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A 57-year-old diabetic male presented with a non-healing wound over the left foot since 4 years. Four years back he had hot water spillage on his left great toe which got secondarily infected and resulted in gangrene. He underwent amputation of great toe (up to metatarsal) for gangrene which left an ulcer over the amputated site. Three months following the amputation, split thickness graft was done for the ulcer. But there was graft failure resulting in a non-healing ulcer. A second split thickness graft was done one year later in January 2012, but the ulcer did not heal []. Since then the chronic non-healing ulcer was managed by debridement and regular dressing without much improvement. On examination, there was a solitary non-tender ulcer over the medial aspect of left foot measuring 5 × 4 × 0.4 cm (width × length × depth) with a well-defined margin covered by granulation tissue and slough surrounded by macerated skin and calluses. Area and volume of the ulcer was 15 cm2 and 6.3 cm3. Wound area was calculated using the formula for an ellipse: Length × width × 0.7854 (an ellipse is closer to a wound shape than a square or rectangle that would be described by simple length × width). The use of an ellipse for calculating wound measurement has been used in randomised controlled trials in wound healing literature.[] The ulcer was foul smelling. His blood sugar levels were under control. His routine investigations were within normal limits. His baseline platelet count was 2.19 lakhs/cumm.\n20 ml of venous blood was taken and anticoagulated by acid citrate dextrose and PRP was prepared by double centrifugation method. The first spin used was hard spin (5000 rpm for 15 minutes) which separates into three layers: Plasma, buffy coat and red blood cells. The plasma and buffy coat was aspirated into a sterile test tube without an anticoagulant and subjected to a second spin (2000 rpm for 5 minutes). The second spin (soft spin) allows the precipitation of the platelets to 0.8 ml to 1.5 ml to fall onto the bottom. 1 ml of PRP was aspirated and activated with 10% calcium chloride (0.3 ml for 1 ml of PRP) and injected to the ulcer and the edge of the ulcer and covered by paraffin gauze and sterile gauze.[] The dressing was covered by Dynoplast® []. The dressing was opened on the third day and PRP was repeated once weekly. After 1 week, there was reduction in area and the volume of the ulcer to 12.5 cm2 and 3.75 cm3. After six sittings of PRP, the ulcer healed completely in 7 weeks [].\nThe patient was advised for bed rest for 2 weeks. He was referred to artificial limb centre for proper foot wear (forefoot support with microcellular rubber) to prevent further complications []. |
A 55-year-old female with a history of recurrent nephrolithiasis was admitted with a 2-month history of progressive fatigue, weight loss, dysuria, and fevers. She had no prior history of urological instrumentation. On examination, she was found to have mild bilateral lower quadrant abdominal tenderness and pronounced costovertebral angle tenderness on the right side. Initial laboratory evaluation revealed pyuria, but no hematuria or leukocytosis. A contrast-enhanced CT of her abdomen and pelvis revealed a soft tissue density in the pelvis and perirenal space along with multiple intrarenal hypodense collections consistent with xanthogranulomatous pyelonephritis. Two small, nonobstructing calculi were identified in the collecting system and the right calyx with associated inflammatory changes. Additionally, a tract extending from the upper pole of the kidney to the second portion of the duodenum was visualized ().\nDuring subsequent percutaneous nephrostomy placement, contrast was visualized extending from the renal calyces into the patient's duodenum and stomach (). Contrast was again visualized flowing to the small bowel and stomach during ureteral stent placement. A small bowel follow-through study confirmed the presence of a fistula extending from the second portion of the duodenum to the superior pole of the right kidney (). Upper endoscopy was performed to rule out a malignant etiology, once again confirming the presence of a renoduodenal fistula. Biopsy of an area of nodularity near the fistula in at the D1/D2 duodenal junction revealed inflammatory changes and no evidence of malignancy.\nAfter ureteral stent placement and eight days of treatment with IV ceftriaxone, the patient's fatigue, dysuria, and costovertebral angle tenderness resolved. A diagnosis of XGP associated with spontaneous renoduodenal fistula and was made, and the patient was discharged in stable condition on oral antibiotics. After discharge, an outpatient open nephrectomy with take-down of the renoduodenal fistula was performed. Gross findings and surgical pathology revealed acute on chronic pyelonephritis with multinucleated giant cells, consistent with XGP. |
A 63-year-old Japanese woman was referred to our department because of an abnormal shadow at the left side of her chest wall on computed tomography. She had undergone total hysterectomy and radiotherapy for cervical carcinoma 4 years prior. One year after the first surgery, three metastatic lung nodules appeared at the upper lobe of her right lung, the lower lobe of her right lung, and the lower lobe of her left lung. Wedge resection for upper and lower lobe of her right lung was initially performed via three-port thoracoscopic surgery. Then, wedge resection for the lower lobe of her left lung was performed via eighth intercostal single incisional thoracoscopic surgery. After the surgery, an intrathoracic chest wall mass developed which increased in size gradually. Her gynecologist introduced her to our department for surgical resection of the mass. Her family, including her parents and two sisters, had been healthy and had no inheritable diseases. She had no symptom, drug history, tobacco smoking history, or psychosocial history, and she was a social drinker. She had not received any medications since the mass developed and until admission to our hospital. She had undergone an operation three times as mentioned above and had been a carrier of type B hepatitis.\nAfter her admission to our department, her general condition was good, and there were three operative scars at both sides of her chest and lower abdomen. Her chest sounds were clear and there was no neurological abnormality. She was 151.1 centimeters tall and weighed 49.8 kilograms. Her heart rate was 77/minute, blood pressure was 135/87 mmHg, and body temperature was 36.1 °C. The laboratory findings were white blood cells 5.25 × 103/μL, hemoglobin 12.7 g/dL, and platelets 156 × 103/μL. A liver function test revealed: albumin 4.6 g/dL, aspartate aminotransferase 15 U/L, alanine aminotransferase 13 U/L, and total bilirubin 0.3 mg/dL. A renal function test revealed blood urea nitrogen 13.6 mg/dL and creatinine 0.79 mg/dL. An electrolyte test revealed sodium 143 mEq/L, potassium 3.8 mEq/L, and chlorine 106 mEq/L. A tumor marker test revealed carcinoembryonic antigen 3.4 ng/mL and squamous cell carcinoma antigen 0.80 ng/mL. Another test revealed positive reaction to type B hepatitis surface antigen and C-reactive protein < 0.1 mg/dL. Computed tomography demonstrated a gradually increasing low-density mass measuring 2.0 × 1.8 cm in diameter (Fig. ). Magnetic resonance imaging demonstrated a low-intensity mass in T1-weighted imaging and a high-intensity mass in T2-weighted imaging (Fig. ). The mass was thought to be a singular cyst; however, this type of cyst was rare and the mass was increasing. Therefore, dissemination of cervical carcinoma could not be excluded, and surgical removal of a part or tissue of the mass was performed.\nIn the right lateral position, thoracoscopic excision of the mass was done with two ports (3 mm and 2 cm access ports) by two general thoracic surgeons (Fig. ). First the 3 mm port was set at the sixth intercostal space on the inframammary line. Most of her left lung was attached to her chest wall; therefore, the second port was set above the cyst and lysis of adhesions was done. After the lysis, the cystic mass was found adhering to the upper lobe of her left lung. The adhesion of the mass to her lung was not strong and could be separated without injury to the visceral pleura. Therefore, the mass was thought to derive from the chest wall pleura and was resected by adhesiolysis.\nThe mass was a unilocular cyst containing mucinous fluid. On microscopic examination, the cyst was lined with a single layer of cuboidal epithelium (Fig. ); immunohistochemistry showed positive staining of calretinin and D2-40 (Fig. ). Thus, the cyst was diagnosed as mesothelial cyst derived from the chest wall pleura. Five years after the surgery, our patient had no evidence of cyst or cervical carcinoma on computed tomography. |
A 3-year-old boy was admitted with complaints of cough, cold, fever, and poor oral intake for 2 days. He had two episodes of febrile convulsions in the past 1 year. The episodes would begin with sudden onset vomiting accompanied by cyanosis and moderate to high-grade fever. After the vomiting, he would develop deviation of eyes to one side lasting for approximately 30 min. This was followed by loss of consciousness of variable duration, ranging from 1 h to 12 h. After the second episode, electroencephalogram (EEG), cerebrospinal fluid studies, and neuroimaging were done which were normal. He was not started on any prophylactic antiepileptic medications, and a diagnosis of atypical febrile convulsions was made. His birth and developmental history was normal. There was no family history of epilepsy. On admission, he was febrile with mild congestion in the throat. Rest of the general and systemic examination was normal. He was started on symptomatic treatment after admission. At 0400 h in the night, he developed high-grade fever and vomiting. This was followed by bluish discoloration of the extremities, tachypnea, and deviation of both eyes to the right side. The episode lasted for about 10–15 min. He was unresponsive during the entire episode. He passed a large quantity of loose stools, had the second episode of vomiting, and became drowsy after the episode. His temperature was 39.9°C, oxygen saturation 90%, blood pressure 72/30 mmHg, respiratory rate 39/min, and heart rate 220/min. Fluid resuscitation was done, and high flow oxygen was started. Loading dose of phenytoin (20 mg/kg) was administered. His electrocardiogram showed sinus tachycardia. He was shifted to Intensive Care Unit for observation and monitoring. Within 2 h, he started regaining consciousness and became hemodynamically stable. Within 5 h of the episode, he was fully conscious with a normal neurological examination. After 24 h, he was shifted out to the pediatric ward. Complete blood count, serum electrolytes, blood gases, serum calcium, serum magnesium, and glucose levels were within normal ranges. Blood and urine culture were normal. C-reactive protein was 0.2 mg/L (normal <5). He was discharged after 3 days from the hospital. Phenytoin was continued for 3 days after discharge and stopped. The manifestation during the episode was similar to that seen in septic shock. However, the rapid recovery, normal investigations, and previous history of similar episodes ruled out septic shock. On follow-up after 2 months, he was asymptomatic and had a normal neurological examination. Neuroimaging and EEG were normal. |
A 65-year-old male was referred to our clinic for evaluation of a bladder mass discovered in the setting of painless gross hematuria. The patient's past medical history was notable for a myocardial infarction 4 years prior, and a cerebrovascular accident 3 years prior with residual right-sided hemiparesis and aphasia, rendering him wheelchair bound.\nCross-sectional imaging via CT abdomen and pelvis revealed an exophytic mass at the bladder dome measuring 7.8 × 5.9 cm with central necrosis and a thick, enhancing wall as well as probable communication with the lumen of the urinary bladder (Figures and ). There was no hydronephrosis or pelvic lymphadenopathy. Metastatic survey was negative. Diagnostic cystoscopy demonstrated a large mass at the dome of the bladder, and subsequent transurethral resection revealed stage T1 mucinous adenocarcinoma arising in a villous adenomatous lesion without the presence of muscle in the specimen. Serum tumor markers revealed elevated CEA at 131 ng/mL (ULN 4.7 ng/mL).\nAfter thorough counseling and informed consent about the potential role of perioperative chemotherapy the patient elected for surgical management and underwent a robotic-assisted laparoscopic partial cystectomy with extended bilateral pelvic lymph node dissection. Although no muscle-invasion had been seen on TURBT pathology, the advanced radiographic appearance of the tumor, with concern for extravesical extension, justified the surgical approach. Intraoperatively, no gross metastatic lesions in the abdomen were seen and the tumor was resected with 1.5 cm margins. All twenty-four dissected lymph nodes were negative for carcinoma. Final pathology revealed pT2b with tumor involvement of the surface urothelium and invasion of the deep muscularis propria. Pathologic evaluation further revealed that, although the location of this tumor at the dome of the bladder suggested urachal adenocarcinoma as the final diagnosis, no urachal glandular remnants were identified and so the features were most strongly suggestive of a primary nonurachal bladder adenocarcinoma (Figures and ).\nPostoperatively, the patient experienced paralytic ileus but otherwise did well and was discharged on postoperative day 5. He was counseled about the role of adjuvant chemotherapy but elected to defer given the lack of robust evidence. Follow-up surveillance imaging at 6 months with CT chest, abdomen, and pelvis revealed no evidence of disease recurrence. He also underwent a normal office cystoscopy and had negative tumor markers, indicating no evidence of disease. The patient remained disease free at 24-month follow-up.\nMolecular profiling of the tumor using the Foundation One CDx test reported microsatellite stability and a tumor mutational burden of 4 mutations per megabase. The test also described four genomic alterations: G12D on the KRAS gene, R201C on the GNAS gene, Q604 on the RMB10 gene, and R248W on TP53 gene. Targeted therapeutic options for this tumor type are limited; however, treatments targeting similar tumor subtypes have shown promise. |
A 37-year-old man presented with a 3-year history of decreased vision in the right eye, which had recently become worse. He reported no previous visual problems and had been diagnosed with a congenital colour vision deficiency at an early age. He had never been treated with antimalarial therapy or retinotoxic drugs, had no night vision symptoms and enjoyed a normal diet. He reported trauma to his head in a soccer game, and subsequent investigation had revealed an apparently incidental colloid cyst of the third ventricle measuring 6 mm. He denied a history of consanguinity in his family background. His family, based in the Middle East, had no history of inherited retinal disease, poor vision or colour vision disturbance; however, his father had recently been diagnosed with chronic central serous retinopathy at a late age, and a facsimile of an otherwise normal fluorescein angiogram with no signs for choroidal darkening or hyperfluorescent flecks was available from this recent assessment. Apparently, his condition had improved significantly with systemic spironolactone treatment.\nExamination revealed a visual acuity of 6/24 in the right eye and of 6/6 in the left eye, normal pupil reactions, 1/15 Ishihara colour vision test plates read correctly with each eye, full ocular motility and an otherwise normal cranial nerve examination. Anterior segment examination showed no abnormalities, and fundus examination (Fig. , ) revealed scattered atrophy and pisciform fundal flecks at both maculae, which were worse in the right eye. Fluorescein angiography (Fig. , ) demonstrated a silent choroid and partial bull's eye maculopathy which was worse in the right eye. Optical coherence tomography (OCT) (Fig. ) showed loss of photoreceptors at both maculae with partial central sparing in the left eye. Photopic and scotopic flash electroretinograms (ERGs) showed reduced amplitudes of responses in the major ERG components in the right eye and amplitudes within the lower range of normal in the left eye. Implicit times in both eyes were within normal limits. The pattern ERG and multifocal ERG demonstrated central retinal dysfunction with the retention of peripheral function. Next-generation sequencing analysis with the Oxford Genetics Testing Laboratory Macular Gene Panel revealed 2 mutations in the ABCA4 genes: c.5882G>A p.(Gly1961Glu), a common ABCA4 mutation, and c.2486C>T p.(Thr829Met), which has not been previously reported in association with a diagnosis of STGD. As the subject's parents were unavailable for segmentation analysis, there remains a possibility that the mutations were on the same allele.\nThe patient was referred for genetic counselling and underwent follow-up for a period of 14 months, maintaining baseline objective and subjective levels of vision in each eye with no change in fundal appearance or OCT. |
A 49-year old male patient received a deceased donor renal allograft in our institution. He was born and raised in Haiti, immigrated in France 2 years before the kidney transplantation, but maintained regular trips to Haiti. He had a history of long-standing hypertension. His chronic kidney disease was discovered in France, when it had already reached end stage. Kidney transplantation induction therapy consisted in rabbit anti-thymocyte globulines, and one steroid pulse of 1000 mg. Maintenance immunosuppression comprised tacrolimus, mycophenolate mofetil and prednisone (20 mg daily for the first 3 months, progressively tapered to 5 mg per day at month 9 post-transplantation). The lowest serum creatinine was of 140 μmol/l. Five months after transplantation, the patient acquired diabetes mellitus which required insulin therapy.\nFive years after the transplantation, while travelling to his hometown in Haiti, a tumour developed in a couple of weeks on the scalp. The tumour was surgically removed, and the histological analysis indicated a sporotrichosis. We were unable to retrieve any data about potential microbiologic culture or molecular biology analysis of the scalp lesion. The patient only brought back the histological slides that were processed in Haiti to our institution. No complementary treatment was administered and the lesion healed completely.\nOne year later, the patient was admitted to our institution after a 1-month stay in Haiti, because of fever and one cutaneous lesion on each thigh. There was no history of local trauma. Clinical examination revealed an erythematous area of 10 × 5 cm on each thigh. There was no crural lymphadenopathy.\nRenal function was stable with a serum creatinine of 165 μmol/l. Plasma C-reactive protein was at 59 mg/L. There was neither anaemia nor leucocytosis. Diabetes was decompensated with an HbA1c at 10.5%. The day after admission, blood cultures yielded Listeria monocytogenes.\nSoft tissue ultrasound and computed tomography showed an abscess of the right vast medial muscle measuring 120 × 45 × 40 mm, and of the left semi-membranous muscle measuring 150 × 70 × 55 mm (Fig. ).\nPuncture of the abscess of the right thigh recovered a serous hematic and cloudy liquid. Direct microscopic examination of this pus evidenced sclerotic body or fumagoid cells (also called Medlar bodies) that are pathognomonic for chromoblastomycosis [] (Fig. ). Pus culture raised both L. monocytogenes and black mould colonies that were identified as Fonsecae sp. (Fig. ). ITS sequencing (using the following primers: forward: 5′- TCCGTAGGTGAACCTGCGG- 3′: reverse 5′-TCCTCCGCTTATTGATATGC-3′ and the Genbank database) revealed F. pedrosoi. Minimal inhibitory concentrations of antifungal drugs could not be determined because of the impossibility to grow the fungus.\nRetrospective re-evaluation in our institution of the histological slides of the scalp lesion removed in Haiti eventually disclosed an aspect of chromomycosis.\nChest and abdominopelvic computed tomography did not show any other infectious localization. In contrast, while the patient showed no neurologic sign, systematic cerebral magnetic resonance revealed a lesion of the peripheral left cerebellum, which was enhanced after gadolinium injection and which contained a necrotic centre (Fig. ). Lumbar puncture was not performed.\nTreatment included percutaneous drainage of the abscess of the right thigh, intravenous followed by oral amoxicillin, and oral posaconazole (suspension; 800 mg/day). The abscess of the left thigh was not drained. The tacrolimus dose was decreased by 2/3, because of posaconazole-induced inhibition of cytochrome P450 3A4. Fever cessed a couple of days after anti-infectious treatment initiation, probably due the resolution of listeriosis with amoxicillin which was maintained for a total duration of 3 weeks. After 3 months of treatment with posaconazole, the abscess of the left thigh had significantly decreased, measuring only 5 × 3 cm, and the lesion of the right thigh seemed heeled. Brain MRI also showed a dramatic improvement after 3 months of treatment (Fig. ). Posaconazole was discontinued after 12 months. Brain MRI showed no cerebellum sequela. Four years after the episode, the patient was asymptomatic with no recurrence of chromomycosis. Serum creatinine was stable at 136 μmol/l. |
A 22-year-old female who reported experiencing recurrent palpitations was seen in our arrhythmia clinic. She had intermittent preexcitation on her ECG. Her palpitations were linked with stressful situations. She had gone to the emergency department a few times before and was found to have sinus tachycardia. Her ECG revealed a midseptal right-sided accessory pathway (. She had a negative delta wave in V1, and there was a transition of the delta wave in V2; this implicated the right septal pathway. Furthermore, the delta waves were negative in lead III and aVF but positive in lead II. These findings suggested a midseptal pathway. While undergoing an exercise stress test, the patient’s preexcitation disappeared completely when her rate increased to 140 bpm (. Her echocardiogram at this time was normal as well.\nGiven the patient’s atypical presentation of orthodromic reciprocating tachycardia, a 30-day event monitor was implanted, which revealed that one episode during this time period correlated with sinus tachycardia presenting at heart rates of between 110 bpm and 120 bpm (; a premature ventricular complex (PVC) was also noted during this episode. However, she mentioned other episodes that were severe had occurred but that she did not activate the monitor manually. Given her overall low risk for sudden cardiac death (suggested by intermittent preexcitation and exercise-induced loss of preexcitation) and lack of finding of SVT, the approach of long-term monitoring using a loop recorder was selected for better symptom–rhythm correlation. It was discussed with the patient that, if she ever did have an episode of SVT, she might benefit from an EPS.\nSince the occurrence of the present case, the patient in question has been followed up with at our arrhythmia clinic and, during the six months since, she experienced multiple manually triggered transmissions from her device, which were all consistent with sinus tachycardia. One of the episodes is shown in .\nIn conclusion, this patient was diagnosed specifically with sinus tachycardia with a warm-up phenomenon. She has experienced no episode suggestive of any SVT to date and is being treated for a presumptive diagnosis of inappropriate sinus tachycardia. This case highlights the importance of better symptom–rhythm correlation before pursuing more invasive treatment options for an arrhythmic etiology in low-risk patients. |
A 35-year-old female with body mass index (BMI) of weight 40.8 kg/m² with no history of any comorbidity underwent UGIE as a workup prior to laparoscopic sleeve gastrectomy (LSG). Endoscopy revealed a gastric body polyp that measured 3.0 × 2.0 cm with central ulceration. Two smaller satellite polyps were seen near the main lesion, and biopsy was taken. Histopathological examination of all lesions was the same and revealed tumor cells that were arranged in small nests with abundant granular cytoplasm and round to oval nuclei (). The tumor cells were positive for cytokeratin and neuroendocrine markers including chromogranin, synaptophysin, and NSE, diagnostic of gastric carcinoid (). Ultrasound was done routinely prior to bariatric surgery and showed a cyst in the right lobe of the liver. Eventually, a computed tomography (CT) scan of abdomen was performed which confirmed the presence of multiple rounded lesions in the greater curvature of the stomach. In addition, there was a solitary, subcapsular mass in segment 8 of the liver measuring 1.8 cm × 1.3 cm × 1.6 cm suggestive of metastasis. A CT-guided liver biopsy targeting the mass was done which showed hepatocytes with preserved architecture and foci of steatosis. Malignancy was not seen. Following the diagnosis of gastric carcinoid with a possible liver metastasis, the plan was changed to laparoscopic total gastrectomy with lymphadenectomy, Roux-en-Y gastrojejunostomy, and simultaneous biopsy of the liver mass. Postoperatively she did well and no leaks on gastrografin swallowed that was performed on the third postoperative day.\nHistopathology of liver lesion again showed preserved architecture with microsteatosis and no tumor deposit. The gastric specimen showed well-differentiated neuroendocrine tumor with lymphatic permeation. The surrounding gastric tissue shows variable degree of G-cell hyperplasia. There were tumor deposits in the serosa, and two out of nine lymph nodes showed metastasis with perinodal fat infiltration.\nPostoperative octreotide scan showed features in keeping with liver metastasis. MRI was performed and revealed a small lesion 1.7 × 1.4 cm in segment 6 of the liver suggestive of metastasis. Another focal lesion in segment 8 was noted, which was peripherally located that measured 2.1 × 1.9 cm probably site of previous biopsy. Based on imaging suspicion of liver metastasis, the patient underwent segmental liver resection in the cancer center, and histopathological examination proofed the presence of metastatic neuroendocrine tumor in the liver. |
A 2 year old female admitted to the emergency department due to 2 days of right postauricular area swelling. The patient was previously treated with antibiotics for a week at a local clinic for acute otitis media. Despite antibiotic treatment, the patient suffered from right otalgia and fever upon admission. Through physical examination, bulging of the tympanic membrane and erythematous change and swelling of the right postauricular area with tenderness were noticed. Further evaluation by temporal bone CT revealed soft tissue density filling the right middle ear and mastoid cavity with erosion of the cortical bone along with formation of subperiosteal abscess (). Under the impression of acute mastoiditis with subperiosteal abscess, percutaneous aspiration was performed on the postauricular area resulting in 3 cc of purulent fluid. The procedure was followed by incision and drainage (I & D) and oral antibiotics were prescribed to the patient. On third day after I & D, the patient showed persistent redness, tenderness, and swelling of the postauricular area () and injected bulging tympanic membrane, regardless of the treatment. Therefore, it was postulated that I & D and oral antibiotics were inadequate and the patient was admitted to our department for intravenous antibiotics therapy.\nOn the second day of hospitalization, the patient underwent surgical treatment of right simple mastoidectomy and ventilation tube insertion because of consistent symptoms and physical findings in spite of 2 days of intravenous antibiotic treatment. Intraoperative finding showed massive granuloma beneath the postauricular area and in the mastoid cavity (). Erosion of cortical bone was also noticed after Palva flap elevation. Intraoperative culture study resulted in Streptococcus pneumoniae which was only susceptible to vancomycin. Intravenous antibiotic treatment with vancomycin was immediately initiated and the child was discharged after 13 days of surgery without any postoperative complications. During the 5 month follow up, the patient showed no signs or symptoms of recurrence. |
A 59-year-old woman was admitted with a black pigmented skin lesion in the right breast. She mentioned that the lesion had appeared at birth but grew rapidly and bled recently. She had no past history of malignancy and no family history of breast carcinoma. The lesion was 2.0 × 1.5 cm in size and located on the periareolar skin in the upper inner quadrant of the right breast which is not associated any breast mass (). The axillary lymph node was not palpated and there was no nipple discharge or retraction.\nShe underwent incisional biopsy a week earlier at a local breast clinic and was diagnosed with malignant melanoma. The specimen was an ulcerative, pigmented lesion, measuring 1.0 × 0.6 cm in size and 0.25 cm in thickness (pT3b). The depth of invasion according to Clark's classification was level IV.\nShe was examined for evidence of metastatic malignant melanoma. Careful examination of the other skin and mucous membranes revealed no areas suggestive of a malignant melanoma. Mammography (MMG) revealed a dense breast. Breast ultrasonography (US) and magnetic resonance imaging showed normal findings. Brain computed tomography (CT) and positron emission tomography-CT showed no evidence of metastasis. She underwent wide local excision of the lesion, including removal of normal appearing skin and underlying subcutaneous tissue to provide a safety margin; and subsequent sentinel lymph node biopsy (SLNBx) using the technetium (Tc)-99m phytate was performed. Grossly, the specimen revealed a sharply defined, black pigmented lesion, measuring 1.9 × 1.6 cm in size including normal skin 2 cm apart from the tumor margin and subcutaneous tissue (). The cut surface revealed a superficial pigmented lesion without ulceration (). The specimen was fixed in 10% neutral-buffered formalin. Paraffin embedded tissue sections were prepared and stained with hematoxylin and eosin. Microscopic findings showed intraepidermal tumor cells and nests that were laterally spreading in a pagetoid manner and tumor nests invaded the superficial dermis 0.12 cm in thickness (pT2a) (). Tumor cells often had large nuclei and nucleoli and abundant cytoplasm with brown-black pigments (). The resection margins were free of tumor cells and there was no regional lymph node metastasis (pN0). The final pathologic stage considering incisional biopsy report was IIB (T3bN0M0) according to the 7th edition of the American Joint Committee on Cancer classification []. Additional chemotherapy and radiotherapy were not performed. Three years after surgery, the patient is alive and shows no signs of local recurrence or distant metastasis. |
The patient is a 42-year-old Caucasian male found to have bilateral renal masses incidentally during work-up for left flank pain caused by an obstructing ureteral stone. After an uneventful ureteroscopy and laser lithotripsy of the left 7 mm ureteral calculus at an outside hospital (OSH), he was referred to our institution 1 month later for evaluation of his renal masses. He was asymptomatic at this time. His medical history was significant for diabetes mellitus type 2 and hyperlipidemia (both controlled with diet and exercise), as well as hypertension managed with losartan. He denied any family history of genitourinary or other malignancies. Physical examination was unremarkable. His laboratory tests, including complete blood count, basic metabolic profile, and liver function tests, were within normal limits. A noncontrast abdominal CT performed at the OSH showed a 7.0 × 7.3 × 5.2 cm anterior, mid-to-lower pole mass on the left kidney, and a 1.5 × 1.9 × 1.6 cm medial lower pole mass on the right kidney. An MRI with contrast confirmed both renal masses with enhancement concerning for malignancy (). Chest X-ray showed no evidence of metastatic disease. The patient underwent an uneventful bilateral laparoscopic partial nephrectomy. The operation was performed using the standard laparoscopic technique in the supine position, and thus, there was no need to reposition, prepare, or drape the patient after the first side was done. The patient was secured on the operative table and we air planed the table toward the contralateral side to facilitate bowel mobilization. The smaller right-sided mass was excised first without clamping the renal vessels and the larger left-sided renal mass was excised next using a hilar vascular clamp for 14 minutes (estimated blood loss = 1200 mL). The pathology report of both renal masses showed clear cell renal-cell carcinoma (ccRCC; right, pT1b Fuhrman grade 2; left, PT1a Fuhrman grade 1) with negative margins. The patient was discharged from the hospital on postoperative day 3. At 6-month follow-up, his serum creatinine was 0.97 mg/dL and he had no evidence of recurrence. |
A 37-year-old man presented in April 2009 with seizures. Magnetic resonance imaging (MRI) brain showed a minimally enhancing left temporal lobe mass. He underwent left temporal craniotomy and debulking of tumor in May 2009 and made an uneventful postoperative recovery. Follow-up scans showed a significant reduction in tumor volume. Histology revealed the lesion to be a Grade III anaplastic oligoastrocytoma, and the patient subsequently underwent radiotherapy using conformal technique receiving 60 Gy in 30 fractions over 6 weeks in July 2009. This was delivered in two phases. The first phase delivered 54 Gy to the whole planning treatment volume in 27 fractions []. The second phase (shielding out critical organs such as brainstem, optic chiasm) was delivered with 6 Gy in three fractions. There was no specific “boost” to the tumor resection cavity/bed. Routine follow-up MRI in August 2010 showed improvement in tumor status with no visible enhancement.\nMRI in June 2011 showed a few areas of nodular enhancement within the primary tumor. This was deemed to be disease progression and chemotherapy using temozolomide was commenced. A routine follow-up MRI in April 2012 showed an entirely new area of enhancement in the left Sylvian cortex adjacent to two prominent vessels thought initially to be further tumor progression [].\nA few days after this MRI, he presented with subacute headache and acute dysphasia, the latter improving rapidly. Computed tomography (CT) brain showed a small 2 mm hyperdensity in the insular cistern corresponding to the new enhancement which had been noted on the recent MRI. CT angiography [] showed a very irregular fusiform aneurysm of a Sylvian branch (supramarginal) of the left middle cerebral artery (MCA) with the hyperdensity thought to be either intra-aneurysmal thrombus or mural/perimural hemorrhage. Digital subtraction angiogram confirmed the large, irregular fusiform aneurysm involving the posterior Sylvian branch of the left MCA []. There was a very short “normal” segment at the origin of the Sylvian branch expanding over a long segment, followed by a short relative stenosis and then a very irregular aneurysmal pouch which tapered to a normal appearing vessel with a runoff supplying the region of the inferior parietal lobule. The adjacent MCA branches showed subtle variation of calibre suggestive of mild vasculitis. MRI brain showed a cap of thrombus around the distal part of the aneurysm suggesting intraluminal or intra-mural thrombus. There was no clear evidence of extraluminal hemorrhage []. These appearances were almost similar to the MRI done a few days before the patient's presentation.\nEndovascular occlusion of the aneurysm was considered the best treatment option, provided vessel occlusion was tolerated clinically. Awake catheter angiography was performed to trial balloon occlusion. Placement of a balloon microcatheter, without inflation, into the origin of the MCA branch was sufficient to arrest flow []. The patient immediately developed profound dysphasia; consequently, the procedure was abandoned.\nThe patient was discussed with neurovascular surgeons and interventional neuroradiologists at two other specialist centers regarding bypass, flow diverters, or stent placement. However, these were deemed to be exceptionally difficult technically with a very high risk of leaving the patient with the permanent neurological deficit. The patient's underlying diagnosis of Grade III oligoastrocytoma and the possibility of generalized vascular fragility due to radiotherapy/vasculitis also served as a detriment while considering the option of bypass or stenting. After detailed counseling, the patient also opted for conservative treatment.\nMRI, 3 months later [], revealed narrowing and partial thrombosis of the aneurysmal segment and the patient remained asymptomatic. Regular follow-up MRI and MR angiography over a period of 42 months has shown stable appearances of the aneurysm and the patient has remained asymptomatic. Unfortunately, the tumor has now started showing signs of radiological progression, and the patient has now been commenced on procarbazine, CCNU, vincristine chemotherapy. |
A 4-year-old boy was brought to the Emergency Department (ED) from a rural area with complaints of fever, headache, and weight loss. He was suffering from these symptoms for the past 4 months, which had worsened in the past week. His brother informed that the child had been unwell since infancy with several episodes of respiratory infection and has had several hospital admissions. Recently, he had started complaining of severe persistent diffuse headaches along with nausea, vomiting, and anorexia. He also suffered from low grade fever intermittently over this period of time. Physicians in his locality who had treated him with antibiotic courses for chest infections started him on antituberculosis (ATT) drugs 2 months back after an MRI, which suggested meningeal enhancement and small ring enhancing lesions. He had no history of seizures or loss of consciousness. One of his brother had passed away at the age of 8 years who was considered to suffer from disseminated tuberculosis. On examination, the child was listless, emaciated, and extremely irritable. He had left-sided weakness in both the upper and lower extremities.\nAn urgent computed tomography (CT) scan of the head showed multiple rings enhancing the left parietooccipital region, with significant surrounding edema and mass effect []. His white blood cell count was 17900/uL, with neutrophils 77.4% and lymphocytes 15.7%. Hemoglobin was 10.9 and erythrocyte sedimentation rate (ESR) was 88 mm/h. We involved pediatric physicians and infectious disease specialists to perform a neuronavigation-guided craniotomy for the excision of these abscesses along with the walls, which contained thick purulent material. Microscopic examination revealed granulomas with fugal hyphae [Figure and ]. Presence of Aspergillus was further confirmed on culture. Postoperatively, he was successfully extubated and shifted to the intensive care unit for postoperative monitoring. His weakness also improved. Considering his chronic cough and repeated chest infections, we obtained a tracheal aspirate for examination, which was positive for Mycobacterium tuberculosis polymerase chain reaction assay. Considering the history, we screened the child for immune deficiency disorders. Nitroblue-tetrazolium (NBT) was found to be negative, which established the diagnosis of CGD.\nIntravenous (IV) voriconazole was commenced at 6 mg/kg/dose every 12 h for 2–3 days, which was later switched to 6 mg/kg/day orally. Five-drug ATT treatment was restarted for the management of pulmonary TB. He was also given broad-spectrum antibiotics. Pneumococcal and meningococcal vaccines were administered as prophylaxis, and co-trimoxazole was included in the regimen for the prevention of pneumocystis carinii pneumonia.\nThe child showed slow improvement, however, the treatment course was complicated by fever, seizures, and hyponatremia. Repeat CT scans did not show residual or recurrent abscesses []. After stabilization of the medical condition, he was discharged. About 10 days after discharge, his family brought him to the ED with complaints of drowsiness. CT scan showed hydrocephalus []. A temporary cerebrospinal fluid (CSF) diversion was performed, followed by a ventriculoperitoneal shunt once the CSF culture was reported negative. The child was again discharged after stabilization. He died 4 months later at home in respiratory distress. The child could not be brought to us, and hence no autopsy was performed to determine the actual cause of his death. |
A 38-year-old woman with a 3-month history of an anterior cervical mass located in the suprasternal region of her neck was referred to our hospital. Physical examination revealed that the 3-cm movable neck mass was firm and slightly tender on palpation and had a distinct margin from surrounding tissues. An ultrasound examination showed that the well-defined oval mass was 31 × 23 × 17 mm in size and exhibited heterogeneity. Furthermore, a non-enhanced computed tomography (CT) scan of the neck revealed that the distinct neck mass in the subcutaneous tissue had a mixture of soft tissue and fatty components (Fig. ). Based on these findings, the cervical tumor was clinically diagnosed to be an unusual lipoma with degeneration; however, we could not exclude the possibility of it being a malignant tumor such as liposarcoma. Thus, fine-needle aspiration cytology of the tumor was performed, but no diagnosis of malignant cells was obtained. Further examinations were conducted to address concerns related to her menstrual pain, and abdominal magnetic resonance imaging revealed bilateral ovarian cysts in the lower abdomen. Under general anesthesia, the patient underwent neck mass extirpation and bilateral ovarian cystectomy; pathological examination of the cysts resulted in a diagnosis of ovarian endometriotic cysts. During the surgery, the cervical mass was well demarcated and did not adhere to the surrounding tissues. The postoperative course was uneventful. The gross pathology report showed that the neck mass measured 3.0 × 2.5 × 2.0 cm. The cut surface of the specimen was heterogeneous, solid, whitish, and yellowish (Fig. ). Microscopically, the tumor was composed of spindle cells, epithelial nests, and mature adipose tissue (Fig. a, b). Immunohistochemical examination revealed that both spindle cells and epithelial nests were positive for cytokeratin (CK) AE1/AE3 (Fig. ). As these histopathological findings were consistent with the features of EHT, a diagnosis of EHT was confirmed. Over a follow-up period of 30 months, this patient exhibited no evidence of recurrence. |
Our patient was female, almost six months old, the first child of unrelated, healthy Caucasian parents. The perinatal history was complicated by gestational diabetes. The girl was delivered on term via spontaneous vaginal labor at 36 weeks of pregnancy due to a premature rupture of membranes. Her birth weight was 2340 g and she received 9 points on the Apgar scale. There were no abnormalities in the healing of the umbilicus. The girl was rehabilitated from the beginning of the third month of life on account of increased muscle tone (hypertonia). Furthermore, she had an episode of acute diarrhea at three months of age, apart from which she did not suffer from any other illness. She was fully vaccinated by 6 months of age.\nThe patient presented with episodic diarrhea, intermittent “sun-setting eye phenomenon” and mild facial dysmorphism to a rural hospital where ultrasonography revealed hepatomegaly with numerous diffuse areas and decreased echogenicity, as well as failure to visualize the spleen twice. An echocardiogram disclosed atrial septal defect (ASD) type 2 and treatment with spironolactone was introduced. The structures of the brain were correctly visualized in a transcranial ultrasound. Moreover, family history of lupus erythematosus was only positive in the mother’s sister.\nThe patient was transferred to the hematology department due to high white blood cell count (WBC), increased hemoglobin and platelet count, with no abnormalities in the peripheral blood smear, accompanied by a suspicion of congenital asplenia. Due to the good clinical condition of the patient, but with a not high persistent WBC (up to 20 K/μl) and Hb (up to 17 g/dl), further scheduled diagnostics were planned. In addition, due to the suspicion of immunity disorders related to congenital asplenia, antibiotic prophylaxis (amoxicillin) and additional vaccinations against Streptococcus pneumoniae and Neisseria meningitidis were ordered, as well as a postponement of “live vaccinations”. Cocoon vaccination was also ordered for the patient's family against influenza with an inactivated vaccine.\nDuring further diagnostics and subsequent hospitalizations, bone marrow trephine biopsy was performed, which showed no hematopoietic abnormalities. Laboratory tests revealed an increase in WBC (up to 41 K/μl) with the normal platelet and red blood cell count, slightly increased activity of aspartate aminotransferase (AST) and Alpha Fetoprotein (AFP) concentration within the age range. Subsequently, a spontaneous normalization of WBC was observed. The peripheral blood smear did not raise oncological concern but revealed Howell-Jolly bodies characteristic of asplenia (Fig. A).\nThe following ultrasonography confirmed the absence of a spleen. Angio-CT revealed a developed, although rather narrow, splenic artery, directed to the left epigastric region, and the absence of a normal splenic vein. Consulting medical geneticist did not confirm any dysmorphic features and performed cytogenetic evaluation revealed constitutional karyotype as normal 46, XX. The absence of any cardiovascular abnormalities (except ASD) helped exclude heterotaxy syndromes such as Ivemark syndrome associated with asplenia []. Subsequent observations at age of one 12 and 16 months revealed resolution of any additional symptoms, which allows us to contribute to the diagnosis of ICA. Due to the high risk of ICA-related immune disorders, a full panel exam of immunoglobulin classes, subclasses and lymphocyte subpopulations was performed. No aberrations were found in the major immunoglobulin classes and IgG1-4 subclasses nor in the percentage distribution of the primary lymphocyte subpopulations. However, absolute values were elevated due to lymphocytosis. In a detailed analysis of the B lymphocyte subpopulation, the percentage of non-switched memory B cells slightly decreased while cells with the CD27-IgD phenotype increased.\nSubsequent observations revealed resolution of symptoms such as disturbances in muscle tension, after rehabilitation. A thorough assessment, including neurological consultation, did not release any defects, and the development was evaluated as adequate to her age. Further clinical and laboratory observations did not reveal any abnormalities therefore, we suspect that haploinsufficiency of SLC25A38, SNORA6, SNORA62 and MOBP genes does not affect the phenotype of patient.\nTargeted Next Generation Sequencing for a panel of genes associated with primary immunodeficiencies and hematological defects was applied [, , ] (complete list of genes in Additional file : Table S1). This included genes associated with asplenia i.e. GJA1, HMOX1, RPSA, STIM1 and AIRE [, , ]. Based on this approach no genetic defect was identified. However, only within a locus of RPSA gene homozygosity of the known SNP were noticed (Additional file : Fig. S1A), which might be suggestive for deletion in respective interval. Thus, this implied to perform a confirmatory searching for copy number variation. A dense genomic SNP microarray approach revealed approximately 50% of signal reduction in 312 SNPs located at RPSA locus which corresponds to a 337.2 kb deletion covering a fragment at the short arm of chromosome 3 (arr[GRCh37] 3p22.2p22.1(39357060_39694267) × 1), affecting five genes, including the RPSA gene (OMIM #150370) which encodes the SA ribosomal protein (Fig. B). This was the only CNV identified in this patient. None of the parents was a carrier of the same deletion (Additional file : Fig S1B and C). This confirms the diagnosis of Congenital Isolated Asplenia (ICAS, OMIM # 271400) resulting from de novo occurring monoallelic deletion of the RPSA gene.\nSearch for copy number variations at 3p22 using the International Standards for Cytogenomic Arrays databases using Decipher version 9.32 revealed that among 40,448 CNV records only 9 affected RPSA, including eight duplications (CNV frequency less than 0.02%). No deletion was found. This suggests that CNVs at the 3p22 are very rare phenomena occurring in germline human genome, and our patient could be treated as unique.\nLiterature review on haploinsufficiency in incidentally founding SLC25A38, SNORA6, SNORA62 and MOBP genes (Table ) revealed possible abnormalities, which, however, did not occur in the described patient. |
A 41-year-old man presented with 7 months history of persecutory delusion' delusion of reference, auditory hallucination, and vagrant-like behavior. A diagnosis of schizophrenia was made. He was commenced on haloperidol 5 mg bid and later increased to 10 mg bid. Four weeks later the medication was changed to olanzapine at a dose of 20 mg daily after delusion persisted and had an extrapyramidal side-effect. The patient developed swelling of both legs, moderate edema up to the knee, 2 weeks after introduction of olanzapine. Physical examination of other system was normal. The urea and electrolyte, creatinine, urinalysis, liver function test, fasting blood glucose, and lipid profile were normal. Abdominal scan and chest X-ray were also normal. The thyroid function test was normal. There was no diurnal variation of edema or skin changes or itching. The olanzapine was reduced to 10 mg. Within 1 week of reducing olanzapine, the edema resolved. Due to the presence of psychopathology, the olanzapine was again increased to 20 mg but edema reappeared by the 5th day. This time around the edema was severe compared to the earlier presentation. Olanzapine was reduced to 10 mg, and the swelling of the lower limbs resolved after 1 week. Naranjo's score was 8.\nIn both cases, the presence of peripheral edema could be attributed to use of olanzapine since edema disappeared in the first case when olanzapine was stopped and in the second case when it was reduced.\nIn the first case, the patient had been on sodium valproate before the introduction of olanzapine without peripheral edema, and edema did reduce with olanzapine stoppage while still taking sodium valproate. In some cases, it had been noted that valproate may increase the plasma concentration of antipsychotic.[] Chen et al.[] and Yalug et al.[] reported edema in patients in which valproate was used as co-medication with quetiapine and olanzapine respectively. By decreasing the hepatic activity of CYP3A4, a metabolizing isoenzyme, it may contribute to higher level of plasma concentration of antipsychotic.[] Valproate on its own has been associated with isolated peripheral edema.[]\nOn the other hand, drug-induced edema may be due to dose-dependent mechanism,[] and not related to co-medication with valproate.[] This is consistent with our second case in which reduction of olanzapine resulted in the disappearance of edema.\nIn the two cases presented, edema can be attributed to olanzapine as other causes of edema such as renal, cardiac, and other drug-induced causes were ruled out. There was a temporal relationship between the start of olanzapine and the development of edema using the Naranjo's adverse reaction scale as causal assessment is regarded as having a score of at least 7.[] |
A 13-year-old male was reported to the Department of Pedodontics and Preventive Dentistry, DJ College of Dental Sciences and Research, Modinagar with a complaint of painless swelling in the right lower back tooth region since 2 months. Extraoral examination revealed a nontender and firm swelling on mandibular right side of face. Intraoral examination revealed a grossly decayed 85. Buccal vestibular expansion extending from distal aspect of 44 to mesial aspect of 46 was noticed. Panoramic radiograph revealed a single well-defined periapical unilocular radiolucency in relation to 85 region, with a radiopaque rim, of about 3 × 2 cm in size, extending from distal aspect of 42 to mesial root of 46 anteroposteriorly, involving tooth bud of 45 and displacing developing tooth bud of 43 inferiorly and mesially. The cyst caused tipping of the developing root of 44 and pushed it mesially causing the crown to incline distally (). Occlusal radiograph revealed a considerable expansion of buccal and lingual cortical plate of mandibular right side which was taken into account (). Based on history, clinical and radiographic examination, a provisional diagnosis of radicular cyst associated with 85 was made.\nAfter the blood investigations and consent of the guardian of the patient, the case was posted for a surgical management of the lesion under local anesthesia. Crevicular incision was made from mesial of 44 to distal of 46 along the gingival margin and mucoperiosteal flap was raised. The socket margins were widened to gain access to the cystic cavity and cavity was decompressed (). The lingual cortical plate was left intact. Eighty-five was extracted (). Part of cystic lining was excised and developing tooth bud of 45 was removed based on operator’s understanding and expert opinions (). Wound irrigation was done with normal saline and excised cystic lining was sent for histopathology examination. The flap was sutured back with simple interrupted sutures. The cystic cavity remained exposed through extraction socket of 85.\nThe surgical defect was packed with ribbon gauze soaked in betadine ointment. An obturator was prepared to promote bone healing, to prevent food accumulation and to maintain a patent surgical opening ().\nRibbon gauze dressing, dipped in betadine and glycerine to keep the cavity moist was replaced every alternate day for a period of 6 months. A bimonthly panoramic X-ray aided in keeping a track on the healing of site. A substantial reduction in the size of the cystic cavity as well as uprighting and promotion of eruption of 43 was seen in a 6 months follow-up panoramic X-ray ().\nHistologically, a nonkeratinized stratified squamous epithelium, arcading pattern of proliferation and dense chronic inflammatory cell infiltrate were evident which were consistent with the diagnosis of radicular cyst ().\nA lingual arch space maintainer was planned and placed to prevent mesial migration of 46 and to promote eruption of 44 into normal occlusion (). |
A 56-year-old male visited his primary care physician to be evaluated for abdominal pain, and the subsequent workup revealed a large retroperitoneal mass measuring 21.8 × 17.5 × 11 cm. A diagnostic workup revealed a poorly differentiated germ cell neoplasm, most likely an embryonal carcinoma. Two months after his initial presentation he began chemotherapy (bleomycin, etoposide and cisplatin). By day 10 of cycle 3 (approximately 4 months after initial presentation), imaging showed a drastic decrease in tumor size.\nSix days after his most recent dose of chemotherapy, he presented to the emergency department noting intermittent fatigue, nausea, vomiting and diarrhea since his last chemotherapy treatment. He also noted headache and fever over the past day. In the emergency department (hospital day 0), he was found to be neutropenic with a total white blood cell (WBC) count of 0.6 × 109 cells/l and an absolute neutrophil count (ANC) of 0.4 × 109 cells/l. He appeared to be in no acute distress but febrile with a temperature of 38.7°C. No obvious signs of an infectious source were noted, including examination of his port-a-cath. The patient was started on cefepime and admitted to the inpatient oncology service for treatment of neutropenic fever.\nThe admitting physician noted tachycardia, but the patient's blood pressure was within normal limits. The only possible localizing sign of infection was a questionable cellulitis of the right lower leg. As the patient had previously been on prophylactic levofloxacin and had frequently been to infusion clinics, vancomycin was added for methicillin-resistant Staphylococcus aureus coverage. The patient was also started on G-CSF (480 μg/day) for neutropenia. Over the subsequent 2 days, his fever and tachycardia continued, and on hospital day 3 the patient became lethargic and confused. A chest radiograph (which showed no sign of pneumonia), blood and urine cultures and a CT scan of the head without contrast showed no acute changes. Throughout this period his ANC remained <1.0 × 109 cells/l.\nBy hospital day 4, the patient was unable to respond to commands and was transferred to our institution for further care; his WBC count had risen to 1.8 × 109 cells/l (ANC not measured) and he remained febrile. His vancomycin was continued while cefepime was changed to ceftazidime out of concern for cefepime-induced encephalopathy []; G-CSF was continued. Shortly after arrival, the patient had 2 tonic clonic seizures, and lorazepam and fosphenytoin were given. He became more lethargic, with periods of apnea, and was transferred to the intensive care unit for endotracheal intubation. An MRI of the brain and MRA of the brain and neck with and without contrast showed an acute punctate infarct in the right hippocampus and no major vascular findings. Continuous EEG monitoring was started. His WBC count shortly after intubation was 11.4 × 109 cells/l with an ANC of 7.0 × 109 cells/l. Fig. shows ANC, WBC count, G-CSF dosing and the patient's symptoms over time.\nA lumbar puncture was performed on hospital day 5 and revealed 64 WBC/μl, 30% neutrophils, 39% lymphocytes, glucose 37 mg/dl and protein 99 mg/dl. No organisms were seen on Gram staining. No cerebrospinal fluid opening pressure was measured. A thorough cerebrospinal fluid workup for infection was sent for evaluation, and empiric acyclovir was started. Cerebrospinal fluid India ink stain was completed in the early evening of hospital day 5 and showed rare encapsulated yeast, and the cerebrospinal fluid cryptococcal antigen (Latex Agglutination, IMMY, Inc., Norman, Okla., USA) was positive at a dilution of 1:32.\nThe infectious disease service was informed of the result, amphotericin B lipid complex was started at a dose of 5 mg/kg, and flucytosine 100 mg/kg/day was also ordered but was not but available until hospital day 7. On hospital day 6, the WBC count rose to 28.0 × 109 cells/l and G-CSF, acyclovir, ceftazidime and vancomycin were stopped.\nThroughout hospital days 6 and 7, the patient was minimally responsive despite no sedation since intubation; however, he did withdraw from painful stimuli. No additional seizures occurred. On hospital day 8, the patient moved his lower extremities on command, and his neurological function rapidly improved. Repeat lumbar puncture was performed on hospital day 9 with an opening pressure of 16 cm H20. By hospital day 10, the patient's neurologic status had improved, and he was extubated. His culture showed Cryptococcus neoformans resistant to flucytosine, and so flucytosine was replaced with fluconazole 800 mg daily.\nAfter 14 days total of amphotericin-based combination therapy, the patient continued on fluconazole 800 mg daily consolidation monotherapy. Repeat lumbar puncture at the time of stopping amphotericin showed a decreased cryptococcal antigen titer of 1:4 and an opening pressure of 22 cm H20. On hospital day 21, the patient was discharged to a transitional care unit. Since hospital discharge, the patient has continued to do well in terms of his CM, although he has required hospitalization for pericarditis. He is being maintained on fluconazole 200 mg daily for secondary prophylaxis, and further chemotherapy has been postponed pending improvement of his other health issues. |
A 50-year-old female was admitted with a paroxysmal boring pain in the left parietal and left frontal region for 9 months, accompanied by progressive vomiting and decreasing of visual acuity and proptosis in the left eye. Headache could be relieved by diclofenac sodium. Two months prior to presentation, her headache worsened extending to the whole left side of the face, accompanied by numbness. No light perception or reflex was detected in the left eye. Horizontal movement was limited in the left eye. Pre-operative CT showed a 29 × 20 mm isodense lesion with heterogeneously enhancement in the region of the left cavernous sinus, left orbital apex, left optic nerve canal, and sphenoid sinus. Bone destruction could be seen in the left wall of the sphenoid sinus. The optic nerve could not be distinguished from the lesion (Fig. a). MRI showed a 21 × 26 mm patchy lesion that was isointense on T1WI and hyperintense on T2WI in the left cavernous sinus region (Fig. b, c). Enhanced-MRI showed noticeable enhancement of the lesion. The lesion was closely related to the dura mater and extended to the sphenoid sinus and optic apex, partly surrounding the left internal carotid artery (Fig. d). MRA suggested stenosis in the cavernous segment of the left internal carotid artery and A1 segment of the left anterior cerebral artery. The patient underwent partial mass excision via a pterional craniotomy due to its close attachment to the ophthalmic branch and maxillary branch of the trigeminal nerve. The lesion was partly fibrotic and partly a milk-white viscous substance. A biopsy was performed immediately and histopathologic examination revealed Aspergillus species (Fig. e). The patient was administered voriconazole after surgery. Post-operative images indicated the resection of the mass (Fig. f). Examination after a follow-up of 15 months showed a complete resolution of the headache. Her vision of the left eye was not regained. |
Our patient, a 42-year-old married, premorbidly well-functioning male with no family history of mental illness being reported, recently visited the dermatology outpatient department along with his wife, for complaints of generalized hypopigmentation of the skin and truncal hair loss, which was gradual in onset of more than 12 years duration. During the examination, he was found to be gesturing and muttering, as if he was in conversation with an imaginary person. He was referred immediately to the psychiatry department where he was admitted for further evaluation.\nHis history revealed that he was apparently normal until 13 years back whereupon he had been admitted to the critical care unit for symptoms of sudden onset weakness, vomiting, dehydration, and melena following a Russell's viper snake bite. He was treated with supportive management. On discharge, our patient was diagnosed to have hypoadrenalism and was managed with oral prednisolone 2.5 mg twice in a day. Two months later, he was admitted again for hypoglycemic shock after complaining of the generalized weakness of the body. He was also diagnosed to have hypothyroidism during this time and was started on oral levothyroxine 50 mcg once in a day. Unfortunately, since the patient belonged to the low socioeconomic status, he dropped from further hormonal supplementation and follow-up after a month. Later over the years, his family gradually noticed personality changes in him. He would have low mood, anhedonia, increased religiosity, poor short-term memory, decreased social interaction, sleep, libido, and appetite. He would stop going for work citing he was being persecuted by the police and for the past 4 years he would be found muttering to himself and gesturing, as if he was in conversation with an invisible person. There was history suggestive of type 2 diabetes mellitus and hypertension in first and second degree relative but no history of other endocrine disorders in the family.\nOn mental status examination, he was conscious and oriented with poor eye contact, decreased psychomotor activity and talk, guarded about revealing the details of his persecutory ideas and described visual hallucinations of seeing ghosts to which he would converse, impaired attention, concentration, and immediate memory with Grade 3 insight. System examination showed generalized hypopigmentation, decreased body hair, 0 and bilateral pitting edema. The blood pressure was 110/80 mm of Hg with no postural hypotension. There were no neurological deficits on examination. His investigations revealed the thyroid stimulating hormone (TSH) levels of 3.73 mIU/L, T4 levels as 0.05 mcg/dl, T3 levels of 31 ng/dl, morning serum cortisol levels of 0.09 mcg/dl, and serum testosterone levels of 1.83 nmol/L. His routine blood examination, routine urine examination, routine blood sugar, serum sodium, calcium, bicarbonate and potassium, renal and liver function test, blood pH, and lipid profile were normal. Cosyntropin stimulation test, assays for growth hormone, follicular stimulating hormone, and luteinizing hormone were not available in our institution. Magnetic resonance imaging scan revealed an empty sella []. The diagnosis of organic delusional (schizophrenia-like) disorder was made as per 10th revision of International Statistical Classification of Diseases and health related problems.\nAfter the consultation with Department of General Medicine, the patient on the day 2 of admission, was started on oral levothyroxine 50 mcg/day and on day 5, after all investigation results had become available, oral prednisolone 5 mg in the morning and 2.5 mg in the evening was initiated. Patient's general condition improved subsequently, and his hallucinations and delusions subsided by day the 10 and discharged on the day 14 with the same medications along with vitamin supplements. He is on follow-up for the past one year, and the patient is maintaining the thyroid hormones and morning serum cortisol levels in the normal limits with the formerly mentioned medications. |
A 28-year-old female presented as an emergency with 1½ months’ amenorrhea and acute pain abdomen. She had had two normal deliveries and one intrauterine death, which had been followed by manual removal of the placenta. The patient also complained of reduced flow during periods since 2 years. On examination, the vitals were stable. Per abdominal examination revealed tenderness in the hypogastrium and left iliac fossa. A cystic mass of about 7×8 cm size was felt in the left iliac fossa; the lower border of the mass could not be felt. Pelvic examination was limited by pain. Per speculum examination showed a cervical fibroid arising from the posterior lip of the cervix. The os could not be visualized. Per vaginal examination revealed extreme tenderness on movement of the cervix. Movements of the mass were transmitted to the cervix. An emergency transabdominal ultrasound revealed an enlarged hour-glass shaped uterus, with sudden narrowing in the region of the lower uterine segment. There was an iso-to hyperechoic collection within the endometrial cavity with an approximate volume of 100 ml [Figures and ]. A transvaginal scan showed the collection in the endometrial cavity clearly [Figures and ]. The urine pregnancy test was negative and routine examination was normal. Hemoglobin was 9 g/l and the white blood cell count 14800/mm. Attempts at dilation of the cervix with Hegar dilators were unsuccessful. The patient underwent diagnostic laparoscopy, which revealed a enlarged cystic uterus. Under anesthesia, the attempt to dilate the cervical os was repeated but was unsuccessful as before.\nAs the patient had requested for hysterectomy, we decided to proceed with a vaginal hysterectomy. During the procedure, while pushing the bladder, the anterior wall of the thinned-out uterus gave way and thick, old, blood was seen coming out of the rent. Histopathological examination of the specimen showed cervical fibroid. The patient was discharged after an uneventful postoperative period. |
A 68-year old female presented with pulsatile swelling over the lower part of right forearm near the wrist joint which was painful and gradually increasing in size for past 8-weeks ().\nShe had undergone coronary artery bypass grafting (CABG) along aortic valve replacement 2 months earlier at a private centre for which coronary angiogram was performed preoperatively through right transradial route. She was receiving aspirin-325 mg, warfarin-5 mg, frusemide-40 mg, and rosuvastatin- 20 mg.\nAfter few days following angiography, small swelling was noted at the radial puncture site for which she consulted her physician. According to her, manual compression was attempted which failed to resolve it. She was advised surgery which she refused as she was already traumatized by her past experience. By the time she visited our hospital, swelling had already progressed. On clinical examination, blood pressure was 112/84 mm Hg in left arm in supine position. Pulse rate was 76/min, regular, normal in volume, with no special character and all pulses were equally palpable. On auscultation, S1 was normal and S2 was normal with aortic click. Her electrocardiogram showed sinus rhythm and chest X-ray postero-anterior view indicated normal cardiac size. Her routine investigation, and biochemistry including international normalized ratio was within the normal range. Transthoracic echocardiogram (TTE) revealed normal systolic function, normal opening and closing of aortic valve prosthesis, and grade II diastolic dysfunction. Duplex ultrasound was performed which confirmed it to be a large pseudoaneurysm arising from right radial artery with a narrow neck (0.18 mm) ().\nHaematoma was ruled on the basis of pulsatile nature and persistent communication of the swelling with the arterial lumen with to and fro flow through its neck. True aneurysm was ruled out on the basis of narrow neck of the swelling.\nAs manual compression had already failed to resolve the swelling and it had been more than 2 months old, compression using ultrasound probe was not considered, and thrombin injection being unavailable, percutaneous endovascular exclusion was planned after taking her informed consent as she refused any surgery further. 6-F sheath was introduced into right brachial artery antegradely and cocktail containing 200 μg nitroglycerin, 2.5 mg diltiazem, and 5000 IU unfractionated heparin (UH) was administered. Radial angiogram was performed through 6-F Judkins Right (JR) guide catheter (Medtronic Inc, USA) which revealed pseudoaneurysm, its narrow neck and large size (A, B). 0.014-inch runthrough wire (Terumo Inc, Japan) was parked into distal branch of radial artery. 3.5x18-mm Graftmaster covered stent (Abott Vascular, USA) was positioned over the neck of pseudoaneurysm (C).\nThe stent was slowly deployed over one minute time at 14-atm pressure (A). Subsequent angiogram demonstrated complete exclusion of pseudoaneurysm without any spillage of contrast into the sac (B).\nThe sheath was removed after 6 hours of keeping the activated clotting time < 180 seconds and replaced by light pressure bandage using dynaplast, which was removed next day. Ultrasonography on the following day revealed partially thrombosed and completely excluded pseudoaneurysm and patent radial artery ().\nThe patient was discharged in stable condition with appropriate advice. In the 6-week follow up, the swelling had completely normalized (). At 1-year follow up, radial artery was patent as Barbeau test was normal. |
A 3-year-old healthy female child presented to her pediatrician’s office with left-sided submandibular neck swelling for 16 days. On clinical examination, a “pillow-feather” on left side of her neck with a tender nonpulsatile swelling was noted (). She was referred to a nearby emergency department (ED), the feather on the neck was removed and was prescribed a course of oral antibiotics. Over the next 3 days, she developed fever and increased left neck swelling. She was seen at another ED where computed tomography (CT) scan of the neck was performed and showed left neck abscess. The patient was subsequently admitted to an outside hospital where incision and drainage of left neck abscess was done, and she received intravenous antibiotic treatment with vancomycin and ceftriaxone for 4 days. Cultures from the neck abscess grew Streptococcus viridians group, Staphylococcus species, and Eikenella, all sensitive to ampicillin/sulbactam. The patient was subsequently discharged home to continue treatment with oral amoxicillin/clavulanate. The night before hospital discharge, she was noted to have some bleeding from the surgical site which was later controlled with pressure so she was discharged as planned.\nAfter a week, she returned to the ED with worsening left-sided neck swelling and restricted neck movement. On examination she was found to be hypotensive and tachycardiac with blood pressure 85/60 mm Hg and pulse of 110 beats/min. Examination of her neck revealed a 1-cm horizontal incision on the left side. There was erythema, induration, tenderness, and extensive soft tissue swelling of the area over the anterior triangle of the left neck. The rest of the physical examination was unremarkable. Contrast-enhanced CT scan of the neck was repeated and showed hyperdense collection in the soft tissues of the left neck region posterior to the angle of the mandible, which demonstrated continuity with a branch of the left external carotid artery. This collection demonstrated mass effect on the adjacent internal and external carotid arteries with medially displaced (). Coronal view showed multiple ill-defined low-attenuation round areas throughout the soft tissues of the left neck region with no distinct enhancing contours. Based on these findings, the patient was transferred to our institution for further management. On route, bleeding from the incision site was noted, but subsided on arrival. |
A 34-year-old Caucasian female presented with an eight-month history of frequent emergency department (ED) visits, related to episodically severe abdominal pain with nausea and vomiting. Within several weeks prior to presentation, she presented at the ED four times for migrating abdominal pain with associated nausea and vomiting. During that time, she was treated for gastroenteritis and irritable bowel syndrome but continued experiencing abdominal pain and nausea. With each ED visit, she underwent similar clinical and imaging evaluations that focused on the chief complaint of nausea, vomiting, and abdominal pain (urine tests, ultrasound, CT of abdomen and pelvis, etc.).\nSeveral ovarian cysts, thought to be physiologic, were identified via abdominal scan. Finally, exploratory laparoscopy was performed. The following day, she exhibited progressive neurological deterioration. Cranial CT demonstrated hydrocephalus. In an obtunded state, she was emergently transferred to neurosurgery service.\nUpon physical examination, she was unresponsive to verbal cue. She was able to moan and to move all four extremities. Her pupils were large and sluggishly reactive.\nShe underwent emergent ventriculostomy and returned to her normal neurological baseline within 24 hours. Cranial MRI evaluation demonstrated a cystic mass near the caudal region of the fourth ventricle, resulting in obstructive hydrocephalus and brainstem compression (Figures and ).\nShe subsequently underwent suboccipital craniectomy for gross total tumor resection. Intraoperatively, after the arachnoid overlying the cistern magna was opened and the cystic mass was punctured and well decompressed, the cyst wall was dissected free from surrounding brain, including the floor of the fourth ventricle and cerebellum. The cyst wall was noted to be particularly adherent to the AP.\nMicroscopic examination of the dissected tissue revealed a cystic mass lined by mucous columnar cells, surrounding proteinaceous material, calcifications, vascular ectasia, and reactive changes with embedded small glandular structures. The diagnosis of NC was confirmed (please refer to ).\nHer symptoms of nausea, vomiting, and abdominal pain were all resolved on postoperative day 1. She was discharged home in 5 days uneventfully. She made an excellent recovery without a clinical need for a ventriculoperitoneal shunt. At her most recent one-year follow-up visit, she reported no neurological concerns. Follow-up cranial MRI confirmed the complete removal of the cystic mass (Figures and ). |
A 44-year-old man with ESRD caused by hypertensive nephrosclerosis on hemodialysis for 15 years was admitted to our hospital for sudden onset of severe left flank pain and nausea. The pain had started suddenly about 2 hours before and worsened rapidly to its peak such that he had never experienced before.\nHis past medical history included hyperthyroidism, bilateral subdural hematoma, and coronary heart disease. He got a coronary artery bypass grafting (CABG) 11 months before his admission. Since then, he took aspirin and warfarin daily.\nOn admission, his blood pressure and pulse rate were 102/65 mmHg and 86 bpm, and he was symptomless except for mildly distended abdomen with severe tenderness over his left flank to back. Laboratory findings showed a low serum hemoglobin concentration 10.0 g/dL. In addition, PT-INR was slightly prolonged to 3.50. Computed tomography (CT) showed a huge hematoma extending from left kidney to perirenal and left retroperitoneal space. The scan also showed multiple cysts in both kidneys ().\nAlthough, fluid resuscitation as well as prothrombin complex concentrates (PCC) for reversal of anticoagulation was initiated, his blood pressure declined to shock. In renal arteriography, we saw very mild extravasation at the periphery of left renal artery. We emergently performed transcatheter arterial embolization (TAE). Shortly after TAE, he recovered completely.\nSeven months later, he was again admitted to the hospital because of fatigue and weakness. His blood pressure had been well controlled after discharge. On admission, no fever, vomiting, or other symptoms were present. His blood pressure and pulse rate were 92/60 mmHg, 102 bpm. His abdomen was mildly distended and mild tender to palpation in the right middle and lower quadrants. Laboratory findings showed a severe low serum hemoglobin concentration 5.3 g/dL, prolonged PT-INR 7.05, under anticoagulation therapy with warfarin. The CT scan showed a large cyst and a huge right perirenal hematoma (). The cyst, which was not found at the previous episode, showed fluid retention with density of blood. This time, in renal arteriography, we demonstrated aneurismal bleeding at the periphery of the right renal artery (). We again emergently perfomed TAE. After TAE, retroperitoneal hemorrhage remitted and he recovered completely in almost the same time course as the previous episode.\nAt outpatient review after 3 months of his discharge, the CT scan showed the contraction and absorption of retroperitoneal hemorrhage. We could not demonstrate any other cause of perirenal hemorrhage including renal cell carcinoma. He continues hemodialysis without any bleeding sign or symptom. |
An otherwise healthy 76-year-old man was admitted as an emergency to the hospital with right hypochondral pain in keeping with cholecystitis. There was no evidence of jaundice or abnormal liver function tests and ultrasound investigation of the abdomen confirmed the presence of gallstones, with a thickened gallbladder wall but normal common bile duct diameter. Acute management involved analgesia, intravenous fluids and antibiotics (erythromycin, given his allergy to penicillin). The patient was submitted for a laparoscpic cholecystectomy when he failed to settle on conservative therapy. It was a technically difficult operation due to the degree of inflammation. The gallbladder was perforated and a subtotal cholecystectomy was performed leaving Hartmans Pouch. Stone clearance was not attempted, due to the absence of deranged liver function tests and the degree of inflammation/infection. The operative field and sub-phrenic spaces were thoroughly irrigated with 5 litres of saline and two wide bore drains were left.\nThe patient was discharged on the seventh postoperative day. He was readmitted on day 11 with general malaise, right upper quadrant abdominal pain, shortness of breath and oliguria. A CT scan revealed bilateral pleural effusions and a right subphrenic collection which, when radiologically drained, revealed frank pus. Further CT scan on the 25thday demonstrated two further collections in the left sub-phrenic and epigastric regions which were radiologically drained. The patient was eventually discharged on the 53rdday.\nOver the following two years, this gentleman had multiple admissions for a discharging sinus in his left chest wall. He underwent repeated radiological drainage with antibiotic cover. Cardiothoracic referral was made and three attempts were made to explore the sinus surgically. These explorations involved extensive excision of sinus tract and segmentectomy of the 10thrib. Laparoscopy and investigative laparotomy were not implemented given the pathology was identified using conventional radiological techniques.\nThree years post-surgery, the patient was admitted with a similar presentation. CT revealed the persistence of fluid lateral to the spleen []. A sinugram identified the sinus tract extending from the left chest wall, to the second part of the duodenum []. At laparotomy the fistula was dissected from the first part of the duodenum to the left upper quadrant requiring an awkward dissection of the stomach from the left lobe of the liver to reveal the fistulous track. Finally, the fistula remnant through the diaphragm and lateral chest wall was irrigated and sclerosed with Hydrogen Peroxide. After a rapid, uneventful recovery, the patient is currently well with no further symptoms. |
An 18 year old Sri Lankan Muslim female, presented with a 9 month history of colicky right iliac fossa pain and a 5 kg weight loss. She did not have diarrhoea, extra-intestinal manifestations of CD, and contact or past history of tuberculosis (TB). Clinical examination was normal.\nColonoscopy revealed an ulcerated ileocaecal valve and a terminal ileal stricture, while the colon itself was macroscopically normal. Biopsy of these lesions was reported as suggestive of CD. TB polymerase chain reaction (PCR) performed on the specimen was negative. Contrast-enhanced CT abdomen showed a segment of terminal ileum with concentric wall thickening. Mantoux test and Quantiferon gold test for TB were negative and chest X-ray was normal.\nShe was started on treatment for CD with a short course of oral steroids followed by azathioprine. However, she failed to tolerate azathioprine and subsequently 6-mercaptopurine (6-MP), and therefore was started on intravenous infliximab. Despite 3 dose of infliximab she failed to respond, and a follow up colonoscopy showed persistence of the ileal stricture. Therefore she was referred for surgical resection of the involved ileal segment. During the laparotomy, multiple terminal ileal and cecal strictures were noted and unexpectedly, multiple omental and peritoneal deposits were also seen. A limited right hemicolectomy including resection of the involved terminal ileum was performed.\nHistology of the resected specimen showed multiple coalescent caseating granulomata involving full thickness of the bowel wall and extending into the mesentery and pericolic fat, suggesting a diagnosis of ITB (Figs. , ). No evidence of dissemination beyond the intestine was found. She was started on anti-TB treatment (ATT), and 2 months later was free of abdominal pain and has regained 3 kg of weight. Three months after commencing ATT a repeat colonoscopy was performed. There were no macroscopic or microscopic changes in the neo-terminal ileum or residual colon. She completed 6 months of ATT and made a full recovery. |
A 58-year-old man was admitted to our Hospitalist Services for medical management of acute small bowel obstruction, which improved. Subsequently, the patient developed diffuse body aches, generalized weakness, and was noted to have a cough with blood-tinged sputum. The Pulmonary Service was consulted for evaluation of this new onset of hemoptysis. His past medical history was significant for underlying diabetes mellitus managed on oral hypoglycemics, end-stage renal disease on hemodialysis, systemic hypertension, Hepatitis C with associated vasculitis, for which he was on chronic oral steroid therapy. He denied any oral or intravenous substance abuse, tobacco smoking or alcohol use.\nOur initial physical assessment revealed normal vital signs without any obvious respiratory distress. His pulse oximetry showed 98% saturation on room air. His eyes were anicteric and neck was supple with no jugular venous distention. Chest auscultation was unremarkable for any extra cardiac or respiratory sounds. The abdominal examination was significant for mild distention with normal bowel sounds. The neurological exam was abnormal and revealed decreased muscle strength throughout, with intact sensations and normal deep tendon reflexes.\nA review of laboratory investigations revealed mild leukocytosis without leftward shift or bandemia. The serum chemistry showed an elevated serum creatinine level and a normal serum bicarbonate level as well as mildly elevated liver enzymes (AST- 110 IU/L, ALT- 42 IU/L and ALP-IU/L, a low albumin level, and normal bilirubin level). A room air arterial blood gas was obtained, which revealed no metabolic or respiratory abnormality. A chest radiograph showed a patchy infiltrate in the right lower lung. A computed tomogram (CT) of the chest, without contrast study, was obtained, which showed a 3.3 centimeter (cm) ×3.3 cm right hilar mass scattered bilateral pleural nodules, and bilateral pleural effusions []. A short interval repeat CT chest with contrast was done to evaluate this mass. This repeat study showed a significant increase in the hilar mass, 5.5 cm in maximum dimension and increasing bilateral pleural nodules. A 1.4 cm right lower paratracheal and 1.6 cm subcarinal lymph nodes were also noted [Figures and ].\nOn account of this rapid expansion of the hilar mass, an underlying infectious etiology was suspected and accordingly the patient was started on broad-spectrum antibiotics. A decision was made to perform EBUS-guided TBNA of the right hilar mass. However, the patient deteriorated rapidly and became progressively delirious over the next 24 hours requiring transfer to the Medical Intensive Care Unit (MICU) for acute respiratory failure, necessitating endotracheal intubation. While in the MICU, he underwent bedside EBUS-TBNA through an 8.0 mm endotracheal tube. The right hilar lymph node (station 10R) was sampled; transbronchial biopsies and bronchoalveolar lavage were performed at the right upper lobe. Rapid onsite cytology showed evidence of fungal elements. The patient was started on an intravenous antifungal agent. As part of the delirium workup, a spinal tap was also performed. The opening pressure was noted to be elevated at 28 cm of water. A cytopathology report from the right hilar lymph node showed a large burden of fungal organisms with thick capsules and tear drop budding consistent with the Cryptococcus genus [Figures and ]. Bronchoalveolar lavage (BAL) showed evidence that Cryptococcus and cerebrospinal fluid (CSF) cultures grew Cryptococcus neoformans. Serum cryptococcal antigen was elevated at a titer of 1:512.\nThe patient continued to rapidly deteriorate with multiorgan failure requiring vasopressor support, mechanical ventilation, and renal replacement therapy; and he expired after two weeks. |
The second case is a 7-year-old boy with right-sided submandibular lymphadenopathy who was transferred from a regional hospital to the Children's University Hospital where he remained for approximately two weeks. His body temperature during this period was normal; however, his parents reported that he had occasional fatigue and poor appetite. It was noted that he had signs of dysmorphism and mild growth delay. He was born at 40 weeks (mother's 5th pregnancy, 5th birth) with a weight of 3,680 g and a height of 53 cm; there were no complications during pregnancy/birth. He had had some mild viral infections after starting kindergarten and had received all his immunizations according to the immunization schedule. His parents did not detail any chronic diseases. He had two healthy brothers, one sister with a double kidney, and one sister that had died at 11 years of age due to some type of congenital pathology, possibly microcephaly (not enough information was available)—family tree shown in .\nUltrasound examination of the boy's cervical lymph nodes and abdominal cavity, thoracic CT scan, and abdominal MRI were performed, and they showed suspicions of lymphoproliferative disease. Consequently, lymph node biopsy, bone marrow aspiration, and trephine biopsy were performed; however, no evidence of lymphoproliferative disease was found, and the boy was discharged in a stable condition. At a follow-up visit, two months later, it was observed that the lymph node conglomerate in his neck area had increased in size. An MRI of the head and neck area was subsequently performed, and the following was reported: large abnormal formation in the cervical soft tissues on the right side, with extracranial parapharyngeal, carotid space with internal carotid artery (ACI), ACE overgrowth, intracranial proliferation through the foramen jugulare in the cerebral cortex with peripheral oedema, and 4th ventricle compression with initial 3rd and lateral ventricle extension. Trepanation, extirpation, and biopsy of the mass were performed, and high-grade B-cell lymphoproliferation with Burkitt lymphoma-specific morphology and immunophenotype was confirmed. Chemotherapy was initiated according to B-NHL-2004 protocol for the R3 treatment group (Burkitt lymphoma IV, CNS +) with prophase + chemotherapy blocks. During chemotherapy, the boy had several very serious episodes of infection with sepsis and bilateral polysegmental lung damage. He was also found to be EBV DNA and IgG and IgM positive.\nAfter chemotherapy, the boy reached complete remission. However, persistent hypogammaglobulinaemia was noted (shown in ), and consequently intravenous immunoglobulin was started.\nAs primary immunodeficiency was suspected, the boy was consulted by a geneticist. The Sanger sequencing analysis for the coding exons of the SH2D1A gene (NM_002351.5) was conducted. A hemizygous mutation was detected in the SH2D1A gene, NM_002351.4:c.5A > G, p.Asp2Gly, rs1556619319. It was classified as likely pathogenic, thus confirming a diagnosis of XLP. The classification was based on ACMG guidelines, fulfilling criteria PM1 (variant located in the functional domain); PM2 (variant not found in gnomAD exomes or genomes); PP2 (missense variant is the pathogenic mechanism in the SH2D1A gene); PP3 (multiple in silico tools suggesting pathogenicity); PP5 (reputable source, ClinVar classifies the variant as likely pathogenic) []. At present, the boy receives intravenous immunoglobulin regularly, and his condition is stable. Genetic testing for other family members were not performed. |
A 13-year-old boy with SHFM was admitted to our department with his fourth episode of purulent meningitis. His family history was significant: he was born as the third child in the family; his sister is alive; his brother, who had a congenital heart malformation and Down’s syndrome, died as an infant. His two cousins had Say-Meyer syndrome. His mother had contact with chemical disinfectants during the first two months of her pregnancy. The boy was born in the 38th week of pregnancy with Apgar scale 9 congenital malformation of all four limbs. When he was 1 year old, his family recognized that he had hearing problems but they did not seek any professional help. In 2006, his chromosome analysis confirmed incorrect karyotype 46, XY, t(7:12)(q21.2;q21.3). The boy had reciprocal translocation between chromosome pairs 7 and 12.***\nThe first episode of invasive streptococcal disease was diagnosed in 2008 when the patient was 6 years old. It was a complication of an inflammation of his right ear. After that, the boy developed two further episodes of meningitis: in 2011, without confirmed aetiology, and in 2012, again streptococcal. In 2011, due to his recurrent infections of the central nervous system, the boy had a consultation with a neurosurgeon and underwent an magnetic resonance imaging (MRI) of the brain and lumbar column, but the radiologist did not find any abnormalities of these structures.\nIn April 2015, the boy started to complain of pain in his left ear. After three hours the laryngologist confirmed the presence of an inflammation in his left ear and prescribed an oral antibiotic. The pain was so severe that the mother took the child to the hospital two hours later. At the time of admission the general status of the child was good: he presented neither a fever nor pathological neurological symptoms. His leucocyte count was 18.7 G/uL; C-reactive protein– 0.39 mg/dL; procalcitonin – 43.07 ng/mL. The patient received a first dosage of intravenous ceftriaxone. Three hours later the general status of the child had worsened: he started to vomit and complained of a headache. He presented positive meningeal signs. His leucocyte count increased to 27.95 G/ul; C-reactive protein – 12.7 mg/dL. A lumbar puncture was performed and confirmed the diagnosis of purulent meningitis (leu – 137 cells/mm3 with 96 % of neutrophils; protein – 3.77 g/L; glucose – 0.01 mmol/L). Therapy with ceftriaxone and vancomycin was started. The next morning the child was sent to actual department. At the time of admission the boy presented severe hyperaesthesia of the skin, neck stiffness of 7 cm and a positive Kernig’s sign. Physical examination of the body revealed median clefts of both hands and feet (Figs. and ). Other systemic examinations were normal except for a mild systemic murmur in the heart apex. The child presented appropriate-to-age development, but because he was deaf and without speech he used only sign language. The laboratory findings were worse: the leucocyte count was 38.26 G/uL with 98 % of neutrophils; C-reactive protein – 40.20 mg/dL; procalcitonin – 43.89 ng/mL. The blood and CSF culture confirmed penicillin-resistant Streptococcus pneumoniae with low sensitivity to vancomycin infection. The patient received high-intensity treatment with cefotaxym (107 mg/kg/day), vancomycin (4 × 10 mg/kg/day) and meropenem (3 × 1 g/day). Over the next few days we observed a slow improvement of the patient’s general status. His therapy was complicated by two episodes of generalized tonic-clonic seizures, which were observed on the 10th and 16th days of treatment. Post-infection epilepsy was diagnosed and levetiracetam was induced.\nDuring his hospitalization we tried to find the reason for his recurrent pneumococcal meningitis. Immunological analysis did not present any abnormalities, and the levels of immunoglobulins G, A and M were correct. The cytometric analysis of the leucocytes presented increased absolute values of lymphocytes CD4-T, CD8-T and B with normal percentages. The expression of adhesive molecules (CD11a, CD11b, CD11c, CD18) was normal. The high-resolution, computer tomography (CT) (axial, coronal, and sagittal planes) of both temporal bones was performed (128-layer Somatom Definition AS apparatus, Siemens, Germany). This indicated: preserved basal turns and absence of the apical turns of the both cochleas, vestibulums wider than usual (Figs. and ). The clinical suggestion of inner ear abnormality was confirmed – Mondini dysplasia. The malformation was also confirmed in magnetic resonance imagination (3-Tesla MAGNETOM Spectra scanner, Siemens Healthcare, Erlangen/Germany) (Fig. ).\nTwo months after the 4th episode the child had the left subtotal petrosectomy performed to protect him against next recurrent bacterial meningitis. A postauricular S-shaped skin incision was done from the temporal region to the mastoid tip. Mastoid periosteal flap was created and external auditory canal was closed as a “blind sac”. Complete mastoidoepitympanectomy was performed with skeletonisation of the dura, sigmoid sinus and facial nerve. The mastoid cells (retrosigmoid, retrofacial, antral, retrolabyrinthine, supra-labyrinthine, infralabyrinthine, supratubal and pericarotid) were all exenterated. The mucosa of the bony Eustachian tube was removed with the diamond burr and coagulated with bipolar forceps. The Eustachian tube was obliterated with fibrin patch and bone wax. Stapes superstructure was removed. The middle ear cavity was obliterated with abdominal fat harvested from the lower quadrant of the abdomen. Both wounds: abdominal and parietal were closed in layers using 3.0 vicryl subcutaneously and monocryl 3.0 to the skin. The patient’s post-operative course was unremarkable. |
A 47-year-old woman with a 24-day history of hypertension followed by a headache was referred to our neurosurgery department. Three days prior, she had been started on oral antihypertensive therapy with azilsartan 20 mg a day at a local hospital, and the patient's headache improved gradually with the antihypertensive therapy. On arrival, her blood pressure was 197/106 mmHg. No focal neurological abnormality was noted. Fluid-attenuated inversion recovery (FLAIR) and T2-weighted MR imaging revealed high-intensity lesions in the pons, bilateral thalamus, bilateral basal ganglia, and periventricular white matter. There was no evidence of SAH. Diffusion-weighted MR imaging detected no signal abnormalities in these regions, except in the bilateral basal ganglia, which exhibited low intensity []. MR angiography revealed a saccular aneurysm located in the right internal carotid artery []. Arterial vasospasm was not observed in the anterior or posterior circulation. She was not admitted to our hospital at this time because MR imaging did not reveal SAH or acute ischemic lesions, and neurological examination revealed no deficits. Two days later, she experienced severe headache and was admitted to our hospital. On arrival, her blood pressure was 274/133 mmHg. She was subsequently started on intravenous nicardipine. Her arterial blood pressure was maintained between 115/63 and 141/76 mmHg. The patient was alert and fully oriented without focal neurological deficits. Initial computed tomography failed to diagnose SAH. FLAIR revealed slightly high-density lesions in the right side of the sylvian cistern and basal cistern consistent with SAH as well as high-intensity lesions in the pons, bilateral thalamus, bilateral basal ganglia, and periventricular white matter []. Two days after the onset of SAH, she successfully underwent neck clipping of an aneurysm via a craniotomy under general anesthesia. Intraoperatively, we did not identify local hemosiderin staining of the pial surface immediately adjacent to the aneurysm dome suggestive of a previous aneurysm rupture. Postoperatively, the patient experienced no cerebral ischemic events. Her systolic and diastolic blood pressure were controlled with intravenous nicardipine and/or oral antihypertensive agents to <150 and 100 mmHg, respectively. Follow-up MR imaging performed 2 weeks later revealed diminution of the lesions []. |
A 36-year-old woman, primigravida, consulted our hospital for investigation of a cystic hepatic lesion in August 2013. The patient had no history of endometriosis. Half a year previously, the patient had presented with severe right quadrant pain in the midnight which only lasted for 3 h just before menstruation. An upper abdominal ultrasound showed a 6-cm lesion within the left lobe of the liver. The patient was diagnosed with a hepatic cystic mass in segment III of the left lobe. A laparotomy had been considered and postponed since the patient had managed to cope with her symptoms. The patient refused further treatment at that moment. The patient was not currently undergoing menopause. The patient had no exposure to hepatotoxic drugs, estrogens, progestins, or oral contraceptives. There were no other significant symptoms. On physical examination, no definite abdominal mass lesion was palpable and no lymphadenopathy was noted. Complete blood count was within normal range as were the liver function tests. Serological tests for hepatitis B surface antigen and anti-hepatitis C virus antibodies were negative. The tumor markers (carcino-embryonic antigen, carbohydrate antigen 19-9, alpha-fetal protein, carbohydrate antigen 125) were normal, as was the routine lab work. A preoperative computed tomography scan showed a well-circumscribed cystic lesion of 6.5 × 6.0 cm located in segment III (Figure ). The wall appeared thick with complex septate. A preoperative tentative diagnosis included liver cystadenoma or liver cystadenocarcinoma.\nAn exploratory operation was performed, which revealed a large cystic tumor occupying segment III of the liver (Figure ). There was no evidence of metastases. Biopsies were taken from the cyst wall, and the intraoperative frozen section histology suggested a diagnosis of hepatic endometriosis. The endometriosis was completely removed by a pericystectomy. An abdominal cavity exploration revealed no other pathologic events. The pelvis was examined and no evidence of endometriosis was found. We did not detect any other abnormalities during the operation. The patient made an uneventful recovery and was discharged on the ninth postoperative day. After 3 months of follow-up, the patient is asymptomatic with no evidence of recurrent disease.\nHistopathology analysis revealed a lobulated cyst with adjacent normal hepatic parenchyma. The cyst wall was partially composed of endometrial glandular and stromal elements (Figure a), characteristic of endometriosis. Immunostaining of the stromal cell and epithelial cells expressed strong coloring for estrogen and progesterone receptor (Figure b,c). This was further confirmed by positive immunostaining of CD10, CK7, as well as HepPar-1, which proved the epithelial origin of the cyst (Figure d,e,f). Since no atypical cells were detected, we concluded the diagnosis to be benign intrahepatic endometriosis. |
A 10-year-old girl visited our affiliated hospital, complaining of pudendal deformity. The patient was born at 39 weeks of gestation by normal delivery as the second child, and the birth weight was 3,144 g. There was no disorder in the course of pregnancy in her mother. Intake of androgenic medicine or the disorders of ovaries and uterus was not observed by a periodic medical examination. No pudendal deformity was clearly observed at birth, but lateral asymmetry of the pudendal region was noticed at about 4 years old. A child care worker pointed out that she pressed her heel to the crotch while sitting on her folded legs. After 5 years old, her mother confirmed that her clitoral hood clearly hypertrophied. After entering primary school, a teacher in charge pointed out that she pressed her crotch to a chair or bar, and the mother told her to stop it, but she repeated this behavior every day. When she strongly wanted to be absent from an overnight school trip at 9 years old, the mother brought her to the Pediatric Department. Various tests were performed suspecting disorders of sex development (DSD). On the first visit, the height was 132 cm and the body weight was 26 kg. The clitoral hood was enlarged. The appearance was similar to the vulva in children with congenital adrenal hyperplasia, and the clitoris size was 8 x 5 mm. Labial fusion or adhesion was not detected, and the urinary tract and vagina were open at the normal positions. No masculinization, such as acne and polytrichosis, was noted (). Intake of androgenic medicine or the disorders of prepuce was not observed. In the blood test, the sex chromosome was 46, XX. The blood count, blood chemistry, and hormonal test were normal (). On abdominal ultrasonography, the uterus and ovaries were present. Abdominal CT and MRI examinations showed no tumorous lesion.\nBased on the above examination and test findings, DSD was considered negative. The patient was diagnosed with clitoral hood enlargement and referred to our department to undergo clitoral hood reduction. For surgery, a longitudinal incision was designed for the dorsal side in order to resect the clitoral hood by cut and try. In the clitoral region, the clitoral hood and corpus cavernosum were dissected through an inverted V-shape incision. The volume of the exposed corpus cavernosum clitoridis was reduced while conserving the neurovascular bundle, following the Marberger method []. The clitoral hood was resected into a triangle shape and used for labial formation. On histopathological examination, lymphedema and venous tasis in a grade consistent with the influence of foreskin excision were observed. No abnormality was noted in the corpus cavernosum. Her postoperative course was uneventful. As of 10 months after surgery, favorable improvement of the appearance was noted (). |
A 44-year-old Japanese man presented to the Maxillofacial Surgery Clinic at the Tokyo Medical and Dental University, Tokyo, Japan, with reports of a hard mass and dull pain in the left mandible. His medical history was generally unremarkable, though his mandible had been accidentally smashed against his young son’s head at the age of 40. No fracture was detected at the time, and no treatment was received.\nClinical examination revealed slight swelling in the left lower molar region and swelling of the left mandible. There was no disturbance of sensitivity in the left lower lip or chin, and he could move his lips normally. Radiographic examination showed large, irregular, multilocular radiolucency of the left area of mandible extending to the premolar and molar area, with no evidence of any impacted tooth (Fig. ). A computed tomography (CT) image revealed a 45 × 30 × 30 mm multilocular cystic mass in the mandible (Fig. ). An axial CT image showed thinning or partly resorption site of the buccal cortical plate. The coronal section images of the CT scan showed the mass lesion displacing the mandibular canal downward, near the inferior border. The roots of the adjacent teeth were resorbed, changing their shapes (Fig. ). The location of the teeth was not changed.\nThe initial clinical impression was an ameloblastoma, myxoma, keratocystic odontogenic tumor or another tumor type lesion.\nThe lesion was removed surgically with the buccal cortical bone and buccal mucosa in contact with the mass of the tumor. The mass fully excised intraorally under general anesthesia, and the inferior alveolar nerve in contact with the mass was preserved.\nThe resected specimen of the primary tumor was a soft, fragile, yellowish-white mass. Microscopically, tumor tissues were composed of plexiform or cord-like nests of tumor cells with round to oval nuclei and eosinophilic cytoplasm in a matrix with prominent myxoid change (Fig. ). On the other hand, the component of solid sheets of tumor cells was limited (Fig. ). In some areas, tumor cells surrounded small blood vessels (Fig. ). Moreover, tumor cells manifested a uniform cell morphology, poor dysplasia, and inconspicuous mitosis.\nImmunohistochemical staining was positive for vimentin (Fig. ), muscle-specific actin/HHF35 (Fig. ), and Calponin (Fig. ), a protein responsible for binding the actin-binding protein. A hairline-shaped area of positive staining for type IV collagen surrounding the tumor cells was also observed (Fig. ). VEGF and D2–40 was also focally positive in tumor cells. In contrast, staining for alpha-SMA, cytokeratin (AE1/AE3), cytokeratin (CAM5.2), CK19, CD34, CD68, p63, S-100, Factor VIII, and desmin, CD56, chromogranin A, synaptophysin was all negative in the tumor cells. Moreover, CD31 was also negative, but we observed many blood cells intervened between tumor nests. The Ki-67 labeling index was almost 1%. No necrosis or tumor invasion into the neurovascular channel was observed. The primary tumor was diagnosed as a glomus tumor based on these findings.\nThe patient remained symptom-free and manifested no signs of recurrence. However, a recurrent tumor was detected in a panoramic radiograph during an 8-year follow-up. The panoramic radiographs taken earlier, after excision of the primary tumor, showed normal healing process, bone regrowth, and increased radiopacity. The follow-up panoramic radiograph 8 years later depicted the recurrence as a radiolucent expansion in the lower area of the mandible. A CT exam showed an expanding lesion exiting the lower site of the mandible and a thinning buccal cortical bone in contact with the tumor (Fig. , ). In magnetic resonance imaging (MRI), the recurrent lesion showed a lower or compatible signal intensity compared to the muscle in T1-weighted images and a low-to-high inhomogeneous signal intensity in T2-weighted images. The apparent diffusion coefficient in the glomus tumor area was 2.0 × 10− 3 mm2/sec, suggesting a low cellular density (Fig. -).\nThe recurrent tumor mass and related teeth were removed under general anesthesia, and the bone surface was shaved. The recurrent tumor had the same microscopic, morphologic, and immunohistochemical features as the primary tumor (Fig. ). The tumor invaded the medullary cavity of the mandible, involving spongiosa and resorbing compressively the cortex bone around tumor.\nThe patient has no symptoms of recurrence as of this writing, 2 years after the second operation, and will be followed for the long term to promptly detect any signs of new tumor growth.\nThe patient is satisfied that the glomus tumor was regulated without resecting the mandibular region. He is pleased that there was no functional deterioration that he was worried about before treatment and there was no aesthetic damage.\nTimeline of patient diagnosis and treatment.\n1. 4 years before the first visit, the patient had been smashed against his young son’s head.\n2. At first visit, the symptom was slight swelling of the left mandible. There was no disturbance of sensitivity. The examinations were performed with X-lay and CT.\n3. First surgery was performed and started clinical follow-up after the surgery.\n4. 8 years later from first surgery, the recurrence was found by panoramic radiograph. The examinations were performed with CT and MR.\n5. Second surgery was performed, and it was passed 2 years with no recurrence from second surgery. |
We present the case of a sixty-two year old gentleman who presented to our emergency department complaining of a 12 hour history of abdominal pain and distension. This was an acute onset central, crampy abdominal pain. He had associated anorexia but no vomiting. He denied any history of weight loss. His laboratory investigations were within normal limits. He had a coronary artery bypass graft performed four years previously and also had haemochromatosis.Computerized tomography of the abdomen demonstrated diffuse thickening of small bowel loops with a doughnut configuration. These findings were suggestive of small bowel intussusception, measuring 12 cm in length. There was a fat density at the centre of the intussusception radiologically consistent with a lipoma (Figure ).He underwent a diagnostic laparoscopy which revealed an intussusception of the proximal jejunum. This was managed intra-operatively by manipulation and laparoscopic reduction without resection. Macroscopically there was no visible pathology in the form of a mass or lesion. Post-operatively the patient did well and was discharged on day three. An out-patient MRI of the small bowel was performed which showed a recurrent dynamic focus of intussusception of the jejunum with dilatation of proximal small bowel loops, although the patient remained asymptomatic (Figure ).Two weeks after his initial surgery this man represented to the emergency department with vomiting and abdominal distension. Clinical examination was consistent with small bowel obstruction. He underwent a laparotomy which found recurrent jejuno-jejunal intussusception (Figure ). This was excised and a primary end-to-end anastomosis was performed. The patient had an uncomplicated post-operative course and remains well.Histological examination of the resected specimen revealed the presence of an inflammatory fibroid polyp (Vanek’s tumour), which acted as the pathologic lead point for intussusception (Figure ). The specimen was a 330 mm long segment of small bowel displaying complete invagination of a 145 mm long segment of it. There was a 42×25mm polypoid lesion at the lead point showing focal surface ulceration. The proximal 150 mm long segment was dilated (perimeter 105 mm). The tumour invaginated into a jejunal segment which had already invaginated. This phenomenon is known as double invagination (Figure ).The tumour invaginated into a jejunal segment which had already invaginated (double invagination). It was a relatively well circumscribed ulcerated submucosal lesion showing variable cellularity, formed by spindle and stellate cells set in a fibro-myxoid background which is scattered by inflammatory cells. The inflammatory infiltrate was composed of mainly eosinophil leukocytes mixed with less number of histiocytes, plasma cells, neutrophil leukocytes and lymphocytes. Among the spindle cells small and medium sized blood vessels were also present containing thickened walled vessels too. Towards the ulcerated surface haemorrhage, granulation tissue pattern and more brisk inflammatory reaction were noted. There was no evidence of dysplasia or malignancy (Figures and ). The immunohistochemical study reveals no staining with Desmin, ER, PR, Synaptophysin and S100. The spindle and stellate cells are positive for Vimentin but negative for CD34, CD117 and SMA. |
A 77-year-old man fell from a height of approximately 3 m into the gutter and struck his pelvis. He was found immobile and was rushed to our hospital.\nMedical history: none.\nPhysical findings: Consciousness was clear, blood pressure was 100/70 mmHg, heart rate was 127/min, SpO2 was 97%, and no injury was noted on either the head or abdomen.\nAs shown in Table , blood work (day 1) showed Hb12.7 g/dl, but no increase in cell ectopic enzymes was observed.\nCT findings on day 1 revealed a right pelvic fracture and a haematoma in the pelvic extraperitoneal space (Fig. ). Neither free air nor ascites was observed in the abdominal cavity, and no damage was found to the intestinal tract. CT findings on day 2 showed the haematoma in the pelvic extraperitoneal space had increased in size from the previous day, and active bleeding was suspected. Blood work from day 2 demonstrated progression of Hb10.3 and anaemia, even after transfusion of 4 units of red blood cells (Table ). For vitals, blood pressure was 78/37 mmHg, and heart rate was 144 bpm, indicating shock, and active bleeding due to the pelvic fracture was suspected. Therefore, transcatheter arterial embolization (TAE) was adopted. Blood flow in the pelvis is supplied from both sides, and the presence of traffic branches may require TAE of the internal iliac arteries on both sides depending on the situation. Angiography identified the responsible vessel, which had embolized the bilateral internal iliac arteries, and confirmed haemostasis.\nCT findings on day 3 revealed thickening of the ascending colon wall, intramural emphysema, and a slight amount of air in the mesenteric vein. In addition, a small amount of ascites was observed near the ascending colon. There was no problem with the contrast effect of the small intestine, and the sigmoid colon was dilated, but there were no obvious necrotic findings. (Fig. ).\nBlood work on day 3 revealed elevated levels of cytopathic enzymes: AST 2363 IU/l, ALT 2233 IU/l, LDH 3181 IU/l, and CPK 4122 IU/l. Based these findings, we rendered a diagnosis of necrosis of the ascending colon and performed emergency surgery.\nIntraoperatively, necrosis was observed in the serosa of the ascending, transverse, and sigmoid colon. Subtotal resection was performed from the ileocecal region to the sigmoid colon. After confirming that there is no necrosis in the mucosa of cut end of small intestine, ileostomy was constructed. Although a slight ischaemic change such as edema and mild redness was observed in the serosa of the entire small intestine, we decided to preserve the entire intestine.\nExcised specimen findings showed scattered necrosis in the ascending, transverse, and sigmoid colon (Fig. ).\nPathological findings indicated necrosis throughout all layers of the intestinal wall in the ascending, transverse, and sigmoid colon.\nPostoperatively, the patient began drinking water 2 days after surgery. However, 6 days after surgery, CT revealed a dilated small intestine, which we diagnosed as paralytic ileus. Subsequently, the paralytic ileus did not improve, and melena was observed 10 days after surgery. CT performed 10 days after surgery revealed extensive small bowel dilation and niveau. In addition, wall thickening and a decrease in contrast effect were observed in partial small intestines (Fig. ).\nWe diagnosed haemorrhage due to necrosis of the residual small intestine. However, due to the postoperative total colectomy and deterioration of general condition, it was decided that intestinal resection was impossible, and treatment with blood transfusion and haemostatic agents was planned. At that time, the colour of stoma did not change greatly and there were no necrosis sites.\nThe patient's condition gradually worsened even after treatment, and over time, he developed liver and renal dysfunction and died 16 days after surgery.\nNOMI is a disorder that causes irreversible ischaemia in the intestine, resulting in intestinal necrosis, despite the absence of organic obstruction in the mesenteric artery trunk. This phenomenon was first reported by Heer [] and includes the following characteristics: (1) no obstruction in the mesenteric arteries or veins governed by intestinal necrosis, (2) segmental discontinuity of intestinal ischaemia and necrosis, and (3) pathological evidence of intestinal haemorrhage and necrosis. We define NOMI as satisfying these conditions. From 12 to 25% of acute intestinal ischaemia is thought to be due to NOMI in Europe and America [, ]. Various triggers increase hypoxia of the intestinal tract tissue, reduce cardiac output, and decrease circulating blood volume. As a result, the sympathetic nerves in the peripheral blood vessels of the mesenteric artery overreact, causing vasospasms and intestinal ischaemia. Since these spasms occur at random, the ischaemic area is sporadic. Fogaty [] suggests that severe congestive heart failure, digitalis intoxication, and blood concentration are important factors affecting this disorder and that dehydration is significantly related to vasopressin due to low cardiac output, haemorrhage, and shock. He asserts that vasopressin and angiotensin increase in the blood due to low cardiac output, haemorrhage, shock, etc., causing catecholamine-induced spasms of the mesenteric artery and resulting in NOMI. In addition, the general risk factors for NOMI are increased age, heart disease, arrhythmia, cerebrovascular disease, diabetes, burns, dialysis, dehydration, haemorrhage, and pancreatitis [–]. In any case, the basis of the pathology is considered a decrease in circulating blood volume. Pelvic fractures generally cause excessive bleeding in trauma, anywhere from 1000 to 4000 ml []. In our patient, bleeding from a pelvic fracture caused a rapid decrease in circulating blood volume, and at one point, the patient was in shock. TAE was performed for active bleeding, and NOMI developed the next day, although haemostasis was attained. When a pelvic fracture with a large volume of blood loss occurs, the risk of NOMI onset should not be ignored.\nNOMI has no specific symptoms. Some of the many non-specific symptoms include abdominal pain, vomiting, abdominal distension, and melena, but these symptoms are often mild during onset. In particular, it is difficult to identify these symptoms in cases of sedation, analgesia, and consciousness disorder. In these cases, diagnosis is likely to be delayed. Similarly, in our case, it was difficult to diagnose intestinal ischaemia based on clinical symptoms, because analgesics are used for pelvic fractures, and systemic contusions are caused by trauma. Blood work showed an increase in deviant enzymes, but the diagnosis was made even more difficult after TAE was performed for active bleeding. In our case, follow-up CT was performed to identify active bleeding, but it is important to consider CT examination as necessary, keeping in mind that NOMI is caused by bleeding from trauma.\nWhen NOMI is considered as a diagnosis, one of the treatments is injection of a vasodilator into the responsible blood vessel using angiography [], but this method is limited by equipment availability and the specific situation. In our case, a vasodilator could not be used due to instability of vital signs from bleeding. If intestinal necrosis is already suspected, as in our case, immediate surgical intervention is needed. At the time of surgery, it is important not only to remove the necrotic intestine but also to evaluate the viability of the remaining intestine. Intestinal ischaemia can be extensive postoperatively, and care must be taken in determining the extent of resection. When deciding the extent of resection, there is a method of observing changes in the colour tone of the mucous membrane using an intraoperative lower gastrointestinal endoscope and blood flow evaluation by fluorescein fluorescence [, ]. However, these methods may be difficult depending on equipment availability and the situation. In our case, we found clear discontinuous necrosis in the serosa of the caecum, transverse colon, and sigmoid colon. Therefore, we decided to remove the intestinal tract from the ascending colon to the sigmoid colon. Since only diffuse and mild oedema and redness in the small intestine were noted and no obvious necrosis was observed, we decided to preserve it and constructed an artificial anus in the terminal ileum. However, the small intestines became necrotic after a few days, eventually resulting in widespread intestinal necrosis. Of note, the prognosis cannot be judged only by the surgical findings. If long-term dilatation of the small intestine is observed after heavy bleeding, it is important to consider intestinal necrosis rather than assuming it as paralytic ileus. In that case, it is necessary to comprehensively judge clinical symptoms, vital signs, and imaging findings, but the diagnosis is very difficult. Importantly, pelvic fractures are typically accompanied by high blood loss, so even if active bleeding subsides and vital stability is obtained, NOMI may develop over time. |
A 77-year-old woman had been diagnosed at the age of 15 years with NS which had started from the left side of her ear and extended to her chest. Her case was followed up after she had presented at a dermatologic clinic. She was referred to our hospital with a 3-year history of black macules on the left ear. She had undergone surgical excision for BCC on the left cheek 2 years before. A physical examination showed yellowish plaques around the left side of the neck and small, numerous yellow papules in the NS. A black macule of 2 mm in diameter was observed on the left auricle (fig. , arrowhead). A dermoscopic examination revealed multiple blue-gray ovoid nests and whitish veils, but arborizing vessels were not found (fig. ). A surgical specimen showed that tumor cells were proliferating from the epidermis to the upper dermis and were arranged in a palisading pattern at the periphery with basal melanosis. Deposits of mucin and clefts were observed in the surrounding area with dilated vessels in the papillary dermis (fig. ). Based on these histological features, the macule was diagnosed as BCC. After surgical removal, no recurrence was observed.\nThree months after the surgery, a small black macule appeared on the left earlobe which slowly enlarged. A physical examination indicated the presence of a small black macule with a diameter of 1 mm on the left earlobe (fig. , arrowhead). A dermoscopic examination revealed a round, homogeneous, brownish structure without arborizing vessels (fig. ). We performed an excisional biopsy. Histopathologically, the surgical specimen showed that the tumor cells were proliferating in the dermis, presented as multiple different shapes, were either clear or basophilic, and were arranged in a partial palisading pattern. The tumor cells also resembled follicular germinative cells without keratinizing cysts, and islands of tumor cells were present as hair bulbs surrounding fibrous stroma (fig. ). The macule was given the final diagnosis of trichoblastoma. |
A 28-year-old male patient presented to the skin out-patient department with history of a huge progressively increasing swelling over the back of the scalp [] and multiple black nodules at various parts of the body. According to patient same kind of lesions were present over the trunk at birth, which were surgically removed when he was 4-year-old. The lesion over the scalp started at the age of 6-7 years and the truncal lesions developed gradually since then. On examination, there was a single bosselated irregular non-tender skin colored swelling of 15 cm × 20 cm with deep folds over the occipital region extending toward the nape of the neck. There were certain areas of depigmentation [ along with blue streaks over the swelling. The surface of the skin was hairless, the skin over the swelling was non-pinchable and the swelling was immobile. There was no skin erosion, dermatitis, infection, odor or tenderness.\nThere were multiple hyperpigmented nodules distributed over different areas of the body such as mandible, flanks, abdomen [], front and back of both thighs, right knee joint, lower leg [], right sole and over volar aspect of first and second finger of the left hand []. The size of the nodules ranged from 0.5 cm × 1 cm to 1.5 cm × 2.5 cm. The nodules were blackish brown in color surface was smooth and hairless. They were asymptomatic and non-tender on palpation. Nails and mucous membrane examination were within normal limits. He had normal intelligence (the full-scale intelligence quotient was 97) and he had no psychiatric problem. Examination of the neurological, ophthalmological and other systems was normal. Laboratory results, including venereal disease research laboratory test, serology for human immunodeficiency virus, growth hormone and thyroid function, were all negative or normal. A punch biopsy from the scalp lesion and an excision biopsy from the nodule over the body was performed. Section from the specimen showed multiple nests and cords of nevus cells in the upper dermis, which contained a moderate amount of melanin []. There was no cellular atypia. Section from the body lesion showed deeply pigmented dendritic melanocytes in addition to nests and fascicles of spindle shaped cells with abundant pale cytoplasm containing little melanin [Figures and ]. The spindle cells were arranged in a storiform pattern. Based on the clinical feature and histology it was diagnosed as a case of congenital melanocytic nevus with cellular blue nevi. |
A 3-month-old male was seen in our pediatric oncology department because of a growing parotid gland mass. The mass was congenital and was followed by the pediatric oncology team with a prediagnosis of hemangioma. The patient’s magnetic resonance imaging (MRI) scan showed an infantile hemangioma in the proliferative phase filling the parapharyngeal space ().\nThe child then received propranolol therapy, but despite this treatment the mass continued to grow, and the child was referred to our department at the age of 10 months. The otorhinolaryngological examination revealed an 8×7-cm mass filling the left parotid region, with normal facial nerve motor functions (). Histopathological diagnosis of the mass was provided by an incisional biopsy as sialolipoma.\nWe planned to perform total excision of the mass with a transparotid approach. The facial nerve was monitored intra-operatively, and surgery was performed under magnification using surgical loops. A standard modified Blair incision was used, flaps were raised (), then the facial nerve was identified in a standard fashion using a tragal pointer and posterior belly of the digastric muscle as landmarks.\nThe main trunk of the facial nerve was found to be unusually elongated before pes anserinus. A soft lobular mass was encountered. A tumor involving the superficial parotid lobe was first dissected, then the deep lobe of the parotid gland and portion of the tumor filling the parapharyngeal space were gently dissected under the facial nerve (). A Jackson-Pratt drain was inserted into the wound after the excision of the tumor.\nOn macroscopic examination, the mass was found to be a lipomatous specimen, 9×8×4 cm in size. The cut surface was consistent with the appearance of a lipoma. Histopathologic examination confirmed the diagnosis of a sialolipoma. The lesion was composed of mature adipose tissue mixed with acinar, ductal structures of a normal salivary gland ().\nThe postoperative period was uneventful. The patient’s facial nerve motor functions were well preserved, and he was discharged on the third postoperative day. The patient is still followed, and has no signs of recurrence in his 24th postoperative month. |
The patient was a 58-year-old man, on treatment for hypertension, who was admitted to a vascular surgery service because of an incidental finding of two aneurysms of the SMA, identified during preoperative imaging exams preparatory to repair of an incisional hernia. The patient had no abdominal symptoms and on physical examination his abdomen was flaccid and painless and with a pulsating mobile mass in the epigastrium. Abdominal ultrasonography indicated a partially thrombosed saccular aneurysm in the retroperitoneal space, with no communication with the aorta. Multislice angiotomography revealed two aneurysms of the SMA, a proximal one measuring 5.9 × 5.2 × 5.0 cm and a distal one measuring 5.3 × 3.5 × 3.2 cm ( \n ). Since multiple collateral branches emerged from both aneurysm bodies, which meant the endovascular treatment would have involved a risk of damaging the intestinal blood supply, the decision was taken to perform open surgical repair. During the operation, by explorative laparotomy, access to the retroperitoneal space was achieved after performing the Cattell-Braasch maneuver, with medial displacement of the ascending colon and part of the transverse colon, exposing the infrarenal aorta and its branches. This revealed two true aneurysms of the SMA, the larger of which was around 3 cm from the arterial ostium and the smaller approximately 2 cm from the end of the first ( ). It was also possible to observe collateral branches (right colic, ileocolic, jejunal artery, and ileal arteries) projecting from the bodies of these aneurysms. A mesenteric-mesenteric, end-to-end bypass was therefore constructed, using a dacron prosthetic graft, excluding both aneurysms but preserving branches distal of the proximal aneurysm. It was decided to ligate and resect the aneurysms – sending specimens for cultures – and their lumens were opened, revealing large quantities of intraluminal thrombi ( ). Inspection of the abdominal cavity found the intestines to be viable and free from any sign of injury. During the postoperative period, the patient suffered gastrointestinal atony, but responded satisfactorily to prolonged conservative measures. Additionally, on the fifth day after the operation, a control computed tomography revealed signs of hematoma in the hepatorenal recess, managed conservatively to resolution. The patient was discharged from hospital to outpatients follow-up in good clinical condition, 18 days after the operation ( ). There was no bacterial growth in the culture of the aneurysm segment. |
A three-month-old female infant, born via normal delivery, was examined for a mass involving the left upper eyelid. Her parents stated that her eyelids appeared normal at birth but at two weeks of age a mass appeared in the infants left upper eyelid which rapidly enlarged over a month.\nOn examination, the fixation pattern was central, steady and maintained in the right eye, and she was able to follow light through a slit-like opening of the left palpebral fissure. There was a 3.5 × 3.00 cm elevated mass with an overlying red spot, involving the medial two third of the left upper eyelid, that caused a marked ptosis in that eyelid []. Ocular motility was full in both eyes with limited elevation in the left eye. There was no afferent pupillary defect. The color of the iris was dark brown in both eyes, and the fundus was normal in both eyes. Cycloplegic refraction of the right eye was +1.50 + 1.50 × 90° and for the left eye was + 5.00 –8.00 × 40°.\nComputed axial tomography with contrast showed a large hyperdense extraconal lesion located at the superomedial aspect of the left orbit causing inferolateral displacement of the globe. The rest of the examination was within normal limits and a diagnosis of infantile capillary hemangioma was made. Because of the high risk of developing amblyopia from visual deprivation and ptosis, the infant was started on a five-week course of tapering oral prednisone starting at a dose of 5 mg, twice a day for two weeks while being monitored by a pediatrician. At the end of treatment, there was some regression in the size of the tumor that now measured 2.50 × 2.00 cm. The cycloplegic refraction was now +3.00 D Sph –6.50 D Cyl × 40°.\nAfter several subsequent visits, there was no further regression in tumor size and astigmatic error remained. Therefore a mixture of 40 mg of triamcinolone acetonide and 8 mg of dexamethasone sodium phosphate was administered within the lesion under general anesthesia. The injection was given using a 27-gauge needle on a 1-ml tuberculin syringe at multiple injection sites throughout the lesion. A patch was applied over the treated eye and she was discharged. The immediate postoperative course was uneventful. At 3-4 weeks after treatment the patient followed light with both eyes, with nor mal fixation with marked reduction in the size of the tumor to 1.50 × 1.00 cm. On examination of the anterior segment, an area of hypopigmentation involving the left iris tissue was noted []. The pupil was normally reactive, funduscopy was anormal and cyclopelgic refraction was plano –6.00 axis 40°. Follow-up visits showed that the area of hypopigmentation remained unchanged []. On the last visit which was one year after treatment her visual acuity was 6/24 in her left eye, using Teller acuity measurements. Ultrasound bio-microscopy of the left iris was unremarkable. Imaging was also repeated which showed a regressed hemangioma with normal brain. |
The patient was a 58-year-old man, on treatment for hypertension, who was admitted to a vascular surgery service because of an incidental finding of two aneurysms of the SMA, identified during preoperative imaging exams preparatory to repair of an incisional hernia. The patient had no abdominal symptoms and on physical examination his abdomen was flaccid and painless and with a pulsating mobile mass in the epigastrium. Abdominal ultrasonography indicated a partially thrombosed saccular aneurysm in the retroperitoneal space, with no communication with the aorta. Multislice angiotomography revealed two aneurysms of the SMA, a proximal one measuring 5.9 × 5.2 × 5.0 cm and a distal one measuring 5.3 × 3.5 × 3.2 cm ( \n ). Since multiple collateral branches emerged from both aneurysm bodies, which meant the endovascular treatment would have involved a risk of damaging the intestinal blood supply, the decision was taken to perform open surgical repair. During the operation, by explorative laparotomy, access to the retroperitoneal space was achieved after performing the Cattell-Braasch maneuver, with medial displacement of the ascending colon and part of the transverse colon, exposing the infrarenal aorta and its branches. This revealed two true aneurysms of the SMA, the larger of which was around 3 cm from the arterial ostium and the smaller approximately 2 cm from the end of the first ( ). It was also possible to observe collateral branches (right colic, ileocolic, jejunal artery, and ileal arteries) projecting from the bodies of these aneurysms. A mesenteric-mesenteric, end-to-end bypass was therefore constructed, using a dacron prosthetic graft, excluding both aneurysms but preserving branches distal of the proximal aneurysm. It was decided to ligate and resect the aneurysms – sending specimens for cultures – and their lumens were opened, revealing large quantities of intraluminal thrombi ( ). Inspection of the abdominal cavity found the intestines to be viable and free from any sign of injury. During the postoperative period, the patient suffered gastrointestinal atony, but responded satisfactorily to prolonged conservative measures. Additionally, on the fifth day after the operation, a control computed tomography revealed signs of hematoma in the hepatorenal recess, managed conservatively to resolution. The patient was discharged from hospital to outpatients follow-up in good clinical condition, 18 days after the operation ( ). There was no bacterial growth in the culture of the aneurysm segment. |
A 79-year-old man presented with a six-month history of numbness in his left upper limb. Left motor weakness developed suddenly, and the patient was admitted to our hospital. Neurological examination on admission revealed left hemiparesis with 2/5 in his upper limb and 3/5 in his lower limb and hypoesthesia in his left side from the neck to the foot. The deep tendon reflexes were increased in his left upper limb. Magnetic resonance imaging (MRI) showed an intramedullary tumor that was homogeneously enhanced with gadolinium at the C1 level in the spinal cord (Figure ). The tumor was also surrounded by a signal change that appeared isointense to the CSF and was clearly differentiated from the spinal cord (Figure ). The signal change seemed to represent a presyrinx state.\nThe patient underwent removal of the tumor under general anesthesia. Laminectomy of the C1 and C2 levels was performed, combined with a suboccipital craniotomy. The tumor was located intramedullary, and the border between the tumor and the neural tissue was unclear. It also invaded into the dorsal root of the C1, and the root was removed with the tumor.\nPostoperatively, his motor function recovered to the level of 3/5 in his upper limb and 4/5 in his lower limb. Histological examination of the tumor revealed a diffuse proliferation of large atypical lymphocytes with B cell markers (Figure ). Based on these findings, the tumor was diagnosed as diffuse large B cell lymphoma. A subsequent MRI of the other regions, a bone marrow puncture, and an 18F-fluorodeoxy-glucose positron emission tomography (18FDG-PET) of the whole body showed no abnormalities (Figure ). The patient underwent high-dose methotrexate (MTX)-based chemotherapy and localized external beam radiation therapy (40Gy) for the level of C1 in the cervical cord. An MRI performed three months postoperatively showed total removal of the tumor and complete disappearance of the peritumoral signal abnormality (Figure ).\nHe has been alive for more than two years since the symptom onset, and without any evidence of recurrence.\nWritten informed consent was obtained from the patient and his next of kin prior to any procedures. |
A three-year-old Chinese girl came to our department in December 2016. She was born in a township hospital and had never received any physical examination until kindergarten admission. She was found to have amblyopia in the left eye at that time and was then diagnosed with congenital cataract at other hospitals, yet had never received any relevant intervention in the past. The girl was a full-term birth by a 36-year-old mother who was diagnosed with thyroid cancer 5 months after delivery. There was no history of similar complaints in the child’s family.\nThe girl’s physical and mental development was within normal range. Her best corrected visual acuity was 20/50 in the right eye and 20/100 in the left eye. The right eye examination was unremarkable, while in the left eye, slit lamp examination revealed posterior pole cataract and posterior capsule opacification as well as a floating horizontal vessel that connected to the tunica vasculosa lentis (Fig. a). Other ocular examinations did not identify any abnormalities; these examinations revealed normal intraocular pressure, cornea diameters and axial lengths, no dragged ciliary processes by ultrasound biomicroscopy, no fibrovascular stalk in the vitreous body by B-scan ultrasonography, and a healthy retina through ophthalmoscopy after mydriasis. Further fundus examination conducted by fundus photography and optical coherence tomography indicated no significant anatomical abnormalities (Fig. ). Altogether, a diagnosis of unilateral anterior PHPV was made.\nConsidering that the fetal vasculature was attached to the retrolenticular fibrous membrane and the plaque was not large enough to fill the pupil [], we decided to manage this case in a conservative manner instead of performing cataract surgery. The amblyopia was treated by 6 h of daily patching the fellow eye according to Amblyopia Preferred Practice Pattern of American Academy of Pediatric Ophthalmology []. Patching was conducted by applying an opaque patch to the contralateral eye and was closely monitored by her parents at home. Bimonthly follow-up examinations were arranged for her to monitor the visual acuity in both eyes as well as other ocular manifestations of the affected eye in order to adjust the treatment regimen timely.\nTwo months later, no changes were noted in the child’s left eye except that the blood flow in the hyaloid artery had slightly decreased (Fig. b); thus, it was decided that the treatment was to be continued. On seven months’ follow-up, the displaced hyaloid artery was noted to be nearly a nonperfusion vessel (Fig. a). An additional movie file showed this artery in more detail (see Additional file 1).\nAt 19 months’ follow-up, the improvement of the visual acuity of the child’s left eye to 20/50 with correction (+ 1.00D diopter cylindrical component at 85°) proved our treatment to be useful. The regression of the floating fetal vasculature into a nonperfusion ghost vessel was also observed (Fig. b). Comprehensive ocular examinations were conducted, and no ocular complications were detected.\nConsidering the patient’s age and visual acuity, she was advised to continue the treatment for amblyopia and the daily patching time was prescribed to be 2 h. Bimonthly follow-up was suggested, and the cataract would be treated with surgery if necessary. |
A 20-year old man was admitted because of nausea, vomiting and severe abdominal pain, accompanied by weight loss. Besides recidivating relapses of herpes infections in the mouth and throat, he suffered no other diseases. The symptoms started at the age of 13 years, when he had a sudden debut of abdominal pain and a collapse. Since then, he suffered occasional periods of abdominal pain and hard stools, alternating with periods of diarrhoea. The results of tests on blood samples taken repeatedly were all within the normal range. The diagnosis IBS was set according to the Rome-II criteria []. Both his mother and aunt suffered from functional dyspepsia since several years; else there is no history of hereditary factors.\nAt the age of 18 years, the symptoms grew worse. The most pronounced symptoms were nausea and abdominal pain, accompanied by weight loss. The symptoms were more and more accelerating, and the patient had difficulties to manage his work and daily life. Vomiting was present some time a week. Blood samples taken repeatedly were all within normal ranges, inclusive cobalamines and folate. Glutenintolerance was ruled out by negative antibodies against endomysium and transglutaminase. Feces haemoglobin was negative, suggesting intact mucosa. Esophago-gastro-duodenoscopy (EGD) showed normal findings in the upper gastrointestinal tract. Treatment with histamine receptor blockers and proton pump inhibitors did not have any impact on the nausea.\nAs the routine examinations turned out normally, the patient was referred to a psychiatric clinic for treatment. He visited the psychologist regularly for support. However, this treatment did not affect his nausea. Because the symptoms grew worse with abdominal pain, nausea, impaired appetite and weight loss, and altered bowel habits with constipation-dominance, he was admitted to the Department of Gastroenterology.\nThe dopamine receptor antagonist metoclopramide was then tested without any effect against the nausea. The muscle relaxantive drug papaverin had a small effect on abdominal pain. Further investigation with gastric emptying scintigraphy performed after ingestion of a meal, consisting of egg and bread, showed an emptying rate within the highest field of the reference value. After 60 min, 55% of the content remained in the ventricle (T50 for healthy controls; 40 ± 28 min) [].\nAs it was impossible for him to keep weight, also the 5-HT3- antagonist ondansetron and the neurolepticum prochlorperazini were tested without any result. Finally, the dopamine receptor antagonist domperidone was prescribed with good result, and the possibility of food intake was slightly improved. As the symptoms aggravated, and could not solely be explained by gastro paresis, the patient was considered for a diagnostic laparoscopy at the age of 23 years. This showed no macroscopic pathology of the small intestine with a normal peristalsis. There were no signs of adhesions, Meckel's diverticulum or tumours. A diamond-shaped, full-thickness wall biopsy was taken one meter proximal to the ileocecal valve and was immediately transported in a wet compress for pathological examination. The histopathological examination showed ganglioneuritis with neuron degeneration. The diagnosis enteric dysmotility was established based on the clinical picture and the histopathology []. |
A 40-year-old Caucasian man was admitted to our emergency department with moderate pain of his left abdomen for 2 days. He also complained of abdominal distension but presented no other signs and symptoms like fever, nausea, vomiting, diarrhea or intestinal obstruction. His medical history included an untreated left inguinal hernia in the last year. A physical examination revealed pain in his left abdomen associated with a palpable mass on his left flank, without rebound tenderness or guarding.\nTwo bilateral reducible inguinal hernias were found. Laboratory results revealed a C-reactive protein of 124mg/L. Given this unspecific clinical presentation, an abdominal and pelvic CT scan were performed, revealing an intra-abdominal abnormality, suggestive of pathologic infiltration of his omentum and the presence of the whirl sign suggesting torsion of his greater omentum (Figure A and B). The distal end of his omentum seemed to be alongside the spermatic cord into a bilateral inguinal hernia sac. Since there was no evidence of acute abdomen or incarcerated hernia and he was stable, we decided on conservative treatment because it has been reported that spontaneous derotation of the omentum can occur []. After he had spent 2 days as an in-patient of our hospital, his pain persisted and his left inguinal hernia became incarcerated. A preoperative diagnosis of secondary torsion of his greater omentum associated with an inguinal bilateral hernia (particularly left inguinal hernia) was established and he submitted to surgery.\nLaparoscopy was performed to evaluate the extent of the damage to his omentum. A wide twist of omentum with large necrotic vascular changes that extended to the inguinal hernia content was found (Figure A). After reducing the hernia content, omentectomy was performed. His abdominal cavity was reached using a 10mm trocar under his umbilicus. Two accessory trocars were placed in his right flank (5mm). A median minilaparotomy (6cm) through the umbilicus port was created for exteriorization and resection of his large necrotic omentum (Figure B). Surgical repair of bilateral inguinal hernia was performed by Lichtenstein technique, since the success of laparoscopic inguinal hernia repair techniques (such as transabdominal preperitoneal) in incarcerated hernias is still controversial [,]. A histopathological examination revealed large areas of interstitial hemorrhage, vascular congestion and thrombosis compatible with omental torsion.\nHis postoperative period occurred without complications and he was discharged on the third day after surgery. One month later he yielded no complaint. |
In December 2006, a 31-year-old woman was referred to a neurologist because of consciousness disorder and fainting. Her main problems were obesity, snoring and waking up with a feeling of suffocation in the middle of sleep. The intraoral examination showed a large soft palate (). The soft palate was scored as class III according to the Mallampati classification (visualization of the soft palate and the base of the uvula) []. The electroencephalogram (EEG) showed focal dysrhythmia during hyperventilation with scattered sharp waves (). The patient was depressed and had sleep disorders such as sleep apnea and myoclonus, especially at the onset of sleep. She had experienced several occurrences of complete loss of consciousness during swimming and at work. The patient was on anticonvulsants and antidepressants (at first, she had been prescribed with Lamotrigine for 5 months, but later she was given 500mg Sodium valproate per day).\nOne of the best treatments for snoring during sleep is UUUP. The success rate of this type of surgery is reported to be between 16% and 83% [].\nWe chose a minimally invasive surgical procedure for the present case since the patient had a proper facial profile and a large soft palate (class III according to the Mallampati classification) [].\nIn May 2007, after analyzing the lateral cephalogram, we evaluated the craniofacial and pharyngeal airway morphology before the surgery. Under general anesthesia, 1cm of the soft palatal mucosa, from the right tonsil to the left tonsil, was removed. The patient’s tonsils were also removed during the surgery, and the anterior and posterior tonsillar pillars were sutured together ().\nThe symptoms were significantly decreased after the recovery. The patient no longer had sleep apnea, and antidepressants and antiepileptic drugs were discontinued. After the surgery, sharp waves were detected on the EEG at the level of the trachea (), but the patient was clinically asymptomatic. The 10-year follow-up showed no symptoms of sleep apnea or seizure. The patient did not lose any weight during the follow-up period. |
A 38-year-old woman with a 3-month history of an anterior cervical mass located in the suprasternal region of her neck was referred to our hospital. Physical examination revealed that the 3-cm movable neck mass was firm and slightly tender on palpation and had a distinct margin from surrounding tissues. An ultrasound examination showed that the well-defined oval mass was 31 × 23 × 17 mm in size and exhibited heterogeneity. Furthermore, a non-enhanced computed tomography (CT) scan of the neck revealed that the distinct neck mass in the subcutaneous tissue had a mixture of soft tissue and fatty components (Fig. ). Based on these findings, the cervical tumor was clinically diagnosed to be an unusual lipoma with degeneration; however, we could not exclude the possibility of it being a malignant tumor such as liposarcoma. Thus, fine-needle aspiration cytology of the tumor was performed, but no diagnosis of malignant cells was obtained. Further examinations were conducted to address concerns related to her menstrual pain, and abdominal magnetic resonance imaging revealed bilateral ovarian cysts in the lower abdomen. Under general anesthesia, the patient underwent neck mass extirpation and bilateral ovarian cystectomy; pathological examination of the cysts resulted in a diagnosis of ovarian endometriotic cysts. During the surgery, the cervical mass was well demarcated and did not adhere to the surrounding tissues. The postoperative course was uneventful. The gross pathology report showed that the neck mass measured 3.0 × 2.5 × 2.0 cm. The cut surface of the specimen was heterogeneous, solid, whitish, and yellowish (Fig. ). Microscopically, the tumor was composed of spindle cells, epithelial nests, and mature adipose tissue (Fig. a, b). Immunohistochemical examination revealed that both spindle cells and epithelial nests were positive for cytokeratin (CK) AE1/AE3 (Fig. ). As these histopathological findings were consistent with the features of EHT, a diagnosis of EHT was confirmed. Over a follow-up period of 30 months, this patient exhibited no evidence of recurrence. |
The patient was a 65-year-old woman who presented with a 1-month history of bloody stool. A digital colonoscopy with biopsies revealed adenocarcinoma in the sigmoid colon. The patient elected to undergo primary laparoscopic colon resection, and the procedure was reported to be uneventful. However, on the 6th postoperative day, the patient noticed a large amount of yellow fluid coming out of a left side abdominal drain. The fluid appeared to be urine. An abdominal ultrasonography showed a collection of fluid in the patient's pelvis. A contrasted computed tomography (CT) scan showed contrast extravasation in the pelvis and around the descending colon (). The patient had decreased serum protein and albumin; however, complete blood count, creatinine, liver functions, and urine analysis were normal. On the 8th postoperative day, the patient was taken to the operating room for a ureteroscopy. The ureteroscopy revealed that the left ureter was completely severed about 4 to 5 cm from the ureteral orifice (, transected distal end of the ureter). The bowels could be seen through the ureteroscope (, intraabdominal cavity with bowels seen through the distal end of the transected ureter). No other obvious injury was identified. With patience, persistence, and some difficulty, the severed upper end of the ureter was identified and entered (, the proximal end of the transected ureter). We estimated that there was a 3- to 4-cm gap between the two ends of the ureter. Two 0.035″ guidewires were first passed, followed by the placement of two 4.5F Double-J ureteral stents. After placing the Double-J stents, the abdominal drainage quickly subsided. An abdominal ultrasonography 6 days after tube placement showed complete resolution of the abdominal fluid collection. The abdominal drain was removed and the patient was discharged. At the 3-month follow-up, a repeat CT scan showed no hydronephrosis, no abdominal fluid collection, and no contrast extravasation. The patient, however, had an asymptomatic urinary tract infection from Klebsiella pneumoniae, which was treated and resolved. A follow-up ureteroscopy over a guidewire showed excellent healing and realignment of the disrupted ureter. The only obvious sign of the transacted ureter was that the mucosa was paler than normal. Because of this unconventional treatment, we decided to continue stenting the ureter with two fresh 4.5F Double-J stents for an additional 4 weeks. Stents were removed 4 months after the injury. Follow-up contrast-enhanced CT scans taken 8 and 14 months after the initial endoscopic treatment showed mild but unchanged residual dilation of the renal pelvis and ureter with good drainage. There were no other abnormalities. |
A 27-year-old man was involved in a road traffic accident (RTA), where he was a passenger restrained with a 3-point seat belt. Initial investigation at the local Emergency Department (ED) revealed posterior rib fractures and a haemothorax, which was treated by insertion of a chest drain. Further imaging of the thorax by computed tomography (CT) and magnetic resonance imaging (MRI) revealed a posterior element fracture at the level of T8.\nFour days later he was transferred to our centre for clinical review and further treatment with regard to his spinal injuries. Review of the imaging by the orthopaedic team at this time revealed a possible combined bony and soft tissue Chance fracture, with a rupture of the ligamentum flavum at T7/8 and a hyperintense signal in the intervertebral disc at T8/9 on MRI (). The patient was counselled as to the possible unstable nature of this type of fracture and was informed of the risks and benefits of both surgical and conservative management. After long discussion with the surgical team, family members, and members of the religious community, the patient opted for conservative management.\nA thoracic brace was fitted on day 7 after injury, and the patient was mobilised. Anteroposterior (AP) and lateral x-rays of the thoracic spine were taken to demonstrate the alignment of the vertebrae. The patient was discharged with paracetamol and dihydrocodeine PRN for analgesia, with follow-up planned one week later to monitor progression of the thoracic kyphosis. At the first follow-up appointment lateral X-ray of the thoracic spine showed no displacement of the vertebrae, and fortnightly follow-up was arranged thereafter.\nThe patient developed pleuritic chest pain four days following this first appointment and was investigated by CT pulmonary angiogram (CTPA) to exclude pulmonary embolism (PE). CTPA was negative for PE and also demonstrated that there continued to be no displacement of the thoracic spine. Further x-rays at three and five weeks after discharge also demonstrated no change to thoracic alignment. However, at ten weeks following initial presentation, the patient complained of increased thoracic back pain, and lateral X-ray of the thoracic spine suggested displacement of the vertebra at the fracture site. MRI confirmed a traumatic spondylolisthesis of T8 on T9 (). The patient had been compliant with brace treatment and had worn the brace day and night for 6 weeks (7 weeks after injury) and then for a further 3 weeks when out of bed and mobilising.\nThe patient was still neurologically intact but was advised by the surgical team that the risks of continuing conservative management now greatly outweighed the risks of surgery. He underwent instrumented posterior stabilisation and fusion with allograft bone from T7 to T10 eight weeks later. |
A 45-year-old male was admitted to the hospital with a one-day history of abdominal pain and dyspnea. The abdominal pain started the day of admission; however, he noted worsening shortness of breath and a productive cough with clear sputum over the course of several days. He also noted a 20-pound weight loss occurring over a three-month period but denied fevers, chills, or night sweats. Physical exam was notable for tachycardia, distant heart sounds, and decreased breath sounds of the lung bases bilaterally. Vitals at presentation included a temperature of 99.2°F, heart rate of 104, respiratory rate of 20, blood pressure 182/106 mmHg, and an oxygen saturation of 95% of 2 liters via nasal cannula.\nOur patient has a past medical history significant for hypertension, chronic anemia, and end stage renal disease, due to focal segmental glomerular nephritis, on hemodialysis three times per week. He has required hemodialysis for approximately four years and unfortunately does not have an arteriovenous fistula due to financial restraints; therefore, his dialysis access is via a left jugular tunneled catheter. He has required multiple tunneled catheters over the past four years.\nInitial metabolic panel was within normal limits except for his creatinine of 7.99 mg/dL. Complete blood cell count revealed anemia with a hemoglobin of 10.6 g/dL and hematocrit of 33.7% but otherwise within normal limits. Chest x-ray was remarkable for bilateral pleural effusions and prominence of the cardiopericardial silhouette consistent with pericardial effusion (). Given his abdominal pain a CT of the abdomen and pelvis without intravenous contrast was performed showing large right and small left pleural effusions and a large pericardial effusion. Since the CT scan was able to capture a majority of both the pleural and pericardial effusions a dedicated CT scan of the thorax was initially deferred.\nGiven his dyspnea and pleural effusions our patient underwent a right-sided thoracentesis by interventional radiology with removal of 1.5 liters of cloudy amber-colored fluid. Body fluid studies revealed a total protein of 3.3 g/dL, LDH 110, glucose 106, RBC 45, WBC 993, lymphocyte predominance of 91%, and pH of 7.0. Serum LDH was 247 and serum protein was of 6.6 g/dL. Using Light's Criteria () our patient did not meet criteria for exudative effusion; however, results were borderline with a pleural fluid protein/serum protein ratio of 0.5 and pleural fluid LDH/serum LDH ratio of 0.445. Acid fast smear and cultures were obtained and resulted negative. Cytology was negative for malignancy but showed many small mature lymphocytes, mesothelial cells, and a few acute inflammatory cells.\nFollowing thoracentesis, he had significant improvement in dyspnea and oxygen saturation improved to the upper 90s on room air. Regarding the pericardial effusion, a transthoracic echocardiogram was obtained revealing a large circumferential pericardial effusion measuring 1.67 cm anteriorly and 1.29 cm posteriorly. The chambers of the right atrium (RA) and right ventricle (RV) were very small, with complete obliteration of the RV chamber during systole, suggesting high intrapericardial pressure. The IVC was collapsible, measuring 1.67 cm, with a 50% collapse during inspiration. No diastolic collapse of the RV or complete systolic collapse of the RA was noted. Cardiology was consulted and during their physical exam he displayed no jugular venous distention, negative Kusmaul's sign, and no clinical signs of tamponade. An EKG was obtained showing sinus rhythm and no electrical alternans; thus pericardiocentesis was deferred.\nOver the next four days he continued his regularly scheduled hemodialysis sessions and showed improvement in the pericardial effusion. Give hemodynamic stability and significant improvement there were no plans for intervention and the decision was made to closely follow with cardiology as an outpatient. However, on the planned day of discharge he developed supraventricular tachycardia and was kept another night for observation and evaluation by electrophysiology. During this time a repeat chest X-ray was performed revealing a reoccurrence of his pleural effusions; therefore, pulmonary medicine was consulted to further investigate.\nPrevious body fluid studies were reviewed, and, given significantly elevated lymphocytes of 91% and borderline Light's Criteria for exudative effusion, there was initial concern for common etiologies including tuberculosis, sarcoidosis, and malignancy. Our patient immigrated from Mexico to the United States approximately 20 years ago but denied any Tuberculosis exposure or previous diagnosis. He did have a 20-pound weight loss but denied other B like symptoms. We elected to further investigate the thorax for the presences of anatomical abnormalities, so a CT thorax was obtained. CT demonstrated a reaccumulation of the right pleural effusion and persistent moderate to large pericardial effusion () but surprisingly showed stenosis or occlusion of the superior vena cava (). This new finding was discussed with radiology that further commented on mediastinal collaterals and evidence of SVC stenosis particularly around the patient's tunneled catheter.\nA repeat thoracentesis was performed and 1.6 liters of milky fluid with a pink tinge was removed. Body fluid was remarkable for total protein of 4.6 g/dL, glucose 72, triglycerides 1,056 mg/dL, cholesterol 116 mg/dL, RBC 423,195, WBC 1,760, lymphocytes 86%, and pH 8.0. These findings were consistent with chylothorax likely form newly discovered SVC stenosis.\nOther labs to evaluate for autoimmune etiology including rheumatoid factor, c-ANCA, p-ANCA, ANA, and hepatitis screen were also obtained and were unremarkable.\nRepeat echocardiogram was performed and worsening pericardial effusion was noted revealing RA collapse and diastolic RV collapse suggestive of cardiac tamponade (). Hemodynamically the patient remained stable and a scheduled pericardial drain was placed with aspiration of 775 mL of bloody fluid consistent with chylopericardium.\nGiven recurrent pleural and pericardial effusions vascular surgery was consulted for SVC stenosis. He underwent angioplasty of the SVC and left brachiocephalic vein using a 12x4 angioplasty balloon and replacement of the tunneled hemodialysis catheter into the left jugular vein. Ultimately, after balloon angioplasty, high triglyceride diet, and draining both the pleural and pericardial effusion his symptoms significantly improved, and he was discharged home. There are plans to establish permanent hemodialysis access by creating an AV fistula in the near future. |
A male infant, who was term appropriate for gestational age, was born via repeat caesarean section to a healthy 26-year-old woman. No family history of bleeding disorders was reported by the mother. Pregnancy was unremarkable, and Apgar scores were 9 and 9 at 1 and 5 minutes, respectively. On initial physical examination, a localized hematoma to the left parietotemporal region was identified. A routine complete blood count (CBC) displayed normal counts. It was decided to repeat the CBC in 12 hours and, in the interim, to observe for dissemination of the blood collection. Prior to the next blood test, the physician was called to the bedside because the infant now had a change in his clinical examination: pale and tachycardic. His examination now revealed a large palpable fluid wave that extended behind the neck and left ear. Given the infant’s change in clinical status and concern for a subgaleal hematoma, the infant was transferred to the neonatal intensive care unit (NICU). The repeat CBC showed a hemoglobin level of 7.6 g/dL, with a hematocrit of 22% and platelets of 169 × 109/L. In the NICU, the patient received a transfusion of 20 cc/kg of O Rh-negative packed red cells. His coagulation profile disclosed a normal prothrombin time but an activated partial thromboplastin time (PTT) of 101 seconds (normal = 25-30 seconds).\nSubsequently, a FVIII level was ordered, which was very low at <0.01 U/mL. He was diagnosed with severe HA and was initially treated with 50 U/kg of recombinant FVIII. His repeat doses were adjusted to bring his FVIII level to 100%. A computed tomography (CT) scan of the head revealed a large subgaleal hematoma, beginning in the left parietal area, extending to the occiput and down the nape of the neck. The infant was discharged at 1 week of age following a repeat head imaging confirming complete resolution of the extracranial hemorrhage (ECH) without any evidence of intracranial bleeding. |
A 34-year-old female Chinese patient was admitted to our intensive care unit due to hypertriglyceridemia and severe acute pancreatitis. A contrast-enhanced computed tomography (CECT) on admission showed an enlarged necrotic pancreas and peripancreatic inflammation and fluid (Figure ). As she suffered from acute respiratory distress syndrome (ARDS) and required mechanical ventilation, a NJT was placed on the fifth day after admission at bedside by transnasal endoscopic technique. The tube position was not determined by an abdominal radiography. We initiated a D5W solution feeding after intubation. No initial discomfort was noted as the peptisorb liquid (of up to 50 ml per hour) was administered. The patient tolerated this for 15 days, and was weaned from mechanical ventilation on the 10th day. However, on the 21st day after hospitalization, she again had a high fever and abdominal distension. So we checked her using an ultrasound (US) at bedside and performed a US-guided percutaneous catheter drainage of the doubtful retroperitoneal abscesses. The left abscess was demonstrated by the aspirate, but the aspirate from the right retroperitoneal abscess was similar to the peptisorb liquid. We injected 20 ml of methylthioninium chloride through the NJT and found it effusing immediately via the right drainage catheter. At first we thought that the right drainage catheter had penetrated the duodenum or superior segment jejunum. An abdominal radiography was performed and showed that the NJT was kinking on the third portion of the duodenum. The duodenum was not visualized when meglumine diatrizoate was injected via the right drainage catheter (Figure ). Meglumine diatrizoate was injected via the NJT and showed that the tip of the NJT was inserted into the right retroperitoneal abscess on the second portion of the duodenum (Figure ). This demonstrated that the duodenal perforation was caused by the NJT. The position of the NJT was adjusted using fluoroscopic guidance and the tip was exactly beyond the ligament of Treitz. After this, the peptisorb liquid from the right drainage catheter disappeared. But her symptoms did not release after two days of conservative treatment. A CT scan of the abdomen showed retroperitoneal emphysema and an abscess. An emergency exploratory laparotomy was performed. The duodenum perforation was not found but peptisorb liquid accumulation was identified. The retroperitoneal abscess was drained. The recovery course was smooth and EN via the NJT was started five days after the surgical procedure. |
A 78-year-old female had been followed up in our Rheumatology outpatient clinic since being diagnosed with rheumatoid arthritis in 1987. Treatment has included occasional intraarticular and intramuscular corticosteroid injections, but never long-term oral corticosteroids. Current immunosuppressive therapy consists of methotrexate and hydroxychloroquine. She also has a background history of an idiopathic kyphoscoliosis, bronchiectasis, and hypertension. She had undergone bilateral total knee arthroplasties in 2007. Osteoporosis was diagnosed in March 2010, when a DEXA scan (Hologic, Crawley, United Kingdom) showed T scores in the lumbar spine (total) and left hip (total) of −2.7 and −2.9, respectively. She has no history of a prior fragility fracture. She was initially treated with oral alendronate, but after a few weeks of intermittent use, this drug was discontinued due to upper gastrointestinal intolerance. She was subsequently treated with Strontium ranelate, but stopped this after a few days due to lower gastrointestinal intolerance. She has also had great difficulty tolerating oral calcium and vitamin D supplements. Other relevant drug therapies on admission included Omeprazole 20 mg once daily, which she had been taking for two years. She was commenced on Denosumab in September 2011. She received a total of 3 doses of Denosumab 60 mg, given subcutaneously at 6 monthly intervals, the last dose being given in September 2012.\nShe presented to our hospital in January 2013 reportedly attempting to stand from sitting on the side of her bed when she heard a crack and felt severe pain in her right thigh. After this, she became unable to bear weight as a result of severe pain. Importantly, she reported that for approximately five weeks prior to this, she had been experiencing increasing pain in both her thighs, more severe on the right, and that she had been increasingly dependent on crutches to mobilise. A radiograph of her right femur was requested on her admission to hospital, which revealed a noncomminuted spiral fracture with a medial spike ().\nFour weeks prior to this admission, a radiograph of her right femur had been requested which was reported to have no abnormality seen with correct positioning of the knee prosthesis and no sign of loosening or a stress reaction (). However, a further review of this radiograph raised the possibility of a subtle periosteal reaction on the lateral cortex of the diaphysis. Blood tests on admission revealed that her albumin-adjusted calcium serum was 2.35 mmol/L (reference range 2.1–2.7 mmol/L), phosphate was 1.11 mmol/L (0.7–1.5 mmol/L), alkaline phosphatase was 68 μmol/L (39–150 μmol/L), and creatinine was 73 μmol/L (60–120 μmol/L). She reported no trauma immediately preceding or in the weeks before developing the fracture.\nManagement of her fracture was complicated as she was found to be suffering from type 2 respiratory failure that necessitated a short period in intensive care unit (ICU) where she was intubated and ventilated. On the following day, after she was stabilized, operative fixation of her femoral fracture was undertaken. An intramedullary nail was inserted () and she was transferred back to ICU where she continued to wean off the bilevel positive airways pressure support and was treated with antibiotics.\nSince her discharge, she has been reviewed in the Orthopaedic and Rheumatology outpatient clinics. The intramedullary nail maintains correct alignment, with moderate callus formation (Supplementary Figure 1(d) in Supplementary material available online at ). There is cortical thickening in the contralateral femur (Supplementary Figure 1(e) in Supplementary material). The patient is now mobilizing with one stick and is taking a nonprescribed vitamin D supplement providing 800 iu daily. Further blood tests showed an albumin-adjusted serum calcium of 2.21 mmol/L, phosphate of 1.24 mmol/L, alkaline phosphatase of 100 μmol/L, ESR of 48 mm/hr, normal protein electrophoresis, and a serum 25-OH vitamin D level of 65 nmol/L (70–200 nmol/L). No evidence of malignancy was observed on histological examination of bone samples taken at the time of surgery. A repeat DEXA scan in June 2013 shows a stable T score in the lumbar spine of −2.4 and a T score in the left hip of −2.9. Bone turnover markers were not done as they are not currently available in our laboratory. Treatment with Denosumab has been ceased indefinitely in accordance with current recommendations published by the Medicines and Healthcare products Regulatory Agency (MRHA) []. |
A 62 years old male presented with recurrent SCC of the left nasal bulbar conjunctiva. The patient's previous medical history detailed multiple interventions at this site. Initially, the diagnosis of a left nasal pterygium (a wing-shaped growth that starts on the conjunctiva and can spread across the limbus to the cornea affecting visual function) was made 3 years prior to the reported presentation, and was surgically removed with local excision. The lesion recurred 1-year post-surgery, and was subsequently managed surgically with a wide local excision. At this time, pathology revealed positive margins for SCC. A further recurrence 6 months later was treated with Plaque Therapy to a dose of 50 Gy in 5 fractions. Subsequently, the lesion was re-excised with adjuvant cryotherapy 7 months later.\nOn presentation 3 months post-cryotherapy, a recurrent lesion (10 × 5 × 2 mm) was detected on the nasal bulbar conjunctiva (Fig. ). The patient declined enucleation following this SCC recurrence. Subsequently, multidisciplinary consultation offered SXRT as an alternative treatment option (Fig. ).\nThe patient also presented with a history of Crohns Colitis (treated with immunotherapy) and a previous excision of a non-melanoma skin cancer from the right temple, and a subsequent skin graft of the area. The patient was on no other medications and had no allergies.\nThe patient was prescribed a dose of 48.4 Gy in 22 fractions, to a depth of 3 mm. This resulted in a skin surface dose of 54.4 Gy. A 2.0 cm diameter direct applicator was used with a 3 mm margin on the treatment region (Fig. ). To allow for an acceptable treatment margin that encompases the target volume and accounts for set up variability, the left inner canthus and tear duct were included in the treatment field. A single en-face beam of 2.0 mm Aluminium energy (2 mm Al) was used to meet the previously articulated dose prescription. The risk of ulceration, scleral perforation, ischemia and nasolacrimal duct obstruction was explained to the patient. This risk was further exacerbated in this case due to re-irradiation of the previously treated area, and a particularly sensitive region post-cryotherapy, surgery and plaque therapy. Topical anaesthesia (Tetracaine eyedrops) was administered to the left eye prior to retractor positioning, to ensure eyelids remained open throughout SXRT delivery. After positioning of the retractors the patient was instructed to maintain gaze on a target positioned to the left side of the room, and the eye was monitored throughout treatment delivery by video surveillance. Lubricating eye drops were prescribed for use prior to treatment administration to prevent drying of the ocular surface, and for the patients self-use between fractions to relieve dry eye symptoms. Total set up and treatment time for this patient was approximately 20 min, with the retractors in place on average less than 10 min. The anaesthetic drops and ocular surface lubricants induced limited sensation and the patient found it mildly uncomfortable. As a precaution, the patient was informed to avoid contact with the anaesthetised eye and wear a protective patch for 60 min post-treatment delivery daily. |
A 39-year-old man was involved in a high-velocity motor vehicle accident. He sustained an open fracture of the right elbow, with significant loss of the external humeral condyle and partial loss of the olecranon. This fracture was classified as a Gustillo type IIIA injury. There was no neurovascular compromise. The patient was treated in a community center, close to the accident, where he received surgical care (debridement and partial excision of the olecranon). The wound was fully closed. The upper arm was then immobilized in a splint (). IV antibiotics (cefazolin-gentamicin) were started for 5 days.\nThe day following his elbow surgery, the patient fell in a staircase and sustained a C7-C8 and C8-T1 fracture-dislocation. This injury caused neurologic damage (quadriparesis), and his right arm became his only functional limb. Following this injury, the patient was moved to our tertiary center to get spinal fusion. During the spinal surgery, the elbow was tested under fluoroscopy. The patient's elbow showed varus instability and a positive pivot shift test. A CT scan of the elbow was obtained the following day and showed bony loss from the external humeral condyle and subluxation of the radial head ().\nWe decided to treat the patient's elbow surgically. The surgery underwent nine days after the initial trauma (after transfer from the community center, spine procedure and elbow imaging). A posterior approach to the elbow was used along with an extensive elbow debridement. A tricortical iliac crest graft was then collected from the patient's right side to replace the humeral condyle bone loss. A tendinous graft was collected from his third and fourth extensor digitorum longus tendons to reconstruct the lateral collateral ligament. The tendinous graft was fixed to the bone graft through two tunnels (anterior to posterior and lateral to medial). The iliac crest graft was then fixed to the humerus with a cancellous screw. A five-hole one-third tubular plate was used as buttress. The graft was left prominent on the lateral side to improve lateral stability of the elbow. The lateral collateral ligament was then reconstructed by fixing the tendinous graft to the proximal ulna with two bundles. We took care to preserve our reconstructed lateral collateral ligament's isometry during flexion and extension movements. Reduction and stability of the elbow joint were verified under fluoroscopy. The wound was closed with staples, and a posterior splint was applied for three weeks. 14 days postoperative X-rays are presented on . At three weeks following the surgery, passive and active ranges of motion were initiated.\nWhile wearing the splint, the patient developed a pressure wound on the lateral side of his elbow (at the site of the reconstruction). This wound later complicated into a Nocardia septic arthritis, which required surgical debridement. Four months after the reconstruction, range of motion was 30-120 degrees with full pronation and supination, without any evidence of instability.\nTwo months later, instrumentation was removed and a rotational flap was done to increase soft tissue coverage of the articulation. Five months after that procedure, the patient developed a Nocardia osteomyelitis of the humerus and was treated with 6 weeks of IV antibiotics (penicillin) and a surgical debridement. The patient responded well to our treatment. Eleven months after the initial intervention, range of motion of the joint ranged from 30 to 120 degrees of flexion, without clinical instability. Osteosynthesis was achieved according to X-rays of the elbow (). |
A 6-year-old boy reported seeking treatment for his abnormally appearing upper front tooth. Patient had previous episodes of spontaneous pain associated with the anomalous tooth, which was relieved by medications. His medical and family histories were non-contributory. Intraoral examination revealed that the patient was in early mixed dentition stage. Mandibular primary central incisors were replaced by the permanent successors. Mandibular primary right lateral incisor was exfoliated; all four permanent first molars were erupted and rest of the primary dentition was intact. Primary maxillary left central incisor had a double crown; the two halves of the crown were like mirror images. They were separated by a deep developmental groove on labial and lingual surfaces running from incisal edge to the sub-gingival region of the crown []. The lingual groove had a carious lesion and the tooth was tender to vertical percussion. Dental midline was shifted to the right side due to the presence of the large anomalous tooth. Intraoral periapical radiograph exhibited primary maxillary left central incisor like a double tooth. A radiolucent line was present between the two halves of anomalous tooth crown from incisal edge to the cervical area of the crown. There were two separate pulp chambers and root canals. Periapical radiolucency and root resorption in the apical region was present with the affected tooth []. Depending on history, clinical and radiographic examinations, a diagnosis of double tooth affecting primary maxillary left central incisor with chronic periapical abscess was made. Treatment was planned after discussion with the parents, which included endodontic treatment of the affected tooth followed by esthetic rehabilitation using direct composite restoration.\nAfter administration of local anesthetic, direct access was gained to the root apices under rubber-dam. Shaping and cleaning of the canals was performed using endodontic K-files and H-files (MANI, INC. Utsunomiya, Tochigi, Japan). Irrigation of the root canals at every step was done with 2.5% sodium hypochlorite and normal saline. The root canals were filled using a combination of calcium hydroxide, iodoform and silicon oil (Metapex, Meta Biomed Co., Ltd. Cheongju Factory, Korea) []. Access cavity was sealed primarily with cavit (3M ESPE AG, Seefeld, Germany) and followed by glass ionomer cement (Ketac™ Molar Easymix, 3M ESPE AG, Seefeld, Germany).\nMesiodistal dimension of the affected tooth was considerably more in comparison with the right central incisor. It was decided to reduce the size of primary maxillary left central incisor and increase the size of right central incisor. Shade selection for the composite material was done. For primary left central incisor, incisal and proximal reduction of 1 mm and 0.5 mm of facial and palatal reduction was carried out. Maxillary right central incisor required no tooth reduction, only smoothening of the enamel margins was done. Preliminary impression was made in alginate and master model was prepared using dental stone. Laboratory wax-up was done using inlay wax, to mimic the final tooth counters []. A composite buildup guide (stent) was fabricated using addition-type silicone elastomer (Affinis putty supersoft, polyvinylsiloxane, Coltene/Whaledent AG, Feldwiesenstrasse, Switzerland) to register the palatal surfaces, proximal contours and the incisal edges against which the composite would be built []. The teeth were etched for 15 seconds with 36% phosphoric acid and rinsed for 10 seconds with water. After removal of the excess water, dentin bonding agent (Adper™ Single bond 2, adhesive; 3M ESPE AG, Seefeld, Germany) was applied for 15 seconds with a brush and then air thinned. The bonding agent was light cured for 20 seconds. The stent was then positioned in the mouth and the palatal, proximal and incisal aspects were built with the composite resin material (Filtek Z250; 3M ESPE AG, Seefeld, Germany) in an incremental manner followed by facial surfaces. Final finishing and polishing of the composite was done using finishing burs and composite finishing kit (SHOFU, SHANK CA, PN 0306, Shofu Dental Corporation, USA). Even though dental midline was not coinciding, reasonably good esthetics was delivered to the patient [].\nPatient was followed up for 1 year at monthly intervals, the patient was asymptomatic throughout the period and root of the primary double tooth was resorbed at a normal rate. After 1 year, the permanent maxillary left central incisor erupted in the oral cavity from the palatal aspect of the affected tooth [], displacing it facially. The primary double tooth showed grade-3 mobility. However, permanent right central incisor was not yet erupted, the primary predecessor exhibited grade-2 mobility. The radiographic examination revealed almost complete root resorption of the primary central incisors. Simultaneously, root canal filling material was also resorbed and the permanent successors were in the path of eruption []. The amount of root formation and the eruption of permanent maxillary left central incisor were ahead of the permanent maxillary right central incisor. Within 2 weeks period after the first year follow-up appointment, the patient reported with physiologically exfoliated primary double tooth. |
A 63-year-old male, 10-day post completion of anti-tuberculosis regimen for pulmonary tuberculosis, known smoker and alcoholic, presented with grade-5 dysphagia associated with food regurgitation, cough and weight loss. On general examination, he was ill looking, cachexic, dehydrated and pale, not jaundiced but no lower limb edema. His vitals were blood pressure of 139/71 mmHg, Temperature of 35.8°C, oxygen saturation of 95% on room air, respiratory rate of 18 cycles per minute and a pulse rate of 73 beats per minute. He had a scaphoid abdomen with tenderness on the right lumbar region. Fine crepitations were heard with reduced air entry on the right side of his chest. Full blood picture was done which was normal with a haemoglobin of 11.3 g/dl, other investigations including renal function tests and liver enzymes were normal. Chest X-ray done showed signs of metastasis (). CT-scan of the abdomen done at the referring hospital with both oral and intravenous contrast reported structures above the diaphragm showing dilated oesophagus measuring 41 × 38 mm due to distal retro-cardiac concentric oesophageal wall thickening amounting to 18 mm with luminal narrowing with possibility oesophageal fistula tract. The gastric cavity was normal without focal lesions. OGD revealed the oesophageal mucosa was hyperemic with a blocking mass at 29 cm from the upper incisors. The scope was able to pass beyond the lesion. The cardiac, fundus and body mucosa was atrophic. There were metaplastic changes at the antrum and pylorus. Multiple biopsies were taken. Histology results revealed oesophageal well differentiated invasive squamous cell carcinoma.\nThe patient was scheduled for GFT insertion. Under aseptic technique, with the patient in supine position, 24 Fr urinary catheter as an improvised GFT was inserted in Stamm manner, haemostasis achieved, abdomen closed in layers and dressed (). There was no ascites and mesenteric lymph nodes were not palpable. After the procedure the patient was discharged home on the second day with analgesics and was booked for a surgical outpatient clinic appointment after 2 weeks.\nThe patient was then seen at the clinic after 2 weeks, she was clinically and vitally stable and the GFT was functioning well. The wound had healed, the tube was patent and there was no leakage from the GFT site. Midline sutures were removed and the patient was discharged.\nThe patient presented to the surgical OPD 3-month later with complaints of GFT blockage at the tip. Under local anaesthesia, the previous tube was removed and another 24 Fr catheter was inserted in a sterile manner and secured and patency was tested. The patient was then discharged and care takers were counselled on use of semi-solid food and to flush the tube with water after every meal.\nHe was reviewed 3-month later, at the OPD, free from complaints and no leakage with a patent tube. He had already begun his first cycle of radiotherapy. |
A 34-year-old male patient presented with a gradually enlarging swelling at the side of his face in front of the left ear of 15 days duration along with a long-standing difficulty in mouth opening [].\nThe patient's medical history was predominantly noncontributory except a history of trauma to the facial region following a fall during childhood. He could however not provide any details about the exact site or provide any prior medical records. General physical and systemic examination was unremarkable. The patient was moderately built and nourished, and afebrile at time of examination. All the vital signs were within the normal limits. The patient gave a history of areca nut and tobacco consumption that he chewed 5–6 times a day (T.I.D) since 8 years. In addition, it was noted that a dull pain was elicited in the left temporomandibular joint (TMJ) region during mouth opening.\nExtraoral inspection revealed a unilateral solitary oval swelling in the left TMJ region measuring 0.5 cm × 1.0 cm in size with normal overlying skin. On palpation, the swelling was found to be firm, tender, and nonfluctuant. There was a noticeable local rise in temperature in the region of the swelling. Tenderness was elicited in the temporalis muscle of the same side with no obvious lymphadenopathy in the head and neck region. Intraorally, the buccal mucosa appeared blanched, occlusion was unaffected with no other significant findings. On palpation, thick fibrous bands were noted in the left buccal mucosa that gave a clinical impression of oral submucous fibrosis. This supposedly could be a contributing factor to the patient's reduced mouth opening apart from the possible hindrance caused by the swelling present in the TMJ region.\nThe patient was referred to an otolaryngologist for otoscopic examination that eliminated the possibility of a middle ear infection.\nThe digital panoramic radiograph exhibited an ill-defined radiolucent area in the region of the left condyle []. The rarefaction in the left condylar region was suggestive of osteomyelitis.\nIn the bone window, there was evidence of erosion with the trabecular destruction of the left mandibular condyle [Figure –]. Contrast computed tomography was suggestive of an abscess formation with peripheral enhancement surrounding the left condyle extending into the left masseter. There were few minimally enlarged left cervical lymph nodes along the jugular chain. The findings correlated with osteomyelitis of the left mandibular condyle with a surrounding abscess and cervical lymphadenopathy.\nBased on the clinical findings, radiographic presentation and a history of trauma, we arrived at provisional diagnosis of chronic suppurative osteomyelitis of the condyle. A routine blood workup revealed an increased erythrocyte sedimentation rate (ESR: 40 mm/h) and raised total leukocyte count (12,000/cumm) suggestive of an infection.\nFine-needle aspiration was done from the dependent part in between the center and the lower border of the swelling with the help of a syringe. Blood-tinged pus was obtained on aspiration that was sent for cytological examination. Meanwhile, the patient was prescribed capsule amoxicillin (with Lactobacillus) 500 mg 3 T.I.D, tablet metronidazole 400 mg T.I.D and tablet diclofenac sodium 50 mg as needed for 5 days. However, there was no relief in the signs and symptoms after 5 days. On microscopic examination, the pus smear showed granulomas with caseous necrosis and dense lymphocytic infiltration. Areas of fibrosis and granulation tissue were also noted. These findings were suggestive of a chronic granulomatous infection raising a strong suspicion of TB.\nWe then advised further investigations such as Mantoux test and culture and staining of acid-fast bacilli. These tests were subsequently found to be positive. The patient was referred to the general physician for a detailed general medical examination, and we arrived at final diagnosis of tuberculous osteomyelitis of the left mandibular condyle.\nThe patient was advised anti-tubercular treatment (ATT) (INH 300 mg 1-0-0 and rifampicin 450 mg 1-0-0) for a 9 months duration and condylectomy.\nThe patient underwent left condylectomy under general anesthesia following strict aseptic condition. Hind's incision marking was placed followed by adrenaline saline infiltration done []. The skin incision was placed by BP blade number 15 and deepened to subcutaneous layer by electrocautery. Blunt dissection was carried out by the small curved artery, parotid gland was identified and reflected superiorly, masseter muscle identified and incised, periosteum reflected ramus and condyle exposed, osteotomy cut placed obliquely from sigmoid notch inferior-posteriorly below the neck of the condyle. Osteotomized condyle was disarticulated by the reflection of lateral pterygoid muscle. Perforation was noted in excised condylar head [].\nHistopathological examination of the excised condyle revealed the presence of granulomas with foci of caseous necrosis, numerous epithelioid histiocytes and Langerhans’ type of giant cells []. The histopathology report was compatible with our diagnosis of tuberculous osteomyelitis of the condyle.\nThe patient responded well to treatment and complete regression of the swelling was attained within 1 month [].\nDigital panoramic radiograph was advised after 4 months follow-up []. Despite a detailed medical examination no other systemic focus of infection was evident, and the patient was placed under long-term clinical follow-up. |
An 85-year-old female patient visited our hospital, complaining chiefly of scalp swelling. Two weeks earlier, the patient had fallen backward. Upon physical examination, we detected a subcutaneous mass in the patient's left posterior parietal area. The 3.5 cm mass was covered by normal skin. She also complained of a little pain on palpation. No tactile pulsation was detected. It was believed to be a posttraumatic subcutaneous hematoma, so we decided to continue observation, and wait for any spontaneous disappearance. The swelling in the patient's scalp decreased in size after one week. She attended no follow-up sessions thereafter.\nTwo months after her first visit, the patient was admitted to the emergency room due to scalp bleeding. The swelling in the patient's scalp had, at that time, increased to 4 cm in diameter with an overlying scalp defect which was oozing blood. Tactile pulsation was detected. The defect was sutured, and the bleeding was controlled via manual compression.\nThe patient was admitted for further evaluation of the pulsatile scalp mass. Brain CT revealed a slight increase in the attenuation of the hematoma in the left parietal scalp, with dense contrast filling (). Upon routine laboratory examination, the level of creatinine in the patient's blood was higher than normal (22 mg/l, normal range 7-14).\nAngiography of the external carotid artery revealed a pseudoaneurysm, which was fed by the left occipital artery (). An aortogram revealed complete occlusion of the right renal artery, as well as severe proximal stenosis in the left renal artery. The radiologist (Y.H.C.) attempted to occlude the pseudoaneurysm via transarterial embolization. However, this attempt failed, due to the complex geometry of the proximal artery. A two-hour attempt was made to occlude the pseudoaneurysm via US-guided manual compression. Thereafter, the pulsation of the scalp swelling became negligible.\nDoppler US, taken five days after the procedure, revealed thrombus formation in the pseudoaneurysm sac. We detected a residual blood flow to the inferior portion of the sac ().\nThe patient's scalp swelling again developed pulsation two days after the taking of the ultrasound. At this time, an attempt was made to occlude the pseudoaneurysm via direct puncture embolization (). The pseudoaneurysm was completely occluded by treatment with a 1:1 mixture of NBCA and iodized oil (Lipiodol; Laboratoire Guerbet, Roissy, France).\nNo regrowth of the scalp swelling or pulsation occurred. Complete occlusion was confirmed by Doppler US, which was taken eight days later (). The scalp swelling subsided, and the wound eventually completely healed. There was no regrowth of the scalp pseudoaneurysm on follow-up for six months after direct puncture embolization. |
A 55-year-old Caucasian woman was attended in the Service of Maxillofacial Surgery for evaluation and treatment of a right upper maxillary lesion affecting canine, lateral and central incisors (teeth #6, #7 and #8). Local clinical examination and on the rest of the orofacial structures was unremarkable. The radiographic study (Fig. ) showed a circular radiotransparency with sclerotic margins enveloping the apexes of canine, lateral and central incisors (teeth #6, #7 and #8) – all of which had undergone endodontic treatment several years ago. In the tomographic reconstruction images of the maxilla, the lesion had a low bone density appearance, close to the floor of the nasal passages and a slight bulging of the buccal and palatal cortical layer – the latter presenting discontinuous margins (Fig. ).\n- Differential diagnosis\nThe present lesion was identified on occasion of a radiographic study of the abutment teeth of maxillary dentures. The patient history and clinical examination yielded no data suggestive of the presence of an odontogenic abscess. No surgery had been performed in the area that would suggest a possible residual cyst or apical scar. The presumed diagnosis was therefore a radicular cyst associated to any of these teeth. On the basis of the location of the lesion, the X-ray study and diagnostic study images, the differential diagnosis included lateral periodontal cyst, keratocystic odontogenic cyst, ossifying fibroma, and other less common odontogenic tumors.\n- Diagnosis and treatment\nThe lesion was removed, with periapical surgery of the affected teeth ( #6, #7 and #8) under local anesthesia and conscious sedation. Surgical specimen measured 1 x 1 cm. The patient is currently under periodic clinical and radiological controls in order to detect any possible relapse (Fig. ).\n- Histopathologic analysis:\nAfter staining with hematoxylin-eosin, the specimen was examined under the light microscope at x40 and x100 magnification (Fig. ). The histological study revealed a cyst lined by hyperplastic squamous epithelium with a chronic inflammatory infiltrate of the fibrous wall and a series of squamous odontogenic cell proliferations forming rounded aggregates surrounded by the fibrous tissue. Based on these findings, a diagnosis of radicular cyst with proliferating squamous odontogenic epithelium was established. () |
A 61-year-old Asian woman was referred to our department with the complaint of painless loss of vision in the left eye. She had a history of hearing loss, headache, dizziness, nausea, and vomiting before the onset of ocular symptoms. Her ocular and medical histories were otherwise unremarkable. The patient’s initial best-corrected visual acuity values were 20/400 in the left eye and 20/20 in the right eye. Intraocular pressure values were normal in both eyes (15 mmHg in the left eye and 17 mmHg in the right eye; normal range, 10–21 mmHg). Both eyes had quiet anterior chamber, clear lens, and no cells in the anterior vitreous cavity. Light reflex was normal in the right eye, whereas it was sluggish in the left eye; the left pupil diameter was 5.5 mm. Fundus examination of the left eye revealed RD in the posterior pole, which involved the fovea. However, no retinal hole or retinal hemorrhage was found in the posterior pole or other regions of the retina. The posterior part of the right eye was normal.\nSubsequently, the patient underwent B-scan ultrasonography and fundus fluorescein angiography (FFA) examinations. B-scan ultrasonography of the left eye showed localized RD temporal to the optic nerve, which involved the macula; it also revealed choroidal thickening. The right eye exhibited normal findings (). FFA revealed stippled pinpoint hyperfluorescence in the upper macula in the left eye, which began in the early phase and lasted into the late phase (). Magnetic resonance angiography to exclude tumors in the brain or retina demonstrated negative results.\nOne week after initial presentation to our clinic, the patient experienced reduced visual acuity in the right eye (best-corrected visual acuity of 20/30). B-scan ultrasonography and optical coherence tomography examinations revealed obvious serous RD below the macula in both eyes ( and ). Thus, a provisional diagnosis of VKH was made, based on the patient’s systemic complaints of headache, hearing loss, and the ocular sign of bilateral RD. However, the patient did not exhibit integument signs, such as poliosis, vitiligo, or alopecia. Notably, 1 week of steroid treatment did not attenuate the RD.\nTwo weeks after initial presentation to our clinic, the patient had high blood pressure (166/93 mmHg) and slurred speech. Hematologic analysis then revealed an elevated leukocyte count (41.96 × 109/L; normal range, 4–10 × 109/L), which primarily constituted lymphocytes (64.4%; normal range, 20%–40%). Elevated numbers of leukocytes and tumor cells were found in cerebrospinal fluid. Furthermore, bone marrow biopsy revealed 77% primary atypical blood cells, 89% of which were immature lymphocytes. The patient was then diagnosed with acute lymphocytic leukemia and transferred to the Department of Hematology for further treatment. However, the patient and her family refused chemotherapy; she died within 6 months. |
A 17-year-old male was an unrestrained passenger in an automobile travelling between 60 and 70 mph. This car was the involved in a road traffic collision at an oblique angle with another vehicle. The patient in question was a rear seat, nearside passenger.\nAt the scene of the collision, the passenger-side front seat trapped the patient for approximately 1½ h. Once released the patient was then transferred by helicopter to a local trauma center.\nUpon arrival, the patient was stable despite tachydcardia with Glascow coma scale of 15/15. Primary and secondary surveys were performed, and trauma team noted open wounds in both right forearm and left thigh. There were bruises and patient was complaining of pain and difficulty in using his jaw. The abdomen was soft and normal oxygen saturations were maintained. The extremities were neurovascularly intact distally, and the patient was complaining of pain in the right leg.\nHe had been administered opiate analgesia, oxygen and tranexamic acid. Once stabilized the patient was transferred for an urgent computer aided tomography (CT) scan, which revealed the following injuries: Subcondylar fracture of the mandible, open fracture of the right ulna and radius, open fracture of the left femur, inferior dislocation of the right hip joint, small splenic laceration, and contusion of left pulmonary base.\nThe same day as admission the patient was taken to the operating theater for reduction under general anesthesia of the inferior dislocation of the right hip joint. With hip abduction traction, external rotation and flexion of the hip joint multiple attempts were made to reduce the hip joint, but unsuccessfully. It was therefore considered necessary to attempt an open reduction of the inferior dislocation. An anterior approach was used and the hip capsule opened anteriorly. The acetabulum was empty and the ligamentum teres ruptured. The femoral head was stuck within the obturator region; inferior to the transverse acetabular ligament. Cuffing of the anterolateral and superior portion of the femoral head was noted. The capsular attachment was torn and buttonholed. The femoral head was reduced into the acetabulum using a bone hook on the neck of the femur. Satisfactory reduction was achieved; several further procedures were also carried out at this visit to theater.\nThe patient kept immobilized with weight bearing instructions on both hips for 6 weeks and without any skin traction. The patient had IM nailing of the contralateral femur and associated ipsilateral foot fracture treated conservatively. Upon follow-up reviews, the patient is progressing well with no hip pain although weight-bearing status is limited by other injuries sustained. A 3 month follow-up X-ray was satisfactory, and there were no reports of re-dislocation or signs of avascular necrosis of the head. |
We present a case of a 66-year old male with prior history of prostate cancer (status post prostatectomy) in remission, CKD stage V status post transplanted live donor kidney (in the setting of ESRD secondary to oxalosis) who presented to the hospital with severe right eye pain, proptosis, and pressure for three days. Three months prior he had recurrent headaches and sensation of fullness in the maxillary sinuses. He sought medical attention after his first episode of large volume epistaxis. On inspection of the nares by otoscope a large mass was seen on the right side. CT noncontrast revealed destructive infiltrative mass in the right nasal cavity eroding through the right lamina papyracea into the right orbit (see Figure ). The mass was biopsied by ENT via a flexible nasopharyngoscopy. Pathology revealed mucosal melanoma. He underwent a positron emission tomography (PET)-CT which showed the primary lesion within the eye/nose with indeterminate nonavid lesions in the left proximal humerus, upper thoracic spine, and liver. Distant metastases could not be excluded. Nuclear medicine bone scan was performed which showed multiple foci of increased activity likely representing metastatic disease within the lateral left fifth rib as well as the third, eighth, and eleventh thoracic vertebral bodies (see Figure ). The patient was evaluated by a multidisciplinary team consisting of oncology, radiation-oncology, and ENT as patient’s symptoms continued to worsen. A CT maxillofacial scan showed increase in size of the mass since prior imaging. The intraorbital component measured approximately 3.5 cm x 1.5 cm which was increased from prior measurements of 2.7 cm x .8 cm (see Figure ). The mass now completely obstructed the right nasal cavity and was eroding the nasal septum; along with leftward deviation. Worsening proptosis of right globe was noted. Radiation oncology recommended palliative course of radiation to the orbital/nasal mass to help with controlling his pain and prevent further worsening of his eye site. He was not considered a surgical candidate to the extent of tumor involvement.\nThe glomerular filtration rate (GFR) for his transplanted kidney was 15. This was due to chronic rejection and progressive fibrosis despite an adequate immunosuppressive regimen. The standard of care would be using the combination of ipilimumab and nivolumab concurrent with the course of RT. Given the potential for worsening renal function we opted to start with a single agent, nivolumab and palliative radiation. Consideration to start ipilimumab would be made based on how the patient tolerated nivolumab. As planned, he completed five fractions of radiation and was started on nivolumab 480 mg IV monthly along with denosumab for the management of hypercalcemia and pathologic bone fractures. Mutational testing for V600E BRAF was negative. Analysis revealed NRAS mutation with DNA change at position 182 adenine to guanine and amino acid change p.Q61R (Glutamine61Arginine). After his second round of nivolumab therapy the patient developed renal failure with transplant rejection requiring permanent hemodialysis. Four months in therapy the patient did have significant improvement in tumor load regression. Ipilimumab has not yet been started. |
A 35 years old (height, 159 cm; weight, 85 kg; gravida 3, para 1) pregnant woman with SAS presented to our hospital and was scheduled to undergo elective CS under epidural anesthesia at 36 weeks of gestation. An echocardiographic examination at 34 weeks of gestation revealed that the diameter of the beginning portion of the aorta (under the aortic valve) was narrowed by about 17 mm. The diameter of the ascending aorta was normal, the average pressure gradient was 40 mmHg, and the ejection fraction was 67%.\nIn the operation room, her baseline vital signs were as follows: heart rate (HR), 82 bpm with normal sinus rhythm; noninvasive blood pressure (BP), 130/76 mmHg; and oxygen saturation (SpO2), 96%. Supplemental oxygen was administrated by a face mask at the rate of 3 L/min. Traditional epidural puncture was performed at the L1–2 and L3–4 interspaces in the left lateral position, and a catheter was inserted to a depth of 4 cm into the epidural space at the cranial and caudal ends of the surgical field. The patient was then placed supine in a left-tilt position; 3 mL of 2% lidocaine was injected as a test dose, and another 7 mL was injected 5 min later through the cranial catheter. Additionally, 3 mL of 1.5% lidocaine was injected through the caudal catheter. At 20 min after the lidocaine injection, the sensory block had reached T6. The patient had no discomfort, and her hemodynamic parameters were stable. The operating bed was adjusted from the left-tilt to horizontal position, and the operation was allowed. Two minutes later, the patient reported chest distress and difficulty breathing (HR, 110 bpm; BP, 80/69 mmHg; SpO2, 96%), and 3 mg of intravenous ephedrine was promptly administered. The patient immediately lost consciousness with no response (HR and BP were depressed to 40 bpm and 53/15 mmHg, respectively). The lowest BP at the time was not measured because the noninvasive BP measurement interval was set to 1 min. The obstetricians sped up the surgery, and in another 2 min, a 2750-g male newborn was delivered with an Apgar score of 10 at both 1 and 5 min. Spontaneous circulation of the patient was returned immediately after delivery, and stable vital signs returned with no other treatment. The postoperative course was uneventful. The mother and neonate recovered uneventfully and were discharged 4 days later. |
A 36-year-old Caucasian male presented to the emergency room complaining of a one-day history of abdominal pain. His main symptoms were that of nausea and vomiting, but he also reported periods of diarrhea. In the emergency room, his initial evaluation was significant for lab studies demonstrating a mild metabolic acidosis with a bicarb of 16.6 mEq/L, an elevation of his creatinine to 1.93 mg/dL, and a serum lactate level of 5.4 mmol/L. A computed tomography (CT) scan of the abdomen and pelvis was obtained and reviewed. The appendix was thought to be normal. There was no evidence of free intraperitoneal air, abscess, or volvulus. There did appear to be evidence of enterocolitis involving the ilium, cecum, and the proximal ascending colon. There was no evidence of pneumatosis or obstruction. Over the course of 12 hours the patient underwent conservative medical management, which included intravenous hydration, intravenous steroids, Toradol and Dilaudid for pain management, and the occasional dose of Ativan for agitation. Failure of conservative management along with medical decline lead to an exploratory laparotomy with a right hemicolectomy for an ileocolic intestinal infarction. Postoperative repeat CT scan of the abdomen incidentally showed pulmonary nodules in the lower lobe. A CT scan of the chest was then obtained, which revealed air in the left chest wall and axilla (Figure ). On physical exam there was very subtle mottling of the left shoulder. The patient was emergently taken back to the OR for further exploration of the shoulder with excisional debridement. Seropurulent fluid and extensive subcutaneous emphysema along the fascial planes of the left chest wall were consistent with necrotizing fasciitis. Both wounds were packed with a gauze bandage roll soaked in saline and then the patient was transferred back to the intensive care unit.\nOver the course of 15 days the patient underwent repeat trips to the OR for re-exploration, incision and drainage of the wounds, and dressing changes (Table ). On postoperative day 17, the wound, ostomy, continence nurse (WOCN) was consulted to evaluate the wound and make treatment recommendations.\nOn exam, there was still a significant amount of nonviable tissue in the base of the left chest and axilla wound. The left chest wound measured 8 x 19 x 8 cm and the left axilla wound measured 6.5 x 25 x 4 cm. NPWTi-d with ROCF-CC was applied to these wounds to aid in the removal of the nonviable tissue. Figures - show the presentation of the wounds prior to the application of the NPWTi-d with ROCF-CC.\nDuring the application of the NPWTi-d with ROCF-CC, care was taken to ensure that the contact layer of the dressing was in contact with the entire wound base. Barrier rings were applied to the skin creases along with any area that presented as a high risk for inadequate seal of the drape (Figure ). The therapy settings for the left axilla were 85 ml of hypochlorous acid solution (Vashe®, SteadMed, Fort Worth, TX), dwell time of 10 minutes with NPWT every hour at -125 mmHg. Therapy settings for the left chest wound were 50 ml of hypochlorous acid solution, dwell time of 10 minutes with NPWT every hour at -125 mmHg.\nDuring the first dressing change after the application of the NPWTi-d with ROCF-CC, there were notable improvements in the quality of the tissue in the wound bed (Figure ). The left chest wound bed was noted to have 100% red healthy tissue and the left axilla wound bed had a decrease in nonviable tissue. There was, however, seropurulent drainage noted deep within the intramuscular structures of the left axilla wound.\nNPWTi-d with ROCF-CC was reapplied to the axilla wound ensuring that the contact layer was placed deep into the wound bed to facilitate the removal of the seropurulent drainage. The left chest wound was transitioned to NPWTi-d to continue to promote granulation tissue (Figure ). The instillation solution was switched to normal saline due to blockage alarms we encountered with the hypochlorous acid solution without resolution.\nThe current treatment regimen was continued for two days until the next dressing change. At that time both wound bases were 100% red with granulation tissue present (Figure ). The seropurulent drainage deep in the intramuscular structures was no longer present. Since there was adequate removal of nonviable tissue and the quality of the tissue had significantly improved, the WOCN made the decision to transition both wounds to NPWTi-d instilling normal saline solution with a dwell time of 10 minutes and NPWT every two hours at -125 mmHg.\nFor the next two weeks the treatment regimen utilizing NPWTi-d to the wounds continued. The dressings were changed three times a week at the bedside by the WOCN. During initial assessment, the axilla wound measured in total volume 650 cm³ and the chest wound measured 1,216 cm³. After 17 days utilizing the combination of NPWTi-d with and without ROCF-CC, the axilla wound measured in total volume 342.25 cm³ and the chest wound measured 554.4 cm³. This presents a significant decrease in overall measurements. At this time, NPWTi-d was discontinued and standard NPWT with black granufoam was utilized to both wounds at -125 mmHg (Figures -).\nNPWT was utilized for approximately a month to promote granulation tissue and assist with wound contraction (Figures -). Once the wound had made vast improvements, NPWT was discontinued and a non-adherent antimicrobial alginate dressing (Silvercel™ Non-adherent, Acelity, San Antonio, TX) was lightly packed into the wound bed and covered with a dry sterile dressing. This dressing was then changed three times a week by a visiting nurse association. The wound measurements prior to discharge showed significant improvement in comparison to the wound measurement at presentation, with the left axilla wound measuring 1.5 x 10.5 x 0.3 cm and the left chest wound measuring 3.5 x 9 x 5 cm. |
This is a case report of a 46-year-old female with a past medical history notable for depression, asthma, and uterine leiomyomas who presented to an urgent care with 5 days of progressive abdominal pain, bloating, nausea, and subjective fevers. The patient endorsed a several month history of gaining weight, though she attributed it to her lifestyle, accompanied with strong, intermittent, crampy right lower quadrant pain. The pain was random in onset and would dissipate very quickly. However, 5 days prior to presentation, her pain dramatically increased and was persistent in nature.\nHer surgical history included a myomectomy performed 5 years prior followed by a laparoscopic hysterectomy 2 years later. She was a regular drinker, consuming four to five alcoholic drinks per night, but stopped when her symptoms worsened and had no history of withdrawal. Family history was significant for a maternal grandmother with breast cancer, mother with skin cancer, and an uncle with colon cancer.\nUpon arrival to the emergency room, the patient was tachycardic, mildly hypotensive, and febrile to 103 °F. Her abdomen was soft, distended, and diffusely tender without peritonitis. Labs were notable for a leukocytosis of 15 K. CT of the abdomen and pelvis demonstrated moderate volume, complex fluid within the abdomen and pelvis with extensive amount of gas and peripheral rim enhancement, a thickened appendix filled with fluid, and a soft tissue/cystic lesion in the anterior abdominal wall (Fig. ).\nDifferential included pseudomyxoma peritonei with a ruptured appendiceal mucocele versus PMP secondary to an adnexal ovarian neoplastic pathology with an infectious component. Per the radiology report, the origin was unclear based upon imaging and stated a ruptured adnexal cyst should be considered given the markedly enlarged septated cystic lesions in the pelvis. The patient was resuscitated, and her blood pressure and heart rate normalized with 2 L of fluid. Given the patient’s hemodynamic stability, the decision was made to attempt conservative management with antibiotics and interventional radiology (IR) biopsy and drainage.\nOn the floor, the patient remained hemodynamically stable with intermittent fevers. Her abdominal exam also remained unchanged, with persistent pain that was under control with pain medication. It was unclear if the abdominal wall mass was neoplastic, and given our initial nonoperative approach, a biopsy was performed for tissue diagnosis to properly guide further treatment. A core needle biopsy of the abdominal wall mass demonstrated a uterine leiomyoma implant, and the cytology aspirate of the peritoneal fluid showed pools of acellular mucoid material. On hospital day 7, the patient’s leukocytosis rose to 23 K, and repeat imaging demonstrated a more organized collection with intraperitoneal air in addition to multiple thick, wall-enhancing, complex cystic multiseptated lesions. Given these findings on imaging as well as her clinical presentation, the patient was taken to the operating room (OR) jointly with surgical oncology and gynecology oncology for a peritoneal washout and bilateral salpingoopherectomy. Intraoperative findings included a large amount of mucin in the abdomen and pelvis with a pocket of purulent fluid in the mid-abdomen, a very large (12 cm) cystic left ovary, dilated right fallopian tube with enlarged right ovary, a very dilated and thickened appendix, and dense, diffuse small bowel intraabdominal adhesions (Fig. ). Her peritoneal cancer index (PCI) score was unable to be determined due to the degree of inflammatory adhesions. She underwent bilateral salpingoopherectomy given the amount of inflammation in the cecum and base of the appendix, the decision was made not to perform an appendectomy to avoid leakage at the staple line and further infectious complication. The patient’s abdomen was hostile, and no larger operation was deemed necessary at this time, as the patient was nonobstructed and the origin of PMP was not yet verified. The primary goal of the operation was to clear the infection, with the intent to return at a later date when her intraabdominal contents could be properly mobilized for a complete PCI score and an appropriate oncologic resection could be performed.\nHer hospital course was complicated by recurrent pleural effusions requiring multiple thoracocenteses, negative for cytology, and persistent need for supplemental oxygen. She also developed a secondary intraabdominal abscess requiring IR-guided drainage. After the patient clinically improved, she returned to the OR 2 months after her initial presentation for completion cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) with 40 mg of mitomycin-C at a target temperature of 42–43 °C over 90 min, as per our institutional protocol []. Her PCI at the second surgery was calculated to be 13. She had minimal peritoneal adhesions, and her intraabdominal infectious process had completely resolved. The patient underwent an appendectomy, omentectomy, and tumor debulking. The appendicular base was healthy and easily stapled across. The patient was discharged home with lovenox on postoperative day 5, saturating well on room air, tolerating a diet with oral pain medication, and with return of bowel function. Six months after the CRS/HIPEC, the patient had surveillance imaging with no evidence of recurrence (Fig. ). The abdominal wall mass was unchanged from prior imaging and, given the biopsy of leimyoma, presumed to be an implant at the port site from the patient’s prior hysterectomy.\nAfter the discharge from the second surgery, the patient’s postoperative recovery was complicated by new-onset shortness of breath after the completion of her 30 day course of lovenox. She was diagnosed with bilateral pulmonary emboli, with no evidence of deep vein thrombus on lower extremity duplex. Therapeutic lovenox was initiated, and her symptoms gradually improved. An interval angio CT of the chest demonstrated resolution of the clot. Anticoagulation has since been discontinued, and the patient will continue with 6-month interval surveillance imaging for PMP recurrence.\nThe pathology of the left ovary and tube following the first operation resulted as an ovarian cyst containing mucin pools and low-grade intestinal type mucinous glands, consistent with metastasis from appendiceal mucinous neoplasm. The right ovary and tube showed mucin, acute inflammation, and adhesions. Immunostains were positive for CK7, CK20, and CDX2 and negative for PAX8 (Fig. ). Following the completion cytoreduction, the appendiceal pathology demonstrated a low-grade appendiceal mucinous neoplasm (LAMN) with acellular mucin outside the appendix and associated fibroinflammatory response. The proximal margin, the appendicieal base, was negative for tumor. Additional specimens sent during the debulking included the falciform ligament, omentum, pelvic tumor nodules, and tumor deposits overlying the sigmoid, small bowel, left colon, and liver. All additional specimens consisted of acellular mucin. Pre-operative carcinoembryonic antigen (CEA) and CA-125 were elevated at 26.8 and 101 respectively. CA 19-9 was within the normal limits. Using AJCC (8th ed.) staging, the final staging was determined to be T4a NX M1a (stage IVA). Since the patient had a complete cytoreduction and the pathology was determined to be LAMN, no further adjuvant therapy was given. |
A 79-year-old male with a medical history of cholelithiasis, for which he underwent cholecystectomy 11 years ago, hyperlipidemia, essential hypertension, and paroxysmal atrial fibrillation was admitted for mild acute pancreatitis. His presentation included severe epigastric pain radiating to the back, lipase of 1840 u/L, and no organ failure. He denied fever, chills, or body aches. The patient denied alcohol consumption and reported to have quit smoking 41 years ago. Liver chemistries, serum bilirubin, serum triglycerides, and immunoglobulin subclasses were within normal limits (). Abdominal CT scan demonstrated peripancreatic haziness consistent with noncomplicated acute pancreatitis (). Home medications included atorvastatin for several years and the addition of 160 mg fenofibrate six months prior to admission. These medications were held upon admission but resumed at discharge. Full medication lists on admission and at discharge are shown in (). The patient was managed with fluid resuscitation and pain control. The patient recovered well and was able to tolerate regular diet without any pain or nausea. The patient was discharged in a stable condition after three hospital admission days, and lipase level at the day of discharge was 307 u/l.\nThree days later, the patient returned with similar symptoms. Lipase levels were >30,000 u/l (), and magnetic resonance cholangiopancreatography showed acute interstitial edematous pancreatitis (). The patient was treated conservatively with intravenous fluid resuscitation, pain control, and nothing per mouth until his symptoms resolved. Four days after his second admission, the patient recovered well and was discharged home in a stable condition. Fenofibrate and atorvastatin were discontinued ().\nTwo months later, the patient remained asymptomatic and returned for further workup to rule out an alternative cause to fenofibrate-induced pancreatitis. Endoscopic ultrasound (EUS) was done, which showed a 12 mm x 20 mm pancreatic head mass without pancreatic duct stenosis, strictures, or dilation. There were no common bile duct abnormalities. Fine needle aspiration was performed, and cytology was negative for malignant cells. Endoscopic retrograde cholangiopancreatography (ERCP) was performed to place a prophylactic pancreatic duct stent. Cholangiogram revealed a normal biliary system without dilation or strictures. The biliary tree was swept, and nothing was found. A prophylactic pancreatic duct stent was placed and a prophylactic sphincterotomy was performed. No recurrence of pancreatitis has occurred as of 6-month follow-up.\nGiven the course of the patient's illnesses in relation to fenofibrate usage, the timespan of drug initiation, and the fact that he had been taking atorvastatin for many years without previous signs or symptoms of acute pancreatitis, we hypothesize that his recurrent disease is probably due to the use of fenofibrate. |
An 11-year-old girl, who was regularly followed up with CF, presented to our intensive care unit with an extended subcutaneous emphysema (left-sided facial, neck, upper third of the chest). CF was diagnosed during the neonatal period after surgical treatment of her meconium ileus. A day before her neck became suddenly painful, her face swelled and she mentioned severe thoracic pain associated with coughing. On auscultation, decreased breath sounds and quiet heart sounds were noticed. There was no shortness of breath; however, she was mildly tachypneic with transcutaneous oxygen saturations in the low 80%. She required oxygen support through nasal prongs to maintain her oxygen saturation above 90%. The chest X-ray showed pneumomediastinum (\n.). On the third day of hospitalization, she became tachypneic again; her oxygen requirement and the size of subcutaneous emphysema increased (neck, chest, and abdominal circumference increased, subcutaneous emphysema became palpable on the arms and the whole trunk); she felt like “a pillow pushing her back.” An urgent chest CT scan was performed, which showed extensive subcutaneous emphysema and a huge pneumomediastinum (\n). She suddenly developed increased respiratory distress, tachycardia, and severe thoracic pain; therefore, an emergency needle mediastinal thoracocentesis was done in the second intercostal space at the midclavicular line on the left side of the thorax with application of local anesthesia. She immediately felt better but this intervention resulted in temporary improvement only. Based on the CF, the possibility of lung injury as a triggering mechanism was raised and discussed with the surgical team. As an emergency procedure, two mediastinal drains were placed through the jugulum under general anesthesia (\n) that were put to continuous suction with 5 to 10 H\n2\nO cm. Her symptoms significantly improved after a few hours and the subcutaneous emphysema decreased considerably. On the first postoperative day, the control chest X-ray described a 2 to 2.5 cm mantle PTX; therefore, a left-sided chest drain was inserted. Her general condition slowly improved, the subcutaneous emphysema decreased gradually, and the PTX resolved. Eleven days later, the chest CT scan was repeated (\n), which showed a significantly smaller pneumomediastinum. At that time, a Heimlich valve was connected to the mediastinal drains, and after 5 days, on the 26th postoperative day, the drains were removed. The patient was transferred to our pulmonology ward, where, in addition to gradually restored inhalation treatments, mucus dissolution, anti-inflammatory therapy, and physiotherapy were commenced. Her oxygen requirement gradually decreased and she was discharged home after 48 days of in-hospital care. |
A 59-year-old man visited the clinic of Dankook University Hospital on 2 March 2016 with the chief complaints of frotteuristic behavior in a public place, a continuous increase in sexual desire, and sexual molestation involving compulsory physical contact that started in 2007. The patient had been diagnosed with paraphilic disorder, intermittent explosive disorder, and borderline intellectual function three years prior by a psychiatrist. The patient had received three years of treatment at the National Forensic Hospital due to a sexually violent crime and uncontrolled paraphilic behavior. In 2014, the patient was arrested by the police for forced sexual molestation by kissing a woman and touching her while sleeping in a sauna facility. The patient had often lied to their family since high school, often went away, and was unemployed. He reported that he had been prostituting twice a month for sexual desire relief. The treatment of the patient was approved by the Institutional Review Board of Dankook University Hospital. A psychiatrist met him face to face, administered a full verbal explanation and written document about the purpose and procedure of this study, and received informed written consent to participate voluntarily. According to the guidelines of the World Federation of Societies of Biological Psychiatry (WFSBP), we rated the patient as Level 5 because of his high risk of sexual assault crimes and a high degree of paraphilic traits after release. We evaluated the patient’s state more seriously than Stage 4, which is the general sex hormone oral administration stage, and administered the GnRH injection according to these guidelines.\nThe patient had been diagnosed with Graves’ disease one year prior and had been taking 75–100 mg/day of propylthiouracil (PTU). In addition, the patient had been diagnosed with osteoporosis 10 years prior and had been taking 70 mg of alendronate weekly and 600 mg calcium/400 IU vitamin D 400 twice a day. The patient had no other disease relevant to his internal medicine or neurology. The patient did not drink and had stopped smoking two years ago. At the physical examination, his height was 164 cm, his weight was 65 kg, and his body mass index was 24.17 kg/m2. In the cardiovascular examination and complete blood count/liver function test examination, no abnormal findings were observed. The values of his hormonal test and thyroid function test at baseline and 6 and 12 months were shown in . His Luteinizing hormone (LH) and follicle-stimulating hormone (FSH) concentrations were increased above the reference level. The level of serum c-telopeptide was within the normal range, which is thought to be suppressed by the administration of alendronate. The baseline thoracolumbar spine X-ray showed degenerative spondylosis, diffuse osteopenia without compressive fracture, and his bone mineral density (BMD) showed osteoporosis ().\nFor the treatment of Graves’ disease, we changed the daily administration of 100 mg PTU to a daily administration of 10 mg methimazole (MMI), which gradually decreased to a daily 2.5 mg according to the result of the thyroid function test (TFT). For the treatment of the patient’s osteoporosis, we educated the patient about appropriate calcium/vitamin D intake and exercise and prescribed a weekly administration of 70 mg alendronate and the daily administration of calcium/vitamin D 500 mg/1000 IU (). We performed alendronate, calcium/vitamin D treatment at the same time as the Leuprorelin depot administration.\nAfter the leuprorelin injection, he reported that his sexual desire was too low. He also reported that he was not interested in sex and that he did not watch any sexual videos, and sexual erections also decreased to 1–2 instances per month. To evaluate the effect of treatment, the patient was assessed with the Clinical Global Impression (CGI), Sex Addiction Screening Test (SAST), and Wilson Sex Fantasy Questionnaire (WSFQ) at baseline and six months and one year after the leuprorelin administration, respectively. The patient was administered with 3.75 mg of long-acting leuprorelin first on 8 March 2016 and every four weeks. In addition, supportive psychotherapy was also implemented for the patient every two weeks. The sexual interest/activity and CGI severity/improvement were evaluated with the sex offender at the baseline state, month 6, and month 12 after starting the Leuprolide injections. The CGI severity score was 5 at the baseline state, 2 in month 6, and 1 in month 12. The CGI improvement score was 1 at month 6, and 3 in month 12 (). The total score for the SAST was 13 in the baseline state, 2 in month 6, and 1 in month 12, and WSFQ was 13 in the baseline state, 2 in month 6, and 1 in month 12 ().\nHis testosterone level was below 0.25 ng/mL after the administration, and it remained at a low level at six months and one year. The weight and blood pressure were continuously within normal limits until one year after the administration of the injection. In the follow-up blood test, the hemoglobin level was 11.4–12.4 mg/dL, indicating mild anemia, and the glucose and lipid levels were found to be within normal limits. The values of bone marrow density (BMD) after 6 and 12 months compared to the baseline were −4.7% and 0% for L1–4, −1.9% and −0.4% for the femur neck, and −6.1% and +4.6% for the total femur, respectively (coefficients of variation 1.0%, the least significant change >2.77%). The serum CTx level increased to 0.084 ng/mL and 0.155 ng/mL after 6 and 12 months, respectively. The serum 25(OH) D level was 27.5 ng/mL after 6 months, but it decreased to 11.0 ng/mL after 12 months. This change was thought to be due to the seasonal effect of less sunshine. Then, we increased the dose of vitamin D to 2800 IU weekly.\nThe patient has not experienced a fracture for more than one year after the administration of the injection, and the weekly administration of 70 mg alendronate and the calcium/vitamin D supplement have been continuously maintained. During the one-year period after the administration of the injection, the patient did not experience any side-effects of the drug (e.g., worsening of osteoporosis and liver function disorder). After the administration of the leuprorelin depot, the patient reported that he was able to have an erection (0–2 times/month), but ejaculation was impossible, possibly due to the effect of this drug. In addition, after drug administration, the patient exhibited a rather feminine appearance change, such as smooth skin and a soft facial contour. There was also an emotional irritation, which was observed in the early stage of the outpatient treatment, and the treatment compliance was also appropriately maintained.\nThe patient provided written informed consent for inclusion before he participated in the study. The study was conducted in accordance with the Declaration of Helsinki, and the protocol was approved by the Ethics Committee of Dankook University Hospital (Project identification code: DKU 2015-12-014). |
A 48-year-old woman was admitted to hospital due to claudication at 250 to 300 meters and tremor in both legs. During history-taking, she reported periodic headaches and vertigo complaints lasting years, she described that she had tremors and muscle weakness during her childhood, she had had one lost pregnancy, and her mother does not have diabetes. She has no history of smoking. Family history for cardiovascular disorders was negative and she explained that her mother’s family has a history of diabetes mellitus. She indicated that her father was hypertensive. However, the patient's own blood pressure was normotensive (120/70 mmHg). Assessment of the patient’s physical and ethnic characteristics determined that she was of Turkish origin (European), 52 kg in weight, and 1.63 m in height. Ankle brachial indexes were calculated as 0.72 on the right side and 0.78 on the left side. Biphasic and monophasic waveform patterns were detected bilaterally in distal (trifurcation arteries) lower extremities with Doppler sonography. Complete blood count and biochemical test results were studied to evaluate systemic risk factors. However, whole blood test results were within normal ranges (). Thereafter, computed tomography angiography (CTA) was planned for the lower extremities, as well as for the cerebrovascular system because of tremor and cerebrovascular complaints. Cerebrovascular CTA revealed bilateral carotid occlusion at the level of supraclinoid segments, and opacifications were detected at the distal segments of bilateral anterior cerebellar and middle cerebellar arteries (). Additionally, abundant collateral formations (puff of smoke sign) were detected in the cerebral parenchyma and basal ganglia (). Lower extremity CTA revealed bilateral segmental occlusions of both popliteal arteries (). The patient was diagnosed with moyamoya disease, and anticoagulant and antiaggregant treatment was started at a dose of 2×1 6000 IU enoxaparin sodium for the first ten days and 300 mg/daily acetylsalicylic acid. Additionally, subsequent 60 mg daily prednisolone treatment was started because of insufficient exclusion of vasculitis. There are no strict guidelines on when to obtain ethical clearance for writing and publishing a case report. We therefore obtained signed informed consent from the patient. |
A 65-year-old woman was referred by her dentist to the emergency department of Spedali Civili Brescia, Italy, with a large swelling of the face and the neck that commenced at the upper left eyelid. Pain was not aggravated on palpation, but dysphagia, dyslalia, and subcutaneous crepitus were evident. An intraoral examination revealed no visible incision; slight bleeding was apparent around an implant in the region of 34. She reported that she had undergone an airflow procedure performed by a dental hygienist in the region of the implant earlier the same day and had felt pain in the left submandibular region during the procedure. She was diagnosed with subcutaneous emphysema (procedural complication).\nHer vital signs were as follows: heart rate 65 beats per minute; blood pressure 145/90 mmHg; respiration 19 breaths per minute; and oxygen saturation 96%.\nTo evaluate the dysphagia and dyslalia, we obtained CT from the maxillofacial region to the thorax. These revealed significant soft-tissue emphysema extending from the left parietal region to the left soft tissue of the face, then bilaterally to the paraspinal muscles of the neck and the pterygoid regions, and posteriorly to the pharynx.\nMore distally, the emphysema splayed the vascular bundle and the thyroid lobes, widened the pectoral muscles posterior to the clavicles, descended to the mediastinum (where it was evident principally in the anterior part of the perivascular adiposity), and surrounded the trachea and oesophagus posteriorly.\nThe emphysema terminated in the region of the upper diaphragm (Figures –).\nTo prevent the expansion of the emphysema, the patient was immediately hospitalised in the Maxillofacial Surgery Unit and was prescribed intravenous antibiotics because of the high risk of infection associated with the access of large amounts of air and water to soft tissue during a dental procedure. Indeed, dental compressed air, and not sterile water, contains Legionella and Pseudomonas, rendering antibiotic therapy and microbiological monitoring even more critical [–].\nThe patient was discharged 4 days later. |
A 41-year-old male presented to the surgery outpatient department in Goa with a 2 day history of painful swellings on the left side of his neck with low-grade fever, malaise, and muscle pain of one day duration. There was no history of accompanying chills and rigor or rash. There were no ear, nose, and throat (ENT) or tooth complaints. There was no history of night sweats, loss of weight and loss of appetite. His past medical and family history was unremarkable. He had no history of tuberculosis (TB) or TB contacts. There was no recent history of foreign travel, insect bites, exposure to cats or other animals.\nOn presentation, his general condition was good, with an axillary temperature of 38°C. On physical examination, he had multiple lymph nodes located in posterior triangle of the neck on left side; the largest being approximately 2 × 1.5 cm in diameter. The lymph nodes were tender, mobile and firm in consistency with a smooth surface. He did not have any other site of significantly enlarged lymph nodes. Thyroid gland was not palpable. ENT examination was normal. Physical examination revealed no other abnormalities. A cervical ultrasound scan confirmed the examination findings and also demonstrated few subcentimetric lymph nodes in the posterior triangle of the neck on right side. Abdominal ultrasound revealed no abnormalities. He was given a course of oral amoxicillin/clavulanate combination, antipyretics, analgesics and was reviewed at the end of a week. There was no change in the symptoms or size of the lymph nodal mass. Hence, a fine-needle aspiration cytology (FNAC) was performed. But the cytological features were inconclusive.\nThe patient was referred to the medicine department with fever increasing to around 40°C. He was advised certain investigations and prescribed another course of antibiotic for 5 days. With this treatment, fever subsided but joint pains appeared after 2 days, first in the wrists and fingers followed by shoulders on both sides. The joint swelling was more at the wrist and fingers. Laboratory investigations revealed normal blood cell counts. The peripheral smear was normal without evidence of atypical lymphocytes. His erythrocyte sedimentation rate (ESR) was 18 mm/hr. Urine analysis and serum uric acid was normal. Search for malaria parasites, antinuclear and double-stranded DNA antibodies, and rheumatoid factor was negative. Serologic tests for hepatitis A, B, and C, human immunodeficiency virus (HIV) and syphilis were negative. The serology was negative for both dengue IgM and IgG antibodies (immunochromatography). The chikungunya IgM antibody was not detected by serum IgM ELISA. The purified protein derivative (PPD) test was negative. There was no evidence of focal lesion on chest radiograph.\nAfter 5 days the lower extremity joints were involved with profound swelling and limitation of movement; the swelling being more at the ankle joint []. He also experienced severe degree of myalgia in the thigh region. Patient was started on non-steroidal anti-inflammatory drugs (NSAIDs) and local application of magnesium sulfate salt. With this treatment the swelling and pain significantly decreased. But the lymph node swelling on the left side persisted and one more lymph node swelling appeared on the right side []. Thus, an excision biopsy was performed, with removal of this enlarged cervical lymph node. Histopathological examination of biopsy revealed necrotizing lymphadenitis with reactive hyperplasia. There was no evidence of histiocytosis, tuberculoid granuloma, or malignancy. A diagnosis of chikungunya was made based on history elicited by the patient and clinical findings. The arthralgia and myalgia persisted for a total period of 3 months following acute infection. |
The second case was a 70-year-old married woman who exhibited wearing off and was depressed after her close friend passed away. Shortly thereafter, she had an injury to her arm caused by a fall 6 months prior to CBT. Her motto was to always be cheerful and to not express her negative feeling toward others. Before she had depression, she regularly went to the gym for social interaction, as well as rehabilitation to maintain her physical ability. This particular participant was dealing with her depression by keeping it secret. We identified her thoughts (“depression is a sign of weakness” and “my friends will forcefully attempt to explore my situation if I share my concerns with them”) and worked to create alternative thoughts by considering several key questions (“If I saw a friend who had the same issue, what would I say to the person? Is there any way to look at the person other than with pity?”). We also created a conversation plan that she would follow with these friends (“If somebody asks how I have been lately, I could simply say, ‘I have not been feeling well for a while,’ and if these friends attempt to explore in more detail, I also could simply reply, ‘I will tell you when I feel I am ready’”). Nevertheless, she hesitated to meet with people and even found that sports gear (as she had previously enjoyed athletics and the gym) triggered her fear of meeting with those people. Thus, we constructed a detailed behavioral plan for actually going to meet with friends at the gym and started the process with small steps in which she gradually gathered the sports gear. Afterward, she experimented with the conversation skills acquired during CBT at a sports gym where many of her friends gathered. With this exercise, she learned that her friends’ perception of her did not change after her depression, and she felt safe to interact with them even though she had depression. Therefore, the identification of an appropriate behavioral experiment was an important step in improving the patient’s QOL. Overall, this patient responded favorably to therapy (GRID-HAMD score decreased from 15 to 2). |
A 76-year-old woman was admitted with abdominal pain and fever. She had been postmenopausal for 28 years without any vaginal bleeding after the onset of menopause. Fourteen years ago, the patient underwent an exploratory laparotomy due to acute abdomen at another hospital. During the operation, 2800 ml of intraabdominal blood clots and non-clotted blood was removed, and a cauliflower-like tumor 3*3*2 cm in diameter with active bleeding was seen around the left tubal umbrella. Complete hysterectomy and bilateral salpingo-oophorectomy and omentectomy were performed. The postoperative pathology report described a diffuse granular follicular cell tumor of the left ovary with invasion of the left fallopian tube. The clinical FIGO stage was II C. After surgery, she received 3 cycles of systemic chemotherapy consisting of Cisplatin + Cyclophosphamide (PC), after which the patient did not return for follow-up.\nFour years later (10 years ago), the patient underwent a second exploratory laparotomy at another hospital due to abdominal pain and the presence of a pelvic mass. During the surgery, a solid-cystic mass 3 cm in diameter was observed between the sigmoid colon and the bottom of the left side of the bladder. In addition, a nodule 1 cm in diameter at the bottom of the left side of the bladder bottom and a nodule 0.5 cm in diameter on the surface of the sigmoid colon were seen. The tumor lesions were completely removed, and the postoperative pathological diagnosis was granulosa cell tumor. After surgery, she received 1 cycle of chemotherapy consisting of Taxol + Carboplatin. However, the patient was then lost to follow-up.\nThe next time, she was admitted for abdominal pain and fever that persisted for 1 day. Ultrasound revealed an irregular, cystic mass 10*8.6 cm in diameter with an unclear boundary in the pelvic cavity; the wall was uneven in its thickness, and the size of the cystic portion was approximately 7.21*6.34 cm. MRI revealed a double capsular structure 88*9*7 cm in diameter on the left side of the pelvic cavity. The mass had a thick wall with a relatively clear edge and was located close to the right bowel. The cephalic size of the lesion was approximately 4*2*3 cm, and the signal of the vesicle was slightly low. Vaginal and abdominal examinations confirmed the presence of a hypertonic, cystic mass with a diameter of 16 cm. The preoperative serum levels of CA125, Estradiol (E2), and Follicle Stimulating Hormone (FSH) were 15.5 U/ml, 25.8 pg/mL and 52.8 mIU/mL, respectively.\nWe performed exploratory laparotomy and secondary cytoreductive surgery. During the operation, a solid-cystic multilocular mass 10 cm in diameter was observed between the sigmoid colon and the left lateral wall of the bladder. After 600 ml of yellow intracapsular fluid was removed, the mass was completely excised. Then, we performed adhesion decompression, repair of the sigmoid colon, and partial ileotomy anastomosis. The tumor was then completely removed, and the pathologic diagnosis was ovarian fibrosarcoma. Immunohistochemical analysis showed the following: Melan-A (+), Vimentin (+), AE1/AE3 (+/-), CD99 (-), Calretinin (-), α-inhibin (-), Ki-67 index 10%.\nAfter surgery, the patient experienced incomplete intestinal obstruction and received conservative treatment. The patient’s condition gradually improved and she fully recovered after 2 weeks. Since the patient was elderly, she did not receive adjuvant chemotherapy. No signs of recurrence or an increase in the serum E2 level have been observed more than 6 years after surgery. |
A 40-year-old woman presented with bilateral symptomatic primary varicose veins and a history of alopecia areata.\nHer alopecia started at the age of 13 during considerable family disruption and emotional trauma and concurrently undergoing menarche. She lost all of her scalp and body hair, retaining eyebrows and eyelashes only. She was otherwise fit and well, on no medication, with no alopecia in parents or four siblings. The alopecia responded to steroid injections, with regrowth a year later.\nAt the age of 19, she went to university and had another traumatic episode in her family’s life, reaching national media attention. These stressful events caused a second episode of alopecia, affecting all of her scalp, eyebrows and eyelashes, and most of the hair on arms and legs. She did retain her pubic and axillary hair. She responded to steroid injections, regrowing her hair over 2 years.\nAt the age of 30, she had the first of three pregnancies, suffering scalp alopecia areata only after the first delivery. She was diagnosed hypothyroid and was given thyroid replacement. Autoimmune thyroiditis was excluded. She responded to steroid injections with regrowth occurring by 6 months. Her subsequent pregnancies passed without any significant hair shedding.\nThese three major episodes of alopecia were related to stress and/or significant hormonal changes. She has suffered from minor episodes of hair shedding, but no other significant episodes until those reported here.\nWith regard to her varicose veins, she had bilateral aching and, as per NICE CG 168 underwent venous duplex ultrasonography by a clinical vascular scientist. On the right, she had significant reflux in both great saphenous vein (GSV) and small saphenous vein (SSV). The proximal GSV was competent with the major route of venous reflux coming from a para-vulval vein emerging from the pelvis. This was refluxing into an incompetent Giacomini vein, which was refluxing into the incompetent proximal SSV – the mid and lower part remaining competent. This suggested that the proximal SSV incompetence was secondary to the Giacomini reflux and hence PVR. Three incompetent perforators were emerging from mid Giacomini vein, through fascia, into posterior thigh varicosities.\nOn the left, there was no deep or superficial truncal reflux. All of the varicosities arose directly from an incompetent left para-vulval vein emerging from the pelvis and one incompetent perforator vein just above the ankle.\nA transvaginal duplex ultrasound scan (TVUS) was performed to identify the source of PVR. This confirmed the commonest pattern of PVR, left ovarian and both internal iliac vein territories exhibiting gross reflux.\nPlanned treated was PVE, followed by endovenous laser ablation (EVLA) of right GSV and SSV with TRansLuminal Occlusion of Perforator (TRLOP) closure of perforators bilaterally and phlebectomies. Any residual varicosities would then be treated with ultrasound-guided foam sclerotherapy.\nPVE of the left ovarian and both internal iliac veins was performed using foam sclerotherapy (2 mL 3% STS mixed 1:3 with 50:50 CO2:O2 1:4 giving 8 mL of foam per injection). The total dose was 10 mL of 3% STS liquid.\nDespite no immediate complication, the patient noticed moderate hair shedding in the days following the PVE although not significant enough to seek steroid injections.\nSix weeks later, she underwent EVLA of right GSV and SSV with bilateral TRLOP closure of perforators and phlebectomies. This is performed under local anaesthetic and passed without complication. Post-operatively, she had no shedding of any hair.\nSeven months after the PVE, she had ultrasound-guided foam sclerotherapy of residual varicosities of legs and labial varicosities. In total, she had 3 mL of 1% STS and 5 mL of 3% STS (1:4 foam with 50:50 CO2:O2). Two days after the sclerotherapy, she underwent substantial shedding from the scalp ( and ). |
A 17-year-old girl was admitted with abdominal pain and distension for the past 6 months. She also had nausea, vomiting and a weight loss of about 10 kg. Physical examination revealed multiple abdominal masses on the left side of the abdomen, pallor, ascites and pleural effusion without any peripheral lymphadenopathy. The patient’s remarkable laboratory findings were severe anemia (hemoglobin: 7.4 g/dL) and a significantly increased ESR (85 mm/h) and serum LDH (835 U/L).\nAbdominal ultrasonography revealed mass lesions adherent to each other in the upper portion of the retroperitoneal space. There was free fluid in the peritoneal and both pleural cavities ( and ). Paracentesis was performed and the ascitic fluid and pleural effusion were bloody. Analysis of both fluids showed exudative features with negative cytopathology.\nAbdominal CT scan confirmed several adherent mass lesions in the retroperitoneum with heterogeneous solid and cystic components (mostly solid) and several curvilinear calcifications (). The left kidney was destructed with no obvious contrast secretion (). The pancreas was displaced anteriorly (). The abdominal aorta, the inferior vena cava and the visceral vessels were encased by the mass lesions ( and ). Moderate hydronephrosis of the right kidney was evident. The liver, spleen, uterus and both ovaries were intact. There was no evidence of metastasis to the stomach in CT scan. Malignant retroperitoneal tumor (probably originating from the left kidney) such as sarcoma, neuroblastoma, or other malignant tumors were suggested as differential diagnoses. The interventional radiologist planned a percutaneous needle biopsy for definitive diagnosis. A 75×70 mm, mass lesion was localized in the left kidney by ultrasound at the posterior axillary line at T11-T12 intercostal space. Under local anesthesia, two biopsy specimens were obtained by a 16G core biopsy needle.\nHistological examination revealed a small rounded cell tumor with hyperchromatic nucleus and scant cytoplasm in a fibrotic stroma. Foci of necrosis were remarkable (). Immunohistochemical finding was compatible with PNET or Ewing’s sarcoma (positive for neuron specific enolase (NSE), vimentin, CD99 and negative for desmin, leukocyte common antigen (LCA) and chromogranin) (). Biopsy of a 5mm nodule in the cardia of the stomach that was discovered during an upper endoscopy for intractable nausea and vomiting showed the same pathology. As a result of local invasion and metastasis, the tumor was unresectable and the patient was referred to an oncologist for chemotherapy. Now, after 6 months, the patient is on multi-drug chemotherapy. The tumor has shown regression in follow-up ultrasonography, but is still unresectable. |
A 24-year-old male suffered from an anoxic brain injury 8 years prior to this study, following cardiac arrest caused by commotio cordis resulting from a blow to the precordial region, leaving the patient without vital signs for ∼30 min. He did not recover normal consciousness following this injury. At the time of data collection, the patient was diagnosed with UWS (CRS-R = 5, ). At the time of publication, this patient remains in UWS.\nMotif analysis. At baseline, motif topographies were dissimilar to conscious controls ( 0.211, 0.451). Motif 1 was spatially dispersed, while motif 7 exhibited the opposite polarity of topographic distribution that we would expect based on observations in healthy controls (i.e. motif 7 was anteriorly instead of posteriorly dominant) (). This was expected as the participant was in a chronic UWS state and might suggest that the inability of the brain network to sustain consciousness is reflected by its motif characteristics. The topographic organization of motifs 1 and 7 shifted in response to anesthesia but failed to return to baseline state post-anesthesia (). Contrary to what we would expect in a healthy individual, high-frequency nodes seemed to become more polarized towards anterior brain regions for motif 1, and posterior brain regions for motif 7. At recovery, node participation in motif 1 regained a disorganized pattern, while motif 7 became insignificant. There were no clear patterns in motif topography or reorganization in the theta () and delta bands (); motifs at these frequencies were often non-significant.\nHub analysis. Alpha network hubs did not resemble conscious controls at baseline 0.101) () or return to baseline state post-anesthesia (). This may further emphasize that the inability of the brain network to sustain consciousness is associated with topographic network properties. There were no clear patterns in hub topography or reorganization in the theta band (), although hubs did undergo anesthetic-induced topographic reorganization in the delta band ().\nPower analysis. At baseline, alpha power topography did not resemble the distribution for conscious controls (0.343) () and did not reorganize under anesthesia (). Neither theta () nor delta () power topography shifted under anesthesia.\nGraph theoretical network properties. At baseline, in the alpha band, global efficiency was within the range of conscious controls, while clustering coefficient, binary small worldness and modularity were all elevated relative to conscious controls (, blue diamonds). While these properties changed across baseline, anesthesia, and recovery, they did not shift in an expected pattern. This was also the case in the theta (, blue diamonds) and the delta bands (, blue diamonds).\nRelative power analysis. Relative power in the theta band was within the range of conscious controls, while relative delta power was higher and relative alpha power was lower than in conscious controls (, blue diamonds). Importantly, this patient, who did not recover consciousness, was the only one with elevated delta power relative to conscious controls. |
An 8-month-old girl without specific past medical history was admitted to the hospital due to nausea and diarrhea. On physical examination, a firm mass was detected in the left upper quadrant of the abdomen. Abdominal computed tomography (CT) revealed a 6.8×5.8×5.3 cm sized left renal mass without lymph node enlargement (). There were no suspicious metastatic lesions in chest CT. Laboratory data showed leukocytosis. Radical nephrectomy with lymph nodes dissection was performed. Gross examination revealed a well circumscribed, ovoid, firm mass in the lower pole of the kidney (). The mass was abutted on the renal capsule, and the surface of the mass was whitish gray with multifocal hemorrhages. The remaining renal parenchyma was unremarkable. Microscopic findings revealed that the tumor was a highly cellular neoplasm composed of tumor cells of varying size with prominent nucleoli and abundant cytoplasm (). Histological examination was consistent with the diagnosis of malignant rhabdoid tumor. Immunohistochemistry showed loss of INI1 protein staining ().\nOne week after the operation, weakness of both lower extremities and voiding failure were noted. On neurological examination, she failed to respond to pain in both legs. Brain magnetic resonance imaging (MRI) did not show evidence of metastatic disease, but lumbar MRI revealed a 6.5×1 cm sized intradural extramedullary tubular mass with heterogeneous enhancement at L1-S1 level (). On the next day from onset of symptoms, laminoplastic laminotomy was performed by a neurosurgeon to remove the tumor and decompress spinal cord. Conus medullaris was displaced by the hypervascular tumor with yellowish color, however the upper margin of the tumor was clear (). The tumor was densely adhered to the left side of L5 nerve root. Near total removal of the tumor was performed using cavitron ultrasonic surgical aspirator, and there was no root injury during the surgery (). The pathologic findings were consistent with metastatic malignant rhabdoid tumor (). After the surgery, the motor power of lower extremity was returned. She received adjuvant radiotherapy for 2 weeks and 13 cycles of chemotherapy (vincristine, doxorubicin, and cyclophosphamide) for 3 months postoperatively. The patient showed general weakness, seizure and changes in mental status during the chemotherapy. Leptomeningeal metastasis with hydrocephalus was noted in brain MRI. A ventriculoperitoneal shunt was placed. However, she did not clinically improve and died 6 months after the surgery. |
A 63-year-old woman had a stage III macular hole in the right eye and a stage IV in the left eye (Fig. ). She had 6 months history of decreased vision in her right eye and 2 months in her left eye. Her corrected visual acuity was 20/50 OD and 20/30 OS. Phacoemulsification and aspiration, intraocular lens implantation, and vitrectomy were performed on the right eye followed by the left eye. The ILM was stained with 0.5% ICG, and a three to four disc diameter area of ILM was peeled and removed. The initial flap for peeling the ILM was created on the temporal side in each eye. Then 1.0 ml of pure SF6 gas was injected to tamponade the retina. The patient maintained a prone position for 24 hours on the first postoperative day and only during the day on the following 7 days.\nOne week after the surgery, the macular holes were confirmed to be closed (Fig. ). However, degenerative changes developed in the macula about 2 weeks after the surgery, especially on the temporal side of the fovea in both eyes. A yellow, round degenerative lesion was noted in the fovea, and irregular degenerative foci associated with pigment deposits were found just temporal to the lesion. The changes were more marked in the right eye (Fig. ,). The surgical procedures and clinical course of left eye was similar to the right eye.\nAlthough retinal vessels appeared ophthalmoscopically normal, fluorescein angiography (FA) revealed areas of hypofluorescein and blockage of the fluorescein in the area of the pigment epithelial cell proliferation. The area surrounding the pigment cell proliferation was hyperfluorescein, but tiny hypofluorescein spots were detected within the hyperfluorescein area (Fig. ,).\nThese pathological changes did not progress, but the visual acuity decreased to 20/60 in the right eye and 20/40 in the left eye at 6 months after surgery. |
A 62-year-old man, with a large nonhealing ulcer of the lower lip since 1 month, was admitted to our clinic. The ulcer presented initially as an inflammatory papule, which gradually transformed into an ulcer with elevated borders smeared with serous fluid. The patient received outpatient treatment consisting of tablet cephalexin (500 mg QID for 7 days) and tablet acyclovir (400 mg 5 times a day for 4 days); however, the patient's clinical condition failed to improve. He denied any history of physical or chemical injuries. However, for the past 4 years, he was suffering from a psychiatry disorder and his drug history included tablet sertraline 50 mg daily. He was not a smoker and did not use any illicit drugs. His other medical history was insignificant. The extra-oral examination on admission revealed no abnormality. However, the intra-oral examination revealed an indurated painless oval ulcer measuring 2 × 1.2 cm in diameter with elevated borders in the central portion of the lower lip with a fibrinous base. The vermilion border adjacent to the ulcer showed desquamation. He had poor oral hygiene with generalized stains, attrition, and carious teeth. The local lymph nodes accessible to physical examination were not enlarged (Figure ). Routine laboratory evaluations did not show any significant abnormal findings. The enzyme-linked immuno-sorbent assay (ELISA) for serum antibodies to HSV-1 and HSV-2 and the rapid plasma reagin (RPR) test for syphilis were both negative. Taking the history into account, a clinical differential diagnosis of traumatic and malignant ulcer was made.\nA deep tissue biopsy was done from the lesion under local anesthesia and sent to two independent pathology laboratories for routine histopathological examination and was stained with hematoxylin and eosin. The histopathology findings revealed ulceration in the epidermis sloughed stratified squamous epithelium with granulation tissue in the dermis composed of fibroblastic and vascular proliferation with a dense, diffuse infiltration of lymphohistiocytes and eosinophils that extended deeply into the submucosal tissue. The above classical features were consistent with EUOM (Figure ). The ulcer resolved spontaneously after 3 months by only reassuring the patient without any medical treatment (Figure ). |
A 70-year-old African American male was seen in the emergency department for acute anxiety and paranoia. He reported that his son gave him melatonin to help him sleep, but he felt the medication was poisonous. He also reported that he was struggling with the death of his partner of 40 years and was feeling anxious. The patient was prescribed risperidone and lorazepam and was discharged shortly thereafter. Twenty-four hours later, he was seen again in the emergency department for worsening anxiety, psychosis, suicidal ideation, and command hallucinations. The patient's son reported that his father “had not slept in weeks.” The son reported progressive agitation, paranoia, and bizarre behavior. Due to the worsening psychosis, he was admitted to the medical floor for further work-up. He reported feeling as though people were watching him and that someone was going to harm him. When questioned about his reported suicidal ideation, he blamed that thought on his post-traumatic stress disorder; but would not elaborate on the event. The patient was given a one-to-one sitter due to his suicidal ideation.\nThis patient lives with his family of seven children. He has a history of prolonged incarceration. His past medical history is significant for hypertension and negative for seizure disorders. The patient had never been hospitalized for psychiatric issues prior to this visit. He denied past suicidal behavior and any history of physically or sexually aggressive behavior. The patient reported a history of excessive alcohol abuse for more than a year following the death of his partner. He reported that he stopped drinking “cold turkey” five months prior to this visit. The patient's family history is significant for a son with an anxiety disorder. He denied a history of physical or sexual abuse in the past.\nThe patient's mental status exam was significant for an anxious affect with referential and paranoid ideations. He denied any thought broadcasting, insertion, or withdrawal. He had some paranoid and persecutory delusions. Insight, judgment, and impulse control were poor.\nThe patient's initial physical exam revealed an inability to ambulate and overall weakness. Further neurological examination revealed mental disorientation with bilateral muscle wasting, sensory deficit, and hyporeflexive ankles. Additionally, the patient exhibited stance and gait abnormalities. A later eye exam revealed bilateral light-near dissociation with accommodation but no reaction to light. Otherwise, all other neurological components were intact and within normal limits.\nInitial routine testing was positive for the Treponema pallidum antibody, suggesting prior infection, but the distinction between treated and untreated syphilis cannot be made as the Treponemal-specific immunoglobulin G (IgG) may remain elevated throughout life. An RPR was then ordered to help distinguish between acute or chronic infection and a Treponema pallidum particle agglutination test was ordered to distinguish between syphilis infection and a false positive screening test. The RPR returned nonreactive but the Treponema pallidum particle agglutination returned reactive, indicating a prior infection of Treponema pallidum. The initial differential diagnosis workup included testing to exclude thiamine, folate and B12 deficiency, insomnia, hypocalcemia, hypothyroidism/hyperthyroidism, HIV encephalopathy, dementia, stroke, drug or alcohol intoxication, and normal pressure hydrocephalus.\nFurther labs showed white blood cell count, sodium, potassium, calcium, anion gap, creatinine, glomerular filtration rate, glucose, mean corpuscular volume, and thyroid stimulating hormone within normal limits. Urinalysis, toxicology screening, alcohol levels, and HIV antibody were also negative. Imaging included a head computed tomography (CT), which showed no abnormalities, a brain magnetic resonance imaging (MRI) that showed cortical atrophy, and an electroencephalogram within normal limits. |
The patient was a 76-year-old woman who had a history of surgery for papillary thyroid cancer and cesarean section with uterus bicornis.\nShe had consulted a previous physician complaining of fever and right lower quadrant abdominal pain. A marked inflammatory reaction was observed and enhanced computed tomography (CT) showed abscess formation around the cecum. An intra-abdominal abscess was diagnosed and the patient was treated conservatively with antibiotics, but her symptoms relapsed after discharge and she was referred to our hospital. The inflammatory reaction showed exacerbation and cancer antigen 125 (CA125) was mildly elevated (78 U/mL). Enhanced CT was performed again and showed a persistent abscess, a tumorous lesion in the mesentery and right hydronephrosis (Figure ). Because malignancy could not be ruled out, surgical treatment was selected.\nAt laparotomy, there were no ascites but encapsulated abscesses were present on the mesenteric side and outside of the ileocecal region (Figure a,b). When we raised the ileocecal region, a tumor was found to be fixed to the right ureter and there was leakage of white, solid tumor content (Figure c). This tumor content was submitted to intraoperative frozen section diagnosis, which demonstrated a ‘carcinoma’. Also, enlarged lymph nodes were observed along the ileocolic artery. For the above reasons, we performed ileocecal resection with D3 lymph node Dissection. A ureteral stent was then inserted into the right ureter, and the tumor was separated and removed from the ureter with scissors (Figure d). The ureteral stent was easily inserted without resistance and urine cytology was negative for carcinoma. After tumor removal, we searched the abdominal cavity thoroughly, but there were no abnormalities of the uterus and adnexa, nor any evidence of peritoneal dissemination.\nThe pathological diagnosis was poorly differentiated carcinoma, but there were no changes involving the mucosal surface of the cecum or the terminal ileum (Figure ). The mesenteric lymph nodes were free of metastasis. Hematoxylin-eosin (HE) staining showed findings of poorly differentiated carcinoma, and alcian blue (AB) staining showed moderate production of mucin accompanied by a partial ductal structure. Immunohistochemistry showed the tumor to be positive for AE1/AE3 (pancytokeratin), cytokeratin(CK)7, CK34βE12 (about 20% of tumor) and Wilms tumor gene 1 (WT-1), with nonspecific reactions for CK5/6, p63, carcinoembryonic antigen (CEA), estrogen receptor (ER) and vimentin, while CK20, thyroid transcription factor 1 (TTF-1), caudal type homeobox 2 (CDX2), gross cystic disease fluid protein-15 (GCDFP-15), calretinin, D2-40 and CD10 were negative (Figure ). No pathogen was detected in bacterial culture of the pus. After surgery, esophagogastroduodenoscopy and colonoscopy were conducted, and no gastrointestinal tract lesions were observed. Gynecologic and transvaginal ultrasonography (US) examinations revealed no abnormal findings. Positron emission tomography (PET) showed no abnormal uptake.\nBecause the tumor content had leaked into the abdominal cavity, we regarded this case as having undergone incomplete resection and six courses of chemotherapy with paclitaxel (PTX, 220 mg/body at day one) and carboplatin (CBDCA, 360 mg/body at day one) were administered at intervals of four weeks. The patient has remained well and disease-free for almost one year, to date, since the primary operation. |
A 56-year-old female, with a background history of hyperthyroidism, osteoporosis, oesophagitis, and hysterectomy, presented to the ED with a one-month history of severe left proximal tibial pain associated with weight-bearing which was worse at night with an insidious onset. Her history comprised of atraumatic pain without any signs or symptoms of infection and without any subjective weight loss or systemic features. She was an ex-smoker for 16 years. She had attended her general practitioner (GP) multiple times for the same pain over the preceding month. The GP organised an MRI of the left knee which was performed eight days before presentation to the ED. The MRI revealed a subcutaneous fat collection at the level of the left tibial tuberosity with no bony or ligamentous injury. Concerned about her symptoms, the patient attended a private orthopaedic consultant who, considering the MRI results, discharged her with simple analgesia. She subsequently decided to present to the ED for further clinical input due to worsening pain.\nOn examination, her vital signs were normal. Systemically, she seemed well without any further symptoms. A focused examination of the left lower leg revealed mild erythema of the medial aspect of the proximal tibia without any swelling, bruising, or deformity. She had point tenderness over the medial aspect of the proximal tibia. Her gait was normal, and she had a good range of motion in her knee and ankle joint. Neurovascular examination of the lower limb was normal. No knee tenderness was elicited. The initial differential diagnoses considered were musculoskeletal pain/injury, stress fracture, gastrocnemius muscle tear/tightness, or fibromyalgia. Deep vein thrombosis (DVT), a common cause of lower limb pain was out ruled by a Wells' score of -2 and a D-dimer value of 0.29 mcg/mL (normal: < 0.44 mcg/mL).\nThe initial impression by the ED clinician was that the clinical area of concern was the tibia rather than the knee. However, the patient went on to have routine blood tests and an x-ray of the left tibia/fibula to further investigate the clinical examination findings elicited on the examination and persistent patient concerns.\nInvestigations\nBaseline blood tests were ordered and the results were all unremarkable (Table ).\nA radiographic image of the left tibia showed an abnormal circular pattern approximately 12 cm below the knee (Figure ). This incidental finding of a well-defined lytic lesion prompted further investigation with computed tomography (CT) scan of the lower leg which supported the possibility of metastatic disease of the bone (Figure ). A chest x-ray was ordered for the staging of the disease as a baseline investigation. This demonstrated the presence of an apical lesion of the left lung apex, suggestive of metastatic bone disease from a possible primary lung cancer (Figure ). A CT scan of the head/neck/chest with contrast was ordered to identify the source and staging of the disease (Figure ). The CT scan of the chest confirmed the presence of an apical mass with no metastasis found in the abdomen, pelvis, or other parts of the chest. A multidisciplinary team approach involving the medical and orthopaedic teams was adopted in this case. A transbronchial needle aspiration (TBNA) was performed from the gland by the respiratory physicians and a sample was sent for cytology and histology. The results indicated a poorly differentiated non-small cell lung cancer (squamous) of the left lung apex with proven central adenopathy (T3N2Mx). Bronchoscopy didn’t yield any cytological evidence of the tumour cells. The primary lung lesion was visualized on endobronchial ultrasound (EBUS) which reported a 12 mm lymph gland in the left lower paratracheal area and a 15 mm lymph gland was noted at the superior left hilum. The multidisciplinary team recommended an urgent positron emission tomography (PET) scan to complete staging and liaison with medical oncology for consideration of systemic chemotherapy (± radiotherapy, depending on the final stage). The PET scan confirmed a 3 cm left proximal left tibial mass with avid uptake.\nDifferential diagnosis\nChronic lower leg pain comprises several etiologies, such as stress fracture, chronic exertional compartment syndrome, medial tibial stress syndrome, nerve entrapment, peripheral vascular disease, DVT, and popliteal artery entrapment syndrome. Symptoms associated with these conditions often overlap, making a definitive diagnosis difficult []. There was little in this patient's history or physical examination to suggest any serious pathology. One of the differential diagnoses was cellulitis of the left upper leg, but the onset and lack of common features of cellulitis, such as redness, pain, swelling, and heat, made it very unlikely []. Necrotizing fasciitis is a diagnosis of choice if associated with a history of rapid progression when the pain is out of proportion to the clinical signs []. This patient’s symptoms, clinical examination, or laboratory data suggested neither cellulitis nor necrotising fasciitis.\nAlthough DVT could have been among the differential diagnoses, there was no evidence of any other cardinal features of DVT, such as asymmetrical swelling in the extremity []. The two-level DVT Wells' score was also unlikely for DVT []. Most myopathic processes are relatively painless (polymyositis, dermatomyositis, toxic myopathies, and body myositis). Muscular pain is usually associated with trauma or repetition. Generally, muscle pain is confined to patients with acute rhabdomyolysis, a few metabolic myopathies, and myopathies associated with connective tissue disease []. Muscular pain was felt to be unlikely following examination. One might consider a diagnosis of a rupture of the distal musculotendinous junction of the medial head of the gastrocnemius muscle (tennis leg). This condition is often seen in athletes performing sudden acceleration and deceleration manoeuvres []. The patient denied any history of trauma, and the physical examination revealed localised pain at the medial aspect of the upper left leg. In stress fractures and medial tibial stress syndrome (MTSS) (known as shin splints), the patient presents with diffuse pain and tenderness over the posteromedial aspect of the distal tibia that occurs with exercise or at rest in more advanced cases []. Our patient’s left leg x-ray showed a lytic lesion of the proximal tibia without any fracture (Figure ). Primary bony tumours or metastases are always considered when patients present with non-traumatic pain. Isolated leg pain is not commonly associated with a PT (lesions in the superior sulcus). Leg pain can direct towards malignancy in the presence of constitutional symptoms or red flags. In the absence of any history of cancer, red flags suggestive of malignancy are usually a new onset of severe pain in a patient over 60 years of age, non-mechanical pain, mechanical symptoms not responding to adequate treatment over an acceptable time, unexplained weight loss associated with fatigue, fever, and night sweats. Our patient’s presentation was atypical, but her perseverance was crucial in prompting further investigations.\nIt is common practice to obtain x-rays of the tibia and fibula in the presence of trauma or suspicion of any bony pathology. There were initially very few indications for obtaining such imaging on this patient. The x-ray of the left leg, in this case, was organised to rule out a stress fracture. The incidental finding of a well-defined lytic lesion prompted the physician to investigate further with a CT scan of the lower leg which supported the possibility of metastatic disease of the bone (Figures -). A chest x-ray was obtained to assist with the staging of the disease. This demonstrated the presence of an apical lesion in the left lung apex, suggestive of metastatic bone disease from a possible primary lung cancer (Figure ). A CT of the chest was ordered to confirm the existence of an apical mass and its relationships with the bony thorax and the thoracic inlet (Figure ) []. To further assess our patient, an MRI of the left tibia was requested, which confirmed an osteolytic bony lesion noted in the upper shaft of the left tibia and with its superior aspect suggestive of an aggressive bony lesion (Figure ). An MRI of the chest remained the best imaging modality to diagnose a superior sulcus tumour and it allowed for the proper assessment of possible neurological impairment [-].\nOutcome and follow-up\nUnfortunately, her outcome was poor, and the patient developed multiple complications. A few months after the diagnosis, she presented to the ED again with respiratory sepsis post-radiotherapy and required admission to the Intensive Care Unit; this was successfully treated with intravenous (IV) antibiotics. A month following her ICU admission, she developed a pulmonary embolism in the right lung and was started on anticoagulants. She also suffered from a pathological fracture of the left proximal tibia which required surgical intervention (Figure ). The patient went on to develop hoarseness due to tumour invasion of the recurrent laryngeal nerve and developed left arm severe pain, likely secondary to brachial plexus compression, confirmed with an interval scan due to increased tumour size.\nAfter an informed and thorough discussion with the caretaker team and the patient, a decision was made in the patient’s best interests to refer her for palliative care. The case was consulted by a palliative medicine physician. She was admitted to the local hospice where she was started on therapeutic enoxaparin, 1 mg/kg twice a day, and alprazolam, methadone, and oxycodone for pain management. She received 10 sessions of palliative radiotherapy and benefited from neoadjuvant chemotherapy post-radiotherapy. |
Ms. AD is a 34-year-old homemaker, who previously, had two manic episodes with no family history of mental illness/bipolar affective disorder. She was not treated for the first episode and remitted within 1 month of the treatment with olanzapine 10 mg/day, and lithium carbonate 600 mg/day for the second episode. The index episode started in February 2014 when she developed fever, nausea, and vomiting acutely. She took paracetamol 500 mg orally with which fever subsided for once, but it recurred after 2 days and did not remit despite taking antipyretics further. She consulted a physician after 1 week of onset of fever (10th day) who diagnosed her with typhoid fever on the basis of serological investigations only (Widal test positive; titers for agglutinin O > 1:160 and agglutinin H > 1:160); prescribed her oral ciprofloxacin 500 mg twice a day for a week. Fever subsided within the next 2 days, and she was asymptomatic for the next 5 days when vomiting and diarrhea recurred along with headache. Ciprofloxacin was continued for 1 more week. Though above mentioned symptoms remitted in the next 2 days, new set of symptoms appeared on the day 10 of treatment with ciprofloxacin. She was noted to get up early in the morning; would bath early and then start doing household chores. She became more active, indulged herself into multiple tasks at the same time, and left them incomplete. Her family reported her to be very cheerful, amusing, and jocular which was unlike her previous self. She talked in excess at a faster pace and difficult to interrupt. She increasingly participated in religious activities, indulged in unnecessary buying of clothes and jewellery. She took ciprofloxacin for 4 more days in that week. Despite stopping the medication, thereafter no improvement was noted. In the next 2 weeks, her symptoms further worsened. There was no history of altered sensorium, disorientation, and any perceptual abnormalities. As she became unmanageable, so she was brought to our center. Her general physical examination did not reveal any abnormality suggestive of either metabolic disorder or cerebrovascular disease. Her metabolic parameters were within a normal range including thyroid function test. Neuroimaging study of brain in the form of computed tomography was normal. She was not receiving any other medications nor did she abuse any psychotropic substances such as cocaine or amphetamines. She was treated with olanzapine 10 mg and lithium carbonate 600 mg/day which was later on increased to 800 mg/day to achieve optimum serum lithium level. She achieved complete remission within 6 weeks.\nIn her past, she had suffered from gastroenteritis at 4–5 occasions but sought treatment only on two occasions. These two episodes of gastroenteritis, which occurred 10 and 8 years back, were treated with ciprofloxacin and ofloxacin, respectively, but prescriptions were not available. On both occasions, she developed similar set of mental symptoms as in the index episode characterized by elated mood, decreased need for sleep, increased goal-directed activity, increased religiosity, over-talkativeness, and increase in goal-directed activities though severity were less. Hence, the diagnosis of medication-induced recurrent mania as per diagnostic and Statistical Manual of Mental Disorders Fifth Edition[] was kept. After she had achieved remission, all the psychotropics were gradually tapered and stopped. The patient and family members were cautioned for the use of quinolones in future as these were implicated as etiological in her mental illness. She has been followed up regularly and was in remission until March 2015. |
A 63-year-old male with a past medical history of non-ischemic cardiomyopathy (ejection fraction 10%-15%), hypertension, chronic kidney disease stage G3A, and severe mitral valve regurgitation status post valve repair initially presented for an upgradation of his biventricular pacemaker to biventricular implantable cardioverter defibrillator (BIV-ICD) and replacement of non-functional right atrial lead. The pacemaker pocket was exposed and identified and the atrial and ventricular leads were pulled back under fluoroscopic guidance. A new left coronary sinus lead was placed. Next, right atrial (RA) and right ventricular (RV) leads were placed and access was obtained via the left coronary sinus as the left subclavian vein was found to be occluded with formation of collaterals. The leads were sutured in fascial planes via silk suture. The pacing and sensing parameters were confirmed. BIV-ICD was placed in the pocket, and the pouch was closed via vicryl sutures. The patient had electrocardiogram (EKG) (Figure ) and chest X-ray (Figure ) performed after the procedure. He had developed small pneumothorax from the procedure, which resolved on its own.\nThe patient was asymptomatic for the next seven months and then he developed increasing shortness of breath. At that point, the EKG (Figure ) revealed loss of appropriate pacing function. It was discovered that the patient kept massaging his pacemaker pocket site in the last seven months but he denied manipulating the device itself. He had also been moving his arm up and down frequently, which would have led all his device leads to be pulled up towards the device in a ratchet-like fashion. The patient was scheduled again for revision of BIV-ICD and on incision of the generator pocket, the left ventricular lead was found coiled up in the pocket beside the generator (Figure ). A new left ventricular lead was placed in the coronary sinus and then access was obtained via the right subclavian vein for right-sided leads. His right atrial and right ventricular leads were also found pulled up. A decision was made to place new right atrial and right ventricular leads. It is to be noted that all his device leads were undamaged and his device was found to be in the pocket in normal orientation. After securing the leads via sutures, the pacing and sensing function was analyzed. The pocket was revised and closed with vicryl sutures and the device was sewn to the pectoral fascia. A chest X-ray (Figure ) and EKG (Figure ) were obtained after the procedure to ensure the absence of pneumothorax and to document the appropriate functioning of the device, respectively. |
A 14-month-old boy was admitted to our department after frequent admissions in other hospitals because of recurrent severe feeding and respiratory problems. His weight was 7.6 kg, height 78 cm, blood pressure 90/75 mmHg, moderate psychomotor retardation was present. His parents were not consanguineous. He was born following an uneventful pregnancy with a birth weight of 2,560 g. The mother was 36 years old.\nChromosomal analysis performed because of facial dysmorphic features (Fig. ) suggesting Down syndrome revealed a 48,XXY +21 karyotype: a double aneuploidy of Down syndrome and Klinefelter syndrome. Soon after birth, he had to be intubated because of severe respiratory problems. Echocardiography, bronchoscopy and CT thorax performed in another centre demonstrated a small atrial septal defect (secundum type) and narrowing of the trachea from its middle to the carina. The X-ray of the small bowel was normal; barium swallow was then not performed. The boy had to be fed by a tube and later exclusively via a percutaneous endoscopical gastrostomy. After extubation, the patient suffered repeatedly from severe respiratory distress in the course of intercurrent infections.\nThis clinical picture accompanied by findings of a topical trachea and possibly also an oesophageal obstruction led to a suspicion of a vascular airway compression. A new echocardiography and a new CT of the thorax with contrast (Siemens Somatom Sensation 64-slice, contrast 25 ml omnipaque 300 intravenous) demonstrated a double aortic arch (Fig. ), compressing both trachea and oesophagus. The anterior left arch appeared smaller with a localised narrowing, as compared to the posterior right arch. The descending aorta was running to the left of the spine. Barium swallow demonstrated a severe indentation in the proximal oesophagus (Fig. ).\nUsing a median sternotomy approach, the left anterior aortic arch was divided at the smallest point between the left carotid and subclavian artery. In addition, the ductal ligament was divided. Complete and thorough mobilization of the trachea and oesophagus has been performed. It was decided not to proceed with open heart surgery in order to close the small centrally located atrial septal defect, because if still necessary, it can later be closed percutaneously.\nThe postoperative course was complicated by a wound infection and pleural empyema for which he needed surgical treatment. The percutaneous endoscopical gastrostomy could be abolished and normal oral feeding restored. |
A 72-year-old female accidentally struck her left forechest 2 months before she was referred to our hospital, and lower left rib fracture was observed on X-ray examination. Her past medical history included hypertension for 30 years and diabetes for 4 years. She was suffering from severe left-sided back pain, which was thought to be apart from the rib fracture site, and the pain was intermittent. The ultrasound sonography demonstrated left hydronephrosis and a simple renal cyst on the lower pole of the left kidney, and urinalysis showed microhematuria. Subsequently, she was referred for urological assessment of severe left flank pain. Routine blood tests were not remarkable except for a slightly elevated serum glucose level of 179 mg/dl.\nIntravenous ureterography (IVU) showed findings compatible with a left sciatic ureter, a dilated ureter with a fixed kinking, which is known as ‘curlicue’ sign (fig. ). The ureter distal to the kink was not well visualized.\nThe abdominal CT showed marked hydronephrosis and hydroureter on the level of the pelvic inlet and revealed the presence of a sciatic herniation of the ureter (fig. ). The ureter was seen to course behind the left hip and through the greater sciatic foramen, anterior to the piriformis muscle. No evidence of inflammatory lesions, compressive tumor or herniation of another pelvic organ with the ureter was seen.\nWe decided to attempt recovery of the herniated ureter using a retrograde approach. Retrograde pyelography demonstrated hydronephrosis with ‘curlicue’ formation of the distal ureter (fig. ). Subsequently, the placement of ureteral stent was performed to decompress the dilated upper urinary tract. Interestingly, the ureterosciatic hernia was relieved with the passage of a flexible guide wire and a double-pigtail stent (fig. ). The patient noted that the back pain experienced before stent placement completely resolved.\nThree months after stenting, we intended to exchange the stent. However, the patient refused continuing to have an indwelling stent and the stent was removed. Thereafter, she was followed by a periodic checkup using ultrasonography and IVU. IVU obtained 3 months after the removal of the ureteral stent revealed the recurrence of the ureterosciatic hernia; however, there was no evident recurrence of hydroureter, hydronephrosis or ureteral obstruction. Since then, abdominal sonography has not shown recurrence of hydronephrosis. The patient is asymptomatic and refuses further intervention but is currently followed with observation. Six years after ureteral stenting, the patient continues to be without hydronephrosis, symptoms or the usage of the indwelling ureteral stent. |
A 41-year-old woman Gravid 4 Para 0 presented to emergency room with complaints of lower abdominal pain and fever for the last 1 week. She also gave a recent history of ectopic pregnancy for which she underwent a laparoscopic right salpingectomy at another hospital. She denied any nausea, vomiting, vaginal bleeding, vaginal discharge, or diarrhea and declined any significant medical problems or medication use. Patient had a history of three spontaneous abortions and one ectopic pregnancy treated with laparoscopic right salpingectomy, 2 weeks back. She denied any other surgical history and also denied any workup for recurrent first trimester miscarriages. She was currently trying to conceive for the last two years. Patient is a nonsmoker and denied any personal or family history of bleeding or thrombotic disorders.\nUpon examination, her abdomen was nontender, nondistended, and without any palpable masses. The rest of the physical examination including inspection of surgical wound, Homans' signs, and Moses' sign was negative. The pelvic examination revealed an anteverted, normal size, nontender uterus, without any adnexal tenderness or palpable masses. Patient was febrile at 101 degrees but other vital signs were within normal limits. Urine pregnancy test was negative. Laboratory examination showed white blood cell count of 13.4 × 109/L with 78% neutrophils and elevated C-reactive protein of 120 mg/dL. The rest of the complete blood count and comprehensive metabolic profile were within normal limits.\nThe remaining workup for fever, including chest X-ray and urinalysis, was noted negative. Patient was admitted with the diagnosis of “fever of unknown etiology” and started on antibiotics. Blood cultures were sent prior to initiating treatment with antibiotics. A hypercoagulable workup performed due to patient's history of recurrent spontaneous abortions returned negative. CT scan of the abdomen and pelvis with intravenous contrast was performed on hospital day 1, which revealed right ovarian vein thrombosis, without extension into the inferior vena cava (IVC) (Figures and ).\nTreatment with therapeutic dose of low molecular weight heparin (Enoxaparin 60 mg, subcutaneous injection twice a day) was started. Patient received Enoxaparin for 2 weeks, following which oral anticoagulation with Warfarin was initiated. Warfarin was continued for 6 months with close outpatient follow-ups. Patient responded very well to the treatment and a follow-up CT revealed absence of thrombus in the right ovarian vein. Warfarin was discontinued at this time and patient resumed care with maternal fetal medicine for preconceptional counseling and management during next pregnancy. |
A 43-year-old man was referred with a 4-year history of drug-resistant epilepsy characterised by focal to bilateral tonic-clonic seizures and refractory myoclonus. There was no other medical history. He had been born at term, attained all developmental milestones normally and had no history of childhood convulsions.\nHe had two sisters, both of whom had childhood epilepsy. One sister had experienced global developmental delay, had never talked and been wheelchair-bound following a diagnosis of congenital muscular dystrophy. The other sister had reached normal milestones until the age of 3 years, after which she developed progressive gait abnormalities. Both sisters had died from pneumonia aged 13 and 16 years old. He had one daughter, aged 8 years old, with no neurological symptoms ().\nOur patient reported developing symptoms when he was 39 years old, initially with episodes of right elbow cramping, which evolved to paroxysmal episodes of right upper limb jerking lasting approximately thirty seconds on each occasion. With time, these attacks became more frequent, and he also began to experience episodes of twitching over the right side of his face. He reported violent episodes lasting up to fifteen minutes with jerking of all four limbs with preserved awareness. He had one episode associated with loss of consciousness, followed by right upper and lower limb weakness lasting several days. When he was 42 years old, he developed bilateral simultaneous progressive visual loss over a period of weeks, to the extent that he had been registered legally blind.\nOn examination, there was high-frequency, stimulus-sensitive multifocal myoclonus predominantly affecting the right side of his face and right upper limb. Awareness was preserved. Facial myoclonus grew more pronounced when talking to the extent he was unable to speak or swallow (). Ankle reflexes were absent and there was a glove-and-stocking distribution of sensory loss.\nInitial visual testing revealed bilateral central scotomata with corrected visual acuities of 6/60 in the right eye and 6/18 in the left eye, relatively preserved peripheral vision and pallor of both optic discs (). Optical coherence tomography showed bilateral symmetric thinning of the nerve fibre layer in the papillomacular bundles. Six months later, there had been considerable deterioration in central vision, with acuities in both eyes at the level of hand movements only. This did not improve despite a trial of high dose intravenous steroids with an oral taper.\nMRI scans over a 2-year period demonstrated interval development of multifocal cortical and subcortical T2/FLAIR hyperintensities involving both cerebral hemispheres (). Nerve conduction studies revealed a symmetrical, length-dependent axonal neuropathy. Prolonged video electroencephalography (EEG) revealed episodes of twitching involving the face, upper body and right arm with no epileptiform correlate. Although excessive muscle artefact made back-averaging impossible, short-duration myoclonic jerks with craniocaudal spread, followed by brief periods of atonia were consistent with cortical myoclonus. Giant somatosensory evoked potentials (N20-P25 24.4µV) were also found, supporting the presence of cortical hyperexcitability.\nA muscle biopsy was performed, which showed minor non-specific changes (). Although there were no definite cytochrome oxidase negative fibres (which may be subject to variation in level of sections), rare fibres deficient in complex I and complex IV components were evident. These changes were not diagnostic, but were in keeping with the clinical impression of a mitochondrial disorder. On the contrary, muscle respiratory chain enzyme analysis showed no evidence of complex I/II+III/IV or ubiquinone deficiency. A 21-gene panel for pathogenic mitochondrial gene mutations was negative. Given clinical suspicion, we proceeded to whole mitochondrial gene sequencing on the muscle sample that revealed a 98% heteroplasmic m.14487T>C p. (Met63Val) mutation. This is a pathological variant which results in an amino acid substitution in NADH dehydrogenase 6 (ND6), a complex 1 subunit of the mitochondrial respiratory chain. |
A previously healthy 10-year-old Asian girl presented to the emergency department with headache, vomiting, and one week of mild nonproductive cough. Her headache started the evening prior to presentation, was gradual in onset and frontotemporal in location, and improved with acetaminophen but subsequently woke her from sleep. It was accompanied by two episodes of emesis. On presentation to the ED, the patient described her headache pain as 3 out of 10 in severity. She denied photophobia, had no further nausea, and denied abdominal pain. She reported that the headache worsened with standing and improved with lying down. Review of systems was significant only for pallor.\nThe patient was otherwise healthy with no prior medical issues and taking no regular medications. She was fully vaccinated and had no known allergies. Her family history was significant for frequent headaches in her mother and maternal aunt. She was living with her parents and brother and attending 4th grade.\nVital signs demonstrated blood pressure 111/56, pulse 104, temperature 37.1°C, respiratory rate 22, and oxygen saturation 100% on room air. Initial exam revealed a well-appearing female and was unremarkable including a normal fundoscopic exam and a normal complete neurologic exam.\nThe patient received ibuprofen and oral rehydration and her headache further improved. A presumptive diagnosis of migraine headache was made and was discharged with primary care follow-up the following day.\nTwo days after her initial emergency department visit, the patient returned to the ED with worsening headache, myalgia, subjective fever, and diffuse weakness. The patient's mother reported that the patient was unable to stand or walk and as a result her mother had been carrying her, including to and from the bathroom. The patient endorsed nausea but no further vomiting.\nVital signs demonstrated blood pressure 105/49, pulse 123, temperature 36.9°C, respiratory rate 30, and oxygenation saturation of 97% on room air. On exam, the patient was moderately ill appearing, lying in bed responding slowly to questions. Her lips were noted to be cracked and with some oozing blood. No oral lesions were noted in the mouth. Pupils were equal, round, and sluggishly reactive bilaterally. Neck was supple with no adenopathy noted. Cardiovascularly, she was noted to be tachycardic with a regular rhythm and II/VI flow murmur. Her respiratory exam was normal. Her abdominal exam was benign with no organomegaly. Neurologically the patient was noted to be slow to respond to questions and moving slowly but without focal deficits. She was, however, unable to walk without assistance. Skin exam revealed diffuse ecchymoses on the lower extremities bilaterally.\nLaboratory studies were ordered along with a rapid brain MRI, and pediatric neurology was consulted.\nLaboratory results were as follows: hemoglobin 2.6 g/dL, hematocrit 8.3%, platelets 10K/uL, and WBC 60.5K/uL with 83% blasts in the differential. CRP was 15.7 mg/L, and ESR was 125 mm/h. Electrolytes showed sodium 139 mmol/L, potassium 4.0 mmol/L, chloride 100 mmol/L, carbon dioxide 24 mmol/L, BUN 11mg/dL, creatinine 0.56 mg/dL, glucose 129, magnesium 2.5mg/dL, and phosphorus 3.6 mg/dL. LDH was 359 U/L, uric acid was 2.3mg/dL. PTT was 29.4 seconds, PT was 19.5 seconds, and INR was 1.7. Fibrinogen was 117 mg/dL and D-dimer was >10,000 ng/mL. Review of peripheral blood smear () demonstrated many primitive cells with round and lobated nuclei, numerous cytoplasmic granules with Auer rods readily identified, and some cells with multiple Auer rods.\nRapid MRI Brain () was obtained which demonstrated leptomeningeal enhancement in the supratentorial parenchyma suggestive of leptomeningeal carcinomatosis, a hemorrhagic lesion in the corpus callosum, multiple subdural hematomas with mild mass effect, and petechial hemorrhages throughout the brain.\nPediatric Oncology was consulted and treatment initiated emergently with ATRA, dexamethasone, allopurinol, cefepime, and blood products including packed red blood cells, platelets, and cryoprecipitate. The patient was subsequently admitted to the pediatric intensive care unit.\nIn the pediatric ICU the patient received several transfusions with platelets, cryoprecipitate, and fresh frozen plasma to manage DIC. She was continued on an induction course of chemotherapy including ATRA, dexamethasone, idarubicin, and arsenic trioxide. Molecular analysis of the peripheral blood was positive for PML-RARA, confirming the diagnosis of APL. After the coagulopathy improved and the patient stabilized, lumbar puncture was performed with administration of intrathecal chemotherapy. The cerebrospinal fluid was notable for the presence of leukemia cells, confirming the involvement of the central nervous system. The patient's encephalopathy gradually improved and she returned to her baseline mental status by day 7 of treatment. The patient was noted to have elevated opening pressure on lumbar punctures, which worsened over the course of induction, attributed to pseudotumor cerebri secondary to ATRA.\nUpon completion of the first 28 days of induction therapy, repeat lumbar puncture and bone marrow studies demonstrated no morphologic evidence of acute promyelocytic leukemia, consistent with remission. |
A 72-year-old male who recently migrated from Dominican Republic was seen in the ambulatory clinic with few weeks of intermittent fevers and bilateral lower limb edema. His medical comorbid condition is significant for hypertension and spondyloarthropathy. He required hospitalization of the further evaluation of echocardiographic finding of aortic valve vegetation.\nPatient's illness started with lower back pain 2 months before presentation, further evaluation anemia, and bone lesion leads to bone marrow biopsy concluding the diagnosis of multiple myeloma. Weight loss of 17 pounds during this period was also associated. Review of system was positive for malaise and constipation. He did not report rectal bleeding, change in stool caliber, or difficulty in swallowing. He had a prior history of remote knee surgery in childhood; only social habit was occasional alcohol use and physical examination was unremarkable except for both diastolic and systolic murmur over the aortic area.\nThe initial laboratory parameters reveal hemoglobin of 8.5 g/dl and white cell count of 9.2 x I09 cell/dl (83.6% neutrophils); transthoracic echocardiogram showed preserved ejection fraction, moderate aortic stenosis, and regurgitation with thickening of the aortic valve and independently mobile echo dense structure on the left ventricular outflow tract measuring 1.61 × 0.82 centimeters (see ). Transesophageal echocardiogram showed tricuspid aortic valve with moderate aortic stenosis, moderate aortic regurgitation, and large aortic valve vegetation of 0.45 cm x 1.3 cm attached to right cusp (see ). Three sets of blood culture reported growth of S. gallolyticus from both aerobic and anaerobic bottles with culture and sensitivity depicting sensitivity to Vancomycin and Ceftriaxone. Infectious disease consultation recommended both intravenous (IV) antimicrobials for six weeks.\nHe was started on intravenous Vancomycin and Ceftriaxone and transferred to a higher tertiary center for further surgical management, where he had aortic valve replacement with an Edwards 23 mm bioprosthetic valve. He had an uneventful recovery after completion of a six-week course of antibiotics (Ceftriaxone and Vancomycin) and was referred to the gastroenterology clinic for a screening colonoscopy. The colonoscopy revealed a 35 mm polyp in the sigmoid colon and another 30 mm polyp in the descending colon (see Figures and ), both removed with hot snare polypectomy. He presented the next day with the high-grade fever with blood cultures positive for Streptococcus mitis; repeat echocardiogram showed no vegetation on the native or bioprosthetic valve. He was treated with a repeat course of antibiotics. Pathology of the sigmoid colon polyp was consistent with invasive adenocarcinoma arising from tubular adenoma and the ascending colon polyp revealed tubular adenoma with high-grade dysplasia. After imaging studies, he staged to colon cancer AJCC Stage 1 (Duke's stage A). Subsequently, he underwent a right hemicolectomy with clean resection margins and no evidence of metastasis and made a remarkable recovery. For the management of multiple myeloma, he is being planned for Bortezomib-based chemotherapy followed by stem cell transplant. |
A 32-year-old female with a history of hyperthyroidism status after radiation resulting in hypothyroidism and no history of seizures presented with an acute onset of behavioral changes and witnessed seizure activity. Family history is remarkable for thyroid disease in multiple relatives, but negative for seizure or psychiatric disorders. Behavioral changes included uncontrolled laughter, screaming, signs of agitation, spitting on the floor, complete lack of appetite, and speaking in a British accent. A further history revealed that the patient is of Caucasian descent, was born in Germany, moved to the United States when she was a baby, and has no ties to Britain.\nShe had two seizures both involving tongue biting and postictal confusion with combative behavior. Her workups for seizures, including but not limited to head CT, urine drug screen, and electrolyte levels, were all within normal limits. She, as well as her family, refused MRI and subsequently was discharged on levetiracetam for new onset seizures. On the fourth day of illness (DOI), the patient was admitted to a local community hospital with continued behavioral changes, where an MRI and lumbar puncture (LP) were found to be unremarkable, with a WBC count of 1 cell per mm3. Other CSF parameters include a RBC count of 10 cells per mm3, glucose of 53 mg per dL, and protein of 26 mg per dL. She was subsequently transferred to our hospital care on the fourteenth day of illness because of persisting and worsening behavioral changes. Upon revisiting the initially unremarkable impression of the MRI, abnormal T2 flair hyperintensity in the mesial temporal lobes was noted, with left lobe hyperintensity greater than that of the right lobe (). These features raised suspicion for limbic encephalitis.\nTiters for serum anti-NMDAR and paraneoplastic antibody panel were sent on clinical suspicion. Video EEG () showed frequent focal onset electrographic seizures from the left frontocentral and left frontotemporal region. Some of these electroclinical seizures showed delta brushes (). With strong clinical suspicion for an anti-NMDAR or paraneoplastic antibody related encephalitis, before even receiving antibody titer results, the patient was started on five-day IV steroids on the fourteenth DOI, and IVIG course started on the fifteenth DOI.\nAt this point, multiple differential diagnoses were being considered including autoimmune encephalitides. During hospital admission, the patient continued to remain afebrile. Repeat LP showed lymphocytic pleocytosis, with a quantitative value of 54 cells per mm3 with 98% lymphocytes, after which she was started on empiric acyclovir until HSV PCR was later confirmed to be negative. All other CSF findings were insignificant, with a normal protein level of 16.8 mg per dL, no RBCs, and an elevated glucose level of 95 mg per dL. She continued to have seizures requiring Lacosamide followed by an addition of Lamotrigine. On the fifth day of IVIG course and twentieth DOI, NMDAR antibody was found to be positive in the serum. CT of the chest, abdomen, and pelvis showed no evidence for neoplasms and a transvaginal ultrasound was negative for ovarian teratoma. CSF was positive for NMDAR antibody with a titer of 1:64. After completion of IV steroids and IVIG, there was no significant clinical improvement. She was started on rituximab on DOI 27 for a total of 4 weeks given weekly, with subsequent clinical improvement in addition to no clinical seizures on EEG and resolution of her new onset British accent.\nThe patient continued to have subclinical focal seizures, catatonia, and orofacial dyskinesias with subsequent gradual improvement in her behavior at discharge. When seen for follow-up as an outpatient, she still was found to have some residual memory and cognitive processing deficits. The patient continues to follow up in the hospital for rituximab infusions which is controlling her anti-NMDAR encephalitis and she has now returned back to baseline health. |
We describe the case of a previously fit 62 year-old man who was diagnosed with RCC after presenting with new-onset haematuria. His past medical history consisted of isolated hypertension for which he took felodopine (5mg OD) and bisoprolol (10mg OD). He was a former smoker but had no other risk factors for, or a family history of, renal cancer. He had an open nephrectomy at which there was no evidence of local or regional metastases. The excised right kidney contained an 11cm diameter clear cell RCC for which the Leibovitch score was 9 – a score associated with a 5 year metastasis free survival of 12.7% [].\nSorafenib prolongs progression-free survival in patients with advanced RCC who have failed other treatments [] and shows efficacy as a first-line agent []. Identification of treatments that reduce relapse rate or extend disease-free remission is of utmost clinical importance. The SORCE study is a phase III randomised placebo controlled double-blind study investigating the role of adjuvant sorafenib in patients with resected primary renal cell carcinoma at intermediate or high risk of relapse (Clinical trial identifier: NCT00492258). The primary outcome measure of this three-armed study is disease free survival; comparing those treated with sorafenib for either 1 year or 3 years with placebo. Secondary outcome measures include metastasis-free survival, disease-specific survival time and overall survival.\nThree months post- nephrectomy the patient was enrolled into the SORCE study. At this point he was well and physically active and his blood parameters were within normal range (Figure ). He was commenced on study medication which was later confirmed to be sorafenib 400mg BD. The initial 3 weeks of treatment were free of adverse effects, but at week 4 he noted increasing fatigue. By week 6 of treatment he had developed grade 1 mouth ulceration and grade 2 plantar erythrodysaesthesia. Additionally he suffered malaise, with gastrointestinal discomfort and mild diarrhoea and had noted an abdominal rash. He remained normotensive with a normal full blood count, normal renal function and normal liver function. By week 7 of treatment his symptoms worsened; with increasing fatigue, loss of appetite, nausea, vomiting and diarrhoea. Treatment was withdrawn but at this point jaundice developed. He had not noted change in urine colour or volume, although he complained of abdominal bloating and loose stools. There was no history of fever but he was anorexic with 2kg weight loss. Apart from his prescription anti-hypertensives and the sorafenib, he had taken no other medications including antibiotics, statins or over-the-counter analgesics. He had consumed no alcohol over the study period and his previous alcohol consumption was negligible. On examination, he was normotensive, euvolaemic and was clearly jaundiced with non-tender hepatomegaly. He was admitted to hospital at this point for further management.\nAdmission blood tests revealed an acute hepatitis with an ALT 6935 (normal 10-45IU), bilirubin 288μM (3-17μM), alkaline phosphatase 577IU (35-320IU) (Figure ) and a prothrombin time of 18.2s (n=12-15s). LDH was markedly raised at 1226IU (n<260IU). The full blood count was normal without leukocytosis whilst CRP was moderately raised (34mg/dl, n<8mg/dl), a level that persisted throughout treatment. His creatinine was moderately raised at 201 μM, the baseline for this patient being 120-140 μM post-nephrectomy. An abdominal ultrasound demonstrated normal hepatic echogenicity with normal calibre common bile duct and patent hepatic vasculature. There was no obstructive uropathy. Viral screening excluded acute cytomegalovirus and Epstein Barr virus and hepatitis viruses. An immunology screen was negative for ANA and ANCA but identified complement consumption with reduced C3 (29mg/dl, 65-190mg/dl) and C4 levels (13.6mg/dl, 14-40mg/dl). A pyrexia of 37.9C was recorded on day 2 of admission and the patient was given empirical ceftriaxone, although a septic source was not identified and blood cultures were uniformly negative. There followed marked deterioration in hepatic and renal function with rising prothrombin time refractory to Vitamin K and requiring repeated plasma transfusions. On day 4, the patient became encephalopathic and was transferred to the intensive care unit where he was intubated. A repeat ultrasound revealed mild peri-hepatic ascites which was subsequently found to be transudative in nature. Further deterioration of renal function precipitated initiation of continuous veno-venous haemofiltration. Over the subsequent three days, despite maximal supportive therapy including N-acetyl-cysteine, there was progressive deterioration. His previous renal tumour excluded him from candidacy for liver transplant. Hypoglycaemia and lactataemia ensued and by day 7 the prothrombin time had risen to >200s and serum ammonium concentration was 120μmol/L (<35μmol), signifying end-stage hepatic failure, and treatment was withdrawn.\nAt autopsy there was no evidence of metastatic disease. A terminal bronchopneumonia was noted although organisms were not isolated. Histology of post-mortem liver showed a lobular hepatitis with mononuclear cell infiltrate and hepatocyte necrosis (Figure a, H&E stain). Histology of the kidneys demonstrated acute tubular necrosis with cellular debris and oxalate crystals within tubules (2b, H&E stain) and collecting ducts (2c, H&E stain). In addition, collecting ducts contained small numbers of myoglobin casts (2d, immunoperoxidase stain). |
A 36-year-old woman with no past medical history was admitted to hospital after slipping on the ice. The patient did not lose consciousness, but she immediately noted drooping of her right eyelid and double vision when she elevated the lid. There was a 3 cm laceration wound on the chin and some abrasions over the neck and the central region of her face ().\nThe right pupil was fixed and dilated, and there was complete right ptosis. The patient could not elevate, depress, or adduct the right eye, but the eye intorted on attempted downgaze (). The right pupil measured 5 mm in diameter and was sluggish reactive to light. Ophthalmologic tests of vision and intraocular pressure were within normal ranges in both eyes. Further physical and neurological examinations were normal. Skull X-rays and orbital computed tomography (CT) scans immediately after the accident failed to show fractures. MRI and MR angiography of the brain did not reveal any pathology on admission (). Contrast-enhanced spoiled gradient-recalled (SPGR) MR sequences with multiplanar reconstruction (MPR) was subsequently performed to produce high-resolution images of the cisternal portions of the cranial nerves. The 3D MR studies indicated a slightly rough-shaped ON near the the posterior petroclinoid ligament (PCL), but no nerve enhancement (). The patient's history, along with the ophthalmologic symptoms and the radiologic findings strongly suggested the diagnosis of an isolated lesion in the course of the ON without further damage in the right oculomotor nucleus.\nShe was then treated with oral prednisone in a tapering dose over several weeks. MPR of contrast-enhanced 3D MRI taken 2 weeks after the event clearly demonstrated strong enhancement and enlargement over the whole length of the right ON (). At the time of follow-up two months after trauma, her ptosis slightly improved. Three months later, ptosis was completely resolved, and there was some restoration of ocular motion in the direction of adduction. Follow-up MRI 6 months post-injury showed complete resolution of the enhancement and swelling of the right ON (). Nine months after the injury, the patient still suffered from symptomatic glare and blurred vision for near objects, although complete recovery of diplopia was seen. Four months later, spontaneous improvement of pupillary dilatation was observed, and ophthalmology examination also revealed no paralysis of accommodation. |
An 18-year-old Greek male student presented for screening test in order to get a health certificate. Physical examination revealed splenomegaly, although the patient was asymptomatic. No other clinical signs were detected. Ultrasonography and Magnetic resonance imaging (MRI) showed a hypoechogenic mass involving the spleen and the splenomegaly (Figure ). Preoperative evaluation led to the possible diagnosis of hemangioma of the spleen. The patient was subjected to laparoscopy, which demonstrated a solid tumor of the spleen. Laparoscopic splenectomy was performed. Operative time was 65 minutes. Postoperative recovery was uneventful, the drain was removed on the second postoperative day and the patient was discharged on the same day. Histologic examination showed a capillary hemangioma of a spleen weighing 850 gr. The patient remains asymptomatic 6 months after the operation.\nFollowing induction of general anesthesia and endotracheal intubation, a nasogastric tube and a urinary catheter were inserted, and compression stockings were applied. The patient was placed on a beanbag in a 60-degree right lateral decubitus position. The right brachial plexus was protected with a pillow roll and the left arm was supported by a splint. The patient was positioned so that the table could be flexed to create a wider working space in a reverse Trendelenburg position. The surgeon and the scrub nurse stood to the patient's right, and the assistants stood to the left. The video monitors were placed on each side of the table, above the level of the patient's shoulders.\nPneumoperitoneum was established using a Veress needle to a pressure of 12 mm Hg. Four ports were used: the first 10-mm trocar was inserted at the left margin of the umbilicus. The other three 5-mm operating trocars were positioned as follows: the first trocar was on the median line, 8 cm above the umbilicus, a second operating trocar in a left subcostal position on the axillary line to enable gastroepiploic mobilization, and a left 5-mm operating trocar was positioned in the left lateral position, 10 cm from the umbilicus (Figure ).\nThe operation is begun with a thorough search of the abdominal cavity. The inferior pole of the spleen was lifted superiorly. The splenocolic ligament was divided (Figure ). This mobilized the inferior aspect of the spleen and allowed the spleen to be retracted cephalad. Great care was taken to avoid rupture of the splenic capsule during retraction. The lateral peritoneal attachments of the spleen, the splenorenal and splenophrenic ligaments were sequentially incised (Figure ). The splenic hilum was approached from the lower pole and dissection was continued cephalad. With the spleen elevated, the short gastric vessels and main vascular pedicle were visualized. The tail of the pancreas was also visualized and avoided at this point as it approached the splenic hilum. The splenic pedicle was carefully dissected from the medial and lateral aspects. After the short gastric vessels had been divided with ultrasonic dissector, the splenic artery and vein were dissected. The vessels were divided by application of endoscopic vascular staplers (ENDOPATH®, ETS Flex 45 Endoscopic Articulating Linear Cutter 45 mm staple line, 2.5 mm Staple Leg Length (Vascular/Thin). 45 MM Vascular/Thin). Each jaw was positioned anterior and posterior to the splenic vessels (Figure ). The instrument was fired two times in sequence. To remove the detached spleen, a nylon extraction bag was introduced through the left lateral trocar site. The bag was opened within the abdominal cavity. An incision adequate to enable removal of the bag containing the intact spleen was made at the left lateral site. The spleen was placed into the specimen retrieval bag. The drawstring was grasped, and the bag was closed and drawn out. The laparoscope was reinserted and the splenic bed was assessed for hemostasis. Finally, peritoneal irrigation was carried out and a Rob drain tube N° 24 was placed at the residual cavity. |
This 40-year-old man developed daily headaches and was referred to an ophthalmologist when these were complicated by visual blurring. Fundoscopic exam revealed papilledema, prompting imaging studies. Head CT and MRI scans demonstrated a 5-cm partially calcified, partially enhancing right frontoparietal mass with extension into the splenium of the corpus callosum. There was associated cerebral edema and mass effect with compression of the right lateral ventricle. On the day of the MRI, the patient developed left sided weakness and had a seizure. The patient subsequently underwent a craniotomy with resection of the contrast enhancing component and a portion of the non-enhancing component. Adjuvant therapy included 12 cycles of temozolomide chemotherapy, but this had to be discontinued after the 12th cycle due to severe rash. The last MRI scan performed 4.9 years after initial presentation showed stable disease with residual non-enhancing tumor and otherwise, the patient is doing well.\nThe pathologic findings in case 3 were nearly identical to those of case 2, except that there was a greater degree of intermingling between the two elements and the zones of infarct-like necrosis within the oligodendroglial component were larger. Synaptophysin staining revealed strong cytoplasmic and Golgi pattern positivity in dysmorphic ganglion cells, while only rare oligodendroglial cells showed cytoplasmic reactivity. There was extensive GFAP positivity with most positive cells showing morphologic features of gliofibrillary oligodendrocytes. However, irregular cells with elongate processes were also seen, suggesting a minor astrocytic component. Both components were Neu-N negative. The CD34 stain highlighted endothelial cells only; tumor cells were negative. The Ki-67 labeling index was low to moderate, focally reaching a maximal staining of 15% in the oligodendroglial component; it was <1% in GGLF. FISH analysis revealed chromosome 19q deletion in both components, while chromosome 1 studies were non-informative due to weak signals. Nevertheless, loss of heterozygosity studies were also performed on this tissue at the Memorial Sloan Kettering Cancer Center and allelic losses of both 1p and 19q were detected. Therefore, this tumor was interpreted as being codeleted. |
We present a 16-year-old boy referred from urology for further evaluation of hypogonadotrophic hypogonadism. He was initially diagnosed with bilateral cryptorchidism aged 2, and his parents opted for conservative management. There was no spontaneous descent of testes in the next few years and he was eventually lost to follow up. He was referred again to urology aged 12, for recurrent urinary tract infections and was diagnosed with vesico-ureteric reflux (VUR) warranting bilateral ureteric stenting and implantation. There were no other structural or functional abnormalities of the renal system identified. Cryptorchidism was reevaluated at this point and he underwent bilateral orchidopexy, with successful descent only on the left side. Routine clinical and hormonal assessment done at this juncture revealed hypogonadotrophic hypogonadism, warranting an endocrine consult.\nDetailed history taking from parents and physical examination elicited the following salient clinical features. He was born full-term with an uneventful antenatal history. His height and weight remained within the 25th and 50th centile till the age of 10. A decline in growth rate was noticed as he approached adolescence, whereby he was one of the shortest amongst his peers in secondary school. He had been a slow learner, with poor academic performance since primary school. His parents had noticed him having low energy levels and fatiguability in the later years. No history of anosmia or hearing deficits was elicited. He had no history of polyuria or polydipsia to suggest cranial diabetes insipidus. A full systemic review did not reveal symptoms to suggest intra-cranial mass effect such as chronic headaches, visual disturbances or neurological deficits. He had no past history of intracranial pathology, trauma or radiation. Apart from the history of VUR and cryptorchidism, he did not suffer from any chronic diseases nor receive long term immunosuppression/steroids or chemotherapy. He has no family history of short stature nor delayed puberty. He has one younger sibling, a 12-year-old female who has achieved puberty and is of appropriate height. His anthropometric measurements of weight and height were charted as 58 kg (between 5-10th centile), 155 cm (below 5th centile) respectively. His height was significantly lower than his mid-parental height of 170 cm. On physical examination, he had no midline facial deformities to suggest a syndromic constellation. Secondary sexual characteristics were also absent. Tanner staging for pubic hair and genitalia was pre pubertal (stage 1) with absent pubic hair, micropenis, minimal hyperpigmentation with no ruggae over the scrotum and testicular volume measuring approximately 1 ml (left) and absent (right).\nHis anterior pituitary hormone panel showed hypogonadotropic hypogonadism with low FSH, low LH and undetectable testosterone levels. Insulin- like growth factor 1 (IGF-1) levels was found to be lower than expected for his age and gender raising the suspicion of concomitant growth hormone (GH) deficiency. His other pituitary hormones were intact. (). Insulin Tolerance Test (ITT) with testosterone priming was carried out to confirm growth hormone deficiency. This test was inconclusive due to failure to achieve adequate hypoglycemia.\nAs an alternative test, Glucagon Stimulation Test (GST) was chosen. GST showed a peak GH of 6.94 ng/ml (>10 ng/ml) at 90 mins confirming his growth hormone deficiency. Bone age assessment showed his skeletal age to lag at the age of 12-13 years with unfused epiphyseal plates. A MRI pituitary ordered showed an enlarged pituitary stalk extending to the tuber cinereum with homogenous enhancement post gadolinium contrast. The stalk measured 13 mm (AP diameter) at the point of insertion at the infundibulum with uniformed pattern of thickening. The pituitary gland itself was not hypoplastic, with no focal enhancement on post contrast study. The optic nerve, chiasm and tract was not thickened, with no evidence of compression. ( and ). Therefore, at this point it was concluded that this patient had thickened pituitary stalk with partial hypopituitarism (hypogonadotropic hypogonadism and growth hormone deficiency).\nThe etiology of thickened pituitary stalk was worked up extensively involving laboratory and radiological investigations. His complete blood count showed no evidence of hematological dyscrasias. His liver and renal biochemistry was normal. Both inflammatory and tumor markers were not raised. There was no evidence of mediastinal or lung mass on chest x-ray. Ultrasound showed very small testes in the right inguinal region and left scrotum measuring 1.2-1.5 cm, both testes had no suspicious malignant features or associated lymphadenopathy. In summary, there were no red flags to suggest an inflammatory or neoplastic process. A multidisciplinary discussion was held to discuss the role of pituitary stalk biopsy for him. The presence of pre-pubertal features representing gonadotroph deficiency early in life coupled with non-suspicious laboratory and imaging workup was highly suggestive of congenital origin of disease. Therefore, a decision was made not to proceed with pituitary stalk biopsy unless there is evidence of disease extension or progressive loss of pituitary function.\nGrowth hormone, norditrophin at a dose of 0.025 mcg/kg/day was initiated in this boy aiming to achieve near adult height. He showed good response to therapy with a 4 cm increment in height after 3 months. Puberty induction will be initiated once acceptable near adult height is achieved. Serial MRI pituitary and pituitary hormone panel will be repeated every 6 months to monitor progression of disease. He is also planned for removal of the right testis due to risk of malignancy and poor function. |
A 60-year-old male was evaluated for insidious onset of right foot pain over 4 months, made worse by walking and standing. Radiographs at an outside facility were reportedly normal. Two corticosteroid injections into the sinus tarsus provided no relief. Upon referral new radiographs were obtained (Fig. ). Magnetic resonance imaging (MRI) showed a complex lesion in the anterior calcaneus with some surrounding edema and potential concern for cortical disruption (Figs. , ). A computed tomography (CT) guided biopsy of the cyst showed a lytic destructive lesion within the central and anterior process of the calcaneus. There was cortical destruction along multiple sites at the margins of the lesion and slight scattered calcifications (Fig. ). The pathologic report revealed cords and clusters of epithelioid cells and foci of spindle cells in a myxochondroid matrix (Figs. , ). Some of the epithelioid cells contained vacuoles and rare erythrocyte (Fig. ). Immunohistochemical stains identified CD31, CD34, and CAMTA1 (Figs. , ). Mixed cytokeratin stain (MCK) showed very focal and equivocal staining and D2-40 and epithelial membrane antigen (EMA) were negative. There was extensive necrosis and some spindling but no invasion of the surrounding bone. These findings supported a diagnosis of epithelioid hemangioendothelioma.\nThe patient was referred to our orthopaedic clinic roughly 6 months after initial presentation. The right lower extremity was neurovascularly intact with no swelling, masses, or skin lesions. There was mild tenderness to palpation of the calcaneus and no palpable nodes in the popliteal or inguinal regions. At this time, radiographs showed a significant cystic lesion with cortical breakthrough and no evidence of collapse. Routine bloodwork was normal. Positron emission tomography/computed tomography (PET/CT) ruled out proximal metastasis; however, bilateral hilar and mediastinal adenopathy were reported along with increased signal in the stomach and bilateral parotid glands.\nThe patient was taken to the operating room for excision and curettage with argon beam followed by cement filling of the lesion and percutaneous pinning. A biopsy taken during surgery showed the tumor to be histologically the same as the previous biopsy, but now involving the surrounding bone. A thorough oncology and pulmonary workup for the mediastinal adenopathy ruled out metastatic disease, as a biopsy showed a benign anthracotic lymph node with marked histiocytosis. The patient returned to weight bearing activity at 3 months and work after 6 months. CT scan and radiographs of the hindfoot at his 6 month and 3 year follow-up showed well fixed hardware without recurrence (Fig. ). PET scan at 3 years illustrated stable hilar lymphadenopathy and absence of enhancement of the calcaneus. Other than tolerable pain with extended walking, the patient has made full and tumor free recovery at 3 years. |
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