2 values
Kallmann Syndrome and X-linked Ichthyosis Caused by Translocation Between Chromosomes X and Y: A Case Report
A 20 year old male () was referred to the department of medical genetics of a tertiary care institute on the 16th of June, 2018. His chief complaints were delayed secondary sexual characteristics, impaired sense of smell, and poor scholastic performance. He was born of nonconsanguineous marriage. Family history was not significant. His weight was 60 kg (0 SD) and height was 151 cm (<−3 Z score). He had narrow palpebral fissures, eunuchoid appearance, gynecomastia, and sexual maturity rating corresponded to Tanner stage 3. His right testis was not identified in the scrotum or inguinal canal and left testicular volume was low (12 ml). Stretched penile length was 3.6 cm (<3rd centile). Mild ichthyosis on abdomen () and back was observed. He did not have any significant facial dysmorphism or any other malformation. Central nervous system examination revealed hyposmia, hoarse voice, and synkinesia of hands.\nEndocrinological evaluation was suggestive of hypogonadotrophic hypogonadism [FSH: 0.733 IU/L, LH: <0.1 IU/L (normal value: 1.7–8.6) and testosterone: 5.59 nmol/L (normal value: 8.6–29)]. Skeletal survey did not reveal any significant findings. Karyotype from peripheral blood lymphocytes was performed and an extra material on p arm of chromosome X was identified (46,Xp+,Y) (). DNA was extracted from peripheral blood and cytogenetic microarray was performed by Cytoscan 750 K array kit (Affymetrix, USA). A 8.3 Mb deletion on short arm of chromosome X {arr[hg19] Xp22.33p22.31(168,551–8,538,809)} including 37 OMIM genes and a 12.8 Mb duplication on long arm of chromosome Y {arr[hg19] Yq11.221q11.23(15,999,473–28,799,654)}including 25 OMIM genes were identified (). The breakpoint on X chromosome resulted in deletion of important and clinically relevant genes including ANOS1 (KAL1) gene (7th–14th exon), STS gene, and 4 other genes including SHOX, ARSL (ARSE), NLGN4X, and VCX (). The duplicated Y segment contained only one clinically important gene namely, DAZ1. Duplication of DAZ1 has not been implicated in any disease causation. Parental blood samples were not available to identify the inherited characteristics of this chromosomal aberration. However, both parents were clinically normal and fertile.
[[20.0, 'year']]
{'7677154': 1, '1302031': 1, '8230160': 1, '745219': 1, '30352392': 1, '4126829': 1, '15772651': 1, '8168818': 1, '17591464': 1, '9713559': 1, '28253503': 1, '23091330': 2, '2602357': 1, '34987993': 2}
{'3468769-1': 1}
Reporting of pneumothorax in association with vaping devices and electronic cigarettes
In October 2019, a 34-four-year-old white male presented to his general practitioner with a 1-day history of back pain, shortness of breath, tightness in his chest, burning pain in his right chest, retrosternal pain and feeling dizzy. Symptoms started after minor exertion during the afternoon on the day before presentation.\nNo specific health problems occurred during adolescence. His previous medical history was otherwise unremarkable apart from a few rib fractures on his right side sustained through a cycling accident 3 years ago. There was no family history of lung disease. As a trained nurse, the patient worked in outpatient palliative care and devoted his free time to strength training and competitive sports. Due to his occupation, he had regular health checks, all negative for infectious diseases. He had no recent sports trauma to his chest. Despite being an active and internationally successful athlete, he smoked 20 cigarettes per day from the age of 21 years to the age of 31 years. Three years ago, he quit smoking by switching to vaping nicotine. He never smoked or vaped cannabis. He reported a pattern of regular deep and slow inhalation with the vaping device throughout the day similar to smoking a pack of cigarettes.\nOn physical examination, we saw an athletic male with normal body mass index (BMI 23.6, 184 cm body height and 80 kg body weight). There were no signs of chronic lung disease such as finger clubbing, chronic cough, wheezing or sputum production. Neurology examination was unremarkable. During the patient’s initial visit to his general practitioner, vitals included a heart rate of 72 beats per minute, blood pressure of 143/91 mm Hg, peripheral oxygen saturation without air supply of 98% and a respiratory frequency of 15 per minute. His ECG demonstrated signs suggestive of myocarditis. The patient arrived at our emergency department 2 hours later with normal peripheral oxygen saturation of 98%, but increasing tachypnoea with 24 breaths per minute. His heart rate and blood pressure remained normal, and there was no enlarged jugular vein pulse or cyanosis. Respiratory frequency returned to normal rates with 3 L of nasal air flow supply. ECG demonstrated again changes in V3–V5 suggestive of myocarditis (). His skin was dry and warm on palpation with a temperature of 36.8°C, and his tongue was of healthy appearance. His heart sounds were regular on auscultation and breathing sounds were absent on the right side of his chest. Percussion revealed tympanitic chest sounds on the right. The patient complained of acute pain in his chest.\nHis chest X-ray demonstrated a large (according to Collins formula) right-sided pneumothorax () and a small right-sided pleural effusion. Immediate insertion of a right-sided chest tube led to pain relief and normalisation of the ECG.\nDiagnostic and therapeutic approaches were discussed with the patient, and a decision was made for a CT thorax scan. The CT scan demonstrated a bilateral inflated lung, the inserted chest tube on the right side, apical bleps of approximately 0.5–1.0 cm in diameter and a small stripe of remaining apical pneumothorax. Blood tests did not reveal any inflammation or other abnormal findings. After successful removal of the chest tube, the patient was discharged and returned for follow-up examination 1 week later in our outpatient department. During the visit, the patient stated his desire to proceed with bullae resection. Being active in martial arts and internationally successful, he feared of another episode of a pneumothorax leading to a further delay in his training.\nThe patient underwent video-assisted thoracoscopic surgery (VATS) resection of the apical bullae and partial apical pleurectomy. Intraoperatively, the apical bullae and a minor clear fluid collection could be demonstrated with no other visible lung pathology. The resection specimen revealed the site of ruptured bullae. The patient made an uneventful recovery. Perioperatively, the patient received routine tyloxapol inhalations four times daily. Standard analgesia contained oral metamizole, ibuprofen and oxycodone/naloxone.
[[4.0, 'year']]
{'27413679': 1, '29609132': 1, '32442559': 1, '32961169': 1, '31826344': 1, '28605480': 1, '29046158': 1, '33778558': 1, '31705470': 1, '33588508': 1, '31753841': 1, '28529185': 1, '32145818': 1, '31827996': 2, '31600764': 1, '30420278': 1, '28983782': 1, '32230711': 1, '7572489': 1, '31725708': 1, '28458928': 2, '28167512': 1, '24155002': 1, '31990264': 1, '25863521': 1, '31495337': 2, '32082682': 2, '32505323': 1, '32970368': 1, '32553826': 1, '34920999': 2}
{'7013298-1': 1, '7013298-2': 1, '7013298-3': 1, '7013298-4': 1, '7013298-5': 1, '6890157-1': 1, '5385216-1': 1, '6732835-1': 1}
Novel walking pole gait pattern improves activity in an older adult with chronic low back pain
The participant was a 65-year-old woman, who had been diagnosed with lumbar spondylolisthesis at age 33. At age 51, she was injured in a motor vehicle crash that left her unable to ambulate in the community without exacerbation of her CLBP and right lower extremity radicular symptoms. The participant had previously undertaken pain interventions such as physical therapy, chiropractic and prolotherapy, but as these interventions did not provide long-term pain relief, she discontinued them. However, on a weekly basis, she received therapeutic massage and participated in Tai Chi classes. The participant also consumed an over-the-counter, non-steroidal anti-inflammatory drug as needed. The participant did not use any prescription analgesics.
[[65.0, 'year']]
{'31210680': 1, '8555924': 1, '25488399': 1, '15928561': 1, '23446066': 1, '26305413': 1, '22135712': 1, '25599309': 1, '20146793': 1, '27131178': 1, '21603199': 1, '5523831': 1, '26742533': 1, '25675896': 1, '10201543': 1, '28756746': 1, '31666220': 1, '27990219': 1, '34920998': 2}
Giant dural arteriovenous fistula in an infant
A one-month-old female presented with irritability and failure to thrive. She was the product of a 35-week pregnancy and was delivered vaginally without complications or a difficult labor. Prenatal ultrasonography was not performed. Her Apgar score was 7. The parents reported failure to thrive and an infant who was minimally responsive although she would open her eyes and was able to follow objects. Her pupils were more or less symmetrical. Blood chemistry panels were within normal limits. On physical exam, the infant moved all extremities to stimulation and grimaced to pinch. The anterior fontanelle was full and tense and the head circumference was 42 cm. The sutures of the calvaria were split. There was an upgaze palsy. Reflexes were found to be hypotonic. No cutaneous stigmata were identified.\nA subsequent magnetic resonance imaging (MRI) identified a large, somewhat spherical mass with significant flow void filling most of the posterior cranial fossa and extending superiorly to the underside of the calvaria (). In total, the mass filled approximately one half of the intracranial volume. No cerebellum was identified and the brainstem was significantly compressed against the clivus (). Hydrocephalus was present, especially involving the temporal horns (). The internal jugular veins were dilated () and the circle of Willis and vertebrobasilar vessels appeared normal () although the resolution of the imaging was poor primarily due to the age of the patient, therefore, based on the findings of MRI, the diagnosis of a giant dAVF involving the transverse-sigmoid sinuses was made. The patient was scheduled for an arteriogram but died before the procedure could be performed.
[[1.0, 'month']]
{'9541321': 1, '28432263': 1, '8683257': 1, '30588182': 1, '22537121': 1, '18628973': 1, '24626343': 1, '7815143': 1, '7862961': 1, '11409515': 1, '28818827': 1, '32753437': 1, '34657838': 2}
Incidentally detected atrioventricular septal defect in an adult
A 34-year-old woman who had planned to be a liver transplantation donor for her son was referred to our hospital for preoperative evaluation. She had no symptoms and had an unremarkable medical history. She had undergone a successful vaginal delivery. Her vital signs were stable upon admission. Cardiac auscultation revealed a grade 3/6 systolic ejection murmur at the second left sternal border and fixed splitting of the second heart sound. Twelve-lead electrocardiography showed sinus rhythm, left axis deviation, and an incomplete right bundle branch block pattern (Figure ). Two-dimensional echocardiography revealed preserved left ventricular contractility, although both atria and right ventricle were dilated. Moreover, there was a defect in the lower part of the atrial septum and a restrictive ventricular septal defect (VSD). Color Doppler echocardiography showed a left-to-right atrial shunt, mild-to-moderate left atrioventricular valve (LAVV) regurgitation, and moderate right atrioventricular valve (RAVV) regurgitation (Figure ). Cardiac catheterization revealed a gooseneck deformity (Figure ). Right-heart catheterization showed a mean pulmonary artery pressure of 16 mmHg and a mean pulmonary capillary wedge pressure of 10 mmHg. The pulmonary-to-systemic flow ratio was 2.08.\nShe was diagnosed with an atrioventricular septal defect. Even though she had no symptoms, she was deemed unfit to be a liver transplant donor, and she was advised to undergo AVSD repair. Median sternotomy, cardiopulmonary bypass, and aortic cross-clamping were performed as usual. We observed a defect in the lower portion of the atrial septum, a small VSD surrounded by fibrous tissue, and a cleft of the LAVV and RAVV (Figure ; white and black arrows indicate a cleft of the LAVV and VSD, respectively). It was diagnosed as an intermediate-type AVSD. We first sutured the pericardium (white arrows) to the junction of the RAVV and LAVV annuli (Figure ). Suturing at the area of the atrioventricular node and coronary sinus was performed very close to the LAVV. Next, the LAVV cleft was closed with interrupted sutures, and edge-to-edge repair was performed to address the RAVV regurgitation.\nPostoperatively, two-dimensional echocardiography showed no leakage around the pericardial patch. Both RAVV and LAVV regurgitations were trivial (Figure ). The patient's postoperative course was uneventful.
[[34.0, 'year']]
{'16631698': 1, '10197679': 1, '31186038': 1, '31008039': 2, '15172287': 1, '29069357': 1, '34987807': 2}
{'6450236-1': 1}
Laparoscopic cholecystectomy for gallbladder torsion in a 3-year-old child
A 3-year-old previously healthy boy was referred to an emergency medical center because of sudden abdominal pain and vomiting. Although he was diagnosed with acute gastroenteritis and prescribed medication, he continued to vomit several times, and his general condition worsened. Therefore, he was referred to our hospital the day after the onset of abdominal pain. Physical examination showed a flat and soft abdominal wall with mild rebound tenderness in the right hypochondrium and no palpable abdominal mass. Laboratory examinations showed a slightly elevated white blood cell (WBC) count of 16,500/μL, while C-reactive protein (0.25 mg/dL) and serum levels of liver enzymes were all within the normal ranges. Abdominal ultrasonography (AUS) revealed gallbladder wall thickness without gallstones. Additionally, a small amount of fluid was observed around the gallbladder and in the pelvic cavity. Because GT was not considered a differential diagnosis at the time of admission, he was admitted to the Department of Pediatrics, and treatment with antibiotics was started with a diagnosis of acute cholecystitis. Simultaneously, examinations for underlying disorders related to cholecystitis were planned; vasculitis, such as immunoglobulin A vasculitis or hemolytic anemia, were ruled out as a result. On day 1 of admission, the WBC count decreased to 12,500 /μl, and his abdominal pain slightly improved. Abdominal enhanced computed tomography (CT) showed swelling of the gallbladder with ascites, as did the AUS images from the day of admission (Fig. ). On day 2 of admission, due to the re-elevated WBC count of 16,000/μl, C-reactive protein level of 3.26 mg/dL, and persistent abdominal pain, a repeat AUS was carried out. An increase in the gallbladder wall thickness to 12 mm and a lack of intramural blood flow was noted (Fig. ), which led to a diagnosis of GT. The patient was referred to our department, and an emergency LC was carried out using the VISERA ELITE system (Olympus). A 12-mm trocar was inserted at the umbilical portion of a flexible laparoscope (LTF-S190-10; Olympus), followed by placement of three 5-mm ports, and the rest of the operation was carried out according to the standard procedure used in adult patients. The gallbladder was blackish, with severe edema, and surrounded by bloody ascites. After resection of the adhesion between the gallbladder and the greater omentum, the gallbladder was observed to be twisted 270° counterclockwise at the level of the cystic duct and mesentery (Fig. ). The gallbladder was easily untwisted clockwise to its natural position, which was classified as type I according to the Gross classification (Fig. ). After resecting a small amount of mesentery between the cystic duct and the liver bed, the cystic duct with the cystic artery was doubly ligated using Endoloop polydioxanone suture II and resected. The operation time was 104 min, and intraoperative blood loss was minimal. Microscopically, the resected specimen showed gangrenous cholecystitis without gallstones. Notably, the gallbladder bile culture was negative. The postoperative course was uneventful without any complications, and the patient was discharged on postoperative day 6.
[[3.0, 'year']]
{'19858599': 1, '20610259': 1, '23925030': 1, '18373471': 1, '20593170': 1, '29052346': 1, '19793492': 1, '8014819': 1, '32939742': 1, '34987833': 2}
Multiple fibro-osseous lesions of the jaws: A report of a rare case with a literature review
A 14-year-old girl from eastern Nepal reported to the authors' clinic with progressive facial disfigurement in the past 3 years due to swelling in the left maxilla. No history of associated trauma or systemic or local infectious conditions was elicited. The associated symptoms of left nasal stuffiness and difficulty in breathing were noted starting 6 months ago. The patient's family and medical history were non-contributory. Upon physical examination, a solitary diffuse, non-tender, bony hard swelling measuring 4×3 cm was noted in the left maxilla. An intraoral examination revealed a swelling in the left maxillary alveolar region that was non-tender and bony-hard on palpation (). The adjacent dentition and oral mucosa showed no abnormalities. A provisional diagnosis of fibrous dysplasia was given, with ossifying fibroma, central giant cell granuloma, and benign odontogenic tumour considered as differential diagnoses.\nPanoramic radiography revealed radiopaque discrete lesions in all 4 quadrants with a peripheral radiolucent rim. The right maxillary alveolus presented with a smaller lesion measuring 1×2 cm that raised the floor of the maxillary sinus in a dome-shaped manner. The mandibular lesions were localized on both the right and left second premolar to first molar regions, and measured 3×2 cm. The most radiopaque area was located centrally, particularly in the mandibular lesions. The lesions showed displacement of the adjacent tooth roots, the mandibular canal, and maxillary sinus floor (). A maxillary occlusal topographic radiograph revealed an expansile radiopaque lesion measuring 4×3 cm extending to the mid-palatine suture in the left maxillary quadrant (). A lateral oblique radiograph of the left mandibular body showed a radiopaque lesion with a peripheral radiolucent rim (). Computed tomography (CT) coronal and axial images revealed expansile osteogenic lesions in the 4 quadrants (), with the lesion in the left maxilla being the largest. No evident breach was noted in the antral walls except for the left lateral nasal wall. Radiographic diagnoses of fibrous dysplasia, ossifying fibroma, and osseous dysplasia including florid osseous dysplasia were considered.\nThe patient was advised to receive an evaluation of serum calcium and alkaline phosphatase levels along with routine hematological investigations, which were within the normal limits. Biopsies of the maxillary lesions and both mandibular lesions were performed under local anaesthesia. The histological examination revealed predominant fibrous tissue with ossifications and cementum-like material suggestive of fibro-osseous lesions. The presence of cementum-like material and ossifications strongly suggested ossifying fibromas (). Surgery was then planned in a phased manner. Considering the age of the patient, a conservative approach with aggressive curettage was performed under general anaesthesia. The most extensive and symptomatic of the lesions in the left and right maxilla were aggressively curetted after obtaining bony access into the maxillary sinus by intraoral approaches on 2 separate occasions.\nOn gross appearance, the left maxillary lesion was friable and obtained as multiple fragmented bits of fibrous tissue (). Histopathology with routine haematoxylin and eosin staining showed a predominantly fibrous stroma with plump spindle-shaped fibroblasts arranged in whorled patterns (). Areas of ossification and irregularly shaped immature cellular bony trabeculae representing woven bone were noted dispersed throughout the lesion. Some areas of spherical eosinophilic calcifications with basophilic concentric reversal lines, suggestive of psammoma bodies, were noted. The histological features were strongly suggestive of ossifying fibroma.\nThe patient remained asymptomatic for 2 years of follow-up. However, she was reluctant to undergo further surgery for the mandibular lesions or any further investigations.
[[14.0, 'year']]
{'20614314': 1, '3112269': 1, '11815929': 1, '9394387': 1, '2594325': 1, '22567440': 2, '16458039': 1, '15292873': 1, '11681486': 1, '2594324': 1, '15141726': 1, '20026707': 1, '34988008': 2}
{'3335520-1': 1}
An unusual presentation of osteoblastoma of the maxilla: A case report
A 17-year-old Hispanic female was admitted to the Stony Brook University School of Dental Medicine for an emergency visit with a chief complaint of dull pain of the left posterior maxilla. Physical examination found a bony swelling located buccal to the left maxillary molars. No erythema, edema, or purulent discharge was noted. Panoramic radiography revealed a moderately defined granular radiopaque area in the periapical region of the left maxillary molars with superior displacement of the sinus floor (). No peripheral radiolucent rim was noted on the panoramic radiograph. For further investigation of the lesion, bone algorithm multidetector computed tomography(MDCT) was performed. The MDCT images also revealed a moderately defined granular hyper-attenuating area in the region of the left maxillary molars. Superior displacement of the left maxillary sinus floor, supero-posterior displacement of the left maxillary third molar, and external apical root resorption of the first and second molars were also appreciated. The entity was confluent with the lamina dura of the affected teeth; however, the periodontal ligament space remained intact. Of note were a thin hypo-attenuating rim, partially surrounding the central portion of the lesion at the antero-medial periphery, and a small area of possible buccal dehiscence adjacent to the left maxillary second molar (). Based on clinical and radiographic features, a preliminary differential diagnosis of osteoblastoma, fibrous dysplasia and cemento-ossifying fibroma was considered. Although unlikely, a remote possibility of osteosarcoma was considered as well. The patient, however, failed to attend her follow-up appointment for further examination.\nThe patient was then referred back to the authors' institution from a private oral and maxillofacial surgery office 13 months after the initial visit. An incisional biopsy had been performed by the referring physician and a histopathologic examination was done prior to readmittance. A histopathologic examination of the specimen revealed well-vascularized fibrous connective tissue containing a tumor composed of osteoid trabeculae surrounded by plump osteoblasts and scattered osteoclasts (). Osteoblast bridges between the bone trabeculae in several areas were also noted. Based on these features, a histolpathologic diagnosis of osteoblastoma was made.\nComplete excision of the tumor was performed via left partial maxillectomy with localized mucoperiosteal advancement flap under general anesthesia (). A histopathologic examination of the surgical specimen showed the histopathologic features of the previous incisional biopsy, and final diagnosis of osteoblastoma was made. The postoperative course was uneventful other than postoperative pain and nasal congestion, which eventually resolved by 4 weeks postoperatively. The patient was last seen 1 year after surgery and showed no sign of recurrence on both clinical and radiologic examinations ().
[[17.0, 'year']]
{'9879220': 1, '17091775': 1, '25663319': 1, '2755691': 1, '27574436': 2, '20737258': 1, '10455381': 1, '13413389': 1, '23604054': 1, '22776732': 1, '17052641': 1, '23459840': 1, '20012077': 1, '18561158': 1, '14697371': 1, '28468168': 1, '22052644': 1, '7823298': 1, '34988007': 2}
{'4993416-1': 1, '4993416-2': 1, '4993416-3': 1, '4993416-4': 1}
Recurrent benign cementoblastoma: A case report and literature review
A 16-year-old male visited the Department of Oral and Maxillofacial Surgery of Kyungpook National University Dental Hospital with pain in the right mandible while chewing. The patient sensed pain on palpation of the gingiva near the mandibular right first molar. All the teeth in the affected area were caries-free. The overlying mucosa appeared normal, with no clinical signs of inflammation. The patient reported that the symptoms had begun several months ago. The patient had no significant family or medical history contributing to pain.\nAn initial panoramic radiograph (Orthopantomograph OP 200 D, Instrumentarium Imaging, Tuusula, Finland) showed a round radiopaque mass with mixed radiodensity surrounded by a radiolucent rim. The lesion was attached to the root of the right first molar and caused displacement of the inferior alveolar canal (). The lesion extended to the periapical area of the mandibular right second molar. Cone-beam computed tomography (CBCT) images (PaX-Flex 3D apparatus, Vatech, Kyungi, Korea) demonstrated a well-circumscribed mass involving the roots of the mandibular right first molar, causing perforation of lingual cortical bone with sclerosing osteitis around the lesion (). We established a differential diagnosis of benign cementoblastoma with secondary infection based on the clinical and radiographic findings. Under general anesthesia, the patient underwent surgical excision of the lesion with extraction of the mandibular right first and second molars (). During surgery, the mass and the involved tooth were well separated. The defect was immediately filled with bone graft material () and the specimen was submitted for a histopathological examination. Macroscopically, the surgical specimen consisted of a round hard tissue mass in conjunction with the first molar roots. Hematoxylin and eosinstained sections showed that the lesion included sheets of cementum-like tissue with basophilic reversal lines surrounded by well-vascularized cellular connective tissue, with large and abundant cementoblasts and irregular lacunae (). The final diagnosis of benign cementoblastoma was established based on the histopathologic examination. Postoperative healing was uneventful.\nRoutine post-surgical follow-up examinations revealed a new lesion at the age of 20 years on the previous operation site. The clinical symptoms included intermittent pain in the right mandible during physical activity. Three years after the initial surgery, a panoramic radiograph showed masses with heterogeneous radiopacity surrounded by radiolucent rims with sclerotic borders at the previous operation site, suggesting recurrence of the lesion (). CBCT showed perforation and expansion of the buccolingual cortical bone (). The patient underwent a reoperation on the mandibular right posterior region. Under general anesthesia, the patient underwent surgical curettage of the lesion, mandibular right second premolar extraction, and grafting with the left iliac bone. The gross specimen included multiple cemental masses near the operation site (). Areas of cementum-like calcified trabecular tissue with benign cementoblastoma and multinucleated giant cells were present along the loosely arranged vascular connective tissue stroma (). Histopathological examination confirmed the diagnosis of recurrent benign cementoblastoma. Six months after the second surgery, there was no further sign of recurrence at a regular follow-up visit ().
[[16.0, 'year']]
{'15533983': 1, '26730374': 1, '1890666': 1, '17238975': 1, '17699708': 1, '8108097': 1, '11203925': 1, '30510344': 2, '28869132': 1, '3482232': 1, '11925541': 1, '17884542': 1, '34988006': 2}
{'6230933-1': 1}
Internal jugular phlebectasia in pediatric patients: a report of two Saudi cases
The first case involved an 8-year-old boy who presented to our clinic with recurrent tonsillitis and was booked for tonsillectomy. He had no contributive medical history. He was found to have an incidental midline neck mass during the evaluation (); this mass was found since the age of eight months. His parents denied any breathing or feeding difficulties. The mass increased with coughing and the valsalva maneuver. The mass was more toward to the right of the neck, and it was soft and compressible with no skin change.\nImaging examination of the patient was done by performing CT and US. US Doppler showed a right dilated internal jugular vein (). The CT scan confirmed this finding, which suggested internal jugular vein phlebectasia ().
[[8.0, 'year']]
{'28474146': 1, '10626865': 1, '7870454': 1, '9051432': 1, '34987755': 2}
{'8711263-2': 2}
Internal jugular phlebectasia in pediatric patients: a report of two Saudi cases
The second case involved a 5-year-old boy scheduled for tonsillectomy and was found to have a midline neck mass several years ago. The mass increased with coughing and valsalva maneuver. He had no contributive past medical history. He had no shortness of breath, dysphagia or snoring. The patient had recurrent tonsillitis and was found to have an incidental midline neck mass during the evaluation.\nOn examination, the midline mass was more toward to the right compressible neck mass. The mass was soft, with no skin change ().\nImaging examination of the patient was done by performing CT with contrast and US. US Doppler showed a right dilated internal jugular vein during coughing and the valsalva maneuver (). CT showed a wide right internal jugular vein (). Both cases managed conservatively with follow-up and no surgical intervention as they did not have any complication or cosmetic concerns.
[[5.0, 'year']]
{'28474146': 1, '10626865': 1, '7870454': 1, '9051432': 1, '34987755': 2}
{'8711263-1': 2}
Case report of combined iliac crest and ASIS apophysis avulsion fracture in an adolescent footballer
A 15-year-old boy presented to our emergency department unable to weight-bear and complaining of severe right hip pain following a non-contact injury while playing football. He described a sudden onset of right hip and groin pain when he over-stretched for a ball with his right leg; his trunk rotated, hip flexed, knee fully extended and his foot internally rotated. He was unable to mobilize or weight-bear and required an ambulance to present to our institution. On physical examination, he had pain on all hip movements with limited hip flexion and internal rotation. There was bony tenderness on palpation along the anterior aspect of the hip and IC. Sensation within the dermatome of the lateral cutaneous femoral nerve (LCFN) was intact.\nThe anterior–posterior plain film X-Ray of pelvis demonstrated an avulsion fracture of the right ASIS and IC apophysis with 1.5 cm displacement (). Lateral view of the hip showed no extension into the joint. There was no radiological evidence of chronic overuse or previous injury. Computed tomography (CT) imaging was performed to better identify the injury; 3D volume reconstruction clearly demonstrated the unique fracture pattern of an ASIS avulsion in addition to an avulsion of the distal iliac apophysis extending ~5 cm with the described displacement (–).\nAfter discussion of the surgical risks of fixation, including hardware irritation and LCFN dysfunction, a shared decision to treat the patient conservatively was reached. The patient was instructed to mobilize with crutches and attempt weight-bearing as comfort allowed. At a 4-week follow-up review, the patient presented with complete resolution of symptoms, full active hip range of motion, nil palpable tenderness and the ability to mobilize independently without walking aids. Radiographs performed showed significant callous formation between the pelvis and the fragment (). Return to sport was achieved by 8 weeks as per medical advice following a period of closed-chain exercises and self-directed rehabilitation.
[[15.0, 'year']]
{'19226032': 1, '28291142': 1, '18382943': 1, '23015945': 1, '25321882': 1, '24246879': 1, '21577152': 1, '10843512': 1, '34987761': 2}
Bilateral papillary thyroid cancer with normal aberrant thyroid tissue
A 43-year-old man presented in 2021, with bilateral thyroid nodules detected by ultra-sonography 5 months earlier. He had no night sweats, no respiratory or dysphagia difficulty, nor other pressure symptoms. An ultrasound scan interpreted nodules in left and right thyroid gland, which were irregular defined, low-echo with calcification. In addition, there was a mass measured about 28 × 5 × 20 mm in front of internal jugular vein. Fine needle aspiration biopsy was advised and histological investigation showed bilateral papillary thyroid cancer. Therefore, total thyroidectomy was recommended.\nThe patient was hospitalized in 22 March 2021. The level of thyroglobulin was as high as 230.80 ng/ml, whereas all other thyroid functional indicators were normal. Chest and abdominal examination as well as preoperative fiberoptic laryngoscopy were all normal. He underwent contrast-enhanced cervical computed tomography (CT) scan, which confirmed bilateral possible malignant nodularity in thyroid gland and an ~1.6 × 0.6-cm hyperdensity deep to the right sternocleidomastoid muscle (SCM, ). Total thyroidectomy and bilateral central neck dissection was operated. A metastatic lymph node encapsulated the left recurrent laryngeal nerve. In the same time, the mass lateral to the right SCM was removed, which was separated from the thyroid gland (). Intraoperative neuromonitoring information was normal. Pathology demonstrated bilateral papillary thyroid cancer (the size of cancer in left lobe was 2.2 cm, right 1.6 cm) with three lymph node metastases. However, the aberrant thyroid was histological free of cancer. Postoperative radioactive iodine therapy (RAI) was recommended for this patient.
[[43.0, 'year']]
{'29697084': 1, '32257713': 2, '34987763': 2}
{'7117606-1': 1}
Distal partial gastrectomy for gastric tube cancer with intraoperative blood flow evaluation using indocyanine green fluorescence
An 80-year-old man developed gastric tube adenocarcinoma after radical esophagectomy for squamous cell carcinoma. He underwent video-assisted subtotal esophagectomy followed by reconstruction of the gastric tube through the retrosternal route and cervical anastomosis 7 years ago. Based on his age and co-morbidities, such as interstitial pneumonia, paroxysmal atrial fibrillation, aortic regurgitation and hypertension, he has been followed up by regular check-ups without adjuvant therapy.\nUpper gastrointestinal endoscopy at the seventh year of follow-up revealed a polypoid tumor of 3 cm in diameter at the pyloric region of the gastric tube with pyloric obstruction (). Endoscopic biopsy from the tumor revealed papillary adenocarcinoma (pap > tub1). Contrast-enhanced computed tomography (CT) demonstrated a solid mass with slight enhancement at the abdominal part of the gastric tube located below the lower border of the sternum (). Neither lymph node metastasis nor distant metastasis was detected, leading to a diagnosis of clinical stage IB (cT2N0M0) gastric cancer according to the seventh edition of the Union for International Cancer Control system.\nTGTR with sternotomy was considered a radical surgery; however, in consideration of surgical invasion and the patient’s physical condition, gastric vessel-preserving partial distal gastrectomy was conducted.\nUnder upper midline laparotomy, the abdominal part of the gastric tube was mobilized along with the RGEA and RGA after careful dissection. The first and second branches of the RGEA and RGA were divided along the gastric wall, carefully preserving the main vessels. Supra-duodenal arteries were also divided. Then, the anal side margin was set at 1 cm from the distal side of the pylorus, and the duodenum was divided using a linear stapler (). Before setting the proximal surgical margin, blood supply in the gastric tube was assessed by ICG fluorescence []. The visualized ICG fluorescence demonstrated an irregular demarcation line at the distal side of the preserved gastric tube, indicating insufficient blood supply at the greater and lesser curvature compared with the center of the gastric wall (). The gastric tube was thus divided at 2 cm on the oral side from the proximal margin of the tumor, confirming complete removal of the ischemic area. Then gastro-jejunostomy was performed for Roux-en-Y reconstruction ().\nThe surgery was uneventful. The operation time was 3 h 49 min, and the amount of blood loss was 60 g. The patient fully recovered and was discharged 16 days after surgery.\nThe resected specimens are shown in . Pathological examination of the surgical specimen demonstrated moderately differentiated adenocarcinoma (pT2), indicating R0 resection. The patient is healthy at 26 months after surgery without recurrence.
[[80.0, 'year']]
{'33024514': 2, '31125790': 1, '18379851': 1, '22331794': 1, '22557942': 1, '32480340': 1, '29951985': 1, '30275750': 1, '32337608': 2, '12022598': 1, '34987762': 2}
{'7520569-1': 1, '7183568-1': 1}
Ectopic uterus? A rare presentation of a giant leiomyoma in the posterior mediastinum
In March 2021, a 17-year-old female from Kilimanjaro region presented with a 3-month history of gradually progressive left-sided chest pain associated with cough, difficulty in breathing and chest tightness. The pain was worse on lying on the affected side. No history of hemoptysis, fever, night sweats or weight loss, neither exertional dyspnea nor orthopnea reported. No contact with tuberculosis reported. No significant past medical history. She does not use tobacco or alcohol. Upon examination, she was comfortable at rest and saturating at 99% in room air. Her axillary temperature, pulse rate and blood pressure were 36.4°C, 100 beats per minute and 116/79 mmHg, respectively. On chest examination, trachea was deviated to the right and there was reduced air entry on the left side. Apex beat was at fifth intercostal space mid-clavicular line and s1 and s2 sounds were heard with no added sounds. Other systems were unremarkable. Her hemoglobin level was 12.9 g/dl; serum creatinine of 72 μmol/l; prothrombin time and international normalization ratio were 11.3 and 0.89 seconds, respectively. Serum electrolytes were within normal range.\nAn initial chest radiograph of the patient confirmed tracheal deviation and revealed a mass in the left hemithorax. Chest CT scan () showed a large mass occupying the entire left posterior hemithorax measuring 12.3 cm (AP) × 14.4 cm (T) × 22.8 cm (CC) with associated partial collapse of the left lung and mediastinal shift to the contralateral side suggestive of pleuropulmonary blastoma vs. neurogenic tumor (ganglioneuroma). This was followed by a CT-guided core needle biopsy which revealed benign spindle cells on a myxoid background. Abdominopelvic ultrasound was normal.\nWe performed a left-sided sixth-interspace thoracotomy. Intra-operatively, we found a huge, firm mass in the posterior mediastinum arising from the parietal pleura adjacent to thoracic vertebrae and fixed to the posterior ribs and intercostal muscles. The mass was in close proximity to the descending aorta from which it had one main feeder vessel. The left lung was collapsed yet normal. The tumor was excised and weighed 2.4 kg. The specimen () was submitted for histopathology examination ( and ). The left lung was inflated and expanded normally.\nPost-operatively, the patient was monitored in ICU and kept on parenteral ceftriaxone 1 g and gentamicin 80 mg 12-hourly, paracetamol 1 g 8-hourly and pethidine 25 mg 6-hourly. She was extubated on post-operative Day 1 and transferred to general ward on the second day. The patient was discharged on 9th post-operative day. She was followed up at the surgical outpatient clinic at 2, 4 and 6 weeks post-operation where she reported full recovery and had normal findings on physical examination and chest X-ray ().
[[17.0, 'year']]
{'11373202': 1, '31521591': 1, '19001649': 1, '27885214': 1, '29607199': 1, '19121206': 2, '34987767': 2}
{'2631530-1': 1}
Right ventricular dominant myocarditis requiring cardiac resynchronization therapy-defibrillator: a case report
A 43-year-old Japanese woman visited a local hospital with a 3 week history of chest discomfort and subsequent dyspnoea, nausea, and epigastralgia. She was previously healthy, had no medical or surgical history, took no regular medications, had no allergies, and had no history of smoking or drinking. Her left ventricular ejection fraction (LVEF) was preserved at 55% on admission; however, she presented with ST elevation in a wide range of leads, troponin T elevation, and pericardial effusion on computed tomography (CT) scan. On the day of her hospitalization, the patient suddenly collapsed from sustained ventricular tachycardia (VT). VT was first terminated through cardioversion, but soon recurred. VTs were refractory despite repeated cardioversions; thus, the patient was transferred to the angiography room and inserted a peripheral veno-arterial extracorporeal membrane oxygenation (VA-ECMO) circuit under cardiopulmonary resuscitation. VT was controlled by the next cardioversion after starting VA-ECMO, intra-aortic balloon pump (IABP), and continuous amiodarone infusion. RV pacing was started against a complete atrioventricular block (CAVB) after VT termination. Coronary angiography showed no significant stenosis. Bilateral ventriculography revealed RV diffuse systolic dysfunction, whereas left ventricular (LV) function was preserved (LVEF 50%). However, on the following morning, LV function was extremely deteriorated (LVEF 20%), and the aortic valve temporarily closed due to extremely impaired left ventricular function. Therefore, she was transferred to Chiba University Hospital via an air ambulance for evaluation regarding treatment with a ventricular assist device.\nOn arrival at Chiba University Hospital, the patient was conscious and alert with the support of VA-ECMO and IABP with sustained VT (Figure\n). After the VT was spontaneously terminated, CAVB was present. An electrocardiogram revealed CAVB with ventricular pacing beats and ST elevation in leads III, aVF, and V1 through V4 (Figure\n). The blood pressure was 84/50 mmHg under stable circulation, whereas heartbeat depended on ventricular pacing with a high pacing threshold. Chest radiography revealed a mildly enlarged cardiac silhouette (cardiothoracic ratio, 60%) without vascular redistribution (Figure\n). Transthoracic echocardiography indicated diffusely reduced biventricular wall motion (LVEF 14%) with diffuse myocardial oedema in the LV [interventricular septum (IVS) thickness: 13 mm, LV posterior wall thickness: 12 mm] without significant valvular disease (Figure\n). Plasma brain natriuretic peptide level was 11.9 pg/mL, serum creatinine was 0.41 mg/dL, and C-reactive protein was 1.75 mg/dL. Rheumatoid and antinuclear antibodies were negative. Endomyocardial biopsy (EMB) from the RV-IVS on the day of transfer demonstrated a complete degeneration of cardiomyocytes with massive infiltration of lymphocytes (Figure\n). She was pathologically diagnosed with lymphocytic myocarditis, although specific viral antibodies were not detected with paired serum examination.\nAs LV function gradually improved, the pacing threshold was lowered. However, CAVB persisted and VTs were easily induced once RV pacing was turned off. Finally, VA-ECMO was successfully weaned off on the 8th hospital day, and IABP was removed on the 11th day. RV temporary pacing was stopped on Day 12 because the heart rate was maintained higher than 70 b.p.m. as ventricular escape rhythm and VT had not recurred.\nEchocardiography on Day 19 showed RV dilatation with akinesis and high echogenicity in the IVS and RV apex, severe tricuspid regurgitation (TR) with valve separation, and thrombus in the RV apex, whereas LVEF improved to 59% (Figure\n). An oral anticoagulant was initiated, which was switched from continuous intravenous heparin. A diuretic was added due to the presentation of right-sided heart failure with concomitant oedema of the extremities, and an angiotensin II receptor blocker was initiated to prevent LV remodelling. On Day 20, contrast-enhanced CT scans indicated pulmonary thromboembolism (PE) along with a thrombus in the RV (Figure\n) and a deep vein thrombosis. Cardiac magnetic resonance (CMR) on Day 30 showed a wide range of late gadolinium enhancement (LGE), including at the RV apex to free wall, IVS, and LV apex (Figure\n). After we confirmed disappearance of the RV thrombus via CT scan on Day 42, a cardiac electrophysiological study was performed. R-wave sensitivity was about 1 mV, and pacing threshold was higher than 5 V/0.4 ms in the RV. In addition, refractory VTs were easily induced. Therefore, she required both biventricular pacing and a defibrillator. She was provided with cardiac resynchronization therapy with a defibrillator (CRT-D) device on Day 49 and was discharged in good condition on Day 56. Serial follow-up echocardiography indicated severe RV dysfunction and dilatation with severe TR. LV was deformed by IVS leftward shift, and apparent LVEF had reduced to approximately 30% after 6 months of CRT-D implantation; however, the patient was able to return to work as a hairdresser without any worsening of heart failure.
[[43.0, 'year']]
{'31679645': 1, '19389557': 1, '24096068': 1, '30045820': 1, '31902242': 1, '19221224': 1, '28496024': 1, '30259175': 1, '11161940': 1, '28040278': 1, '34655274': 2}
Suppression of acute heart failure rehospitalization by biventricular pacing in wide QRS and mid-range ejection fraction
A 66-year-old man with complete left bundle branch block and mid-range left ventricular EF at rest had he had recurrent hospitalization (three times within a 6 month period) due to clinical scenario 1 acute HF. Treatment with a nitrovasodilator immediately compensated his HF. He had been diagnosed with symptomatic sick sinus syndrome 2 years prior and was treated with the implantation of a dual-chamber pacemaker. After a thorough review of the examination results, he was diagnosed with dilated cardiomyopathy. The right ventricular pacing burden was marginal (<0.1%) owing to his normal atrioventricular conduction and the use of an algorithm of Intrinsic rhythm support (IRSplus) and Ventricular pacing suppression (VpS) of Biotronik pacemaker. The patient had progressive dyspnoea on exertion [New York Heart Association (NYHA) Class III] even though he had received an implant of a rate-responsive pacemaker and optimal medical therapy (beta-blocker with a vasodilator activity, angiotensin receptor blocker and aldosterone antagonist at maximally tolerated doses) for the treatment of hypertension and heart failure. His home blood pressure at rest was 110/70 mmHg on average. Electrocardiogram showed an atrial pacing rhythm (60 ppm) with a complete left bundle branch block (QRS duration: 185 ms; Figure\n). A transthoracic echocardiogram showed moderate left ventricular systolic dysfunction with a mid-range EF of 42%, trivial to mild MR, with a structurally normal valve at rest. Remarkable left ventricular (LV) dyssynchrony was observed between the septal and postero-lateral walls on speckle-tracking echocardiography (Figure\n). However, the exercise stress echocardiogram demonstrated a decrease in LVEF with exercise to 33%, with an increase in systolic blood pressure (from 110 to 190 mmHg) and MR from mild to severe as the QRS width slightly increased (from 185 ms to 195 ms; Figure\n). In addition, LV dyssynchrony was further worsened (Figure\n) compared with the findings of resting echocardiography (Figure\n). Interestingly, a phenylephrine-induced increase of afterload enhanced the elevation of left ventricular end-diastolic pressure (LVEDP), pulmonary capillary wedge pressure (PCWP), pulmonary arterial V-wave augmentation and MR, which was confirmed via intra-cardiac pressure measurement and echocardiography (Figure\n) and QRS widening appeared.\nTaking into consideration the patient's debilitating symptoms, we offered him the option of a biventricular pacemaker. His paced electrocardiogram is demonstrated in Figure\n (QRS duration: 155 ms). A follow-up evaluation using the same methods as used previously demonstrated that LV dyssynchrony at rest was attenuated by biventricular pacing (Figure\n) and stress echocardiography did not show exacerbation of dyssynchrony (Figure\n); further, biventricular pacing reduced phenylephrine-induced MR and elevation of LVEDP, PCWP and pulmonary arterial V-wave augmentation (Figure\n).\nDuring the 2 year follow-up period, the patient reported a dramatic improvement in his exercise capacity, with NYHA Class I symptoms. We also noted an improvement in resting EF to 55%. The patient has not had any recurrent hospitalization due to HF.
[[66.0, 'year']]
{'21486916': 1, '22975672': 1, '20150328': 1, '32239646': 1, '34499431': 2}
A rare case of pancreatic metastasis of lung adenocarcinoma
We reported a case of 69-year-old male who was diagnosed since a year for lung adenocarcinoma. His treatment based on chemotherapy according to the line treatment of medical oncology of the hospital. He was in good health without any symptomatology. During his follow-up, thoraco-abdominal-pelvic CT scan revealed a roughly rounded, poorly defined lung mass with irregular in shape, enhanced at the periphery after contrast media injection ().\nTwo bilateral adrenal masses were also discovered as hypodense, poorly defined, irregularly, and enhanced after injection of contrast medium ().\nA hypodense pancreatic corporal mass, roughly rounded, with irregular contours, enhanced after injection of contrast medium, without dilation of the Wirsung. There is associated dilatation of the intrahepatic bile ducts and the main bile duct (). On ultrasound, this mass corresponds to a rounded, heterogeneous, echogenic lesion with discrete peripheral vascularization on color Doppler (). A endoscopic biopsy for pancreatic mass was performed to confirm the diagnostic of non-primary tumor of pancreas. Histology confirmed a metastatic mass of lung cancer.\nThe adrenal and pancreatic metastases were diagnosed. There were no surgical indications according to the multidisciplinary consultation. The patient changed the treatment line due to multiple metastases, essentially involving chemotherapy without any radiotherapy.
[[69.0, 'year']]
{'18981550': 1, '21713016': 1, '34040687': 1, '17249510': 2, '9713480': 1, '15405683': 1, '15057558': 1, '24462547': 1, '28928103': 1, '9536484': 1, '2928909': 1, '34987855': 2}
{'3891033-1': 1}
Bilateral renal infarction with COVID-19 pneumonia: a case report
A 37-year-old male presented to the emergency department with complaints of bilateral flank and suprapubic pain since 2 days. The pain was sudden in onset, continuous, 5/10 in severity and aggravated with coughing and deep breathing. His past medical history was significant for hospitalization for urinary tract infection with development of ureteral strictures requiring stents 5 years ago. There was no family history of cancer or blood disorders.\nAt presentation, his vitals were normal with blood pressure of 100/75 mm Hg. On examination, he was non-toxic, alert, oriented and in pain. Physical examination was significant for bilateral costovertebral angle tenderness. Imaging showed bilateral pneumonia on X-ray and computed tomography (CT) of chest. Contrast enhanced CT of abdomen showed brisk excretion of contrast, no hydronephrosis and bilateral wedge-shaped non-enhancing areas in the renal parenchyma consistent with infarcts ().\nLabs were significant for positive COVID-19 infection on reverse transcription-polymerase chain reaction of nasal swab and negative for IgG antibodies. Serum creatinine, blood urea nitrogen (BUN) and urinalysis were normal. Hypercoagulability workup including platelet count, prothrombin time (PT), partial thromboplastin time (PTT), international normalized ratio (INR), Factor V levels and mutation analysis, antineutrophil cytoplasmic antibodies (ANCA), complement, anti-double-stranded DNA, cardiolipin antibody, beta-2-microglobulin and serum homocysteine were all normal (). Renal arterial and venous duplex showed preserved renal perfusion and no significant stenosis in the visualized segments of renal arteries.\nPatient was admitted for COVID-19 pneumonia and bilateral renal infarction. He was started on dexamethasone (6 mg) daily, Lovenox (70 mg) twice daily subcutaneously, IV fluids and supplemental oxygen at 5 liters/min through nasal canula. Because of his deranged liver enzymes, the patient was not considered a candidate for remdesivir. He received 1 unit of convalescent plasma. Pain gradually subsided and patient recovered saturating at 98% on room air at the time of discharge. He was discharged on apixaban (5 mg) daily for renal infarction.
[[37.0, 'year']]
{'32327202': 1, '32558620': 1, '32788101': 1, '33524866': 1, '33560401': 1, '32339221': 1, '34987851': 2}
Decoding enigma: Turner syndrome with ring chromosome
A 17-year-old female presented to us with anasarca developed over the past 2 months, which started with bipedal edema gradually developing into anasarca for the last 15 days associated with intermittent fever for the same. Tracing backward this patient had a history of progressive exertional breathlessness for the past 5 years and had a history of weakness of the right side of the body and facial deviation of angle of mouth to the left, which recovered spontaneously within 24 h, suggestive of transient ischemic attack (TIA), 1 month back. She also had developed jaundice throughout the last 2 weeks and orthopnea in the last 1 week.\nFever was associated with productive cough and expectoration. There was no history of rash, diarrhea, vomiting, joint pain, dysuria, hematemesis, melena, altered sensorium, renal failure or proteinuria. Her birth history was uneventful, developmental milestones were delayed along with poor scholastic performance and she didn’t achieve her menarche yet.\nOn examination, she had sinus tachycardia, pallor, fever with 100.8°F, anasarca, suffering from cardiogenic shock with a blood pressure of 70/40 mm of Hg, raised Jugular Venous Pressure (JVP) and poor capillary filling. Cardiological auscultation revealed a systolic murmur of grade III over the left parasternal area with loud P2; chest auscultation revealed basal crepitations. She was malnourished with stunted growth. There was no organomegaly or clubbing or any abnormal fundoscopic picture. The patient was resuscitated, vasopressors added, blood cultures sent, empirical intravenous antibiotics started and urgent echocardiography planned in accordance with the infective endocarditis protocol. Electrocardiogram showed sinus tachycardia with features of biatrial enlargement. Echocardiogram revealed levocardia with situs solitus; a 1.8 cm × 1.8-cm sized mass suggestive of thrombus within the left ventricular (LV) cavity near apex was found with dilated all four cardiac chambers with no evidence of vegetation. Global hypokinesia of LV, right ventricular free wall with left ventricular ejection fraction of 21% associated with severe tricuspid regurgitation and severe pulmonary arterial hypertension were found. No abnormalities were found in the aortic valve cusp or aortic root. ().\nGiven the working diagnosis of heart failure due to DCM with intracardiac thrombus, we started the patient on anticoagulation with low molecular weight heparin 20 unit subcutaneously twice daily. After initial stabilization, we searched for the underlying etiology behind this early DCM and intracardiac thrombus. Anti-Nuclear Antibody (ANA)-Hep2 and anticardiolipin, antibeta-2glycoprotein antibody was negative along with normal protein C, S values, which ruled out some common causes of the thrombotic phenomenon.\nOn detailed anthropometric analysis, she had short stature (height 125 cm), underweight (17.5 kg), upper segment:lower segment of 0.87 and arm span being 110 cm. Other notable findings were short fourth toe in the bilateral lower limb and low intelligence [Intelligence Quotient (IQ) 72 (borderline) as per Wechsler Adult Intelligence Scale (WAIS-IV) classification] []. There was a history of amenorrhea, associated with scanty axillary and pubic hairs and breast development showed tanner stage 1 with widely spaced nipples. She had a low posterior hairline with micrognathia, a webbed neck and cubitus valgus (). Hormonal assay revealed normal levels of estradiol (129 pg/ml), follicle-stimulating hormone (6.21 mIU/ml), luteinizing hormone (0.56 mIU/ml) and low insulin-like growth factor-1 level (102 pg/ml).\nThe skeletal survey revealed short fourth metacarpal and metatarsal in all four limbs. We had ruled out pseudohypoparathyroidism with normal serum calcium, phosphate and iPTH values along with the absence of other classical Albright’s hereditary osteodystrophy phenotype morphology (early obesity, round face, heterotopic calcification) and sent sample for karyotyping from the blood that showed a mosaic pattern, with 45, X/46, X,r(X)(p22.3q28); i.e. Turner syndrome karyotype with a very rare variety that is ring chromosome [] ().\nShe was treated for heart failure with reduced ejection fraction as per standard protocol conservatively, with subsequent anticoagulation. The patient was cured of intracardiac thrombi and congestive cardiac failure and finally was discharged with oral contraceptive pills for secondary sexual character development, iron-folic acid supplementation therapy for iron deficiency anemia, was put on dabigatran for 3 months and regular follow-up is being done periodically.
[[17.0, 'year']]
{'6883789': 1, '3059758': 1, '10664223': 1, '22707402': 1, '25404835': 1, '33452775': 1, '17047017': 1, '34987848': 2}
Extensive triple vessel coronary artery disease in a young male with juvenile idiopathic arthritis
We present a case of a 27-year-old male with a 1-month history of neck and lower back pain associated with worsening dyspnea on exertion. He had no prior history of angina, syncope, palpitations, flu-like illness, vomiting or diarrhoea. In addition, he had no family history of premature CVD or traditional ASCVD risk factors. Specifically, he reported no history of cigarette smoking or any form of substance abuse.\nOn clinical examination, he had conjunctival pallor, grade three pedal oedema and abdominal ascites. Examination of the cardiovascular system revealed an elevated jugular venous pressure, a myopathic displaced apex beat with a right parasternal heave and an S3 gallop. He was in New York Heart Association functional class III. His respiratory examination was normal.\nThe musculoskeletal examination revealed loss of cervical lordosis, tender and swollen joint count of eight, fixed flexion deformity of the elbows, ankylosis of the wrists and the proximal interphalangeal joints of both hands. The clinical disease activity index score was 34, signifying a high rheumatologic disease activity.\nAll inflammatory markers were elevated (), and the rheumatologic serological studies were unremarkable (). The Human immunodeficiency virus ELISA screen was negative. A resting electrocardiogram (ECG) showed a narrow QRS complex sinus tachycardia (). Echocardiography demonstrated four-chamber enlargement with global hypokinesis of the left ventricle and a left ventricular ejection fraction of 20–25% (). The diagnostic coronary angiogram revealed extensive triple vessel disease (), with a syntax score I of 36.5. Furthermore, computed tomography angiography of the neck and brain revealed large vessel vasculitis of the common carotid and middle cerebral arteries. The vasculitis flare was subsequently treated with two cycles of cyclophosphamide at 13 mg/kg, administered 2 weeks apart.\nThe patients’ rheumatic symptoms were treated with pulsed solumedrol 1 g for 3 days, followed by prednisone at 0.5 mg/kg. As part of the heart failure therapy, the patient was acutely initiated on furosemide, low dose carvedilol, an angiotensin-converting enzyme inhibitor and spironolactone, to which he responded well. The patient was also treated with simvastatin, baclofen, chloroquine, methotrexate, folic acid and carbamazepine. The ECG did not show any features suggestive of chloroquine-induced QT prolongation (QTc > 450 ms) as both the QT and corrected QT interval were 308 and 429 ms, respectively.\nThe differential diagnoses were ischaemic cardiomyopathy secondary to accelerated atherosclerosis and a vasculopathy of the coronary arteries as a sequela of the juvenile idiopathic arthritis (JIA). A differential diagnosis of cyclophosphamide-induced cardiomyopathy was also entertained.\nThe institutional heart team decided to refer the patient for elective coronary artery bypass graft surgery once the inflammatory markers had settled. Unfortunately, 3 weeks later, the patient experienced a sudden cardiac arrest while at home. The likely cause of death was a lethal arrhythmia such as ventricular tachycardia. An intracardiac defibrillator would have been an ideal therapy to prevent his sudden cardiac death. However, in our local clinical setting, such devices are not readily available due to their high cost.
[[27.0, 'year']]
{'21905249': 1, '10789823': 1, '23606026': 1, '23731870': 1, '3533179': 1, '27697765': 1, '31073128': 1, '11254238': 1, '23502074': 1, '14760812': 1, '34987849': 2}
A fatal case of infective endocarditis complicated by acute COVID-19 pneumonia
In November 2020, a 74-year-old man with no co-morbidities presented to the emergency department of his local hospital with a 3-day history of diarrhoea and vomiting. On examination, he was agitated with a Glasgow Coma Scale (GCS) score of 12/15 (E4, V3 and M5). He had a pansystolic murmur that was heard loudest at the apex, which radiated to the left axilla. He had a number of physical signs suggestive of IE, which are shown in and . He also had evidence of impaired oral hygiene, which is shown in . His inflammatory markers were elevated. His peripheral blood cultures were taken, and he was started on intravenous ceftriaxone immediately afterwards. His blood culture grew Staphylococcus aureus. A routine SARS-CoV-2 nasopharyngeal swab taken on admission was negative. A transthoracic echocardiogram showed severe mitral regurgitation () as well as vegetations on both mitral and aortic valves (). Consequently, the patient satisfied two major criteria (new valvular regurgitation and the presence of vegetations) as well as three minor criteria (multiple Osler nodes, a Janeway lesion and a single positive blood culture) of the modified Duke’s criteria for IE []. Hence, definite IE was confirmed.\nshows the trend in his white cell count and estimated glomerular filtration rate (eGFR) over Days 1–9. During this time, his GCS ranged between 11/15 (E4, V2 and M5) and 15/15. An abdominal ultrasound on Day 5 showed no evidence of renal obstruction, hence, his renal failure was attributed to worsening sepsis. By Day 8, he had become anuric and he was treated with haemodialysis on Days 8 and 9.\nOn Day 10, he was transferred to a tertiary hospital for mitral valve replacement. He was admitted to the intensive care unit for continuous venovenous haemofiltration. However, a routine admission nasopharyngeal swab for SARS-CoV-2 on Day 11 was positive, thus delaying his surgery. We believe this infection may have occurred either during transit or during the final few days of the patient’s admission at his local hospital. On admission to the tertiary hospital, he underwent serial nasopharyngeal swabs for SARS-CoV-2 at twice weekly intervals. A plan was made to operate as soon as he tested negative, or as soon as we could be satisfied that his COVID-19 pneumonia incubation period had elapsed.\nHowever, on Day 24, he developed nausea and vomiting. Computerised Tomography (CT) imaging of his chest showed peripheral ground glass change with bilateral pleural effusions in keeping with COVID-19 pneumonia (). He was started on supplementary oxygen, oral dexamethasone and intravenous remdesivir. This was in accordance with local guidelines for the management of COVID-19 pneumonia at the time. He initially maintained an oxygen saturation >92% without the need for invasive ventilation. However, he died on Day 31 from acute respiratory distress syndrome (ARDS) secondary to COVID-19 pneumonia.
[[74.0, 'year']]
{'10770721': 1, '14726169': 1, '32479829': 1, '33069268': 1, '6438003': 1, '32574691': 1, '32598501': 1, '34987853': 2}
A case of bacterial keratitis caused by multi-drug-resistant Shewanella algae without marine exposure
A 66-year-old man was sent to the emergency department from a nursing hospital because of desaturation. He had been hospitalized for more than 2 years in an unconscious state due to a cervical spine injury. He was unable to close his eyes voluntarily. Neither the patient nor his family had a history of marine exposure. At admission, pneumonia was suspected, and conjunctival injection, purulent discharge and central white opacity were detected in his right eye. He was admitted to the respiratory department, and treatment with piperacillin–tazobactam was started for pneumonia. An ophthalmologist examined the patient at the bedside. Examination of his right eye revealed 6.0 × 5.0 mm corneal opacity and hyphema with hypopyon in the anterior chamber. Fundus examination was impossible due to corneal haziness. Corneal swabs were sent for Gram staining, bacterial culture and antimicrobial sensitivity testing, KOH mount smear and fungal culture. Empirical treatment with tobramycin, moxifloxacin and ofloxacin eye drops was started. The KOH smear and Gram stain were negative. After a 2-day incubation, medium-sized gray and dark pink colonies grew on blood agar plate and chocolate agar plate (). Other types of colony did not grow on the plate. S. algae was identified by matrix-assisted laser desorption/ionization time-of-flight mass spectrometry using the Vitek system (bioMérieux, Marcy-L’Étoile, France). 16S rRNA sequencing revealed 99.93% (1410/1411 bp) identity with S. algae JCM 21037(T) (GenBank accession number ). Susceptibility testing of the isolate indicated susceptible to amikacin, aztreonam, cefepime, ceftazidime, colistin and gentamicin, resistant to cefotaxime, imipenem, piperacillin and piperacillin/tazobactam, and intermediate to ciprofloxacin, levofloxacin and meropenem. We added steroid eye drops to control the inflammation and continued the antibiotic eye drops. After 14 days, his keratitis had improved.
[[66.0, 'year']]
{'25566408': 2, '19949681': 2, '17985805': 1, '30574928': 2, '27998732': 1, '23690548': 1, '34987857': 2}
{'4285008-1': 1, '6324159-1': 1, '2775873-1': 1}
Utility of [18F]-fluoroestradiol (FES) PET/CT with dedicated brain acquisition in differentiating brain metastases from posttreatment change in estrogen receptor-positive breast cancer
A 41-year-old woman presented with recurrent, metastatic, ER-positive invasive ductal carcinoma. The patient had initially presented 10 years prior with a palpable breast lesion. Pathology at the time of initial diagnosis was remarkable for a 7-cm invasive ductal carcinoma with lymphovascular invasion, as well as positive metastatic lymph nodes with evidence of extracapsular extension. Immunohistochemistry demonstrated the tumor to be ER-positive (70%), progesterone receptor-negative, and HER2-positive.
[[41.0, 'year']]
{'29602822': 1, '32374053': 1, '33995598': 1, '27276226': 1, '32448947': 1, '31437274': 1, '28912144': 1, '32680923': 1, '9816253': 1, '28747642': 1, '27853813': 1, '30846327': 1, '34086424': 1, '31016232': 1, '34139575': 1, '34988456': 2}
In-utero gastric perforation from combined duodenal and esophageal atresia without consistent polyhydramnios
A 24-year-old G2P0010 woman was referred to our fetal care center at 21- and 2/7-weeks’ gestation following routine ultrasound that identified ascites, dilated small intestine and absent versus right-sided stomach. Fetal ultrasound performed at our institution demonstrated a dilated stomach with possible ‘double-bubble’ sign (). Fetal magnetic resonance imaging (MRI) confirmed dilation of the stomach and proximal duodenum without clear distinction between the two structures (), suspicious for duodenal atresia. Polyhydramnios was not seen on either ultrasound or MRI. She was scheduled for amniotic fluid index (AFI) evaluations () and biophysical profiles (BPP) every 2 weeks with monthly growth ultrasounds, and induction was planned at 39-weeks’ gestation. At 30- and 2/7-weeks’ gestation, her AFI was mildly elevated at 29.7 cm (normal 5–25 cm). The ‘double-bubble’ sign was not identifiable on any follow-up evaluation. At 34- and 5/7-weeks’ gestation, she presented with decreased fetal movement. Her BPP was abnormal with absent fetal breathing episodes and a non-reactive non-stress test, necessitating further surveillance and ultimately Cesarean section. The infant was initially apneic, cyanotic without grimace and bradycardic. There was no response to continuous positive airway pressure ventilation requiring intubation with improvement in hemodynamic status. Oro-gastric sump tube placement was attempted but could not be advanced past the proximal esophagus. Radiographs noted coiling of the sump tube within the proximal esophagus and a gasless abdomen. On day of life two, the infant was taken to the operating room for rigid bronchoscopy and laparoscopic gastrostomy tube placement. Bronchoscopy demonstrated normal tracheal and bronchial anatomy. The bronchoscope was used to evaluate the esophagus and confirmed a blind-ending esophageal pouch. A gastrostomy tube was placed laparoscopically without issue but upon insufflation of the stomach, froth was noted along the lesser curve just proximal to the pylorus. A supra-umbilical midline incision was made to further evaluate, and a gastric perforation was noted. A red rubber catheter was passed through the perforation and met obstruction within the second portion of the duodenum. The perforation was repaired, and a standard duodenoduodenostomy was performed.\nOn day of life 15, an esophageal gap study was performed, which demonstrated a proximal esophageal pouch ending at the T2 vertebral level and a 7-mm distal esophageal stump, with an estimated esophageal gap of 6 cm (). The infant remained in the neonatal intensive care unit (NICU) awaiting long-gap esophageal atresia repair until 4 months of age when repeat esophageal gap study demonstrated a gap of approximately six vertebral bodies. He then underwent gastric pull-up, pyloroplasty and jejunostomy tube placement. Post-operative esophagram demonstrated no leak, and the infant was discharged home 3 weeks post-operatively with close follow-up.
[[24.0, 'year']]
{'19551391': 1, '19572291': 1, '8015045': 1, '7229841': 1, '15185215': 1, '17498283': 1, '24851753': 1, '34987752': 2}
Coronary artery bypass graft for stent occlusion in a patient with essential thrombocythemia
A 38-year-old man known case of hyperlipidemia, hypertension and ET diagnosed 5 years previously by bone marrow biopsy with JAK2 mutation (Janus Kinase 2) presented with angina. He was on hydroxy urea therapy since that time. He underwent multiple percutaneous coronary interventions to left anterior descending artery (LAD) and first diagonal artery (D1) for past 5 years. He presented with unstable angina. He had no past surgical history and no family history of ischemic heart disease. He was non-smoker. Coronary angiography showed total occlusion of LAD and D1 arteries with normal right coronary and circumflex arteries (). Echocardiography showed ejection fraction of 50% with no significant valve disease. Hemoglobin was 16 g/l and platelet count was 495 * 109 per litter with normal renal and liver function tests. His hydroxyurea medication was continued at a dose of 500 mg orally daily until day of surgery.\nHe underwent total arterial coronary artery bypass graft surgery with left internal mammary artery anastomosed to LAD and radial artery anastomosed to D1. The procedure was performed on cardiopulmonary bypass with cardioplegic arrest. The operation was uneventful and he was extubated 9 hours post-operatively. His total drainage from mediastinal and pleural drains were 500 cc over 24 hours. He required no blood or blood product transfusion. He was commenced on dual antiplatelet treatment (DAPT) using aspirin and clopidegrol Day 1 post-operative. Immediate post-op platelet count was 295 * 109 per litter and Day 1 platelet was 464 * 109 per litter. Hydroxyurea was increased Day 1 post-operatively to a dose of 500 mg orally twice daily. His platelet counts on Day 2 until Day 6 were 489 * 109, 365 * 109, 395 * 109, 650 * 109 and 780 * 109 per litter, respectively. He was discharged home Day 6 post-op with no complication. At 3 weeks post-operatively, his platelet count was 750 * 109 per litter on same treatment and dose. Patient was then seen at 4 months post-operatively with platelet count is 467 * 109 per litter and hemoglobin was 147 g/l. He was back to all daily activities and walking daily for one and half hour with no symptoms. He remained on the same dose of hydroxyl urea (500 mg orally twice daily) and DAPT.
[[38.0, 'year']]
{'29705369': 1, '7803745': 1, '20730008': 1, '25425991': 1, '7700286': 1, '17111201': 1, '28219560': 1, '34987766': 2}
Ileocecal intussusception as the initial presentation of endometriosis: case report
A 33-year-old woman was admitted to the emergency room (ER) with abdominal pain lasting 5 days. The patient did not present comorbidities, previous abdominal surgeries or pregnancies. She reported that the abdominal pain started in the epigastrium and later migrated, localized and intensified in the right lower quadrant (RLQ). It was associated with constipation and poor flatus elimination. The symptoms were not related to menses.\nOn admission, the patient presented a pulse of 65 beats/min, blood pressure of 140/90 mm Hg, and temperature of 36.5°C. On physical examination, rebound tenderness and abdominal guarding were recognized in the RLQ, and a palpable mass was identified.\nBlood tests showed leukocytosis 12 × 109/L with 75% segmented neutrophils, C-reactive protein 8 mg/dL and hemoglobin of 14.2 g/dl. Human chorionic gonadotropin hormone beta-subunit 2.0 mUl/ml. Levels of serum CA 19–9 2.0 U/ml, and carcinoembryonic antigen 0.5 mg/ml. Metabolic panel and liver function tests were within normal limits.\nAbdominal ultrasound showed in the ascending colon, a heterogeneous mass composed of a conglomerate of intestinal loops measuring 10 × 8 × 5 cm.\nComputed tomography (CT) of the abdomen and pelvis with oral and intravenous contrast was performed (), and emergency surgery was decided due to the suspected ileocolic intussusception with a probable tumor origin (). The preoperative diagnoses were malignant cecum tumor (adenocarcinoma, sarcoma) or benign tumor (lipoma, villous tumor).\nThe initial approach was laparoscopic, recognizing a tumor in the cecum associated with ileocolic intussusception. The abdominal cavity was explored without detecting other foci of probable neoplastic origin. However, due to technical difficulties, the decision was made to convert to conventional surgery. Due to the appearance of the lesion and the suspicion of a malignant tumor, an oncological right hemicolectomy was performed with ileotransverse manual anastomosis (). The operative time was 3 hours, with an estimated blood loss of 100 cm3. It did not require red blood cell units.\nThe immediate postoperative follow-up was carried out in the intensive care unit. Later the patient went to the inpatient room, where she remained for eight days.\nThe microscopic study reported ileocolic intussusception and cecal endometriosis ( and ).\nPeriodic clinical controls were performed. Five years after surgery, the patient continues under follow-up without presenting new foci of endometriosis.
[[33.0, 'year']]
{'22676322': 2, '16937103': 1, '9464437': 1, '22096677': 1, '17651575': 1, '15705388': 1, '17572918': 1, '30689680': 1, '9296505': 1, '14734195': 1, '34987756': 2}
{'3534626-1': 1}
Graves Disease Following the SARS-CoV-2 Vaccine: Case Series
A 38-year-old woman received the SARS-CoV-2 vaccine (Pfizer-BioNTech, New York, New York) and 5 days later presented to the emergency department with sudden onset left lower quadrant abdominal pain. Her presentation was consistent with thyroid storm given her fever, tachycardia to 140 beats per minute, and gastrointestinal symptoms (Burch-Wartofsky score 55).\nThyroid function tests revealed suppressed thyrotropin (TSH) of <0.008 µIU/mL, elevated free thyroxine (fT4) of 108 pmol/L, and elevated total triiodothyronine (T3) of 10.3 nmol/L. Thyrotropin receptor antibody (TSHrAb), thyroid-stimulating immunoglobulin (TSI), and anti-thyroid peroxidase (TPO) levels were all elevated. Ultrasonography revealed a diffusely enlarged thyroid gland with heterogeneous echogenicity and increased vascularity consistent with Graves. She has no personal or family history of thyroid or autoimmune disease. A computed tomography (CT) angiogram performed 1 year prior incidentally revealed thyroid gland enlargement measuring 7.3 cm in transverse diameter. She was treated with methimazole at a total daily dose of 60 mg titrated up to 80 mg along with propranolol 40 mg 3 times daily. After 2 months, fT4 decreased to 66 pmol/L, and after 3 months, it normalized to 23 pmol/L, while on methimazole, 80 mg daily in divided doses. After a discussion of the risks and benefit of administering the second vaccine, she decided to postpone the dose. She reported improved energy level and denied recurrence of palpitations or abdominal pain.
[[38.0, 'year']]
{'33543236': 1, '33674759': 1, '34533769': 1, '34106438': 1, '34015983': 1, '34342859': 1, '33858208': 1, '34451940': 1, '28167927': 1, '33489841': 1, '32265102': 1, '32686042': 1, '33584709': 1, '34939881': 2}
{'8724979-2': 2, '8724979-3': 2}
Graves Disease Following the SARS-CoV-2 Vaccine: Case Series
A 63-year-old woman received the first dose of the SARS-CoV-2 vaccine (Moderna, Cambridge, Massachusetts) and 1 week later developed a pruritic rash on her neck and upper chest. She had no palpitations, heat intolerance, distal tremor, or diarrhea. She received her second vaccine dose 31 days after the first dose. Four days after receiving the second vaccine dose, thyroid function tests were drawn, which revealed a suppressed TSH of 0.011 µIU/mL and elevated fT4 of 30.9 pmol/L and T3 of 4.6 nmol/L. She had elevated TSHrAb, TSI, and TPO antibody levels. Thyroid ultrasound demonstrated a heterogeneous hypervascular gland along with 2 solid isoechoic nodules measuring 1.4 and 2.3 cm. She has no personal or family history of thyroid disease. Except for a sister and aunt with lupus, there is no other family history of autoimmune disease. She received no recent intravenous contrast. No antithyroid medication or bet-blocker was initiated as the patient was asymptomatic. After 6 months, the TSH remained suppressed at 0.01 µIU/mL with a high-normal fT4 of 21 pmol/L. On thyroid scintigraphy, radiotracer activity was high throughout both thyroid lobes with an elevated 24-hour uptake of 41%, consistent with Graves. The patient remained asymptomatic and no antithyroid medication was initiated.
[[63.0, 'year']]
{'33543236': 1, '33674759': 1, '34533769': 1, '34106438': 1, '34015983': 1, '34342859': 1, '33858208': 1, '34451940': 1, '28167927': 1, '33489841': 1, '32265102': 1, '32686042': 1, '33584709': 1, '34939881': 2}
{'8724979-1': 2, '8724979-3': 2}
Graves Disease Following the SARS-CoV-2 Vaccine: Case Series
A 30-year-old man received the second dose of the SARS-CoV-2 vaccine (Pfizer-BioNTech) and 4 weeks later developed irritability, palpitations, tremors, and restless sleep. These symptoms were accompanied by a 15-pound weight loss over 6 weeks. Thyroid function tests revealed a suppressed TSH of <0.005 µIU/mL, fT4 minimally elevated at 22.9 pmol/L, and T3 normal at 161 pmol/L. He had an elevated TSI and negative TPO and anti-thyroglobulin antibody (ATA). He has no history of thyroid disease and had a normal TSH level 3 years prior. His mother developed Graves disease postpartum. He has no personal or family history of autoimmune disease. Medication was initiated with methimazole 5 mg and atenolol 25 mg daily. Six weeks later, the TSH remained fully suppressed and fT4 was in the normal range at 14.9 pmol/L. His irritability and restless sleep improved; however, he still endorsed occasional palpitations.
[[30.0, 'year']]
{'33543236': 1, '33674759': 1, '34533769': 1, '34106438': 1, '34015983': 1, '34342859': 1, '33858208': 1, '34451940': 1, '28167927': 1, '33489841': 1, '32265102': 1, '32686042': 1, '33584709': 1, '34939881': 2}
{'8724979-1': 2, '8724979-2': 2}
Heart of the Matter: Syncope as a Rare Presentation of Lung Cancer Invading\nthe Heart
A 65-year-old Caucasian man with a history of tobacco dependence presented with a brief\nepisode of dizziness while standing in line at the bank, which was immediately followed by\nwitnessed loss of consciousness for 30 seconds. The dizziness was only “seconds-long” and\nimmediately preceded “passing out.” He reported one previous episode of syncope last summer\nwhile “walking in the heat,” which also was sudden without any prodromal or other associated\nsymptoms. His review of systems was negative for seizures, angina, orthostatic hypotension,\nstroke, or trauma. Over the past 3 months, he experienced an 18.1 kg weight loss with\nanorexia and constipation. The patient was a retired construction worker, had smoked 50\npack-years, and drank alcohol, but denied intravenous drug use.\nOn physical examination in the emergency department (ED), the patient was afebrile, with a\npresenting heart rate of 63 beats per minute (bpm), blood pressure range of 134/73,\nrespiratory rate of 20 breaths per minute, and normal oxygen saturation of 98% on room air.\nThere was no evidence of orthostatic hypotension based on blood pressure and heart rate\nmeasurements in the supine, sitting, and standing positions. The patient appeared thin and\nchronically ill with temporal-wasting, barrel chest, mild egophony of the right lung base,\nand a scaphoid abdomen. There was no evidence of dry mucus membranes, delayed capillary\nrefill, heart murmur, jugular venous distension, lymphadenopathy, clubbing, cyanosis, edema,\nor neurological deficits. In addition, there was no evidence of gastrointestinal bleeding,\nscleral icterus, or jaundice.\nLaboratory studies were notable for white blood cell count of 16.9 K/mm3,\nhematocrit of 28% with normal mean corpuscular volume (MCV), platelets of 650\nK/mm3, albumin of 2.6 g/dL, normal glucose and corrected calcium, and negative\ncardiac enzymes. There were no other electrolyte or biochemical laboratory abnormalities\nwith respect to liver and renal function. A chest radiograph showed a right peri-hilar\nmass-like dense opacity concerning for malignancy (). Electrocardiogram (ECG) revealed\nbradycardia, variable junctional and ectopic atrial rhythms, ventricular bigeminy, QT\nprolongation, low voltage, T-wave abnormalities, and short PR interval (). Additional rhythm strips in the ED showed\nventricular bigeminy followed by a witnessed episode of worsening bradycardia (heart rate in\nthe range of 30-35 bpm) with associated near syncope.\nChest computed tomography (CT) revealed a large cavitating necrotic mass in the right\nmiddle lobe contiguous with the mediastinum, invading the superior vena cava and right\natrium with surrounding patchy consolidation (). Echocardiogram showed an extrinsic\npericardial mass at the right atrial roof () with an atrial mass measuring 5.0 × 4.4 cm (). Bronchoscopy showed erythema and\ncompression within the right bronchus intermedius and external compression of the right\nupper lobe bronchus ().\nEndobronchial biopsy revealed invasive well-differentiated squamous cell carcinoma and a\nclinical picture consistent with stage IIIB disease ().\nUnfortunately, the patient missed several chemotherapy appointments to treat his lung\ncancer. He did not maintain regular follow-up and we do not know whether he had recurrent\nepisodes of syncope. On hospital readmission 6 months later, the patient developed atrial\nfibrillation and flutter (with frequent ventricular ectopy) and electrocardiographic\nevidence of right atrial enlargement (, ), a right\npleural effusion, and hypoxemic respiratory failure. The patient eventually expired from\nprogressive lung cancer opting for comfort care rather than continued medical treatment.
[[65.0, 'year']]
{'10744016': 1, '4024175': 1, '690555': 1, '23673126': 1, '7924080': 1, '16636177': 1, '9118728': 1, '26064126': 2, '34939449': 2}
{'4443876-1': 1}
A multidisciplinary collaborative model based on single-port\nthoracoscopy for the treatment of giant mediastinal lymph node hyperplasia: a\ncase report
The reporting of the present case conforms to the CARE guidelines.\n A 30-year-old male patient was admitted to our hospital because of chest\ntightness after exercise of 1 month’s duration. He had been diagnosed with chronic\nhepatitis B >10 years earlier, for which he was taking entecavir. His hepatitis B\nvirus DNA titre was within the normal range. Computed tomography (CT) revealed a\nwell-defined mass in his right upper mediastinum that was compressing his superior\nvena cava (). Subsequent CT thoracic aortic angiography demonstrated that the\nmediastinal mass was mainly supplied by the brachiocephalic trunk, and more\nspecifically, most of the large and tortuous vessels surrounding the mass originated\nfrom the right subclavian artery (). Using arteriography of the thoracic aorta and subclavian\nartery, we determined that the bilateral branches of the subclavian artery and the\nT3 and T4 intercostal arteries were the main supply arteries. His two distal\nintercostal arteries, right internal mammary artery and left thyrocervical trunk\nwere embolised (),\nbut we were unable to successfully embolise the right subclavian artery branch\nbecause of its narrow lumen.\nSurgery was scheduled for the day after the arteriography. A double-lumen\nendotracheal tube was placed and the patient was positioned in the left lateral\ndecubitus position. Single-lung ventilation was applied, and a 4-cm long incision\nwas made in the fourth intercostal space along a right anterior axillary line.\nVia single-port thoracoscopy, we disconnected the thin\nnutritive vessels by ultrasonic scalpel and coagulation hook, gradually freed up the\nthicker branches connecting to the mass and clamped these using vascular clips, and\nfinally removed the tumour completely. Because the mass was hard and lacked the\nflexibility of lung tissue, we could not remove it via a\nnormal-length incision. Therefore, to avoid the mass rupturing, we extended the\nincision appropriately. The mass was 7 × 5 × 4 cm in size (), and was later confirmed to be\nhyaline vascular Castleman disease by postoperative haematoxylin and eosin-staining\n(),\nimmunohistochemistry, and in situ hybridisation (ISH) (). However,\nimmunostaining for human herpesvirus (HHV)-8 and ISH for Epstein–Barr virus (EBV)\nwere both negative, which is consistent with the findings of most previous studies\nthat neither HHV-8 nor EBV is involved in hyaline vascular CD.\nIn addition to the vascular embolism, acute normovolaemic haemodilution and\nautologous blood transfusion were also used. After the induction of anaesthesia and\nbefore the surgical incision was made, blood was slowly drawn from the left jugular\nvein of the patient and an equal amount of balanced salt solution was simultaneously\nprovided via a peripheral vein until the haemoglobin concentration\nhad decreased from 167 g/L to 130 g/L. Blood reinfusion was performed when the\nsurgical wound had stopped haemorrhaging. A precise thoracoscopically-guided\nintercostal nerve block was performed. The entire procedure lasted ∼1 hour, and the\ntotal blood loss was ∼200 ml. The patient was discharged on the third postoperative\nday, when he demonstrated an absence of cough, haemothorax, chylothorax, and severe\npain. During the following year there were no complications or recurrence.
[[30.0, 'year']]
{'31253137': 2, '27533770': 1, '25536724': 1, '29225882': 1, '24198836': 2, '22367441': 1, '26380750': 1, '25928278': 1, '11306326': 1, '30300638': 1, '32522182': 1, '13165944': 1, '21933401': 2, '24429696': 1, '17702325': 1, '17278035': 1, '12842545': 1, '23841009': 2, '19202940': 1, '21110277': 1, '32359709': 1, '24266334': 1, '31517014': 1, '29490420': 1, '21997995': 1, '14689419': 1, '23558227': 1, '10819491': 1, '34918977': 2}
{'6599372-1': 1, '3806124-1': 1, '3184047-1': 1, '3691905-1': 1}
Resection of small acoustic neuroma using the transcanal transvestibular\nendoscopic approach
The first patient, a woman in her 50s, presented with a 1-month history of tinnitus and\nhearing loss. She was unable to hear loud sounds in her right ear. She had no headache,\ndizziness, or facial paralysis. The patient showed no significant improvement in the hearing\nloss or tinnitus after taking an oral glucocorticoid for 1 week. Twenty days after drug\nwithdrawal, the patient visited our hospital again. A physical examination revealed complete\neardrums and no effusion in the tympanum. Pure-tone audiometry indicated a threshold of\n70 dB in the right ear. Preoperative gadolinium-enhanced MRI showed a small (11- × 6-mm)\nright-sided tumor ().
[[55.0, 'year']]
{'30169471': 1, '20193244': 1, '28816690': 1, '32728903': 1, '16708737': 1, '29021412': 1, '33229882': 1, '24266334': 1, '31028535': 1, '27565388': 1, '7580712': 1, '27625137': 1, '30201576': 1, '34929111': 2}
{'8725232-2': 2}
Resection of small acoustic neuroma using the transcanal transvestibular\nendoscopic approach
The second patient, a man in his 30s, presented with a 2-month history of right-sided\nhearing loss and right-sided facial paralysis. He had persistent tinnitus, occasional\nvertigo, no ear discharge, and no ear pain. Oral glucocorticoids and neurotrophics were\nadministered for 2 weeks; however, the patient’s symptoms did not improve, and he visited\nour hospital again 1 month later. A physical examination revealed complete eardrums and no\neffusion in the tympanum. The right-sided facial paralysis was House–Brackmann stage III.\nPure-tone audiometry indicated a threshold of 75 dB in the right ear. Preoperative\ngadolinium-enhanced MRI showed a small (12- × 10-mm) right-sided tumor.\nBoth operations were performed using oral tracheal intubation under general anesthesia.\nBoth patients were placed in the supine position with the head tilted to the contralateral\nside. The operative team comprised a chief surgeon, an anesthetist, a circulating nurse, and\na scrub nurse. The surgical site was exposed through the external auditory meatus. A 0º,\n3-mm-diameter endoscope (Karl Storz, Tuttlingen, Germany) was inserted via the external\nauditory meatus. Under endoscopic assistance, a circular incision was made in the skin of\nthe external auditory meatus at its bony–cartilaginous junction. The skin was elevated and\nremoved together with the eardrum to gain access to the tympanic cavity. The external\nauditory meatus bone and scutum were ground with an endoscopic ear drill, and the ossicular\nchain was removed to expose the whole medial wall of the tympanic cavity (including the\nvestibule and the round window) (). The anatomical boundaries of the surgical field were observed; the anterior\nboundary was adjacent to the basal turn of the cochlea, the upper boundary was the tympanic\nsegment of the facial nerve, the lower boundary was the jugular bulb, and the posterior\nboundary was adjacent to the mastoid segment of the facial nerve (). The vestibule was exposed to its depth, and\nthe spherical recess was used as a landmark for the fundus of the internal auditory meatus\nbecause this recess represents the insertion of the inferior vestibular nerve. The\nperivestibular bone was removed to widen the vestibular window, and the promontorium tympani\nwas then carefully ground, opening and exposing the basal turn of the cochlea. The cochlea\nmiddle turn and top turn did not have to be opened, and the scala tympani was preserved.\nOnce the extensions of the incision were complete and the borders of the tumor could be\nclearly seen (), the facial\nnerve and cochlear nerve were located deep in the tumor. They were identified and carefully\nprotected while removing the tumor (). Finally, the tumor was successfully separated from the internal auditory\nmeatus, and all anatomic areas were thoroughly checked for hemostasis. The cavity was closed\nusing a fat pad harvested from the abdomen to occlude the inner ear and middle ear\ndefects.\nBoth tumors were removed completely without residual tumor tissue, and damage to the facial\nnerve was avoided. No patients developed postoperative vertigo, postoperative aggravation of\nfacial paralysis, severe pain, or permanent postoperative complications. All wounds healed\nwithout issue. The patients were followed up for 6 months, and none developed recurrence.\nPostoperative gadolinium-enhanced MRI showed that the tumor was totally removed by the fully\nendoscopic technique ().
[[35.0, 'year']]
{'30169471': 1, '20193244': 1, '28816690': 1, '32728903': 1, '16708737': 1, '29021412': 1, '33229882': 1, '24266334': 1, '31028535': 1, '27565388': 1, '7580712': 1, '27625137': 1, '30201576': 1, '34929111': 2}
{'8725232-1': 2}