been present for several years and grew slowly overtime. He also noted progressive swelling and disfigurement of his face and noticed a hard mass along his mandible which had also grown overtime. The mass was not associated with pain and because he did not have insurance, he did not go to the doctor for further evaluation. He did not know what was causing the changes in his face. Oral maxillary surgery was consulted and they requested a computed tomography (CT) maxillary face [] to be performed for further evaluation. It revealed severe enlargement of the maxilla and mandible with a coarse, marbled appearance of the bony matrix seen as alternating wavy bands of lucency and sclerosis. The bony expansion was most prominent at the maxilla and mandible with a bulky expanded hard palate accounting for the mass seen during extubation. These abnormal bony changes were also seen in other bones of the face, though less prominent, and at the skull base.\nOn review of the patient’s chart and its documentation during multiple prior admissions for emergent dialysis, additional imaging and laboratory tests were analyzed. The patient had a long history of significantly elevated creatinine levels up to 17.5 mg/dL (normal 67–1.17 mg/dL), low glomerular filtration rate (GFR), and elevated blood-urea-nitrogen up to 125 mg/dL (normal 6–26 mg/dL) documented for over 7 years. Extremely elevated parathyroid hormone (PTH) was also noted, which had also increased over the years and was now >5000 pg/mL (normal 15–35 pg/mL), possibly greater as more definitive quantitative analysis was not routine beyond 5000 pg/mL. There was progressive, marked elevation of alkaline phosphatase reaching 740 units/L (normal 40–129 nits/L) and new hypocalcemia that was seen only in the past 7 months. Serum calcium was borderline decreased ranging from 8.1 to 8.3 mg/dl over the past few months (normal 8.5–10.1 mg/dl). There was also documentation supporting severe cardiovascular disease resulting in heart failure and diabetes mellitus.\nA review of CT abdomen [] reveals an atrophic appearance of the kidneys containing multiple small cysts typically seen in patients with end-stage renal disease who are on chronic hemodialysis and atherosclerotic calcification throughout his vasculature. Imaging [-] also showed decreased bone density in the spine and pelvis and bands of sclerosis along the vertebral endplates, a characteristic feature of metabolic bone disease specific to hyperparathyroidism. In light of end-stage renal disease, laboratory and imaging findings as well as the history of progressive, painless expansion of the bones of the face over several years, uremic leontiasis ossea was diagnosed. After the cardiac event and subsequent cardiac cath, the patient left the hospital against medical advice.
A 47-year-old male presented with severe pain characterized with radicular behavior spreading on the neck and right arm and an onset of numbness in the first and second fingers of the right hand. It was learned that the patient had slipped and fallen down on his back about 20 days before but had no complaints afterward, and 10 days after the minor trauma, he suddenly started to have very severe pain in the neck root. His pain had gradually grown progressively and begun to spread to the right arm. Radiological findings showed compression on the spinal canal and deterioration of the cervical lordosis due to height loss in the C6 vertebra corpus [–].\nIn the present case, it was thought that the pathological compression fracture (collapse) in C6 corpus may be secondary to infection or due to metastasis. In the examination for a primary etiologic cause, a large mass with a diameter of 10 cm was found in the left kidney in abdominal computed tomography (CT) images. Due to his severe pain, a cervical sixth vertebrae corpectomy was performed through the anterior without delay. A 7–8 cm bone autograft was taken from the middle section of his own right leg fibula to put it in the gap created after the corpectomy. The top and bottom ends of the fibula graft were smoothened with a high-speed drill. The size of the graft was tried to be kept high, especially to ensure physiological lordosis. During the surgery, the fibula was implanted at the distance cleared by applying traction on the patient's head. After ensuring that the lordosis was obtained, and the graft was in the right place, the fibula was supported using an anterior plaque to reduce the likelihood of the fibula to come loose. Postoperative cervical vertebra was controlled by lateral cervical X-ray and CT [ and ].\nPostoperative pain occurring on the leg of the case did not prevent mobilization on the 1st day. On the 1st day after the operation, his radicular pain in the neck and in the arm was gone almost completely. The pain in his leg gradually diminished at the end of the 1st week. The definitive pathology was evaluated as RCC metastasis []. Radical nephrectomy was performed 2 weeks later for the other, newly identified kidney mass. At the end of the 6th year of the case, there was still no recurrence of a tumor lesion on the cervical vertebra and the rest of the body. At the end of the 6th year, radiographs of the patient were being followed up to see how well the fibula was fused [].
A 28-year-old primigravida was referred to our hospital at 31 + 2 weeks of gestation for further evaluation as a result of abnormal findings on a fetal echocardiography. At 29 weeks of gestation, blindly ended vessels originating from the heart or transposition of the great arteries was first noticed. However, no comments were made regarding any abnormal findings of the fetal lung on a fetal ultrasonography. In addition, the medical and obstetric histories were unremarkable.\nAn ultrasound examination performed on the day of the first visit at our institute demonstrated an elevated diaphragm, raising the suspicion of associated lung abnormalities (). The thoracic cavities, especially on the right, were reduced in size. The left thoracic cavity was largely occupied by the heart with a severely left-deviated axis, resulting from the remarkable displacement of the diaphragm cephalad. However, it was not evident whether any lung tissue was present, or whether there were any lung abnormalities or pulmonary veins connecting to the left atrium as well as the right and left branches of the main pulmonary artery. As a result, no specific structural abnormalities of the heart were identified.\nFor further evaluation of the lung abnormalities, a fetal MRI study was performed. Similar to the results of the ultrasound examinations, a significantly decreased thoracic volume associated with a bilateral elevation of the diaphragm was identified, thus resulting in an upward migration of the heart and rotation of the cardiac axis. Moreover, no definite lung was identified and the trachea ended blindly near the level of the cricoid cartilage without bronchial branching (). Together, these findings strongly supported the diagnosis of BPA.\nA male fetus weighing 2,530 g was delivered vaginally at 39 + 2 weeks of gestation after the induction of labor and in the presence of neonatologists. The Apgar scores at 1, 5, and 10 minutes after birth were 2, 2, and 2, respectively. Attempts at resuscitation were unsuccessful and after 15 minutes, the cardiac asystole persisted despite all efforts. The neonate died at 44 minutes after delivery. A postmortem infantogram was taken and the results of the study were consistent with the prenatal diagnosis (). The karyotype of the neonate was 46, XY.\nThe body size of the fetus was appropriate for its gestational age. There were no congenital abnormalities noted on external inspection; however, multiple abnormalities were noted on an internal examination. The intact diaphragm was elevated bilaterally. A complete agenesis of both lungs was identified along with the absence of lung tissue and bronchial rudiments (). In addition, neither pulmonary arteries nor veins were identified. An atretic trachea ended blindly at the level of the cricoid cartilage and esophageal stenosis was noted. The cardiac axis was rotated due to a mediastinal shift to the left. Four chambers of the heart had developed, however the main trunk of the pulmonary artery had no branches and joined the aorta at the site of the persistent ductus arteriosus. Lastly, a large atrial septal defect was present.
Mr B was a 62 year old normally fit and active man who was referred to the chest clinic for an ovoid lesion on his chest X ray. He initially presented to another district general hospital 1 year prior with fever and abnormality on chest radiograph (). At that time the lesion was aspirated and thought be an abscess. He was treated with antibiotics and discharged. The aspirate showed no organisms and no malignant cells.\nHe then represented to his general practitioner ten months later who referred him to our chest clinic. He presented with a six week history of cough and some left sided chest discomfort. There was no history of sputum production. His weight and appetite were stable and there were no fevers, night sweats or haemoptysis.\nHe was an ex-smoker having smoked heavily in the past. There was no known asbestos exposure. He had no significant past medical or family history and he currently lived with his male partner and was still working for a leading supermarket.\nOn examination there was no finger clubbing or lymphadenopathy. Chest examination revealed reduced breath sounds at the left base.\nChest radiography and CT examination ((a),(b),(c)) revealed a 10 cm ovoid lesion in the left lower lobe adjacent to the pleura. The penetration suggested it was a fluid filled structure. A similar looking lesion was also noted in the left adrenal (). He underwent a fibre-optic bronchoscopy. Some brown adherent material was seen at the orifice of the posterior and lateral segment of the left lower lobe. Washings and biopsies were taken. Cytology and microbiology were all negative for malignancy. He also underwent aspirations of the left lower lobe lesion and adrenal lesion. Only blood was aspirated and cytology was once again negative. HIV test was performed and was negative.\nA few weeks later he developed a painful 5 cm left axillary lymph node and had this biopsied too. The biopsy confirmed metastatic adenocarcinoma suggestive of primary lung origin (). At this stage his performance status was still 0 and following discussion at lung MDT, it was agreed to refer him to the clinical oncologist for palliative chemotherapy. Mr B subsequently developed symptoms of headache. Imaging of the brain (CT followed by MRI) revealed a space occupying lesion in the left parietal lobe surrounded by moderate oedema (). This was also felt to have the appearance of blood filled cystic lesions when reviewed by the radiologist. Prior to commencing chemo- or radiotherapy his case was discussed with the neurosurgeon who felt surgical intervention would be associated with high risk of mortality and can leave him with significant physical disability. Unfortunately, the day following his neurosurgical review, this lesion progressed quite rapidly causing a dense hemiplegia and slurred speech and at this stage it was felt Mr B was not fit for any active treatment. He was treated by means of symptom control and died some weeks later.
A 65-year-old Hispanic female presented to emergency department (ED) with a 6-month history of right ear pain that did not respond to ciprodex and keflex. The patient described initial drainage of clear fluids which became serosanguineous over the last 3 months. The patient denied any history of smoking or alcohol abuse.\nIn the ED, otoscopic examination of the right ear showed that the external auditory canal was edematous with clear discharge and bulging of the tympanic membrane. The patient was found to have decreased sensation over the right side of the face in the second and third trigeminal region but normal sensation over the right forehead. She had right eye ptosis and paralysis. The patient was unable to protrude her tongue, and there appeared to be a facial droop over the right side.\nA CT scan of her right inner ear and temporal bone showed opacification of the right external auditory canal and bony destructive changes of the anterior wall of the right external auditory canal, right tympanic and epitympanic spaces, and mastoid antrum. The CT also showed destructive changes of the anterior wall of the right external auditory canal, scutum wall of the right carotid canal and foramen lacerum, and wall of the right semicircular canal ().\nA magnetic resonance imaging scan of the neck revealed extensive heterogeneous signal abnormality and enhancement centered about the right external auditory canal and middle ear region (). Similar abnormality and enhancement were noted in the right temporal bone extending into the carotid and parapharyngeal spaces. There was also an equivocal 9 × 7 mm fluid collection in the carotid space and associated dural enhancement in the sigmoid sinus. The patient was thought to have a severe otitis media and was treated with antibiotics and was to follow up with the ear, nose, and throat clinic as an outpatient.\nHowever, the patient was readmitted again three weeks later with symptoms of nausea and vomiting and worsening of the right ear pain. On readmission, the patient was found to be hyponatremic with altered mental status. The lesion of the right external auditory canal was biopsied. The pathology report showed a well-differentiated squamous cell carcinoma ().\nThe patient underwent a positive electronic transmission scan of the full body which showed that her tumor was confined to the right external and middle ear with no metastasis.\nThe patient had stage T4NO disease based on radiological CT imaging of the right ear which showed that the tumor has penetrated through the right external auditory canal into the scutum and walls of the right carotid and foramen lacerum. There was also evidence on the CT and MRI showing local invasion of the facial nerve in the middle ear (); this was thought to be the cause of her facial paralysis.\nThe patient was evaluated by head and neck surgery, and her tumor was found to be unresectable due to the extensive local invasion. The patient received induction chemotherapy consisting of carboplatin, paclitaxel, and cetuximab for 2 cycles before getting palliative short-course radiation therapy. The patient was referred to alive hospice for palliative care and died several weeks later.
At the end of January in this year, a 46-year-old woman noticed for the first time a change on the skin of her upper lip resembling “a spot, the size of a wheat grain”, which grew rapidly and during the period of two weeks attained a size of 0.6 cm. The change was red, hard and painful to touch, attached to the skin. It was surgically removed. Primary histopathological finding was follicular keratosis. Recurrent tumor appeared in surgical incision area only seven days after the operation, as well as the onset of extreme swelling of the upper lip tumorous tissue (size < 2 cm). The patient was sent to a tertiary hospital. After the hospital admission, the fungal or bacterial infection of the lesion was excluded. Soft, reddish nodular tumor with the size of 5 × 2 cm with sebaceous cysts and telangiectasias was clinically observed ( and ). Swollen lymph nodes (< 13 mm diameter) were detected on both sides of the neck and in the right axillary fossa there was one swollen lymph node around 10 mm in size. All biochemical and serological blood tests were within normal reference ranges. Well differentiated, keratinized, squamous cell carcinoma of the upper lip was diagnosed after the histopathological audit. Tumorous tissue infiltrated all papillary and reticular dermis, muscle fibers and perineural spaces and is visible on the lower resection line ( and ). With the second surgical intervention the upper lip skin cancer was removed and the defect reconstructed. Final postoperative result was shown in the . In the time of the second surgical intervention, lymph nodes on both sides of the neck could not be identified either on palpation or by ultrasound examination. Earlier detected lymph node, with almost normal ultrasound characteristics, could not be seen or palpated, thus there was no need for a lymph node biopsy. Postoperative findings of lymph nodes showed only transient reactive lymphadenopathy. Further oncological treatment was not advised for the patient, only subsequent medical check-ups of the operated area.
This is a case of a 68-year-old female presented to the clinic for a 2-month history of recurrent left ankle pain, associated with swelling and edema. The patient had a history of thyroid follicular adenocarcinoma which was treated surgically with total thyroidectomy and postoperative iodine treatment 25 years prior to presentation. At 12 years post thyroidectomy, she was diagnosed with a calcaneal mass of the same pathology and diagnosed with delayed distant thyroid carcinoma metastasis to the calcaneus. 10 years after calcaneal mass excision, the patient was diagnosed with a proximal tibial mass that turned out to be also delayed metastasis of the same pathology.\nAt the first presentation 15 years ago, she initially presented complaining of mild edema of the left ankle with intermittent pain upon daily activity. Physical exam showed full range of motion of the ankle joint with intact motor power and intact neurovascular status. There were no cutaneous lesions.\nPlain radiographs of the left ankle showed a 3-centimeter oval lytic lesion in the anterior aspect of the calcaneum ().\nAn MRI of the left ankle showed a 3.2 cm well-defined benign-looking lytic lesion of the calcaneal neck reaching the cortex which appeared to be mildly irregular with mild degenerative disease of the posterior subtalar joint consistent with an intraosseous ganglion cyst of the calcaneus ().\nMedical treatment with NSAIDs and paracetamol was initiated, along with partial weight bearing and relative rest with no improvement at follow-up at 4 weeks. Surgical intervention was decided due to the persistent pain. The cyst was resected with a margin of surrounding fibro-osseous tissue and the bone grafted. Histopathological evaluation revealed a metastatic carcinoma of the thyroid gland. Immunostaining showed that the cells expressed cytokeratin, cytokeratin 7, and thyroglobulin, all of which confirm the diagnosis ().\nFor further confirmation, the recently excised cyst slides were compared to the pathology slides of the thyroid excision undertaken 12 years prior to the calcaneal presentation and were found out to be of the same pathology ().\nThe patient had a smooth postoperative hospital stay and clinical recovery from pain before discharge; postoperative follow-up showed necrosis of the upper part of the wound which healed by secondary intention.\nThe patient had a complete bone metastasis workup; chest X-ray showed a right upper lobe nodule for which an FNA biopsy under CT scan was done demonstrating the same pathology as for the calcaneus. A bone scan was ordered showing no definitive sign suggesting metastasis with no specific abnormality of the manubrium-sterni joint and the right proximal metaphysis of the right tibia which was nonspecific for distant metastasis according to the nuclear radiologist. After the discussion with the oncologist, decision for radioactive iodine therapy was made.\nAt 3-month postoperative follow-up, while the patient was undergoing chemotherapy, she recomplained of ankle pain upon ambulation, associated with edema. The patient had 5/5 motor strength, no numbness, and no sign of infection. A left ankle X-ray and MRI were ordered () to rule out any recurrence at the surgical site. The new investigations showed oval-shaped lytic lesion of 2.8 cm, and MRI showed increase in size of the calcaneal mass without pathologic fracture.\nThe patient was treated symptomatically; a follow-up MRI at 8 months showed postoperative enhancement in the surgical bed suggestive of viable tissue, and at 10 months, a follow-up MRI showed that there was near total healing of the calcaneal region.\nAfter 10 years post calcaneal mass excision, keeping in mind that the patient was symptom-free with respect to her ankle since the surgery, the patient presented to the clinic complaining of recurrent right knee pain. Investigation showed right proximal tibial metastasis with invasion of the patellar tendon. Surgical resection of the metastasis with repair of the patellar tendon was performed, and pathology was also consistent with thyroid follicular cell carcinoma (Figures and ).\nThe oncologist was consulted, and the decision for treatment with teroglobulin as well as adjuvant radiation therapy was made.
A 40 year old man presented with a painful mass at the medial side of his arm. On clinical examination there was a painless solid mass 6–7 cm in length, little sensitive to pressure, firmly attached to deeper tissues. Percussion over the mass produced a Tinel’s like sensation along the median nerve. The patient experienced mild numbness in the distribution of the median nerve in the palm but no motor weakness or muscle atrophy was detected. No cutaneous pigmented lesions were found. The patient reported he first palpated a nodule 10 years ago that grew up very slowly to the current size. No history of significant trauma was recalled.\nAn MRI examination revealed a 6 cm long, well circumscribed mass in close proximity to the neurovascular bundle of the arm. The lesion had a relatively homogenous low signal, slightly lower to the muscle, on T1 images. On T1 fat suppressed images, increased gadolinium enhancement was noticed at the periphery of the mass with a nonenhancing low signal central area (Fig. a). No peri-tumoral edema was present and a line of fat surrounded the tumor. Based on the long history of the patient, and the clinical and MRI features, the diagnosis of a nerve sheath tumor arising from the median nerve was supported and the patient was scheduled for excisional biopsy. A longitudinal incision centered over the tumor bulk was performed. The tumor had an eccentric position and was firmly attached to the median nerve, (Fig. b). An incisional biopsy was performed and tissue sample was taken from the proximal pole of the mass (where more viable tissue was seen on MRI). The sample was considered to be representative of the entire pathology macroscopically and was sent for frozen section. Intra-operative biopsy examination revealed nuclear atypia. Howeve, significant mitotic activity was not seen and the lesion was characterized as a benign peripheral nerve sheath tumor. The decision to preserve the median nerve and perform a marginal tumor excision was undertaken. Under high power magnification, the epineurium was longitudinally incised and the soft tumor mass dissected from surrounding nerve fascicles. Internal neurolysis started from the poles of the mass and a few nerve fascicles entering to the mass had to be sacrificed. Immediately post-operatively the patient experienced minor sensory loss in the median nerve territory of the palm without noticeable weakness of the muscles innervated by the median nerve. At 6 months follow up the wound is well healed with no pain upon palpation. The upper extremity is neurologically intact except for the partially improved numbness mainly in the long finger. The patient returned to full labor work as a fisherman.\nThe final gross pathology evaluation demonstrated an ovoid tumor measuring 5.5 × 3 × 2.5 cm. The center of the mass was cystic filled with a brown colored fluid. Histologically there were alternating hypocellular and hypercellular areas (Fig. ). The hypercellular areas showed elongated spindle cells with nuclear palisading forming occasional Verocay bodies, whereas the hypocellular areas showed loosely arranged neoplastic cells in a myxoid background with hyalinized thick-walled blood vessels and foci of hemorrhage with hemosiderin-laden macrophages. In these areas significant nuclear atypia and pleomorphism with multinucleation and nuclear invaginations were seen, however mitoses were scarcely found and atypical mitotic figures or necrosis was not observed. The S-100 stain was positive. Immuno-staining with the proliferation index Ki67 (MIB-1) displayed a low proliferating index. The final pathology report was consistent with ancient schwannoma.
A 39-year-old male farmer was referred to our emergency department with generalized pain on the right leg with loss of the skin throughout the lower leg leaving the soft and hard tissue exposed to the environment shown in . He could not move his right leg and feel no sensation at some part of the limb. No history of loss of consciousness, amnesia, and headache was reported. He was a smoker and occasional drinker but did not have allergic and vascular risk factors such as hypertension, diabetes, or dyslipidemia. There was no history of medication use.\nTwo weeks earlier, he had a motorcycle accident and crashed into a truck with the right leg as the focus of impact. He was immediately brought to the hospital, where he was taken plain X-Ray on the right leg with suspicion of open fracture of the right lower limb. Clinically, the patient was alert and complained that he had intense pain and unable to move the right leg. The doctor explained the urgent need for operation as there was an open fracture observed, but the patient decided to discharge against medical advice and sought home treatment with a TBS. During the treatment, the leg was directly manipulated by holding the patient down, while the TBS performed the manipulation without using analgesics. The traditional herbs were used in the mixture and blended into a cream that was applied to the lower leg right after the manipulation. Afterward, the splint was applied over the herbal cream, and a bandage was put around tightly to hold the splint in place.\nAfter 24 h, he started to feel intense pain, swollen and numbness throughout the right leg. He had a fever that developed in the following days with limb exuding foul-smelling discharge. Progressive darkening of the limb was noticed, and the leg was swollen. He was subsequently brought to the hospital and treated for five days. Before referred to our hospital, his vital signs were reported as blood pressure 90/60 mm Hg, pulse rate 110 beats/min, respiration rate 20 breaths/min, temperature 38.3 °C. Laboratory results in indicated severe anemia (hemoglobin was 4.6 g/dl) and severe infection (white blood cell count was 29.3×109/l). He was given intravenous fluid, analgesics, antipyretic, broad-spectrum antibiotics, and blood transfusion.\nPhysical examination at presentation showed he was alert with vital signs showing a blood pressure of 100/60 mm Hg, a pulse rate at 96 beats/min, respiration rate at 20 breaths/min, a temperature of 37.8 °C. The Visual Analogue Scale (VAS) was 7. The results of the general examination were within normal limits. Essential findings in the musculoskeletal system of the right leg were the loss of skin tissues from the knee to the foot. The bone and the soft tissues such as muscles and tendons were exposed, dirty, darkened, and dead, leaving a foul smell. The knee was held in a fully extended position. Palpation of the limb showed crepitation at the level of the femur, and the temperature of the gangrenous part was cold. Range of motion (ROM) examination showed there was no active movement below the knee joint. Normal pulse was palpated at the femoral artery, but there was no pulse at dorsalis pedis, posterior tibial, and popliteal artery. No sensation felt throughout the lower leg and no active movement below the knee joint. His laboratories at the time of admission showed: hemoglobin 8.6 g/dl, hematocrit 26.5%, erythrocyte 3.36×106/mm3, white blood cells 9700/μL (diff. Count of neutrophils 90.7%, lymphocyte 2.8%). All other laboratory values were within normal limits, shown in . Anteroposterior (AP) and lateral (L) view X-Ray of the upper leg showed displaced transverse fracture of the middle third of the femur. AP/L view of the lower leg showed an incomplete fracture of the middle third of the fibula, soft tissue defect at the proximal third and distal third of the tibia, and gas density around soft tissue suggestive of gas gangrene. AP/oblique view of the foot showed an oblique fracture of the 5th metatarsal, dislocation of the middle phalanx of the 2nd digit, subluxation of the distal phalanx of the 4th digit, and gas density around the soft tissue suggestive for gas gangrene. . Biopsy could not be performed due to financial reason.\nWith the above physical and imaging characteristics, a diagnosis of dead limb secondary to bone setter's treatment was made. The patient and his family were informed about the urgent need for amputation after stabilizing the patient's condition. He was given intravenous fluid, antibiotics (ceftriaxone, 1 g every 12 h; gentamycin 80 mg every 8 h), analgesics (ketorolac, 30 mg every 8 h), antipyretics (paracetamol, 1 g every 8 h IV drips), and blood transfusion one bag every 24 h until the hemoglobin reaches the minimum of 10 g/dl. He underwent above-knee amputation by an orthopedic surgeon in our center on the 3rd day of admission . This was considered as the limb was non-functional, the inconvenience of wound care, and the risk of becoming a focus of infection.\nPostoperative recovery was excellent, surgical drain and the urethral catheter were removed after 72 h, and on the 5th postoperative day, he was discharged with suggestions for routine wound control in the orthopedic outpatient department. He was given oral antibiotics (cefixime 200 mg every 12 h, metronidazole 500 mg every 8 h), analgesics (mefenamic acid 500 mg every 8 h), ferrous sulfate 200 mg every 12 h, and calcium lactate 500 mg every 24 h. One week follow-up of the patients showed satisfying wound control, and he was referred to rehabilitation for exercises such as; standing, balancing, and endurance exercise. After two weeks, he presented without any additional complaints and was grateful for his condition.
A 78-year-old male patient with coronary artery disease status post coronary stent placement was found to have a lung nodule on the chest radiograph at that time. The patient underwent a computed tomography (CT) scan, and bronchoscopy, and was found to have a 9 mm fatty endobronchial lesion in the bronchus intermedius above the middle lobe with 2 cm extraluminal fatty lesion into the right hilum. An endobronchial ultrasound with biopsy of the mass was performed, which showed benign bronchial epithelial cells. Since endobronchial resection of the mass would lead to a large defect in the right bronchus intermedius, the decision was made to perform robotic-assisted resection of the lesion (Video ).\nWe used the Da Vinci Xi robot to perform resection of the endobronchial lesion and hilar mass with right lower lobe superior segmentectomy to remove the lesion. The patient had a “five on a dice” port placement for the operation [, ]. First, we performed the right lower lobe superior segmentectomy to obtain adequate exposure of the hilar mass. We mobilized the superior segmental branch of pulmonary artery and superior segmental branch of the right lower lobe going to the inferior pulmonary vein and divided them with the vascular robot stapler. We divided the superior segmental branch of right lower lobe bronchus with the robot blue load stapler. We used indocyanine green angiography to define the borders of the superior segment of the right lower lobe, which was divided using the robot blue load stapler. This provided access to the hilar fatty tumor, which allowed for removal of the hilar mass and subsequent resection of endobronchial lesion with scissors. The frozen section on both lesions was negative for malignancy. We confirmed complete resection with intraoperative bronchoscopy that also showed a large opening in the airway.\nIn order to reconstruct the airway, we placed two 3-0 vicryl stay sutures at the proximal and distal ends of the airway and placed the suture through the posterior ports to pull the airway posteriorly away from the main pulmonary artery. We closed the opening with 4-0 PDS (polydioxanone) in an interrupted fashion eight times. This provided good closure of the opening. We performed a bronchoscopy that showed no abnormalities and the air leak test demonstrated no appreciable air leaks.\nThe patient went home on postoperative day 3 without any complications. The final pathology report was lipomatous hamartoma.