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A 17-year-old Motswana boy of the Tswana ethnic group with a primary school education was brought to our psychiatric hospital with a 5-year history of tobacco smoking and delinquent behaviors. He is the first in a family of three children; both his parents are alive but separated. He lives with his mother who is of low socioeconomic status; she has a busy schedule and works full time. There is no history of mental illness or substance abuse in the family. He was observed at the age of 11 years to exhibit conduct disorder behaviors such as disobedience, stealing, truancy, and hanging out with “street kids.” He was first introduced to tobacco smoking by his friends at age 12 years. He started with a Peter Stuyvesant (nicotine content of 1.3 mg/cigarette) brand of cigarette which was initially unpleasant; however, he continued with persuasion from his friends. He gradually stepped up his use from one cigarette/day over the next 2 to 3 years to approximately 20 to 30 cigarettes per day to sustain the relaxing and stimulating effect which improved his daily performance. He admitted to craving for this substance to the extent of doing dirty jobs for people to sustain the habit and neglecting other previous forms of enjoyment, such as watching television with family members. He has had several unsuccessful attempts at controlling the amount he took in a day despite the knowledge of its harmful consequences. His longest period of abstinence was 3 months in a rehabilitation center which was approximately 3.5 years ago. He had once experimented with cannabis and alcohol, but he never enjoyed these substances and so did not continue.\nTwo months prior to his index presentation at our hospital, he progressively neglected his personal hygiene and food, became emaciated, and spent more time cigarette smoking (that is, smoking continuously); he decided to seek medical attention at this time.\nThere were no psychotic symptoms on admission, but he was very restless, irritable, and had intense craving for this substance. In addition he complained of headache and insomnia.\nHe has had no previous treatment or admission for any psychological disorder and was never on any psychotropic medication before his index presentation at our hospital. A year after he started smoking cigarettes, his mother decided to seek spiritual help when he was observed to be smoking cigarettes at the expense of other activities, pilfering, and playing truant, but there was no significant improvement. His cigarette smoking subsequently became excessive over the next 6 months and he consequently started neglecting his personal hygiene, withdrawing from family activities, and preferring to smoke cigarettes rather than eat; thus, he was becoming emaciated. As a result, his mother was advised to try a rehabilitation center. He spent 3 months in a rehabilitation center 3 years prior to his presenting at our hospital. He went through drug education, counselling, and was abstinent for only this period. He had neither psychotic nor mood symptoms before or during the period of rehabilitation, and did not experience any abnormal movement. He only complained of restlessness and tension; nevertheless, he was not placed on any medication other than multivitamins. While he was in the rehabilitation center, he was completely abstinent and his appetite and weight improved considerably. On leaving the rehabilitation center, he attended follow-up only once before he defaulted. Afterwards, he went back to smoking cigarettes and has had no period of abstinence until his index admission to our mental health facility.\nBefore he started smoking cigarettes, he was described as an easy child, quite cheerful, and an outgoing person who enjoyed the company of other children.\nA mental state examination at his index admission to our hospital revealed agitation, but there was no abnormality of speech, thought, or perception. He described his mood as fine, but objectively it was anxious.\nA physical examination revealed no significant abnormality. His blood pressure was 110/70 mmHg, pulse rate was 90 beats/minute, and his temperature was 37 °C. Investigations such as full blood count, liver function test, thyroid function test, as well as computed tomography (CT) scanning of his brain revealed no significant abnormality. Urine drug screening was negative for substances which included cannabis, cocaine, and phencyclidine, except for benzodiazepines, which was given to reduce restlessness and to improve sleep.\nThe working diagnosis made was mental and behavioral disorder due to psychoactive substance use; nicotine dependence with comorbid conduct disorder.\nThree days postadmission, he was observed to be having some abnormal involuntary movement such as occasional chewing movements, trunk twisting, and truncal tremor. During an interview he tried to conceal the involuntary movements of his hands by holding his chair with a firm grip. According to the nurses’ reports, these movements often disappeared during sleep and briefly whenever his attention was called to them. He admitted to the fact that he first experienced these movements approximately 2 years ago and has also observed their disappearance whenever he smokes. This claim was supported following some relief which he experienced with nicotine gum (Nicorette); an offer which he previously refused.\nHe was on admission for 4 weeks with scheduled sessions with a psychologist on drug counselling and education. In addition he was placed on diazepam 10 mg on the first night and twice daily for 5 days in addition to nicotine gum which was made available to him on demand.\nHis level of hygiene as well as his appetite improved approximately 2 weeks after admission in response to therapy. In addition, his abnormal movements reduced after 3 weeks on nicotine gum after which he was discharged home with the gum. He was to continue with monthly psychological sessions on an out-patient basis since there was no formal rehabilitation program in the facility. He was seen only once on follow-up during which it was noted that he did well on nicotine gum without any adverse effect. His appetite, level of hygiene, and weight were also well maintained but he then defaulted. |
The patient is a 21-year-old African American man with a past psychiatric history of schizophrenia who was transferred from the psychiatry unit to the medical floor to rule out sepsis after the development of fever and tachycardia. The patient was reported to have decompensated on account of nonadherence with his home medications resulting in frequent hospitalizations and poor functioning. As per the patient's admission records, the patient's initial labs and urine toxicology were within normal limits. The patient had been on various antipsychotics since his diagnosis with schizophrenia at the age of 19, including haloperidol, olanzapine, and risperidone, but he continued to decompensate despite adequate medication trials. The decision was made to start the patient on clozapine due to possible failure of these previous antipsychotics. Clozapine was started at 25 mg PO twice daily and titrated up to 150 mg PO twice daily over the next 12 days. His current daily dose was 300 mg when he was transferred to the telemetry unit following a sudden development of fever and tachycardia on the psychiatric inpatient floor.\nOn the medical inpatient unit, the patient was mostly selectively mute and did not appear to be in any pain, was not vomiting, and had no diarrhea and no reported loss of consciousness or seizures. His admission vitals were a temperature of 102.4 degrees Fahrenheit, BP of 115/81 mmHg, HR of 114 beats per minute, and an oxygen saturation of 97% on room air. Physical examination did not reveal any significant findings other than a mild dehydration, no skin rash, no jugular venous distension, basal crepitation or pedal edema. Cardiac auscultative findings were largely normal except for a tachycardic heart rate; apex beat was in the 5th intercostal space, midclavicular line. The patient's heart sounds, S1 and S2, were normal, no rubs, or murmurs, and no obvious gallop rhythm. His peripheral extremities were warm with a normal capillary refill.\nThe patient was given an intravenous bolus of normal saline and a stat dose of intravenous antibiotics (vancomycin and meropenem), and full workup including 2 sets of blood cultures, urine analysis, and a chest radiograph was ordered. His complete blood count (CBC) revealed a normal leukocyte count of 7000/µl with elevated eosinophils of 500 (reference 0.0–400, see for trend) and normal platelet count. He had an elevated erythrocyte sedimentation rate (ESR) of 26 (reference 0–15 mm/Hour) and CRP was 147.4 mg/dl (reference 0.0–4.9 mg/dl).\nIn view of the patient's current clozapine use, a presumptive diagnosis of myocarditis was made and troponin I was requested which turns out to be markedly increased, 1.12 ng/dl (reference: 0.00–0.05 ng/dl). His NT-pro B-type natriuretic peptide (BNP), lactic acid, and creatine kinase levels were normal. His electrocardiogram (EKG) showed sinus tachycardia without specific ST-T changes ( below). His chest radiograph was unremarkable. Urinalysis and urine toxicology were negative. Initial transthoracic echocardiogram (TTE) revealed a left ventricular systolic dysfunction with apical hypokinesis (ejection fraction 45%) and mild tricuspid regurgitation. Coronary angiogram showed patent coronaries; ventilation/perfusion scan resulted as low probability for pulmonary embolism.\nClozapine was discontinued on admission to the medical unit. Subsequently, the patient's fever and tachycardia resolved. Troponin I trended down (lowest being 0.22 ng/ml). Eosinophil count initially increased but later normalized as shown in . Repeat transthoracic echo after one week of discontinuing clozapine revealed normal left ventricular systolic function with mild apical hypokinesis (ejection fraction of 60–65%). The patient was clinically stable throughout the hospital course. The psychiatry consult liaison team started the patient on aripiprazole which was well tolerated by the patient without any side effects. The patient later underwent an uneventful discharge to the community outpatient clinic. |
An obese 33-year-old male patient with no significant past medical history presented to the emergency room (ER) complaining of left-leg pain after a recent COVID-19 infection. He had tested positive nearly three weeks earlier and had remained asymptomatic, not requiring hospitalization. Repeat testing on admission via antigen and polymerase chain reaction (PCR) was negative. He developed acute onset severe pain and swelling in the left leg and foot nearly one week before presentation, which progressed to numbness. He did not seek medical attention previously until the current presentation when his pain became unbearable. Five days before arriving at the ER, he also had a motor loss of the toes and ankle. The patient denied any coughing, had no shortness of breath or chest pain. The patient was afebrile and vital signs were stable on presentation. On physical exam, the patient had positive Homan’s sign and palpable cord of the left lower extremity with minimal swelling. The right and left dorsalis pedis (DP) and posterior tibial (PT) pulses were palpable. The popliteal pulses were palpable on the right side and noted to be monophasic on the left. The femoral pulses were palpable bilaterally. The left foot was noted to be cool in temperature with diminished sensation at the level of the ankle. The patient also had a foot drop, was unable to flex the ankle, minimal toe flexion/extension, and early mottling of the skin was noted. The rest of the physical exam was within normal limits.\nUltrasound of the left lower extremity showed evidence of acute deep venous thrombosis in the popliteal (partial) and gastrocnemius (nearly occlusive) veins. Subcutaneous edema and rouleaux flow were seen throughout the extremity. Nearly occlusive arterial thromboses were also discovered throughout the distal femoral, popliteal, posterior tibial, anterior tibial, and peroneal arteries with very low flow velocities to absent flow overall (Figure ). More proximally, triphasic waveforms were observed with moderately reduced velocities through the common femoral, deep femoral, proximal, and mid-femoral arteries. Heparin infusion was immediately started. Vascular surgery was consulted, and the patient was taken to the operating room for an open thrombectomy of the superficial femoral artery, popliteal, anterior tibial, posterior tibial, and peroneal arteries under general anesthesia. Heparin infusion was maintained throughout the procedure and the patient was also heparinized using 100 U/kg heparin which circulated for three minutes before the activated clotting time (ACT) was measured. The ACT was maintained between 250-300 throughout the procedure. Despite appropriate anticoagulation, he had recurrent thromboses. The posterior tibial artery lost signal within a few minutes of closing and was reoccluded. These tibial vessels were subsequently reopened, and he underwent repeat thrombectomy. After the re-thrombectomy, the patient developed signs and symptoms of impending respiratory failure with oxygen saturations dropping down to the low 70s despite a 100% fraction of inspired oxygen (FiO2) and tachycardia. As such, the patient was intubated. The posterior tibial artery was reoccluded, but there was a signal in the dorsalis pedis. However, because the patient was deemed to be in danger of significant decompensation, a third thrombectomy was not attempted.\nPost-operatively, the patient developed worsening respiratory failure, increased work of breathing, and apparent ST elevation on the monitor. EKG confirmed an inferior ST segment elevation myocardial infarction (STEMI) with reciprocal anterior ST depression (Figure ). Unfortunately, the doppler signal of the anterior tibial artery was also lost around the same time. He was taken emergently to the cardiac catheterization lab and was deemed unstable for return to the OR for another attempt at tibial thrombectomy.\nIn the cardiac catheterization lab, he was found to have triple vessel disease; percutaneous coronary intervention was attempted but was unsuccessful. The ejection fraction based on the left ventriculogram was 20%. He experienced atrial flutter during the procedure requiring chemical cardioversion with amiodarone. He was noted to have a possible left apical and right mural thrombus both of which were confirmed on a subsequent echocardiogram. His left foot remained cold and pulseless. Patient remained on a heparin gutta (gtt) (heparin infusion 25,000 units in 500 mL 0.45% NaCl continuous at 0-30 units/kg/hr, titrated per protocol while monitoring activated partial thromboplastin time [aPTT]). Clopidogrel (Plavix) 300 mg per os (PO) was administered once after the patient sustained a STEMI and then continued at 75 mg PO daily thereafter. The patient demonstrated signs of heparin resistance as he could not reach goal aPTT despite maximal heparin gtt with a mean activated partial thromboplastin time of 55.7 seconds and an average anti-Xa assay of 0.24 IU/mL. Argatroban infusion was initiated at 2 mcg/kg/min and aPTT level within 24 hours of initiating argatroban was 114.8 seconds, achieving the therapeutic range indicated for anticoagulation. His left foot was not deemed salvageable and given his poor overall condition, ongoing hypercoagulability, and mortality risk, a guillotine amputation of the left foot just proximal to the ankle was performed. No arterial inflow was present. An acute clot was extracted from the posterior tibial artery with the restoration of pulsatile inflow. The clot was also extracted from the anterior tibial artery with the return of weak inflow.\nFour days following the STEMI, the patient developed pulseless ventricular tachycardia and was successfully resuscitated and started on amiodarone. While he remained on mechanical ventilation, he required intermittent pressor therapy for undifferentiated shock. Three days later, the patient was successfully extubated and transferred out of the ICU and began to work with physical therapy. One week later, the patient developed recurrent acute hypoxemic respiratory failure, requiring re-intubation, and he was transferred back to the ICU. Anticoagulation with warfarin was initiated after bridging with fondaparinux. Three days later, the patient was extubated and then three days thereafter, he again went into acute respiratory distress, requiring BiPAP. Despite attempts at non-invasive ventilation using BiPAP, he became somnolent and required intubation, and transition to full mechanical ventilatory support was altered and he was intubated. During the peri-intubation period, the patient lost pulse and went into pulseless electrical activity and advanced cardiac life support (ACLS) protocol was initiated. Thirty minutes of ACLS was completed and a cardiac ultrasound thereafter showed no cardiac movement with the presence of a ventricular thrombus. The patient was pronounced dead.\nHematology-oncology was consulted during the hospitalization to investigate potential underlying etiologies of the patient’s hypercoagulability, given his young age and unusual clotting symptoms. Further lab workup to investigate causes of hypercoagulopathy included antiphospholipid antibodies, anticardiolipin antibodies, beta 2 microglobulin antibody, lupus anticoagulant, and factor II DNA analysis that all resulted negative. Flow cytometry testing for paroxysmal nocturnal hemoglobinuria was also collected and the results were negative. Janus Kinase 2 (JAK2) kinase mutation looking for evidence of polycythemia vera or other myeloproliferative disorders was also negative. Other hypercoagulable syndromes more related to venous clotting, specifically with factor V Leiden, prothrombin gene mutation, protein C deficiency, protein S deficiency, and antithrombin III deficiency were worked up (although it was expected that the antithrombin III level was not reliable, as the patient remained on heparin in the setting of acute clotting) and no test came back positive. Screening for underlying connective tissue disorders/rheumatologic disorders was conducted inclusive of the antinuclear antibody (ANA), rheumatoid factor, and serum protein electrophoresis (SPEP) with quantitative immunoglobulins. This extensive workup for hypercoagulability, while the patient was hospitalized, was non-diagnostic, suggesting COVID-19 syndrome of hypercoagulability as the most probable etiology in the post-acute infection setting.\nThe patient also notably had elevated blood glucose levels during his hospitalization ranging in the 200s. An A1C was then obtained and resulted to be 12.0%. Though the patient had not been formally diagnosed with diabetes, his hemoglobin A1c (HbA1C) and elevated blood glucose readings in the hospital confirmed the diagnosis of type 2 diabetes. His blood glucose levels were controlled in the inpatient setting with sliding scale insulin. |
A 44-year-old African American man presented at an outside hospital with history of neck pain and left arm pain in a nondermatomal distribution associated with left hand numbness/tingling and left-sided weakness for a couple of months. Examination revealed presence of subtle left sided hemiparesis (4+/5 on Medical Research Council (MRC) grade) and considering a diagnosis of cervical spondylosis, a plain MRI of cervical spine was ordered which revealed presence of moderate cervical stenosis associated with T2 signal changes with no significant focal compression and he was treated conservatively. He however had progression of symptoms over the next few weeks due to which he was referred to our institution. Examination revealed presence of worsening weakness involving the left side (3/5) with brisk reflexes in bilateral upper and lower extremities. Sensory exam showed no gross dermatomal sensory deficits with presence of Hoffman's reflex. These neurologic findings prompted us to repeat an MRI with contrast enhancement due to presence of signal changes on the MRI without significant compression and disconcordance between clinical examination and imaging findings. MRI revealed presence of an approximately 1.5 cm patchy enhancement involving the left half of the cervical spinal cord at C4 level with presence of T2 signal changes spanning three to four segments in the cervical spine along with presence of moderate to severe spinal canal stenosis [Figures and ]. In light of the clinical symptoms and neuroimaging abnormalities, the diagnosis of spondylotic compressive myelopathy was questioned and a differential diagnosis of a neoplasm (astrocytoma/ependymoma), inflammatory, ischemic, and demyelinating lesions was entertained. Initial laboratory workup including lumbar puncture revealed that the patient had a mildly elevated protein level (43.5 mg/dl), a normal immunoglobulin (Ig)G/albumin CSF ratio (0.2), negative oligoclonal bands, and no malignant cells on CSF cytology studies. His CSF also demonstrated 110 red blood cells and 15 white blood cells per ml with 90% lymphocytes. Quantiferon gold tuberculosis (TB) test, aerobic, anaerobic, fungal, and TB cultures were all negative making infection an unlikely diagnosis. Erythrocyte sedimentation rate and C-reactive protein were slightly elevated with values being 19 (N: 0-17 mm/h) and 17 (N: 0.0-8 mg/dl). He was started on pulse dose of steroids with a likely diagnosis of myelitis and his clinical examination improved with improvement in motor strength to 4+/5 and he could ambulate better. However, while the steroids were being tapered, he had recurrence of symptoms with worsening weakness (3/5). The steroids were restarted, but because it was not possible to make a definitive diagnosis based on laboratory studies, in the face of neurological worsening on tapering steroids, despite the risks of an intramedullary biopsy, this was thought to be the best option for diagnosis. A C3 through C6 laminoplasty and excisional biopsy of the C4 intramedullary lesion was performed to address the cervical stenosis and biopsy the intramedullary lesion []. Intraoperatively, the cord appeared normal except that it was quite swollen laterally on the left-hand side at C4 level and a dorsal root entry zone myelotomy was performed and abnormal tissue was immediately encountered which was quite distinct from the normal spinal cord tissue. This was sent for frozen section, which revealed gliosis versus neoplasm. Given the fact that this could be neoplastic tissue, further dissection was then carried out and a dissectible plane was found separate from normal spinal cord and a gross total resection could be achieved. The patient initially had worsening of his strength on left side and became essentially hemiplegic, but started improving gradually. Final pathology demonstrated non-necrotizing granulomatous disease consistent with sarcoidosis []. After the diagnosis was confirmed, an MRI of the brain [] and CT of the chest abdomen and pelvis were done to rule out any other site of involvement which demonstrated slightly, but nonsignificant mediastinal and retroperitoneal lymph nodes. Serum and CSF angiotensin converting enzyme (ACE) levels performed were within normal limits. He was subsequently treated with pulse dose of intravenous (IV) methylprednisolone, methotrexate, and induction infliximab (chimeric monoclonal antibody against tumor necrosis factor (TNF)-α) therapy and was discharged home with significant improvement in his motor strength. He remained in clinical follow-up with rheumatologist and neurologist and was treated on an outpatient basis with prednisolone 60 mg/day for 6 months along with methotrexate and infliximab. At his last follow-up at 9 months, he was on tapering dose of prednisone with continued weekly oral methotrexate and eight weekly maintenance infliximab therapy. He maintained neurological improvement in his motor strength except residual left ankle dorsiflexion weakness required an ankle foot orthosis and could ambulate independently with occasional use of walker for stability. |
A 58-year-old man presented with productive cough and fever. His medical history was significant for hepatitis C and a maternal family history of colon cancer. The patient had no past history of cancer or surgery. He was an ex-smoker (40 pack/year), and worked as a screenwriter and photographer. He had no history of asbestos exposure. He was initially treated with antibiotics, and his symptoms were resolved. However, due to persistent abnormal chest X-ray findings, a CT scan of the chest was carried out revealing a 5 cm × 4 cm paraspinal mass in the upper right chest, which was also intensely hypermetabolic on a corresponding positron emission tomography scan without evidence of lymph node metastasis. No pleural effusion was detected []. A radiologic differential diagnosis included a posterior mediastinal neurogenic tumor and a metastatic carcinoma. Because the mass was located adjacent to the esophagus, an esophagogastroscopy was arranged to rule out esophageal cancer, and this was normal. A magnetic resonance imaging of the brain and a bone scan were negative. Laboratory studies were all within the normal range. The mass was aspirated under CT guidance using coaxial technique and a 22-gauge needle. Air-dried and alcohol fixed smears were stained with Romanowsky and Papanicolaou method. A cell block was prepared from sample rinsed in saline, using the histogel method. Rapid on-site assessment provided by a cytopathologist was recorded as an adequate sample showing an epithelioid neoplasm.\nThe smears showed a hypercellular specimen consisting of loosely cohesive “lobules” of heavily vacuolated epithelioid cells displayed against a background of myxoid material, which was highlighted on Field's and Giemsa stained direct smears, suggesting the possibility of chordoma []. The vacuoles in the cells were filled with the same myxoid material seen in the background [] and were negative for mucicarmine stain. Occasional cells with intracytoplasmic vacuoles displacing their nuclei to the periphery resembling signet-ring cells were also seen within the lobules, expanding the differential diagnosis to include the possibility of adenocarcinoma []. Individual microcysts were fused, resulting in secondary cystic dilatation. The epithelioid cells showed moderate to marked nuclear pleomorphism out of keeping with chordoma, hyperchromatic nuclei, prominent nucleoli, a dense chromatin pattern, and abundant cytoplasm. Mitotic activity was easily identified []. There was no evidence of necrosis. The working differential diagnoses included chordoma, benign adenomatoid tumor, epithelioid hemangioendothelioma, adenocarcinoma, and epithelioid MM.\nImmunohistochemical studies performed on cell block sections showed tumor cells were strongly immunoreactive for calretinin, WT-1, D2-40, cytokeratin (CK) 7, and AE1/AE3; and moderately positive for high molecular weight keratin (CK5/6), vimentin, and epithelial membrane antigen, which supported a mesothelial origin. Negative stains included thyroid transcription factor-1, Ber-EP4, carcinoembryonic antigen, S100 protein, CK20, and CDX-2, which excluded adenocarcinoma and chordoma and further supported the diagnosis of mesothelioma []. In the context of radiologic findings, a diagnosis of localized MM, microcystic (adenomatoid) variant, was made. The patient subsequently underwent right pneumonectomy. Examination of the lung found a localized, pleural-based 4.8 cm tumor located adjacent to the right upper lobe. The tumor involved parietal and visceral pleura, and focally invaded the underlying lung parenchyma and overlying chest wall soft tissue, confirming the diagnosis of MM. The patient remains disease free 29 months after the pneumonectomy. |
A 72-year-old female patient was admitted to our outpatient department complaining of back pain associated with severe neuropathic radicular pain to her both lower extremities, incomplete paraplegia at the levels of L5 and S1 and low back fistula with serous secretion since several weeks. The patient had extensive surgical and medical history in another hospital and brought us all the medical reports from her previous admissions.\nNine years before her admission to our department, she had undergone several anterior and posterior lumbar spine surgeries for L3-L5 spinal stenosis and neurologic claudication. The first operation had occurred at the age of 63 years. It was an anterior decompression and interbody fusion L3-L4, via a left-sided XLIF approach with neuromonitoring. While placing the intervertebral cage at the segment L4/L5, there was a pool of blood coming from the surgical site and the patient soon became hemodynamically unstable. The vascular surgeon on-site applied a hemostatic agent immediately along with packing and the decision to abort the posterior planned stabilization was made. Emergent angiography was performed, as the suspicion for major vascular injury arose; it showed laceration of the terminal aorta along with large expanding hematoma pressing on the lower abdominal aorta and the right common iliac injury. Following angiography, an intravascular mesh stent was successfully inserted by the interventional radiologist on site (Figure ). The patient became hemodynamically stable and repeat angiography showed no blood escape from the aorta laceration site. Immediately postoperatively, the patient complained of decreased motor strength in all muscles below the knee (L5, S1) bilaterally and severe neuropathic pain. The patient remained stable and the decision to proceed to the aborted XLIF was made. Two months after the initial L4/L5 XLIF, the surgeon proceeded to the posterior percutaneous MIS stabilization.\nThe subsequently performed CT and MRI of the thoracolumbar spine disclosed a significant spinal stenosis with myelopathy signs at the level T11-T12, so that the previous surgeons advised a wide decompression in the lower thoracic spine since it was considered a main source of the persistent lower extremities pain and neurologic deficit (Figure ).\nTherefore, a wide posterior decompression, including laminectomy and facetectomy was performed by the same surgeons at the level of T11-L3 with posterior pedicle screw fixation and fusion from T10-S1 levels (Figures -).\nOn admission to our outpatient clinic, the patient was mobilized using a wheelchair and claimed severe pain in the lower extremities. She had been in pain-relief protocol with pethidine and morphine in a private pain clinic by an experienced anaesthetist with only temporary relief. Physical examination revealed a patient with marked muscular atrophy in both lower extremities and flexion contracture in her right knee of 30 degrees associated with severe osteoarthritis. There were two draining sinuses emerging from the back over the old posterior midline surgical scar. The lateral (XLIF) scar in the left side was without signs of infection. Neurovascular examination revealed motor deficit in the lower extremities as follows: iliopsoas bilaterally 3/5; quadriceps bilaterally 3/5; foot dorsal extensors and flexors bilaterally 1/5 and 2/5 respectively. Furthermore, sensation from L1 to S2 was decreased, worse at the levels of L4-S1 bilaterally. Therefore, only minor improvement was seen compared to the situation after the multiple operations in the first institution.\nWound cultures from the sinuses were collected on admission that disclosed E. coli and intravenous antibiotics were started. A CT showed completed fusion in all instrumented segments (Figures -), but also revealed remarkable abscess formation underneath the lumbar fascia (Figure ).\nDuring her admission in our department, she underwent a posterior revision surgery from T10 to S1 including removal of the fistulae that emerged from underneath the deep lumbar fascia. Pus was draining from the subfascial area and was drained and debrided meticulously. No findings of meningocele or pseudomenigocele were disclosed elsewhere. Complete removal of the posterior spinal implants (screws, rods etc.) was performed since the fusion was completed and a chronic deep infection persisted (Figure ).\nTissue samples taken from the posterior lumbar surgical site were cultured. Tissue culture grew E. coli and Pseudomonas strains. The antibiotic scheme was adjusted and continued for a total of four weeks until the patient was discharged; oral antibiotics followed for additional two months after discharge. She remained stable during her hospitalization, repeat blood cultures were negative and the patient was finally discharged to a rehabilitation facility.\nIn the first six months following this surgery, the patient reported slow but gradual improvement of her lower extremities neuropathic pain. Thirty months following this last surgery the patient was admitted again to our outpatient clinic. She was almost pain-free and was mobilizing with leg braces for the lower extremities. The ESR was 12 mm/1st hour and CRP was 0.5, within normal limits (<0.5). |
A 54-year-old man came to the private dental clinic with complaint of difficulty in mastication and esthetical concern for his upper anterior teeth. He was a nonsmoker and was diagnosed with IgG-kappa type MM in November 2011. In the physical examination, he was diagnosed with MM. Bony metastasis was present at the time of diagnosis of the disease. A full radiographic skeletal survey showed multiple bony lesions at the ribs, femurs, and hip (Figures and ).\nPanoramic view revealed bony lytic and punch out lesions at the right side of the mandible. This patient had no history of surgery. His weight had decreased by 7 kg, following 22 months of acute intravenous injection (IV) BP treatment after the last chemotherapy treatment session. His blood pressure was 130/80, and he had a normal breathing and pulse rate. Preoperative examination of his oral mucosa revealed no evidence of pathological lesions, and overall oral hygiene was good. The patient was felt healthy and was well nourished, alert, and cooperative. After thorough clinical examination, maxillary right first premolar was found missing.\nAfter meticulous consulting sessions with the patient and discussing the advantages and disadvantages of all treatment options, he accepted to receive dental implant.\nAccording to the patient's physician, the appropriate time for the surgery relied upon the patient's regular blood cell counts. This patient did not undergo any radiotherapy phases in the entire duration of his active IV BP treatment. He underwent chemotherapy for two separate sessions. After the last session of chemotherapy, the patient received monthly infusion of 3.5 mg of the IV BP drug zoledronate (Zometa; Novartis Pharmaceuticals Corporation) for a period of 22 months (from May 2014 to March 2016). As per the physician's recommendation, C-terminal cross-linking telopeptide (CTX) examination was carried out 6 months after stopping IV BP therapy. The CTX above of more than 150 was considered to be safe, in that the CTX was 289 pg/mL.\nBefore surgery, the patient was premeditated with 2 g of amoxicillin/clavulanic acid and 50 mg of diclofenac. A root form titanium dental implant (Superline; Dentium) of 3.6 mm in diameter and 10 mm in length was inserted under local anesthesia (Figure ). The patient well-tolerated the procedure and his vital signs were regularly monitored. Postoperative medications including antibiotics (1000 mg amoxicillin/clavulanic acid twice daily for 7 days, starting on the day of surgery), an analgesic (600 mg ibuprofen as required every 6 hours), and mouthwash (0.2% chlorhexidine twice daily for 2 weeks, starting on the day after surgery) were prescribed to the patient. Postoperative course and healing were unremarkable and typical. He was instructed to resume normal oral hygiene and chewing by week six. Postsurgical cleaning protocols, including oral hygiene instructions, were implemented at weeks 1, 2, 6, and 12.\nFour months after the implant insertion, the patient returned for punch removal of the gingiva overlying the implants. After 1 week, the appropriate impression copings were connected to the fixture. Polyether (Permadyne light and regular body; ESPE, Plymouth Meeting) was injected around the transfer copings and placed inside the custom tray using the dispenser. After laboratory procedure, abutment were positioned and torqued according to the manufacture's guidelines at 30 Ncm. After the surgical and prosthetic treatments were completed on February 2017, the patient was placed on a regular follow-up for peri-implant maintenance. The patient resumed IV BP therapy on May 2017. The oral hygiene regimen was implemented for this patient in a 6-month recall. The last follow-up (12 months after prosthetic delivery) showed minimum bone loss, as compared with the X-rays taken immediately after the prosthetic delivery and the implant, and its restoration was successful. The patient was satisfied with the treatment (Figure ). |
A 43-year-old man presented with a progressive deterioration of visual function for the previous seven years. The patient had no other ocular symptoms such as nystagmus or photophobia. His past history showed stable vision of 20 / 40 since trauma to his right eye when he was approximately 14 years of age. No other systemic abnormalities or malformations were recorded. His best-corrected vision was 20 / 400 in the right eye and 20 / 20 in the left, and his intraocular pressures were 25 mmHg in the right eye and 23 mmHg in the left eye at the time of his initial visit. Under slit lamp examination, a diffuse haze composed of a flaky pattern of stroma was noted throughout the entire cornea. The right eye had decreased vision and exhibited relatively denser homogenous opacities than the left ().\nThe family members stated that corneal changes had been detected only in the patient's mother at 69 years of age, and no specific issues had arisen in any other family member or relative. The patient's father had reported no ophthalmic abnormalities before his death, and his mother had been diagnosed with diffuse corneal opacities of unknown etiology in both eyes three years previously (). She explained that she had experienced decreased vision since childhood, but these deficiencies produced no difficulties in her daily life. The patient's brother and sister had no symptoms at all and no ophthalmic or systemic abnormalities. As far as the family knew, no one in the paternal or maternal lineage or offspring of the patient had experienced any eye problems except for the patient's mother ().\nThe endothelium and Descemet's membrane of the right eye were identified as normal following slit lamp examination. No gross abnormalities, such as Haab's striae or features of posterior polymorphous corneal dystrophy, were detected in the right eye. The patient's past medical records from another hospital demonstrated that his endothelial cells of both eyes presented with a normal shape and numbers under a specular microscope about six years ago. However, endothelial cells were found as indeterminate forms using specular microscopy due to the barrier of stromal opacity at the time of our study. The endothelial cells of the left eye were counted using a Konan Noncon Robo-8400 noncontact specular microscope (Konan Medical Inc., Hyogo, Japan) as 2564 cells/m2. We assumed that the right eye would have a similar amount of endothelial cells and a relatively uniform morphologic pattern as those of the left.\nUltrasound corneal pachymetry (Humphrey Instruments Inc., San Leandro, CA, USA) revealed a central corneal thickness of 658 µm in the right eye and 632 µm in the left. The patient was suspicious for CHSD based upon clinical evidence, and he was scheduled for penetrating keratoplasty of the right eye. A corneal button was sent for light and electron microscopic analysis. There was no problem with corneal wound healing after keratoplasty, and the grafted cornea restored its transparency within two weeks. After 12 months, the corneal graft remained clear, and the patient's best-corrected visual acuity was 20 / 50 in the right eye.\nLight microscopy with hemotoxylin and eosin staining revealed a normal epithelium and uninterrupted Bowman's membrane. The stromal lamellae were separated slightly from one another, forming a relatively compact space in between the anterior and the posterior stroma (). No Descemet's membrane or endothelium was detected in the original corneal button, apparently as the result of inappropriate specimen handling. Any infiltration, vessels, inflammatory, or storage material could not be detected.\nElectron microscopy revealed a criss-crossing pattern of corneal collagen fibers with a relatively electron dense and lucent structure and collagen fibers irregular in shape and size (). Keratocytes extended widely through the zone of low filaments ().\nBlood was sampled from the patient and family members for DNA collection and analysis []. DNA sequencing analysis of the decorin gene in chromosome 12q22 was positive in both the patient and his mother. The novel mutation of a heterozygous, nucleoside substitution (c.1036T>G) point mutation in the decorin gene was detected in both patients (). Lumican and keratocan sequence variants, which are closely located within the decorin gene, did not reveal any mutations. The c.1036T>G mutation resulted in a change of amino acid sequence (p.Cys346Gly). However, no genetic mutations were detected in other family members. |
An 81-year-old Japanese woman with a 2-week history of abdominal distension presented to our hospital for assessment. The patient did not have a past history of malignancy, with only a cesarean section as a relevant feature in her history. Endoscopic examination at a previous hospital revealed the presence of early carcinomas in the stomach and distal esophagus. The patient was referred to our hospital for endoscopic resection.\nLaboratory data, as well as serum carcinoembryonic antigen, squamous cell carcinoma antigen, and cytokeratin-19 fragment levels, were close to normal limits. Endoscopic examination revealed mild granular elevated lesions, with slightly depressed irregular mucosa, extending from the anterior wall to the right wall of the distal esophagus (Fig. ). This irregular mucosa further extended from the anterior wall to the left wall, with the boundary on the oral side being unclear (Fig. ). A superficial elevated tumor-like lesion was also observed in the lower body of the stomach, with a diameter of about 10 mm (Fig. ). Based on the endoscopic biopsy specimen, this gastric lesion was diagnosed as a well-differentiated tubular adenocarcinoma. On the other hand, the preoperative biopsy specimens of the esophageal tumor showed intraepithelial tumor cells, which were isolated or in clusters, and consisted of large clear cells with atypical nuclei and prominent nucleoli. No glandular structures and no obvious intracytoplasmic mucin were observed. These histological findings were consistent with a malignant melanoma, with a pagetoid spread of invasive adenocarcinoma or squamous cell carcinoma, and Paget’s disease as a differential diagnosis. Immunohistochemically, the tumor cells diffusely stained positive for CK7 and partially for CK20, with negative staining for S100 protein and HMB-47. On the basis of these results, a diagnosis of malignant melanoma was excluded. All human mucin core proteins examined (MUC2, MUC5AC, and HIK1083) were also negative. Furthermore, p53 overexpression was observed in all tumor cells. From these results, we diagnosed the tumor as Paget’s disease or a pagetoid spread of an esophageal carcinoma. On enhanced computed tomography (CT) and [18F]-fluoro-deoxy-glucose positron emission tomography (FDG-PET)/CT imaging, no lymph node and distant metastases were identified (Fig. ). FDG uptake was observed only in the lower body of the stomach, with these lesions considered to reflect past endoscopic submucosal dissection (ESD) for early gastric cancer (Fig. ). Although we could not define the margin of the tumor, previous reports of esophageal Paget’s disease indicated a wide extension of Paget cells in the esophageal mucosa. On the basis of these findings, we planned ESD for the treatment of the gastric lesion, followed by a thoracoscopic esophagectomy (TE) and hand-assisted laparoscopic proximal gastrectomy (HALPG) for the treatment of esophagogastric Paget’s disease. Histological examination of the ESD specimen revealed a well-differentiated mucosal adenocarcinoma (11 mm × 8 mm) without lymphovascular involvement. The lateral and vertical margins of the resected tissue were free of tumor cells, and ESD was considered as a curative resection.\nTE and HALPG, with lymph node dissection, were performed at 43 days after the gastric ESD. Regional lymph nodes were dissected, with no metastatic invasion identified in the thoracic and abdominal lymph nodes. Reconstruction with a gastric tube was performed after esophagectomy, using a hand-assisted laparoscopy procedure via a post-sternal route.\nHistological examination of the surgically resected specimen was performed. Macroscopically, the mucosa of the lower thoracic and abdominal esophagus was slightly irregular and depressed, with submucosal capillary hyperplasia (Fig. ). No tumor mass or ulceration was observable in the resected material. With iodine staining, the mucosa of the lower esophagus, which was congruous with the irregular and depressed area, did not stain. Furthermore, isolated small iodine-stained foci were observed in the gastric mucosa adjacent to esophagogastric junction (Fig. ). Microscopically, these foci consisted of squamous metaplasia of the gastric mucosa. The sectioned tissues were stained with hematoxylin and eosin (HE) and periodic acid-Schiff (PAS)/Alcian blue. As well, immunohistochemical staining for CK5, CK7, CK20, CDX2, MUC2, MUC5AC, HIK1083, p53, p63, S100, and HMB-45 was performed. Microscopic examination revealed neoplastic cells, with a large atypical nucleus and pale-staining cytoplasm, in the lower part of the esophageal epithelium, occurring either singly or in clusters (Fig. ). Reserve cell hyperplasia (Fig. ) and squamous metaplasia (Fig. ) were observed in the gastric mucosa, adjacent to the esophagogastric junction, and an intraepithelial squamous cell carcinoma (SCC) was observed within the squamous metaplasia (Fig. ). Components of the intraepithelial squamous cell carcinoma were identified following the Paget cells in the esophageal squamous epithelium. Only a few Paget cells stained positively for PAS/Alcian blue. Immunohistochemically, negative staining for CK5 (Fig. ) and p63 was identified in Paget cells, with positive staining for CK7 (Fig. ). The Paget cells showed no reactivity for intestinal mucin (MUC2) and gastric foveolar mucin (MUC5AC), but a few Paget cells were positive for gastric gland mucin (HIK1083). On the other hand, the intraepithelial SCC showed positive reactivity for CK5 and p63, but no reactivity for CK7 and CK20. Overexpression of p53 was observed in both Paget cells (Fig. ) and the intraepithelial SCC. Histochemical and immunohistochemical results are summarized in Table , and schematic representation of the distribution of Paget cells and squamous cell carcinoma of the esophagogastric junction is shown in Fig. . Because there were any findings of Barrett’s esophagus neither endoscopically nor pathologically, macroscopic esophagogastric junction and pathological squamocolumnar junction were identical. Regional lymph node metastases were not identified on pathological assessment.\nAt the last follow-up, conducted 2 years and 8 months after surgery, the patient’s health status was fairly good, with no recurrence of the EMPD or carcinoma.\nEMPD was first described in a patient with urinary bladder carcinoma in 1889 []. Since this initial report, EMPD has been described in various sites of the body, most commonly the vulva, perianal region, scrotum, penis, and axilla []. EMPD is subdivided into primary and secondary types on the basis of the presence or absence of associated malignancies. Primary EMPD is thought to be derived from an underlying neoplastic transformation of the intraepidermal portion of a sweat gland, whereas secondary EMPD is caused by the intraepidermal spread of neoplastic cells, typically derived from an underlying adenocarcinoma [, ].\nEsophageal Paget’s disease is quite rare, with only a few cases having been reported [–]. Yates and Koss [] described esophageal Paget’s disease associated with a poorly differentiated squamous cell carcinoma of the distal esophagus, whereas Norihisa et al. [] reported a case of adenosquamous carcinoma of the esophagus with pagetoid extension of the adenocarcinoma component. Therefore, both of these cases were diagnosed as a pagetoid growth of an advanced esophageal carcinoma. On the other hand, Nonomura et al. [] and Matsukuma et al. [] reported esophageal Paget’s disease associated with an early underlying carcinoma, one being an intraepithelial carcinoma and the other, a minimally invasive adenocarcinoma of the esophagus. Ishihara et al. [] also reported a case of an early invasive carcinoma, which consisted of pagetoid squamous cell carcinoma in situ combined with early invasive components and choriocarcinoma at the metastatic site. Abraham et al. [] reported a close relationship between Paget cells in the esophagus and an underlying poorly differentiated adenocarcinoma in the esophagus or esophagogastric junction. From these reports, all previously reported cases of Paget’s disease of the esophagus were thought to be secondary to an underlying carcinoma, although the malignant component varied in each case. In our case, we identified an SCC component, with squamous metaplasia and reserve cell hyperplasia, in the gastric mucosa of the esophagogastric junction, which was followed by Paget cells. However, unlike typical Paget’s disease, only few Paget cells were positive for PAS/Alcian blue staining and immunohistochemically positive for gastric gland mucin, whereas a strong p53 overexpression was observed in both SCC component and Paget cells.\nReserve cells are small undifferentiated cells found as a single layer beneath the endocervical columnar epithelium. They have the capacity to transform into both endocervical columnar and squamous epithelium in the endocervix []. Reserve cell hyperplasia and epithelial dysplasia are frequently observed in the squamocolumnar junction of the cervix uteri, and squamous cell carcinoma of the cervix uteri is considered to be derived from these changes []. Reserve cell hyperplasia and squamous cell metaplasia of the gastric mucosa are rare phenomena. In 1981, Takubo [] reported the resemblance of squamous cell metaplasia to reserve cell hyperplasia in the cervix uteri and considered squamous metaplasia or reserve cell hyperplasia with atypical change as a precursor of SCC in the esophagogastric junction. However, this hypothesis has not been fully elucidated. From histological findings and immunohistochemical results in our case, we speculate that the Paget cells were derived from the squamous cell carcinoma, developing in the squamous metaplasia and reserve cell hyperplasia of the esophagogastric junction. The difference in the pattern of expression of cytokeratin and p63 might reflect the glandular differentiation of tumor cells.\nFDG-PET/CT imaging is currently accepted as the most accurate technique for exploring metastatic lesions of a solid tumor. The combination of metabolic and structural information provided by the PET and CT portions, respectively, has improved the accuracy of tumor staging, detection of recurrence, and therapeutic monitoring, having an enormous impact on patient management [, ]. In patients with EMPD, 18F-FDG PET/CT diagnosis of primary lesions is mainly dependent on the thickness of the lesions, whereas it is more sensitive for the diagnosis of lymph node and distant metastases []. In this case, thick primary lesions showed an intense uptake of 18F-FDG (SUVmax 14.9 and 7.5), whereas thin primary lesions showed only a mild 18F-FDG uptake (mean SUVmax 3.25 ± 0.24). Three of the 10 cases reported, however, showed no 18F-FDG uptake at primary site, as in our case. In 3 of these 10 cases with lymph node invasion and distant metastases of EMPD were upstaged by PET/CT, rather than conventional staging examination. To determine the appropriate treatment strategy for EMPD based on staging, PET/CT may play an important role, although some EMPD might be 18F-FDG negative.\nTraditionally, EMPD has been surgically managed, especially in the early stage of the disease. Achieving adequate margins for the primary lesions is an important factor in reducing the risk of recurrence. In patients unfit for radical surgery, radiotherapy is proposed as alternative treatment, as long as invasive disease has been excluded []. In the surgical treatment of esophageal cancer, thoracoscopic esophagectomy is generally regarded, and accepted, as a minimally invasive surgery []. Biere et al. reported on the short-term benefits of minimally invasive esophagectomy for patients with resectable esophageal cancer, with prevention of pulmonary infection being an important benefit. Furthermore, thoracoscopic esophagectomy with three-field lymphadenectomy, pursuing best loco-regional control by surgery, is a feasible and safe alternative treatment commonly performed in Japan []. Our case was diagnosed as early stage Paget’s disease of the esophagus by endoscopic, CT, and PET/CT findings. But because of the unclear and extensive proximal margin of the tumor, a thoracoscopic esophagectomy was performed to obtain a wide local excision of the EMPD. However, in the pathological diagnosis, Paget’s disease and squamous cell carcinoma were identified in the mucosal layer. Therefore, curative resection with ESD could have been possible. ESD is an effective treatment for superficial esophageal neoplasms. Funakawa et al. [] reported a success rate of 99.4% (164/165) for en bloc resection and 90.9% (150/165) for complete en bloc resection, with no instance of fatal complications. However, the reported incidence of esophageal strictures after ESD for near-circumferential or circumferential esophageal neoplasms is extremely high at 88–100% []. Post-ESD strictures seriously lower patients’ quality of life, being associated with several symptoms, including dysphagia, nausea, vomiting, weight loss, and even cachexia. In contrast, esophagogastric junction cancers have a high rate of submucosal invasion, irrespective of size, compared to non-junctional cancers []. Furthermore, the rates of positive lymphatic and/or venous invasion were remarkably higher in junctional cancers []. Therefore, when ESD is performed for near-circumferential junctional cancer as in our case, attention must be paid to the occurrence of esophageal stricture. It is important to evaluate the risk of recurrence by pathological diagnosis and to consider whether additional treatment, including surgical resection, should be performed. |
A 25-year-old Japanese female presented to our emergency department with the chief complaints of dyspnea and palpitations on exertion, starting 1 month ago. Upon arrival, physical examination revealed systolic murmur. The bedside ultrasound examination demonstrated moderate tricuspid regurgitation and possible pulmonary hypertension and the patient was hospitalized. A contrast-enhanced chest CT showed dilatation of the main PA, filled with a hypodense area with calcification adjacent to the right and left PA. The lumens of the main PA and the hilar areas of the right and left PA appeared almost obliterated by the mass; however, the mass was not attached to the pulmonary valve and did not extend into the peripheral parts of the right and left PA (Fig. ). The differential diagnosis included primary PA tumor and pulmonary thromboembolism, but we suspected it to be a PA tumor based on the radiological findings: a relatively poor contrast effect on the lesion with calcification. Lung perfusion scintigraphy revealed decreased blood flow in the whole bilateral lungs, except for the left lung upper lobe. Due to critical symptomatic obliteration of the pulmonary circulation, an emergency surgery was performed on the second day of hospitalization. Preoperative FDP D-dimer was 1.9 μg/mL, slightly higher than the normal limit (within 1 μg/mL).\nFollowing a median sternotomy and institution of cardiopulmonary bypass, deep hypothermic circulatory arrest was induced for the removal of the tumor. The longitudinal incision was made on the main PA extending into the left PA (Fig. ). A whitish shiny mass filled the lumens without any attachment to the surrounding intima, except that the tumor was attached to the intima of the left interlobar PA. The tumor was completely removed from the vessel lumen (Fig. ). Next, the longitudinal incision of the right PA behind the aorta and the superior vena cava was extended to the right interlobar PA. The neoplasm had no attachment to the intima in this area and was obliterated by the segmental branches of the right PA. The tumor was extracted and completely removed from the vessels, and the peripheral ends of the tumor demonstrated a finger-like appearance (Fig. ). After complete removal of the tumor and copious irrigations, the incisions were simply closed using 6-0 polypropylene sutures. The postoperative CT scan confirmed that no tumorous mass was left behind in the PAs.\nGross pathology showed a soft-to-hard whitish-brown tumor. Microscopically, spindle cells with marked cytological atypia proliferated with tumor osteoid formation. There were also lobular proliferations of chondroid islands composed of atypical chondroblasts (Fig. ). Based on the pathological findings as well as the results of the clinical examination that there was no possible primary tumor, it was diagnosed as a primary ISCOS of the PA.\nShe received adjuvant chemotherapy, but 5 months later, a contrast-enhanced chest CT scan showed a hyperdense lesion with calcification at the upper hilum of the right lung, indicating the recurrence of the disease. Right upper lobectomy was performed, and the resected specimen contained a neoplastic lesion with similar pathological features to the primary lesion. Intraoperatively, a pleural metastatic nodule was also found and resected, which was of the same pathological characteristics. The patient is currently being followed up in an outpatient clinic without any known complications 16 months after the initial surgery. |
An 18-year-old male patient was hospitalized because of a 1-week history of cough, fever, and shortness of breath. Both past medical history and family history were unremarkable. He was diagnosed with pneumonia, but antibiotics did not improve his condition, and he needed oxygen inhalation for respiratory distress. One week later, a cardiovascular examination revealed 2/6 diastolic murmur over the apex and a transthoracic echocardiogram revealed a left atrial mass. He was emergently referred to our hospital. A chest radiograph revealed cardiomegaly and pulmonary vascular congestion. A transthoracic echocardiogram showed a large left atrial mass occluding the mitral inflow. A thoracic computed tomography presented a left atrial mass but no other tumors elsewhere (Fig. ). Soon after admission, his condition rapidly deteriorated after massive hemoptysis to necessitate mechanical ventilation and percutaneous cardiopulmonary support. The patient underwent an emergent surgery under a putative diagnosis of left atrium myxoma. Median sternotomy, aortic-bicaval cannulation, and standard cardiopulmonary bypass were performed. Through a right interatrial groove incision, a large pedunculated hard mass occupying the left atrium was excised; the pedicle was traced to the left superior pulmonary vein. The cardiac surgeons indicated that the tumor might have originated from the left superior pulmonary vein near the left atrium and may have directly invaded the atrial wall to the junction of the right superior pulmonary vein and left atrium. Although the mass was removed as much as possible, the en bloc removal of the tumor, with both superior pulmonary veins and their corresponding pulmonary lobes and atrial wall, could not be performed.\nHistologically, the tumor comprised pleomorphic cells with bizarre nuclei and some spindle cells with blunt-ended nuclei. In addition, multinucleated giant cells and occasional coagulative necrosis (<50% in the examined area) were observed. Besides, mitotic figures were also found with a frequency of 5–7 per 10 high-power fields. The immunohistochemical staining with the proliferation marker Ki-67 revealed a proliferation index of 50%. While the tumor cells were α-smooth muscle actin, h-caldesmon, desmin, and vimentin positive, epithelial membrane antigen, keratin, CD34, and factor VIII were negative (Fig. ). Hence, the diagnosis of leiomyosarcoma was determined.\nAfter the operation, he required the assistance of percutaneous cardiopulmonary support for 5 days and mechanical ventilation for 12 days. However, the remaining tumor grew rapidly during this interval. A transesophageal echocardiography revealed a mass, measuring 1 × 1.5 cm in the left superior pulmonary vein 7 days after the operation, and the tumor grew up to occupying two-thirds of the left atrium at postoperative day 40. Because the curative en bloc excision of the tumor was impracticable, chemotherapy and combined radiation therapy (total amount of 60 Gy) were initiated at postoperative days 46 and 59, respectively.\nOne course of IFO/DOX: ifosfamide, 2.5 g/m2 × 5 days, doxorubicin, 20 mg/m2 × 2 days; 2 courses of CYVADIC: cyclophosphamide, 1,200 mg/m2 × 1 day, vincristine, 1 mg/m2 × 2 days, doxorubicin, 40 mg/m2 × 1 day, dacarbazine, 225 mg/m2 × 4 days; 1 course of MAID: ifosfamide, 2.5 g/m2 × 3 days with MESNA, doxorubicin, 20 mg/m2 × 3 days, dacarbazine, 225 mg/m2 × 3 days; and selective tumor-specific intra-arterial infusion of melphalan (50 mg) via internal thoracic artery could not achieve any response. For salvage chemotherapy, a PAX-containing regimen, ifosfamide, 1 g/m2 × 3 days, doxorubicin, 30 mg/m2 × 1 day, PAX, 140 mg/m2 × 1 day (IFO/DOX/PAX), was initiated, and it achieved dramatic tumor regression (Fig. ). The tumor almost disappeared after 2 courses with IFO/DOX/PAX. After 4 courses of IFO/DOX/PAX, we conducted a tandem PAX-based high-dose chemotherapy (HDC) with autologous peripheral blood stem cell transplantation (PBSCT); the first HDC regimen comprised ifosfamide, 3 g/m2 × 3 days and PAX, 370 mg/m2 × 1 day, and the second comprised etoposide, 300 mg/m2 × 3 days, carboplatin, 400 mg/m2 × 3 days, and PAX, 420 mg/m2 × 1 day, respectively. The patient achieved complete regression of the tumor.\nThe patient enjoyed a sustained complete remission for 2 years after the therapy, but he suffered a brain metastasis followed by bone and pulmonary metastases. No local recurrence was noted. The lesions were refractory to chemotherapy, including PAX, and he underwent palliative surgery and radiation therapy. However, he died 4 years after the onset of symptoms and an autopsy was not allowed. |
In January 2018, a 79-year-old man was referred to our genitourinary medical oncology clinic for management of his prostate adenocarcinoma metastatic to multiple bones. His case was complicated by a concurrent diagnosis of melanoma metastatic to a distant skin site, for which he had started pembrolizumab immunotherapy the week previously. His prostate cancer had originally been diagnosed in November 2011 after a transurethral resection of the prostate performed for urinary obstruction revealed Gleason score 7 prostate adenocarcinoma. In June 2015, he was found to have biopsy-proven prostate adenocarcinoma from a rib metastasis. The patient elected to defer all medical therapy and had his rib metastasis treated with radiation alone. He then had definitive radiation to the prostate in June 2016. His prostate cancer spread to additional bony sites, which were likewise treated with radiation, including radiation to the thoracic spine in July 2017 and the proximal humerus in August 2017, rather than any systemic therapy. In December 2017, he had prophylactic nipple irradiation to prevent gynecomastia in preparation for noncastrating medical therapy with single-agent bicalutamide. On meeting the patient for the first time in January 2018, we elected to continue with the plan for single-agent bicalutamide 50 mg per day, given the patient’s preference and the uncertain prognosis from his metastatic melanoma. His prostate-specific antigen (PSA) was 30.8 ng/mL at the time of treatment initiation and responded rapidly to bicalutamide, reaching a nadir of 1.1 ng/mL in September 2018 ().\nHis melanoma was diagnosed from a shave biopsy of the right superior lateral lower back in August 2015 as localized ulcerated malignant melanoma, unclassified, with nevoid features. It was invasive to at least 1.45 mm and at least Clark level IV, and it had five mitoses/mm2. He subsequently had a microstaging excision and a right back excision with a negative sentinel lymph node in the right groin. In 2017, the patient noticed a new lump on his right lower back scar in 2017. In November 2017, excisional biopsies of his right lower back and right groin both showed metastatic melanoma, as confirmed by immunohistochemistry (IHC) showing positive staining for SOX-10 and MART-1. A 7-mm lesion was also detected on his posterior scalp and was also visible on magnetic resonance imaging of his brain and metabolically active on positron emission tomography (PET)/computed tomography (CT); hence, it was diagnosed as stage IV melanoma. Mutation testing showed an atypical N581I mutation in the BRAF gene, but wild-type status at the BRAF V600 codon. His PET/CT showed extensive osteosclerotic lesions, most of which were not fluorodeoxyglucose (FDG) avid, consistent with metastatic prostate cancer. The most avid lesion in the right ischium had a maximum standardized uptake value (SUV max) of 7.2 but was biopsied and found to be metastatic prostate adenocarcinoma, as confirmed by IHC assessment that demonstrated NKX3.1 expression. He began treatment of his metastatic melanoma with pembrolizumab 200 mg intravenous every 3 weeks and received four treatments before the pembrolizumab was stopped because of the development of pneumonitis. This treatment resulted in a complete response in his melanoma as assessed by physical examination of his scalp lesion. His pneumonitis was treated with a taper of prednisone, which was reduced to physiologic dosing by August 2018.\nHowever, despite evidence of response in his melanoma by physical examination, there was concern for progression on PET/CT in June 2018, with FDG update in the descending colon/small bowel wall, perisplenic region, a mildly FDG-avid left internal iliac lymph node, and a right external iliac nodal conglomerate encasing the right ureter, 4.1 cm in diameter and with an SUV max of 11.7. By December 2018, the right pelvic mass had increased in size to 4.4 cm and in metabolic activity to SUV max 19.8 (). At this time, he was also noted to have multiple small lung nodules of unclear etiology. Because the appearance of the right pelvic mass was judged to be atypical for either prostate cancer or melanoma, it was biopsied by pelvic laparoscopy in November 2018 and was found on surgical pathology assessment to be consistent with a high-grade carcinoma with squamous features (). IHC assessment demonstrated neoplastic cells positive for p63 and GATA3, but negative for PSA and NKX3-1 expression. Urine cytology also showed atypical urothelial cells. We performed exome sequencing using the University of Michigan OncoSeq panel (MI-OncoSeq) and whole transcriptome analysis on formalin-fixed paraffin-embedded tissue from this biopsy. All patients enrolled in the MI-OncoSeq study provided written informed consent approved by the University of Michigan Institutional Review Board. Consent is inclusive of publishing information and/or images from participants (or their designate). However, results were limited because of low tumor content, estimated to be less than 10%. The variant allele fraction of mutations spanned 1% to 6%, and no copy number aberrations were detected (). Because the pelvic mass was causing pain and urinary obstruction, we elected to treat it with a platinum doublet active in urothelial carcinoma and carcinoma of unknown primary, despite not knowing the tissue of origin. In January 2019, we began carboplatin area under the curve 5 mg/mL/minute on day 1 and gemcitabine 1,000 mg/m2 on days 1 and 8 of 21-day cycles, and we completed six cycles before stopping because of progressive fatigue. The patient experienced some improvement in pain, but the size of the right pelvic mass was largely unchanged ().\nIn June 2019, while being treated only with bicalutamide, the patient was noted on CT scan as having a 1.4-cm nodule in the superficial anterior abdominal wall, which subsequently became easily palpable and caused skin erythema. This nodule, together with smaller adjacent nodules, was removed by excisional biopsy in July 2019, where surgical pathology assessment demonstrated tumor features consistent with metastatic squamous cell carcinoma (). A fresh portion of this specimen was sent for analysis using the MI-OncoSeq platform. This specimen showed a much higher tumor content of 54%, allowing the discovery of additional molecular alterations.\nResults were discussed at the University of Michigan Precision Medicine Tumor Board in September 2019. Somatic aberrations detected in the previous sample were detected in the new biopsy specimen, suggesting clonal relatedness, and additional alterations consistent with the increased tumor content were noted as well (). Most curiously, we noted a few reads of chimeric transcripts supporting a gene fusion between TMPRSS2 exon 1 and ERG exon 2, accompanied by a focal deletion located in the intergenic region between the two genes and breakpoint visible on the copy number profile ( and ). Fusions between the TMPRSS2 locus and Ets family transcription factors occur in nearly one half of prostate cancers in the United States and, to our knowledge, do not occur in other cancers. Given the presence of this pathognomonic gene fusion and the patient’s history of prostate adenocarcinoma, we concluded that his squamous cell carcinoma had arisen from his prostate cancer. To further confirm the findings, we subsequently performed IHC analysis, which showed positive ERG expression on the patient’s initial prostate transurethral resection, which was strongly positive for ERG protein (). We further performed ERG IHC assessment on the patient’s recent abdominal biopsy, which demonstrated focal and weak-to-moderate ERG protein expression, supporting a clonal phenotypic origin and evolution from the patient’s original conventional (acinar) prostatic adenocarcinoma ().\nArmed with the knowledge that this patient’s squamous cell carcinoma was either a trans-differentiated or metaplastic variant of prostate cancer, we discussed the possible therapeutic implications at this point, when the patient was taking only single-agent bicalutamide. For prostate adenocarcinoma, the addition of medical castration, likely in addition to either abiraterone and prednisone or a nonsteroidal second-generation antiandrogen, would have been a reasonable next line of therapy. However, the transcriptomics data of the MI-OncoSeq platform showed low expression of the androgen receptor and androgen responsive genes KLK2, KLK3 (PSA), TMPRSS2, ACPP, and SLC45A3 (). In keeping with these findings, his PSA level was only 3.1 ng/mL at this point. Therefore, we concluded that additional therapy targeting androgens or the androgen receptor was unlikely to be successful. Similarly, we examined markers for neuroendocrine carcinoma, the most common nonadenocarcinoma type of prostate cancer. However, expression of the neuroendocrine markers SYP, CHGA, CHGB, and NCAM1 were also low (). Therefore, we did not plan chemotherapy with a regimen such as carboplatin and etoposide, which is active against small-cell neuroendocrine carcinomas.\nWe examined the remainder of the molecular results in an effort to find alternative, clinically actionable molecular targets. We noted two alterations associated with PTEN and Akt signaling: an activating mutation in PIK3CA and a homozygous deletion in PTEN itself (). Inhibitors of mammalian target of rapamycin (mTOR) have been suggested for use in PTEN-deficient tumors. However, overall, the results for mTOR inhibitors in prostate cancer have been disappointing. Some randomized data support the use of PI3K inhibitors in metastatic castration-resistant prostate cancer. However, this was in combination with abiraterone, which made PI3K inhibitors less attractive in this case, given the patient’s near total lack of androgen signaling. Last, the homozygous deletion of CDKN2B could theoretically sensitize to CDK4/6 inhibitors. However, we decided against this option because CDK4/6 inhibitors have thus far shown disappointing results in prostate cancer. Therefore, we elected to treat with docetaxel, the most common cytotoxic chemotherapy drug for castration-resistant prostate cancer.\nAt this point, the patient’s lung nodules, which were previously indeterminate, had enlarged greatly (). We started treatment with docetaxel at 75 mg/m2 for one cycle, then decreased the dose to 60 mg/m2 because of fatigue, and completed three more cycles. We also attempted bicalutamide withdrawal for the adenocarcinoma component of his disease but resumed bicalutamide and added leuprolide a month later after his PSA continued to rise. The patient reported improvement in right groin pain shortly after initiation of docetaxel. CT completed after three cycles showed an interval decrease in the size of multiple bilateral lung nodules, and a decrease in size of his right pelvic mass, compatible with a therapeutic response ().\nIn this case, we used next-generation sequencing to determine that the patient’s squamous cell carcinoma was actually of prostate origin, because of the presence of a gene fusion between TMPRSS2 and ERG. Transcriptomic and histologic analysis showed minimal evidence of androgen receptor signaling, but it also showed a lack of evidence of neuroendocrine differentiation. Such prostate cancers without evidence of androgen receptor signaling and without neuroendocrine markers have been termed “double-negative” prostate cancers. In more recent work profiling rapid autopsy specimens, investigators identified a squamous subtype of double-negative prostate cancer present in eight of 98 patients. Squamous histology prostate cancer was reported previously but was a rare finding before the development of advanced antiandrogens. However, the detection of squamous and other nonadenocarcinoma prostate cancer subtypes has become more common since the development of abiraterone and nonsteroidal second-generation antiandrogens, possibly as a means to escape continual selective pressure against androgen signaling, a phenomenon that had been described rarely in the past. Some of these double-negative prostate cancer (DNPC) tumors seem to be driven by fibroblast growth factor (FGF) alterations, and trials of FGF inhibitors have recently begun in advanced prostate cancer. Our patient did not have an FGF abnormality. Platinum-based chemotherapy is also commonly used to treat squamous cell neoplasms. In the current case, despite having squamous differentiation, this patient had only stable disease in response to platinum-based chemotherapy.\nAfter using whole exome sequencing and RNA sequencing to identify this tumor as a squamous neoplasm of prostate origin, we elected to treat him with an agent approved for prostate cancer (docetaxel), an agent we would not have elected to use without knowing the tissue of origin. We elected not to pursue the therapies that could target molecular alterations in his tumor because of the known survival benefit of docetaxel in men with advanced prostate cancer, but therapies targeting his tumor’s molecular alterations remain options down the road if his disease progresses. In summary, this report demonstrates a case of transdifferentiation of a prostate adenocarcinoma to a DNPC tumor with squamous differentiation without evidence of an FGF alteration. Consideration should be given to docetaxel in patients with tumors of a similar phenotype. |
A 17-year-old Pakistani girl presented to our hospital with the complaints of productive cough, vomiting and high grade fever for one week. A diagnosis of acute bronchopneumonia was made on the basis of physical examination (tachypnea, basilar pulmonary crackles, fever) and postero-anterior view (PA) chest X-ray (right apical cavitation). She was admitted to the hospital and treated with intravenous antibiotics. Her sputum cultures grew Pseudomonas aeruginosa and her antibiotics were modified accordingly.\nPast medical history of the patient was significant for recurrent respiratory tract infections since childhood; many of these episodes were associated with otitis media without perforation of the tympanic membrane. She had visited multiple doctors in the past few years and had been treated for tuberculosis up to three times in the past for a total of twenty four months in addition to receiving multiple courses of antibiotics. Her sputum smears and cultures for acid fast bacilli had not been positive. Her past history was negative for signs and symptoms of malabsorption, recurrent cutaneous infections or regular nasal drip. She had a history of primary amenorrhea at the time of initial presentation to us. She weighed 41 kg and her body mass index was 19.5 kg/m2 at that time.\nAfter an uneventful discharge from the hospital for the bronchopneumonia, the patient was followed up on an out-patient basis for further workup. In view of the history of recurrent infections, the possibility of bronchiectasis secondary to a variety of underlying pathologies such as post-infection, immunodeficiency syndromes or ciliary dyskinesia disorders was considered. Cystic fibrosis was also an important consideration. There was no history of consanguineous marriage in her parents. Computed tomography scan obtained at that time didn’t show features of bronchiectasis. Her sweat chloride test was done as part of the workup. Chemical analysis of a 58 mg sweat sample from the patient showed a result of 22 mmol/L. Her blood analysis for immunoglobulins were performed next, showing a deficiency of IgA, IgG subclass 2 and 4 while her IgE and IgM levels were all normal.\nWithin the next two years, she was readmitted multiple times for severe gastroenteritis, bronchopneumonia and maxillary sinusitis. In addition to several courses of intravenous and oral antibiotics, she also received intravenous immunoglobulins (IVIG) on four separate occasions to help her cope with crisis secondary to severe systemic infections. She showed a successful resolution of the crisis after administration of intravenous immunoglobulins. The possibility of regular monthly administration of IVIG was discussed with the patient but not opted for due to financial constraints.\nAbout five years after the initial diagnosis of primary immunoglobulin deficiency was made, she presented with localized cervical lymphadenopathy and a month’s history of fever. Her laboratory tests showed anemia (hemoglobin = 9.4 g/dl), leukocytosis (total leukocyte count = 14.7 × 109/L), thrombocytosis (platelets=441 × 109/L) and a Lactate Dehydrogenase (LDH) of 404 IU/L. In view of her immunodeficiency, we immediately biopsied the cervical lymph nodes. Histopathological examination of the lymph nodes showed scattered cells with vesicular nuclei, occasionally prominent nucleoli and mitosis in the background of histiocytes, plasma cells and lymphocytes. Based on positivity of LCA, CD 20, CD 3 and CD 30 along with a proliferative index of 30-40, a provisional diagnosis of intermediate grade non-Hodgkin’s lymphoma was made. Bone marrow biopsy confirmed these findings.\nA complete radiological work-up was done using CT with contrast. It showed no mediastinal lymphadenopathy, multiple enlarged lymph nodes in the neck at levels 1, 2, 3 and 4 bilaterally along with left supraclavicular lymph nodes, bilateral enhancing axillary lymph nodes, hepatosplenomegaly, multiple large enhancing notes in peri-pancreatic, aorto-caval, celiac axis, para-aortic and mesenteric locations. She is currently receiving chemotherapy for intermediate grade non-Hodgkin’s lymphoma.\nHer last chest X-ray showed development of fibrotic changes in right upper, middle and lower lung zones as well as bronchiectatic changes in the left basilar region. This most likely occurred in association with the multiple respiratory infections the patient has had in the past. |
A 52-year-old Chinese man was admitted to the Department of Neurology at The First Hospital of Changsha on 1 August 2019. He had been continuously taking warfarin at a dosage of 3.125 mg/day since having undergone mechanical mitral valve replacement surgery 3 years previously. His most recent international normalized ratio (INR) had been measured 1 week before presentation, and the result was 2.2. Two days before admission, the patient suddenly developed low back pain with no obvious cause followed by nausea and vomiting. He was diagnosed with acute myelitis at a local hospital. The next morning, the patient developed a lack of bilateral leg strength, numbness spreading across his feet and chest, urinary incontinence, and a fever of 38.44°C. At the time of admission to our hospital, a CT scan showed no brain abnormalities. A urinary catheter was inserted, and the patient was transferred to the Department of Neurology at our hospital for further examination and diagnosis.\nUpon admission, the patient was conscious and alert with a good sense of direction. His back pain had become less severe than that experienced during the occurrence of paraplegia and numbness; however, he developed a mild headache. Physical examination showed flaccid paraparesis in both legs and an obviously rigid neck. His cranial nerves remained intact. Based on the patient’s history and neurological test results, we performed a lumbar puncture on the day of admission. The cerebrospinal fluid (CSF) was bloody at all three puncture sites; therefore, we concluded that the attempts had been unsuccessful because of puncture injury. The patient underwent a spinal CT scan, which revealed degenerative change in the lumbar spine and a Schmorl node at T9 (). However, no cauda equina or nerve root compression was observed. An urgent brain CT scan showed no abnormalities. Because the patient had a history of mechanical valve replacement surgery, magnetic resonance imaging was contraindicated. Examination of the peripheral blood showed that the INR was 1.95 and that the prothrombin time (PT) was 1.96 s. On the second day, the patient’s brain CT results suggested SAH (). We performed another lumbar puncture, and bloody CSF was retrieved again in three test tubes, thus confirming SSH (). On the third day, the patient experienced more frequent headaches, and the back of his neck grew increasingly more painful. A second brain CT scan showed a high-density area in the left posterior occipital region that was compatible with SAH (). Repeated blood tests revealed a PT of 21.3 s and INR of 1.86.\nIn view of the negative spinal CT results, we performed spinal angiography. Still, no arteriovenous malformation or other vascular abnormalities were found (). Considering the patient’s medical history and clinical manifestations, we temporarily stopped his anticoagulation treatment and administered vitamin K to reverse the anticoagulation, aminocaproic acid to induce hemostasis, and nimodipine to prevent vasospasm; we also performed CSF replacement. Continuous lumbar punctures produced fluid containing hemorrhage and yellow pigmentation (). The high-density area in the left posterior occipital region and the SAH markers were negative after 12 days of follow-up (). Therefore, the patient restarted his warfarin treatment at 1.25 mg/day. At this time point, his PT and INR were 16.5 s and 1.44, respectively. Three days later, we adjusted his warfarin dosage to 2.5 mg/day. Another 2 days later, the patient developed nasal hemorrhage with a PT of 17.3 s and INR of 1.52. Nasal endoscopy examination revealed chronic rhinitis with no other abnormalities. Therefore, we adjusted the warfarin dosage to 1.875 mg/day. Neither nasal bleeding nor cerebral or spinal hemorrhage occurred thereafter.\nThe SSH in this case was presumed to have developed as a result of the oral anticoagulant therapy based on the rise in the PT and INR. Therefore, the dosage was altered accordingly. Because the patient’s general condition was relatively weak and continuous anticoagulation treatment is more effective than sporadic treatment, we performed no surgical treatments. The patient was finally discharged 32 days after admission. Some functional improvements were observed after discharge; he became able to move himself with a standing turner and the assistance of another person instead of entirely depending on others. |
An 81-year-old female patient presented to our emergency department with acute onset of hematemesis and melena. On admission, the patient appeared to have a poor general health condition and was hemodynamically compromised. Her initial laboratory exams revealed a hemoglobin concentration of 7.7 mg/dl without leukocytosis or C-reactive protein (CRP) elevation. The patient had a history of a 6.5 cm gastrointestinal stromal tumor (GIST) of the cardia, for which she initially received downsizing treatment with imatinib 400 mg/d, followed by surgical resection of the gastroesophageal junction and reconstruction with a jejunal interposition (Merendino procedure) 4 years earlier (). Histopathological studies of the tumor revealed a T3-tumor with a positive c-Kit mutation in exon-11 and a Ki-67 proliferation rate of 15%. The risk for disease progression based on Miettinen's criteria was determined as high (size > 5 cm; mitosis rate > 5/HPF), and the patient was subsequently placed on 1st line adjuvant therapy with imatinib 400 mg/d []. After two years on 1st line treatment, the patient developed hepatic and peritoneal metastasis and was placed on sunitinib as 2nd line therapy for metastatic GIST. Treatment with a proton pump inhibitor (PPI) was suspended for an unknown reason two years prior to presentation.\nThe patient was immediately transferred to our intensive care unit, where she was intubated and received two units of packed red blood cells (pRBCs). An emergency gastroscopy was carried out, which revealed active bleeding from a vessel stump in the jejunal interposition, corresponding to a Forrest stage I b upper gastrointestinal bleed. The bleeding was successfully stopped endoscopically by local injection of adrenaline and the application of polymer powder. A CT scan of the thorax and abdomen showed no signs of active bleeding or free abdominal fluid (). The known hepatic and peritoneal metastasis were described as constant in size, but increasingly necrotic compared to a previous CT scan. Due to a renewed drop in the hemoglobin concentration during the course of the day, a repeat gastroscopy was performed. This time, it showed diffuse bleeding without a circumscribed source. As a result, we acted to stabilize the coagulopathy by transfusing the patient with 9 units of pRBCs, 6 units of fresh frozen plasma (FFPs), and 6 units of platelet concentrates. In addition, the patient received 3 g of fibrinogen and 4000 IU of PPSB®, a prothrombin complex concentrate containing the coagulation factors II, VII, X, and IX.\nOn the second day after admission, a temporary improvement in the clinical condition of the patient was observed. It was possible to extubate the patient, who was hemodynamically stable with no signs of active bleeding. A phase of atrial fibrillation was cardioverted following treatment with a beta blocker, digoxin, and amiodarone. On the third day following her admission, the patient's condition deteriorated rapidly with the occurrence of fever, gross hematuria, and decreased oxygen saturation. A delayed hemolytic transfusion reaction was suspected, and positive Rh antibodies (anti-c antibody) were detected. Clinically as well as biochemically, the patient was suffering from a hemolysis with a decline of the hemoglobin concentration to 4.8 mg/dl and an increase of lactate dehydrogenase (LDH) to 3842.0 U/l. Given the lack of a septic focus, only a marginal increase in the inflammatory parameters, and pending blood culture results, no antibiotic treatment or surgical therapy was initiated. Due to imminent respiratory failure, the patient was reintubated. Vasopressors, atropine, and crystalloid solutions were administered to treat bradycardia and shock. However, the patient died on the same evening, following an unsuccessful cardiopulmonary resuscitation.\nThe results of blood cultures taken on the day of the patient's death revealed gram-labile rods without bacterial growth after two days. The subsequent external analysis confirmed bacteremia with C. perfringens and the detection of the alpha toxin gene by polymerase chain reaction, but without any traces of the beta toxin, enterotoxin, epsilon toxin, or iota toxin. The autopsy of the patient revealed a 6 cm sized local recurrence of the GIST and multiple necrotic liver metastases. In addition, a diffuse spread of C. perfringens in multiple organs with advanced tissue lysis was histologically confirmed (Figures –). The mucosal ulcer of the jejunal interposition was located 1.5 cm distal to the esophagojejunal anastomosis, which itself was intact. Death due to a septic-toxic shock caused by C. perfringens sepsis was determined as the cause of death. A contamination of the administered blood products with C. perfringens as the source of the infection was excluded by a subsequent analysis, which was confirmed by an external laboratory. |
The patient is a 57-year-old female who underwent cardiac catheterization via the right common femoral artery two weeks prior to developing a large, symptomatic right common femoral artery pseudoaneurysm ().\nThe patient began complaining of groin pain two weeks after cardiac catheterization. She has a past medical history of aortic valve replacement secondary to aortic valve infective endocarditis, hyperlipidemia, and hypertension.\nShe underwent two attempts of ultrasound-guided thrombin injection of the pseudoaneurysm. On ultrasound, the size of the pseudoaneurysm was found to be 5 cm × 3 cm × 4.6 cm. The neck of the pseudoaneurysm was measured to be 0.8 cm long. The two attempts involved using a 21 gauge needle to administer 1000 units and 2000 units of thrombin, respectively, into the pseudoaneurysm under ultrasound guidance and with the assistance of compression. Due to the size of the aneurysmal cavity and a relatively large pseudoaneurysm neck, injections were found to be unsuccessful on follow-up ultrasound (Figures and ). It was then decided to attempt endovascular closure of the neck of the pseudoaneurysm. All risks were discussed with the patient.\nAfter identification by the attending surgeon, the patient was transferred to the procedure room table in the catheterization lab. The patient received IV sedation, and local anesthesia was used prior to ultrasound-guided percutaneous access to the left common femoral artery. During the procedure, vital signs, including blood pressure, heart rate, respiratory rate, and oxygen saturation, were monitored by an ACLS certified nurse.\nAfter a 21 gauge needle was placed into the projection of the vessel lumen, a guidewire was placed into the left iliac artery. An angiographic catheter and guidewire were used to perform selective cannulation of the contralateral right common iliac artery. Then, a 6 French long access sheath was placed to perform an angiogram. The neck of the pseudoaneurysm was visualized (), and a 0.014 guidewire was placed into the proximal portion of the neck.\nA 21 gauge needle was used to cannulate the proximal portion of the neck percutaneously from the right groin. The previously placed guidewire was used as a landmark to place the tip of the 21 gauge needle into the pseudoaneurysm. After blood return was noticed from the needle, a 0.018 guidewire was placed into the lumen of the right common femoral artery. A 6 French access sheath was placed over the guidewire. Fluoroscopy was then used to visualize the deployment of a vessel closure device (VASCADE 6 French). This was done without difficulty, and the collagen patch was positioned outside the vessel wall in the area of the pseudoaneurysm neck. Interval angiogram revealed partial occlusion of the pseudoaneurysm neck ().\nIt was then decided to place an occlusive 8 mm balloon into the lumen of the right common femoral artery to facilitate pseudoaneurysm thrombosis. The balloon was insufflated up to 8 ATM for 600 seconds. This was done twice in total. Interval angiogram then revealed complete occlusion of the pseudoaneurysm blood flow (Figures and ).\nAll wires and catheters were removed at this point, and a left common femoral artery access sheath was kept in overnight. Postoperatively, the patient had no complications, and formal ultrasound confirmed complete thrombosis of the pseudoaneurysm. The access sheath was then removed without issue. There were no ischemic complications due to balloon occlusion in the immediate postoperative period. |
Our patient is a 67-year-old Hispanic male who presented to our hospital for the chief complaint of progressive worsening of shortness of breath of 3 weeks duration and was admitted for acute respiratory distress due to interstitial lung disease of unknown etiology. His medical history includes coronary artery disease, status postpercutaneous coronary intervention in 2010, hypertension, hyperlipidemia, and diabetes mellitus type 2. He has a significant smoking history of 20 to 30 pack-years and a significant history of alcohol drinking consuming 12 cans of beer per day for 30 years. He has quit smoking and drinking for 10 to 15 years. He works as a janitor and has no significant occupational exposure to asbestos or silicone.\nSix months prior to the day of admission, the patient had complaints of dry cough for which he visited his primary care physician and was prescribed over-the-counter cough suppressants with no relief. The patient had a chest X-ray done at this time which showed mild hazy changes in bilateral lung fields (). The patient continued to have dry cough and eventually developed progressive shortness of breath with no dyspnea at rest. The patient continued to ignore his symptoms until few weeks prior to the day of admission he had significant shortness of breath to the point to which he could not walk to the bathroom in his house. Concerned of this he came to the hospital for further evaluation.\nIn the ED, the patient was brought in by his daughter and he had significant shortness of breath during ambulation which caused him to rest after every few steps. His initial SpO2 on room air was 77% and he was put on 3-litre O2 on nasal canula and his SpO2 improved to 93%. His blood pressure was 134/76 mm of hg, heart rate was 78/min, and respiratory rate was 22/min. On physical examination the patient was awake, alert and oriented, his neck was supple with no jugular venous distension, his heart sounds were audible with no murmurs or gallops, and auscultation of his chest revealed bilateral breath sounds with significant velcro rales on all the lung fields bilaterally. Abdomen was soft and nontender and the patient had no focal neurological deficits. Examination of his extremities revealed no pedal edema but grade 3 clubbing of his finger nails. The patient denied fever, chills, hemoptysis, orthopnea or paroxysmal nocturnal dyspnea, recent travel, or sick contacts.\nChest X-ray in the emergency department showed reticular and hazy markings throughout the both lungs, being worse compared to the previous chest X-ray (). A CT scan of the chest showed extensive honeycombing and bronchiectasis of both lungs which were markedly worse when compared to a CT scan done 4 years ago (). X-rays of both hands and wrists showed early inflammatory arthropathy but the patient denied any joint pain (). 2D echocardiogram showed ejection fraction of 59% with mild mitral regurgitation and no pulmonary hypertension, which was not consistent with CHF.\nConcerning his chest X-ray and CT scan findings, ILD was now the working diagnosis. The differential at this time was idiopathic versus rheumatoid arthritis. Laboratory data showed elevated erythrocyte sedimentation rate 98 (N 0–20), elevated C-reactive protein 17.4 (N ≤ 7.0), elevated rheumatoid factor 275 (N ≤ 10), and elevated cyclic citrullinated peptide >250 (N < 20). To exclude causes of falsely elevated rheumatoid factor, hepatitis C Ab was done which was negative. ANA and DsDNA were both negative.\nThe complete blood count, electrolytes, and renal and liver functions were within normal limits. Lung biopsy was avoided due to the complications of an invasive procedure.\nA diagnosis of interstitial lung disease of the usual interstitial pneumonia (UIP) variant due to rheumatoid arthritis was made. The patient was given intravenous solumedrol 40 mg TID which was tapered and changed to oral prednisone 60 mg daily upon discharge. During his hospital stay he was on nasal O2 3 litres and had episodes of desaturation on ambulation; hence he was discharged with home oxygen. He was advised to continue the rest of his medications for his comorbidities and to follow up with his primary care physician and pulmonologist as outpatient. Eventually the patient was referred to a tertiary care center for lung transplant. The patient is currently on the waiting list for his lung transplant. |
A 25-year-old man presented to our epilepsy center for evaluation of seizures. He was born at term without any developmental delays and had no risk factors for epilepsy including traumatic brain injury, brain surgery, febrile seizures, central nervous system infections, or family history of seizures and no significant past medical or psychiatric comorbidities. Three years prior to his presentation he had his first seizure. He did not remember the event, but while attending basic training in the Army, he was reportedly found in the shower confused by his fellow soldiers. There was no tongue bite or urinary incontinence, but he was disoriented afterward for much of that day. He had another episode within the same month while he was performing physical training exercises, whereby he collapsed and remained confused for hours, but no report of witnessed convulsions. An evaluation at that time was unrevealing. He had 12 episodes in the next 3 years. They were all similar, some associated with lateral tongue laceration suffered during the event. He was seizure-free for 6 months and then began to have spells at least monthly. He denied an aura or premonition preceding his seizures. His wife reported at night that he would “cry” at the onset and then appears to have clonic jerking bilaterally and symmetrically, up to 3 minutes in duration. He was reported to be distressed for a few minutes after the episodes. Brain MRI was reportedly normal and EEG abnormal, but the reports were unavailable. He had been taking levetiracetam 3000 mg daily with topiramate 50 mg daily. He had also tried valproic acid but reportedly had abnormal labatory studies so this was discontinued. At his appointment, it was determined that he would continue his current regimen of levetiracetam, and topiramate was increased to 100 mg total daily. A presumptive diagnosis of epilepsy was made upon clinical grounds though the classification included focal epilepsy localized to the frontal head region or genetic generalized epilepsy manifest as recurrent nocturnal generalized tonic-clonic seizures. At his follow up appointment, a high-resolution 3-T brain MRI was performed and was normal without intracranial abnormalities. EEG demonstrated 3–4 Hz generalized polyspike-and-wave discharges supporting a clinical diagnosis of genetic generalized epilepsy. The patient and his wife had recorded a video of his habitual seizures, which was reviewed an epileptologists (WOT). As noted in the video, he appears agitated and combative and is thrashing his extremities in a non-rhythmic and discontinuous manner with side to side head movements with eyes closed (). He and his wife were clear that this was the semiology of his typical seizure. The side to side head movements, eye closure, and discontinuous nonrhythmic hypermotor activity suggested FS . He was subsequently admitted to the epilepsy monitoring unit for LTVEM for differential diagnosis and classification of recurrent events. During the admission, EEG redemonstrated interictal generalized spike and polyspike and slow wave complexes noted previously. He had one seizure with clinical semiology suggesting a focal to bilateral tonic-clonic seizure due to head version, yet lateralized and focal seizures are known to occur in genetic generalized epilepsies . Despite the appearance of focal features, the ictal EEG demonstrated a generalized seizure onset. Immediately following a definitive diagnosis of epilepsy with electroclinical support from a electroclinical bilateral tonic-clonic seizure, he exhibited the exact same post-ictal behavior that was witnessed in clinic while reviewing the smartphone video. This behavior observed on the smartphone video was therefore able to be linked to his habitual postictal state with violent thrashing that simulated a FS (). In discussion with the patient and his wife, the difference between his seizure and a postictal state with confusion and combativeness was underscored to define a sequence of events rather than separate events. LTVEM was therefore able to establish a diagnosis of genetic genealized epilepsy despite the history suggesting focal epilepsy and the smartphone video suggesting a FS. |
The 71-year-old female was a healthy housewife with no record of medical interventions. She had a family history of cerebral cancer. August 4, 2018, marked the onset of a series of symptoms, including an altered state of consciousness, disorientation and sleepiness and no presence of fever. She first consulted a doctor in private practice and was diagnosed with transient cerebral ischemia. The onset of memory loss and the persistence of the previous symptoms led the patient to seek medical attention in a public hospital where she was admitted and blood analysis was performed. The only alteration in the basic blood panel was high blood pressure, with a value of 149/100 mmHg. Pallor was observed in the skin and integuments. Neurological examination only showed cognitive impairment with bradypsychia, disorientation in time and space and difficulty in carrying out simple calculations, with no fever or meningeal signs. Nuclear magnetic resonance imaging using gadolinium contrast (NMRI) of the brain revealed multiple bilateral cystic lesions containing varying amounts of fluid (white arrows in Fig. Ab). The lesions were detected in several brain locations: the frontal, temporal and occipital lobes (Fig. Aa-d) and in the supra- and infratentorial zones (Fig. Ba-d). Since some of the lesions were compatible with a diagnosis of colloidal vesicular phase neurocysticercosis, because the hospital did not have a stereotaxic frame and due to the multiple locations of the abscesses, the patient was submitted to a right temporal craniotomy under general anesthesia on August 25, 2018. The layers of tissue were separated, working from the skin to the brain and through the superior temporal sulcus. A cyst (without capsule) was removed from the right temporal lobe, which had a diameter of approximately 5 mm, contents with a milky not suppurative aspect and a periphery composed of soft whitish tissue (see supplementary video). A fragment of biopsy-extracted tissue was fixed in formaldehyde at 10% to be processed for histopathological examination. The surgical lesion was closed in layers from the dura to the skin.\nThe patient was discharged on September 3, 2018 with a diagnosis of probable neurocysticercosis and possible hydatid cysts. The sample was not grown in bacterial culture, and the medical ethics committee decided to perform a histopathological study and ELISA to obtain a definitive diagnosis.\nBrain biopsy tissue showed a large necrotic area with an amoeboid structure (red arrow) on the periphery of the brain tissue abscess (Fig. ). The presence of E. histolytica trophozoites in cerebral biopsy specimens was confirmed by immunohistochemistry using a rabbit polyclonal anti-E. histolytica antibody [] (Fig. a) and mouse anti-140 kDa fibronectin (FN)-binding protein (EhFNR) [] (Fig. c). Furthermore, staining with rhodamine phalloidin revealed amoebic structures rich in actin filaments that formed adhesion plaques and macropinosomes (Fig. b, yellow arrows). The rest of the brain tissue was positive for glial fibrillary acidic protein (GFAP) (Fig. d) by immunofluorescence.\nThe presence of E. histolytica in the cerebral tissue was corroborated by PCR, and an 128 bp amplicon of the E. histolytica rRNA gene (NCBI Accession number X65163.1) was cloned from cerebral tissue with the CloneJET PCR Cloning Kit (Thermo Scientific). DNA sequencing was performed in the Unit of Molecular Biology of the Institute of Cellular Physiology (National Autonomous University of Mexico) (Fig. ). Interestingly, the ELISA of the patient serum did not find IgG antibodies against E. histolytica or amoebic proteins. Absorbance data analysis showed a cutoff for the negative control of 186.38; the median for amoebic cerebral abscess patients was 111.5, a number below that of the negative control; however, the median for the positive control was 477.3 (Fig. a and b). Based on a diagnosis of amoebic brain abscess, the patient was treated with ceftriaxone (2 g IV every 12 h), metronidazole (750 mg IV every 8 h), and dexamethasone (8 mg IV every 8 h) for 4 weeks, and no antiepileptic drugs were administered. A deteriorating condition led to her readmission to the hospital on October 14, 2018, and she died four days later. |
A 12-year-old female patient was referred by a private practitioner who detected a mass of soft tissue on the palatal aspect of tooth 11. The patient had a history of alveolar cleft repair done about 1 year back. Clinical examination revealed an extensive soft tissue mass in a palatal defect of tooth 11 []. Gentle exploration revealed that besides loss of the palatal enamel there was also loss of enamel from the interproximal areas of the tooth. However, the labial surface of the tooth was intact. The tooth responded to thermal and electric pulp testing within normal limits. A periapical radiograph revealed a large irregular radiolucency in the coronal region of the tooth extending mesiodistally and slightly into the coronal radicular dentin []. A diagnosis of Class 2 external cervical resorption was made.\nGlycerol was applied to the adjacent tissues to form a protective film. Isolation was achieved using the cuff rubber dam technique. A small cotton pellet was then dipped in 90 percent trichloroacetic acid and the excess was removed by dabbing it on a piece of gauze. The cotton pellet was then applied over the resorptive tissue mass with gentle pressure for about 1 minute. The tissue mass underwent necrosis with repeated application of trichloroacetic acid which was curetted out till a sound dentinal base was revealed []. Care was taken to see that the interproximal areas of the tooth were thoroughly debrided. The cavity was then refreshed with a high speed bur and restored with glass ionomer cement [].\nTrichloroacetic acid is a chemical escharotic agent that causes coagulation necrosis and renders the resorptive tissue avascular.[] The use of trichloroacetic acid does not necessitate raising a flap to gain access to the lesion. This eliminates the possibility of active resorbing cells present in the flap from being repositioned over the repaired root surface.[] However, due to the caustic nature of the acid, certain guidelines need to be followed as outlined by Heithersay:[] a. The adjacent soft tissues should be protected with glycerol, b. A thin glycerol impregnated cotton roll should be placed into the gingival sulcus for added protection, c. The cuff rubber dam technique should be employed to prevent slippage of the acid impregnated cotton pellets into the oral cavity, d. Very small cotton pellets or mini applicators must be used and excess solution should be dampened on gauze, e. Tweezers used to carry the cotton pellets must not be used for other purposes during the procedure.\nA reduction of the microhardness in both dentin and enamel has been reported following the use of trichloroacetic acid.[] Hence, restorations that will reinforce the weakened tooth structure are recommended. Chemical adhesion to tooth structure favours glass ionomer cement as the restorative material of choice.[] Recently a ‘reverse sandwich restoration’ comprising of microfilled resin composite and resin modified glass ionomer cement has been proposed to overcome the hydrolytic instability of glass ionomer cement. The microfilled resin tends to flex with the tooth thus reducing the chances of debonding.[]\nTrichloroacetic acid etches dentin and enamel and hence, conditioning is not recommended prior to insertion of the glass ionomer cement.[] However, refreshing the tooth surface with a bur is necessary because dentin that has been treated with trichloroacetic acid is severely demineralised and is not suitable for bonding with either dentin-bonding agents or glass ionomer materials.[] |
A 61-year-old woman was seen in a clinic complaining of fever and dyspnea lasting 2 weeks. She was diagnosed with left pneumonia by computed tomography (CT) and referred to our institution. At the time of presentation, she had dyspnea and a fever over 38 °C. Her Eastern Cooperative Oncology Group (ECOG) performance status score was 2, and her Hugh-Jones classification was IV. Rhonchi were evident in the anterior chest. Laboratory studies showed an increased inflammatory response. All tumor markers were within normal limits. The chest X-ray revealed an infiltrative shadow in the lower left lung field. CT imaging showed a solid left main bronchial tumor with carinal involvement. Cartilage destruction was apparent, and the boundaries between the tumor and the esophagus and descending aorta were unclear (Fig. ). Therefore, tumor infiltration into the esophagus and descending aorta was suspected. We diagnosed her with obstructive pneumonia due to a tracheobronchial tumor. For the purposes of securing the airway, performing a tissue diagnosis and evaluating the extent of tumor progression, rigid bronchoscopy was initially performed. The tumor almost completely occluded the left main bronchus, and tumor hemorrhage was evident. By coring out the tumor, the left main bronchus was reopened, and detail of the involved area was revealed. The tumor originated from the left main stem bronchus and occupied almost the entire left main stem bronchus. Two tracheal cartilage rings above the carina and one right main stem bronchial ring distal from the carina were invaded by the tumor (Figs. and ). The pathological examination showed three typical types of histology (cribriform, tubular, solid pattern), and she was diagnosed with tracheobronchial adenoid cystic carcinoma. The rigid bronchoscopic treatment resulted in a significant improvement in the patient’s general condition and cardiopulmonary function (PS 2 → 0, H-J IV → I). Fluorodeoxyglucose-positron emission tomography (FDG-PET) showed abnormal enhancement in the tumor with a maximum standard uptake value of 5.3. At the other sites, abnormal FDG uptake was not observed. We determined that if the tumor did not infiltrate the surrounding organs, complete resection would be possible via LSP. Even if a microscopic lesion remains in the resected stump, we can expect improvement in prognosis by administering additional postoperative irradiation. Therefore, we decided to perform the surgery.\nTo evaluate the presence of out-of-wall invasion, we proceeded with the left-side operation in the right lateral decubitus position by complete VATS with three ports in a look-up setting (Fig. ). A right-sided double lumen endotracheal tube was used. Initially, the pleura was opened along the subaortic window. We observed no tumor infiltration into the left recurrent laryngeal nerve, esophagus, or descending aorta. Next, we performed a left pneumonectomy by complete VATS. The left pulmonary artery and vein were dissected into their extrapericardial sections by staplers. Subsequently, the left main stem bronchus was cut near the second carina (where the tumor had not progressed) using the stapler. A protective bag provided easy removal of the left lung from the pleural cavity without enlarging the skin incision. After that, the tracheal carina was exposed circumferentially, and the lower trachea and right main stem bronchus were identified by forceps manipulation. The left chest was closed. After exchanging for a 7.5-Fr single-lumen endotracheal tube, the surgical position and approach were switched to the left lateral decubitus position with a posterior lateral incision and a fourth intercostal thoracotomy. Low volume ventilation with small expansion of the right lung simplifies the operation. Prior identification via left thoracotomy around the carina provided easy circumferential exposure of the trachea, carina, and right main stem bronchus after dissecting the azygous vein. Then, the right main stem bronchus was dissected at the two rings distal from the carina followed by surgical field intubation using a 6.5-Fr spiral tube with a short cuff. The tracheal carina was removed after dissection of the three rings above the carina (Figs. and ). Under surgical intubation, the trachea and right main stem bronchus were anastomosed with a telescope technique using interrupted sutures with full-thickness bites and 4-0 PDS. After completing a left-side semicircle anastomosis by surgical intubation, a right-side semicircle anastomosis was performed under intermittent removal of the tube after sufficient oxygenation during one or two stiches. There was no requirement for prepared jet ventilation. The anastomotic site was wrapped with the intercostal muscle pedicles, and the operation was terminated. To avoid tension on the anatomic site, the chin was tagged to the anterior chest wall by two sutures for 2 weeks. Pathological analysis revealed no tumor component in the resected stump, and we achieved complete resection. After the operation, the patient experienced a panic attack, and hospitalization was prolonged. She improved with psychiatric intervention. She was discharged and walking independently on postoperative day 79. Unfortunately, recurrence via bone metastasis to the left humerus was observed 6 months after surgery, and palliative irradiation is underway. We are currently monitoring her progress.\nLSP is one of the most challenging operations in thoracic surgery, and surgical approaches need to be individualized. Bilateral thoracotomy and median sternotomy are often favored; [] however, as thoracoscopic surgery becomes mainstream, newer and less invasive approaches for extended surgery, such as LSP, are employed.\nCases for which LSP is indicated are generally locally advanced malignant tumors that often involve surrounding organs, and proper assessment is critical. If tumor invasion to the surrounding organs is suspected and preservation of the left lung cannot be expected upon initial diagnosis, this minimally invasive combined thoracoscopic approach has several advantages. With initial left-sided VATS, resectability can be evaluated in advance and in a less invasive manner than with thoracotomy. Confirmation of no invasion to the surrounding tissue makes left pneumonectomy beneficial. Right thoracotomy provides safety and precise anastomosis at the time of carinal reconstruction.\nAn initial right thoracotomy could be considered, but it is difficult to evaluate tumor involvement in the left thorax []. Initial right thoracotomy requires tube intubation through the narrowed left main stem bronchus with tumor invasion. It is difficult to insert a large caliber tube enough to maintain ventilation and oxygenation. In recent years, the usefulness of the clamshell approach for carinal reconstruction has been reported, [] but it has several disadvantages, such as poor visibility of the esophagus and descending aorta and the requirement of extensive detachment of respiratory muscles. The disadvantage of poor visibility is similar in anterior approaches such as the transsternal and hemi-clamshell approaches. However, our approach requires a position change, and there are also disadvantages relative to providing ventilation during airway anastomosis, which is complicated and difficult to address in an emergency. In our case, small volume ventilation from the surgical field provided precise anastomotic maneuvering. Because of the difficulty of laryngeal release, this approach is not suitable when the resection length of the trachea is relatively long. |
A 4-year 7-month-old boy and his parents were referred to the Pediatric Dentistry Postgraduate Program Clinic in June 2016, requesting dental treatment due to multiple dental caries cavities, local infectious processes, and associated pain. Two years previously, the patient had been diagnosed with early infantile GS, confirmed on the analysis of the beta-GAL both in peripheral blood leucocytes and in cultured skin fibroblasts (sequencing of the CTSA gene was not carried out). Previously, the child was insufficiently treated by a pediatric dentist, due to the child's very poor level of cooperation. Only the upper right anterior segment was treated: a pulpectomy procedure on the lateral incisor and extraction of the root remnant of the central incisor. However, the patient did not continue the treatment.\nMedical and dental history revealed that when the child was 1 year of age, his parents noticed the existence of a mild soft outpouching swelling in his lower abdomen, which progressively increased in size. The patient was evaluated at a local public hospital, and the condition was diagnosed as peritoneal ascites, together with three abdominal hernias, due to enlarged liver and spleen.\nAt the moment of the patient's first dental visit, the presence was evident of a huge abdominal growth due to ascites (). According to the treating medical team, this anomaly was unable to be surgically repaired. Because of the significant swelling, the patient had difficulty in maintaining a straightened body posture, and he could not be adequately positioned on the dental chair. In addition, the patient manifested mild mental retardation, language delay, severe bilateral hypoacusia, hepatic damage, and bilateral hydrocele (swelling in the scrotum).\nThe patient's head exhibited a squared form, coarse face, and short neck. The facial profile was markedly convex with an increased lower third, retrusive chin, protruding maxilla, closed nasolabial angle, and manifested lip incompetence (mouth permanently open) (). Intraorally, the examination showed both arches with interdental spacing, carious cavities in all primary molars, a root remnant of the upper left lateral incisor with related abscess fistula and gingival swelling, and macroglossia associated with an evident anterior open bite (). Oral hygiene was very poor, and halitosis was significant.\nThe programmed treatment plan consisted of the placement of composite restorations, pulpotomies and preformed metallic crowns, and extraction of the root remnant. Due to the greatly reduced level of cooperation exhibited by the patient (rated as Frankl's scale level I, definitely negative), it was not possible to obtain X-rays. The patient was very fearful, with clear evidence of treatment refusal, forceful crying, and extreme negativism. Therefore, it was decided to start the treatment with an oral examination, dental prophylaxis, topical fluoride-varnish applications, and the teaching of tooth brushing. Traditional behavioral management techniques, such as conditioning, desensitization, “tell-show-do,” and positive reinforcement, were persistently employed. On the other hand, the patient was unable to maintain a supine or horizontal position on the dental chair due to pain caused by the abdominal hernia. Thus, the patient was approached when he was in a 90-degree seated position, with the aid of his mother. However, all these efforts were unsuccessful. Then, it was decided to treat the patient under general anesthesia, in agreement with the parents, who signed a special informed consent document.\nThe patient was managed according to the American Academy of Pediatric Dentistry (AAPD) guidelines on sedation and general anesthesia. First, the child was sent to the pediatric anesthesiologist for a physical examination and a presurgical health and risk evaluation; respiratory, cardiovascular, and gastrointestinal systems were exhaustively assessed, and blood and urine laboratory tests were indicated; only coagulation times appeared slightly increased. The patient was classified as American Society of Anesthesiologists (ASA) physical status classification III, with lower pulmonary capacity, limited open aperture, macroglossia, and challenging airway access due to decreased diameter. The parents were instructed, through printed guidance, regarding their child's eating and drinking on the day prior to the intervention.\nThe surgical intervention was carried out in August 2016 at the university hospital. After placing routine monitors, according to the American Society of Anesthesiologists standards, general anesthesia was induced via facemask with inhaled fentanyl, lidocaine, propofol, rocuronium bromide, and sevoflurane. Supplemental local anesthesia was also provided at the site of the root-remnant extraction. The extraction site was fully sutured with fine absorbable 6-0 Dexon in order to prevent a potential hemorrhagic episode. The whole surgical procedure lasted approximately 2 hours and ensued without complications. However, the extubation procedure was not possible due to respiratory restriction, and the patient was subsequently transferred to the pediatric intensive care unit (PICU). After 4 days under pharmacological management (dexamethasone, metamizole, ephedrine, clindamycin, and midazolam), together with assisted mechanical ventilation, the extubation could finally be performed. The patient was remitted to the pediatric area, where he was maintained with oxygen nebulization; the case proceeded uneventfully thereafter. He was discharged from the hospital 2 days later.\nThe patient was evaluated at our clinic 15 days after the intervention conducted under general anesthesia. Restorations were found to be in place adequately, and the cicatrization process at the extraction site was uneventful. Then, an individualized oral preventive program was initiated, including dental hygiene practice with a fluoridated paste (1,450 ppm), topical fluoride varnish, MI Paste Plus® applications, and diet counseling. Since then, the patient has been reviewed closely, every month; at each of the visits, the previously mentioned behavior modification techniques were applied in depth. The last control appointment took place in mid-November 2017, during which an excellent oral condition was observed. Currently, the patient is considered a poor candidate for treatment with orthodontic appliances, particularly for treating his anterior open bite. In the meanwhile, the eruption process and occlusal development will be continuously assessed. |
A 10-year-old boy (height, 120 cm; weight, 20 kg) presented with an 8-year history of arteriovenous malformation (AVM) involving the right lower limb. He had been started on sclerotherapy 4 years earlier because of leg length discrepancy causing gait disturbance. However, this treatment was stopped because there was no remarkable improvement. A year before presentation, the AVM had undergone rapid expansion causing significant pain and high-output cardiac failure. Therefore, an amputation of the affected lower limb was recommended at the other hospital.\nOn admission to Kyungpook National University Hospital, an examination revealed that AVM with soft tissue hypertrophy had spread all over the right leg (). A chest X-ray showed marked cardiomegaly with increased pulmonary vascularities (). Electrocardiography showed normal sinus rhythm with right ventricular hypertrophy. Two-dimensional echocardiography showed an enlarged right atrium, right ventricle, and left ventricle, but relatively good left ventricular contractility. The pulmonary artery was also enlarged, but there was no pulmonary edema. Computed tomographic (CT) angiography of the right lower extremity revealed extensive AVM with feeding arteries from the branches of the right profunda femoris and superficial femoral arteries. The venous drainage was through the superficial femoral and deep femoral veins, and there was a marked dilatation of the pelvic vein and inferior vena cava (). The blood investigations were found to be normal.\nOne week before surgery, the patient was scheduled to receive preoperative selective embolization to reduce the size of the AVM and minimize the risk of uncontrolled intraoperative bleeding. For embolization, the radiologist performed catheterization through the left common femoral artery and installed a tourniquet over the right proximal thigh. Soon after, remarkable bradycardia developed due to a baroreceptor reflex-induced abrupt increase in the systemic vascular resistance (SVR), and this procedure was cancelled. After a thorough discussion with the plastic surgeons, orthopedic surgeons, and vascular surgeons, right hip disarticulation was considered to be the best option for improving the patient's quality of life. It was anticipated that the large feeding vessel branches of the right profunda femoris artery, superficial femoral artery, and the large veins draining the limb would be difficult to control during a hip disarticulation. To minimize the chances of torrential hemorrhage, a disarticulation was planned under cardiopulmonary bypass.\nAfter anesthetic induction, an intra-arterial catheter 22G was inserted into the left radial artery to check the invasive arterial pressure and cardiac output by using an arterial pressure waveform-derived cardiac index sensor (FloTrac; Edward Lifesciences LLC, Irvine, CA, USA) and monitor (Vigileo, Edward Lifesciences LLC). Further, a central venous catheter was positioned into the left subclavian vein.\nAfter induction, the mean arterial pressure was maintained between 60 and 80 mmHg. The cardiac index (CI) and the central venous pressure (CVP) were recorded at 10 and 18, respectively, which were considered to be high.\nAfter heparinization, the right iliac artery was cannulated using an 18 French (Fr) cannula, and a 24 Fr venous cannula was placed through the right iliac vein. Partial cardiopulmonary bypass (CPB) was commenced at a flow rate of 1,200 mL/m2/min. The patient's body was maintained at a normal temperature, and the activated clotting time was greater than 400 seconds throughout the CPB. During a partial CPB, a continuous intravenous infusion of milrinone 0.5 µg/kg/min was used to improve the right ventricle function by decreasing the pulmonary vascular resistance. After the initiation of CPB, the CI and CVP were lowered to 2.5 and 3, respectively. The amputation was performed at the level of the proximal one-third of the right femur. The CPB time was 180 minutes. After heparin neutralization using protamine, the patient was weaned off of the bypass with 0.1 µg/kg/min of norepinephrine and 0.5 µg/kg/min of milrinone. Despite the use of CPB, the blood loss was extensive, and a transfusion of 8 units of packed red cells, 6 units of fresh frozen plasma, and 5 units of cryoprecipitate was required.\nHe was extubated on the following day. The postoperative period was uneventful, and he recovered fully with no neurologic deficit. A two-dimensional echocardiography, which was performed a week after surgery, showed a significantly smaller cardiac chamber. Two months after surgery, no residual lesion was observed in a CT angiography (), and a chest X-ray was unremarkable without cardiomegaly (). |
A 67-year-old Caucasian man presented to our hospital after an accident and emergency with a history of five hours of sudden-onset lower abdominal pain. Nine months previously he had been admitted to our hospital with a stroke due to vertebral artery dissection. He developed acute urinary retention at the time, with a residual of 550 mL of urine. He was unable to sense normal bladder filling until he experienced the pain of bladder over-distension. Previous to this he had had no lower urinary tract symptoms. His urological history included an incidental finding of an 11 mm mass upon CT in June 2009 that raised clinical suspicions of a renal cell carcinoma that was under active surveillance. His other pertinent medical history included a left inguinal hernia repair in 2008 that was initiated by using a totally extra-peritoneal approach but was converted to an open repair because of pneumoperitoneum. The patient was a recent ex-smoker, had no significant family history of urological disease, and lived independently. He was taking latanoprost and prednisolone eyedrops.\nHis digital rectal examination revealed a moderately enlarged prostate, and a prostate-specific antigen test returned values within normal age-related limits. He underwent anti-coagulation with warfarin as treatment for the stroke and fitted with a long-term urinary catheter that was left on free drainage.\nFour months after he was fitted with the long-term catheter he had an episode of frank hematuria upon a routine catheter change. A cystoscopy was subsequently performed, which showed edematous urothelium but no focal lesions, as well as an open prostatic fossa. A trial without catheter was performed to determine whether his bladder function had recovered. This resulted in the patient's going back into urinary retention with abdominal pain. Re-catheterization drained 500 mL of urine. The catheter was replaced, and the patient was discharged with an out-patient appointment to discuss future management options.\nIn the interim, the patient presented to the emergency department with acute-onset lower abdominal pain. This pain was associated with diarrhea and vomiting over the preceding 24-hour period. His indwelling urinary catheter was changed without resolution of symptoms or drainage of a significant volume of urine.\nAn examination revealed that he was afebrile and cardiovascularly stable. His abdomen was non-distended but tense with guarding over the lower abdomen. Bowel sounds were heard. Urethral re-catheterization had drained 100 mL of urine with some light hematuria and debris in the catheter bag. Urine analysis showed 4+ blood, 4+ leukocytes, 1+ protein, and +ve nitrites. His blood tests showed neutrophilia (12.3 mL × 109/mL) with a raised C-reactive protein level of 67 mg/L. He was in acute renal failure with a creatinine level of 186 mmol/L (compared with 51 mmol/L three months previously). Plain X-rays showed distended small bowel loops over the central part of the abdomen with a collapsed large bowel and no focal lung lesions or subdiaphragmatic gas. A provisional diagnosis of a urinary tract infection was made, and he was admitted under the care of the physicians. He was treated with intravenous antibiotics (piperacillin/tazobactam combination) and fluid resuscitation.\nHis symptoms failed to settle over the next two days, with continued loose stool, nausea, and vomiting. His urine output was good throughout (> 60 mL/hour), and his renal function normalized. However, he had regular spikes of fever reaching 38.4°C, and his inflammatory markers were raised further. A urological opinion was sought. A consultant urologist diagnosed intra-abdominal sepsis and requested general surgical involvement. CT of the abdomen and pelvis was requested.\nCT showed small bowel obstruction with a transition point just above the dome of the bladder. The patient's bladder was abnormal and diffusely thickened with gas within it that tracked through the bladder dome and into the soft tissues superior and anterior to the bladder, where it was contained and formed several gas pockets that tracked toward the umbilicus. Extensive stranding was present around the dome of the bladder at the point of transition with the small bowel.\nThe patient was taken immediately to the surgical theater for an exploratory laparotomy. A rigid cystoscopy was first performed, which showed a large defect in the dome of the bladder with a possible fistular or urachal mouth in close proximity. Biopsies of the bladder wall were taken close to the defect in the bladder dome. Laparotomy revealed a large defect in the dome of the bladder adjacent to a thickened and abnormal possible urachal remnant (Figure ). The small bowel was dilated without any site of obstruction or bowel pathology. The bladder defect was excised with part of the wall of the bladder to allow repair. Stents, a suprapubic catheter, and two drains were placed. No obvious tumor was seen.\nA histological examination of the bladder wall showed severe transmural inflammation and necrosis predominantly outside the bladder but also involving peri-vesical adipose tissue. The urothelium was reactive but unremarkable. Acute inflammation of the urachal segment extended focally to involve the mucosa, which was lost extensively. In a single section of the bladder wall, a urothelium-lined structure was identified within the lamina propria that was surrounded by smooth muscle. This may have represented a urachal remnant. No tumor, definite urachal remnant, or underlying cause of the inflammation and necrosis was identified.\nFollowing a three-day post-operative stay in the intensive therapy unit, the patient was discharged to a general ward. His recovery was complicated by a post-operative ileus requiring total parenteral nutrition and some superficial wound dehiscence. He was then discharged to rehabilitation in a community hospital 26 days after admission and eventually fully recovered. |
A 29-year old female diagnosed with SLE for 4 years complicated with grade II lupus nephritis presented with status epilepticus. She denied a history of fever on admission, but was treated with cyclophosphamide 1 month prior for an episode of cerebral lupus. She had noticed a papule over the left deltoid region which progressed to an ulcer over 1 week. Fever was noted following several days of hospital admission and the ulcer site became painful. She had worked in paddy fields several months prior to the admission when she was in good health. However, she could not recall any precipitating injury at the affected site during working. She is a mother of two and both pregnancies were uncomplicated. She denied history of alcohol abuse or smoking.\nOn examination she was emaciated and had a GCS score of 15/15 following recovery of status epilepticus. There was no obvious lymphadenopathy. At presentation, the size of the ulcer was about a 3 cm lesion and it gradually developed in to an ulcer with a necrotic center with surrounding erythema. A tentative diagnosis of pyoderma gangrenosum was made with the appearance of the ulcer (Figure ). It gradually advanced into the underlying muscle over 3 weeks of onset despite the antibiotic treatment. Examination of the cardiovascular, respiratory systems, and the abdomen was normal.\nHer full blood count, blood picture, and other supportive investigations showed evidence of microangiopathic hemolytic anaemia, which was suggestive of thrombotic thrombocytopenic purpura which resolved following plasmapheresis. Her ESR was persistently normal. Renal functions were stable during hospital stay, so were the liver profile. Chest radiography revealed evidence of bilateral mild pleural effusions and echocardiography revealed a thin rim of pericardial effusion and good cardiac function. MRI, MRA brain showed evidence of Posterior Reversible Encephalopathy Syndrome. Repeat imaging showed resolved changes.\nA punch biopsy of the skin was done from the lesion and sent for fungal studies and histopathological studies. The direct microscopy examination revealed wide and irregular ribbon-like nonseptate hyphae with right-angle branching suggestive for Mucormycete fungi. Culture was done on Sabouraud dextrose agar with chloramphenicol (at 26°C and 37°C) yielded a white aerial mold, which covered the entire surface of the agar and came up to the lid of the culture bottles after 4 days of incubation (Figure ).\nThe lactophenol cotton blue mount of the growth revealed broad, nonseptate hyaline sterile hyphae. The slide culture test has been attempted with the hope of sporulation, however it was not successful. They only resulted in broad, nonseptate hyaline sterile hyphae without spores. Then the isolate was subcultured on to potato dextrose agar (PDA) and Rose Bengal (RB) agar for induction of sporulation. However, they yielded only sterile mycelia.\nThe isolate was inoculated on nutritionally deficient medium, tap water agar and incubated for 14 days at 37°C. It provided a hazy view of flask shaped sporangium with rhizoids in lactophenol cotton blue mount. Then floating agar method was used and it yielded characteristic flask-shaped sporangium in short sporangeophore with rhizoids after 10 days of incubation (Figure ).The sporangia had a long neck and the apex of the neck closed with a mucilaginous plug. The sporangiospores were cylindrical, with rounded ends. Those morphological features were suggestive for S. vasiformis and the isolate was identified as S. vasiformis.\nThe histopathology of the punch biopsy of the skin also reveled broad aseptate hyphae suggestive of Mucormycetes group of fungi.\nBased on the histopathological evidence of broad aseptate hyphae, suggestive of Mucormycete fungi, the patient was started on IV amphotericin B deoxycholate. Repeated surgical debridement was done and samples were sent for fungal studies. However, local application of antifungals was not included in the management. Her second tissue biopsy, which was taken during debridement after 5 days of IV amphotericin B also had similar direct microscopy findings and yielded S. vasiformis. However third tissue sample which was obtained after 10 days after IV amphotericine B deoxycholate became negative for fungal studies. Following the confirmation of sterile cultures from the subcutaneous biopsies, superficial skin grafting was done which was completely accepted from the wound site. She was treated with intravenous conventional amphotericin B for 28 days and she was asymptomatic when she was discharged from the ward. |
This is a case of 77-year-old male patient who presented for lower limbs edema and abdominal pain with diarrhea of 1-month duration.\nThe patient was an ex-smoker and nonalcoholic. His past surgical history included cholecystectomy and prostatectomy. His medical history was significant for hypertension controlled on Bisoprolol and Irbesartan, dyslipidemia treated with Atorvastatin, and a transient cerebral ischemic attack 5 months prior to presentation for which he was put on aspirin and clopidogrel. At that time, he was incidentally found to have peripheral eosinophilia of 1050 eos/mm3 (13.7% of total leukocytes) without going into further investigations. As family history, he had a brother diagnosed with Non-Hodgkin Lymphoma who died few months earlier after a prolonged hospitalization for unexplained dyspnea, attributed later on to a Strongyloides stercoralis infection detected in his bronchoalveolar lavage only one day before his death.\nHis history went back to 3 months prior to presentation when he was admitted to another hospital for dyspnea, cough, and high grade fever that started few days after inadvertent inhalation of pesticides while working in his garden. CT chest was done and showed bilateral patchy interstitial infiltrates suspicious of allergic pneumonitis (). Bronchoscopy was also done and showed normal bronchial mucosa. Bronchoalveolar lavage (BAL) analysis showed an inflammatory smear and the culture detected few Alcaligenes spp. Blood culture was negative. So patient was considered having pulmonary infection on top of allergic pneumonitis and was discharged home on levofloxacin and prednisone 1 mg/kg for 1 week to be tapered down over 3 weeks thereafter. Although his dyspnea and chest infiltrates were improving during steroids tapering, our patient developed severe diffuse pruritic skin rash that persisted for about 10 days and he started experiencing a progressively increasing epigastric discomfort associated with diarrhea. In addition, he noted an increasing bilateral lower limbs edema. After stopping steroids, his skin rash disappeared but without improvement of other symptoms.\nHis diarrhea was watery and nonmucoid accompanied sometimes by fine streaks of blood and having a variable frequency between 1 and 7 episodes per day. It was associated with moderate to severe left lower quadrant abdominal pain not related to food, as well as epigastric pain and many episodes of postprandial vomiting. He also reported a decreased PO intake and a significant weight loss of 12 kg over the last 2 months. His lower limbs edema was progressively increasing, even after stopping steroids. He had no significant dyspnea or cough and no urinary symptoms.\nOn physical examination, the patient's heart rate was 80 beats per minute, blood pressure was 110/70 mmHg, and temperature was 37°C. He was looking ill without scleral icterus or palpable cervical lymph nodes. His chest auscultation was unremarkable. His abdomen was soft with moderate epigastric and left lower quadrant tenderness and normal bowel sounds. Lower limbs examination showed severe 4+ pitting edema extending from the ankles till the knees. There were no skin rashes and the neurological exam was unremarkable.\nInitial tests showed a white blood cell count of 8200 per mm3 with 14% eosinophils, hemoglobin around 10 g/dL, MCV around 84 fL, creatinine level around 2 mg/dL, and sodium level of 127 meq/L, total serum proteins were 4.5 g/dL with albumin of 1.8 g/dL, liver enzymes were normal, and International Normalized Ratio (INR) was slightly prolonged (1.5). Direct fecal examination was done once and showed no parasites. Stools culture turned out to be negative. Chest X-ray was within normal limits. Twenty-four-hour urine collection revealed only 180 mg of proteins.\nIn view of his unexplained eosinophilia associated with diarrhea and abdominal pain, together with his history of dyspnea and skin rash that flared up while on steroids and his brother's history of strongyloidiasis, we strongly suspected an underlying strongyloides infection. Thus, we requested strongyloides serology test and upper and lower endoscopies. Upper endoscopy showed severe edematous bulboduodenitis with areas of erosions and whitish villi (Figures and ) and lower endoscopy showed multiple patchy erythematous lesions separated by areas of normal mucosa that appeared all over the colon, predominantly in the cecum, and in the ileum (Figures and ). Duodenal biopsy showed severe erosive duodenitis with eosinophilic infiltration and strongyloides larvae (Figures and ). Colonic and ileal biopsies showed severe eosinophilic inflammatory changes without parasitic detection. Serology test showed antistrongyloides antibody titer of 4.5 (normal <1.2). So patient was diagnosed to have strongyloidiasis.\nIvermectin was started at a dose of 200 mcg/kg/day. Patient's diarrhea and abdominal pain disappeared within 3 days. However, patient was still having high eosinophil count after 1 week. Since then, we extended Ivermectin course to 2 weeks until disappearance of eosinophilia. Stool test was obtained 2 weeks after initiating treatment and turned out to be negative. Albumin level was progressively increasing to 3.1 g/dL in about 1 month. |
In May, 2015, a 61-year-old right-handed woman presented at our clinic complaining of dull right posterior pain and intermittent radiating pain from the shoulder to the anterior aspect of the upper extremity with a 3-year history. She often performed repetitive tasks in the overhead position as an orchard farmer. She complained of difficulty in performing work because of the loss of muscle strength in her right upper limb while working for a prolonged time in the overhead position 6 months prior to visiting our clinic. She was diagnosed as having impingement syndrome at another clinic and was treated with physical therapy, medication, and steroid injection. However, no improvement of the symptom occurred. The patient had no trauma history or evidence of systemic diseases. The range of motion in the involved right shoulder was 180° of active forward flexion, 50° of external rotation, and T7-level internal rotation at the back. The range of motion in the uninvolved left shoulder was 180°, 85°, and T7 level, respectively. The impingement signs I and II were positive, but no signs of instability were found. The Obrien test was positive but other rotator cuff tests were negative.\nPlain radiography of the right shoulder showed a radiolucent, elliptical-shaped lesion with a well-defined margins and sclerosis of the surrounding bone (Fig. ). Cervical spine plain radiographs did not show abnormal findings. Computed tomography of the right glenoid revealed a cystic lesion with sclerotic margins measuring 25 × 20 × 20 mm in diameter in the posterosuperior border of the glenoid, but with no communication between the cyst and the glenohumeral joint (Fig. ). Magnetic resonance imaging (MRI) of the lesion demonstrated a low signal intensity on T1-weighted spine-echo images and a high signal intensity on T2-weighted spine-echo images within and around the scapular neck. The cystic lesion within the scapular neck was a multilobular lesion and protruded into the spinoglenoid notch. The suprascapular nerve was compressed by the lesion through a cortical defect in the posterosuperior area of the glenoid (Fig. ). The EMG study confirmed compression of the suprascapular nerve with reduced recruitment in supraspinatus and infraspinatrus muscles (Fig. ).\nWe decided on surgical treatment based on clinical symptoms and radiologic findings. First, with the patient under general anesthesia in the lateral decubitus position, diagnostic arthroscopy of the glenohumeral joint was performed. Severe degenerative tearing of biceps tendon was observed in the intertubercular groove portion. Meanwhile, the biceps tendon showed fraying and degeneration of the free edge of the superior labrum without detachment of the biceps anchor from the superior glenoid tubercle upon probing, hence only biceps tenotomy was performed. The glenoid cartilage and capsule in the vicinity of the affected area showed no abnormal findings. As for the intraosseous ganglion, it was considered impossible to approach from the joint and to completely decompress because it was multiply lobulated in the scapular neck. Thus, surgical exploration of the infraspinatus fossa was performed by an incision inferior to the spine of the scapular with partial detachment of the deltoid. Gentle approach through the fibers between the infraspinatus and teres minor muscle revealed a large cyst. The cyst compressed the suprascapular nerve and artery with adhesion in the spinoglenoid notch (Fig. A). Cystic lesion of 2.5 × 2.0 × 2.0 cm was removed after gentle detachment and release of the ligament (Fig. B). Histological examination revealed an intraosseous ganglion with myxoid change in the wall of the cyst (Fig. C). The infraspinatus fossa dull pain subsided immediately after surgery. No recurrence of the cystic lesion was noted on follow-up plain radiograph and MRI performed 18 months postoperatively (Fig. ). The patient was pain-free during her work activities. Shoulder external rotation strength was graded as 5 of 5. |
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 73-year-old female patient visited the hospital due to left-sided hemiparesis. She did not have a family history of stroke and had been taking antihypertensive medication for the past 10 years and angina medication for the past 6 years. She had a chronic headache for past few years. She had frequent headache with nausea or vomiting on 15 days per month. A neurological examination was conducted, and left hemiparesis, paresthesia, and dysarthria were found (Fig. ). The muscle power of the left upper and lower limbs was Medical Research Council (MRC) grade III and the brain diffusion-weighted MRI (diffusion weighted image [DWI]) showed a right lenticulostriate artery territorial infarction. Obstruction and stenosis of the main vessel were not observed. Multiple CMBs were found in the bilateral deep gray matter and pons on GRE MRI (Fig. A). Transthoracic echocardiography was normal. Cilostazol 50 mg twice daily was administered for secondary prevention of stroke in consideration of the multiple CMBs. The muscle power of the patient's left upper and lower limbs improved to MRC grade IV on the 7th day of hospitalization so she was discharged. Outpatient follow-up examination found that the muscle power of patient's left upper and lower limbs improved to MRC grade V 1 month after discharge. However, her hypertension was not controlled. Therefore, the dose of existing hypertension medication was increased and the follow-up examination found that her blood pressure was well controlled afterward.\nThe patient presented with numbness in the left upper limb 6 months after discharge, and DWI and GRE brain MRI were performed. The newly taken DWI and GRE brain MRI were not different from previous images (Fig. B). Eight months after discharge, the patient experienced acute left hemiparesis and paresthesia with headache and she visited the emergency room within 1 hour of its onset. Neurological examination revealed that the muscle strength of the left upper and lower limbs was decreased to MRC grade IV. The blood pressure of the patient was 200/110 mm Hg when she visited the emergency room and electrocardiography did not show any abnormal findings except sinus bradycardia. The blood test was normal. The recurrence of cerebral infarction was suspected so brain MRI and DWI were performed but an acute infarction was not found. However, a new microbleed was observed in addition to previous CMBs in the right thalamus on the GRE sequence (Fig. C). The muscle power of the patient's left upper and lower limbs improved to MRC grade V from the 2nd day after admission and the patient was discharged 3 days later. After admission, the patient's blood pressure was not well controlled and she complained of headache. Therefore, the dose of previous hypertension medication was adjusted again, and the blood pressure was well controlled afterward. Outpatient follow-up found that the left paresthesia improved to normal, as well improvements with headache.\nTwo months after the occurrence of the new CMBs, the patient experience left-sided paresthesia and visited the emergency room within 2 hours of its occurrence. Neurological examination was performed on presentation and found that the muscle power of the left upper and lower limbs was normal (MRC grade V) but paresthesia, which was previously improved, and headache occurred again. Her blood pressure was 210/110 mm Hg on admission and electrocardiography and blood test results were normal. Laboratory studies and abdominal ultrasonography for evaluation of secondary hypertension were normal. Brain MRI was obtained again to confirm the recurrence of cerebral infarction. No new lesions were seen on DWI. However, it was confirmed that another new microbleed had occurred, in addition to the 2 existing CMBs in the right thalamus on GRE MRI (Fig. D). After admission, her blood pressure was too high and the dose of hypertension medication had to be adjusted again. The blood pressure was well maintained afterward. The patient's paresthesia and headache improved from the next day, and the patient was discharged 5 days later. The left-sided paresthesia began to improve from the 2nd week after discharge and she has been treated as an outpatient without recurrence of neurological symptoms. Patient was followed up for 12 months at the outpatient clinic. Blood pressure was well controlled and there was no abnormal neurological symptoms. |
A 17-year-old female patient visited the Department of Oral Medicine and Radiology with a complaint of swelling in the lower jaw that had begun 5-6 months earlier. The lesion had progressively increased in size and been painful beginning two months earlier. There was no anesthesia or paresthesia of the lower lip, chin, or jaw, and there was no history of trauma.\nOn extraoral examination, the swelling in the mandibular anterior region measured about 8 cm×5 cm extending about 4 cm from the midline bilaterally (). Superoinferiorly, it extended from the lower lip to the lower border of the mandible. The skin over the swelling and the surrounding area appeared normal and the margins were diffuse. On palpation, there was no local rise in temperature, but the swelling was tender, especially in the anterior region. Intraoral examination revealed swelling 7 cm×5 cm obliterating the buccal and lingual vestibule extending bilaterally to the buccal sulcus of the first permanent left mandibular molar and first permanent right mandibular molar and measuring about 3.5 cm from the midline bilaterally (). The expansion of the lingual cortex in the midline region was remarkable. The swelling was bony hard in consistency. The crowns of the mandibular anterior teeth were tipped mesially. The left lateral incisor, right central incisor, and right lateral incisor were grade 2 mobile. Grade 1 mobility was present in the permanent first molar in the left mandibular region and the permanent central incisor in the left mandibular region was clinically missing. The teeth were nontender on percussion. The teeth associated with the swelling - the incisors, canines, premolars and first molars of the left and right side of the mandible - gave a negative response to a vitality test. Since the expansile lesion with clinically aggressive behavior was associated with a missing tooth, a working diagnosis of ameloblastoma was considered. Other odontogenic cysts and tumors including dentigerous cyst and odontogenic keratocyst were considered in the differential diagnosis.\nThe patient was subjected to radiographic examination including panoramic and intraoral radiographs. The panoramic radiograph revealed a large radiolucent lesion of the mandible extending from the roots of the permanent first molar in the right mandibular region to the permanent first molar in the left mandibular region with well-defined corticated borders (). The lesion was associated with an impacted permanent central incisor in the left mandibular region, which appeared to be displaced towards the inferior cortex of the mandible. A few radiopaque flecks of about 1-2 mm were noted. The roots of the premolars and molars in the right and left mandibular region showed resorption and all the roots of teeth associated with the lesion showed loss of the lamina dura and widening of the periodontal ligament space. The roots were tipped distally. The lower border of the cortex showed endosteal resorption. The mandibular occlusal radiograph revealed a striking cortical expansion, especially that of the lingual cortex with dense septae in the lingual region giving a multilocular appearance (). Several radiopaque foci of about 1-2 mm were seen scattered, which were less evident in the panoramic radiograph. Cortical thinning and resorption were evident.\nThe patient was further subjected to a computed tomography (CT) examination, which demonstrated a large expansile radiolucent lesion with multiple flecks of calcification of varying sizes, which were predominantly peripherally distributed and impacted the permanent central incisor (). The remarkable lingual and buccal expansion, perforation of the lingual cortex, and dense septae in the lingual region were evident in the CT. The soft tissue density at the periphery suggestive of a capsule was also appreciable (). The dimensions of the lesion measured 5.82 cm×3.28 cm×3.25 cm (). A multiplanar three-dimensional reconstructed image showed a multilocular appearance with thick lingual septae and the position of the impacted tooth ().\nA radiographic diagnosis of adenomatoid odontogenic tumor (AOT) was arrived at considering the multiple scattered radiopaque flecks in the lesion associated with an unerupted impacted tooth and a soft tissue capsule. However, a multilocular appearance and large size in an AOT is unusual. Hence, a differential diagnosis of other multilocular mixed lesions such as calcifying odontogenic cyst and calcifying epithelial odontogenic tumor was also considered.\nThe lesion was surgically enucleated. Macroscopically, the mass was well encapsulated with cystic areas along with an embedded permanent mandibular central incisor in the tumor mass. Histopathological examination revealed sheets, ducts, and whorls of darkly staining ovoid to round epithelial cells suggestive of odontogenic epithelial cells (). The duct-like structures were lined by columnar cells. A few basophilic calcifications were also observed. Small cystic areas containing degenerated cell debris were noted in the focal areas. The supporting connective tissue stroma was loose and less cellular in nature. Based on these findings, a histopathologic diagnosis of adenomatoid odontogenic tumor was made.\nThe patient was under follow-up and had not shown any signs of recurrence six months after surgery (). |
A 14-year-old male presented to the emergency department with complaint of left lower extremity pain for 5 days. The pain was localized to the left thigh, worsening over time despite analgesic intake. Patient also complained of swelling of the thigh, difficulty in ambulation for 2 days, and numbness for 1 day. There was no history of trauma, recent surgery, medication use, or prolonged immobilization. There was no family history of clotting or bleeding disorder or venous thromboembolism. He denies any history of smoking or illicit drug use.\nPatient is a known case of type 1 diabetes mellitus diagnosed at age of 10 years, currently on insulin pump. He was diagnosed with hypertension at age 9 and is on enalapril. He was born in Jamaica, via normal spontaneous vaginal delivery at term and had shoulder dystocia at birth for which he stayed in the hospital for 10 days. A sling was applied and no other intervention was done. Patient's mother denied any other complications at birth.\nOn examination he was noted to have marked asymmetry between the two lower extremities. There was tense swelling of the left posterior thigh and the left calf, which was tender to palpation. No erythema, warmth, varicose veins, or ulcers were present. Peripheral pulses were palpable and equal bilaterally with normal neurological exam.\nHis initial laboratory results in the emergency room showed normal complete blood count, basic metabolic panel, prothrombin time, and activated partial thromboplastin time. A lower extremity ultrasound showed the left common femoral, left superficial femoral, and left popliteal vein were noncompressible and demonstrated no vascular flow, with intraluminal echogenic thrombus suggestive of deep vein thrombosis of the left lower extremity ().\nHe was admitted to the pediatric floor and started on low molecular weight (LMW) heparin and warfarin after hematology consultation. His chest X-ray was normal. A thrombophilia workup was done which showed no prothrombin gene mutation, normal levels of Factor V Leiden, antithrombin III, and protein S. Protein C was low 51.9 (normal 55–123 units IU/dL). Low protein C in the setting of a large DVT was attributed to consumption of coagulation factors. LDH, uric acid, and homocysteine level were normal. Anticardiolipin and lupus anticoagulant were normal.\nA CT of the abdomen and pelvis was done to determine the extent of the thrombosis in the pelvis. The CT showed the suprarenal IVC and the hepatic segments of the IVC were patent. There was absence of infrarenal IVC (). There was an anomalous course of the external iliac veins communicating with lumbar veins. There was heterogeneous material within the left common femoral vein and left external iliac vein and hypodensity within the left lumbar vein consistent with thrombus (). There were prominent azygous and hemiazygous veins. The left kidney was small in size and there was compensatory hypertrophy of the right kidney (). The renal veins were not thrombosed and the origin of the left renal vein was normal in caliber. There was calcification of the right adrenal gland noted consistent with prior adrenal hemorrhage, the etiology of which could not be ascertained.\nOn review of patients past medical records it was noted that as a workup of hypertension he had a CT angiogram done which demonstrated atrophic left kidney supplied by two hypoplastic renal arteries arising from the abdominal aorta. The origin of the more inferior renal artery had a short segment of stenosis (). The right kidney was normal. A DMSA renal scan done subsequently demonstrated left renal uptake of approximately 13% and right renal uptake of approximately 87%.\nThe patient was continued on low molecular weight (LMW) heparin until his international normalized ratio (INR) reached more than 2. His pain and stiffness improved and he was discharged on oral warfarin therapy. Patient and mother were made aware that he may need lifelong anticoagulation therapy. In view of the fact that the patient had venous and arterial anomalies, prior to discharge the patient received a brain MRA/MRV to look for any other vascular anomalies, which were normal. A genetic evaluation was also normal. The patient is being followed by hematology team for venous thrombosis as an outpatient and is on oral warfarin therapy with therapeutic INR. |
A 72-year-old female with a medical history of AS, diabetes mellitus type 2, chronic kidney disease stage 3, gout, colon cancer treated with resection and colostomy bag placement 18 years prior, hyperlipidemia, hypertension, and morbid obesity presented with GI bleeding evidenced by black tarry stool in her colostomy bag for 7 days. Associated symptoms included fatigue, nausea, and decreased appetite. The patient reported no vomiting, abdominal distension, or abdominal pain. She had not recently taken any nonsteroidal antiinflammatory drugs. Echocardiography 1 month prior to presentation showed aortic valve area of 1.24 cm2, aortic valve mean gradient of 39 mmHg, and aortic orifice peak velocity of 4.11 m/s, indicative of moderate to severe AS.\nThe patient was initially seen at a regional hospital where her hemoglobin (Hgb) level was as low as 6.0 g/dL. During her stay at the regional hospital, she underwent push enteroscopy, colonoscopy, tagged red blood cell (RBC) scan, and angiography. The RBC scan showed delayed images for tracer accumulation in the right colon/terminal ileum. She was transfused 2 units of packed RBCs on day 4 of hospitalization and 3 units of packed RBCs on day 6. She was transferred to our tertiary care center on day 7 of hospitalization.\nOn arrival at our hospital, the patient's vital signs were within normal limits. She was alert and oriented. Her colostomy bag was located on the right lower quadrant of the abdomen and contained black tarry stool. Erythema was visible around the colostomy site. She had an existing colectomy midline wound with a linear bandage beginning below the xiphoid process and crossing the umbilicus. Other significant abdominal findings included abdominal distension and tenderness at the periumbilical and hypogastric region on deep palpation. A large hernia protruded from the pelvic region. Cardiovascular examination was significant for systolic ejection murmur, III/VI in intensity on the Levine scale, in the aortopulmonary area. Eye examination revealed conjunctival pallor.\nOn admission, the patient's laboratory results were significant for Hgb of 8.3 g/dL and a platelet count of 96 platelets/μL. Video capsule endoscopy showed blood in her proximal small bowel, and double-balloon enteroscopy (DBE) showed jejunal angiodysplasia that was treated with argon plasma coagulation. Repeat DBE on day 3 of hospitalization at our facility showed another small jejunal angiodysplasia that was also treated with argon plasma coagulation. Her Hgb remained stable during admission. She was discharged after 3 days of hospitalization with home health to resume wound care for her abdominal wound.\nThe patient continued to have dark stools after discharge. She was readmitted to the regional hospital 10 days after discharge. Repeat complete blood count showed Hgb of 6.2 g/dL. She was transferred back to our hospital the next day after being transfused another 2 units of packed RBCs. Repeat DBE showed a 1-mm focus of active bleeding in the proximal jejunum consistent with a Dieulafoy lesion ().\nThe lesion was treated with argon plasma at 1 L/min and 25 watts. Two homeostatic clips were placed to prevent further bleeding. India ink 0.3 mL was injected to tattoo the area. The patient's Hgb remained stable at 7.9 g/dL during the postoperative observation period. After 2 days, she was discharged home. At follow-up 1 year later, she reported no GI bleeding symptoms since discharge. |
A 43-year old female (gravida 3, para 1) presented to an outside hospital with pelvic pain and vaginal bleeding in December 2012, for which she was admitted to the emergency room; following a physical examination and CT scan of the pelvis, a 20 × 10 × 15 cm pelvic mass was identified. The lesion was compressing the rectum, bladder and left ureter, which caused severe, ipsilateral hydronephrosis. The patient's medical history was significant for a supracervical hysterectomy in 2008 to address uterine fibroids; her most recent Pap smear and pelvic exam, both of which were negative for malignancy, coincided with the aforesaid hysterectomy.\nIn January 2013, the patient underwent an exploratory laparotomy, biopsy and lysis of adhesions, which revealed a 10 × 8 cm necrotic, vaginal mass with a 5 × 5 cm pedicle at the vaginal apex. Subsequently, the mass and lysis of adhesions were biopsied. Abdominally, she had a 10 × 15 cm solid mass posterior to the bladder, normal-appearing right tube and ovary; there was a large amount of omental adhesions, which were lysed. The patient received 2 units of blood prior to the surgery and received an additional 2 L of blood intra-operatively; ultimately, she tolerated the procedure well.\nPathology of the pelvic mass revealed a hypercellular lesion without significant mitotic activity, atypia or necrosis, consistent with a cellular leiomyoma. The vaginal mass was composed predominantly of non-viable/necrotic tissue and only focal viable atypical tissue was available for evaluation (a); albeit suspicious for malignancy, given the scant nature of the atypical focus (b), a definitive diagnosis of malignancy could not be rendered at that time. Consequently, in lieu of a re-biopsy, the oncology team opted for complete removal of the disease.\nThe patient continued to suffer from intractable vaginal bleeding and thus, she underwent an abdominal aortogram, selective bilateral internal iliac arteriography and embolization of the pelvic mass in January 2013. Selective left hypogastric branch vessels demonstrated significant tumor vascularity; they were catheterized and then embolized, which effectuated a substantial reduction of tumor flow. Moreover, super selective injection of one branch demonstrated active arterial extravasation; this was successfully resolved following coil embolization.\nA follow-up CT of the chest, abdomen and pelvis revealed a large thrombus in the inferior vena cava (IVC) and left iliac veins, which extended into the right atrium (); there was also evidence of enhancement, indicative of tumor thrombus or benign metastasizing leiomyomatosis. In consideration of the lesion's presence in the right atrium, cardiovascular surgery consultation was emergently recommended and an ensuing transthoracic echocardiogram was performed ().\nInterestingly, the patient's medical history was negative for any cardiac or thoracic symptomatology. She also denied any specific knowledge of having a myocardial infarction, dysrhythmias, palpitations, or cardiac murmurs. Nonetheless, the patient reported increased dyspnea in the few months preceding her hospitalization; her sitting blood pressure was 132/92 mm Hg and pulse rate was 133 beats/min.\nIn February 2013, the patient was admitted to the emergency room due to asthenia, vertigo and a urinary tract infection; she also reported moderate bleeding. The patient was febrile at 102 °F; her hematocrit was 29% and creatinine was 1.2 mg/dL. The patient was administered antibiotic therapy and transfused with 2 units of packed red blood cells to ameliorate her symptoms.\nIntra-cardiac leiomyosarcomatosis is an extremely complex and precarious condition and thus, the patient's management necessitated a multidisciplinary, formulated approach; this involved substantive pre-operative discussion among the cardiovascular, vascular, and gynecologic oncology surgeons. Since significant patient blood loss was anticipated, a comprehensive transfusion protocol was thereby instituted. Moreover, the operating room was reserved for the entire day to accommodate this multifaceted surgical procedure.\nInitially, the patient underwent cardiovascular surgery, comprising a sternotomy via a combination of central and peripheral cannulation in the right common femoral vein, extending up to the common iliac artery. The heart was arrested; cardioplegia was administered at 30-minute intervals and hypothermic circulatory arrest was performed after the head was packed in ice. Simultaneously, a right atriotomy was performed with removal of the right ventricular and atrial components of the mass; there was no tumor identified in the ventricle and/or coronary sinus.\nThe tumor was removed en bloc approximately 2 cm into the IVC; however, the mass was friable and had to be removed in discrete sections (Fig. 4), indicative of a malignant process. A Foley catheter was used as a Fogarty arterial embolectomy catheter into the IVC, of which successive passes were negative for evidence of tumor or thrombus. The right atrium was closed with 5-0 Prolene suture in a running technique; the IVC was closed and cardiopulmonary bypass was reinstituted; the aortic crossclamp was removed, allowing for cardiac resuscitation.\nThe tumor was removed en bloc approximately 2 cm into the IVC; however, the mass was friable and had to be removed in discrete sections (Fig. 4), indicative of a malignant process. A Foley catheter was used as a Fogarty arterial embolectomy catheter into the IVC, of which successive passes were negative for evidence of tumor or thrombus. The right atrium was closed with 5-0 Prolene suture in a running technique; the IVC was closed and cardiopulmonary bypass was reinstituted; the aortic crossclamp was removed, allowing for cardiac resuscitation.\nVascular surgery consultation was then indicated; thereafter, a vena cava thrombectomy, right common iliac tumor thrombectomy and left common and external iliac tumor thrombectomy were planned. Total circulatory arrest was necessary to extract the tumor from the patient's ventricle and IVC. The neoplasm's removal from the iliac system occurred in 2 stages. During total circulatory arrest, the heart and vena cava were opened. The tumor was extracted; similar to the cardiovascular surgery, the mass was incohesive and thus, removed in distinct segments; estimated blood loss was approximately 4 L.\nThe final operative component encompassed gynecologic oncology surgery. Initially, the retroperitoneum on the right side was entered. Once the vagina was identified, the posterior region was divided, entered and the rectovaginal space was developed. An ovarian cyst was visualized and subsequently removed using blunt dissection; the bladder and ureters were both mobilized. Accordingly, the pelvic mass was completely mobilized.\nOnce the lesion was completely unencumbered, the neoplasm was extracted from the pelvis. Hemostasis was appreciated although there was large volume blood loss (10 L), necessitating a massive transfusion. The patient received 34 units of packed red blood cells, 30 units of cryoprecipitate, 8 units of fresh frozen plaza, 7 units of platelets and one dose of Recombinant Factor VIIa. When the procedure was concluded, she was taken to the Cardiovascular Intensive Care Unit in stable condition.\nDespite the initial pre-operative pathology results (i.e., a cellular leiomyoma), additional evaluation of the patient's gynecologic and vascular specimens revealed a high grade leiomyosarcoma of uterine origin. At the conclusion of surgery, the patient did well; she did not suffer from any significant complications and fulfilled all appropriate recovery criteria prior to discharge, which occurred on postoperative day 18. Consequently, she began six cycles of gemcitabine (1000 mg/m2) and docetaxel (75 mg/m2) chemotherapy. |
The patient was a 42-year-old woman. She had suffered from migraine and tension-type headaches since her twenties. The migraine headache was described as pulsatile, bilateral, and on the forehead, persisting from a few hours to half a day. It occurred seven to eight times a month irrespective of menstruation and was accompanied by aura (partial deficit of the left visual field lasted approximately 10 minutes), light sensitivity, and nausea. She took oral loxoprofen 60 mg to treat the headache, on average, <15 days a month, which did not meet the standard of mediation-overuse headache. The tension-type headache was followed by muscle stiffness from the shoulders to the neck and was exacerbated by fatigue. The frequency of pain attacks was one per week. The duration was 1 or 2 days. The headache was bilaterally located, of pressing quality, was not aggravated by walking, not associated with nausea and photophobia.\nEight days before admission, the patient had engaged in farm work. During this work, she reported that grass fragments had entered her right eye while operating a mower. She experienced strong pain and a foreign body sensation but stated that there had been no bleeding or inflammation. The next morning, she reported general malaise and a persistent pulsatile headache on both sides of her forehead, accompanied by a fever of 38.5°C by the evening. The headache was accompanied by nausea and occasional vomiting; it was aggravated by turning her face downward and was not associated with photophobia and phonophobia. The effect of loxoprofen was inadequate and lasted only a few hours. The symptoms gradually worsened over the following 3 days, and the nature of the headache changed to a pain that tightened around the whole head. Nausea appeared in addition to the headache, so she presented to a nearby clinic. Head computed tomography was performed and showed no evidence of cerebral hemorrhage. She was discharged with reassurance; however, her headache gradually worsened and she consulted the clinic again 2 days later and was referred to our hospital with suspected meningitis.\nNeurological examination, laboratory data from blood and spinal fluid (Table ), and contrast-enhanced head magnetic resonance imaging (Figure A) showed neither meningitis nor any other abnormality that could explain the headache. The serum antibody of tsutsugamushi disease, which is a kind of Lyme disease, was negative. Systemic reactions including BHL, serum Ca high values, which suggest sarcoidosis, were negative. Head computed tomography (Figure B) and computed tomography angiography (Figure C) also revealed no cerebral hemorrhage, vertebral artery dissection, or cerebral aneurysm. At this time, she described the headache as 10/10 on a numeric rating scale (NRS). Intravenous infusion of 1000 mg acetaminophen over 2 days reduced the severity of the headache to an NRS of five. Although the patient reported a considerable improvement in the headache, she stated that the mild occipital pain remained. A stinging pain was described that lasted for several minutes and was mixed with a constant and background occipital pain. We considered occipital neuralgia at this point and started treatment with 400 mg of oral carbamazepine, which improved the headache to an NRS of two by the following day.\nOn the fifth day of admission, the patient reported difficulty in opening her mouth. The distance between the upper and lower incisors was 5 mm. Temporomandibular joint MRI showed no abnormality and excluded temporomandibular joint disease (Figure D). Based on the history and characteristic symptom, she was diagnosed with tetanus and treatment was started with tetanus toxoid vaccine, human tetanus immunoglobulin (3000 units), and penicillin G (12 million units). By the next day, this treatment had improved the remaining headache that encircled the whole head to an NRS of 0, but an occipital headache remained.\nDuring the subsequent disease course, the patient developed various symptoms, including facial nerve paralysis, stiffness of the tongue base, photophobia, and cardiac autonomic nervous disorder (Figure ). She developed facial nerve palsy and stiffness of the tongue base the day after the appearance of trismus. The facial nerve palsy was bilateral and peripheral. Her nasolabial grooves were equal on the both sides, but the weakness of the orbicularis oculi muscle was left-side dominant, which caused leakage from the corner of her mouth when she took fluid orally. Although the stiffness of the tongue base caused a sensation of throat obstruction, she could breathe and swallow normally. She developed dysarthria due to the trismus and weakness of orbicularis oris muscle. Photophobia appeared in the order of the left to the right side, and she experienced a loss of taste.\nAlthough most symptoms began to improve with treatment, she reported palpitations under mild exertion on the 20th day. The coefficient of variation of the R-R intervals on an electrocardiogram was 1.71% at this point, indicating autonomic dysfunction, but this improved to within the normal range 1 week later (3.14%). She was followed as an outpatient, and after 7 weeks she had regained full strength of the orbicularis oris muscle and her persistent occipital pain had improved. |
A 5-year-old previously healthy, VZV unvaccinated boy presented to our emergency department with typical varicella skin lesions which had developed 2 days prior. He had a history of fever and poor oral intake. Furthermore, he complained of pain around the left thigh and was reluctant to bear weight. The child was in a mildly reduced general condition with normal heart rate, respiratory rate and blood pressure for age. He was febrile with a temperature of 39.6°C. The cardiopulmonary examination was unremarkable. Next to multiple crusted skin lesions there was a tender and discolored area (3–5 cm) on the left buttock (). The boy refused to sit or lie on his back.\nLaboratory work-up showed a white blood cell count (WBC) of 7.2 G/L, platelets of 131 G/L and a CRP of 195 mg/L. Blood cultures were drawn and intravenous Cefuroxime and Clindamycin were started for suspected bacterial soft tissue infection. Growth of GAS from the blood culture was reported with a time to positivity of 2.6 h by the microbiology laboratory. Ultrasound showed signs of soft tissue inflammation around the painful area at the buttock. On the second day of hospitalization the patient had progressively worsening pain of the left thigh. A CT scan revealed inflammation and swelling of the gluteal muscle. Urgent surgical debridement was performed and intraoperatively necrotising fasciitis was confirmed. Tissue swabs grew GAS. Although antimicrobial treatment was started promptly, GAS was still detected in the tissue samples 48 h after initiating betalactam and lincosamide antibiotics at the first debridement. Further blood cultures were not taken at this time. As there was little improvement during the following days, an MRI was performed showing multiple abscesses in the gluteal muscle but no osseous involvement. Overall the child needed two further debridements on days 3 and 4 of hospitalization with application of a vacuum assisted closure (V.A.C.) therapy. On day 5 of hospitalization the patient presented with respiratory distress and required supplementary oxygen. He was transferred to the Pediatric Intensive Care Unit (PICU). On clinical examination a new systolic murmur was heard. Echocardiography revealed mitral valve prolapse with regurgitation. Assuming an endovascular infectious complication, a further set of blood cultures was drawn (which remained sterile) and antibiotic treatment was changed empirically to Gentamycin and Ceftriaxone. Two days later the boy's general condition deteriorated further and a second echocardiography revealed progressive prolapse of the mitral valve, assuming rupture of the chordae tendineae (). X-ray of the chest revealed pulmonary infiltrations due to mitral regurgitation (). The child was intubated and transferred to a tertiary pediatric cardiac surgery center where the mitral valve was reconstructed the next day and neo-chordae were implanted. Endocarditis was confirmed intraoperatively (small proliferative inflammatory changes of the endocardial tissue) and antibiotic treatment was adjusted to intravenous amoxicillin and continued for 4 weeks. At the day of transfer to the cardiac surgery tertiary center, CRP was 32 mg/l, WBC 15.9 G/L and the child was afebrile. The last documented laboratory findings after 4 weeks of antibiotic treatment showed a CRP <4 and WBC 3.97 G/L and a blood sedimentation rate of 28 mm/h. The wound on the buttock was successfully closed 2 weeks after placement of the V.A.C. Four and a half weeks after primary admission the patient was discharged home in good clinical condition. Cardiology follow-up 1 month later revealed good biventricular function and only mild mitral regurgitation. Screening investigations for an underlying immunodeficiency (quantitative and qualitative humoral and cellular testing and HIV screen) were unremarkable. S. pyogenes M serotyping was not done by our laboratory. |
An 85-year-old Caucasian woman presented to our hospital with right flank pain 10 years ago. She had a past medical history of type 2 diabetes mellitus and essential hypertension. She denied any history of thyroid disease and neck irradiation. She had no family history of any cancer. She was a housewife and had no history of tobacco smoking or consuming alcohol. A physical examination at the time of presentation was not significant except for right costovertebral angle tenderness. Her heart rate was 96 beats per minute and blood pressure was 155/90 mmHg. The findings of laboratory tests, which were complete blood count, liver and renal function tests, and urine analysis, were within normal range and they did not help us find the etiology of her right flank pain. Abdominal screening with computed tomography (CT) revealed a mass on her right kidney, which was considered a primary renal cell carcinoma and she underwent a right nephrectomy. Unexpectedly, PTC metastasis was diagnosed from demonstrative histopathological findings, such as positive immunoperoxidase staining for thyroglobulin (Tg). After further examinations of her thyroid and neck with ultrasonography (USG), a total thyroidectomy was performed. Pathological examination of thyroid tissue revealed a 5 cm tumor with capsular invasion and a strong positive immunoperoxidase staining of cytokeratin-19, HBME-1, and galectin-3. She was diagnosed as having metastatic PTC. Orally administered levothyroxine 75 mcg daily was initiated in addition to the metformin 1000 mg twice daily and amlodipine 10 mg daily treatments she received prior to PTC diagnosis. Postoperative serum Tg was above 300 ng/ml and anti-Tg was negative. Afterward, she was screened with unenhanced thoracic CT and skeletal scintigraphy. They revealed bilateral multiple nodules in her lungs and bone metastasis on T10 vertebra and right sacroiliac joint. Initially, 30 Gy radiotherapy was implemented to her T9–10 vertebrae for 12 days. We also started treating her with L-thyroxine to keep her thyrotropin (TSH) level below 0.1 mIU/L. After 2 months, she was treated with 200 mCi RAI for ablation. A RAI whole body scan (WBS) showed extensive RAI uptake in lungs and bones. A second 200 mCi RAI was applied 8 months after the first treatment. A post-ablative WBS showed progression. Serum Tg was still above 300 ng/ml and 200 mCi RAI administration was applied for the third time. A WBS was still displaying high radioactive activities in multiple areas of her body. Because of the existence of increased uptake, we planned a fourth RAI treatment but our patient was lost to follow-up for 2 years.\nWhen she presented again in 2015, her serum Tg was above 300 ng/ml again and a fourth 200 mCi RAI WBS of our patient was done. Unexpectedly, the WBS revealed diminished RAI uptake compared with the previous ones (Fig. ). However, a neck USG showed two solid thyroid nodules at the previous thyroid area and bilateral lung metastases were identified by thoracic CT. For the next 3 years, she was lost to follow-up, again. Finally, in February 2018, she was referred to our clinic and presented with a huge hemorrhagic draining cervical mass (Fig. ). Of interest, besides this finding, she did not have any other complaints other than a little dyspnea when lying down. Summing up all previous RAI treatments, cumulative 800 mCi RAI was given to her in the past 10 years; however, a physical examination and screening findings were not yet promising at the last follow-up (Fig. ). Eventually, considering her elderly age, harboring multiple metastases, and the absence of severe complaints, we planned radiotherapy to the giant mass on her neck. After applying radiotherapy, she was lost to follow-up again and at the end of the year her sons reported that she was dead. |
A previously healthy 42 years old male presented to our institute with history of gradually progressive and painless swelling over left calf since two months. He was a non-smoker, laborer by occupation. The patient noticed a firm swelling in calf region of left leg 5 years ago. No history of trauma or infection prior to the appearance of the mass was reported. No family history of any such swelling in the past. Patient was operated for swelling over calf region 20 years back but no records were available. There was a history of gradual increase in deformity of left foot since 3 years and the patient had started walking on toes on left side with no dorsiflexion at ankle joint.\nOn examination, there was a single, non-tender, hyperpigmented scar of size 8×3 cm over mid-calf region fixed to underlying structures. A large, well defined non-tender, firm, swelling was palpable in posterior aspect of left leg measuring about 28×8 cm extending from tendoachilles region up to 5 cm distal to popliteal fossa and medially and laterally up to border of tibia and fibula respectively. The overlying skin was normal with no discoloration and local raise of temperature. Movement of knee joint was normal. There was fixed equinus deformity of left foot (). No inflammatory signs, skin changes or adenopathies were present. No bruits were heard on auscultation. Neurovascular examination of left leg and foot was normal. Laboratory findings were within normal limits. Radiological examination revealed large soft tissue mass with linear and streak-like ossification around the left tibia. MR Angiography () showed arteriovenous malformation in left calf with multiple feeding arteries arising from popliteal, peroneal and anterior tibial artery and large draining veins draining deep into venous system of leg. The tibia and fibula marrow showed normal signal intensity.\nBecause of patient symptoms and with clinical diagnosis of a vascular malformation, a wide surgical excision of the lesion was done. Through a 25 cm longitudinal incision across the calf, posterior compartment muscles were exposed. The mass was found completely involving superficial group of posterior compartment muscles sparing the deep compartment with no attachment to periosteum or bone (). Peroneal artery and vein were found to be embedded in the lesion and thus sacrificed. Plane of dissection was between superficial and deep muscles.\nThe lesion was completely removed along with overlying cutaneous scar with wide surgical margins leaving posterior tibial artery in continuity. Intraoperative, complete dorsiflexion of foot was achieved with intact vascularity of leg. The excised specimen was very hard like bone and had to be cut longitudinally with saw (). Grossly the resected specimen showed ossified tissue covered with skin and soft tissues including muscle, tendons and adipose tissue measuring 15×7×5 cm. The cut surface of the ossified area was grey white, gritty and congested (). Microscopically, it revealed features of a vascular malformation with numerous blood vessels of variable size and shape composed of arteries and veins which were dissecting soft tissues and interstitial planes of skeletal muscle.\nMany of the vessels were thin walled with anastomosing and a sinusoidal appearance. Some of them showed fresh and organized thrombi within this vascular background, extensive osseous metaplasia characterized by mature lamellar bone formation was seen. (). The diagnosis was consistent with arterio-venous malformation with extensive osseous metaplasia. At the time of recent follow up after one year from the operation, no local recurrence of the tumor was demonstrated clinically and radiologically. No restriction of motion of ankle joint was found. Patient is presently walking with a normal gait. |
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