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A 58-year-old male patient came to the Oral Medicine and Radiology Outpatient Department at Government Dental College, Raipur, with chief complaints of pain and swelling on the lower left side of the face and mobility of teeth in the lower jaw for more than 2 months. History revealed that the patient had got his left mandibular first molar extracted about 2 months back for treatment of a similar complaint. The patient was a habitual bidi smoker for more than 20 years.\nOn oral examination, the swelling was observed in relation to lower left first molar region mucosa with bone involvement and also in lower left anterior region gingivae [Figures and ]. An extraoral, irregular submental swelling was also noted due to palpable, mobile and firm sublingual and submental lymph nodes. On inspection, a proliferative mass with surface ulceration was present in the left mandibular first molar region as well as the lower left anterior labial sulcus.\nAn orthopantomogram X-ray image of the patient's jaws and his lesion's clinical picture were also provided to the Oral Pathology Department. Radiograph showed an irregular, radiolucent lesion extending from the lower left central incisor to the left first molar in the mandibular alveolar region extending as poorly defined radiolucency into the body of the mandible region [].\nAn incisional biopsy was sent to the Oral Pathology Department with a provisional diagnosis of osteomyelitis or carcinoma of the alveolus based on the clinical appearance of the lesion and habit history of the patient. However, since the histology did not show any feature to ascertain the provisional diagnosis, a repeat biopsy was requested with full depth of the lesional tissue.\nThe incisional biopsy was done again, this time with incisional tissue from both left anterior and left molar regions of the mandibular gingiva. The repeat biopsy tissue showed normal stratified squamous surface epithelium and normal, fibrocellular, juxtaepithelial connective tissue. However, deep connective tissue showed an exuberant presence of dark, large lymphocyte-like cells and vacuolar histiocyte-like cells which made the oral pathologist suspect a lymphoma or probably a leukemia.(Figures –: different magnifications & microscopic fields). Blood parameter analysis of the patient for differential white blood cell count was done which was normal. This made it essential to try to find out what exactly was the lineage of these cells. Hence, immunohistochemistry (IHC) panel for lymphoma and leukemia was done which also surprisingly were negative and increased our perplexity.\nThus, histologically, this biopsy tissue was reported as an undifferentiated tumor on the basis of hematoxylin- and eosin-stained slides. This term has been used in reference to a heterogeneous group of tumors with little or no evidence of differentiation.[]\nAn undifferentiated malignant tumor represents either a metastasis of unknown origin or a primary neoplasia without obvious cell line of differentiation. The terminology, “undifferentiated tumor,” generally implies a high-grade malignancy, frequently associated with pleomorphic to anaplastic appearance. It is also used to describe tumors lacking evidence of lineage differentiation on the basis of routine light microscopic morphology alone.[]\nEven in undifferentiated tumors, subtle features of epithelial versus mesenchymal differentiation can often be appreciated, which assist the immunohistochemical approach to these tumors. Hints for epithelial differentiation include epithelioid cells (round-to-oval cells) with nesting arrangement and a desmoplastic stroma with feeding vessels separating tumor cell nests. Mesenchymal differentiation is suggested by a diffuse arrangement of spindled cells, without reactive stroma, but with feeding vessels in between tumor cells. Some tumors, however, may not fit into either of these two categories because of their overlapping histologic features, for example, sarcomatoid carcinoma, melanoma, lymphoma, neuroendocrine tumors and sarcoma with epithelioid cells.[]\nIHC of undifferentiated tumors helps to categorize them into small round blue cell tumors or large cell tumors. The latter group is further divided into (a) carcinomatous tumors, (b) sarcomatous or sarcoma-like tumors and (c) tumors with overlapping features.\nEach category entertains a broad list of entities from epithelial, mesenchymal, hematopoietic or melanocytic lineage in the differential diagnosis.[]\nIt was only a few days later that the patient's relative mentioned that the patient was being given radiotherapy for operated prostate carcinoma that made us think in terms of metastasis. Subsequently, IHC was done to evaluate this suspicion. IHC markers used were cytokeratin 7 (CK7), CK-high molecular weight (HMW) and P-63.\nCK7 and CK-HMW were negative, and P63 had a very mild and patchy positivity [Figures –]. Therefore, it became clear that the gingival and alveolar lesions were metastatic lesions of prostate carcinoma.\nMore recently, antibodies to p63 have been reported to be more sensitive than HMW-CK for the detection of prostatic basal cells. p63, a homolog of tumor suppressor gene p53, is essential for prostate development and is selectively expressed in the nuclei of basal cells of normal prostate glands. |
A 39-year-old man presented in 1998 with an 18-month history of left-sided nonprogressive hearing loss. He had no symptoms of discharge, pain, tinnitus, or disequilibrium and was otherwise fit and well. On examination the left ear canal was occluded with a tumour arising from the superior aspect of the external auditory canal. The lesion did not blanch on compression or appear to pulsate on examination. Pure tone audiogram showed a mild to moderate conductive hearing loss in the left ear and normal hearing in the right ear.\nA Computed tomography (CT) scan of the temporal bones showed a well-defined soft-tissue mass in the external auditory canal extending into the middle ear. There was no distortion of the ossicles or facial nerve involvement. A magnetic resonance imaging (MRI) scan with gadolinium suggested that the lesion was not vascular in nature.\nSurgical exploration was carried out via an endaural approach and the tumour was excised leaving a complex perforation of the tympanic membrane. There was further tumour excised from the middle ear with preservation of the ossicular chain and the tympanic membrane was grafted with a temporalis fascia underlay graft.\nPostoperatively the patient's hearing improved such that he had normal hearing in the left ear with no conductive deficit. The surgically removed specimens were a 1 × 0.5 × 0.5 cm nodule from the ear canal and further fragments excised from the middle ear. All were analysed histologically. All specimens contained poorly differentiated, most probably ceruminous carcinoma.\nThe patient was discussed at the joint clinicopathological multidisciplinary meeting and it was decided that the best clinical course of action would be a more radical surgical resection to clear the middle ear. This approach would likely cause further hearing loss.\nThe patient declined any further surgical treatment and requested active observation (1999). He was followed up at regular intervals both clinically and radiologically. At the last review he was asymptomatic and disease free clinically and radiologically on MRI scan. |
The patient was an 18-year-old Chinese American female being evaluated in the Adoption Medicine Clinic (AMC) at the University of Minnesota. She had been adopted at age 3 years from China. Prior to adoption, she had lived in an orphanage after being relinquished by her biological parents at the age of 5 months. Her previous medical history included a ventricular septal defect (VSD) that was repaired prior to adoption and bilateral strabismus that had been repaired during childhood. She had a resting bilateral hand tremor that was diagnosed as psychogenic by Pediatric Neurology. There was a history of delayed developmental milestones, including gross motor and speech delays. The patient did not start walking until approximately age 24 months. She did not start speaking in phrases or sentences until age 4. Following adoption, the patient’s adoptive mother attempted to obtain resources to help support the patient’s growth and development. She received occupational therapy services from age 3 to 4 years, but this had been discontinued due to lack of insurance coverage. Throughout childhood the family had difficulty obtaining appropriate therapeutic support, and the patient was moved to multiple school districts to identify and obtain resources. She was able to secure financial support from county services at age 16. Despite these challenges, the patient was able to continue to generally progress with her developmental milestones.\nThe patient had a history of intellectual disability. She had a school individualized education program (IEP) evaluation at age 13 that revealed a full-scale intelligence quotient (IQ) of 70. She was provided school services under the category Developmental Cognitive Disability, mild-moderate. She was evaluated by Pediatric Neuropsychology at age 15 due to ongoing concerns for intellectual disability and developmental delays and was diagnosed with mild intellectual disability and static encephalopathy. She was subsequently evaluated by Pediatric Neurology, who agreed with these diagnoses. The patient also underwent diagnostic testing for autism spectrum disorder (ASD) at age 15, which was not consistent with an ASD diagnosis.\nAs the patient reached adolescence, she began to have significant difficulty with emotional regulation and mood and was diagnosed with severe depression. She was hospitalized on two separate occasions due to concerns for anxiety, depression, self-injurious behaviors, and suicidal ideation. She was referred for psychotherapy and had been maintained on mood-stabilizing medications, including fluoxetine and escitalopram. She was taking escitalopram at the time of evaluation. The patient previously had dialectical behavioral therapy (DBT) and eye movement desensitization and reprocessing (EMDR) therapy. She had also been treated for an eating disorder at an outpatient program. Throughout adolescence, she continued with social isolation and self-injurious behaviors, including skin picking and cutting, although her mood had improved following initiation of medications and therapy. She had recently started with a mental health coach prior to her evaluation. The patient and adoptive mother had ongoing attachment difficulties. The patient also reported a history of poor sleep related to anxiety and use of electronics prior to bedtime. She was taking melatonin.\nAt the time of her visit to the AMC, despite the presence of known global delays, diagnosis of mild intellectual disability, and a history of congenital cardiac disease, the patient had not undergone genetic testing. The family had established contact with the patient’s biological family. There was no known consanguinity between parents. She had an older sibling with a hand deformity and a younger sibling without identified medical concerns. Neither sibling was reported to have developmental delays. There was no family history of substance abuse or mental health issues. The patient had an adaptive IEP with occupational therapy support and a plan to transition to adulthood following her senior year of high school. Of note, the patient had spent the majority of ninth grade homebound due to anxiety symptoms and was in a half-day therapy program throughout tenth grade.\nOn clinical evaluation, the patient displayed appropriate growth and physical development. Weight-for-age was 67th percentile and height-for-age was 37th percentile. Body mass index (BMI) was age-appropriate at 72nd percentile. Vital signs, including blood pressure (91/70) and heart rate (71), were age-appropriate. She did not display any dysmorphic features. She had evidence of self-injurious behaviors, including healed scars on her arms and evidence of skin picking on her scalp. She had a healed surgical incision from her VSD repair. She did not display physical findings consistent with fetal alcohol syndrome. Examination of head and neck was unremarkable. Lungs were clear to auscultation. She demonstrated a quiet systolic heart murmur best heard at her left upper sternal border. Her abdomen was soft and non-tender. Cranial nerves were intact. Deep tendon reflexes were symmetric. She demonstrated appropriate bilateral muscle strength and tone. Laboratory values, including complete blood count (CBC) and vitamin D levels, were within normal limits. She had evidence of stage 2 iron deficiency with low ferritin and elevated iron-binding capacity. She was started on an iron supplement. As part of her clinical workup, the patient was evaluated by a genetic counselor, who recommended obtaining a comparative genomic hybridization (CGH) and single-nucleotide polymorphism (SNP) microarray and testing for Fragile X syndrome, given her history of developmental delays and diagnosis of intellectual disability. She was referred to occupational therapy for management of her sensory sensitivities and fine motor delays.\nChromosomal microarray identified a 6.8 Mb deletion at chromosome location 5p15.31-5p15.33 (arr[GRCh37] arr[GRCh37] 5p15.33p15.31(26142_6858476)x1). This area of chromosome 5 is included in the larger deletion associated with Cri-du-chat syndrome. Although smaller than the typical Cri-du-chat deletion, this patient’s deletion was a likely explanation for her history of developmental delays, intellectual disability, and short stature. It may also have contributed to her congenital heart defect. Interestingly, on further discussion with the adoptive mother and review of medical records, it was found that the patient displayed the characteristic high-pitched cry of Cri-du-chat syndrome during infancy. This patient was referred for further evaluation by a medical geneticist and encouraged to contact her biological family to discuss the genetic testing results and whether any further testing of her siblings was warranted. She was formally seen and assessed by a geneticist and genetic counselor. At that time, additional genetic testing through a karyotype was pursued, which was able to rule out the possibility that this terminal deletion was the result of a translocation. That visit was also critical in determining whether any additional testing or healthcare management was indicated based on the genes included in the deleted region, and to discuss implications for inheritance and future childbearing. She was evaluated by Neurology and underwent brain magnetic resonance imaging (MRI) that revealed no intracranial abnormalities other than a presumed incidental cavernoma in the right thalamus that did not correlate with her symptoms. |
A 67-year-old female with past medical history of congenital deafness presented to the emergency room with complaints of right-sided facial droop and right upper extremity weakness, tingling, and numbness. These symptoms were sudden in onset and lasted for a few minutes. Symptoms had completely resolved at the time of presentation. She did not have a history of any atherosclerotic risk factor including hypertension, diabetes, or hypercholesterolemia. The initial set of vital signs were normal; routine laboratory tests including complete blood count and basic metabolic panel were unremarkable. A computed tomography (CT) of the head without contrast as well as a magnetic resonance imaging (MRI) of the brain with and without contrast did not show any acute intracranial hemorrhage or infarction.\nThe patient was diagnosed with TIA, and further investigations were planned to determine the etiology. A magnetic resonance angiogram (MRA) of the head and neck with and without contrast did not show any arterial flow limiting stenosis or occlusion. A transthoracic echocardiogram (TTE) with bubble study using agitated normal saline contrast was performed and was found to be normal. Patient's heart rhythm was monitored with continuous cardiac monitoring, and no arrhythmias were noted during her stay at the hospital. At this point, the patient was identified as having cryptogenic TIA, having failed to determine the precise etiology from routine workup. Patient was started on aspirin therapy and discharged from the hospital on day 3 with further outpatient workup planned. Outpatient workup for hypercoagulability showed a high factor VIII activity of 153%, which potentially put her at increased risk of venous thromboembolism. However, this test was performed just one week after the thrombotic event and was hence difficult to interpret. Subsequently, a transesophageal echocardiogram (TEE) was performed that revealed a patent foramen ovale with right-to-left shunt. This raised the concern for paradoxical embolism as the cause of patient's TIA. Lower extremity duplex venous ultrasound showed no evidence of deep vein thrombosis. However, magnetic resonance venogram (MRV) of pelvis showed compression of the left common iliac vein just after its origin, which was suggestive of May-Thurner syndrome (). There was no evidence of venous thrombosis on the MRV. May-Thurner syndrome was recognized as the probable source of paradoxical embolism causing TIA in the patient. The patient was eventually referred for percutaneous PFO repair, which was performed without any complications. The patient had been regularly followed yearly at the cardiology clinic for 5 years now. She remains in good health with no further episodes of TIA. |
A 50-year-old Caucasian female visited the regional dental office due to uncertain pain in the right mandibular region. No significant diseases were mentioned in the anamnesis. The patient was diagnosed with gingivitis, and treated with a non-steroid anti-inflammatory drug and oral rinsing with chlorhexidine. The condition of the patient did not improve significantly. When she returned a week later, she had no alleviation, but labial paresthesia on the right side of her lip. As the cause of the new symptom remained unidentified, she was referred to the oral surgery group. Initially, the paraesthesia affected only the right side of the lower lip, but by this time it had spread to the skin of the right side of her chin. The oral surgeons decided to extract the lower right wisdom tooth, but the labial paraesthesia still persisted. Since there was an extended composite restoration in the lower right first molar, and translucency around its apical region was visible, the lesion was diagnosed as periapical granuloma. The lesion on tooth 46 was not close to the mandibular canal; this can be seen on Fig. . Therefore, root canal treatment was carried out, despite the uncertain result of the percussion, palpation and sensibility test. The transparent lesion around the apex and the negative sensibility test lead to the decision of endodontic treatment. Diagnosis was made by a dentist who is not a staff member of our Department. The treatment revealed that the pulp was vital. Our oral diagnostic team examined the patient, and we diagnosed the case as cemento-osseous dysplasia based on the panoramic radiograph showing apical translucency lesions around the anterior teeth with vital pulp (Fig. ). The paraesthesia has been continually present ever since. Because the lesion around the lower right canine had already involved the cortical bone by CBCT scan (Fig. ), a root canal filling and removal of the apical lesion were performed. On the CBCT image the involvement of the entire periapical region can be seen including the lingual region and the buccal cortical (Fig. ). It became clear during the planning of the surgery that the blood vessels and nerves of tooth 43 would be injured.\nThe root canal treatment was carried out on the lower right canine in a single-visit treatment using local anaesthaesia. We explored the entire root canal length using a size #15 hand instrument. The working length was determined using an apex locator, (Woodpex III, Guilin,China), then the length was also confirmed with radiographic imaging. Following the length determination, the root canal was shaped using Wave One (Densply Maillefer, York, USA) rotary instrument. The root canal was obturated using guttapercha and AH Plus sealer (Dentsply DeTrey GmbH, Konstanz, Germany) with lateral condensation technique. Glass ionomer cement (Fuji IX GP, GC Co., Tokyo, Japan) was then applied to seal off the access cavity, while the permanent restoration was done.\nWe prepared an intraoral mucoperiostal flap using an L-shaped incision and the surgery was carried out by using a surgical microscope (Aspheron, Schmidt and Bender Hungaria, Budapest, Hungary).\nWe opened up the buccal cortical bone using a surgical bur, thereafter we removed the lesion surrounding the apex of the root. The root apex was resected and a retrograde root canal filling was placed using mineral trioxid aggregate (MTA+, Cerkamed, Stalowa Wola, Poland).\nThe area of the lesion was augmented using gentamicin impregnated BoneAlbumin (OrthoSera Dental Zrt., Gyor, Hungary). We seeked to lower the chance of osteomyelitis occuring with the use of gentamicin. Sutures were then carefully placed to achieve tensionfree closure of the flap for optimal healing.\nThe 19-year-old Caucasian woman is the daughter of the first patient. CBCT showed a radiolucent lesion around the root of the lower right incisor (Fig. ). She is presently asymptomatic after a one-year follow-up, but she occasionally felt tension and moderate pain in the right side of the mandibular region eradiating to her ear, approximately 2 years earlier. The symptoms had no dental background, and ceased gradually.\nPulp tests and periapical pathology diagnoses were made by authors on teeth 33,32,41,43 with the use of percussion, palpation and sensibility test.\nPanoramic radiograph and CBCT scans of the first patient showed radiolucent lesions located in the periapical bone, specifically on lower incisors and canines – in the premandibular, and in the right molar region of the mandible with radiopaque parts showing the lesions inside (Figs. and ). The affected teeth were asymptomatic, CBCT proved the presence of apical pathology. The buccal cortical involvement was discovered with the aid of CBCT, which also affected the treatment plan because 2D imaging methods provide no information on bucco-lingual dimension. Prior to the surgical intervention the use of CBCT was motivated by the fact that only 3D imaging could ascertain the precise shape, location and involvement of surrounding anatomical landmarks of the lesion.\nFigure shows the postoperative condition (Fig. ). At the 6-months periapical follow-up, the bone healing is being processed (Fig. ). 18 months later it can be seen on the CT image that the buccal cortical bone surrounding the root of tooth 43 has regenerated (Fig. ). In the case of the second patient, there were no notable changes in the radiographic status 1 year later, and she has no complaint at present.\nThe removed tissue pieces contained both connective tissue and calcified areas. In the calcified area, beside the irregular trabecular – lamellar bony formations, oval and globular cementum-like structures were present. In the non-calcified area connective tissue was found and connective tissue filled out the centre of another bony sample as well. No elements or remnants of a capsule were visible. The histological diagnosis was cemento-osseous dysplasia (Fig. ). |
A 20-year-old woman presented to the emergency department with complaints of severe abdominal pain, multiple episodes of vomiting and obstipation for one day. She was a known case of abdominal tuberculosis and had received anti-tubercular treatment for two months. Vital signs showed a blood pressure of 100/64 mm Hg, heart rate of 110 per minute, respiratory rate of 18 per minute, oxygen saturation of 96% on room air and a temperature of 100.5°F. She was cachectic with a weight of 28 kg which was less than 5th percentile for gender and age matched normal population. Physical examination revealed diffuse abdominal tenderness and rigidity. Her erect abdominal radiography showed gas under right dome of the diaphragm. Clinical diagnosis of perforation peritonitis was made and the patient was wheeled to the operating room for emergent laparotomy. Intraoperatively, a 3 cm x 2 cm single ileal perforation was found 1 foot proximal to the ileocecal junction, with unhealthy bowel margins and multiple mesenteric lymphadenopathy. Resection of perforated bowel segment with proximal ileostomy and mucus fistula of distal ileum was performed. Later, histopathology report confirmed tubercular etiology showing caseous necrosis in mesenteric lymph nodes and epithelioid granuloma in ulcer edge biopsy. Two days after the surgery, the patient was started on oral feeds and was accepting meals orally subsequently. Ten days following surgery, the patient had multiple episodes of bilious vomiting with stoma output decreasing to less than 100 mL per day. Her abdomen remained flat with no increase in bowel sounds. Abdominal radiography showed dilated stomach with no air fluid levels. Oral feeds were stopped and a nasogastric tube was placed for drainage of gastric contents. A contrast enhanced computed tomography (CECT) abdomen was done to determine the cause of obstruction which revealed a decreased aortomesenteric angle of 15° (Figure ).\nAs a result of this decreased angle, an abrupt collapse of the third part of the duodenum was seen along with proximal duodenal and stomach dilation (Figure ).\nBased on the clinical features and CECT findings, diagnosis of SMA syndrome was made and the patient was managed conservatively. The patient was not tolerating enteral feeds and no relief of postprandial abdominal pain was noticed even on changing to left lateral decubitus or prone position. Considering the patient’s nutritional condition, total parenteral nutrition (TPN) was started. Anti-tubercular drugs were continued with oral sips. She was started on 1500 kcal/day (50 kcal/kg/day), taking care of refeeding syndrome and was gradually titrated up to provide maximal caloric support. Monitoring of weight, blood glucose, electrolytes, liver function test, and lipid profile was done to prevent any TPN complications. A good clinical response was seen after starting TPN. The patient’s weight gradually increased to 34 kg in three weeks and gradually the stoma output increased and nasogastric drainage decreased. TPN was tapered off gradually with concurrent starting of oral feeds. On discharge, the patient was advised a high caloric and protein diet with small frequent meals. A follow-up CECT was done after six weeks from discharge which showed an increase in aortomesenteric angle from 15° to 21° (Figure ) with the patient being asymptomatic and a total weight gain of 12 kg post-surgery. |
A 53-year-old black male with end-stage renal disease on chronic haemodialysis for 12 years presented to his nephrologist with acute onset of hypotension, weakness and gastroenterological symptoms. The patient had a history of hypertension, hepatitis C, prostatic hyperplasia, gastroesophageal reflux disease, a previous translumbar dialysis catheter placement due to multiple upper chest vascular thromboses and a remote trial of peritoneal dialysis for 2 years that was stopped for unclear reasons. He was in his usual state of health until 1 day prior to presentation when he underwent a successful mechanical thrombectomy using a percutaneous thrombolytic device (Arrow-Trerotola, Arrow International, Reading, PA) for a severe venous stenosis of his left thigh AV graft. Upon presentation to his outpatient haemodialysis unit the following morning, his blood pressure was 89/48 mmHg, markedly different from his baseline systolic pressure of 140–150 mmHg. Symptomatic hypotension precluded ultrafiltration that day. Though the patient's symptoms were initially attributed to the residual effects of anaesthesia, his course over the ensuing 2 weeks worsened as he manifested nausea, constipation, progressive weakness to the point of requiring a wheelchair and continued inability to remove any fluid due to persistent hypotension. A chest X-ray revealed only parenchymal chronic scarring. Twelve days after the thrombectomy, the patient was admitted to the hospital for further evaluation.\nUpon admission, an abdominal and pelvic CT scan with contrast was performed to evaluate his GI symptoms. It revealed a thrombus within the IVC extending from the renal to the hepatic veins (). Extensive venous collateralization was also noted, suggesting a chronic condition. Two days later, a diagnostic venography revealed acute on chronic thrombus material that completely occluded the IVC. An IVC thrombectomy was performed, and a repeat venography demonstrated the presence of a pre-occlusive chronic stenosis in the midportion of the IVC (). An IVC stent was placed that resolved the stenosis and normalized blood flow. Of note, angiography did not reveal any residual stenosis in the patient's patent left thigh AV graft.\nThe IVC thrombectomy and stenting were accompanied by immediate normalization of the patient's blood pressure, leading again to successful ultrafiltration. The patient was discharged in excellent condition soon thereafter. |
A giant renal angiomyolipoma (AML) measuring 18 × 13.2 × 22.4 cm was found in the left kidney of this 43-year-old female (with no evidence of tuberous sclerosis [TS]). Her other kidney was atrophic contributing only 3% of total renal function (damaged by chronic pelvi-ureteric junction obstruction). The grossly hypertrophied left kidney was causing displacement of the ureter, pancreas and spleen (Figure ). There was gross neovascularization within the AML, and the patient suffered three life threatening episodes of haemorrhage requiring interventional radiological procedures which attempted to coil the arteriovenous malformations. On one of these occasions the procedure caused a renal artery aneurysm and a radiological attempt at repair resulted in contrast induced anaphylaxis thereby precluding any further radiological attempts. Owing to the risk of further haemorrhagic episodes, a difficult decision was made to attempt a partial nephrectomy to excise the angiomyolipoma – at this risk of rendering the patient effectively anephric and dialysis dependent if total nephrectomy had to be performed to control bleeding.\nIntraoperatively the surgeons were able to successfully resect a segment of AML measuring 14 × 19 cm. Postoperatively there was a prolonged period of hypotension requiring inotropic support and blood product replacement. She underwent three further abdominal operations to investigate and treat ongoing blood loss and abdominal compartment syndrome. This was subsequently exacerbated by intra-abdominal sepis – perihepatic collections necessitating ultrasound guided drainage and episodes of pneumoniae with multiple courses of intravenous antibiotics. During her ITU course she required haemofiltration, ventilation via a tracheostomy and total parenteral nutrition. Upon clinical improvement a right sided homonymous hemianopia was noted, with evidence of a left posterior circulation artery territory ischaemia demonstrated on head MRI scan (Figure ). The patient made a very slow recovery and was discharged 50 days later, with normal renal function, a large open abdominal wound (left to heal by secondary intention) and a new visual field defect. She has subsequently developed an incisional hernia and the visual defect has failed to resolve. |
A 41-year-old gentleman presented with sudden onset of pain and swelling over his right groin and upper thigh for a period of 10 days. He was a known patient with Type II diabetes mellitus and acute myeloid leukemia M2 diagnosed on the basis of bone marrow morphology and immunophenotyping 2 years ago. He received induction chemotherapy with cytosine and daunorubicin, followed by consolidation chemotherapy and peripheral autologous bone marrow transplant. Fourteen months post bone marrow transplantation, he had remission and developed the above complaints. Examination revealed mild tenderness over the right proximal femur with no significant restriction of movements. Plain radiographs of the right proximal femur and hip joint were normal. Magnetic resonance imaging (MRI) of the right hip revealed altered signal intensity in the right proximal femur with no cortical breach or evidence of abscess. There was edema in the surrounding muscles, especially the illiopsoas. He was treated conservatively with analgesics for the same.\nHis complaints gradually increased in the next 3 weeks and he had difficulty in weight bearing and walking. On clinical examination, he had severe tenderness over the right proximal femur with painful restriction of movements at the hip joint. Plain radiographs revealed a 6 × 3 cm, ill-defined osteolytic lesion in the proximal shaft of right femur extending into the greater trochanter with wide zone of transition and adjacent soft tissue swelling []. MRI showed altered signal intensity in proximal metadiaphysis of right femur, including greater trochanter, with focal collection of 5 × 2.6 cm with cortical breach. There were two small abscesses measuring 1.5 and 0.7 cm, respectively, in the soft tissues [].\nBone scintigraphy with Technetium 99 m methylene diphosphonate (Tc 99 m MDP) demonstrated intense increase in tracer uptake in the proximal third of right femur with normal tracer distribution in rest of the skeleton, suggestive of an infective pathology [].\nAn open biopsy of the right proximal femur was done. Intraoperatively, black discoloration of the right proximal femoral metaphysis with foul smelling discharge was noticed. The tissue was smeared with 10% KOH solution and calcofluor white solution initially and then cultured in Sabraoud's Dextrose Agar (SDA) and lactophenol cotton blue (LPCB) mount which revealed broad aseptate fungal hyphae suggestive of mucormycosis. Histopathology of the cancellous bone and soft tissue exhibited large areas of necrosis and hemorrhage, replaced by inflammatory granulation tissue with dense infiltrates of lymphocytes, plasma cells, histiocytes, neutrophils, and few multinucleate giant cells. There were many broad, twisted, and few branching fungal filaments suggestive of mucormycosis [].\nAfter the diagnosis of mucormycosis was confirmed, patient was evaluated for primary infection. He had no cutaneous lesions. ENT evaluation ruled out infection of nasal sinuses. Computed tomography scan of paranasal sinus did not reveal any signs of active infection. There was no history of gastrointestinal infections and previous biopsy from gastric mucosa was negative. There were no pre-existing wounds or deterioration of the same. Hence, primary source of infection could not be traced.\nIn the immediate postoperative period, the patient developed pathological fracture at the same site and was put on skeletal traction for 19 days before definitive fixation. He was treated with liposomal amphotericin 3 mg/kg/day for 19 days, following which radical debridement of the lesion proceeded by skeletal stabilization with 95° condylar blade plate for the right proximal femur. Considering the location of the pathological fracture (proximal femur) and the need for aggressive debridement and stable fixation, a 95° condylar blade plate fixation was achieved after adequate debridement. To prevent the usual complications of external fixation instead of infection such as pin tract infection, difficulty in mobilization and also considering adequate preoperative antifungal therapy and aggressive debridement, internal fixation was decided.\nSurgery was supplemented with amphotericin mixed with polymethylmethacrylate (PMMA) beads. 300 mg of fungitericin (amphotericin B) in powdered form was mixed with 40 g of simplex cement polymer and the mixture was then added with monomer, and while setting, cement beads were made into chains using stainless steel wire (two strings with 15 beads each) [].\nIntraoperatively, he had torrential blood loss amounting to 2.5–3 litres and went into hypovolemic shock. He was resuscitated with five units of packed red cells, eight units of cryoprecipitate, and eight units of fresh frozen plasma. Radical debridement, inability to use the tourniquet, and difficulty in differentiating infected and uninfected marrow were the causes for unexpected blood loss. After aggressive debridement, bone to bone cortical contact was achieved for healing which led to loss of metaphyseal bone loss of 3 cm.\nPostoperatively, liposomal amphotericin 3 mg/kg/day (180 mg/day) was given for 3 weeks and downgraded to 1 mg/kg/day (60 mg/day) for the next 9 weeks. Patient had received a total dosage of 7560 mg of liposomal amphotericin for a total period of 12 weeks. He was advised postoperatively physiotherapy and toe-touch weight bearing crutch walking for 12 weeks. The beads were removed 12 weeks after bead insertion. Bone grafting was done along with beads removal and the tissue sent during surgery for histopathology and microbiological review was negative for mucormycosis.\nAt 3-year followup, there was 3 cm limb shortening noticed for which a shoe raise was given. Plain radiographs revealed healed fracture with no residual/recurrent lesion in the metadiaphysis region of right proximal femur []. |
A 52-year-old man with a history of cirrhosis secondary to hepatitis C. Surgical history revealed banding of esophageal varices and a right inguinal hernia repair twice over the past 2 years. The last repair of inguinal hernia was 7 months ago in which he required mesh placement. He presented to the emergency department with an excruciating stabbing pain in the right lower abdomen and pelvis area for 3 weeks. The pain was worse on getting up from a supine position and was not relieved by bowel movements. It slightly increased upon urination as well. Physical examination revealed moderate tenderness at the lower abdomen, over the urinary bladder, and over the site of the previous inguinal hernia surgery. There were no signs of hernia recurrence. The testicles were descended and nontender. Computed tomography showed hepatic cirrhosis in addition to an extraperitoneal soft tissue of 2.6 cm × 2.3 cm in the pelvis adjacent to the urinary bladder and extending into the right inguinal canal []. A diagnosis of Meckel diverticulum was initially suspected, but it was later ruled out by a negative radio-nuclear Meckel scan. Blood workup revealed white blood cell count of 8700/mm3, bilirubin of 1.6 mg/dL and aspartate aminotransferase of 83 U/L. During a laparoscopy, a cystic mass was viewed attached to the urinary bladder and the right inguinal area []. The mass was subsequently excised from the urinary bladder surface and then removed from the site of the previous inguinal hernia repair. The superficial layer of the bladder was then repaired. The mass measured 4.5 cm × 2.5 cm in diameter. Histological sections demonstrated a cystic lumen of diameter of 1.5 cm filled with old blood and surrounded by thick muscular mass. When viewed microscopically, the lesion consisted of thick, smooth muscle fibers that concentrically surround a central lumen that was bordered by endometrium-like glands and stroma []. Immunostains were positive for estrogen and progesterone receptors in the glandular component and for CD10 in the stroma, which was consistent with endometriosis. The patient had an uneventful postoperative course and his pain resolved completely. |
The patient was a 47-year-old male with non-contributory past medical history. He noticed weakness in the left lower extremity upon rising from bed in the morning, but the symptom gradually resolved. On the night of the same day, he suddenly developed headache and mild paralysis of the left lower extremity and presented to our hospital. On admission, the patient was alert and had mild paralysis of the left lower extremity. His blood pressure was 138/78 mm Hg. There were no abnormalities in the blood biochemistry tests taken upon arrival. The head CT and MRI showed cerebral infarctions in the right frontal lobe and subarachnoid hemorrhage in the frontal convexity and anterior interhemispheric fissure. In MRA, DAs were observed in the bilateral anterior cerebral arteries. In the 3D-CT angiography, dissecting intima was observed in the area with saccular enlargement (). The patient was diagnosed with a combination of cerebral infarction and subarachnoid hemorrhage caused by the rupture of the DAs in the bilateral anterior cerebral arteries. In the left internal carotid angiography, saccular cerebral aneurysm was observed in the left A1–A2 junction (). In addition, stenosis was seen in the left A2 segment and opaque vascular bulging was seen in its peripheral in the late arterial phase. Right internal carotid angiography showed cerebral aneurysm at the left A1–A2 junction (). The right A2 segment was occluded and its peripheral vessels showed retrograde flow during the venous phase. The treatment plan included surgery to prevent recurrent hemorrhage and to reinforce the walls of the DAs in the anterior cerebral arteries. Surgery was performed on the fifteenth day after admission when the patient’s neurological symptoms stabilized. First, anterior interhemispheric approach in a supine position was used to confirm the presence of aneurysms in the bilateral areas from A2 to A3 segments. The arterial wall was dark purple, and the vascular diameters were irregular with bulging in one area. Wrapping was performed from A2 to A3 segments for the left anterior cerebral artery. The right peripheral vessels had weak walls and vascular anastomosis was difficult. Therefore, trapping was performed at the A2 segment. In the postoperative course, the patient had no apparent neurological deterioration. One month after the surgery his symptoms had resolved completely, and he could return to work. |
A 40-year-old female presented with chronic headache with infrequent exacerbations. She presents with worsening headache for three months with associated vertigo, nausea, and vomiting not responding to analgesics or vestibular sedatives. Her symptoms initially started twelve years ago as a diffused mild headache, which persisted through the day. Gradually, the headache worsened to a severe headache episodically associated with vertigo, nausea, and vomiting. These episodes lasted for two to three days and got resolved. She was treated with flunarizine for suspected basilar migraine but did not show any response. From the last year, she had monthly exacerbations of headache associated with distressing vertigo, unsteadiness of gait, and right-sided body numbness. In between these episodes, she had a significant dull diffuse headache not responding to simple analgesia. She did not complain of fever or night sweats but had constitutional symptoms lasting for several months. All of these symptoms severely affected her daily activities and functionality.\nDuring the last 17 years, she had repeated episodes of neurological deficits. Even before the headache appeared, she has presented with visual impairment of the right eye and right lateral rectus palsy and was treated as retrobulbar neuritis with good response to methylprednisolone. One year later, she developed left-sided visual impairment, which fully responded to methylprednisolone. MRI imaging at that time revealed normal results. Few months after this event, she got admitted with right hemisensory loss with hemiplegia, and a demyelination disease or hemiplegic migraine was suspected. Second MRI was performed at this admission, and no abnormalities were detected again. Eight years ago, she had developed a left lower motor type facial nerve palsy, which was attributed to Bell's palsy. Within the last year, she was diagnosed to have depression and anxiety for which she was treated for few months. Other than the first two instances, she was not treated with steroids thereafter. She did not complain of weight loss and did not have constitutional symptoms or chest symptoms during these periods.\nOn examination, she is an averagely built female with a BMI of 23 kg/m2. She is afebrile, pale, and did not have lymphadenopathy. Her GCS was 15/15, and she was conscious and rational with normal pupillary response, visual acuity, visual field examination, and fundoscopy. There was no neck rigidity, and she had residual left lower motor VII palsy. She had an ataxic broad-based gait with unsteadiness. Upper and lower limb examination is clinically normal. Her respiratory, cardiovascular, and abdomen examinations were unremarkable.\nInvestigations revealed a hemoglobin count of 9 g/dL with normal white cells and platelets. ESR was elevated to 86 mm/1st hour. Renal- and liver-related biochemical investigations were normal with an alkaline phosphatase within the normal range. Initial MRI scans of the brain done 10 years ago did not reveal any abnormalities such as demyelination, optic nerve enhancement of focal lesions in the cerebrum, or cerebellum. CSF examination revealed an elevated protein level of 55 mg/dl with normal glucose and cells with negative oligoclonal bands or TB-PCR. Vasculitis investigations including ANA, ANCA (ELISA and Immunofluorescence), and RF were negative. Syphilis serology and HIV testing were also negative. Chest radiograph, ultrasound abdomen, and CT scan of chest and abdomen did not reveal any mediastinal lymphadenopathy or focal lesions in visceral organ or evidence of any malignancy. Serum ACE levels (19 µ/l) and ionized calcium levels were normal. We performed a new MRI scan of the brain with contrast, which revealed a diffuse and patchy meningeal thickening and enhancement mainly in the right frontoparietal and left occipital regions with a minor enhancement of bilateral optic sheaths (). Her NMO antibodies were normal, and the MRI did not show any areas of demyelination. Therefore, she underwent a dural biopsy from the thickened dura, which revealed large areas of caseous necrosis surrounded by epithelioid histiocytes and lymphoid cells with a few isolated giant cells in the adjacent vicinity (). There were no features of vasculitis or sarcoidosis. TB-PCR of tissue and acid-fast bacilli were negative. Final conclusion was necrotizing granulomatous inflammation suggestive of dural tuberculosis. This diagnosis was presumed by the presence of necrotizing granulomatous necrosis with caseation with a strongly positive Mantoux test of 25 mm (), and later was supported by a marked response to antituberculous medication.\nWe initiated her on antituberculous therapy (all four drugs for 3 months and 9 months of rifampicin and isoniazid) without streptomycin as she is already having vestibular symptoms. Steroids were added concurrently (1 mg/kg) and was continued for 6 weeks and was tailed off over a month. She experienced a marked improvement of her headache, and she could do her daily activities normally. After a year of anti-TB medication and follow-up, she did not complain of any worsening of symptoms. |
A 30-year-old lady presented with complaints of right lower abdominal pain since seven days which was insidious in onset and gradually progressive in nature. There was a history of difficulty in micturition and also a history of intermenstrual bleeding which were on and off for the past one year. There was no history of altered bowel habits. An abdominal examination did not reveal any abnormalities. On preoperative perivaginal examination when the cervix was pulled up, a mass was felt through the posterior fornix. Whereas on postoperative perivaginal examination, a small cystic mass was felt in the left fornix. Ultrasonography of the whole abdomen showed a large mass occupying the whole of the pelvis just superior to the fundus of the uterus arising from the right adnexa (not arising from the uterus) measuring 12.5 x 13.2 x 14.3 cc. There was also moderate free fluid seen in the peritoneal cavity with moving internal echoes (). The chest x-ray did not reveal any major abnormalities except for marginal increase of cardiac size in transverse diameter without any clinical cardiac abnormalities (). The magnetic resonance imaging (MRI) of the pelvis showed a large oval, well-defined, altered signal intensity mass lesion measuring approximately 108 x 146 x 147 mm in size which occupied the lower abdomen and upper pelvis extending from upper L3 to S2 level superior to uterus. This lesion was displacing the adjacent bowel loops and was posteriorly abutting the iliac vessels and vertebrae. The lesion showed adequate heterogeneous enhancement more on the posterior and right lateral aspects along with enhancement of a tag-like structure and with mild heterogeneous enhancement in the centre. The left ovary was easily visible, measuring 19 x 15 mm, whereas the right ovary was small and ill-defined, measuring 10 x 16 mm. On the basis of these findings, a provisional diagnosis of leiomyoma, possibly pedunculated, was made (). However, in view of the ill-defined right ovary and moderate ascites further workup was suggested including ascitic fluid aspiration and cytology and CA-125 for the confirmation of the findings. The preoperative CA-125 level was 69.9 U/mL. The patient underwent laparotomy followed by right salpingo-oophorectomy and peritoneal lavage with perioperative findings revealing 15 cm x 15 cm ovarian mass with clotted blood (), areas of hemorrhage, and necrosis. The patient further underwent total abdominal hysterectomy with left salpingo-oopherectomy after the first course of adjuvant chemotherapy. Finally the patient was diagnosed as primary leiomyosarcoma of right ovary based on histopathological examination and immunohistochemistry (). Immunohistochemistry of the tissue revealed positive for vimentin (), smooth muscle actin (), desmin, and it was negative for inhibin. Then the patient was planned for adjuvant chemotherapy with vincristine, epirubicin, and cyclophosphamide intravenously three weekly for six cycles. Six months after the treatment completion, the patient is on regular follow-up and is disease-free on clinical and radiological examination (). |
A 37-year-old female patient with no previous medical history underwent a Cesarean section early in the morning at a local medical center, and then experienced chest pain and dyspnea. She was referred to the Yeungnam University Hospital when these symptoms worsened and when a systolic blood pressure of 60 mmHg and a heart rate of 40 beats/min were recorded. She had undergone a normal pregnancy two years prior and experienced short breath during her most recent pregnancy, but she thought nothing of it. On arrival, besides mild fever and chest discomfort, her vital signs were stable. A chest computed tomography (CT) was performed to evaluate the dyspnea and revealed sinus of Valsalva dilatation at 78 mm in diameter and descending aorta dissection from the left subclavian artery to the renal artery, but there was no dissection of the aortic arch (). On physical examination, a grade IV/VI diastolic murmur was heard, and rales in both lung fields were also heard. A transthoracic echocardiogram revealed severe aortic regurgitation (grade IV), dilated sinus of Valsalva (74 mm), and left ventricular enlargement; however, left ventricular systolic function was maintained with an ejection fraction of 56%.\nConservative treatment of the descending aorta and the Bentall procedure for the ascending aorta were planned because the descending aorta was measured at 4 cm in diameter, both the true and false lumen blood flows were stable, malperfusion symptoms were absent, and the blood pressure in both lower extremities was stable. Surgery was performed through a median sternotomy. Above the aneurysm of the ascending aorta, there was a normal aorta where aortic cross-clamping was possible; therefore, arterial cannulation was performed on the proximal aortic arch, and venous cannulation was performed on the right atrium. After aortic cross-clamping and aortotomy, the dissection flap at the aortic root was observed, and aortic dissection, which is thought to cause huge aneurysms, had occurred (). A composite graft (SJM valved graft conduit 25 mm; St. Jude Medical Inc., St. Paul, MN, USA) was used for the operation. With no total circulatory arrest, the total pump time was 208 minutes and the aortic cross clamp time was 160 minutes. After 30 hours in the intensive care unit, the patient was weaned from the ventilator and transferred to the general ward. An echocardiogram on postoperative day 20 revealed a normal graft and valve function, and the left ventricular end-diastolic volume decreased from 68 to 48 mm. The patient was followed up on postoperative day 34 with 3D CT, which revealed a well-maintained surgical region of the ascending aorta and a stable lesion on the descending aorta (). The patient has been monitored since she was discharged. |
A 42-year-old male presented to the Department of Head and Neck Surgery of a tertiary oncological centre because of right mandibular swelling and trismus. The patient had already been admitted to a secondary care hospital 3 months earlier with complaints of right mandibular discomfort and slight tumefaction. A biopsy was then performed and the diagnosis of ameloblastic carcinoma was made histologically. The patient was otherwise healthy with no significant past medical history, including alcohol, smoking or tobacco abuse.\nA complete head and neck examination revealed a painless, firm and fixed right mandibular mass with no cutaneous inflammatory signs. No ulcers or mucosal lesions were found in the oral cavity. Laboratory evaluation, chest radiograph and respiratory function tests were unremarkable. The patient underwent bronchofibroscopy, which revealed only mild laryngeal hyperaemia.\nBoth neck CT () and MRI () were performed, showing a large, solid tumour arising from the ramus and posterior body of the right mandible. The lesion extended to the surrounding soft tissues, with invasion of the masseter and medial pterygoid muscles and caused bulging of the buccal mucosa. The soft tissue component was hypointense on T1 weighted and hyperintense on T2 weighted MR images and showed avid enhancement after gadolinium administration on MR examination. CT scan disclosed striking sclerosis and irregularity of the mandibular ramus with some gas bubbles inside the medullary cavity and an expansive lytic component in the posterior body and angle with some bone-forming matrix inside. Prominent periosteal reaction was also identified, particularly in the outer cortical surface of the mandibular ramus with the typical pattern of a ruptured Codman triangle. No associated cystic lesion was found in the mandible. No enlarged lymph nodes were detected and the evaluation of the remaining cervical spaces was unremarkable. A thoracic CT scan was also performed, with no parenchymal lesions.\nThe patient underwent a right hemimandibulectomy and ipsilateral cervical lymph node dissection. Surgical resection also included the right submandibular gland and a segment of buccal mucosa that was swollen by the mandibular mass. Reconstruction was performed with free fibula graft. The surgical specimen included a large, white and solid tumour with 11 × 7.5 × 6.5 cm, corresponding to an invasive, moderately differentiated (G2) SCC (). Focal positive margins were found at the medial surface of the specimen. The resected buccal mucosa, submandibular gland and lymph nodes had no neoplastic tissue. Taking into account the imaging staging examinations and the post-surgical histological report, the final TNM stage was stated as pT3 N0 Mx. After surgery, the patient underwent adjuvant chemotherapy (cisplatin-based regimen) and radiotherapy. |
Mr P was a married man in his 40s with one child. He had served in the UK army and was deployed to the Bosnian conflict in the mid-1990s. During his deployment he was stationed at a morgue that had been set up to aid the identification of bodies recovered from a mass burial site. He described how the morgue contained a large number of bodies in various states of decomposition, with varying degrees of physical trauma. He reported re-experiencing fragmented intrusive images of the morgue and daily emotionally distressing nightmares of which he could not recall the content. He experienced high levels of anxiety that triggered his dissociative symptoms. These appeared to function as a coping strategy to avoid distressing emotions connected to his memory of the morgue. Other avoidance symptoms included feeling detached from people around him and feeling emotionally numb. Mr P avoided family occasions and busy places which could trigger his hyper-arousal symptoms and had never spoken to anyone about his experiences. He reported coping historically through the use of alcohol to block out his emotions. His avoidance behaviours had maintained his PTSD symptoms.\nAt the start of therapy sessions, Mr P was reluctant to talk about his past experiences stating he only felt able to discuss vague details about his intrusive memories. Mr P attempted to use physical objects such as stones and leaves, or his e-cigarette, as grounding strategies in the clinic room. However, he also reported feeling unable to tolerate any emotions connected to the events and found it difficult to identify peri-traumatic cognitions. Mr P appeared to experience intrusions of one particular fragmented image and could not recall any other contextual information from before or after this moment.\nIn both cases Mr C and Mr P experienced difficulties in engaging with traditional methods of imaginal reliving and prolonged exposure, due to high levels of avoidance and low thresholds for dissociation, and subsequently had difficulties accessing their cognitions for updating. For both clients the prior use of multiple grounding methods in the therapy room were unsuccessful. They experienced their traumatic memories as fragmented images with a sense of ‘nowness’ (), which indicated a distinct lack of contextualization.\nAn adaptive approach was then trialled to address these hindering factors. This involved three stages: (1) walking the client through the imaginal scene outdoors to address dissociation, (2) viewing the imaginal scene from multiple perspectives to facilitate contextualization of the memory and (3) identifying and reappraising the cognitions with frequently used approaches in trauma-focused therapy. |
We report a 64-year-old Japanese woman with no past medical history, no family history of PoLMT or PLMT, and no obesity who did not smoke or drink alcohol. She felt uncomfortable but did not have pain in her left foot about 3 months before onset. Although she did not hit her foot, did not have joint deformities, and did not have morning stiffness, she developed a slight swelling and a hot feeling in the dorsolateral part of the left foot that resolved spontaneously over several days about 2 weeks before onset. Upon neurological examination, she presented with repetitive involuntary toe movement of the left foot in the absence of pain. The movement comprised nonrhythmic extension, flexion, abduction, adduction, and torsion, and was most prominent in the 4th and 5th toes (video). She was able to consciously suppress the movement to some extent, but not completely. The movement was exacerbated when she was distracted. This occurred throughout the day at rest and disappeared during sleep. She had no sensory disturbance, reduced tendon reflexes, weakness, or muscle atrophy, which indicated that she had no other neurological deficits. CT and MRI did not show tumors, infarction, hemorrhage, or any other lesions. Whole spinal cord MRI did not reveal compression or any other lesions. Nerve conduction studies and somatosensory evoked potentials were within normal limits. Cerebrospinal fluids and blood examination, including vitamin B1 and B12, ceruloplasmin, iron, thyroid function, and autoantibodies indicating collagen diseases and rheumatic arthritis, were also all within normal limits. The patient's movement did not fulfill either periodic limb movement or restless legs syndrome because of the absence during sleep and the lack of urge to move her legs and toes, respectively. We excluded psychogenic movement disorder because she had no secondary gain and spontaneous remissions, her onset was not sudden, and the movement had no variability over time. Considering the form of the movement without pain and other clinical points, we diagnosed her with idiopathic PoLMT. The patient was treated with a low dose of clonazepam (0.5 mg/day). Thereafter, the involuntary movement improved and disappeared completely several days after treatment. She discontinued clonazepam because of the complete resolution. PoLMT recurred several days after the withdrawal, although to a lesser extent. Written informed consent was obtained from the patient for the publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal. |
A 21-year-old woman presented in 1996 with right arm pain and weakness. She underwent total laminectomy at C7-T1 with gross total removal of the intradural tumor and partial removal of the extradural tumor. The pathologic diagnosis was Ewing's sarcoma, and it showed the expression of MIC-2 antigen according to immunohistochemistry. The patient presented with recurrent right side weakness the following year and so she underwent resection of the recurrent mass followed by chemotherapy and radiation treatment. No metastatic lesion was found at the time of the first or second operation. The patient was healthy until August 2004 when she complained of headache. No recurrent tumor was found in the spine at that time. In March 2005, she presented with slowly progressing left side weakness and paresthesia. MRI was performed at 3.0 T, and this showed a mass in the right cerebellopontine angle cistern with extension into the ipsilateral Meckel's cave (). The mass showed a slightly heterogeneous lobulated contour with similar or slightly higher signal intensity than the cortex on the T2-weighted images, with heterogeneous enhancement. Several spots of dark signal-intensity were noted within the mass on MRI, suggesting the presence of hypervascularity, hemorrhage or calcifications. Edema was noted in the pons and cerebellum adjacent to the mass. Noncontrast CT showed a spot of subtle high attenuation within the mass, suggesting the possibility of calcification or hemorrhage (). CT also demonstrated a high-attenuation curvilinear area near the cerebellum, and this showed low signal intensity on T2-weighted imaging and no enhancement (). The ipsilateral internal auditory canal and petrous bone were free of tumor on both CT and MRI (). Catheter angiography showed no blood supply from the meningeal arteries. Surgical resection was performed via a suboccipital approach with partial removal of the ipsilateral mastoid. No petrous bone or adjacent brain parenchyma involvement was found. The surgeon observed focal hemorrhage within the mass. Complete resection of the mass was done and this was followed by radiation treatment. The pathologic diagnosis was a metastatic Ewing's sarcoma with a MIC-2 antigen expression (). The curvilinear area of high attenuation on CT was determined to be fibrosis on the pathologic examination. The patient again presented with headache in January 2006. MRI revealed a new enhancing lesion abutting the anterior falx cerebri, and left fovea ethmoidalis was also noted (). The FDG PET scan demonstrated increased metabolism in the mass, suggesting the recurrence of tumor. Radiation treatment was performed for the lesion. |
Herein we report a case of a 35-year-old male from Nawalparasi, Nepal, who had undergone a craniotomy and evacuation of acute subdural hematoma following an automobile accident 2 months before admission to our institution. He presented with complaints of an abnormal gait, with a tendency to fall backwards and also with features of frontal lobe-related incontinence. There were no significant past medical illnesses. He was taking Sodium Valproate (300 mg oral three times daily) as seizure prophylaxis following the traumatic head injury and surgical intervention for the same 2 months previously. Fundus examination revealed the presence of papilledema. A head computerized tomography (CT) scan revealed the presence of evolving hydrocephalus. To rule out hydrocephalus\nex vacuo due to volume loss and changes in CSF dynamics subsequent to the previous accident, external ventricular drainage (EVD) was placed which revealed egress of CSF under pressure. The reasons for opting to choose EVD prior to VP shunting are threefold. Firstly, we had to rule out post traumatic hydrocephalus\nex vacuo by measuring the opening pressure of the CSF egress and looking for the neurological improvement in the patient following CSF diversion. Secondly, since we not have the programmable VP shunting available, we need to appropriately choose the Chabra shunt depending on the opening pressure so as to prevent either over drainage or under drainage of CSF. Lastly, since it was a post traumatic case, we need to measure the CSF protein (as it may be increased from the lysed traumatic subarachnoid blood) and also we need to rule out subclinical meningitis. Both these may be the reasons for shunt failure. Following EVD, the patient showed gross improvement in his previous deficits. CSF sugar and protein was within range. Gram stain was negative for any bacteria. Repeat CT scan post EVD did not reveal any pneumocephalus. Thereafter he was scheduled for insertion of a VP shunt. EVD was clamped for 6 hours prior to the procedure to facilitate the ventricular tap. During insertion of the VP shunt, there was dry tap during an attempt of ventriculostomy from the Kocher’s point. We made two further attempts to ensure the correct trajectory of the shunt end and also to reflush the shunt end to prevent blockage due to blood clots and cell debris. We placed the shunt tip in the presumed location of the foramen of Monro of the frontal horn of ipsilateral lateral ventricle. We did not remove the EVD, hoping that it would act as a safety channel for CSF bypass had we missed the correct trajectory for the VP shunt.\nA postoperative scan revealed the presence of tension pneumocephalus and pneumoventriculi (\n and\n). The patient was managed with 100% oxygen for 3 days and was continued on antiepileptic medications at the same dose intravenously. Stringent neurological monitoring was undertaken to evaluate early neurological deterioration due to tension pneumocephalus. Pupils were routinely assessed to look for hippus (a clinical marker of epilepsy). Patient was extubated the following morning. A repeat CT scan on the 6\nth day post-operation showed that the proximal shunt was in the third ventricle (\n) and there was complete resolution of the condition. The EVD was subsequently removed with no neurological deterioration of the patient on 7\nth day after operation. The patient then started to walk with support from the 8\nth day post-operation, and he slowly improved in gait. Patient went home walking with minimal support on the 14\nth day post-operation. Patient had also regained his bladder control within that time. Patient returned, walking on his own 1 month later for his follow up in the outpatient department His gait was normal with no features of retropulsion. The shunt chamber was functioning well and his bowel habits were normal. Compliance in continuation of Sodium valproate therapy (at the aforementioned dose) was also ensured. |
A 58-year-old female patient presented with pain in abdomen and urinary incontinence with hematuria in April 2012 for which she was evaluated. Past medical history revealed that she had bilateral hydronephrosis for which she had undergone stenting. She also had Parkinson's disease and rheumatoid arthritis for which she was under regular medical management. A CT scan was taken which revealed a large ill-defined irregular mass in the uterine cervix with bilateral parametrial infiltration. The mass measured 5 × 4 cm at its widest point, infiltrating the lower uterine myometrium, extending anteriorly infiltrating the posterior wall and trigone of the bladder involving both vesicoureteric junctions. Posteriorly, the mass had infiltrated the anterior wall of rectum. Few enlarged internal ileac nodes were present bilaterally. Biopsy revealed the mass to be squamous cell carcinoma. She also had bilateral L5 spondylosis with grade II anterolisthesis of L5 over S1. She was treated with external radiation to the pelvis with a dose of 6000 cGy in May 2012. After radiotherapy, the PET-CT in June 2012 () showed residual cervical thickening and residual retroperitoneal lymphadenopathy with increased F18-FDG uptake in the aortocaval and retrocaval lymphnodes with the largest measuring 1.5 × 1.4 cm. Degenerative changes were also noted in the spine. For the residual lymphadenopathy, considering the factors like invasive nature of the surgery and the comorbid conditions of the patient along with fragile nature of her physique making chemotherapy, a high-risk one, the treating physicians recommended autologous natural killer (NK) cell and activated T lymphocyte based immunotherapy termed as autologous immune enhancement therapy (AIET). The first AIET cell transfusion was done in July 2012 and the second and third cell transfusions in August 2012. For each transfusion, approximately 70 mL of peripheral blood was withdrawn and the isolation, in vitro expansion cum activation of the NK cells and T lymphocytes were done as reported earlier []. After the three transfusions, an MRI was taken in September 2012 which showed regression of the aortocaval node to 1.4 × 1 cm (earlier 1.5 × 1.4 cm) and the retrocaval node measured 0.9 × 0.5 cm. The fourth and fifth AIET transfusions were done in October 2012 and the sixth transfusions in November 2012. An average of 282.5 million NK cells and 478.5 million activated T lymphocytes were transfused in these six AIET cycles. A PET-CT scan in November 2012 after the completion of all the six cycles of AIET showed complete resolution of the retroperitoneal lymphnodes with no evidence of local lesion in the cervix (). The PET-CT also showed sclerotic lesion in the C7 vertebral body. However biopsy of the lesion was not done and the patient is still under followup. In addition to the above changes, the patient also reported improvement in quality of life after the AIET. There were no adverse reactions reported after AIET. |
A 46-year-old female presented in 2017 with symptomatic right leg pain and aesthetic complaints relating to the right calf. Medical history showed a previous head trauma (car accident) with brain hematoma drainage and a saphenous vein operation. Physical examination revealed edema in the perimalleolar area and painful varicose veins, in the right calf (with considerable aesthetic impact) and left calf (with minor aesthetic impact). Venous scores at the first visit to our clinic were the following: Venous clinical severity score VCSS 10 and Aberdeen quality of life questionnaire 27.7.\nDuplex examination conducted before the original venous operation (which had been performed in a different clinic in January 2016) had shown reflux in the left great saphenous vein and significant reflux in the right small saphenous vein. However, the operation actually performed was stripping of the right great saphenous vein. Both the left great saphenous vein and the right small saphenous veins were left in place untreated. After this procedure, symptoms had exacerbated progressively, and the aesthetics of the leg had deteriorated progressively.\nPreoperative evaluation was normal. We performed a complete duplex scan, according to our routine, as published elsewhere. The patient had type 1b+2a shunt in the right leg and 4+2d shunt in the left leg. We suggested operating to treat the small saphenous vein in the right leg and the great saphenous vein in the left leg. We treated the patient using the CHIVA technique to preserve the remaining saphenous veins.\nWe performed the CHIVA procedure on both legs during the same operation. Local anesthesia was provided with a solution containing 10 mg/mL 20 mL of ropivacaine and 2% lidocaine, using 20 mL and 60 mL of saline. We routinely have an anesthetist in the operating room to guarantee patient safety and comfort, who is always advised to avoid sedation as much as possible. When necessary, an opioid-free sedation technique is employed. In the right leg, we ligated the small saphenous vein at its junction with a calf vein and ligated two N3 collaterals, leaving the small saphenous vein draining through two perforators. In the left leg, we ligated a collateral draining to the great saphenous vein from the inguinal ligament and an N3 draining reflux from the great saphenous vein to the calf. A total of 5 small incisions were made. The patient was discharged two hours after the operation wearing compressive stockings and taking 40 mg enoxaparin per day for 3 days, according to our postoperative routine.\nOn the sixth postoperative day, duplex scanning was performed, showing minor continuous reflux in the small saphenous vein of the right leg and even less reflux in the great saphenous vein on the left. The right small saphenous vein had been 7.4 mm before the operation and was 3.8 mm after. The left great saphenous vein had been 4 mm before the operation and had not decreased in size during the initial postoperative period. The patient scored pain at 3 on a 0-10 pain scale and had taken one 750 mg paracetamol tablet during the entire postoperative period. We made a full photographic record before and after the operation ( \n ). There were no photographs or records of symptoms available from the original operation.\nIn relation to the wrong-site surgery, we comforted the patient and reported the case to both the previous surgeon and the patient safety surveillance team at the hospital where the operation had been performed. |
A 35-year-old man consulted on the appearance of a right gluteal mass. He had noticed the mass some months before, but he was not concerned about it for he had considered it the result of the intensive training due to his job as a bodyguard. However, the mass had grown so much that he could no longer sit and he decided to seek medical assistance. The patient was in good general health, had no other symptoms and reported no weight loss. The physical examination confirmed a 40 × 15 cm mass in his right gluteal area and the MRI suggested a possible adipose nature for it. Incisional biopsy showed a similar histological image to that described in the previous case with myxoid and round cell areas. Diagnosis was myxoid liposarcoma and material from the biopsy was sent for cytogenetic analysis and subsequently confirmed the translocation t(12;16) (q13;p11) typical of myxoid and round cell liposarcomas. During the extension study the CAT showed a 7-cm extrapulmonary mass in the posterior mediastinum. The core needle biopsy showed the same histological image as the gluteal mass. As surgical therapy would imply amputation of the leg including the hip and the pelvis (hemipelvectomy) and the patient had a second mass in the mediastinum, neodajuvant chemotherapy was initiated with iphosphamide, adriamycin and DTIC. The control MRI indicated partial response of the mass to chemotherapy and two months after diagnosis simple resection of the gluteal mass was attempted. The histological analysis of the enucleated mass showed less than 10% of tumour necrosis related to chemotherapy and confirmed the existence of widespread areas of round cells, comprising almost 40% of the tumour. After surgery local radiotherapy was initiated with good tolerance. For one year the patient remained well and apparently disease-free, but 13 months after initial diagnosis he consulted on the appearance of a right laterocervical mass. Fine-needle aspiration suggested an adipose tumour and a cervical lymphadenectomy confirmed involvement by myxoid liposarcoma in one of twelve identified lymph nodes, without any evidence of disease elsewhere. No further therapy was initiated and the patient remained well, but 8 months after this last surgery he suddenly developed abdominal pain and the imaging techniques revealed a 18 cm adipose mass involving the left kidney. The patient was operated again for bulk reduction and underwent wide resection of the retroperitoneal mass including the left adrenal gland and kidney (Figure ). However, macroscopic residual disease persisted. Today, 25 months after initial diagnosis he remains alive with progressive disease and is under palliative care. |
A 51-year-old man came to the hospital with pain in his left shoulder while sitting and when elevating his arm, present for 2 months. While moving there was also a snapping of the left scapula. He also described slow growing tumors among the scapula on the right side for 3 years and for 2 years on the left. Former diagnostics including magnetic resonance imaging (MRI) did not show any pathological findings. His profession is a paver. There was no family history of soft tissue tumors. The physical examination showed two bilateral tumors on the inferior angle of the scapula, only visible when the arms were adducted and elevated to the front. Magnetic resonance imaging was performed to evaluate the tissue of origin and measure the size. There were two 8 × 6 × 2.5 cm tumors on the dorsolateral thoracic wall near the scapula, located between the dorsolateral middle ribs and the serratus anterior muscle. shows the MRI scan of the patient. Because no criteria of malignancy were met, no preoperative puncture was done.\nAs recommended in the literature, a surgical resection was performed in general anesthesia. The patient was placed in a prone position and the arms were elevated to let the inferior angle of the scapula slide laterally. A semilunar incision of the skin was made around the angle of the scapula, first on the left side (). Then the latissimus muscle was cut and the tumor was removed with some surrounding fat tissue. The same procedure was performed on the right side.\nThe pathological and histopathological examination of the tumors showed two mesenchymal tumors, 7 × 5.5 × 3.4 cm on the left and 7.6 × 7.4 × 3.0 cm on the right side, with fibrosis and an increased amount of elastic fibers. Both tumors were diagnosed as elastofibroma dorsi ( and ].\nPostoperative vacuum drainage of the wounds was performed for 2 days. However, a hematoma and later a seroma on the right side occurred. The hematoma was evacuated and later the seroma has to be drained. After drainage for 3 days there was no more wound secretion. No long-term complications were reported in a 6-month follow-up. |
A 67-year-old female with a past medical history of hypertension and dyslipidemia presented with nausea, vomiting, and epigastric pain for 7 days. She admitted to a history of multiple drug intolerances. A few days prior to the presentation, the patient was started on oral rosuvastatin 10 mg daily for dyslipidemia at the discretion of the primary care physician. She denied alcohol abuse or a family history of gastrointestinal disease. Upon presentation to the emergency room, vital signs were stable and physical examination was remarkable for epigastric tenderness without guarding or rigidity and normal bowel sounds. The rectal examination was unremarkable, and the stool occult blood was negative.\nThe laboratory data upon presentation to the emergency room is shown in . The serum lipase was mildly elevated at 82 U/L. Liver enzymes, including serum bilirubin, were normal. The CT scan of abdomen, with intravenous contrast, showed mild edema of pancreatic and peripancreatic tissue that was confined to the head and body of the pancreas, which was consistent with acute pancreatitis. The abdominal ultrasound showed a normal biliary tree without choledocholithiasis. Rosuvastatin was held at the time of admission. She was treated conservatively with bowel rest, intravenous fluids, and parenteral pain management. She improved clinically over next 3 days, tolerated regular diet and was discharged home. Her serum lipase levels trended down to normal (i.e., 27 U/L) during the hospital course. She was restarted on rosuvastatin at the time of discharge, as there was no strong evidence of its correlation with AP at that time except for one case report. After detailed discussions regarding the risks and benefits, the patient agreed to restart rosuvastatin at the time of discharge from the hospital.\nEight weeks later, the patient presented to the emergency room with vomiting and epigastric pain for 7 days. Her vital signs were stable, and the physical examination was remarkable for epigastric tenderness and normal bowel sounds. The serum amylase and lipase were elevated at 168 U/L and 100 U/L, respectively. The laboratory data upon presentation to the emergency room is shown in . The CT scan of abdomen, with intravenous contrast, showed mild edema of the head and body of the pancreas and mild stranding of the adjacent fat, consistent with acute pancreatitis and there was no evidence of any pancreatic mass lesion. Magnetic resonance cholangiopancreatography (MRCP) did not reveal biliary sludge or microlithiasis. The serum IgG4 levels were normal, which ruled out autoimmune pancreatitis. The patient responded to bowel rest, intravenous fluids, and pain management. She clinically improved over the next three days and was able to tolerate regular diet. Serum lipase and amylase trended down to 22 U/L and 44 U/L, respectively. After reviewing case reports of statin-induced pancreatitis and ruling out other differential diagnoses, it was determined that rosuvastatin would be stopped at the time of discharge. The strength of association between rosuvastatin and AP increased during this admission as this could be considered as a drug rechallenge and all other possible diagnoses were ruled out. After treatment with rosuvastatin was terminated, the patient did not have any further episodes of AP and was completely asymptomatic during the 18-month followup period. She remained on a strict, fat-free diet and was never started on any medication for dyslipidemia, as her LDL cholesterol remained at less than 100 mg/dL during followup. |
This is a case of a 75-year-old lady who is known to have diabetes mellitus, hypertension, hypothyroidism, adrenal suppression, previous history of stroke and a history of aortic valve replacement with mechanical prosthesis 15 years prior to presentation. She was admitted urgently to the cardiac care unit with shortness of breath on exertion class III and lower limb edema. Chest X-Ray was compatible with pulmonary edema. Echocardiogram showed a well-functioning aortic prosthesis but severe mitral valve stenosis (MS) and mitral regurgitation (MR) with severe Mitral annular calcification (MAC) (video , 2).\nThe patient was stabilized medically then underwent Cardiac CT to characterize the MAC that appeared as a dense horseshoe calcification occupying most of the circumference of the mitral annulus (Video , ). The case was discussed in the multidisciplinary rounds and she was deemed very high risk for surgical intervention with a calculated STS risk score of (19.5%). Given the patient age and frailty, it was not reasonable to decalcify the mitral annulus and reconstruct the annulus that may lead to high risk of Atrio-ventricular (AV) groove dissociation. So the decision was taken to implant the Sapien 3 valve in the mitral position under direct vision after redo sternotomy. To predict the risk of post procedure LVOT obstruction, the neo LVOT was assessed preoperatively during systole by measuring the distance from the inter- ventricular septum to the frame of the simulated transcatheter valve and then calculating the neo LVOT area which was 211 mm2 in this case. However, there is no set threshold for LVOT obstruction when this procedure is done using transatrial approach and the anterior mitral leaflet is excised since the calculated neo LVOT is underestimated due to the fact that there is flow through the cells of the valve stent frame.\nIntraoperatively, and after exposing the mitral valve, the anterior leaflet was excised and was thickened and fibrosed (Video 5). The posterior calcium bar was so prominent that made the MV orifice very small not even accommodating size 25 regular MV prosthesis sizer. We decided preoperatively to use the largest Sapien 3 valve (size 29) based on the mitral orifice size from CT scan with additional 20% oversizing. We loaded the valve on the balloon in a similar way to the transapical aortic approach and we advanced the balloon inside the ventricle until the end of the valve stents was just across the mitral annulus (Video 6). We elected not to perform a balloon predilatation to avoid fracture of the calcium body or to induce atrioventricular groove rupture. Subsequently one operator was responsible for inflating the balloon and another one was responsible for stabilizing the position of the valve across the annulus with fine tuning the direction of the valve during inflation of the balloon. The balloon was inflated until an atmospheric pressure of 4 and kept inflated for 15 s then deflated (Video 7). The valve was nicely deployed and stable in position with normal movement of the leaflets. We did not use supportive sutures to fix the valve nor utilized any patches or skirts around the valve. The patient was weaned smoothly from bypass and intraoperative transesophageal echo was performed and showed that the valve is well seated in position with no evidence of any paravalvular leak (Video , 9). The mean gradient across the valve was 4 mmHg. |
A 77-year-old male with hypertension, diabetes mellitus, was referred to our hospital for elective internal carotid artery stenting in asymptomatic severe subtotal occlusion of the left internal carotid artery (). He had a past medical history of radiation to the head and neck for laryngeal cancer. No coronary artery disease or cardiac arrhythmias were reported.\nPrevious CT-angiography had shown a type III aortic with bovine arch (). The patient was considered to be a poor candidate for carotid endarterectomy (CEA) due to a history of head and neck irradiation and a short neck, another distance of 3 cm from carotid bifurcation, made more difficult a surgical dissection (). Percutaneous carotid artery stenting (CAS) was initially attempted with access via the femoral and right radial approach. However, this procedure was unsuccessful due to a very tortuous and calcified type III aortic arch.\nCarotid artery stenting (CAS) via the transcervical approach was then performed. The patient was given general anesthesia with the head turned towards the right side. The common carotid artery was exposed using surgical cutdown and exploration at the base of neck (). A sheath was introduced into the common carotid artery with passage through the skin of the incision proximally to allow for improved stability of the sheath (). To avoid a clamping post stenting, a gate access on common carotid was prepared with the surgiclose technique, an easy and fast trick, applicable to all vessels to avoid a damage of artery , . The area of stenosis was identified with a carotid angiogram and, previous clamping of common carotid having a flow reversal, was crossed with a filter wire where a RX Spider Filter 6 Fr (Medtronic, Minneapolis, MN) was used distally in the left internal carotid artery, re-opening right after the artery. The lesion was treated with release of a 7 × 30 mm RX Xact carotid stent (Abbott Vascular, Chicago, IL). Post-dilation was then performed with a 5.5 × 30 mm Rx Submarine balloon catheter (Medtronic Minneapolis, MN), with good results (). The filter was retrieved followed by sheath removal. The arteriotomy site was easier closed thanks to surgiclose technique and hemostasis was successfully achieved (). The patient tolerated the procedure well and was successfully extubated. He was neurologically intact with National Institutes of Health Stroke Scale score of zero. Following the procedure, he remained on dual antiplatelet and statin therapy. At 3-months followup, Ultrasound doppler showed lack of restenosis with good flow intrastent (). |
A 43-year-old woman without any specific previous medical or surgical history presented to Asan Medical Center with a chief complaint of a skin tumor on her right nasal ala. A punch biopsy conducted at another institution indicated the presence of a squamous carcinoma with positive margins. On the metastasis work-up, no evidence of distant metastasis was detected. Singlestage wide excision and reconstruction was scheduled, with microsurgical reconstruction of the right ala using a helical free flap.\nPrior to surgery, the vessels adjacent to the defect and donor sites were assessed via computed tomography (CT) angiography. The superficial temporal artery and its branch to the upper portion of the helical rim could be identified on axial CT angiography (). Hand-held Doppler tracing also indicated the superficial temporal arterial branch to the helix and angular artery trunk, as well as its branch to the alae.\nWe determined the excision margin according to the methods previously described by Thomas et al. []. Briefly, after wide excision with a margin of 0.7 cm, an alar defect with a size of 1×1×0.5 cm was obtained, which included the full thickness of the skin, mucosa, and lower lateral cartilage (). Moreover, approximately 80% of the ala was missing. We designed an ipsilateral ear helical free flap nearly the same size of the defect, hoping there would be minimal contracture after microanastomosis. Vessel dissection was performed in a straightforward manner, starting from the incision margin for flap harvest, without any further dissection of reach the greater trunk of the superficial temporal artery (). The harvested flap had a vascular pedicle of an anonymous branch, running from the branch to the helix of the superficial temporal artery. Pedicle dissection was performed under microscopic magnification and a ×4 loupe.\nFor recipient vessel dissection, we dissected a branch of the angular artery through the excision defect, without the need for any additional incision to identify the trunk. An anonymous branch of the angular artery was dissected. The diameters of both the recipient and donor vessel were <0.8 mm, consistent with our inclusion criteria related to supermicrosurgery.\nMicroanastomosis was conducted for one artery and one vein. The perforator of the superficial temporal artery was anastomosed to the branch of the angular artery in an end-to-end fashion. Moreover, vein anastomosis was performed between both the venae comitantes in an end-to-end fashion (). The flap was inset in order to match the contour of the contralateral ala. We closed the donor site via rotation and advancement. shows the immediate postoperative findings after insetting.\nThe flap was monitored with inspection of the color and contour. Doppler imaging was also used to assess the state of the pedicle. In the present case, the flap survived without any venous congestion or necrosis. Moreover, the donor site did not exhibit necrosis or deformity. At 6 months and 1 year () postoperatively, no donor site morbidity was observed, despite the presence of a small scar that could easily be covered with hair. The alar contour was satisfactory, and the patient was satisfied with the results. However, the caliber of the nostril was reduced compared to the preoperative dimensions. |
A 67-year-old male had a motor scooter accident causing him to fall to the ground, landing on his shoulder. Due to excruciating pain, the subject consumed ibuprofen (200 mg) and hydrocodone 7.5 (mg) up to six times per day as required. Although the subject had surgery to repair the damage scheduled, he wanted to try an alternative to surgery. As such, about 2.5 months following the accident, the subject received one injection of PRP into the right shoulder in the hope that the PRP would alleviate the pain and induce healing. By 7 days following the PRP injection, the subject’s pain had decreased significantly, and by the end of the following 7 days he had stopped use of all analgesics. The scheduled surgery was canceled. After an additional 3 months, the patient had slight but annoying residual pain. The patient received a corticosteroid injection, and after 3 weeks was about 98% pain-free. The patient remained virtually pain-free more than 2 years and 2 months following the PRP injection.\nAbout 7 months following the accident, the subject began to feel pain in the left shoulder contralaterally to the one that had caused the extreme pain. Although the pain was intense, it was not as severe as the pain of the first shoulder. Magnetic resonance imaging indicated that the pain was due to a partial tear of the left supraspinatus muscle. The pain was treated with ibuprofen 400 mg four times per day and hydrocodone 7.4 mg every day as needed, but the pain did not decrease with time.\nAfter about 3 months of persistent pain, the patient received an injection of PRP into the second shoulder. By 1.5 months following the PRP injection, no pain relief had developed and the pain persisted at a relatively constant level for about another 5 weeks. Seeking pain relief, the subject received a corticosteroid injection into the shoulder, and within 12 days the pain had disappeared and the patient went off all analgesics. At 15 months following the corticosteroid injection, the second shoulder remained almost 100% pain-free. The subject still suffers some muscle weakness and there are some tasks, such as lifting heavy objects above his head, which he is unable to do. However, the patient considers the treatment a complete success. The patient provided written informed consent to have the case details published. |
A 10-year-old female sustained a twisting injury to her left knee as she was descending a spiral playground slide. She presented to the ED with her leg locked in extension and experiencing severe pain. She was unable to bear weight on her left leg. The patient had no other injuries but did complain of tingling in the lower left leg. She had no muscle weakness and no loss of sensation from the knee distally. On physical examination, the patella was obviously mal-positioned and was rotated 90°, facing perpendicular to its natural plane (). The performed radiograph confirmed a rotation of the patella of 90° about its vertical axis, positioned within the trochlear groove between the femoral condyles (). The Orthopaedic Service was consulted and evaluated the patient in the Emergency Department. Given the patient’s thin body habitus, the patella could be easily palpated and it was determined that the articular surface was facing laterally. A reduction was attempted in the Emergency Department under conscious sedation with etomidate. The patella was successfully reduced via vertical traction to lift the medial side and lateral pressure on the anterolateral edge of the patella to allow it to rotate back to its anatomic position. Reduction was confirmed with radiographic imaging () and restoration of normal motion of the knee passively. After waking from sedation, the patient reported that her pain had markedly improved. A knee immobilizer was placed after reduction and she was discharged from the ED.\nUpon follow-up in clinic, an MRI was ordered and revealed medial patella and anterolateral femoral condyle bone contusions, mild femoral trochlear dysplasia, and medial patella femoral ligament sprain. There were no osteochondral defects or loose bodies detected in the MRI. The knee immobilizer was then transitioned to a hinged knee brace and she was able to begin range of motion exercises. She was referred to physical therapy for strengthening and patella stabilization. At four-month followup, the patient had returned to normal activities with full range of motion and no limitations in function nor pain symptoms. She subsequently had no recurrent episodes of dislocation or pain symptoms and had not needed follow-up at 24-months post-injury |
A 62-year-old man, affected dysphagia, was endoscopically diagnosed with lower esophageal cancer confirmed squamous cell carcinoma on biopsy, and was reffered to our hospital (Fig. ). He had no medical history. His lifestyle has included 1500 ml beer consumption per day and 40 cigarettes per day for the past 40 years. Computed tomography (CT) showed thickening of the wall in the lower esophagus as the primary lesion was demonstrated and the tumor formed a mass with the solitary metastatic abdominal lymph node, and invaded pancreas body and gastric body (Fig. ). No other distant metastasis was detected on CT. He was diagnosed with lower esophageal cancer cT4 N1 M0, with pancreatic invasion, cStage IIIC according to 7th edition of the Union for International Cancer Control system []. At first, we considered definitive chemoradiotherapy. However, radiation oncologists evaluated that the tumor was less candidate for chemoradiotherapy because of the risk of gastric mucosal damage. For the purpose of definitive therapy, radical esophagectomy with distal pancreatectomy was planned. As neoadjuvant chemotherapy, CF therapy (cisplatin and 5-fluorouracil therapy; cisplatin was dripped 80 mg/m2 plus 5-fluorouracil was infused 800 mg/m2 on day 1 through 4 continuously) was started according to standard therapy of localized advanced esophageal cancer []. However, after once administration, he could not continue chemotherapy for the exacerbation of dysphagia, and underwent radical surgery. Preoperative evaluation of tumor was similar to initial findings on endoscopy and CT.\nIn findings on laparotomy, abdominal lymph node was infiltrated directly to pancreas body. As the radical surgery, Ivor Lewis esophagectomy with distal pancreatectomy and splenectomy, followed by reconstruction of gastric conduit. Two fields lymphadenectomy was performed according to the treatment strategy of the abdominal esophageal cancer. Reconstruction of gastric conduit was possible although the lymph node was adherent to lesser side of gastric body, which was resected when reconstruction of gastric conduit. In addition, partial resection of lung was performed simultaneously owing to involvement of bilateral pulmonary ligaments to the primary tumor (Fig. ). The operative time was 528 min. The estimated blood loss was 2850 ml, and the patient was transfused 4 units of red blood cell concentrates.\nThe patient had an uneventful postoperative course and was able to take orally. He was discharged 16 days after operation with tube-free. After discharge, he underwent two courses of CF therapy as adjuvant chemotherapy. At present, he is still alive and has no recurrence for 7 years after surgery.\nIn the resected specimen, the primary lesion was observed from lower esophagus to esophagogastric junction, and metastatic lymph node was fixed to stomach and pancreas body (Fig. ). Pathological examination revealed that primary lesion was not infiltrated to lung (Fig. a, b). Massive metastatic lymph node (over 5cm) was observed in the lesser curvature of stomach, and infiltrated to pancreas and gastric wall with extranodal extension (Fig. c, d). The tumor was diagnosed with squamous cell carcinoma, moderately differentiated type. The vascular and lymphatic invasion was confirmed. The surgical margin was negative. The pathological stage was ypT4 N1 (1/61) M0 (metastatic lymph node invasion into pancreas), ypStage IIIC. The histopathological response of chemotherapy was grade 1a, which was equivalent that proliferable cells were 2/3 or more, in 7th edition of the Union for International Cancer Control system []. |
A 76-year-old man with a past medical history of coronary artery disease, hypertension, and severe septal hypertrophy suspicious for hypertrophic cardiomyopathy, presented to the hospital with progressive symptoms of heart failure. He had been suffering from worsening dyspnea and lower extremity edema for several months. Lab work over the preceding months had shown deranged liver function tests, concerning for right heart failure. He was admitted for intravenous diuretic and inotrope therapy. At his cardiology clinic appointment prior to this hospital admission, an ECG showed typical atrial flutter and diffuse low voltage []. A right heart catheterization showed moderately elevated right greater than left filling pressures with a right atrial pressure 18 mmHg, pulmonary capillary wedge pressure 21 mmHg and a Fick cardiac index 1.6 liters/min/m2. The ScvO2 was 48%.\nOf note, a year prior to this admission, a transthoracic echocardiogram (TEE) had shown severe asymmetric septal hypertrophy (2.6 cm), suspicious for hypertrophic cardiomyopathy, but had not been further evaluated. A repeat TEE at the present admission showed a left ventricular ejection fraction (LVEF) of 35% as well as the aforementioned septal hypertrophy []. However, due to the significant low voltage on ECG, despite the significant LVH on echocardiogram and history of hypertension, a cardiac MRI was ordered to rule out infiltrative cardiomyopathy as opposed to hypertrophic cardiomyopathy.\nThe images showed normal left ventricular chamber size with severe, asymmetric left ventricular hypertrophy, primarily involving the septal wall with a maximum septal thickness of 27 mm []. The LVEF was measured at 40%. Also noted was diffuse, global late gadolinium enhancement of the left ventricular myocardium, consistent with a diagnosis of cardiac amyloidosis []. The only extracardiac clinical finding suggestive of systemic amyloidosis was carpal tunnel syndrome. Serum and urine electrophoresis did not detect a monoclonal protein, and serum free light chain ratio was low, which significantly lowered the suspicion for AL-amyloidosis. An abdominal fat pad biopsy was ordered which came back negative. Finally, an endomyocardial biopsy was performed which showed a pink amorphous interstitial infiltrate exhibiting apple green birefringence with Congo red stain, confirming cardiac amyloidosis. The subtype was likely wild-type ATTR or variant ATTR amyloidosis.\nIncidentally, the cardiac MRI also found a left upper lobe mass which, on further workup, was characterized as adenocarcinoma. No further differentiation of the subtype of ATTR amyloidosis was pursued, as this would not have changed management, especially in view of the poor prognosis portended by the adenocarcinoma. |
A 32-year-old primigravida was referred for fetal echocardiography at 23 weeks of gestation in view of right heart enlargement and inability to demonstrate drainage of pulmonary veins to the left atrium. Fetal echocardiography performed at our center showed significant right-left chamber asymmetry with dilatation of the right-sided chambers in the four-chamber view [ and ]. The left atrium was small with a smooth posterior wall, and the pulmonary veins were not seen connecting to the left atrium []. The outflow tracts were normally related and unobstructed and there were no abnormal systemic venous connections. The area behind the heart showed a common chamber measuring 1.5 mm in diameter between the LA and the descending aorta []. The pulmonary veins were demonstrated as joining this common chamber []. The superior vena cava (SVC) appeared dilated, and a channel with turbulent high velocity was seen draining into the SVC in the three-vessel trachea view [ and ]. Doppler evaluation of this channel showed continuous flow into the SVC, suggesting obstruction at the site of joining of the channel into the SVC []. A diagnosis of the obstructed type of supracardiac TAPVC was made. The findings were re-confirmed in a repeat scan at 34 weeks, and the parents were counseled about the expected postnatal course and the need for urgent surgical intervention after birth. Following prenatal counseling, the family opted for postnatal cardiac care and surgical correction. Hence, the delivery was planned in a tertiary pediatric cardiac surgical facility for expediting postnatal cardiac care and avoiding the risks of neonatal transport.\nAt 39 weeks' gestation, the mother was admitted in our hospital with a history of premature rupture of membranes. In view of significant cephalopelvic disproportion, an emergency cesarean section was performed, and a male baby weighing 2.4 Kg was delivered. The Apgar score at 1 and 5 min after birth was 8 and 9, respectively. The baby presented with significant cyanosis, tachypnea with a respiratory rate of 60–70 breaths/min, suprasternal and subcostal retractions. Pulse oximetry in room air showed a saturation of 70% immediately after birth. The baby's respiratory distress worsened rapidly, requiring mechanical ventilation. The chest radiography revealed signs of severe pulmonary venous congestion. Postnatal echocardiography confirmed the prenatal findings. The SVC was markedly dilated []. The suprasternal view revealed a vertical vein ascending to the right of the SVC; the vertical vein appeared to have a tortuous course before draining into the SVC []. The drainage appeared obstructed at the ascending vertical vein on color flow mapping []. There was evidence of severe pulmonary arterial hypertension with a moderate-sized ASD. Computed tomography (CT) angiogram was done in order to confirm the pulmonary vein anatomy and delineate the anatomy of the vertical vein more clearly. CT revealed the presence of reasonable sized individual pulmonary veins which joined together from both sides at multiple levels with a small confluent chamber. The vertical vein was narrow and tortuous and it ascended to the right of the midline to join the right-sided SVC [Figure –]. A tight stenosis was noted at the confluence site measuring approximately 2.2 mm. Three-dimensional rendering showed that the vertical vein was very thin along the entire length, causing obstruction throughout its course.\nIn view of the unstable hemodyanamics and low oxygen saturations despite ventilation, the neonate underwent an emergency open-heart surgery within 2 h after birth. The anatomic findings were confirmed on surgery. A side-to-side anastomosis was performed between the pulmonary venous confluence and left atrium. The vertical vein was identified lateral to the right SVC and looped. ASD was closed using pericardial patch leaving behind a small ASD. The intraoperative course was uneventful, except for systemic PA pressures while coming off bypass. The patient had supra systemic PA pressures in the immediate postoperative period, and hence, the sternum was kept open for 3 days. Poststernal closure, the baby was gradually weaned and extubated on the 11th postop day with stable hemodynamics. The baby required high flow nasal canula support for about 6 weeks due to persisting mild respiratory distress. The child was discharged on the 45th postoperative day and at the time of discharge, the baby was active, alert and feeding well. Postoperative echocardiogram revealed unobstructed pulmonary vein anastomosis with no residual PAH. At 5 months follow-up, the baby had gained weight (4.8 kg) and there were no residual pulmonary vein obstruction or pulmonary hypertension on follow-up echocardiogram. Cardiac medications were weaned off, and the baby is scheduled for further follow-up for the reassessment of PA pressures at 1 year of age. |
A 57-year-old female with widely metastatic high-grade serous adenocarcinoma of the ovary was referred to our tertiary palliative care clinic by her oncologist for management of severe cancer-related pain. Following her original diagnosis, she underwent a number of multimodal disease-directed therapies, including surgical resection and chemotherapy. She had significant ongoing mixed somatic and neuropathic pain in the left lower back as well as her pelvis, both sites of known metastatic disease. The back pain was noted to be a deep sharp pain without radiation to her extremities, located primarily around the region of her nephrostomy tube and into the abdomen. She would also have sharp lancinating neuropathic pain in her pelvis due to tumor burden with prolonged periods of sitting, walking, or activity.\nInitial treatment consisted of multimodal pharmacologic therapy including short-acting and long-acting opioids with nonopioid and adjuvant neuropathic agents. Despite these therapies, she had progressive pain and was ultimately increased to an opioid combination of morphine sulfate controlled-release (120 mg by mouth every 8 hours) and morphine sulfate immediate-release (30–45 mg by mouth every 3 hours as needed). Early changes yielded acceptable analgesia. Conversations and medication adjustments continued over the phone, given the distance between the patient's home and our institution. However, over several weeks' time, pain progressed despite further opioid increases. Given concern for escalating pain and poor response to several attempts at altering her opioid therapy, it was advised that she present for a consultation in the palliative medicine clinic. Fortunately, focused discussions with the patient and her family around the patient's goals of care were addressed with her palliative medicine team at every meeting. She was very clear that her top priority in her ongoing medical care was to achieve acceptable analgesia. During the interview in the clinic, the patient noted significant pain with generalized discomfort, a sense of restlessness, and new muscle fasciculations. Her husband accompanied her and noted intermittent confusion. Her physical exam featured delirium (Confusion Assessment Method positive) with diffuse myoclonus.\nOut of concern for opioid-induced neurotoxicity (OIN), the patient was admitted to the hospital for analgesic control and treatment of what was felt to be a toxic encephalopathy. Despite the known shared metabolic pathway (phase II metabolism) between morphine and hydromorphone, the patient's opioids were rotated from oral morphine to parenteral hydromorphone as the clinical picture continued to unfold. Further workup revealed an unremarkable head CT and EEG. Clinical evaluations and laboratory assessments suggested that, in the setting of her declining physical and renal function (creatinine 1.0 mg/dL with prior baseline 0.6 mg/dL and estimated glomerular filtration rate (eGFR) 55 ml/min/BSA with prior baseline >60 ml/min/BSA), the delirium could be the result of an accumulation of active neurotoxic morphine metabolites (morphine-3-glucuronide). In addition, she was dehydrated, constipated, and had not slept in several days. Unfortunately, her delirium persisted despite correction of metabolic derangements, hydration, and an aggressive bowel regimen.\nUrology was consulted, and a nuclear medicine dimercaptosuccinic acid (DMSA) scan revealed minimal function of the left kidney, despite the presence of a nephrostomy tube. Subsequent vascular radiological investigations revealed tumor-induced thrombosis with surmised infarction of her left kidney. It was concluded that the altered renal function due to infarction likely resulted in an inability to adequately excrete the morphine, and the accumulation of polar morphine metabolites resulted in OIN. As exemplified in this case, even with opioid rotation, centrally active metabolites of hydromorphone (hydromorphone-3-glucuronide) theoretically exist, given that it follows a similar phase II metabolic glucuronidation pathway as morphine, although the relative potency and effect of such hydromorphone metabolites are thought to be significantly less than morphine. The patient continued to have signs of OIN and was eventually transitioned to parenteral fentanyl, given that fentanyl is a unique medication that is largely hepatically metabolized into inactive metabolites. Despite common lore that fentanyl and methadone are agents that do no produce active metabolites and therefore do not elicit OIN, case reports of synthetic opioids eliciting OIN do exist in the literature [, ]. Unfortunately, despite aggressive supportive measures and conversion of parenteral hydromorphone to parenteral fentanyl without reduction for cross-tolerance, she continued to have persistent pain with minimal resolution of her systemic neurotoxicity.\nGiven the concern for poorly controlled cancer-associated pain, the palliative care and pain medicine teams collaborated from the initial visit with concerning symptoms of OIN. At our institution, the palliative clinic shares space within the pain clinic, and the collaboration required a simple conversation followed by same day pain consultation. Initially, the shared thought was moving directly to intrathecal (IT) TDD therapy, as this had been discussed over numerous palliative care visits as a potential option with her physicians and was in line with her goals to achieve enhanced pain control with limited side effects. However, given the underlying metastatic disease process and concurrent cancer treatments, she had a metabolic coagulopathy due to nutritional and micronutrient deficits that required correction with vitamin k to assist in the reversal of her international normalized ratio (INR), thus allowing for any type of procedural intervention.\nUltimately, given the persistent multifactorial delirium and rapidly changing situation, the pain medicine and palliative care teams convened a family conference with the husband, the patients' health-care power of attorney, to again address the current situation and make recommendations that seemed consistent with the patient's predetermined goals. Given the rapid progression of her symptoms despite aggressive attempts to correct potentially remediable factors, the shared medical decision-making discussion included moving forward with TDD, given that this therapy was thought to be the best chance for meeting the patients' goals for comfort and hope to have meaningful interaction with family by reducing the burden of systemic side effects from oral or parenteral opioid therapy. The patient's advanced directive was clear that she appointed her husband to make decisions on her behalf if she was ever in a situation where she was unable to provide consent. Informed consent was obtained through her appointed surrogate decision maker and designated health-care power of attorney.\nAfter correction of her metabolic coagulopathy yielding an INR <1.2, she proceeded with placement of an intrathecal TDD system (Medtronic SynchroMed™ II 40 mL pump and Ascenda catheter). The pump reservoir was placed in her right lower abdominal quadrant, and the catheter tip was placed at T10. Her TDD system was efficiently titrated to achieve acceptable levels of analgesia using a combination of opioid (hydromorphone 2 mg/mL) and local anesthetic (bupivacaine 10 mg/mL). The initial postoperative settings were 0.5 mg/day of hydromorphone in a simple continuous mode without bolus dosing (2.5 mg/day of bupivacaine). Within 24 hours of TDD placement, the patient experienced near complete resolution of her toxic encephalopathy and was able to engage in meaningful conversation with her family and health-care teams. There were no signs or symptoms of opioid withdrawal. In the early postoperative phase, she required additional dosing of oral hydromorphone, but was responsive to 2 mg orally every 3 hours as needed for breakthrough postsurgical pain totaling three to four doses per day. Given the robust response to TDD therapy, the patient was able to participate in goals of care discussions with her interdisciplinary palliative care team and elected to discharge closer to home under the provision of hospice services postoperative day (POD) two. Prior to leaving the hospital, the TDD rate was increased to 0.8 mg/day of hydromorphone, and she was provided with a personal therapy manager (PTM) allowing 0.1 mg/dose every six hours totaling four doses per day. The dose remained stable for seven days. As her oncologic disease continued to evolve, her TDD system was adjusted by her hospice provider to accommodate her daily use of oral opioid therapy. The pump was again adjusted on postoperative day nine, yielding a basal rate of 1.4 mg/day of hydromorphone with four PTM boluses of 0.15 mg/dose of hydromorphone every six hours. This dose remained for POD 9–13, and she passed away peacefully with acceptable analgesia at home with her family under the auspices of hospice two weeks after TDD implantation. |
A 58-year-old male was admitted to our hospital in April 2007 with a suspected metastasis of a sigmoid cancer in the Couinaud segment (CS) 7 found during a follow-up examination using ultrasound. The patient had undergone a resection of the colon sigmoideum due to carcinoma in 2003. In addition to the liver metastasis, the patient suffered from mild hypertension and non-insulin-dependent diabetes mellitus. The only preoperative imaging was an abdominal ultrasound; thus, an additional CT scan of the abdomen was performed in preparation for the surgical intervention. The metastasis in the CS 7 had a size of 2.2 × 2.4 cm and was closely attached to the right hepatic vein (Figure ). After careful analysis of the CT scan, it became clear that the proximal part of the middle hepatic vein was completely missing, whereas its distal branches seemed to be open. In order to get more detailed information about the anatomical situation, an additional 3D imaging of this case was performed. It was evident that the middle hepatic vein was missing. Most likely this anatomical pattern was not tumour related because there was a close proximity of metastasis and right hepatic vein (Figure ) making the resection of the right hepatic vein inevitable. As the non-existence of a middle hepatic vein may have led to insufficient drainage of remnant hepatic parenchyma following a bisegmental resection of CS 6 and 7, it was decided to perform a right-sided hemihepatectomy. The pathologic examination confirmed the diagnosis of a colorectal liver metastasis. The surrounding liver parenchyma did not show any distinctive features, and in particular, no evidence of liver cirrhosis. The intra- and postoperative courses of the patient were uneventful. The patient was discharged on the eighth postoperative day.\nThe according CT scan was made available by the Radiology Department of the German Cancer Research Center, Heidelberg. The image data was acquired preoperatively during the routinely performed CT scan.\nImage data were acquired with a Toshiba Aquilion 16 slice multidetector CT scanner (Toshiba, Japan). A standard bi- or triphasic liver scan with an optimized portal venous phase was performed. SureStart bolus tracking technique (130 ml Imeron 300, Altana, Germany, flow rate 4–5 ml/s) was used in all protocols to optimize vascular contrast [].\nImaging data sets were transferred to the Department of Medical and Biological Informatics of the German Cancer Research Center using the CHILI [] teleradiology system (CHILI GmbH, Heidelberg, Germany). Segmentation of the liver was performed using interactive region growing techniques as previously described by our group []. The vessels were segmented using a grey-value-based volume-growing technique. The segmentation was then transformed automatically into a symbolic representation of the vascular anatomy containing the vessel paths, and locations of bifurcations as well as the vessels diameters []. The origin of the left, middle, and right hepatic veins were identified, clicked upon and all depending vessels were marked automatically as branches of that particular vein. Portal veins and arteries were defined in the same manner. All results were validated by a team consisting of surgeons, radiologists and medical computing specialists.\nSince the whole process from CT data to 3D viszualization preserves all positional information, 3D visualization and original CT data can be presented in a consistent manner. Several tools are available for the interactive exploration of the individual patient anatomy. One of these tools, the "re-localization tool", allowed us to define any volume element (voxel) in the CT scan and find this particular voxel within the 3D visualization. This is of special importance because the localization of intrahepatic venous shunts is more easily achieved with the help of 3D images. |
A 49-year-old man was referred to our department with persistent serous fluid discharge via a Penrose drain that had been intraoperatively placed into the operation site and had remained until postoperative day 10 after orthopedic internal fixation with metal screws for multiple pelvic bone fractures. The creatinine and blood urea nitrogen values of the discharged fluid were 25.56 mg/mL and 86 mg/mL, respectively. Subsequently, intravenous urography was performed, which showed urinary extravasation arising from the right distal ureter suspicious of a right distal ureteric injury (). Renal ultrasonography was also performed, which confirmed grade I hydronephrosis of the right kidney. Following this, a percutaneous nephrostomy (PCN) tube was successfully inserted to divert the urine output into an external drainage bag. To further specify the location and severity of the right distal ureteric injury, an antegrade nephrostogram was performed via the PCN tube, which clearly demonstrated extravasation of contrast material around the right distal ureter (). However, an antegrade attempt to internalize the right ureter by placement of a double J ureteric stent failed. After detailed discussion with the patient, it was decided to proceed with an emergent reconstructive operation to repair the right distal ureteric injury. A midline laparotomy was performed to obtain adequate surgical exposure of the injured right ureter. After careful dissection, the right ureter was found to be displaced medially by a well-formed urinoma secondary to urinary leakage along the right pelvic side wall. In addition, reactive inflammatory changes adjacent to the injured ureter had resulted in adhesions that made ureteric dissection difficult. Two penetrating holes leaking urine at the same level of the right distal ureter were intraoperatively identified. After complete evacuation of the urinoma, approximately 2 cm of the injured ureteral segment was excised and an additional ureteral dissection was made so that tension-free ureteral anastomosis could be safely achieved. Eventually, an end-to-end ureteroureterostomy was performed over a 6-French double J ureteral stent (). The patient recovered fully without complications or adverse sequelae. |
We present here the case of a 26-year-old female who attended the outpatient department seeking consultation for a growth in the floor of the mouth that had existed for about 3 years, although she had not noted any significant increase in size over the last few years. She had been having altered speech and functional difficulty since last two years and she believes that the tumoration on the floor of his mouth was the cause of it all. On extraoral inspection, her neck appeared short and thick, giving a double chin appearance []. There was no cervical lymphadenopathy. Intraoral examination showed a large swelling involving the submental, submandibular, and sublingual areas measuring 3 × 3 cm2 in diameter, adhered to the deep planes, painless, and displacing her tongue upward. The tongue was elevated to the extent that the soft palate could not be visualized. The swelling was found to be smooth, nontender, and the mucosa on the floor of the mouth was normal []. On application of pressure in the submental region, the growth was getting displaced upward. The size and appearance of the orifices of Wharton's ducts were normal.\nAn ultrasonography was performed, which revealed cystic area with echogenic material and internal echoes within it. There was no evidence of flow within the cystic area on the color doppler []. MRI showed an encapsulated cystic mass without any calcification [].\nThe differential diagnosis included lipoma, ranula, thyroglossal duct cyst, cystic hygroma, branchial cleft cysts, and benign and malignant tumors of the floor of the mouth and adjacent salivary glands. Surgical excision of the lesion was performed through an intraoral midline incision under general anesthesia []. Blunt dissection was utilized to free and remove the mass, and then the wound was sutured in layers with a corrugated rubber drain placed in position. Macroscopically, the lesion appeared encapsulated and contained a keratin-like yellow material []. The anatomical pathology diagnosis was a dermoid cyst. Histopathology revealed that it is an epidermal cyst lined by stratified squamous epithelium with the lumen containing kerati [] histopathology course was uneventful, and there was no evidence of recurrence at a period of 4 months after surgery]. |
A 31-year-old man was admitted to our hospital, complaining of hematochezia which had lasted for 1 month. His past history involved a high anterior resection of the rectum in our hospital due to intussusception caused by Peutz-Jeghers polyposis (Fig. ). He was hospitalized for 1 month after the procedure. Unfortunately, details of the surgical procedure that had been performed and the reason for his extended postoperative hospitalization were unknown, because clinical records from his previous admission were not available. He was followed up for 3 years after the procedure, during which he had no abdominal symptoms. He remained symptom-free until the month prior to readmission when he began to suffer from hematochezia. On this admission, hematochezia was his only symptom and there were no abnormal abdominal findings on physical examination. Blood test results, including levels of tumor markers, were all within normal limits. Colonoscopy revealed a hemorrhagic tumor with a smooth surface protruding from the anastomosis of the previous high anterior resection, at a distance of 10 cm from the anal verge; a second examination 4 days later revealed that the tumor had disappeared (Fig. ). The biopsied tumor and other small polyps were histologically diagnosed as adenocarcinoma and hamartomatous polyps, respectively (data not shown). We performed a low anterior resection of the rectum, including the anastomotic site with the adenocarcinoma, combined with a resection of the ileum for strong adhesion. The patient was discharged from our hospital 42 days after the operation.\nGross appearance of the resected rectum showed a defect of the rectal mucosa with a smooth edge and a mucosal bulge located at the anastomotic site (Fig. ). Cut surfaces demonstrated a submucosal tumor mainly occupying the proper muscle layer under the defect (Fig. ). Microscopically, the submucosal tumor comprised an adenocarcinoma and a bone lesion at the anastomotic site (Fig. ). The surface of the tumor was covered with granulation tissue (Fig. ). The bone lesion not only included the carcinomatous glands but also normal glands in the bone tissue (Fig. ). In addition, we identified the incorporation of the normal mucosa in the submucosal fibrosis at the anastomotic site (Fig. f). |
A 32-year-old man was referred to Seoul National University Dental Hospital for further evaluation and treatment of bilateral nodular lesions of the neck that had been present for several months. He had visited a local plastic surgery clinic with a chief complaint of a large lesion and pain in the right cervical area. Documentation from the clinic stated that he had only agreed to a drainage procedure and had refused surgery to remove the lesions. According to the patient, the pain and swelling had subsided as a result of the aspiration performed during his visit to the clinic. Additionally, while the patient had an operative history of cardiac septal defect repair performed when he was 10 months old, he gave no family history associated with skin lesions like those that he exhibited.\nPanoramic radiography showed abnormal dentition. First, the deciduous second molars were in a state of prolonged retention and submergence on both sides of the maxilla and the right side of the mandible. Second, multiple permanent teeth were missing, including the maxillary second premolars on both sides and 2 mandibular incisors on both sides and 1 mandibular molar on the right side. Finally, microdontia of 2 maxillary lateral incisors (peg lateral incisors) and 2 mandibular premolars was observed ().\nTo accurately evaluate the soft-tissue lesions, contrast-enhanced computed tomography (CECT) was performed. The images revealed multiple well-defined round to ovoid, smooth or scalloped, nodular lesions with diameters ranging from 2 to 20 mm in the subcutaneous layer on both sides of the cervical region. The internal structure showed fat attenuation on the level of −110 to −100 Hounsfield units (HU) and some lobulation. Additionally, the large lesion of the right upper neck, where the patient experienced the pain, displayed peripheral enhancement with mild swelling, suggesting that it was accompanied by inflammation. The epidermis of that lesion was elevated, and the adjacent platysma muscle and external jugular vein were displaced medially (). In other words, the benign lesion exerted a pushing force on adjacent structures. Based on these radiographic findings, the initial diagnosis was lipomatosis.\nFor microscopic examination, excisional biopsy was performed on a lesion (7×5×4 mm) on the right side of the neck, and hematoxylin and eosin staining was used. The histological finding was a subcutaneous cystic lesion containing sebum-like material. At the lowest magnification (×12.5), a cavity surrounded by a thin epithelial lining that formed part of the cyst wall was observed. The middle magnification settings (×40 and ×100) showed the specimen to have a wall lined by 3 to 5 layers of keratinized stratified squamous epithelium lacking a granular layer, and many sebaceous lobules were located very close to, as well as within, the cyst wall. In addition, a wavy luminal surface and sebum-like material or keratin debris were detected. The highest magnification (×400) showed numerous lobules of pilosebaceous glands on the cyst wall. The granular layer was completely absent (). Accordingly, the analysis resulted in a diagnosis of steatocystoma multiplex.\nThe patient revisited with bleeding of the above-mentioned large lesion of the right neck 6 months after the biopsy. The aspiration contained serous fluid, blood, and sebum-like material. His discomfort was relieved with medication a few days later, and he required no further visits. |
A 25-year-old female Irish patient presented with recurrent episodes of rhabdomyolysis since childhood. The first episode occurred at the age of 22 months following a respiratory tract infection. At that time, her creatine kinase (CK) serum concentration was noted to be 250 000 U/L.\nThe family history indicated that both parents were well. However, two siblings (brother and sister) had sudden death at the age of 2 and 4 years more than 20 years ago following a short infectious illness with sudden deterioration over a period of hours. In both cases, the children became progressively weak with severe muscle pain and had evidence of rhabdomyolysis and myoglobinuria of uncertain etiology. Skeletal muscle histology and electron microscopy studies at postmortem evaluation were normal in both children, cardiac evaluation demonstrated dilated cardiomyopathy. Notes pertaining to their clinical episodes were not available. On first presentation, our patient had no hypoglycemia and no ketosis during the acute illness, but was initially treated as a possible long chain fatty acid oxidation defect due to the family history.\nThe patient had numerous subsequent admissions with similar presentations of extremely elevated CK concentration (1 000 000 U/L at the age of 9) associated with muscle pain. This was despite aggressive carbohydrate supplementation including nocturnal cornstarch. Her fat intake from food was continually restricted to approximately 40 g/d. Her total fat intake was supplemented with the use of MCT Oil (medium chain triglycerides supplement) and essential fatty acids in the form of walnut oil. Coenzyme Q10 at a dose of 100-200 mg daily was provided on an ongoing basis. The CK concentration was also raised between the episodes (500-2000 U/L). She was advised to limit her exercise to 20 minutes per day and have high-calorie drink prior to any physical activity.\nExtensive investigations were performed over the presenting years with the lack of a definite etiology. Serial urine organic acid profile and the acylcarnitine profiles were normal. A fibroblast fatty acid oxidation study showed normal myristate and palmitate oxidation studies in fibroblasts. Genetic analysis for fatty acid oxidation defect (LCHAD, MCAD, CPT I, and CPT II) and McArdle disease was uninformative.\nAt the age of 16 years, mitochondrial respiratory chain activity measured in a muscle biopsy was normal, however morphological findings, such as intramyocellular lipid, were compatible with lipin-1 deficiency (Figure ). Her echocardiogram and electrocardiogram did not show any abnormal findings.\nAt the age of 19 years, DNA sequence analysis of the LPIN1 gene (all coding exons and flanking intron boundaries corresponding to the canonical transcript variant NM_145693.3) revealed the presence of a common pathogenic intragenic deletion within the LPIN1 gene (c.2295-866_2410-30del1763) encompassing exon 18 (HGMD accession: CG085181). However, a second mutation could not be identified.\nSubsequently, the LPIN1 coding region was analyzed by reverse-transcriptase PCR (RT-PCR) from total RNA isolated from muscle tissue and conventional DNA sequencing (Figure A,B). In addition to transcripts lacking exon 18 or exons 18-19 (corresponding to the allele harboring the genomic exon 18 deletion), an alternative exon spliced in between exon 5 and exon 6 was detected in a significant proportion of transcripts (Figure C,D). Because this alternative exon, named exon 5a, corresponded to an alternatively spliced in-frame exon annotated only in an N-terminal LPIN1 transcript variant (NM_001261428.2), further targeted DNA-based sequencing was performed. Indeed, this identified a second heterozygous variant (NC_000002.11:g.11916284C>A), which was formally regarded a nonsense mutation introducing a premature stop-codon within exon 5a (which would correspond to NM_001261428.2:c.942C>A, NP_001248357.1:p.[Cys314*]; Figure ). RT-PCR of total RNA isolated from healthy skeletal muscle detected exon 5a also in transcripts containing the first (noncoding) exon of the canonical isoform (data not shown). However, because exon 5a is currently not annotated to be contained in this main reference transcript (NM_145693.3), further studies will be needed to clearly establish pathogenicity of the novel variant.\nSegregation analysis in the patient's family revealed that her father and younger sister are heterozygous for the common pathogenic intragenic deletion (NM_145693.3:c.2295-866_2410-30del1763), while her mother was a heterozygous carrier of the variant within the alternative exon. Thus, the results of these molecular genetic analyses were formally consistent with the clinical diagnosis of LPIN1-associated rhabdomyolysis, due to compound heterozygosity for a known pathogenic deletion and potentially pathogenic novel mutation.\nAt the age of 25 years, the patient presented with acute muscle pain and weakness following prolonged fasting and strenuous exercise. A rhabdomyolysis crisis was confirmed with a CK of 500 000 U/L. She was admitted to intensive care unit (ICU) for a 2 week period over which period she lost a significant amount of weight. During her critical illness, she received an intravenous infusion of 10% dextrose at 1.5 times maintenance with added electrolytes, sodium bicarbonate, morphine, and dexamethasone. Intravenous carnitine was also provided as the patient had previously observed this to be clinically beneficial. She was treated symptomatically, with shortened fasting periods, corn-starch at night and an “unwell dietary regimes” with a mild restriction of fat (40 g per day) and supplementation with MCT oil and walnut oil for essential fatty acids and Liquigen 5 g daily. On day 11, her CK was monitored 4 hourly and was 1248 U/L. On discharge from ICU, this patient had generalized muscle weakness, stiff lower limb muscles (gastrocnemius), and bilateral drop foot requiring orthotic splints. Muscle weakness gradually improved after months of rehabilitative physiotherapy. Her drop foot has improved somewhat (power from 0/5 to 3/5 for dorsiflexion and extensor hallicis longus over 1 year) with areas of altered sensation anterolaterally below the knees consistent with bilateral residual common peroneal neuropathies (CPN). Serial nerve conduction/electromyography studies demonstrated bilateral CPN palsies and a background generalized myopathy. The most likely cause of this patient's weakness was a critical care neuromyopathy, which has improved with time, in addition to CPN palsies related to significant weight loss while critically ill. However, we cannot exclude the possibility that both defects were related to her lipin-1 deficiency. While myopathy has been reported in a few cases, CPN damage is not a known association of this rare metabolic disorder. |
A 37-year-old man was admitted to our hospital with long-standing, persistent, lone atrial fibrillation refractory to medical therapy and several attempts of cardioversion. The patient underwent a total thoracoscopic epicardial ablation without any intraoperative or postoperative complications, followed by a percutaneous postprocedural electrophysiologic evaluation that included confirmation of pulmonary vein isolation and a cavotricuspid isthmus block on the tenth postoperative day. He was discharged in sinus rhythm six days after the hybrid procedure.\nTwo weeks after discharge, the patient was admitted to the emergency room with right-sided motor weakness and paresthesia of both upper and lower extremities. His fever reached 40°C, and he had a white blood cell count of 17,690/μL. An electrocardiogram showed atrial fibrillation. Computed tomography imaging of the chest and brain was performed. Chest computed tomography imaging revealed a collection of loculated air measuring up to 2 cm in diameter between the right side of the left atrium and the esophagus, with no definitive evidence of a fistulous connection (). Brain computed tomography revealed an acute-onset left middle cerebral artery infarction. Emergency echocardiography revealed many floating microbubbles.\nAn urgent surgical intervention was performed due to our high level of suspicion for a left atrial or pulmonary venous-esophageal fistula. The right femoral area was prepared for the possibility of an emergent cardiopulmonary bypass. The chest was opened through the fifth intercostal space via a right posterolateral thoracotomy under one-lung ventilation with a double lumen endotracheal tube. After anteriorly retracting the lung, a meticulous dissection of the posterior mediastinum between the esophagus, right upper and lower pulmonary veins, and left atrium was performed. A fistula 2 to 3 mm in diameter was discovered between the right lower pulmonary vein and anterior surface of the esophagus. The fistula was divided with Metzenbaum scissors (). Surprisingly, there was no bleeding from the fistula on the side of the right lower pulmonary vein. The internal orifice was very small, with a diameter of approximately 1 mm. The right lower pulmonary vein was repaired with a bovine pericardial patch, covered, and fixed with continuous poly-propylene sutures. After repairing the right lower pulmonary vein, we consulted our hospital’s thoracic surgeon about repairing the fistula opening on the esophageal side. He decided to carry out a primary repair of this defect because the defect site was small and relatively clear. The fistula was carefully debrided and repaired with intermittent 4-0 black silk sutures.\nThe operation was successful and the postoperative course was uneventful. The patient’s rhythm was converted to sinus rhythm and sustained after cardioversion one day after surgery. The patient became alert on the third postoperative day, and was extubated on the fifth postoperative day. He was discharged 39 days postoperatively, with a motor power score of three out of five on the Medical Research Council scale after rehabilitation therapy. On his last outpatient visit, two and a half months after surgery, motor power was fully recovered without any sequelae. |
A 37-year-old Caucasian woman who was 35 weeks pregnant with a history of 2 previous spontaneous abortions experienced a sudden, spontaneous, and very heavy epistaxis from the left nostril. Conducted to the emergency department of the local hospital, she presented hemodynamic instability and fetal monitorization compatible with severe fetal distress. She underwent an emergency cesarean section preceded by a focal convulsion upon the induction of anesthesia (attributed to induction of anesthesia in the context of severe hypovolemic anemic shock leading to cerebral hypoperfusion). The epistaxis continued and she received 5 units of red blood cells and 1 unit of fresh frozen plasma over 2 days. Because of failure to control the nasal bleeding with a local hemostatic balloon, she was transferred to a tertiary hospital for angiography with eventual embolization. During this period, she did not present any changes in her mental state or hemodynamic stability, and the local hemostatic balloon in the left nostril was kept in place.\nThe first angiography was performed using 50mL of low osmolality and nonionic organic contrast media administered in the internal and external carotid arteries. There was an abnormal vascular blush in the left nasal hemifossa dependent on branches of the sphenopalatine artery, and embolization was performed. However, the epistaxis returned shortly after, and a second angiography was performed 2 hours later, excluding the presence of any remaining vascular blush and confirming the overall success of the prior embolization. A total of 90mL of nonionic isosmolar contrast was used in both angiographies. Local tamponing was kept in the left nostril.\nImmediately after the second angiography, during the postanaesthetic recovery, the patient started to be disoriented, with sleepy speech but no formal visual complaints or motor deficits. Within 2 hours of the second procedure, she remained alert but cried continuously, developed hyperventilation, was not able to follow orders, directed her gaze but could not fix it and presented repetitive speech without intelligibility or the ability to make sentences. She was hemodynamically stable, afebrile and without any meningeal signs. The patient was transferred to the intensive care unit (ICU) due to her clinical evolution and the eventual need for airway protection in the context of an acute confusional state of unknown cause.\nGiven the patient’s agitation and noncooperation, it was not possible to properly evaluate her language and content of thought or humor, and it was not possible at that time to exclude the possibility of aphasia. A formal psychiatric evaluation, performed within a few hours of presentation, ruled out any major psychiatric illness, as well as the more common peripartum psychosis (PPP). There were no motor deficits, cranial nerve changes, or other focal signs suggesting extensive focal neurological injury. Given the acute onset, brain magnetic resonance imaging (MRI) was performed to evaluate possible complications of angiography, and a lumbar puncture was performed to exclude an infectious or inflammatory disease of the central nervous system. An electroencephalogram was also performed.\nMagnetic resonance imaging revealed a small focus of hypersignal on the diffusion weighted imaging at the level of the left corona radiata white matter (), with hyposignal on the apparent diffusion coefficient map, with a discrete hypersignal in the fluid-attenuated inversion recovery. There was also smooth swelling of the posterior temporo-occipito-parietal cortex bilaterally, with a discrete hypersignal on the fluid-attenuated inversion recovery () but without restricted diffusion, suggesting vasogenic edema. Her cerebrospinal fluid was crystal clear, with a normal opening pressure, and presented four cells, a glucose of 72mg/dL, proteins 53mg/dL and a lactate dehydrogenase (LDH) < 30UI/L. Polymerase chain reaction was negative for an extensive panel of neurotropic viruses; all bacterial cultures were negative. There were no electrolytic disturbances, and systemic tests for autoimmunity were negative.\nAn electroencephalogram showed predominant theta activity in the posterior projection, beta activity in the anterior and theta-delta activity infrequent in the frontotemporal region, and a left predominance, sometimes with a rhythmic morphology followed by base suppression. The traces revealed diffuse slowing of base electrogenesis to a moderate degree. There was fronto-temporal slow activity of the left hemisphere associated with a scarce paroxysmal component but it was mild and noncontinuous.\nSedoanalgesia and mechanical ventilation were maintained for 2 days, being withdrawn only after stabilization of the clinical picture and reviewing the results of the MRI and electroencephalogram to exclude further brain damage that could need a different approach.\nExtubated, she maintained a very poor vocabulary (with a maximum of 40 spoken words) but with no focal deficits. In less than 24 hours, a progressive improvement of her speech and reasoning content was observed, without other changes in the examination. Her renal function was normal throughout.\nA second MRI was performed 48 hours after the first, showing the presence of a few bilateral upper cortical/subcortical millimetric restricted diffusion lesions, probably related to the previous angiography. There was a complete resolution of her posterior cortical edema (). The rest of her brain parenchyma did not presented other relevant changes.\nClinically, the patient progressed very well, with quick recovery of speech and all neurological changes. Tamponing was withdrawn on the third day, and the patient was discharged home 5 days after the C-section. |
A 72-year-old woman without a previous history of cardiac disease underwent radiofrequency ablation of year-long paroxysmal AF consisting of a pulmonary vein isolation (PVI) without any LA substrate modification. Five months after the ablation she was admitted to our hospital for a cough and slight fever refractory to medical therapy. An ECG exhibited recurrent paroxysmal AF with a mean of 112 b.p.m. and no evidence of myocardial ischaemia. Chest computed tomography revealed mild pleural and pericardial effusions without any pulmonary emboli. For the first time, laboratory testing revealed an elevated C-reactive protein level (57 mg/L). Transoesophageal echocardiography revealed mild dilatation with an LA diameter of 38 mm despite there having been no dilatation of the LA just after the ablation (29 mm). Transoesophageal echocardiography revealed wall thickening limited to the LA. Cardiac magnetic resonance imaging showed there was wall thickening with marked oedema. Cardiac PET after a 24-h carbohydrate-free diet revealed an LA FDG uptake consistent with myocarditis (Figure ). An extensive infectious work up was negative. An LA biopsy under intracardiac echocardiography (ICE) guidance revealed granulomatous and lymphocytic inflammation of the myocardium, which was consistent with atrial GCM (Figure ). After treatment, starting with only prednisolone 40 mg per day to tapering to 15 mg, she returned to a normal life after 2 months of follow-up. Atrial fibrillation was the only detected arrhythmia during the course of the atrial GCM. Atrial fibrillation recovered to sinus rhythm during treatment under the administration of dabigatran and cibenzoline. Following that, no further AF recurred during 16 months of follow-up.\nThis case exhibited a new variant of GCM with lone involvement of the LA and a preserved left ventricular function. The isolated atrial involvement was implicated as a cause of the AF in spite of an AF ablation., However, this ablation procedure might not have been associated with the development of atrial GCM, because the sudden onset of the disease was found more than at least 5 months after the ablation, and LA wall thickening could also be observed in the LA body and LA appendage ().\nAtrial cardiomyopathies generally may have long been overlooked. Atrial GCM may be considered as a cause of recurrent AF after an AF ablation. Cardiac imaging including FDG-PET and an atrial wall biopsy under guidance with ICE may contribute to understanding the atrial pathology. |
A 44-year-old man was admitted to our hospital due to a sudden onset of lower right abdominal pain. The patient had a history of hypertension and hyperlipidemia. Computed tomography (CT) revealed a spindle-shaped and dilated right colic artery, intra-abdominal bleeding, and a large mesenteric hematoma (Fig. ). A diagnosis was made of rupture of the aneurysm of the right colic artery, and catheter embolization of the ruptured right colic artery was performed (Fig. ). Although cessation of bleeding was achieved, a large hematoma, with a major axis of 150 mm, remained in the ascending colic mesentery after catheter embolization (Fig. A). As the patient had no associated symptoms, he was discharged from the hospital on day 6 after onset. On day 16, however, the patient developed sudden onset of severe right lower abdominal pain. Although there was no evidence of re-bleeding on enhanced computed (CT) imaging, a collapse of the residual hematoma wall was observed, with visible leakage into the intra-abdominal cavity (Fig. B). Figure shows a little air existed in the hematoma, although the position and amount of air have not changed between both Fig. A, B. Therefore, we considered that the air mixed during catheter and the hematoma did not have infection. After readmission, the patient developed a small bowel obstruction (Fig. ), with an elevated level of biliary enzymes from day 20 (Fig. ). CT and ultrasound images revealed dilation of the gallbladder and biliary sludge, but with no sign of inflammation or biliary obstruction. These findings were suggestive of cholestasis. Figure shows the levels of serum bilirubin and biliary enzymes over this period. As the small bowel obstruction and cholestasis did not improve with conservative treatment, we proceeded to surgical treatment on day 32 after the initial onset.\nWe performed a laparotomy using a midline incision. Adhesions between the small bowel and the abdominal wall were observed, which were likely the cause of the small bowel obstruction. The release of these adhesions removed the cause of the small bowel obstruction. We also observed a collapse of the caudal wall of the hematoma in the ascending colic mesentery, with a pooling of the leaked blood in the pelvis (Fig. ). We removed as much of the hematoma as possible and placed a drain in the pelvis and intra-mesenteric cavity. The culture taken from the hematoma during operation showed no growth of bacteria.\nThe small bowel obstruction improved after surgery and the patient was started on a diet. However, on day 45, the patient developed epigastric pain and fever. CT and ultrasound imaging revealed dilation of the gallbladder, with surrounding inflammation. From blood tests, the white blood cell count and levels of biliary enzymes and total bilirubin were elevated. The diagnosis of cholecystitis was made, and we proceeded with percutaneous transhepatic gallbladder aspiration (PTGBA). After PTGBA, the patient’s symptoms improved, with a decrease in white blood cell count and biliary enzymes and total bilirubin; the postoperative course of these levels is shown in Fig. . The patient was discharged on day 70 from the initial onset of symptoms, 38 days after surgical treatment. |
A 30-year-old female with a BMI of 37.9 was diagnosed with IIH seven months prior to this presentation. Her MRI at diagnosis demonstrated 4 mm of cerebellar tonsillar descent () with flattening of the posterior globes, mild distension of the optic nerve sheaths, and a partially empty sella turcica. At the time of diagnosis, her LP revealed an opening pressure of 42 cm H2O. She was prescribed regular oral acetazolamide and regularly underwent therapeutic drainage of 15 mL of cerebrospinal fluid (CSF) for symptom control without complication. Her opening pressures were consistently between 35 and 50 cm H2O. MR venogram demonstrated an aplastic left transverse sinus with a right transverse sinus stenosis with extrinsic compression, for which she was being considered for a stenting procedure ().\nShe presented to the emergency department with four days of severe headache, nausea, and vomiting. Her past history included renal tubular acidosis diagnosed two years prior and treated with sodium bicarbonate and electrolyte replacement. In the emergency department she had bilateral papilloedema with no new focal neurology. She was normothermic with a CRP of 2.8 mg/L. A therapeutic LP was performed with an opening pressure of 43 cm H2O. 18 mL of clear CSF was drained and she was admitted to the neurology ward for observation and analgesia.\nOvernight she had a medical emergency team (MET) call for hypotension attributed to hypovolemia due to ongoing vomiting, which was treated with intravenous fluids. She had a further MET call overnight for transient visual disturbance and reported being unable to appreciate light/dark and had severe headache. Her pupils were bilaterally dilated but responsive to light. She was alert and interactive and had no motor deficits. The visual changes resolved without intervention and she was taken for a CT venogram, which demonstrated bilateral tentorial herniation with 10 mm of cerebellar tonsillar herniation. There were no features of dural venous thrombosis.\nShe was reviewed on the morning of day 1 and found to be agitated, Glasgow Coma Scale (GCS) 13 (M6, V4, E3), with a fixed dilated right pupil 7 mm. Her left pupil was 5 mm and reactive. Plantar response was upgoing bilaterally and her neck was hyperextended with extreme pain on neck flexion. Heart rate was 65 bpm and blood pressure was 112/66 mmHg. She was transferred urgently to the intensive care unit (ICU) and intubated for anticipated decline whilst awaiting a CT brain. During the CT scan she became bradycardic with a heart rate of 30 and asystolic pauses, treated with osmotic therapy of 10 mL 20% sodium chloride. Her BP was then recorded as 147/77 mmHg. The CT scan again showed 10 mm of tonsillar descent and an increase in fourth ventricle size with posterior fossa crowding (). Her initial LP result showed a mildly elevated white cell count of 7 × 106/L (normal < 5 × 106/L) and she was commenced on aciclovir empirically.\nShe was treated with a further 20 mL of 20% sodium chloride and taken for urgent insertion of an external ventricular drain (EVD), a technically difficult procedure due to small ventricular size, which rapidly improved right pupil reactivity although it remained sluggish. She returned to ICU with the EVD on free drainage 15 cm above the tragus. Her ICP remained persistently high at >45 cm H2O and the EVD was lowered to 10 cm to allow further drainage. As her pressure on previous LPs was 35–50 cm H2O, a target of 35 cm H2O was agreed upon for initial management.\nOn day 2 she had persistently high ICP >60 cm H2O despite aggressive management with hyperventilation to PaCO2 of 35 mmHg, osmotic therapy with 20% sodium chloride, deep sedation with morphine and midazolam, and aggressive temperature management, targeting normothermia. Her ICP did not respond to intermittent paralysis; thus a paralytic infusion was not commenced. CT scan demonstrated 12 mm of tonsillar herniation. She was treated with 16 mg dexamethasone and taken for rescue bifrontal decompressive craniectomy. The brain was noted to be grossly swollen, hyperaemic, and herniating with marked cortical venous distension. The craniectomy was insufficient alone to provide cerebral decompression due to the inelasticity of the dura; thus the latter was opened with multiple slits during which a cortical vein was lacerated requiring diathermy. The EVD was removed and a fibre-optic pressure monitor was placed.\nFollowing this, her ICP remained <10 cm H2O. Postoperative CT scan showed 8 mm of tonsillar herniation and a significant increase in size of the third ventricle. CSF culture was negative and viral PCR for HSV1, HSV2, enterovirus, and VZV were also negative and her empiric aciclovir was ceased.\nOn day 3 high dose sedation was ceased with slow recovery including transient diabetes insipidus treated with desmopressin and reintubation for poor sputum clearance. She had a gradual neurological improvement and was discharged to the neurology ward with a GCS 15 and no focal neurology on day 15 of her admission.\nUnfortunately she developed CSF rhinorrhoea and pneumocephalus due to a CSF fistula through the frontal air sinus requiring surgical repair. She subsequently developed a venous infarct complicated by seizures and the requirement for intubation for reduced level of consciousness.\nAfter rehabilitation, she was discharged home on day 69 after admission and was able to function independently, although she had not returned to work. At the time of publication she is undergoing neuropsychological testing to evaluate her readiness to return to work. Her cerebellar tonsils had returned to a normal position on a follow-up outpatient CT scan (). |
A 51-year-old man was admitted for subarachnoid hemorrhage caused by a rupture of the left anterior communicating artery aneurysm. After acute treatment for a month, he was transferred to the department of rehabilitation for intensive therapy. Physical and neurological examinations were done. The score of the Korean version of the Mini-Mental Status Examination (K-MMSE) was 16. There were no abnormal findings on cranial nerve function tests, and no definite extraocular movement limitations or facial palsies were noted. Manual muscle power test showed that the Medical Research Council (MRC) grade of both the upper and lower extremities was grade II and I, respectively. The patient had been suffering from teeth grinding and jaw clenching, which had begun 3 weeks after the onset of a cerebral hemorrhage. Dystonia of the tongue, face or any other part of the body was absent. This symptom was aggravated during the daytime, especially during meals, and subsided during sleep. Teeth grinding worsened as time went on. An injection of botulinum toxin was given 5 weeks after the onset of bruxism; 40 units to each side of the bilateral masseter, 15 units to each side of the bilateral lateral pterygoid, and 15 units to each side of the bilateral medial pterygoid muscles. The symptom was followed up after 2 weeks, and the injected botulinum toxin showed no effect. His bruxism was also refractory to bromocriptine therapy. Thus, a 10 mg intramuscular metoclopramide HCl injection was administered for treatment of the bruxism. The bruxism subsided dramatically 5 hours after the injection and did not recur for the subsequent 12 hours. Oral metoclopramide medication in dosages of 15 mg per day was initiated the next day, which successfully abolished his bruxism after 2 days. Bruxism did not recur during the metoclopramide therapy. Magnetic resonance imaging of the brain showed a subacute intracranial hemorrhage at the left basal frontal lobe (). Positron emission tomography (PET) also showed a metabolic defect at the left basal frontal lobe, along with diffuse hypometabolism at the bilateral frontal, temporal, and parietal cortexes. A blink reflex study and electroencephalography (EEG) were normal. Transcranial and carotid Doppler were also normal. Anticholinergic agents, which are known to decrease the effectiveness of metoclopramide, were not administered for any reason. Any drug that induced bruxism or decreased the effect of levodopa was not administered either. |
The incidental clinical findings of central accessory cusps were observed in a 5 years old Bengali girl who reported to the Outpatient Department of Pedodontics and Preventive Dentistry, Guru Nanak Institute of Dental Science and Research, Kolkata. Her major complain was of pain in the right and left lower back tooth region since 1 month. The pain history revealed aggravation of pain at night with an associated swelling in relation to the affected teeth. No significant relevant medical and dental histories were reported by the parents.\nOn clinical examination, patient had no relevant extraoral findings. The intraoral presentation revealed that the patient was in a primary dentition stage. There was presence of dental caries leading to dentoalveolar abscess with pus discharging sinus in relation to 75 and 85 (). The central cusps were discovered during the examination, found in both the maxillary second molars with deep pit and fssure caries (). No other relevant clinical findings were observed.\nThe central cusps were presented as a central projection (3 mm by 4 mm in size) on the occlusal surfaces of both the right and left maxillary second deciduous molars () respectively. These cuspal projections were situated on a triangular base in the center of the oblique ridge and were identical on both sides.\nThe established treatment plan was pulpectomy of lower second molars followed by stainless steel crown; and light cure composite restoration of 55 and 65.\nIn the first phase of treatment, pulpectomy of 75 and 85 were done after draining the sinus followed by placement of stainless steel crowns in both the teeth respectively.\nIn the second phase of treatment, occlusal pit and fissure caries of 55, 65 were removed with the help of spoon shaped excavator (maliper) and no. 2 round diamond bur. The prepared teeth were then restored with the matched shade of packable composite (3M) preserving the cuspal morphology (). Next, proper postoperative instructions were given and periodic follow-up was advised to the parents. Three months and 6 months follow-up showed no eventful changes of these affected teeth. |
A 41-year-old Japanese man became aware of abdominal swelling in December 2008 but did not seek a medical opinion until he developed marked leg edema, a 30 kg weight gain, and cough. He was hospitalized the same day after a retroperitoneal tumor was detected by computed tomography (CT). After hospitalization, the patient was diagnosed with a liposarcoma by CT-guided needle biopsy. The patient was emergently transferred to our hospital due to worsening respiratory status. On admission his body surface area was 2.18 m2. A CT scan detected a 43 × 37 × 31 cm tumor in the right retroperitoneum, with the right kidney significantly displaced to the left and anterior side (). Whole-body CT scan detected no metastasis. His condition was diagnosed as respiratory failure due to elevation of the diaphragm as a consequence of the rapid growth of the tumor ().\nWe concluded that surgical resection was the only option for saving the patient's life, so we performed a tumorectomy 3 weeks later through a midline incision. The operation took 3 hours and 20 minutes, and the estimated blood loss was 3990 mL requiring 800 mL blood transfusion. During surgery, the tumor and right kidney were completely resected.\nPathological examination showed that the resected mass weighed 22 kg and was 45 × 40 × 30 cm in size (). Grossly, the tumor was well encapsulated and transverse sectioning revealed a solid multinodular tumor mass. Histopathologic analysis demonstrated many spindle cells with high-grade atypia; some of which were lipoblasts on a myxoid background; there were no split signals on FISH analysis using CHOP DNA probes, thus leading to the diagnosis of dedifferentiated liposarcoma (). Microscopically, no tumor cells were identified in the right kidney and this was an R0 resection. He declined adjuvant therapy. The patient was discharged from our hospital after 18 days. His body surface area at discharge was 1.66 m2. A CT scan performed 12 months after surgery detected no recurrence. |
A 67-year-old female presented to our hospital with a symptom of gradually aggravated dyspnea. Clinical examination confirmed the grade 3/6, subaortic, midsystolic murmur and increased respiration rate of 28 breaths/min. A 12-lead electrocardiography showed left ventricular hypertrophy in voltage criteria. A chest radiograph demonstrated marked cardiomegaly with pulmonary edema ().\nEight years ago, the patient had come to our hospital with similar symptoms. On TTE, the LV interventricular septal wall thickness and LV posterior wall thickness were 15 mm and 10 mm at diastolic phase, respectively, and papillary muscle was hypertrophied. There was no significant calcification, thickening or motion limitation of aortic valve to increase flow velocity. Continuous wave (CW) Doppler spectrum did not show late peaking appearance but symmetrical appearance and the velocity was increased up to 6 m/sec at the LVOT level during the resting state. Therefore we had regarded the patient as having HCMP accompanied by flow acceleration caused by narrow LVOT (). In this time, TTE was of suboptimal quality but suggested the presence of hypertrophied interventricular septum and turbulent flow at the basal interventricular septum, which findings were similar to those by the previous TTE. The CW Doppler showed slightly late peaking configuration and the peak pressure gradient between the LV and the ascending aorta was 151 mmHg. However, there were no definite aortic stenosis and systolic anterior motion (SAM) of anterior mitral valve leaflet or chordae to induce the high pressure gradient between the LV and the ascending aorta. TEE was performed to find out the cause for the high pressure gradient between the LV and the ascending aorta; confirmed the flail subaortic membrane which disturbs the forward flow toward the ascending aorta and causes severe subaortic stenosis (). To identify the hemodynamic significance of the flail subaortic membrane, we performed cardiac catheterization. We simultaneously recorded left ventricular pressure and aortic pressure using right radial long sheath. There was a pressure drop at systolic phase on the pressure curve of the LVOT. The pressure drop coincided with the notch which was measured at systolic phase of ascending aorta pressure curve (). These pressure curve changes implied that the subaortic membrane of interventricular septum has a critical role in inducing high pressure gradient between the LVOT and the ascending aorta. She had an open heart surgery for the resection of subaortic membrane. After original planned resection of subaortic membrane, the operator thought that interventricular septal myectomy and mitral valvular replacement would be helpful. Because she had severe LV hypertrophy due to longstanding subaortic membrane, it looks like HCMP. Aortic valvuloplasty and papillary muscle release were done due to incidental papillary muscle rupture. Her symptoms were improved after the resection of subaortic membrane and she was discharged without major complications. |
A 65-year-old gentleman with no comorbidities underwent elective laparoscopic cholecystectomy for symptomatic gallstones in 2013. His operation was complicated by intra-operative spillage of gallstones, which were retrieved. The patient remained well until 2017 when he developed a long-standing dry cough. Computed tomography (CT) scan chest showed a right lung base mass lesion with a large area of contact with diaphragmatic pleura, also abutting the right lobe of the liver. A small area of calcification was seen in the mass lesion thought to be part of the malignant process ().\nSubsequent positron emission tomography scan and right pleural biopsies only showed inflammatory changes, and no evidence of malignancy was found. This mass lesion was followed up with a CT scan at 6 months interval that showed complete resolution.\nTwo years later, the patient developed fever and painful swelling over the back in the right paraspinal region. CT scan abdomen showed a large abscess in the right paraspinal region with no apparent cause demonstrated. This abscess was drained with a pigtail drain under ultrasound guidance.\nA year later, the abscess recurred, and the patient required acute hospital admission. Inflammatory markers were observed to be elevated, C-reactive protein of 125 mg/L. His liver function test remained unremarkable. Abdomen was soft but tender in right flank.\nA CT scan abdomen was performed, which demonstrated a large retroperitoneal abscess behind the liver tracking through a small intramuscular opening onto the skin over the upper aspect of the back ().\nBecause of previously identified calcification in the mass lesion and history of laparoscopic cholecystectomy, a non-contrast CT scan abdomen was obtained to ascertain if spilled gallstones were responsible for the recurrent abscess formation ().\nIncision and drainage of the back abscess were performed under general anaesthesia. Almost 200 mL of frank pus were drained, and after a washout, a corrugated drain was left in place. Also, during the procedure, a small gallstone was identified and removed ().\nSince the operation, the patient has done very well and is symptoms free to date. |
A 53 year-old, edentulous, African-American woman with longstanding FCOD presented to her dentist with a 1 month history of swelling, pain, and purulent discharge involving the region of the left lower mandible. The drainage was described as white, thick, and malodorous. Radiographs revealed multifocal diffuse bony changes consistent with FCOD and a new radiolucency in the bone of the mandible underlying the swollen and draining soft tissues. She was treated with a chlorhexidine gluconate oral rinse and a two-week course of oral levofloxacin but did not improve. A biopsy performed approximately two months after the onset of symptoms revealed dead bone but no specific diagnosis. Two months after this biopsy the patient noted what appeared to be a bone fragment erupting from the same pus-draining ulcer. The ulceration and drainage continued and seven months after the onset of symptoms the patient was referred to our institution for further management. She denied fevers, chills, night sweats or other constitutional symptoms. Her dental history was significant for FCOD, a condition shared with her mother. The diagnosis of FCOD was established twenty five years prior to her presentation to the University of Michigan by biopsy coincident with the extraction of her remaining teeth. Her present dentures were more than 10 years old. Her medical history was significant for multiple sclerosis treated with interferon 1β and a venous thromboembolic disorder managed with oral warfarin.\nOn physical examination the patient's poorly-fitting mandibular denture was removed and the alveolar tissues revealed slight swelling along the anterior border of the ramus with an area of exposed bone measuring approximately 1 cm along the external oblique ridge laterally. There was soft tissue edema and congestion in this area. Her neck was otherwise supple without lymphadenopathy. She had no other oral lesions. Panoramic radiographs revealed bilateral lesions consistent with FCOD involving both the maxilla and mandible (Figure ). A maxillofacial computed tomography (CT) scan revealed midface, and mandibular findings consistent with FCOD (Figures and ). On the patient's left, there was bony sequestrum within a radiolucent capsule to the anterior border of the ascending ramus (Figures and ).\nBecause of concern for chronic infection, the patient underwent debridement with bone biopsy and cultures of the diseased left mandibular ramus. The ulcerated soft tissues were repaired with a simple advancement flap. Purulence and necrotic bone were observed during this procedure. Histopathological examination of the biopsied bone revealed irregular trabeculae and bosselated cementum droplets in a fibrous stroma, typical of cemento-osseous dysplasia (Figure ). Changes consistent with osteomyelitis were also observed (Figure ). A Brown-Hopps tissue Gram stain revealed abundant gram-positive filamentous organisms consistent with Actinomyces (Figure ), which also stained positively with GMS silver stain (Figure ). Operative cultures yielded numerous (in quantity) Actinomyces species (not further identified to the species level) along with a mixture of oral anaerobic bacteria (table ). Coagulase-negative Staphylococcus was the only aerobic bacterium isolated from the bone biopsy. Cultures for acid fast bacteria, fungi, and Nocardia were negative.\nThe patient was initially treated for one week with oral amoxicillin combined with clavulinic acid 875 mg twice daily. However, because of the complicated polymicrobial nature of the infection, including bone involvement, therapy was changed to intravenous ertapenem one gram daily and the patient was treated for eight weeks. An additional 10 months of oral therapy were instituted with amoxicillin/clavulanic acid 875 mg twice daily. The patient responded well to antimicrobial treatment with complete healing of her operative site, and to date denies any further pain, swelling, or drainage.\nThis case newly demonstrates an association between two chronic, destructive and disfiguring conditions of the mandible, FCOD and Actinomyces osteomyelitis. It also emphasizes the utility of performing biopsies with appropriate aerobic and anaerobic cultures to establish a diagnosis in complicated cases such as this one. Initially our patient was felt to be suffering only from a progression of her FCOD coupled with ulceration caused by her poorly-fitting dentures. This led to a delay in her diagnosis. However, the eruption of purulent fluid from the ulcer and the eventual radiographic demonstration of an enlarged radiolucent ring surrounding more poorly-defined lesional tissue/sequestrum suggested that a superimposed osteomyelitis was present. While radiographs did not show a progressive osteolysis of the bone as might be expected typical osteomyelitis, the diagnosis was confirmed histopathologically. We speculate that our patient developed Actinomyces osteomyelitis following the mandibular ulceration.\nActinomycosis of the head and neck is an indolent infection that generally presents as a soft tissue swelling, mass, abscess, or ulceration of the oral-cervical region []. "Lumpy jaw" is caused when an actinomycete resident of the mouth (or other mucosal site) invades the underlying tissues through a loss of mucosal integrity []. The most common region for lesions to occur is the perimandibular area, but involvement of the bone is rare []. Anaerobic cultures are necessary to isolate the bacterium from pathological specimens, but culture-independent means of establishing the presence of these bacteria, such as PCR, are increasingly utilized []. Gram stains are more sensitive than cultures, perhaps because a lack of strict anaerobic processing or previous antibiotic use can render the cultures negative []. In cases of perimandibular infections, actinomycetes are often present in the setting of multiple other species of bacteria []. Although it is not always known how much the other bacteria are participating in the pathogenesis of infection, antimicrobial therapy is generally broad enough to cover these microbes. Although biopsy clearly demonstrated invasive actinomycosis, our patient had malodorous, purulent drainage from her mandible, suggesting that anaerobes or pyogenic bacteria were also present, which was confirmed by culture (table ). It is likely that her infection was driven by this polymicrobial collection of pathogens and was not simply due to actinomycosis alone.\nAs evidenced in the present case, the treatment of cervicofacial actinomycosis requires attention to other identified pathogens, since these infections can be polymicrobial []. However, in many cases co-pathogens (such as oral anaerobic bacteria) are susceptible to the agents targeting the actinomycete. In cases where Actinomyces is the only identified pathogen then targeted therapy is warranted. Fortunately, actinomycetes remain exquisitely susceptible to beta lactam drugs and intravenous penicillin G remains a first-line agent []. For patients with intolerance to beta lactam agents, tetracycline agents can be substituted [,]. As detailed in a recent review [], parenteral penicillin G, 10 to 20 million units daily divided every 6 hours for 4 to 6 weeks can be used for complicated cases, followed by oral penicillin V, 2 to 4 g/d divided four times daily for 6 to 12 months to prevent relapse []. The ultimate length of treatment depends upon clinical and pathologic response []. A number of alternatives to penicillin are available and include macrolides, tetracyclines, clindamycin, and carbapenem agents []. Importantly, many commonly used antimicrobials are not active against Actinomyces species and these include metronidazole, aminoglycosides, aztreonam, trimethoprim-sulfamethoxazole, penicillinase-resistant penicillins (e.g., nafcillin and oxacillin) and cephalexin [,]. In the present case ertapenem was used for the parenteral therapy because of the polymicrobial nature of the osteomyelitis and the convenience of once daily dosing.\nIt is interesting that our patient developed actinomycosis while on an immunomodulatory agent, interferon 1β, which was used to treat her multiple sclerosis. We were unable to identify previous reports of osteomyelitis associated with this medication. Infectious complications of interferon 1β are uncommon []. Although neutropenia can be a complication of therapy, our patient did not have any record of this while on therapy (data not shown).\nFCOD is a benign lesion that can be disfiguring and lead to tooth loss, jaw fractures, and chronic infections. Although the etiology and pathogenesis of FCOD are unknown, the disease represents a disturbance in bone metabolism where normal bone becomes replaced by a connective tissue matrix that gradually develops cemento-osseous tissue []. As in our patient, the disease is more common in persons of African ancestry and can be familial []. FCOD is typically diagnosed through incidental findings on routine radiographic evaluation and biopsy often avoided to avoid the risk of chronically infected, non-healing wounds. The lesions are characteristic of a diffuse bilateral involvement of the maxillary and mandibular bones. Pathologic evaluation is often free of inflammation, however superinfection can occur and progress to osteomyelitis due to the limited blood supply of the dense bone [,,]. Traumatic extractions and pressure ulceration caused by poorly fitting prosthetics can cause bone exposure which is susceptible to inflammation and infection of various organisms. Prior to the characterization of FCOD by Melrose in 1976 [] these lesions were believed to represent CDSO [-]. Due to the dysplastic anatomy of the lesions, surgical intervention is often necessary for treatment of chronic infections combined with prolonged antibiotic therapy [,,].\nAs observed in this case report, FCOD is characterized by fibrovascular stroma with a "ginger-root" pattern of irregular curvilinear trabeculae, as well as droplets of cementum ("cementicles") that fuse to form bosselated structures [-]. Coexisting bone cysts with fibrovascular lining may also be present [,], although these were not observed in the present case. Early-stage lesions of FCOD display fewer trabeculae and more prominent fibrovascular stroma, often with hemorrhage []. In the case presented here, these typical findings of FCOD were seen in conjunction with key features of Actinomyces osteomyelitis including filamentous gram-positive organisms, acute inflammation, and necrotic bone [,]. |
The patient was a 23-year-old male suffering from schizophrenia for 5 years (diagnosed according to the International Classification of Diseases-Tenth Revision), who had undergone psychiatric hospitalization four times in the past, most recently 3 years earlier. The first episode involved a picture of typical paranoid syndrome and was induced by amphetamines. Subsequent psychotic episodes were not related to drug use. During a stable period, the patient presented mild severity of negative (blunted and inappropriate affect, social withdrawal) and depressive symptoms and moderate cognitive problems (memory and attention), which were the main indication for administration of sarcosine. Among positive symptomatology, the patient described only periodic mild racing thoughts without typical delusions or hallucinations. Both parents were diagnosed with schizophrenia. After receiving information about sarcosine, the patient signed an informed consent form and started receiving the amino acid. Basic laboratory tests were performed (complete blood count, electrolytes, liver and renal parameters, lipids, prolactin, and thyroid stimulating hormone), which showed no deviations from reference values. The patient had no neurological, endocrine, or other chronic comorbidities at the time of treatment. We excluded ongoing alcohol and drug dependence or use in the previous 6 months (using a panel drug testing), but the patient smoked about 15 cigarettes per day. He was unemployed at that time and on a disability living allowance. Before addition of sarcosine, the patient has been receiving quetiapine 500 mg per day for 2 years and citalopram 10 mg per day for several months. Citalopram was prescribed due to panic attacks and negative symptoms.\nDuring the first 2 weeks of sarcosine administration at 2 g per day, the patient reported improvement in overall activity, concentration, and mood. After a further 2 weeks, he reported moderate inner tension with mildly increased drive, excessive activity and irritability, and a significant increase in sexual tension. Further examination ruled out a diagnosis of hypomania. The patient found these symptoms to be very unpleasant and asked for modification of his treatment. The overall impact of sarcosine he described as positive, and therefore it was decided to maintain the sarcosine supplementation but to reduce the dose by half to 1 g per day. After lowering the dose of sarcosine without changes in the dosage of his concomitant medications, the intensity of his excitation and irritability decreased, and the patient subjectively described his overall mental state as better in comparison with the period before sarcosine and treatment with 2 g of the amino acid (see ). There were no external circumstances to explain the observed intensity of psychomotor agitation symptoms. |
A 76-year-old man with a past medical history of coronary artery disease, hypertension, and severe septal hypertrophy suspicious for hypertrophic cardiomyopathy, presented to the hospital with progressive symptoms of heart failure. He had been suffering from worsening dyspnea and lower extremity edema for several months. Lab work over the preceding months had shown deranged liver function tests, concerning for right heart failure. He was admitted for intravenous diuretic and inotrope therapy. At his cardiology clinic appointment prior to this hospital admission, an ECG showed typical atrial flutter and diffuse low voltage []. A right heart catheterization showed moderately elevated right greater than left filling pressures with a right atrial pressure 18 mmHg, pulmonary capillary wedge pressure 21 mmHg and a Fick cardiac index 1.6 liters/min/m2. The ScvO2 was 48%.\nOf note, a year prior to this admission, a transthoracic echocardiogram (TEE) had shown severe asymmetric septal hypertrophy (2.6 cm), suspicious for hypertrophic cardiomyopathy, but had not been further evaluated. A repeat TEE at the present admission showed a left ventricular ejection fraction (LVEF) of 35% as well as the aforementioned septal hypertrophy []. However, due to the significant low voltage on ECG, despite the significant LVH on echocardiogram and history of hypertension, a cardiac MRI was ordered to rule out infiltrative cardiomyopathy as opposed to hypertrophic cardiomyopathy.\nThe images showed normal left ventricular chamber size with severe, asymmetric left ventricular hypertrophy, primarily involving the septal wall with a maximum septal thickness of 27 mm []. The LVEF was measured at 40%. Also noted was diffuse, global late gadolinium enhancement of the left ventricular myocardium, consistent with a diagnosis of cardiac amyloidosis []. The only extracardiac clinical finding suggestive of systemic amyloidosis was carpal tunnel syndrome. Serum and urine electrophoresis did not detect a monoclonal protein, and serum free light chain ratio was low, which significantly lowered the suspicion for AL-amyloidosis. An abdominal fat pad biopsy was ordered which came back negative. Finally, an endomyocardial biopsy was performed which showed a pink amorphous interstitial infiltrate exhibiting apple green birefringence with Congo red stain, confirming cardiac amyloidosis. The subtype was likely wild-type ATTR or variant ATTR amyloidosis.\nIncidentally, the cardiac MRI also found a left upper lobe mass which, on further workup, was characterized as adenocarcinoma. No further differentiation of the subtype of ATTR amyloidosis was pursued, as this would not have changed management, especially in view of the poor prognosis portended by the adenocarcinoma. |
A 34-year-old Malay, gravida 4, para 3, Rh-negative woman was referred from a private hospital at 13 weeks owing to accreta suspicion for further management. She had a history of three previous lower segment cesarean sections, and all operations were uneventful. At 5 weeks of pregnancy, she presented with per vaginal bleeding and unresolved suprapubic pain at a private hospital. Her urine pregnancy test was positive, and ultrasound examination showed an empty uterus with evidence of intraperitoneal bleeding. A diagnosis of a ruptured ectopic pregnancy was made. She underwent emergency laparotomy, and hemoperitoneum with clots and fresh 500 ml of bleeding were found. This was due to bleeding from a ruptured vessel of an engorged and swollen left Fallopian tube. Left salpingectomy was performed. Postoperatively, her per vaginal bleeding had stopped, and, on day 3 postoperation, she was discharged from the ward. A week later, she had had obvious morning sickness symptoms; she then returned to her doctor and discovered she had an intrauterine pregnancy with a viable fetus of 7 weeks gestation.\nThe gestational sac was located at the lower part of the uterus; however, there was no suspicion of abnormal placentation at that time. The patient was given 4 weeks until her next appointment. She experienced intermittent minimal per vaginal bleeding associated with suprapubic discomfort during this period. At 12 weeks of gestation, a repeat ultrasound showed that a viable fetus was located at the lower part of the uterus, and the placenta was covering the internal os, which was accompanied by loss of the hypoechoic border between the placenta and uterus; thus, a diagnosis of placenta accreta was made. The patient sought a second opinion from another consultant. Magnetic resonance imaging (MRI) was performed, and the gestational sac was found to occupy the lower half of the uterine cavity. Moreover, superior to the gestational sac was a sizeable heterogeneous lesion, suggestive of a multi-age blood clot occupying the other half of the uterine cavity. The placenta was located at the lower part of the uterus covering the os. She was counseled for a hysterectomy and then was referred to our center. A repeat ultrasound examination revealed similar findings with increased subplacental vascularity at the uterine bladder interface (Fig). Per-abdominal examination revealed that the uterus was at 20 weeks gravid uterine size.\nAn elective hysterectomy was decided upon, and the procedure and possible complications were explained to the patient and partner. The patient was started with an intravenous antibiotic because of her prolonged per vaginal bleeding. The challenge in managing the case was in deciding the best approach to minimize the patient’s complications. A large amount of Rh-negative blood is not readily available in our blood bank. If an additional amount is required, a regular donor needs to be called, or Rh-negative blood is collected from another hospital blood bank. The surgery could only be performed after at least 6 pints of blood group O Rh-negative was obtained in preparation for any bleeding intraoperatively. The apprehension was more regarding the adhesion of the uterus to the anterior abdominal wall, the difficulty of separating the urinary bladder, the possible injury to the urinary bladder, and intraoperative bleeding. The transfusion department of our institution managed to gather eight units of a packed cell of Rh-negative blood group O on the operation day. The urology team was on standby during the operation. A midline subumbilical vertical incision was made. There were adhesions between the right anterolateral peritoneal wall with the omentum, the anterior surface of the uterus, and the bowels. Adhesiolysis was carried out slowly.\nAn enlarged uterus was visible, with tortuous vessels on the serosal surface of the lower part. The total hysterectomy was performed successfully. The estimated blood loss was 2 L, with bleeding mainly from the raw areas at the vesicouterine fold. Two pints of the packed cell were transfused intraoperatively. A gross histopathological examination showed that the placenta appeared to extend up to the serosa (Figs. , ). It was microscopically confirmed that the chorionic villi invaded the myometrium with an absence of decidual tissue, while no invasion toward or penetration of the serosal layer was found (Fig. ). Our patient recovered uneventfully. She was discharged on the fifth postoperative day in good condition, and she was in excellent health during a follow-up visit 2 weeks later. She was seen again after 1 month: she had no complaints, the wound was healed, and she was discharged from the gynecological clinic. |
A 63-year-old female patient was admitted to our hospital with intermittent epigastric abdominal pain for the past three months. Results of the physical examination on admission indicated no icteric sclera. The abdomen was soft with no palpable abdominal mass. The patient had experienced a weight loss of about five kg in the past two months and had no history of hepatitis B or C. She had been exposed to dogs and sheep and denied any history of exposure to infected cases from the epidemic area. After admission, CA19-9 was measured and was 796.20 U/mL. No serological examination for echinococcosis was performed because of the limited conditions of our hospital. Test results from pelvic ultrasound indicated menopausal uterus and uterine fibroids. Test results from gastroscopy showed chronic non atrophic gastritis. Colonoscopy revealed multiple polyps in the large intestine (basically removed); intestinal histopathology (cecum, biopsy) showed severe chronic inflammation of the mucosa and adenomatous hyperplasia of the individual glands. Contrast-enhanced computed tomography of the upper abdomen (Fig. A) revealed a more homogeneous thickening of the gastric wall in the antrum. Round unenhanced low-density foci with a diameter of 4.6 cm was seen in the S7 segment of the liver. Nodular calcifications were also observed. No significant dilatation was noted in the intrahepatic and extrahepatic bile ducts. The size and shape of the gallbladder were normal, the wall was not thick, and no significant abnormal density was observed in the cavity. The pancreas, spleen, and adrenal glands showed no significant abnormalities. Test results from computed tomography indicated liver cyst and intrahepatic calcifications. Contrast-enhanced magnetic resonance imaging of the liver and gallbladder (Fig. B, ) revealed a normal size and shape of the liver and proportion of each lobe, and the intrahepatic and extrahepatic bile ducts and flow vessels ran naturally. A long T1 and long T2 cystic signal with a diameter of about 4.7 cm was observed in the right lobe of the liver, with liquid level, short T1 high signal intensity in the lower layer, high signal intensity on diffusion-weighted imaging sequence, and enhancement of the cyst wall on the enhanced scan. Test results from magnetic resonance imaging showed that the space-occupying lesion of the right lobe of the liver had been considered to be more likely a hepatic hydatid cyst. Preoperatively, three-dimensional reconstruction demonstrated the location of the tumor and its relationship with the surrounding vessels (Fig. A).\nThe patient underwent laparoscopic S7 segmentectomy. Intraoperative findings showed that the tumor was located at the S7 segment of the liver and was about 5 × 4 cm in size and partially protruding from the surface of the liver, with an intact capsule and clear boundary with normal liver tissue. The tumor compressed the right hepatic vein and its tributaries and densely adhered to the right hepatic vein (Fig. B). On postoperative pathology, a mass was observed immediately adjacent to the liver capsule, with a volume of 5 × 5 × 4.5 cm. The section surface showed a brown turbid fluid, a smooth inner wall, and greyish red, greyish yellow, and soft section surfaces of other liver tissues. The pathological section showed a low-grade mucinous cystic neoplasm (volume 5 × 5 × 4.5 cm) in the S7 segment of the liver, with steatosis in the surrounding hepatic tissue area, chronic inflammatory cell infiltration in the portal area, and no tumor cell involvement in the margin of the liver resection.\nImmunohistochemistry demonstrated tumour cells CK7 (+), CK19 (+), and CEA (−); stromal cells ER (+), PR (+), α-inhibin (a small amount +), vimentin (+), desmin (+), and actin (+) (Fig. A–C). This study was approved by the Ethics Committee of Yantai Affiliated Hospital of Binzhou Medical University. |
A 77-year-old man with a past medical history of type 2 diabetes, hypertension, and ESRD underwent deceased donor renal transplantation. Two months following his renal transplant, the patient was admitted for an acute kidney injury discovered on routine follow-up laboratory testing. From a baseline creatinine of 1.3 mg/dl after the transplant, he was noted to have a creatinine of 2.7 mg/dl and a renal biopsy was obtained. Histopathology was suggestive of mildly active cellular rejection and acute tubular injury but with no concerns for antibody-mediated rejection. The patient received three days of methyl-prednisolone 250 mg daily intravenously and was subsequently transitioned to high-dose oral prednisone with a taper.\nThe patient was readmitted within three weeks of his renal biopsy with worsening renal functions, now with a creatinine level of 3.8 mg/dl. At this time, he was on prednisone five mg daily, tacrolimus four mg twice daily, and mycophenolate 500 mg twice daily. His family reported poor oral intake and that he had been taking furosemide at home. He received intravenous (IV) normal saline and diuretics were held. However, as his renal functions did not improve beyond a creatinine level of 2.1 mg/dl, the decision was made to perform a repeat renal biopsy. During ultrasonography for the renal biopsy, concerns were raised for a possible renal artery aneurysm. An ultrasound of the right lower quadrant and transplant kidney subsequently showed a 3 cm x 3.4 cm x 4 cm aneurysm proximal to the renal artery anastomosis to the right external iliac artery (Figure ). The right external iliac artery to renal artery anastomosis was patent and the renal vein was noted to be patent as well. No peri-transplant kidney fluid collections were noted and no hydronephrosis was seen.\nSubsequently, a pelvic arteriogram was performed, which showed patent pelvic and iliac arterial flow. However, a large pseudoaneurysm arising directly off the right external iliac artery was re-noted and the renal transplant artery was noted to be filling from the distal side of the aneurysm and remained patent, although with sluggish flow (Figure ). A second known renal transplant artery was not seen and was presumed to be thrombosed.\nWhile the interventional radiologist considered closing off the pseudoaneurysm by stent placement, the origin of the aneurysm was noted to be too close to the anastomosis to allow for stent placement. Hence, transplant surgery was consulted. The patient was taken to the operation room for a re-exploration of his transplanted kidney and revision of the arterial anastomosis. The patient underwent exploratory laparotomy, and intraoperatively, significant inflammation and scar tissue surrounding the hilum of the transplanted kidney was seen. Once the pseudoaneurysm was entered, necrotic tissue and purulence within the pseudoaneurysm were noted. It was felt that the arterial flow to the transplanted kidney could not be restored and, therefore, a decision was made to proceed with the resection of the infected pseudoaneurysm and transplant nephrectomy. The transplanted kidney was explanted. A portion of the pseudoaneurysm was sent to pathology for further characterization, and a portion was sent to microbiology for culture. The explanted kidney was also sent to pathology for further analysis. The patient was transferred to the intensive care unit (ICU) postoperatively for further management and close monitoring. During the surgery, the patient received crystalloids and multiple blood products.\nOn arrival to the ICU, the patient had temporary dialysis access placed and started on continuous renal replacement therapy (CRRT). Given concerns for an infected pseudoaneurysm, he was empirically started on renally dosed vancomycin, piperacillin-tazobactam, and fluconazole. All immunosuppressive agents were discontinued at this time. Due to persistent vasopressor requirements while in the ICU, the patient was switched to vancomycin, meropenem, and micafungin. The infectious disease team was consulted for recommendations. Blood bacterial and fungal cultures remained negative. However, fungal cultures from the pseudoaneurysm grew Aspergillus flavus on three separate samples. The patient was then transitioned to IV isavuconazonium 372 mg every eight hours for six doses, followed by 372 mg daily with a plan to continue for at least six weeks. Vancomycin and meropenem were discontinued. The pathology of the explanted kidney showed transmural necrosis of the renal artery, no evidence of rejection, and scattered micro-abscesses within the parenchyma. As the patient improved clinically, he was transitioned from CRRT to intermittent hemodialysis. He was subsequently transferred out of the ICU in stable condition. His hospitalization was complicated by colitis secondary to Clostridium difficile for which oral vancomycin was initiated. Preparation was made to discharge him to an acute rehabilitation facility; however, a day prior to discharge, he was found to be unresponsive by his nurse and despite prolonged resuscitation attempts for cardiac arrest, could not be revived. The exact etiology for this sudden demise could not be determined. |
In June 2011, a 70-year-old female Caucasian patient presented with weight loss and hematuria in a local hospital. Further diagnostics revealed lung tumor in the left lobe with multiple pulmonary, liver, left adrenal gland and bone metastases, including pathological fracture of the 11th vertebra. The tumor cytology showed adenocarcinoma. Thus, palliative chemotherapy with carboplatin/paclitaxel was administered. A further genetic analysis of a tumor biopsy proved EGFR mutation at exon 19. After another hospital attendance due to urinary infection with complications, the chemotherapy was stopped and targeted therapy with gefitinib at a dose of 250 mg/m2 was started on August 27, 2011. In September 2011, a palliative course of radiotherapy with daily single doses of 3 Gy to a total dose of 36 Gy was administered to vertebrae 8-12 for symptomatic metastatic spread to thoracic vertebrae 9 and 11 without extraosseous and intraspinal tumor manifestations. Daily concomitant administration of gefitinib was continued. On the last day of radiotherapy, the patient was reported to be completely free of metastasis-related symptoms.\nIn December 2011, restaging proved partial response of primary tumor and lung metastases with complete response of liver and adrenal metastases. Maintenance therapy with gefitinib was continued. In March 2012, the patient presented with back pain approximately at level Th 6-7. Restaging with thoracic and abdominal computed tomography in April 2012 showed stable disease as compared to December 2011. In August 2012, the bisphosphonate therapy was stopped due to no otherwise specified adverse events and was switched to denosumab. Due to progressive disease revealed in September 2012, systemic therapy was changed to pemetrexed and continued after achieving stable disease in December 2012.\n16 months after radiotherapy (January 2013), the patient presented with pain in her right hip radiating down to the lower leg. Spinal computed tomography in February 2013 did not reveal any progression of bony spinal metastases. The patient did not respond to analgesic therapy with Tapentadol 3×100 mg/die. Due to neuropathic complaints in the right leg and muscular weakness, the patient was hospitalized to the Neurological department of the regional clinic in February 2013. Spinal MRI with gadolinium contrast agent showed T2-hypertintense spinal cord lesions in segment Th 7-10 with a little contrast enhancement on T1 and isointense in native T1 (Figure a, b). Cranial MRI revealed one small cerebellar lesion suspected to be of metastatic origin. Spinal fluid taken was negative for tumor cells. On neurological examination, pyramid signs were negative. The muscle tonus, and the deep and superficial sensitivity of the right leg were diminished. Electromyography of right m. tibialis anterior and m. rectus femoris showed no pathological spontaneous activity. Controlled muscular activity was sluggish.\nThe patient was consulted in our Radiological department. We consulted with our Radiologist on the MRIs and discussed differential diagnoses. A diagnosis of radiation-induced myelopathy was suspected and dexamethasone was proposed as a therapy for myelitis. The patient did not respond to intravenous dexamethasone 24 mg/die. A few weeks later, she became urinary incontinent. Some pain relief could be obtained with pregabalin 75 mg bid. The patient continued her palliative chemotherapy in an outpatient setting.\nThe neurological symptoms were progressive in August 2013 with the right leg being completely plegic. The left leg was incompletely paralyzed. Deep sensitivity was completely absent in the whole right leg and up to the knee on the left side. Superficial sensitivity was also diminished in accordance with deep sensitivity bilaterally. The patient was completely urinary and anally incontinent. Contrary to the clinical findings, a follow-up MRI (July 2013) showed amelioration of the former signal alterations in the spinal cord (Figure c, d). The diagnosis of paraneoplastic myelopathy was refuted by a negative test for autologous antibodies performed in our institution.\nAt the last visit (May 2014), the neurological symptoms were stable in comparison with the state in August 2013. At the last restaging (April 2014) of the thoracic CT, the primary tumor was slightly progredient. No intraspinal changes were described in the thoracic CT. The current targeted therapy contains erlotinib in the highest tolerable dosage for this patient of 125 mg daily. Before the last progression, the patient received erlotinib 100 mg daily with diarrhea being a dose-limiting toxicity. |
An 80-year-old Caucasian man who initially presented with back pain was found to have a 9.8 cm infrarenal abdominal aortic aneurysm on computed tomography (CT). This was suitable for endovascular aneurysm repair (EVAR) but the left common iliac artery (CIA) was short, necessitating the extension of the left iliac limb into the external iliac artery (EIA). The decision was made to proceed with an on table coiling of the left internal iliac artery (IIA) and EVAR. However, a tortuous right CIA made coiling of the left IIA extremely difficult and following a prolonged attempt, the decision was made to proceed with EVAR without coiling the IIA (Fig. ).\nOn a 1-month follow up CT the left limb extension had disengaged from the main body, resulting in a large Type 3 endoleak. The disengagement was presumed to be due to the tortuosity of a heavily calcified external iliac artery returning to its anatomical position on removal of the Meir wire (Boston Scientific). The left IIA remained patent and acted as an outflow for the type 3 endoleak. The left limb was then realigned successfully with termination of the type 3 endoleak.\nOn a follow up CT 1 month post repair, the left IIA remained patent with a large type 2 endoleak demonstrated but the sac size remained static (Fig. ). The failure of the IIA to occlude was presumed due to the cavity created within the aneurysm sac from the type 3 endoleak. Due to the large size of the aneurysm and the relatively large size of the type 2 endoleak the decision was made at multi-disciplinary team meeting to embolise the type 2 endoleak.\nAs antegrade access to the left IIA was not possible due to the presence of the stent graft, an attempt was made to access the internal iliac artery via the buttock. Direct sac puncture was not possible as the endoleak was located within the pelvis and surrounded by bony structures and pelvic viscera. The intention was to puncture the posterior division of the IIA but visualisation with ultrasound was limited and a vessel was punctured, which on subsequent angiography was shown to be the anterior division of the left IIA (Fig. ). The left IIA was embolised using Spirali (Pyramed) coils in a retrograde manner. Due to the absence of a solid structure which to compress the access vessel against, especially as the anterior division of the IIA had been punctured, the arteriotomy needed to be closed using an Angio-Seal (Terumo). Correct intraluminal placement of the Angio-Seal (Terumo) is dependent on backflow of blood into the Angio-Seal (Terumo) sheath from the artery. However, as the left IIA had been embolised, backflow from the Angio-Seal (Terumo) was non-existent. The angioseal was not visualised on ultrasound and therefore a 4 French dilator was inserted within the Angio-Seal (Terumo) sheath through which contrast was injected to directly visualise the tip to the sheath within the artery (Fig. ). The sheath was then withdrawn until just inside the vessel lumen and the plug deployed with immediate haemostasis (Fig. ). This was an off-label use of the Angio-Seal (Terumo).\nA follow up CT 1 month post procedure revealed resolution of the Type 2 endoleak. |
A healthy 21-year-old man was admitted to our hospital after being referred from another medical institution because of worsening left eye (LE) uveitis. He was treated 3 weeks earlier with oral acyclovir and topical steroids because of suspected LE herpetic anterior uveitis as serological tests revealed positive immunoglobulin M to herpes simplex virus-1. Initially he responded to treatment but 2 weeks later, his vision declined from 6/9 to counting fingers (CF), resulting in his referral to our center.\nThe patient denied any relevant past medical history. However, he mentioned that a month earlier he was examined for LE discomfort and diagnosed with allergic conjunctivitis following minor blunt trauma from a tree branch.\nOn examination, visual acuity (VA) was 6/6 in the right eye (RE) and CF at 1 meter in the LE. Intraocular pressure was 14 mmHg in both eyes. RE anterior and posterior segments were normal. There was a remarkable LE anterior chamber reaction with dust-like keratic precipitates, cells (4+), flare (2+), and some iris nodules. Fundus assessment was not possible because of dense vitritis. B-scan and high-frequency ultrasound did not reveal any intraocular foreign body. Aqueous tap was performed and the sample was sent for culture and polymerase chain reaction analysis. It was negative for all herpes viruses and for 16S rDNA. Meanwhile, with oral steroids and valacyclovir, the patient showed signs of improvement and LE VA improved to 6/15. Despite the remarkable improvement, it was insufficient as the patient continued to have marked anterior uveitis and vitritis. A white shadow was noted in the peripheral temporal retina of the LE which again could not be assessed properly due to vitreous opacities (). The patient eventually underwent pars plana vitrectomy and laser retinopexy was performed around the white peripheral temporal lesion, which was later believed to be the site of penetrating injury by a thorn on the tree branch from the previous trauma described by the patient. Gram staining of the undiluted vitreous samples showed gram-positive cocci. 16S rDNA was positive for S. epidermidis, and blood agar and chocolate agar cultures confirmed the result with moderate growth.\nThe patient was treated with intravitreal antibiotics (vancomycin 1 mg/0.1 mL and ceftazidime 2.25 mg/0.1 mL) and intravitreal dexamethasone 400 mcg/0.1 mL as well as intravenous (IV) vancomycin (1 g twice/day), and oral prednisone was continued.\nAfter 48 hours of treatment, the patient showed remarkable clinical improvement. LE VA was 6/12. There was no need to administer more intravitreal antibiotics.\nAfter 5 days of IV antibiotic treatment, the patient was discharged on topical and a tapering regimen of oral steroids.\nAfter a follow-up period of 3 months, LE VA was 6/6 with complete resolution of the infectious process (). |
This 18-year-old male patient presented at the Oral and Craniomaxillofacial Surgery Clinic to consider surgical treatment options for reducing an enlarged tumor of the back. The patient had more than six café au lait spots on the trunk and extremities, axial and inguinal freckling and several cutaneous tumors that were slightly raised above the level of the skin. The patient had no physical discomfort, no motor or sensitive deficits. The patient stated that he had been operated 2 years earlier on a tumor of the back in another hospital. More detailed information was not available. Despite this previous treatment of the tumor, the remaining tumor mass disturbed him both physically and in his self-perception. The patient stated that the tumor had been growing again since the first operation.\nOn the back there was a tumorous protrusion of the intact skin with a maximum above the spine, which extended from the lower thoracic region close to the edge of the pelvis (Figure 1 A ). The tumor was clearly prominent under tight-fitting clothing. The skin in this area was darker pigmented throughout the lumbar region and showed hirsutism. The tumor was insensitive to touch and pressure, showed no fluctuation on palpation, and the covering skin moved with the tumor.\nB-scan ultrasound revealed an inhomogeneous mass with focal, partly string-like reflections inside the space occupying lesion. Borders were poorly defined and the tumor mass reached to the spinous processes of the spine. The tumor appeared as solid mass and contained no cavities suggestive of necrosis. The tumor was resected in general anesthesia. When the lesion was exposed, a black pigmentation became apparent, which was partially arranged in a stripe-like pattern and frayed at the edges (Figure 2 ). The tumor was resected and the contour of the back reshaped. Despite dense suturing of the wound margins a hematoma developed, which was emptied. Secondary wound healing took 21 days and led to a stable healed wound (Figure 1 B ). There was no movement restriction of the patient after the wound had healed.\nUpon neuropathological investigation a spindle-shaped, 22x9x2 cm3 large skin sample with centrally located 6 cm long scar was seen. Cutting the skin exposed white and slightly greasy tissue on both sides of the scar with spotty brown-black pigmentation.\nHistological examination revealed a diffusely grown neoplasia of medium to high cellular density in the subepidermal connective tissue, consisting of roundish and oblong cells with delicate cytoplasmic extensions and slightly pleomorphic, small, round-oval, sometimes comma-shaped nuclei. The cells showed different degrees of pigmentation. Repeatedly, pseudo-Meissner corpuscles were observed. There was no evidence of mitoses and no Turnbull-positive hemosiderin pigment was detected. Immunohistochemistry demonstrated labeling of the tumor cells with antibodies against S100-protein and melan-A and to a lesser extent also with antibodies against HMB45. The Ki-67-proliferation index was less than 3%.\nA subepidermal diffusely grown pigmented (melanotic) neurofibroma WHO grade I was diagnosed (Figure 3 ). |
A 6-year-old male child with hypoplastic left heart syndrome (HLHS) (mitral and aortic stenosis) underwent staged palliation culminating in an extracardiac nonfenestrated Fontan procedure when he was 3 years old. The pre-Fontan hemodynamics assessment showed a normal Glenn circuit pressure at 10 mmHg. Eight months after his Fontan procedure he was found to have mild intermittent facial edema with no evidence of biochemical signs of protein-losing enteropathy. Cardiac catheterization showed mild angiographic left pulmonary artery stenosis and low pressure in the Fontan circuit (10 mmHg). One year and 2 months after his Fontan procedure, he developed clinical and biochemical signs of protein-losing enteropathy. Repeat catheterization revealed mean Fontan pressure of 12 mmHg that increased to 18 mmHg after 10 mL/kg fluid bolus. There was 1 mm mean gradient across the left pulmonary artery stenosis that was successfully treated with stent implantation. Over the next 18 months, he had clinical and biochemical evidence of protein-losing enteropathy that initially responded to therapy with budesonide. Unfortunately, he was finally refractory to high dose budesonide with side effects from therapy and growth failure. Interestingly, he was noted to have new development of restriction across the native atrial septum with 8-10 mm mean gradient by echocardiogram. Throughout his course, he had adequate systemic ventricular function, no significant systemic atrioventricular valve regurgitation, and an unobstructed aortic arch. After a discussion at a multidisciplinary conference, a decision was made to proceed with the attempt of transcatheter relief of atrial septal restriction and creation of a fenestration to avoid additional stress of cardiopulmonary bypass.\nHis weight on the day of cardiac catheterization was 19.6 kg. The procedure was performed under general anesthesia and showed elevated mean Fontan pressure of 18-20 mmHg without any stenosis across the Fontan pathway including the previously placed left pulmonary artery stent. A 6-mm mean gradient was recorded across the atrial septum (measured by retrograde catheter from the femoral artery across the systemic atrioventricular valve into the atrium). At this point, we considered three options to relieve native atrial septum restriction: Retrograde arterial via the systemic atrioventricular valve, transconduit Brockenbrough puncture from the femoral vein/transhepatic or from the pulmonary artery to the atrial chamber, and finally hybrid approach. Based on our experience, when the systemic ventricle has right ventricular morphology, access to the atrium across the systemic atrioventricular valve can be easily performed. We decided to balloon size the atrial defect and determine the hemodynamic stability with this approach. A pigtail catheter was advanced retrograde to the right ventricle via the neo-aortic valve with the open end directed to the systemic atrioventricular valve. The pigtail was opened using a Glidewire (Terumo Medical Corporation, Somerset, NJ), which was directed across the systemic atrioventricular valve into the right atrium and the pigtail was exchanged for a Glide catheter (Terumo Medical Corporation, Somerset, NJ). The catheter was then advanced across the atrial septum into the left upper pulmonary vein. This was exchanged over a SV5 guidewire and a 12-mm Tyshak II angioplasty catheter (Braun Interventional System Inc., Bethlehem, PA) was advanced over the wire and centered across the atrial septum under transesophageal and fluoroscopic control []. There was no hemodynamic perturbation with the wire and angioplasty catheter in place. Balloon sizing revealed a 7-mm waist. However, in order to place a Diabolo stent, the size of the arterial sheath was felt to be prohibitive. Next, the Brockenbrough puncture across the Gore-Tex tube graft was attempted; however, we were unable to advance the introducer after multiple attempts (including reshaping the needle) and in fact during one of the attempts the needle tip fractured without embolization. Transhepatic approach was considered but not used because of the relative contraindication secondary to ascites. Based on the relative ease of puncture between the native floor of the neo-left pulmonary artery and the adjacent atrial roof, this approach was felt to be optimal. A pulmonary artery angiogram through the right internal jugular vein and simultaneous atrial angiogram through the retrograde atrial catheter confirmed anatomic proximity of the pulmonary artery to the atrium. Brockenbrough puncture from the neo-left pulmonary artery to the atrium was performed under fluoroscopic and transesophageal guidance []. A 0.035 Amplatzer super stiff wire (Boston Scientific Corporation, Marlborough, MA) was positioned in the right atrium and an 11-Fr Mullins sheath (Medtronic, Minneapolis, MN) was advanced into the right atrium []. A Diabolo stent was then prepared using a 5-mm Amplatz Gooseneck Snare (ev3 Endovascular Inc., Plymouth, MN) positioned in the middle of a 16-mm BIB balloon (NuMed, Hopkinton, NY). A 2510 Genesis XD stent (Johnson and Johnson Health Care System Inc., Piscataway, NJ) was mounted over the centered snare. Fluoroscopy was used to confirm a proper position of the snare at the middle of the balloon. The distal end of the stent (up to the snare) was unsheathed in the right atrium and the angioplasty catheter was inflated. The entire system while the balloon was still inflated was pulled back to oppose the flared distal end against the atrial septum. Next, the remainder of the stent was unsheathed and the proximal portion was inflated. The stent with the atrial septum was constrained at 5 mm. It however became apparent that the large sheath distorted the anatomy and the stent was actually deployed in the left atrium []. The snare and balloon were removed and the stent was still over the wire. To reposition the stent, a 25-mm sizing balloon catheter was chosen and this was advanced across the stent and positioned so that most of the balloon was distal to the waist in the stent (toward the septum). The rationale behind this approach was to hold the stent using the balloon and then use it as an introducer to facilitate advancement of the stent. Once inflated in this position at low pressure, the larger distal portion of the balloon dilated the atrial septum and allowed the stent to be advanced without catching on the atrial tissue. Once the stent was nicely seated across the atrial septum the waist was further dilated to 8 mm []. Repeat hemodynamic assessment showed no residual gradient and significant drop in the Fontan pressure to 14 mmHg. Next, the fenestration was created by deploying another Diabolo stent. A 7 mm × 22 mm I-CAST covered stent (Atrium Medical Corporation, Hudson, NH) was taken off the existing catheter and was remounted on a 12 cm × 3 cm Optapro angioplasty catheter (Cordis Corporation, Bridgewater, NJ) and a 5 mm snare that was positioned at the middle of the balloon. The stent was re-crimped over snare then advanced through the long sheath into the left atrium. The stent was deployed in the same manner described earlier creating a Diabolo configuration across the pulmonary artery to atrial access site [Figure and ]. The covered stent prevented bleeding into the potential space. A repeat hemodynamics was performed and showed a reduction in Fontan pressure to 14 mmHg with systemic saturation in high 80% range. He had an uncomplicated post-procedure course and was discharged home the next day. He was bridged with Lovenox to Coumadin for systemic anticoagulation. He had rapid clinical and biochemical improvement (2 weeks) with increase in albumin levels from 2.2 to 3.2 gm/dL and resolution of ascites, facial edema, and diarrhea. |
A 72-year-old man, well apart from a history of eczema, underwent a left radical nephrectomy in 2006. Histology confirmed a T3b Fuhrman grade 3 clear cell renal carcinoma. Five years later he developed multiple lung nodules, biopsy of which confirmed metastatic clear cell carcinoma. He was treated for 2 years with Sunitinib, switching on progression to Axitinib, which he took until further progression in February 2015.\nHe became troubled by symptoms 2 years later with a persistent cough and lumbar back pain. A new baseline computed tomography (CT) scan of his chest, abdomen and pelvis showed a significant increase in size and number of the pulmonary nodules; increasing hilar and mediastinal lymphadenopathy; two suspicious right renal deposits and a new lytic bone lesion in the L1 vertebrae.\nHe consented to Nivolumab and Cycle 1 was given at a dose of 3 mg/kg. Nine days after this, he presented to the Emergency Department with fever, chest pain and shortness of breath. A cardiovascular event was excluded on the basis of a normal ECG and troponin level. Blood tests were notable for a high CRP of 442 mg/L. He was treated with antibiotics and given 2 mg/kg of methylprednisolone. A high resolution CT chest excluded pneumonitis. He improved to the point of discharge 6 days later. No source of infection was isolated, it was postulated that his symptoms could represent a response related inflammatory process.\nIn the outpatient clinic one week later he reported symptoms of cough, weight loss and a poor appetite. Bloods showed liver and renal dysfunction with an ALT of 124 U/L and creatinine 160 umol/l. A random glucose level was raised at 21.6 mmol/L. Insulin therapy for diabetes was initiated, thought to be either as a complication of immunotherapy or more likely of steroid treatment. Both his liver and renal function had normalized by the time he was seen in clinic a month later. At this point his breathing was improved, his cough mostly resolved and he was weaning the prednisolone dose. After 1 cycle of Nivolumab he elected to stop therapy and discontinue oncology follow up.\nTen months later, in February 2018, he presented to his GP with acute symptoms of shortness of breath and cough. A chest X-ray showed resolution of the original lung metastases and mediastinal lymphadenopathy ().\nHe had previously declined repeat CT scans but agreed to have one 2 years after the immunotherapy. The images showed that original pulmonary metastases and mediastinal lymphadenopathy were no longer visible; there was a stable para-tracheal node and reduction in the presumed right renal metastases. A pathological fracture was seen affecting the bony metastasis at L1. There were no new sites of disease (). |
A 79-year-old gentleman with Stage I NSCLC of the lower lobe of the left lung, who underwent lobectomy 12 months earlier, presented with new onset hemoptysis. A new friable ~5 cm tumor along the bifurcation of anterior and the posterior segments of the right upper lobe was noted on bronchoscopic examination, with features of squamous cell carcinoma (SCC) confirmed on biopsy. Positron emission tomography nor contrast-enhanced computerized tomography (PET/CT) imaging showed the newly diagnosed endobronchial cancer or any other sites of PET avid disease elsewhere. The patient now had a diffusing capacity of the lung for carbon monoxide of <40%, therefore, he was not considered a surgical candidate. SBRT was also not possible because of the radiologically occult nature of this new cancer that precluded accurate target delineation for radiotherapy.\nThe patient agreed to go forward with HDR-EBBT. He underwent planning CT simulation after placement of brachytherapy catheters by the pulmonologist. Dose calculation was based on the bronchoscopic evidence of extent of endobronchial disease that was ~5 cm, with 1 cm of additional length proximally and distally. After carefully evaluating the CT-based brachytherapy treatment plan, a dose of 7 Gy was prescribed at a depth of 0.8 cm from the source axis based on the adequate coverage of the mucosal lesion and without under dosing the endobronchial tumor. A total length of 7 cm was treated []. The patient received 4 weekly sessions, for a total of 28 Gy. CT-based planning was done for each brachytherapy session. He tolerated the treatment very well without any acute or late side effects and was followed every 3–4 months with bronchoscopy examinations for 6 months and subsequently with routine surveillance imaging. Seven and a half years later in July 2018, the patient again developed episodes of hemoptysis that warranted evaluation with EBUS which revealed no evidence of cancer or bleeding from the previously treated lesion but revealed a new 2.1 cm spiculated nodule (standard uptake value maximum of 19.3) in the right upper lobe of the lung (located in the peripheral, posterior segment close to the fissure) that was confirmed on PET/CT as well. This was located at least 3.5 cm away from the previous location of the treated endobronchial SCC []. Given its location, and long duration of time from his prior treatment, this was considered a third primary lung cancer, which was successfully treated using SBRT in August 2018. His most recent CT scan in July 2019 shows no evidence of new or recurrent disease. An informed written consent was obtained from the patient for publication of his case. |
A 67-year-old Caucasian man presented to our hospital after an accident and emergency with a history of five hours of sudden-onset lower abdominal pain. Nine months previously he had been admitted to our hospital with a stroke due to vertebral artery dissection. He developed acute urinary retention at the time, with a residual of 550 mL of urine. He was unable to sense normal bladder filling until he experienced the pain of bladder over-distension. Previous to this he had had no lower urinary tract symptoms. His urological history included an incidental finding of an 11 mm mass upon CT in June 2009 that raised clinical suspicions of a renal cell carcinoma that was under active surveillance. His other pertinent medical history included a left inguinal hernia repair in 2008 that was initiated by using a totally extra-peritoneal approach but was converted to an open repair because of pneumoperitoneum. The patient was a recent ex-smoker, had no significant family history of urological disease, and lived independently. He was taking latanoprost and prednisolone eyedrops.\nHis digital rectal examination revealed a moderately enlarged prostate, and a prostate-specific antigen test returned values within normal age-related limits. He underwent anti-coagulation with warfarin as treatment for the stroke and fitted with a long-term urinary catheter that was left on free drainage.\nFour months after he was fitted with the long-term catheter he had an episode of frank hematuria upon a routine catheter change. A cystoscopy was subsequently performed, which showed edematous urothelium but no focal lesions, as well as an open prostatic fossa. A trial without catheter was performed to determine whether his bladder function had recovered. This resulted in the patient's going back into urinary retention with abdominal pain. Re-catheterization drained 500 mL of urine. The catheter was replaced, and the patient was discharged with an out-patient appointment to discuss future management options.\nIn the interim, the patient presented to the emergency department with acute-onset lower abdominal pain. This pain was associated with diarrhea and vomiting over the preceding 24-hour period. His indwelling urinary catheter was changed without resolution of symptoms or drainage of a significant volume of urine.\nAn examination revealed that he was afebrile and cardiovascularly stable. His abdomen was non-distended but tense with guarding over the lower abdomen. Bowel sounds were heard. Urethral re-catheterization had drained 100 mL of urine with some light hematuria and debris in the catheter bag. Urine analysis showed 4+ blood, 4+ leukocytes, 1+ protein, and +ve nitrites. His blood tests showed neutrophilia (12.3 mL × 109/mL) with a raised C-reactive protein level of 67 mg/L. He was in acute renal failure with a creatinine level of 186 mmol/L (compared with 51 mmol/L three months previously). Plain X-rays showed distended small bowel loops over the central part of the abdomen with a collapsed large bowel and no focal lung lesions or subdiaphragmatic gas. A provisional diagnosis of a urinary tract infection was made, and he was admitted under the care of the physicians. He was treated with intravenous antibiotics (piperacillin/tazobactam combination) and fluid resuscitation.\nHis symptoms failed to settle over the next two days, with continued loose stool, nausea, and vomiting. His urine output was good throughout (> 60 mL/hour), and his renal function normalized. However, he had regular spikes of fever reaching 38.4°C, and his inflammatory markers were raised further. A urological opinion was sought. A consultant urologist diagnosed intra-abdominal sepsis and requested general surgical involvement. CT of the abdomen and pelvis was requested.\nCT showed small bowel obstruction with a transition point just above the dome of the bladder. The patient's bladder was abnormal and diffusely thickened with gas within it that tracked through the bladder dome and into the soft tissues superior and anterior to the bladder, where it was contained and formed several gas pockets that tracked toward the umbilicus. Extensive stranding was present around the dome of the bladder at the point of transition with the small bowel.\nThe patient was taken immediately to the surgical theater for an exploratory laparotomy. A rigid cystoscopy was first performed, which showed a large defect in the dome of the bladder with a possible fistular or urachal mouth in close proximity. Biopsies of the bladder wall were taken close to the defect in the bladder dome. Laparotomy revealed a large defect in the dome of the bladder adjacent to a thickened and abnormal possible urachal remnant (Figure ). The small bowel was dilated without any site of obstruction or bowel pathology. The bladder defect was excised with part of the wall of the bladder to allow repair. Stents, a suprapubic catheter, and two drains were placed. No obvious tumor was seen.\nA histological examination of the bladder wall showed severe transmural inflammation and necrosis predominantly outside the bladder but also involving peri-vesical adipose tissue. The urothelium was reactive but unremarkable. Acute inflammation of the urachal segment extended focally to involve the mucosa, which was lost extensively. In a single section of the bladder wall, a urothelium-lined structure was identified within the lamina propria that was surrounded by smooth muscle. This may have represented a urachal remnant. No tumor, definite urachal remnant, or underlying cause of the inflammation and necrosis was identified.\nFollowing a three-day post-operative stay in the intensive therapy unit, the patient was discharged to a general ward. His recovery was complicated by a post-operative ileus requiring total parenteral nutrition and some superficial wound dehiscence. He was then discharged to rehabilitation in a community hospital 26 days after admission and eventually fully recovered. |
A 64-year-old man visited the emergency room for abrupt-onset chest pain. He had undergone a closed thoracostomy for left hemothorax in another local clinic. Whole body computed tomography imaging indicated a possible ruptured thoracic aortic aneurysm; the maximal diameter of the thoracic aorta was 65 mm (). The patient underwent an urgent thoracic endovascular aortic repair procedure the next day. Since the proximal landing zone of the stent graft was estimated to be between the innominate artery and the left common carotid artery (zone I), he concomitantly underwent the debranching of the arch vessels. All procedures were performed in the operating room. The extrathoracic exposure of the arch vessels was performed via two small supraclavicular incisions. The debranching of the arch vessels was done by bypassing the left common carotid artery and the left subclavian artery to the right common carotid artery using a single 8 mm vascular graft (InterGard Woven; InterVascular, La Ciotat, France). The course of the graft crossed above the trachea and above the left internal jugular vein. The thoracic endovascular aortic repair procedure was carried out using a SEAL thoracic aortic stent (SEAL Thoracic 38/34×180 mm; S&G Biotech Inc., Seongnam, Korea) via both femoral arteries. All procedures were uneventful. The patient underwent perioperative cerebrospinal fluid drainage to prevent paraplegia.\nPostoperatively, the patient twice failed to be weaned off the ventilator because of intractable stridor and desaturation immediately after extubation. He underwent reoperation on postoperative day four due to suspected airway edema caused by the obstruction of venous return. When neck ultrasonography was performed in the operating room, a large amount of thrombi was found in the left internal jugular vein and no flow was found through the left internal jugular vein. In the operative theater, the left internal jugular vein was found to be totally obstructed by thrombosis (), which might have been caused by compression from the bypass graft. The reoperation was done by thrombectomy and left internal jugular vein intervenous bypass using an 8-mm ringed graft (EXXCELL SOFT ePTFE vascular graft; MAQUET cardiovascular LLC, San Jose, CA, USA) (). After reoperation, the patient’s upper airway symptoms improved, but he still displayed delirium and poor spatial orientation. He was weaned off the ventilator five days after the second operation and transferred to a general ward on postoperative day eight. He was readmitted to the intensive care unit because of stridor and delirium on postoperative day nine. On an upper airway exam, bilateral vocal cord palsy was noted. Thereafter, he was given steroid medications and was weaned off the ventilator four days later. On postoperative cardiac computed tomography, a patent debranching graft was noted and the thoracic stent graft was well positioned with no evidence of endoleak (). On a follow-up vocal cord exam, his bilateral vocal cord palsy had improved. He was discharged from hospital on postoperative day 30, but was prescribed oral aspirin for anticoagulation to maintain the patency of the intervenous graft. |
An 80-year-old Caucasian man who initially presented with back pain was found to have a 9.8 cm infrarenal abdominal aortic aneurysm on computed tomography (CT). This was suitable for endovascular aneurysm repair (EVAR) but the left common iliac artery (CIA) was short, necessitating the extension of the left iliac limb into the external iliac artery (EIA). The decision was made to proceed with an on table coiling of the left internal iliac artery (IIA) and EVAR. However, a tortuous right CIA made coiling of the left IIA extremely difficult and following a prolonged attempt, the decision was made to proceed with EVAR without coiling the IIA (Fig. ).\nOn a 1-month follow up CT the left limb extension had disengaged from the main body, resulting in a large Type 3 endoleak. The disengagement was presumed to be due to the tortuosity of a heavily calcified external iliac artery returning to its anatomical position on removal of the Meir wire (Boston Scientific). The left IIA remained patent and acted as an outflow for the type 3 endoleak. The left limb was then realigned successfully with termination of the type 3 endoleak.\nOn a follow up CT 1 month post repair, the left IIA remained patent with a large type 2 endoleak demonstrated but the sac size remained static (Fig. ). The failure of the IIA to occlude was presumed due to the cavity created within the aneurysm sac from the type 3 endoleak. Due to the large size of the aneurysm and the relatively large size of the type 2 endoleak the decision was made at multi-disciplinary team meeting to embolise the type 2 endoleak.\nAs antegrade access to the left IIA was not possible due to the presence of the stent graft, an attempt was made to access the internal iliac artery via the buttock. Direct sac puncture was not possible as the endoleak was located within the pelvis and surrounded by bony structures and pelvic viscera. The intention was to puncture the posterior division of the IIA but visualisation with ultrasound was limited and a vessel was punctured, which on subsequent angiography was shown to be the anterior division of the left IIA (Fig. ). The left IIA was embolised using Spirali (Pyramed) coils in a retrograde manner. Due to the absence of a solid structure which to compress the access vessel against, especially as the anterior division of the IIA had been punctured, the arteriotomy needed to be closed using an Angio-Seal (Terumo). Correct intraluminal placement of the Angio-Seal (Terumo) is dependent on backflow of blood into the Angio-Seal (Terumo) sheath from the artery. However, as the left IIA had been embolised, backflow from the Angio-Seal (Terumo) was non-existent. The angioseal was not visualised on ultrasound and therefore a 4 French dilator was inserted within the Angio-Seal (Terumo) sheath through which contrast was injected to directly visualise the tip to the sheath within the artery (Fig. ). The sheath was then withdrawn until just inside the vessel lumen and the plug deployed with immediate haemostasis (Fig. ). This was an off-label use of the Angio-Seal (Terumo).\nA follow up CT 1 month post procedure revealed resolution of the Type 2 endoleak. |
A 23-year-old male patient from India, without any past medical history, presented with pain and swelling in small joints of right hand with morning stiffness lasting for more than one hour along with feeling of warmth of the hands. The symptoms were of six-week duration, and within the week prior to being seen he also developed similar pain in the joints of his left hand. The pain was constantly present with exacerbation on touch and on movement at joints of hands. There were no constitutional or systemic features and he denied any history of trauma.\nOn examination there was marked tenderness over and around small joints of both hands, particularly on right side. There was diffuse swelling of the right hand which was more over and around the joints, and also the hand was slightly warm to touch (). There was decreased range of motion at the wrist and small joints of hand. Similar findings with lesser severity were also present on the left hand. Apart from marked joint tenderness there was no significant dysaesthesia or sensory loss over the hands. He reported difficulty in performing routine activities with his hands due to severe pain.\nPrior to his presentation to us his family physician had him tested for rheumatoid factor and anticyclic citrullinated peptide antibodies, and both were negative. He had also received multiple analgesics and anti-inflammatory medicines without any significant relief.\nOn further evaluation, his laboratory parameters including haemoglobin, blood counts, kidney and liver function tests, and urine examination were normal. His erythrocyte sedimentation rate was 5 mm/hour (normal range 0–10 mm/hour) and C-reactive protein was 1.8 mg/dL (normal range 0–0.75 mg/dL). Plain radiographs of the chest and both hands (including wrists) were normal.\nMusculoskeletal ultrasonography was done which revealed diffuse subcutaneous oedema of both hands (more on the right side) without any evidence of synovitis (). In view of no concrete evidence of inflammatory arthritis in his laboratory and radiological investigations and poor response to anti-inflammatory medicines, his history was retaken and on direct questioning he revealed the history of venipuncture for blood sampling from right antecubital region, done for his routine health check one day prior to the onset of symptoms. Venipuncture was done skillfully and he denied any history of sudden electric current like sensation, pain, tingling, weakness, or loss of sensation after the procedure.\nA possibility of complex regional pain syndrome was strongly considered as the patient fulfilled the Budapest diagnostic criteria; he had continuing pain disproportionate to the inciting event, hyperalgesia, vasomotor changes in the form of rise in temperature of the hands, sudomotor changes in the form of oedema of hands, and motor finding of decreased range of motion at wrist and small joints of hands. No other condition could be found that could have explained the clinical scenario.\nIn order to further strengthen the diagnosis a triple-phase radioisotope bone scan was done which revealed findings highly suggestive of CRPS in both upper limbs (). The bone site of increased uptake was exactly the site that Sudeck first described periarticular demineralization [].\nPatient was treated with pregabalin, a single intramuscular depot preparation of 80 mg methylprednisolone, physiotherapy, and counseling. His symptoms gradually abated within two months of therapy and swelling of the hands subsided (). His pregabalin was tapered off without any recurrence in the next one year of follow-up. |
A 39-year-old female was brought to our emergency room by a private vehicle after being shot. An evaluation revealed a wound at the lower left back and at the mons pubis. Exploratory laparotomy and resection of the sigmoid colon, left ovary, and fallopian tube with a colostomy was performed. Postoperative deep venous thrombosis prophylaxis in the form of enoxaparin, 30 mg every 12 hours, was given and then changed to heparin, 5,000 units subcutaneously every eight hours, due to worsening renal function. The patient did well until postoperative day 4 when she complained of increased abdominal pain out of proportion to the clinical findings. Her symptoms worsened the following day. A computed tomography (CT) scan of the abdomen and pelvis was obtained, the results of which were consistent with postoperative ileus and raising concerns for right colon ischemia.\nThe patient was managed conservatively, and bedside drainage of the superficial wound infection was done on postoperative day 6 with improvement in abdominal pain. On the following day, a repeat CT of the abdomen and pelvis raised more concerns for bowel ischemia; however, the patient refused reexploration (Figure ).\nA computed tomography angiogram (CTA) of the chest was obtained for worsening pulmonary symptoms on postoperative day 8 and was consistent with lower lobe pneumonia.\nFeculent drainage around the stoma was noticed on postoperative day 9, and an exploratory laparotomy was performed with resection of multiple small bowel ischemic loops, cholecystectomy, and right hemicolectomy. The rest of the small bowel appeared dusky and friable. After multiple visits to the operating room, she was left with only a few inches of the proximal jejunum. A CTA of the abdomen was done on postoperative day 13 from her initial surgery and was consistent with a superior mesenteric artery occlusion (Figure ).\nAdmission platelet count was 281, down to 82 on postoperative day 11 when she tested positive for heparin-induced antibodies. The pre-test probability for HIT score was high (2 for thrombocytopenia, 1 for timing, 2 for thrombosis, 2 for no other cause = 7). The heparin was stopped and argatroban was used. The patient did survive her injury and was referred to a small bowel transplant center. |
A 61-year-old Caucasian male was brought to the ER with an initial diagnosis of acute ST-elevation myocardial infraction by the emergency medical services (EMS). On further questioning, the patient denied history of chest pain, but complained of sudden onset generalized weakness and numbness. The patient had developed weakness of his legs and difficulty in passing urine one hour before arriving in the ER. Patient denied recent infections, headaches, nausea, vomiting, trauma or a similar neurological episode before. The patient had end stage renal failure due to poorly controlled diabetes and hypertensive heart disease for many years. He had been on routine hemodialysis thrice a week with each dialyzing session lasting for four hours for the past three years. The last dialysis was done the day prior to presentation. His medications included metoprolol 100 mg twice a day, simvastatin 40 mg daily, aspirin 81 mg daily and 70/30 insulin 30 units subcutaneously twice a day.\nOn examination, the patient's had a temperature of 98.2°F, respiratory rate of 20 per minute, heart rate of 90 beats per minute and a blood pressure of 150/90 mmHg. Examination of the cardiovascular system showed normal heart sounds with no murmurs. The respiratory system and gastrointestinal systems were normal. Central nervous system examination revealed an alert, awake and oriented patient with normal cranial nerve function. He had symmetrical and equal weakness of the lower limbs more than in the upper limbs. The muscle tone and the reflexes were also weaker in the lower limbs compared to the upper limbs. There was no sensory deficit. In the ER, blood tests including chemistry and complete blood count were sent. A bed side EKG and a chest X-ray were performed.\nBased on the initial two EKG strips done by the EMS and the subsequent two EKGs done in the ER (Figure: , , , ) and the clinical back ground of end stage renal failure the diagnosis of hyperkalaemic flaccid paralysis was made. The patient was given 10 ml of 10% calcium gluconate intravenously followed by 10 units of regular insulin, and 50 ml of 50% dextrose. The patient also received 30 ml of sodium polystyrene sulfonate (Kayexalate) orally that was repeated every hour till the patient developed a diarrhea.\nSubsequently the patient's blood results showed potassium of 6.2 mEq/L, blood urea nitrogen (BUN) of 152 mg/dl and creatinine level of 8.50 mg/dl. Patient was rushed for emergency dialysis. After three hours of dialysis, the patient recovered completely from his weakness and was able to walk. His post dialysis potassium level was 4.0 mEq/L, BUN was 47 mg/dl and creatinine was 3.57 mg/dl. The EKG had reversed back to sinus rhythm, heart rate of 88 per minute, normal T waves and ST segment (Figure ). |
A 50-year-old woman was referred to the Infectious Disease Clinic for nonhealing painful hand wounds, twelve days after sustaining dog bites on the dorsum of both hands. She had a history of insulin-dependent diabetes mellitus for two years and hypertension. She was initially evaluated at an urgent care center in Rhode Island on the day of the dog bite. At that time, she was noted to have open wounds on the dorsum of both hands which were subsequently cleaned and stitched. She was given a fourteen-day course of amoxicillin. Six days after the initial dog bite, she returned to the urgent care center to have the stitches removed. At that time, she was noted to have an enlarged, draining nodule on the left hand. As a result, the stitches were only removed from the right hand. The bloody drainage from the left hand wound was sent for culture. Three days later, nine days after initial dog bite, she was called back to the urgent care center and was told mold and bacteria grew in the culture and that the mold was likely a contaminant. At this visit, a small eschar replaced the draining nodule on the dorsum of her left hand. The stitches were removed from the left hand and a black, stringy material was noted in the wound. This material was sent to the microbiology laboratory for culture, and the wound was packed. The patient continued her amoxicillin and eleven days after the dog bite, she returned to the urgent care center for the fourth time and once again was told that the culture grew the same bacteria and mold, which was later identified as Enterobacter cloacae and Mucor species (). The patient was referred to the Infectious Disease Clinic.\nThe patient denied any other unusual exposures following the dog bites. She denied fever or chills. Her current medications were insulin, metformin, aspirin, and lisinopril. She had no known drug allergies. At the Infectious Disease Clinic, her exam was remarkable for normal vital signs and bilateral hand ulcers, left greater than right (). The patient was sent from the Infectious Disease Clinic to the Emergency Department (ED) in order to be evaluated for debridement. We recommended that the patient receive meropenem 1 mg/kg and 5 mg/kg of amphotericin B (lipid complex; Abelcet; Enzon Pharmaceuticals, Bridgewater, NJ, USA).\nIn the ED, the patient's white blood cell (WBC) was 7900/uL, with a normal differential and normal chemistry panel. Her hemoglobin A1c was 7.8%. Blood cultures were drawn, which were negative for growth. Within three hours of arrival to the ED, the patient was taken to the Operating Room (OR) for debridement of left dorsal hand wound. The surgeons debrided down through the subcutaneous tissue to the level of the peritenon, which are the connective tissue structures attached to and surround the tendon (). The specimen was sent to pathology and microbiology. This time, the cultures did not grow. The pathologist found chronic active ulcer and organizing abscess in the surgical specimen.\nThree days after surgical debridement, the patient's wound was healing well, and her intravenous antibiotics were switched to ciprofloxacin 500 mg orally every twelve hours for two weeks and posaconazole 100 mg orally every twelve hours for two weeks. |
A 60 year old diabetic, non smoker, housewife presented to us with complaints of gradual onset, progressive dysphagia initially for solid and later for liquid food for one and a half year and foreign body sensation in throat for 6 months. She had lost 10 kg over last 6 months, despite having a good appetite. She did not have significant medical or surgical illness or intervention in the past. General and systemic clinical examination was normal. Her hematological and biochemical investigations were normal too. Barium esophagogram revealed smooth narrowing of the mid thoracic esophagus with proximal dilatation (Fig. ). Esophagoscopy was suggestive of a submucosal growth with intact mucosa from 18 to 25 cm from central incisors, involving half of the circumference of esophagus, located at 12 to 6 o’ clock position (Fig. ). Contrast enhanced computed tomography (CECT) scan of neck, chest and abdomen revealed a longitudinally oriented, well defined, non-enhancing, homogenous lesion involving the lower cervical and upper thoracic esophagus, causing significant luminal narrowing (Fig. ). The patient underwent a right lateral thoracotomy. Esophageal dissection was done and longitudinal incision was made in the esophagus, over the lesion which was deepened through the muscle layer and enucleation of the lesion was performed. It was a solid, submucosal mass measuring 8.5 × 6 × 2.5 cm (Fig. ). While dissecting the mass out, there was a 6 cm long clear rent in the mucosa but as the tissue was healthy, uninflamed and well vascularised, it was primarily repaired in two layers and reinforced with adjacent pleural flaps. She was kept on partial parenteral nutrition, intravenous Pantoprazole, nasogastric drainage and enteral feeding was done through feeding jejunostomy. Contrast esophagogram on seventh postoperative day revealed normal esophagus (Fig. ). She was started on liquid diet initially and later she could swallow both solid and liquid food without dysphagia. She was discharged from hospital on tenth postoperative day. Gross pathology demonstrated an encapsulated solid mass with cut surface showing solid white areas without hemorrhage, necrosis and calcification. The microscopic examination of the mass revealed proliferation of spindle cells with stroma of proliferative blood vessels and lymphoplasmacytic infiltration with formation of lymphoid follicles (Fig. ). The cells were immunonegative for Anaplastic Lymphoma Kinase (ALK). The patient is on regular follow up. At 6 months postoperatively she is doing well, without recurrence of her symptoms. Esophagoscopy done revealed normal esophagus. |
A 15-year-old male child presented with history of worsening right-sided weakness of 2 years duration. He had progressive vision loss in the left eye for the past 1 year. He had multiple episodes of focal-onset generalized tonic clonic seizures with poor drug compliance. Headache with projectile vomitings had started lately. On clinical examination, the patient was blind in the left eye, with a normal right eye. There was mild right-sided seventh nerve weakness and hemiparesis. A magnetic resonance imaging scan of the brain revealed a left-sided large supratentorial tumor extending from the brain surface in the frontal and parietal cortex to the atrium and frontal horn of the lateral ventricle, causing significant compression []. There was brain edema with evidence of tentorial herniation. The tumor had both solid and cystic components []. The tumor showed heterogenous contrast enhancement after gadolinium injection []. Taking into consideration the radiological findings, a possibility of primitive neuroectodermal tumor was made. At surgery, after the bone flap was elevated, the cystic component of the tumor was found to be infiltrating the duramater near the lateral parietal region, causing an impression over the inner surface of the bone. The cystic component of the tumor contained xanthochromic fluid. The solid component was firm, moderately vascular and poorly suckable. A gross total resection of the tumor was performed along with resection of the infiltrated duramater overlying the parietal lobe. The patient had complete relief of headache and vomiting in the postoperative period and was free of seizures on phenytoin. A postoperative computed tomography (contrast) scan of the brain [] revealed total excision of the tumor. Histopathology of the tumor revealed a diagnosis of CCM WHO grade II. The tumor was composed of lobules of loosely arranged cells set in a very loose myxoid stroma. The cells had scanty cytoplasm with indistinct cell margins and slender ovoid nuclei, some with intranuclear cytoplasmic inclusions []. The tumor cells were positive for Epithelial membrane antigen (EMA) [] and vimentin and negative for S-100, synaptophysin, Glial fibrillary acidic protien (GFAP), cytokeratin and desmin. The MIB -1 index of the tumor was 8%. The patient is doing well at 18 months of follow-up. |
A 37-day-old girl was admitted to our Pediatric Surgery Unit with abdominal obstruction.
The patient was born at 38 weeks’ gestation (birth weight 2755 g). She was breastfed at birth. On day 13, the physical evaluation in the primary pediatric care setting revealed tachycardia with a heart rate (HR) of more than 200 beats per minute (bpm). No vomiting, constipation or abdominal distension were noted. The asymptomatic girl was admitted to the Neonatal Intensive Care Unit for further investigation.
On admission, the 13 days old term infant had a SVT of 278-294 bpm which was refractory to vagal stimulation, electrical cardioversion, and repeated doses of adenosine. She was reverted to sinus rhythm with amiodarone and flecainide on the 4th day of admission. Echocardiography showed normal heart anatomy, borderline and dyskinetic cardiac function and a left ventricle ejection fraction of 50%. Day 23 of life she again developed SVT, and for the 3rd time on day 25, both episodes reverted to sinus rhythm by adenosine. In conclusion she had refractory SVT initially for 4 days followed by 2 episodes of recurrent SVT, which were not refractory and responded to adenosine.
Abdominal distension was initially noted on day 16 (3 days after admission) while still in the phase of refractory SVT, for which she received glycerin suppository, this was followed by decreased activity which was attributed to sepsis and treated with antibiotics. The infection markers remained static and did not drop as expected in spite of 14 days on antibiotics. On day 32 of life, surgical evaluation was undertaken due to worsening abdominal distension. Intestinal obstruction was strongly suspected. Imaging showed dilated loops of bowel with no free air in the abdomen. On contrast enema severe stenosis at the transverse colon, with dilated small bowel loops, were seen (). During preparation for laparotomy, she developed fecal vomiting (sign of distal small bowel or colonic obstruction). At laparotomy the terminal ileum and a jejunal loop with oment |
A 51 year old man presented to our outpatient department with an increasing swelling in the right distal upper arm. He reported about local pain without radiation. The patient´s medical history was without previous infections, surgeries or other diseases. The mass in the arm presented solid and relocatable. The examination showed full strength in all upper extremity muscles, especially in the forearm flexors, in M. pronator, M. abductor pollicis brevis, M. flexor pollcis brevis, M. opponens pollcis and Mm. lumbricales I and II. No sensory loss in the upper arm, the forearm, the palm and dorsum of the hand and the fingers could be found.\nMRI of the upper arm showed a spindle-shaped homogeneously contrast enhancing mass. It was located some centimeters above the crook of the arm within the medial sulcus bicipitalis. In the imaging it showed a relationship to the median nerve main branch of the forearm or seemed to originate from part of its fibres, respectively. Its diameter was about 11 × 4 centimeters (). The primary diagnosis from the radiologist was schwannoma.\nSurgical extirpation was indicated and performed. In its middle part the exposed tumour had a smooth capsule which was opened (). In its equator the surface had a good boundary to the surrounding tissue (). It did not extend to the muscles or tendons. In its distal and especially in its proximal ending the tumour showed a more infiltrative growth (). A feeding fascicle could be identified and was cut after ensuring by electric stimulation that it had no motor function. But with the intention to set no damage at the main nerve trunk approximately twenty percent residual tumour was left ().\nThe postoperative course was uneventful. The patient suffered a light hypesthesia in the forearm. This did not match to the supply territory of the median nerve which is the palmar hand. It rather corresponded to another skin nerve, possibly damaged by the approach. There was no new motor function deficit in the forearm flexors, in M. pronator, M. abductor pollicis brevis, M. flexor pollcis brevis, M. opponens pollcis and Mm. lumbricales I and II. A local upper arm pain vanished in the course of two weeks.\nThe final histological examination of the tumour showed typical criteria of the Castleman disease with an effaced architecture of a lymph node with regressed germinal centers and typical high endothelial venules (). Immunohistochemistry demonstrated regressed atrophic germinal centers () and aberrant network of follicular dendritic cells (). The combination of these features ensured the diagnosis.\nTo exclude a multicentric disease the patient was admitted to the internal medical department. Entire virus tests including HIV were negative. A bone marrow biopsy showed a normal hematopoiesis without evidence for an infiltration by pathologic cells. A staging PET-CT showed no further organ manifestations. An unicentric form was approved in synopsis of all findings. In regard to the tumour rest and the curative approach of an unicentric M. Castleman the patient finally underwent a selective radiation of the upper arm [].\nIn a 6-month follow-up, the partly sensory loss in the forearm had remained. Except for this, the patient had no nerve related problems or restrictions in everyday life except for the sensory loss in the forearm. In a 18-month follow up he reportet on full functionality of the arm. Currently, the area of the tumour is regularly examined with sonography. |
KS, a 31-year-old woman with mild gastroesophageal reflux disease requiring no home medications, presented for planned open left hepatectomy (our preferred technique at the time) for liver donation. During the procedure, a replaced left hepatic artery was visualized and dissected to the level of its origin from the left gastric artery. To completely mobilize the replaced artery, neurovascular branches supplying the lesser curvature of the stomach were divided. Upon examination of the hepatic hilum, a standard left hepatic artery was found, as well as a small segment-4 artery from the proximal right hepatic artery. These additional arteries were not seen on preoperative imaging. Due to concern for increased risk of graft failure, the decision was made to convert to a right hepatectomy. The operation proceeded without complications. After removal of the right lobe graft, omentum and colon were placed in the resection bed, and Seprafilm adhesion barrier was applied to the cut liver surface. During the first postoperative week, the patient complained of nausea, heartburn, reflux, and nonbilious vomiting. The patient gradually improved with medical management including a proton pump inhibitor, sucralfate, and metoclopramide, and she was discharged home on postoperative day (POD) 9. On POD 31, the patient was readmitted for severe nausea, bilious vomiting, and burning epigastric pain. A computed tomography (CT) scan of the abdomen did not suggest bowel obstruction or adhesion formation between the stomach and cut liver surface (). Endoscopy revealed retained food products in the stomach, and a gastric emptying study demonstrated absent emptying over one hour (). Following initial improvement with medical management as described above, the patient experienced worsening emesis on POD 43 with solid food trial. During repeat endoscopy, 200 units of botulinum toxin were injected at the pylorus. Afterward, the patient experienced no further emesis and tolerated oral medications. An upper gastrointestinal series with small bowel follow-through showed no abnormalities. The patient was discharged home on POD 49. She underwent a repeat gastric emptying study one week after discharge, which showed marked improvement and normal gastric emptying (). In subsequent follow-up, she reports feeling well and eating normally. She is currently over four years postdonation. |
A 78-year-old African American man with medical history of hypertension was found to have an enlarged prostate associated with complaints of urinary hesitancy by his primary care physician. He was referred to urology and later underwent a TRUS-guided prostate biopsy. A few hours after the procedure on his way home, he had a syncopal episode and was transferred to the ER.\nHe was found to have copious amount of fresh blood clots per rectum with significant hemodynamic instability. He was diaphoretic with a blood pressure of 80/30 mmHg and a pulse rate 100 - 120/min. Patient was aggressively resuscitated with fluid and received a total of 5 units of packed red blood cells. He was transferred immediately to the intensive care unit.\nA flexible sigmoidoscopy performed revealed multiple diverticulosis in the left colon and a raised non-bleeding erythematous mucosal lesion in the rectum (most likely the site of prostate biopsy). The lesion was injected with 10cc of 1:10000 epinephrines (). We planned a full colonoscopy at later date when patient was more stable.\nWhile in the unit the patient had no further rectal bleed and his hematocrit remained stable (between 30-35%) and as such was transferred to the medical floor.\nFive days later, on the day prior to planned discharge he developed multiple episodes of massive rectal bleeding with a drop in hematocrit to 25% and drop in systolic blood pressure of 60 mmHg. A central line was placed and the patient received transfusion with 4 units of packed red blood cells and was transferred back to the intensive care unit. As he continued to bleed massively with hemodynamic instability we opted for an immediate angiography rather than a colonoscopy. A complete mesenteric angiography was performed ().\nThe selective inferior mesenteric artery angiogram revealed a brisk blush in distal portion of the superior hemorrhoidal artery and collection of contents in the rectum. Gel foam embolization of the superior hemorrhoidal artery with successful hemostasis.\nA follow-up selective inferior mesenteric artery angiogram revealed filling of proximal superior mesenteric artery without any filling in distal superior hemorrhoidal artery indicating an effective superior hemorrhoidal artery thrombosis ().\nFollowing the successful angiography and embolization, the rectal bleeding stopped. The patient was monitored in the ICU for another 48 h during which his hematocrit was stable and developed no further bleeding. He was discharged in a stable condition. |
A 60-year-old African American female was following up for her chronically elevated alkaline phosphatase levels. She had a history of hypertension, hyperlipidemia, type 2 diabetes mellitus, allergic rhinitis, and chronic lower back pain. Patient has a family history of arthritis, cardiovascular disease, and diabetes mellitus; she denies ever using alcohol or tobacco.\nWith the onset of elevated alkaline phosphatase level and vague abdominal pain in 2013, an abdominal ultrasound performed in December showed hepatic steatosis. Viral serologies for hepatitis during 2013 were negative, as a gastrointestinal consult was required to determine the need for a liver biopsy. A liver biopsy was subsequently performed, which showed focal mixed micro and macrovesicular steatosis. Portal tracts showed minimal focal chronic inflammation, no significant fibrosis, and no iron deposition.\nThe vague abdominal pain that she was experiencing waxed and waned for two years. Additionally, the patient experienced some vague chest pain and dyspnea that prompted an echocardiogram in February of 2015, which demonstrated a left ventricle ejection fraction of 44%. Consequently, a left heart catheterization in the following month showed no significant coronary disease with a dilated left ventricle with an ejection fraction of 50%. A 2-year follow-up in July of 2015 showed suspicious cirrhosis by Computed Tomography (CT) scan (), possibly due to granulomatous changes and chronic inflammation. A CT scan was determined to be necessary for our patient because of rising alkaline phosphatase without other explainable etiologies, in addition to the patient's appetite suppression and vague abdominal pains. Patient's weight during this time was 207 lbs (93.89 kg) and was advised to diet and exercise. After 4 months of continuous symptoms, especially with abdominal pain, a laparoscopic cholecystectomy was performed with a liver biopsy that showed subsequent granulomatous changes in September of 2015. The liver biopsy showed coalescing periportal nonnecrotizing epithelioid granulomas with associated multinucleated giant cells and chronic inflammation. The chronic and patchy inflammation is representative of the granulomatous hepatitis, despite not having elevated transaminases. The granulomatous changes suggested possible sarcoidosis (). The liver biopsy was not histologically suggestive of nonalcoholic steatohepatitis, with no steatosis noted. Chest X-ray at that time showed no significant findings.\nInflammatory bowel disease (IBD) was not explored in the patient because she never had clinical signs on past or present examinations, denying any symptoms of IBD including alternating bowel habits, predominant constipation, or diarrhea. Laboratory studies 2 years and 6 months since the onset, at the end of 2015 showed negative antinuclear antibodies (ANA), negative rheumatoid arthritis factor, negative cyclic citrullinated peptide (CCP) antibodies (IgG/IgA), elevated C-reactive protein of 8.9 mg/L, normal complement C3/C4, elevated B-type natriuretic peptide 688.6 pg/mL, and negative mitochondrial (M2) antibody. Subsequent office visits and additional laboratory results are shown in .\nAdditionally, patient experienced peak weight loss with a weight of 154 lbs. (69.85 kg) in 2016. During this time period, an echocardiogram showed a decreased ejection fraction of 26%. This result and a history of having ventricular tachycardia resulted in the patient having an automated implantable cardioverter-defibrillator (ICD) placed.\nUrinalysis in June 2017 showed RBC 0-5/hpf, WBC 0-5/hpf, bacteria 2+, and moderate calcium oxalate crystals. Additionally, patient's weight increased in 2017 to 190 lbs. (86.18 kg). |
A 16-year-old Malay boy was admitted to our hospital in December 2003 for surgical management of increased intracranial pressure. He underwent external ventricular drainage and stereotactic biopsy. The histological report was consistent with demyelination. He had recurrent episodes of acute onset severe headache and vomiting with a mild unsteady gait, first in August 2003, then in September 2003 and the last in October 2003 leading to hospital admission. In the first two occasions, CT and MRI scans revealed obstructive hydrocephalus with a lesion, enhanced with contrast in the region of the fourth ventricle. He was treated with steroids since he refused surgery and recovered fully in 1 to 2 weeks. He was well until early October 2003 when he had recurrent headache and subacute onset of a severe unsteady gait. Because of this, he became bedridden. The symptoms continued and in November 2003, he complained of blurring of vision and lost his sight 3 weeks later. The examination revealed bilateral optic neuritis with multiple left cranial nerve palsy (IX, X and XII), bilateral pyramidal tract signs (more prominent on the right side) and cerebellar signs. Autoimmune antibodies and serology screening for HIV, syphilis and hepatitis B and C were negative. VEP was absent in both eyes. BAEP was suggestive of bilateral auditory pathway dysfunction. The MRI brain scan showed multiple small lesions in the thalamus, centrum semiovale and brainstem and an ill-defined lesion in the cerebellum which appeared to be hypointense on T1-weighted images and hyperintense on T2-weighted images, not suppressed by fluid-attenuated inversion recovery (FLAIR), and enhanced with contrast (Figure ). In view of the remitting and relapsing course of the illness, involving multiple sites in the central nervous system - optic nerve, brainstem, cerebellum and cortex - his diagnosis was revised to MS. The cause of his hydrocephalus was a strategically located demyelinating plaque causing compression of the fourth ventricle. He was treated with intravenous methylprednisolone for five days and tapering oral prednisolone for one month and showed some improvement to the extent that he was able to perceive light and sit up without support. |
A 63-year-old Caucasian man presented to his primary care physician with a 6-month history of intermittent right anterior neck and intraoral pain. The patient noted a tongue mass, which had grown substantially over the last several months. The mass made eating difficult at times and resulted in one episode of mild oral bleeding that resolved spontaneously. He was referred to our institution's department of otolaryngology/head and neck surgery for further evaluation.\nThe patient's past medical history is significant for RCC of the right kidney diagnosed 4 years prior and treated with right radical nephrectomy. An appropriate work-up at that time included a CT scan of the chest, abdomen, and pelvis and liver functions tests, all of which were negative for metastatic disease. He did not follow-up with his urologist as recommended after the surgery.\nThe physical exam revealed an erythematous, indurated 3 cm mass in the right anterior floor of mouth region that was tender to palpation. It was not fixed to the mandible and appeared vascular. The neck exam was positive for a 3 cm firm mass in the right thyroid lobe with no pathologic lymphadenopathy otherwise.\nBiopsy of his anterior floor of mouth lesion was notable for persistent bleeding and revealed clear cell carcinoma, consistent with the patient's previous history of renal cell cancer (Figure ). Histologic evaluation revealed the presence of a solid nest of epithelial cells with clear cytoplasm and small, round hyperchromatic nuclei (Figure ). A rich vascular network was also noted. Immunoperoxidase testing was positive for CD10 and vimentin and negative for gross cystic disease fluid protein (GCDFP), S-100, HMB-45, muscle-specific antigen, and desmin, supporting the diagnosis of metastatic RCC (Figure ).\nOriginal surgical, pathology and postoperative records were eventually obtained revealing the discovery of suspicious lymph nodes near the renal hilum during his original nephrectomy. The resected lymph nodes were found to harbor metastatic carcinoma and the patient was referred to a medical oncologist at that time to discuss additional therapeutic options. Unfortunately, he did not seek further care for his metastatic RCC. After receiving the news of his biopsy results in our clinic, the patient decided to return to his original institution for further care and died of metastatic disease within several months. |
A 16-year-old girl presented with transient left upper and lower limb numbness and headache while participating in chorus and sports. She was referred to our department for surgical treatment. She had a past medical history of immunoglobulin A nephropathy and was being treated with steroids. Magnetic resonance (MR) images showed no ischaemic changes in her brain, but MR angiography showed intracranial vascular stenosis []. Digital subtraction angiography showed bilateral stenoses from the terminal portion of the ICA to the proximal parts of the middle cerebral and anterior cerebral arteries []. Extended perforating branches were also noted. Single-photon emission computed tomography (SPECT) imaging showed decreased cerebral blood flow (CBF) on the right side [], which worsened after an acetazolamide challenge []. Based on these findings, she was diagnosed with Suzuki Stage 2 moyamoya disease bilaterally[] and underwent revascularisation on the symptomatic right side.\nDuring surgery, both direct and indirect bypasses were successfully performed; however, involuntary movements appeared immediately after the operation, with her right upper limb episodically shaking at a frequency of 3 to 5 Hz []. These episodes lasted for a few minutes up to a few hours, with no associated disturbances in her level of consciousness and no incontinence. The involuntary movements were limited to the right upper limb and did not occur during sleep. On the next day, MR angiography showed good patency of bypass vessels, and MR images showed neither cerebral infarction nor hemorrhage [ and ]. Electroencephalography (EEG) showed no abnormal brainwaves suggestive of epilepsy. SPECT performed on postoperative day 3 revealed decreased CBF in the bilateral frontal lobes [].\nThe frequency of her involuntary movements gradually decreased, and SPECT imaging showed slight improvement of the hypoperfusion in her bilateral frontal lobes; however, this symptom and her abnormal imaging findings persisted, though to a lesser degree. Therefore, a left-sided revascularisation procedure was performed on postoperative day 47. The following day, her symptoms disappeared and did not recur since then. Furthermore, blood flow in the bilateral frontal lobes was improved according to SPECT imaging performed 7 days after the second surgery []. |
A healthy 21-year-old man was admitted to our hospital after being referred from another medical institution because of worsening left eye (LE) uveitis. He was treated 3 weeks earlier with oral acyclovir and topical steroids because of suspected LE herpetic anterior uveitis as serological tests revealed positive immunoglobulin M to herpes simplex virus-1. Initially he responded to treatment but 2 weeks later, his vision declined from 6/9 to counting fingers (CF), resulting in his referral to our center.\nThe patient denied any relevant past medical history. However, he mentioned that a month earlier he was examined for LE discomfort and diagnosed with allergic conjunctivitis following minor blunt trauma from a tree branch.\nOn examination, visual acuity (VA) was 6/6 in the right eye (RE) and CF at 1 meter in the LE. Intraocular pressure was 14 mmHg in both eyes. RE anterior and posterior segments were normal. There was a remarkable LE anterior chamber reaction with dust-like keratic precipitates, cells (4+), flare (2+), and some iris nodules. Fundus assessment was not possible because of dense vitritis. B-scan and high-frequency ultrasound did not reveal any intraocular foreign body. Aqueous tap was performed and the sample was sent for culture and polymerase chain reaction analysis. It was negative for all herpes viruses and for 16S rDNA. Meanwhile, with oral steroids and valacyclovir, the patient showed signs of improvement and LE VA improved to 6/15. Despite the remarkable improvement, it was insufficient as the patient continued to have marked anterior uveitis and vitritis. A white shadow was noted in the peripheral temporal retina of the LE which again could not be assessed properly due to vitreous opacities (). The patient eventually underwent pars plana vitrectomy and laser retinopexy was performed around the white peripheral temporal lesion, which was later believed to be the site of penetrating injury by a thorn on the tree branch from the previous trauma described by the patient. Gram staining of the undiluted vitreous samples showed gram-positive cocci. 16S rDNA was positive for S. epidermidis, and blood agar and chocolate agar cultures confirmed the result with moderate growth.\nThe patient was treated with intravitreal antibiotics (vancomycin 1 mg/0.1 mL and ceftazidime 2.25 mg/0.1 mL) and intravitreal dexamethasone 400 mcg/0.1 mL as well as intravenous (IV) vancomycin (1 g twice/day), and oral prednisone was continued.\nAfter 48 hours of treatment, the patient showed remarkable clinical improvement. LE VA was 6/12. There was no need to administer more intravitreal antibiotics.\nAfter 5 days of IV antibiotic treatment, the patient was discharged on topical and a tapering regimen of oral steroids.\nAfter a follow-up period of 3 months, LE VA was 6/6 with complete resolution of the infectious process (). |
A 62-year-old man, affected dysphagia, was endoscopically diagnosed with lower esophageal cancer confirmed squamous cell carcinoma on biopsy, and was reffered to our hospital (Fig. ). He had no medical history. His lifestyle has included 1500 ml beer consumption per day and 40 cigarettes per day for the past 40 years. Computed tomography (CT) showed thickening of the wall in the lower esophagus as the primary lesion was demonstrated and the tumor formed a mass with the solitary metastatic abdominal lymph node, and invaded pancreas body and gastric body (Fig. ). No other distant metastasis was detected on CT. He was diagnosed with lower esophageal cancer cT4 N1 M0, with pancreatic invasion, cStage IIIC according to 7th edition of the Union for International Cancer Control system []. At first, we considered definitive chemoradiotherapy. However, radiation oncologists evaluated that the tumor was less candidate for chemoradiotherapy because of the risk of gastric mucosal damage. For the purpose of definitive therapy, radical esophagectomy with distal pancreatectomy was planned. As neoadjuvant chemotherapy, CF therapy (cisplatin and 5-fluorouracil therapy; cisplatin was dripped 80 mg/m2 plus 5-fluorouracil was infused 800 mg/m2 on day 1 through 4 continuously) was started according to standard therapy of localized advanced esophageal cancer []. However, after once administration, he could not continue chemotherapy for the exacerbation of dysphagia, and underwent radical surgery. Preoperative evaluation of tumor was similar to initial findings on endoscopy and CT.\nIn findings on laparotomy, abdominal lymph node was infiltrated directly to pancreas body. As the radical surgery, Ivor Lewis esophagectomy with distal pancreatectomy and splenectomy, followed by reconstruction of gastric conduit. Two fields lymphadenectomy was performed according to the treatment strategy of the abdominal esophageal cancer. Reconstruction of gastric conduit was possible although the lymph node was adherent to lesser side of gastric body, which was resected when reconstruction of gastric conduit. In addition, partial resection of lung was performed simultaneously owing to involvement of bilateral pulmonary ligaments to the primary tumor (Fig. ). The operative time was 528 min. The estimated blood loss was 2850 ml, and the patient was transfused 4 units of red blood cell concentrates.\nThe patient had an uneventful postoperative course and was able to take orally. He was discharged 16 days after operation with tube-free. After discharge, he underwent two courses of CF therapy as adjuvant chemotherapy. At present, he is still alive and has no recurrence for 7 years after surgery.\nIn the resected specimen, the primary lesion was observed from lower esophagus to esophagogastric junction, and metastatic lymph node was fixed to stomach and pancreas body (Fig. ). Pathological examination revealed that primary lesion was not infiltrated to lung (Fig. a, b). Massive metastatic lymph node (over 5cm) was observed in the lesser curvature of stomach, and infiltrated to pancreas and gastric wall with extranodal extension (Fig. c, d). The tumor was diagnosed with squamous cell carcinoma, moderately differentiated type. The vascular and lymphatic invasion was confirmed. The surgical margin was negative. The pathological stage was ypT4 N1 (1/61) M0 (metastatic lymph node invasion into pancreas), ypStage IIIC. The histopathological response of chemotherapy was grade 1a, which was equivalent that proliferable cells were 2/3 or more, in 7th edition of the Union for International Cancer Control system []. |
Our patient who is a 68-year-old ethnic Chinese woman had a prior history of intraductal papillary mucinous neoplasm (IPMN) manifesting as an 8 mm cystic side branch communicating with the main duct in the body and 4 mm cystic lesion in the uncinate process of the pancreas. These were detected incidentally on CT urogram when she presented with a complicated urinary tract infection in 2013. She has no other significant medical history of note. The IPMN was documented to be stable based on MRI after 1 year and in subsequent biennial MRI scan of the pancreas from 2013 to 2018.\nShe started experiencing abdominal discomfort in mid-2020. MRI scan done a month after onset of abdominal pain revealed a mass in the head of pancreas measuring 2.5 cm by 2.1 cm encasing both the superior mesenteric artery and vein. It had also compressed on the common bile duct causing biliary drainage obstruction and the tumour had invaded the third part of the duodenum. The radiological features of multiple liver nodules in segments 6, 7 and 8 were consistent with metastatic disease. The diagnosis of adenocarcinoma of the pancreas was confirmed by ultrasound guided biopsy of the segment 6 liver metastasis.\nShe started treatment with chemotherapy combination capecitabine/oxaliplatin (CAPOX) in view of her poor performance status. Her performance status was ECOG 2 at presentation. After two cycles of CAPOX, she deteriorated further with severe loss of appetite, abdominal pain and rapid loss of weight of 10 kg within 1 month. Progressive disease was then documented on CT imaging. The tumour specimen obtained from the biopsy of the liver metastasis was sent for comprehensive tumour genetic profiling. When she was admitted to hospital for intravenous hydration and sodium repletion, her performance status was ECOG 4. Her inability to retain sufficient enteral nutrition as a result of the progressive duodenal obstruction from tumour invasion resulted in grade 3 hyponatraemia (serum sodium=120 mmol/L).\nTumour profiling detected an FGFR2-TACC2 fusion in the tumour specimen and oral erdafitinib 5 mg per day was started on knowledge of the profiling result. The specific FGFR2-TACC2 (NM_000141)-TACC2(NM_006997) fusion was at (F17; T7). We postulated that this fusion may be associated with a clinical response to FGFR inhibitors such as erdafitinib. There was complete resolution of nausea and abdominal pain within 3 days of starting erdafitinib. She was discharged well and walked out of hospital without assistance after 1 week of treatment using erdafitinib. |
We present a 64-year-old Hispanic male with Rai Stage II, IgH mutated, and trisomy 12 positive CLL, diagnosed 13 years prior to admission. He was initially observed for 3 years and then received first-line fludarabine, cyclophosphamide, and rituximab (FCR) for 3 cycles with good response. He remained on observation for 4 additional years and then received an additional 4 cycles of FCR followed by 5 cycles of maintenance rituximab. Five years later, he was found to have 17p deleted recurrent disease and he was started on ibrutinib with good response. He presented to an outside facility with acute onset altered mental status after stopping ibrutinib 2 months earlier due to financial constraints. Cerebrospinal fluid (CSF) analysis was initially concerning for CLL infiltration of the CNS with neoplastic-appearing lymphocytes identified by cytomorphology; however, serological tests for West Nile virus indicated acute infection based on positive IgM and negative IgG; however, PCR could not be performed. The patient received therapy for presumed CNS involvement by CLL with intrathecal methotrexate along with intravenous rituximab and methylprednisolone before being transferred to our institution. On admission, his complete blood count revealed leukocytosis with a normal differential and thrombocytopenia, after reviewing the patients' historical trends, we could discern that the platelet count had been within normal limits in the past and had likely decreased due to the acute illness; additionally, we can appreciate the increasing trend in WBCs until the start of treatment around 10 years prior to this admission (). Peripheral blood smear showed normal platelet morphology and moderate leukocytosis with lymphocytosis with coarse, block-like chromatin pattern concerning for prolymphocytoid transformation which had not been present previously. Bone marrow biopsy revealed hypercellularity with diffuse infiltrate of the medium to large-sized mononuclear cells with irregular nuclear contours and prominent nucleoli similar to that seen in the peripheral blood smear (). Repeat CSF studies at our hospital showed lymphocytes accounting for 49% of total events per flow cytometry, positive for CD19, CD20, CD5, and CD23, with lambda light chain restriction, and negative for CD10 and FMC-7 (). The morphology of lymphocytes in CSF was consistent with neoplasia; however, given the admixture of reactive lymphocytes and the presence of known CNS infection, it was deemed that this most likely represented peripheral blood contamination and reactive infiltration of neoplastic lymphocytes.\nHe was treated supportively during his hospital stay and improved without any further CNS-directed therapy. Ibrutinib was later reinstated after the resolution of thrombocytopenia and BTK mutational testing proved negative. The patient continued to improve during outpatient follow-up, prolymphocitoid morphology disappeared from peripheral smear, and brain MRI showed no evidence of CNS disease. |
A 35-year-old woman presented to the infertility clinic with secondary infertility. At laparoscopy a pelvic tumour was noted in the right uterosacral region abutting the side wall with spread into the pouch of Douglas. This mass was hard, irregular, and white in appearance and biopsy confirmed a carcinoid tumor with a low Ki-67 index (measure of cell division activity) (grade 1) (). She had no symptoms of flushing, diarrhea, or local discomfort. Endocrine screening showed a nonsecretory tumor with normal fasting gut hormones and normal urinary 5H1AA levels. Tumor markers (CEA, CA-125, AFP, and calcitonin) levels were within normal limits. The patient was transferred to a tertiary centre for further investigation and management. A CT scan of her abdomen and pelvis confirmed the right-sided pelvic mass and peritoneal deposits, but the origin of the tumor was difficult to identify, though the appendix was felt to be the presumed origin of disease ().\nDuring this period the patient became pregnant. As imaging at that time indicated radiologically static disease, a neuroendocrine tumour multidisciplinary team decision was made to postpone any surgical intervention until after delivery. The patient was monitored by the endocrinology, surgical, and obstetric teams throughout an uneventful antenatal period. A healthy baby was born by elective Caesarean section, with this delivery method chosen to avoid complications from the tumor.\nInvestigations resumed postpartum for the purpose of staging and determining treatment options. An indium111 octreotide study, three months postpartum, showed increased tracer uptake in the lower pelvis, correlating to the original tumor as well as a focal increase in the region of the terminal ileum suggesting the location of the primary lesion (). Having identified a likely primary site, surgical resection was planned. A repeat CT scan, five months postpartum, was undertaken () which failed to detect the original mass. Following MDT review the decision was for repeat laparoscopy. This demonstrated no macroscopic pelvic or abdominal abnormality. A colonoscopy to the terminal ileum with random biopsies was also normal. Nine years later the patient has remained asymptomatic with no evidence of disease recurrence. Surveillance consisted of two-yearly pelvic MRI imaging. The most recent scan has shown no evidence of abnormality noted in the adnexae bilaterally or around the ileocaecal region suggesting full regression of the previously noted carcinoid tumor (). No chemotherapy, radiotherapy, or alternative medicine (including herbal medicine) was given before, during, or after the pregnancy. |
An 83-year-old man underwent surgical excision of a malignant peripheral nerve sheath tumor (MPNST) at the right knee at another hospital and was then referred to our hospital for further chemotherapy. Three months after the start of chemotherapy, the patient complained of severe pelvic and low back pain. A PET CT was performed which showed multiple bony lesions with a variable (moderate to high) degree of metabolic activity raising suspicion that these were metastases. The lesions, however, had a very unusual appearance and evolution as seen on CT.\nInitially, the lesions presented as small rounded lytic lesions with a well-defined sclerotic margin (Figure ). They exhibited a rapid growth but the initial appearance of central lysis and surrounding sclerotic margin was preserved. Follow up PET CT scan was performed after seven weeks to evaluate the response to chemotherapy. It revealed a second concentric band (halo) around some of the lesions. This band (halo) had a ground-glass appearance and was surrounded by a second sclerotic rim. This rim was thinner and less sharply demarcated than the more central rim (Figure ). The multiple lesions were in different phases of evolution and as such had a different appearance at any given time.\nPresence of multiple lesions, a known primary tumor and moderate to high uptake of FDG tracer on PET scan (Figure ) suggested metastatic disease, despite the unusual appearance of the lesions.\nOther bony metastases were also evident on CT. Some were entirely sclerotic and a few had an ill-defined lytic appearance. Collapse of a vertebral body and fracture of the upper endplate of another vertebra due to metastases were also present.\nBone biopsy of a lesion with the above described doughnut appearance located in the left pubic bone was performed. A biopsy was obtained in the central lytic area and another biopsy in the peripheral halo (Figure ). Histological evaluation of both the samples showed infiltration of bone marrow by malignant spindle-shaped cells arranged neatly in bundles. Histological and immunohistochemical studies were compatible with metastases from a spindle cell tumor (in this case MPNST). |
A 24-year-old female presented with a massive fungating mass over the left upper back and shoulder. The patient gave a history of swelling over the left upper back, which was insidious in onset and gradually progressed over the past 2 years. Incisional biopsy over the most prominent aspect of the swelling was performed elsewhere about 1 year ago, a provisional diagnosis of ES of the left scapula was made and the patient was started on chemotherapy and operated following 3months of chemotherapy. The wound following surgery did not heal and an ulcer developed over the operated site which rapidly increased in size. Neoadjuvant chemotherapy was started and the regimen was changed multiple times over the next 9months as the tumor did not respond and swelling continued to progress in size. In view of the size and nature of the lesion, wound, and associated complications, the patient was advised forequarter amputation of the left upper limb at multiple centers in her country, which she refused and was finally referred to our hospital for further management. On presentation, a huge fungating mass was noticed over the left upper back and shoulder region with active bleeding and foul-smelling discharge from the ulcer (). The patient was febrile and unable to use the left upper limb due to the size of the lesion and excruciating pain. Magnetic resonance imaging of the shoulder and upper back showed a T1 isointense and T2 heterogeneous high-signal intensity lesion which measured 18 cm ×27cm × 26cmin size, with multiple fluid levels, degenerative cystic changes, hemorrhage, areas of necrosis, and infiltration of periscapular musculature (). On computed tomography (CT) scan, tumor was ill defined with heterogeneous attenuation, multiple areas of cystic changes, and gross osteolysis of the scapula. However, the shoulder joint, chest wall, and the brachial plexus were not involved, axillary vascular bundle was displaced anteriorly without signs of infiltration. Whole body positron emission tomography-CT scan showed no signs of pulmonary or distant metastasis. Diagnosis of Ewing’s sarcoma was made by histopathological examination of needle biopsy sample from the swelling, which showed sheets of small, round, and uniform cells with scanty cytoplasm, separated by fibrous strands with few mitosis and minimal stroma (). CD99 was positive on immunohistochemistry. In view of the clinical condition of the patientupfront surgery was performed, following antibiotics to control infection, blood transfusions to correct anemia, and selective arterial embolization of feeding vessels. The patient underwent enbloc excision of the tumor mass bytotal scapulectomy. The ulcer was covered with an adhesive sterile surgical drape; circumferential incision was made around the tumor mass including 3cm of pinchable uninvolved skin. Axillary vascular bundle and the brachial plexus were dissected and isolated from the tumor mass. Arthrotomy of the left shoulder was performed as a layer of the capsule of the joint was also removed. Periscapular musculature was also removed along with the tumor mass. Frozen section from the surrounding soft tissue and chest wall was negative for residual tumor cells. The humeral head was secured to the lateral end of the clavicle (), and the defect over the back following resection was closed by negative pressure dressing and subsequentlycovered2days later with latissimus dorsi free pedicle flap(). The humeral head was plain radiograph of the left shoulder region following resection. Diagnosis of ES was confirmed by histopathological examination of the resected specimen and all the resected surgical margins were free of tumor (). 3 weeks after surgery, concomitant radiotherapy and adjuvant chemotherapy (6 cycles of vincristine, doxorubicin, and cyclophosphamide alternating with if osfamide and etoposide) were started. The patient also underwent autologous stem cell transplantation following adjuvant chemotherapy. There were no neurological or vascular deficits in the operated limb following the procedure and the patient on follow-up had good functional range of motion of the left shoulder and full range of motion of the left elbow, wrist, and fingers. On final follow-up of 3years, the patient showed no signs of local recurrence (LR) or metastasis and had returned to her prior vocational status (). |
A young boy aged 8 years was reported to a private dental practitioner for treatment of multiple carious lower primary teeth. After the clinical examination, extraction of lower teeth was planned. To extract teeth, the inferior alveolar nerve block on left side was achieved using local anesthetic solution containing 1.8 mL of 2% lignocaine hydrochloride with 1:80,000 adrenaline. Immediately after the block, the child started screaming and crying. Thinking it to be a compensatory cry by the child, the practitioner ardently administered the whole solution. The patient became quiet restless and started complaining of severe burning sensation over the left midface region. The solution was cross checked by the practitioner and it was found that by mistake the attendant had loaded 40 percent formalin instead of lignocaine which was kept in a similar vial for a biopsy specimen. Without delay, an Oral and Maxillofacial Surgeon and Pedodontists who are the authors of this paper were consulted for further management.\nAt first local anesthetic solution was administered in the same region to relieve pain and all vitals were recorded. Extra oral swelling was evident on left side of face. Dental procedure was postponed to be done at a later stage. Isotonic saline was injected in the region of nerve block using a large bore needle (18 gauge) and the content was aspirated. The maneuver was repeated for several times to eliminate the formalin from the tissue spaces. Intravenous saline infusion was started along with oral dexamethasone (5 mg) and patient was kept under observation. After 24 h, the size of swelling increased showing features of chemical facial cellulitis. Subsequently the patient developed trismus and peri-orbital edema indicating spread of cellulitis to adjacent spaces ().\nThe following medicines were prescribed: i) amoxicillin 250 mg daily; ii) metronidazole 200 mg three orally times daily for 5 days along with analgesics, vitamin B complex and chlorhexidine mouthwash. The oral dexamethasone was continued twice daily for 5 days with tapering off the dose. After 4 days, when patient was able to open the mouth, after improvement in symptoms, an ulcer was noted at the site of injection (). It was decided to wait and watch the progress of the lesion as the condition was improving. The affected area was regularly irrigated with normal saline and povidone-iodine (1% w/v) solution. Antibiotics were continued for another five days and patient was kept on follow up every alternate day. After 15 days, the extra oral swelling started reducing progressively () and the site of ulcer showed signs of healing. Wound swabs showed negative results for any microbes throughout the course. At the end of four weeks, the patient was completely comfortable with no signs of paresthesia and no functional deficit. Mucosal wound healing was complete without any complication ( and ). |
Patient is a 22-year-old female who was referred for psychiatric consultation by her therapist for the symptoms of low mood, poor motivation, and anhedonia. She reported these symptoms have been present for the last one year but has been progressively getting worse. She started therapy a month ago but missed several appointments. She also had difficulties at work for not being able to complete her assignments. Her initial complaints were that she was not able to understand self, often gets bored when among people, and at the same time fearful of being alone. During assessment, she reported about deep interest in quantum physics as early as seventh grade. She was a honor roll student throughout her academic career and has won prestigious awards for physics at the national level. However, she struggled to maintain friendships and also stated that on many occasions she felt no need to have friends. In high school she was the victim of bullying due to the way she dressed and failed to maintain romantic relationships. She experienced emotional and sexual abuse by her boyfriend at the age of 19. She was uncomfortable with the development of secondary sexual characters during late adolescence and felt difficult to fit in with her peers. She liked reading science books and learning German language as a coping strategy to deal with stressful situations. She also had one previous trial on antidepressant medication (sertraline 50 mg daily) for two months without any benefit. After the consultation, we discussed the possibility of underlying ASD, and she agreed for a CARS2-HF assessment. She scored 30 on CARS2-HF, signifying mild to moderate symptoms of autism spectrum disorder. After the psychoeducation session, she requested more material to read about high-functioning autism. She returned to the clinic after a week and reported feeling relieved and accepted her clinical diagnosis. She agreed to pursue weekly supportive psychotherapy. During her subsequent one-month follow-up visit, the patient reported subjective improvement in the affective symptoms with therapy. |
A 63-year-old female patient was admitted to our hospital with intermittent epigastric abdominal pain for the past three months. Results of the physical examination on admission indicated no icteric sclera. The abdomen was soft with no palpable abdominal mass. The patient had experienced a weight loss of about five kg in the past two months and had no history of hepatitis B or C. She had been exposed to dogs and sheep and denied any history of exposure to infected cases from the epidemic area. After admission, CA19-9 was measured and was 796.20 U/mL. No serological examination for echinococcosis was performed because of the limited conditions of our hospital. Test results from pelvic ultrasound indicated menopausal uterus and uterine fibroids. Test results from gastroscopy showed chronic non atrophic gastritis. Colonoscopy revealed multiple polyps in the large intestine (basically removed); intestinal histopathology (cecum, biopsy) showed severe chronic inflammation of the mucosa and adenomatous hyperplasia of the individual glands. Contrast-enhanced computed tomography of the upper abdomen (Fig. A) revealed a more homogeneous thickening of the gastric wall in the antrum. Round unenhanced low-density foci with a diameter of 4.6 cm was seen in the S7 segment of the liver. Nodular calcifications were also observed. No significant dilatation was noted in the intrahepatic and extrahepatic bile ducts. The size and shape of the gallbladder were normal, the wall was not thick, and no significant abnormal density was observed in the cavity. The pancreas, spleen, and adrenal glands showed no significant abnormalities. Test results from computed tomography indicated liver cyst and intrahepatic calcifications. Contrast-enhanced magnetic resonance imaging of the liver and gallbladder (Fig. B, ) revealed a normal size and shape of the liver and proportion of each lobe, and the intrahepatic and extrahepatic bile ducts and flow vessels ran naturally. A long T1 and long T2 cystic signal with a diameter of about 4.7 cm was observed in the right lobe of the liver, with liquid level, short T1 high signal intensity in the lower layer, high signal intensity on diffusion-weighted imaging sequence, and enhancement of the cyst wall on the enhanced scan. Test results from magnetic resonance imaging showed that the space-occupying lesion of the right lobe of the liver had been considered to be more likely a hepatic hydatid cyst. Preoperatively, three-dimensional reconstruction demonstrated the location of the tumor and its relationship with the surrounding vessels (Fig. A).\nThe patient underwent laparoscopic S7 segmentectomy. Intraoperative findings showed that the tumor was located at the S7 segment of the liver and was about 5 × 4 cm in size and partially protruding from the surface of the liver, with an intact capsule and clear boundary with normal liver tissue. The tumor compressed the right hepatic vein and its tributaries and densely adhered to the right hepatic vein (Fig. B). On postoperative pathology, a mass was observed immediately adjacent to the liver capsule, with a volume of 5 × 5 × 4.5 cm. The section surface showed a brown turbid fluid, a smooth inner wall, and greyish red, greyish yellow, and soft section surfaces of other liver tissues. The pathological section showed a low-grade mucinous cystic neoplasm (volume 5 × 5 × 4.5 cm) in the S7 segment of the liver, with steatosis in the surrounding hepatic tissue area, chronic inflammatory cell infiltration in the portal area, and no tumor cell involvement in the margin of the liver resection.\nImmunohistochemistry demonstrated tumour cells CK7 (+), CK19 (+), and CEA (−); stromal cells ER (+), PR (+), α-inhibin (a small amount +), vimentin (+), desmin (+), and actin (+) (Fig. A–C). This study was approved by the Ethics Committee of Yantai Affiliated Hospital of Binzhou Medical University. |
A 46-year-old male came to our surgical oncology clinic presenting with a chief complaint of a lump in the anterior compartment of the neck, located in the front and slightly to the right, which the patient had noticed since a year ago and has been increasing in size in the last 6 months. The patient denied any complaints of difficulty in swallowing, difficulty in breathing, change in voice, significant weight loss, or any signs of hyperthyroidism. The patient reported to have a history of dyslipidemia, and during inpatient care, we discovered that the patient had hypertension. The patient also reported to have undergone sinus surgery twice in the last 20 years. There was no significant family history.\nPhysical examination on the patient showed a neck mass in the front area, in the midline slightly located to the right, mobile, with soft surface, painless, solid, with a well-defined border, and sized approximately 5 × 5 × 4 cm. During palpation, no lymph node enlargement was noted. Based on the findings above, the patient was given a working diagnosis of suspected benign right nontoxic goiter (struma nodosa nontoxic).\nThyroid gland ultrasonography was performed. The result showed that there was no abnormality in the thyroid gland; however, a cystic lesion in the anterior midline area was found, sized approximately 3.4 × 3.5 × 4.5 cm with debris sediment located inside the lesion which raised suspicions towards the thyroglossal duct cyst. To obtain a more accurate picture, a CT scan with contrast for the neck area was performed, which then showed a complex cystic mass in the front neck area located in the right parasagittal area, sized approximately 3.9 × 3.8 × 5.5 cm, attached to the right infrahyoid muscle, appearing to bulge into the larynx, with differential diagnosis of the thyroglossal duct cyst or epidermoid cyst (). The thyroid gland appeared to be within normal limits. The scan also discovered multiple lymph node enlargement in the submental area and along the right jugular chain that measured largest in size 22 mm in the right area and 17 mm in the left. The thyroid function test was within normal limits.\nThe patient was then diagnosed with a thyroglossal duct cyst and planned to undergo Sistrunk procedure. The surgery went well, and the patient was discharged the day after. The excised specimen was then sent to pathology for histopathology examination. Macroscopic appearance of the specimen showed a cystic lesion that measured 4 × 3 × 3 cm with part of the specimen resembling white cauliflower and appeared to be fragile (). Microscopic examination showed that the cystic lesion consisted of fibrous tissue lined by a single layer of epithelial cells that was erosive in most area. This area was the remnant of the thyroglossal duct cyst. Other areas showed that there were tumors in papillary structures with a fibrovascular stalk and some in a follicular pattern, lined by cells that were stratified, and showing ground glass nuclei, nuclear grooves, and eosinophilic cytoplasm (). The histopathology conclusion was a thyroglossal duct cyst with a classic and follicular variant of papillary thyroid carcinoma.\nOn the 6-month follow-up after surgery, the patient reported no clinical complaints, and there were no locoregional complication nor clinically palpable lymph node enlargement; however, there was a lymph node enhancement discovered by cervical contrast CT scan, and the patient was planned for a lymph node biopsy; if the biopsy result showed to be malignant, selective neck dissection and total thyroidectomy would be the treatment of choice. |
The patient was a 57-year-old male, with ESRD secondary to diabetes and hypertension, on hemodialysis for 20 months. He was evaluated to undergo living unrelated donor kidney transplant. The donor was a 54-year-old female with unremarkable past medical history. She was medically and surgically cleared after full assessment. Preoperative computed tomography (CT) angiography for the donor revealed a small complex cyst (Bosniak IIF), a short right renal vein (2 cm), and a right renal artery with an early bifurcation of an upper pole artery. Surgical approach deemed best by the living donor selection committee was to remove the right kidney with the complex cyst via laparoscopic approach.\nThe risks of surgery and potential complications were explained to both donor and recipient. Written informed consent was obtained prior to surgery from both patients.\nA standard hand-assisted laparoscopic living donor nephrectomy (LLDN) was performed to retrieve the right kidney and the right ovarian vein for reconstruction. The length of the donor renal vein was reduced by 10-15 mm due to the standard use of vascular stapler device to control the renal vein stump.\nStatus after procurement upon visualization in the back table is that the length of the donor renal vein was 3 mm. In order to elongate the vein, the donor ovarian vein was dissected and used for reconstruction. It was opened longitudinally, folded over, and anastomosed to the donor renal vein using end-to-end anastomosis with an 8-0 Prolene ().\nThe donor kidney had a renal artery with early bifurcation of an upper pole artery. After mobilization of the inferior vena cava as much as possible, the endovascular stapler was placed after the bifurcation from the renal artery and the length of the vessel was reduced by 10-15 mm. At the back table the donor kidney presented two individual arteries: the main renal artery and the short upper pole arterial branch. Consequently, the recipient IEA was used as an extension graft to perform an end-to-side anastomosis between the upper pole arterial branch and the main renal artery using 8-0 Prolene ().\nThe complex cyst was dissected all the way down into the calyces and substantial margin of healthy parenchyma was removed. Distal margin was sent to pathology to rule out malignancy. The calyces and the renal parenchyma were oversewed with 5-0 PDS and the edges of the defect with U-stitch of 4-0 PDS using pledget ().\nThe extended renal vein and the main renal artery were anastomosed end-to-side to the external iliac vein (EIV) and artery (EIA) respectively, using 6-0 Prolene ().\nWe performed an extravesical ureteroneocystostomy and a Jackson Pratt drain was placed. The warm ischemia time was 33 minutes. Postoperative course was uncomplicated and the patient has maintained adequate renal function with stable serum creatinine (1.32 mg/dl) at 5-month follow-up. The final pathology report and the intraoperative frozen section of the margin were negative for malignancy. |
A 37-year-old, gravid 2, para 2, woman with no significant past history was referred from a gynecologic clinic because of bilateral vulvar swelling. The vulvar swelling had developed for several years but she never visited hospital for evaluation because she felt that she gained body fat in the groin. She recognized palpable and painless mass on both vulvae 3 weeks before visiting gynecologic clinic in March 2010. Physical examination revealed a whole vulvar swelling and about 3-cm-size non-tender masses on bilateral labium majus without a protruding lump. Other external genitalia showed no significant abnormality. No signs or symptoms suggesting inflammation was found. Hematological and biochemical values were within normal range. Pelvic magnetic resonance imaging with contrast revealed fatty masses suggesting lipoma () and positron emission tomography/computed tomography scan of whole body showed no distant metastasis. We performed surgical resection of bilateral vulvar masses and the masses () were about 15 and 20 cm in maximum length respectively. It was not easy to clearly demarcate the tumor and fat tissue around. Both inguinal lymph nodes were not enlarged. Histopathologic exam confirmed the diagnosis of well-differentiated liposarcoma which showed adipocytes of various sizes and lipoblasts ().\nAdjuvant radiotherapy was given because we were not sure the tumor was completely resected. After postoperative recovery, the patient was referred to the radiotherapy department in May 2010. The patient was prescribed a dose of 60 Gy to be delivered in 30 fractions of 2 Gy to the vulvae and pelvis, using 6 MV linear accelerator (linac). Treatment was daily, except for the weekend. At 40 Gy the patient presented with erythema arising within a radiation field of perineum and reported persistent anal pain, so radiotherapy was discontinued. The lesion and symptom resolved after a week, not requiring any intervention. The treatment was restarted with same dose and completed without additional side effects. Neither recurrence nor metastasis has occurred up to 44 months after initial surgical treatment and subsequent radiotherapy. |
A 65 year old male with an extensive operative history for Crohn’s disease, including 4 laparotomies with small bowel resections to ameliorate small bowel obstructions presented to us. His first surgery was performed in England in 1991, and the next 3 were performed in Peru, one in 1998 and two in 2007. Over the past few years he has been suffering from nutritional deficiencies and osteopenia associated with his resultant short bowel syndrome. He has had, in addition, intermittent drainage from his previous midline incision. The drainage was non-bilious, serosanguenous fluid that would egress approximately every 2–3 days. Several sutures were removed through the tract in the past but the wound still failed to heal. The drainage volume and quality did not change significantly over the past year. Although he did suffer from short bowel syndrome, he has had no change in his gastrointestinal symptomatology or body weight over the past year. On physical examination, his vital signs were within normal limits. His chest and abdominal examinations were unremarkable, except for an infra-umbilical surgical scar with, what appeared to be, a chronic sinus draining a minimal amount of serous fluid. A number of subcutaneous sutures were palpable, adjacent to the orifice of the sinus but no erythema or purulence was observed. A CT fistulogram done in 2008, 1 year after his most recent bowel resection, revealed a possible enterocutaneous fistula (Figure ).\nGiven his history of inflammatory bowel disease, multiple prior surgical procedures and physical findings, the working diagnosis was a chronically draining abdominal wall sinus tract most likely secondary to a previously infected suture. The possibility of an enterocutaneous fistula was also entertained; however, his clinical picture, with minimal and intermittent drainage of non-succus fluid made the latter possibility less likely. The fistula noted on the CT fistulogram 5 years ago was believed to have closed given his presentation.\nAfter explaining the risks, benefits and possible complications of surgical intervention, our patient elected to have a wound exploration with possible laparotomy under general anesthesia for the purpose resecting this sinus tract.\nAn elliptical incision was made around the suspected sinus tract and followed down to the fascia. A hemostat was placed in the lumen of the sinus tract which traveled below the fascia, necessitating entry into the abdomen. Sharp dissection along the previous midline incision revealed moderate to severely thickened abdominal wall adhesions, most likely a result of prior obliterative peritonitis. Extensive lysis of adhesions and mobilization of the small bowel took approximately 10 hours. Once the extensive adhesions were lysed and the bowel mobilized, the tract was traced to a 15 cm blind ended portion of small bowel, closed on one end with the fistulous tract emanating from the other. After running the bowel from the Ligament of Trietz to the peritoneal reflection multiple times revealing the rest of the bowel to be fully continuous, it was clear that the suspected sinus tract, was in fact, a fistula tract connected to a blind segment of small bowel which had its own mesenteric blood supply. A second surgeon was called into the OR to confirm the continuity of bowel and the intraoperative findings. Only after multiple confirmations of bowel continuity, was the tract and blind segment resected.\nPostoperatively, our patient regained bowel function and promptly improved in overall health. At follow-up his wound was healing well without complications with resolution of his chronic draining sinus. |
A 25-year-old male, who sustained accidental industrial exposure to Iridium192 while repairing a gamma camera at his work place in Bahrain in March 2000, presented with a non-healing painful ulcer of size 5×5 cm over the right groin to our centre in October 2000 []. Further enquiry revealed that, immediately after exposure he developed recurrent attacks of hemetemesis requiring supportive treatment in a local hospital. Three weeks after the exposure he developed burns at the contact areas, which included the left hand, left side of the chest, abdomen and right inguinal region. All except the inguinal burn healed with unstable scars. At the fourth week he developed severe bone marrow depression with a platelet count of 61 × 109/liters and a WBC count of 2.5 × 109/liters, which also recovered with supportive treatment at the local hospital.\nThe patient was admitted at the pain and palliative care ward and started on morphine and adjuvants, which included NSAIDs, steroids, antibiotics, and antidepressants after routine investigations were found to be normal. Due to inadequacy of pain relief with increasing doses of oral and intravenous morphine an epidural catheter was inserted through the second lumbar intervertebral space to deliver morphine buprinorphine and bupivacaine for round the clock analgesia. Once adequate analgesia was attained, the ulcer I the groin was excised with a margin of 0.5 cm all around, except at the depth where the femoral neurovascular bundle was situated, and covered by the adjoining groin flap under general anesthesia. An ipsilateral Tensor Fascia Latae (TFL) myocutaneous flap was used for cover of the groin flap donor site and the post operative period was uneventful []. The epidural catheter was removed on the fourth day post-operatively, and he was discharged on the 14th day with NSAIDs for pain management []. However he was warned of further scar breakdown and hence advised regular follow up.\nOn 4th April, 2001 he presented with another painful ulcer, 4 × 4 cm with surrounding unstable scar over the left side of the anterior abdominal wall. The groin flap had settled very well. The abdominal scar and ulcer were again excised with 0.5 cm margins up to the underlying muscle. An ipsilateral Latissimus Dorsi (LD) myocutaneous flap supplemented by a random transposition flap of size 6 × 4 cm was used to reconstruct the defect created by the excision. The LD donor site was closed primarily. Pain was managed by a protocol similar to that followed earlier, and he was discharged after an uneventful postoperative period with advice on further follow-up every two months.\nOn 17th October 2001 he again presented with a 3 × 3 cm ulcer with surrounding scar over the ulnar aspect of the left wrist. This time it was decided to prophylactically excise the unstable scar on the chest in addition to the ulcer on the wrist. The ulnar nerve was intact. Both defects were reconstructed with local transposition flaps. A fasciocutaneous transposition flap based on radial forearm perforators was used to close the wrist defect. The post-operative period was uneventful. There were no further episodes of scar breakdown and the patient returned to his job in Bahrain. On 2nd March 2002, he presented with an ulcer on the volar aspect of the thumb tip for which a reconstruction with a Littler’s flap was advised. However, he did not report for surgery and on July 2002, presented with a fresh ulcer on the lateral aspect of the right heel. The thumb tip ulcer had almost healed with a healthy scar. After three months of pain and conservative management, the leg defect also started showing signs of healing. He is now on further follow up []. |
A 28-year-old male patient who lost his right ear in an electric burn reported to the Department of Prosthodontics, Saveetha Dental College and Hospital, for further treatment. Examination and history revealed that the patient had suffered from electric burns with scar formation, but some rudimentary ear was present in the tragus region of missing right ear (). Hearing capability was not compromised on both sides. A prosthetic reconstruction was decided to fulfil the patient's desire of cosmetic correction without surgery.\nExamination of the defect showed healthy tissue with scar formation and rudimentary tissue tag at the height of the tragus, and the contralateral ear was normal. Before the impression making, three horizontal markings are made on the normal ear at the junction of helix with the side of the head as the first marking, the second at the middle of the tragus, and the last one at the junction of the ear lobe with the side of the head. Similar markings were made on the defective side, so that these markings were accurately transferred to the working cast. The borders were confined and closed by hair and smeared with vaseline. While making an impression, the external auditory canal was blocked with a cotton plug and the impression was made by irreversible hydrocolloid and with the gauge in between, and backing is done by quick-setting plaster to provide support for the impression (). The impression of the donor ear was made with light body additional silicone, and the impression was poured with the dental stone to get the master cast of the defect site and the cast of the normal ear ().\nThe prosthesis should have a lifelike appearance, so sculpting was done with utmost care to complement the aesthetics. The prosthesis can be sculpted from the beginning or the donor technique may be used. The donor ear can be selected from a sibling or from any person whose ear matches with the patient's ear. For this patient, the donor technique was used and the impression was made and filled with modelling wax and later retrieved from the impression ().\nAfter the wax pattern try-in was done, it was oriented on the master cast using the markings placed on the defect site and necessary modification was done to match the contralateral ear. The wax pattern was then sealed in position on the master cast and leading edges were thinned as much as possible so as to allow the silicone edges to feather into the natural skin and when used in conjunction with adhesive they disappear. A three piece mould was fabricated for easy placement of silicone in the mould. To get the three piece mould, top portion of the dental flask was used as the base, the master cast along with the wax ear pattern was placed on this top portion of the flask, and dental plaster was poured to flush with the surface of the cast leaving no undercuts (). Once set, two grooves were created on the plaster at the back portion of the ear to reorient the piece of mould. Separating media was applied as a separating agent and the dental stone was mixed and filled on the back side of the ear wax pattern to flush just underneath the superior margin of the helix extending till the base of the helix and junction of the lobe with the side of the head without leaving any undercut. Once the second pour was set, similar grooving procedure was carried out and the third pour was done using plaster, and the lid was placed and clamped and allowed to set.\nDewaxing was done in the usual manner. After keeping the flask in hot water for fifteen minutes, the flask was opened carefully and all three pieces of mould were thoroughly cleaned with hot water to remove traces of petroleum jelly and wax. Cold mould seal was diluted with water in 1 : 1 ratio and applied. The moulds were allowed to dry completely as traces of vaseline, wax, or water will interfere with setting of silicone and the prosthesis will have the tacky surface that will invite catching of dust at a later date. The three piece mould was now ready for silicone packing. Medical-grade factor-II room temperature vulcanising (RTV) silicone was used and was mixed as per the manufacturer's instruction. Thixo was added to prevent porosity in the prosthesis. To fabricate a lifelike silicone prosthesis, the patient must always be present for the colour match. It requires great care and patience from the doctor along with an understanding of colour matching for a successful result. As the silicone is translucent, desired skin colour can be obtained using primary colours in proper proportions. Using red, yellow, and blue primary colours of intrinsic colouring system, the first base shade was prepared, and then as per the requirement, additional shades were prepared. Care was taken while adding colour, as colour loading will lead to opacity and lifeless appearance (). To match with the patient's normal ear, the dark and light shade silicone was poured in the mould. To create the characteristics, red flocks were placed on the surface layer and the mould was packed and allowed for bench curing for 24 hours. Cured prosthesis was retrieved from the mould, cleaned thoroughly with soap, and the excess silicone flesh was trimmed from the margins (). The prosthesis was tried on the patient and margins were trimmed as per requirement, and then the prosthesis was attached to the spectacle frame and placed in position ().\nAn instruction leaflet with “Do's and Dont's” explained in it was given to the patient with an instruction that the prosthetic ear should be replaced every few years as the old one wears off. The skin surface should be maintained clean. |
Here, we present a case of bilateral congenital IEM. A four-year-old female patient who was diagnosed with congenital profound SNHL at the age of 18 months presented to our otology clinic for evaluation and advice. No medical problems in the mother were recorded during pregnancy. The child was delivered at full term via a caesarian section. The mother and baby were not admitted to the intensive care unit. The normal pediatric examination was performed, and the child was sent home three days after delivery.\nHearing screening test was not performed after delivery. Hearing loss was observed by the parents at six months of age. Apart from the hearing loss, the child had normal growth and development with a normal facial appearance. Microscopic examination revealed normal bilateral tympanic membrane.\nThe otoacoustic emissions test was performed at 12 months of age, and no emissions were detected. The auditory brainstem response (ABR) was assessed at the age of 12 months, and no reproducible waves at 90 dB presentation level were observed. Hearing aids for both ears were used for three months with no benefit. Furthermore, magnetic resonance imaging (MRI) of the temporal bone revealed a bilateral CA with a dilated vestibule and a malformed semicircular canal located posteroinferiorly to the IAC, both of which contain two nerves that may represent both the facial and cochleovestibular nerves with larger sizes in the left ear (Figures - show the axial and the parasagittal view of both sides, respectively). According to the IAC grading system, grade II represents the presence of nerves in the parasagittal plane on both sides [].\nTreatment plan\nBased on the clinical history and audiological and 3D reconstruction of the radiological findings (Figure ), the CI committee recommended unilateral CI for the left ear due to bigger cystic cavity and thicker eighth cranial nerve before considering an auditory brain stem implantation. After the parents provided signed informed consent, the child received a CI at the age of 2.7 years. Form19 electrodes from MED-EL (Innsbruck, Austria) were used for the CI procedure. A single-slit trans-mastoid labyrinthectomy (TML) approach was used with a smooth insertion of the electrode. No oozing or gushers were observed during the TML approach. Figure shows the approach, insertion technique, and closure of the labyrinthectomy opening.\nThe intraoperative objective measurements showed an electrically evoked compound action potential (ECAP) in two of the 12 electrodes (3 and 7) with normal impedance values for all electrodes. Intraoperative mastoid radiation (X-ray) showed complete insertion of the electrode with an electrode located in the lateral wall and no penetration inside the IAC. Normal wound healing with no adverse events occurred after the CI surgery.\nAudiological and speech assessment outcomes\nThe activation of the audio processor was performed three days after the CI surgery. Early activation of CI audioprocessor showed to be safe approach and more economic compared to classical activation that performed one month after the surgery []. Impedance field telemetry (IFT) was normal, and the ECAP was present only at electrodes 3 and 7. Four progressive maps were obtained. The child started to hear loud noise one month after the activation of the device. Based on the data logged, the average number of hours of wearing the audio processor was 12 h per day. Six months after the surgery, the pure tone average of the child was 32.5 dB, the speech reception threshold (SRT) was 20 dB, and the child tried to initiate some words, but the ECAPS was present in 7 of 12 electrodes (Figures , ).\nEight months after the CI surgery, the child produced 16 words, and the language age judge was 20 months. Sixteen months after the CI surgery, the child could produce 200 words and say a sentence with two words. LittlEars Questionnaire was found to be valid and reliable screening tools for auditory skills in infants and toddlers who received cochlear implants []. The responses of the parents to the LittlEars Questionnaire, when the child was 4 years old, showed a score of 32, indicating normal hearing for the corresponding age. |
The patient is a 45-year old caucasian female without serious previous disease or family history of cancer. In October 2004, she had noted a few days of hypesthesia in her left leg, followed by slight hemiparesis and a seizure resulting in hospitalisation. A magnetic resonance imaging (MRI) scan of the brain revealed a tumor in the right parietal lobe, presumably representing a glioma (Figure ). In November 2004, a partial resection (because of the proximity to the motor cortex) was performed. The symptoms improved completely and the patient continued on anticonvulsant therapy. Histology demonstrated a metastatic tumor with pigmented cells and positive immunohistochemistry for S100, HMB45 and Vimentin.\nStaging including examinations of the eyes, head and neck mucosa and total skin, gynecological evaluation, bone scintigraphy and computed tomography (CT) scans showed an enlarged left adrenal gland as the only pathological finding (Figure ). All routine blood tests and hormonal levels were within normal limits. The adrenal mass was removed completely by laparoscopic surgery and histology corresponded to that of the brain metastasis. Treatment proceeded with whole-brain radiotherapy (WBRT), 10 fractions of 3 Gy. In February 2005, the patient noted headaches and a decreasing general condition. A MRI scan disclosed two new brain metastases in the left parietal and temporal lobe, respectively (Figure ). While the parietal tumor could be resected completely, the temporal lesion was treated with gamma-knife radiosurgery (RS). The peripheral minimum dose was 15 Gy. In March 2005, the patient developed abdominal symptoms and a CT scan showed a right abdominal mass presumably representing inflammation in and around the vermiform appendix and ovary (Figure ). Surgery including ovarectomy and appendectomy was performed and the histology demonstrated again the same type of melanoma with all 3 positive markers as mentioned. The tumor was limited to the vermiform appendix without spread to peritoneum or lymph nodes and was judged to be removed completely.\nAfter a symptom-free interval, routine MR evaluation in November 2005 disclosed tumor progression of the temporal lesion and a second gamma-knife procedure was performed. Since then, the patient returned to repeated follow-up examinations including MR and CT scans. The last one was performed in July 2007. As shown in Figure , there is a stable residual abnormality with contrast-enhancement in the temporal lobe after repeated gamma-knife treatments, possibly representing treatment effects rather than active tumor. No other potential signs of disease were detectable. The patient has a Karnofsky performance status (KPS) of 80% resulting from slight concentration and endurance problems. She has never received systemic treatment during the whole course of disease, although she initially was interested in participation in a clinical trial. However, all trials that were open in Norway at that time did explicitly exclude patients with brain metastases. |
A five-year-old boy presented with rapid-onset proptosis and inferior displacement of the left globe for two weeks with associated with pain and discharge. There is history of the same condition in contralateral eyes that spontaneously heal without any treatment. It was previously treated with intravenous antibiotic for three days without any improvement. There was no history of trauma, upper respiratory tract infection, sinusitis or immunosuppression. The vital signs were within normal limit. The left eye showed proptosis, periorbital edema, chemosis in all quadrants of the conjunctiva and severe exposure keratopathy (). The swelling was tender and soft in consistency. Proptosis and displacement of the left globe to inferior were present. There was limitation of all movement of the globe. Examination of right eye was within normal limit. Based on history and clinical examination, clinical diagnosis of rhabdomyosarcoma was made with orbital cellulitis as differential diagnosis.\nA multislice computed tomography (MSCT) scan showed lesions involving the lateral orbit () and the retro bulbar space (). Complete blood count showed leukocytosis with signs of infection. Systemic screening results for metastasis were negative. Consultation with ear, nose and throat (ENT) specialist confirm no clinical and radiological signs of sinusitis. A dental consultation also showed no odontogenic infection. Community acquired pneumonia (CAP) was confirmed by a pediatrician from clinical and thorax radiologic findings. A biopsy was not performed due to suboptimal condition for anesthetic procedure.\nOn three days' administration of broad-spectrum cephalosporin antibiotic 750 mg twice a day, there is slight improvement of the patient's condition (). Patient shows resolution of clinical presentation after an additional intravenous administration of anti-inflammatory dexamethasone 5 mg twice a day for three days (). The patient made a good response after additional intravenous administration of broad-spectrum aminoglycoside antibiotic 40 mg twice a day for one week (). Simultaneously with intravenous therapy, an alpha receptor antagonist sympathomimetic and antibiotic were given topically to prevent further damage to the anterior segment of eyeball. After three weeks' therapy, the globe recovered, proptosis and displacement disappeared and ocular motility became normal. The visual prognosis was poor but with topical treatment, the ocular surface began to show better results. The patient’s legal guardian signed the written informed consent for publication of the case details and accompanying images. Institutional approval was not necessary. |
A 71-year-old male patient visited Yeungnam University Hospital with abnormal chest computed tomography (CT) findings on a routine medical examination. The patient was taking medications for hypertension, diabetes mellitus, and hypothyroidism and had a history of traumatic abdominal aortic aneurysm surgery 40 years previously. Chest CT revealed a 3.5-cm oval mass in the posterior mediastinum at the level of the eighth to ninth thoracic vertebrae in addition to multiple pulmonary nodules (). Magnetic resonance imaging (MRI) of the abdomen showed multiple poorly enhanced masses in the liver. The liver masses were smaller than 2 cm and emitted a low T1 signal and a high T2 signal (). We considered the possibility that the lung and liver lesions were metastatic, but blood tumor marker tests were normal. Percutaneous needle biopsy (PCNB) of the pulmonary lesion failed to yield a meaningful sample. With the possibility of neurogenic tumor or pulmonary and hepatic metastases in mind, we decided to perform surgery. We planned to resect the mediastinal tumor and biopsy the pulmonary lesion for diagnosis. We decided to determine the treatment plan for the hepatic lesions based on the surgical pathology results. We performed surgery with the patient in the left lateral decubitus position via video-assisted thoracoscopic surgery. We made 3 ports (5 mm, 10 mm, and 12 mm) using a 10-mm telescope. We identified the mediastinal tumor as observed on chest CT. The mediastinal tumor was located in the posterior mediastinum, close to the sympathetic chain and supplied by feeding vessels from the intercostal vessel. The tumor was well-demarcated without invasion into adjacent structures. After ligation and division of the feeding vessel with a hemoclip, the tumor was removed without difficulty. A pulmonary examination revealed multiple well-circumscribed nodules of different sizes, diffusely distributed on the lung surface. We performed wedge resection to obtain tissue for diagnosis. After insertion of a 28F chest tube, the operation was terminated. On gross examination, the mediastinal tumor was a 3.5-cm oval nodular mass surrounded by hardened tissue. There were 5 lung nodules in the resected lung tissue, and the cut surface showed a honeycomb-like appearance. A microscopic pathological examination revealed at both sites dilated blood vessels that varied in size and were filled with varying amounts of blood components (). A histopathological examination of the specimens, lung nodules, and mediastinal tumor revealed cavernous hemangioma. Based on the liver MRI and histopathology of the mediastinum and lung, we decided that the hepatic nodules were also likely cavernous hemangiomas and decided to investigate further. The patient’s postoperative course was uneventful. The chest tube was removed on postoperative day 3, and the patient was discharged on postoperative day 4. One year after surgery, chest CT and abdominal MRI revealed no changes in the pulmonary and hepatic lesions (). The patient is doing well without any specific clinical symptoms. The patient provided written informed consent for publication of his clinical details and images. |
A 19-year-old male patient, an electrician by occupation, presented to us with complaints of pain in the left eye associated with a mildly diminished vision since the past 2 days following ocular injury with a ball. He also gave a history of grossly diminished vision in the right eye since the past 2 months following an electric shock; the point of contact was the right thumb. The approximate voltage of the current was 440 volts. There was an associated history of fall following the electric shock which was from a height of about 10 feet and was not associated with any bony injury. His general physical evaluation revealed presence of a healed burn on the dorsum of the right thumb extending to the lateral aspect of the index finger [].\nOcular examination revealed a visual acuity of 20/400 (OD) and 20/30 (OS). On anterior segment examination of the left eye, a note was made of 1+ cells and flare in the anterior chamber with 0.8 mm of hyphema. A relative afferent pupillary defect was noted in the right eye. The remainder of the anterior segment was normal in both eyes with a clear lens.\nFundus examination of the right eye [] showed presence of a clear media with multiple cotton-wool spots in the nasal half of the macular region. There were multiple areas of retinal whitening with associated areas of perivascular clearing noted in the nasal macula depicting the classical Purtscher flecken. A note was also made of the solitary flame-shaped hemorrhage and a sclerosed vessel in the area of the papillomacular bundle. The peripheral fundus was within normal limits. The fundus evaluation of the left eye was normal.\nAn optical coherence tomography (OCT) evaluation of the right eye showed disorganization of the inner retinal layers nasal to the fovea with atrophy of the ganglion cell layer. The outer retina also shows a discontinuity in the ellipsoid zone in the same area []. OCT-angiography (OCT-A) analysis (6 × 6 mm) showed presence of multiple areas of capillary drop-out in the nasal macula which were more pronounced in the deep capillary plexus as compared to the superficial capillary plexus []. To confirm the findings of the OCT-A, a fluorescein angiogram (FFA) was carried out, which revealed an area of capillary nonperfusion supero-nasal to the fovea. A note was also made of sluggish perfusion in the area of the papillomacular bundle with perfusion being complete at around 65 s following dye injection []. OCT evaluation and FFA of the left eye was essentially normal.\nA diagnosis electric shock induced Purtscher-like retinopathy in the right eye with a contusion injury in the left eye was reached. The patient was started on topical steroids and mydriatics in the left eye, while he was explained about the poor visual prognosis in the right eye.\nAt 2 months of follow-up, the visual acuity in the right eye was stable at 20/400, while it improved to 20/20 in the left eye. The iritis in the left eye had resolved while fundus examination of the right eye revealed diminished number of cotton-wool spots with a temporal pallor of the optic nerve head. |
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