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A 53-year-old African American man was brought in by his wife with new-onset seizures. The patient was unable to provide an accurate history due to altered mental status. Per the patient's wife, the patient had stopped drinking alcohol 24–48 hours prior to admission due to financial constraints, after consuming two pints of whiskey per day for the previous 10 years. The seizures began the evening prior to admission and continued throughout the night. These episodes lasted 1-2 seconds, were tonic-clonic in nature, and were associated with urinary incontinence and foaming at the mouth. His past medical history was significant only for hypertension. He had smoked one pack of cigarettes per day for the past 48 years. Upon arrival, his temperature was 37.1 degrees Celsius, pulse was 94 beats per minute, and blood pressure was 160/98 mmHg. Cardiovascular exam revealed a regular rate and rhythm with normal S1 and S2, abdominal exam disclosed hepatosplenomegaly, and neurologic exam was significant for tremulousness. The patient was disoriented on mental status exam. Mean corpuscular volume was 92.3 fL, platelet count was 58 × 103 per μL, AST was 199 units/L, and ALT was 89 units/L. An ECG done upon arrival revealed a normal sinus rhythm and QTc interval of 546 ms.\nGiven the recent onset of seizure-like episodes, history of abrupt discontinuation of chronic alcohol use, exam findings, and lab values consistent with ethanol abuse, a diagnosis of acute alcohol withdrawal was suspected. The patient was admitted to the medical intensive care unit. Treatment was initiated with diazepam 5 mg IV every 8 hours and lorazepam 2 mg IV every 1 hour as needed for agitation and anxiety. The patient was also given 100 mg of thiamine, 1 mg of folic acid, and 2 gm of magnesium sulfate IV every 24 hours.\nDuring the 1st hospital day, the patient exhibited witnessed tonic-clonic seizure activity, as well as significant anxiety and agitation. During the 2nd and 3rd hospital day, the patient's mental status fluctuated significantly. Intermittently, the patient was severely agitated, attempting to dislodge his indwelling urinary catheter and requiring 4-point soft restraints. Throughout this period, temperature peaked at 37.4 degrees Celsius, pulse ranged from 71 to 99 beats/minute, and blood pressure ranged from 118 to 154/88–103 mmHg. Diazepam was changed to 7.5 mg PO every 6 hours; however the patient still required lorazepam 2 mg IV every 4 hours for anxiety and agitation.\nOn the 4th hospital day, the patient developed hypotension. Blood pressure was 90–100/70–80 mmHg, and pulse was 82–99 beats/minute. The patient was significantly altered and was therefore unable to report subjective symptoms. ECG disclosed symmetric deep coving T waves in leads II, III, aVF, and V2–V6, as well as a prolonged QTc interval of 645 ms. Serum troponin I was elevated at 1.25 ng/mL. Repeat ECGs continued to show symmetric T wave inversions in the inferior limb and lateral chest leads. Serial troponin levels, however, trended downward. An urgent transthoracic echocardiogram revealed multiple left ventricular regional wall motion abnormalities, notably akinesis of the mid-distal anteroseptal, mid-distal anterolateral, mid-distal inferoseptal, mid-distal inferolateral, and apical segments (). Of note, right ventricular systolic function was normal, and there was no mitral regurgitation. The left ventricular ejection fraction was estimated to be 40–45%. The clinical instability of the patient precluded emergent coronary angiography. Given the widespread repolarization abnormalities by ECG, diffuse wall motion abnormalities involving myocardial segments circumferentially throughout the entire cardiac apex, presence of an identifiable stressor, and downward trend of cardiac troponins, the diagnosis of stress cardiomyopathy was suspected. The patient continued to require additional doses of lorazepam 2 mg every 2 hours due to agitation and anxiety. On the 5th hospital day, adjunctive treatment with the alpha-2-adrenoreceptor agonist dexmedetomidine was initiated to reduce central sympathetic outflow, directly targeting the presumed pathobiology of the stress cardiomyopathy. A loading dose of 1 μg/kg IV was given over 10 minutes, followed by a maintenance infusion of 0.2 μg/kg/hr titrated by 0.1 μg/kg/hr every 15 minutes to maintain a Richmond Agitation-Sedation Scale of −1. The mean infusion rate was 0.31 μg/kg/hr, administered over a period of 23.93 hours ().\nOn the 6th hospital day, the patient showed marked clinical improvement. Per the patient's family, mental status was at baseline. The patient denied shortness of breath or chest pain. Cardiovascular and pulmonary examinations were unchanged, and neurologic examination revealed no agitation, anxiety, or tremulousness. The patient was awake, alert, and oriented to person, place, and time. Dexmedetomidine therapy was weaned by 0.1 μg/kg/hr every 15 minutes. The patient was treated thereafter with tapering doses of diazepam PO, and no additional doses of lorazepam were required. A coronary angiogram was offered to the patient to rule out coronary artery obstruction; however the patient refused the procedure. One month after hospitalization, repeat ECG revealed improvement of the T wave inversions when compared to the ECGs during the event. The QTc was shortened to 495 ms. Coronary CT angiography was performed, which revealed absence of coronary artery disease. Repeat ECG two months later revealed a normal sinus rhythm with no significant T wave inversions. Subsequent echocardiography performed 14 months after the event revealed resolution of the acute regional wall abnormalities and confirmed a full recovery of ventricular systolic function, further supporting the diagnosis of stress cardiomyopathy (). |
A 67-year-old woman presented with severe paroxysmal cramp-like pain on the right side, including the head and both upper and lower extremities. The pain started 5 years ago, and there was no obvious cues that preceded or elicited the episodes. The pain was initially mild and occasional, but gradually intensified to an unbearable degree with an average of 10–15 daily episodes, each lasting for 5–10 mins. The pain had profound impact on daily life: the patient was practically confined to a wheelchair due to muscle stiffness. She was profoundly anxious and depressive, and attempted a few unsuccessful suicides.\nThe patient had a “hemorrhagic stroke” 10 years ago that resulted in hemiplegia on the right side. Both the right arm and legs were completely paralyzed, but voluntary movements gradually recovered. She was able to walk alone slowly using a cane and manage an independent life. Five years after the stroke, cramp-like pain emerged. Despite of treatments with NSAIDs, tramadol, duloxetine and gabapentin, the pain gradually increased in intensity and episode frequency. Motor cortical stimulation and deep brain stimulation were offered but refused.\nUpon assessment using a 10-point visual analogue scale, the pain intensity was 7–10. The Barthel activity of daily living score was 40 (the 100 maximum indicates full functioning) (). Hospital anxiety and depression scale (HADS) was 12 for anxiety and 14 for depression. The patient reported stiffness throughout the right side of the body, and heavy sweating and on the right side of the face. Thermosensation in the right arm and leg, particularly to cold stimuli, was impaired. Muscle tone was significantly increased, with intact but diminished deep tendon reflex. Cranial CT and MRI were consistent with lesions to the left dorsal thalamus and medial temporal lobe (). Extensive hemosiderosis was apparent, indicating hemorrhagic stroke. She had hypertension for 10 years, but blood pressure was well controlled.\nStellate ganglion block was performed, as described previously. Briefly, 2-mL 2% lidocaine was injected between the internal carotid artery and the longus colli at the C7 level under ultrasound guidance ()., The block was repeated once every day, and the pain subsided rapidly in both intensity and frequency along with the advancement of daily treatment. On the seventh day after treatment initiation, the patient no longer had pain episode. Thermosensation normalized, and she was able to walk for 200 m on her own. A physical workup showed reduced muscle tension and normalized deep tension reflex. Stiffness persisted, but at a reduced degree that could be readily managed with gabapentin at small doses. At the last follow-up 9 months later, the patient was free from pain and only took gabapentin on-demand occasionally. A repeat cranial CT () was unremarkable. ADL score increased to 80. HADS score was 6 for anxiety and 8 for depression. |
A 39-year-old Asian male presented to our department with a chief complaint of a painless swelling involving the left lateral border of the tongue. History revealed that the growth began as a pea-sized nodule and gradually increased in size to its present state over a period of 4 months.\nThe past medical history did not reveal any cardiovascular, respiratory, genitourinary, gastrointestinal, endocrine, haematological, neurological, or any other medical history of relevance. The past dental or familial history was not of any consequence to our particular case. A complete blood count was performed and no abnormality was detected. No history of trauma, bleeding, pain, or paresthesia was present. There were no signs of any cervical lymphadenopathy noted. Orthopantomographic examination did not reveal any bony abnormalities. Extraoral examination revealed multiple soft cutaneous nodules involving either side of the face, back, trunk, and the lower and upper extremities. They were round to oval in shape and were of various sizes ranging from a few millimetres to centimetres across. On palpation they were found to be sessile and some pedunculated also, soft to firm in consistency, nontender, and noncompressible and showed no signs of fixity. Around 15 café au lait (coffee in milk) macules were present over 15 mm in diameter throughout the body with increased prevalence in the back and trunk. The largest one among these was located over the right arm measuring a whopping 22 × 13 cm across with smooth borders. It was roughly ovoid in shape and there was a brownish macule with long thick dark hair involving the surface of the lesion and it extended from the acromioclavicular joint all the way down to the upper arm (). The distribution of the lesion did not follow the lines of Blaschko. Rubbing the affected area exhibited a pseudo-Darier sign which consisted of a transient piloerection. Neither axillary freckling (Crowe's sign) nor Lisch nodules were noted in our case.\nIntraoral examination revealed a sessile lesion with a lobulated appearance and measured 2 × 1.5 cm in greatest dimensions. It was roughly ovoid in shape with irregular borders and exhibited a smooth surface. The periphery was non-erythematous in appearance. On palpation it was found to be nontender, firm in consistency, and fixed to the underlying tissue. It was noncompressible, nonreducible, and nonpulsatile in nature (). The slow-growing, asymptomatic nature of the lesion with the presence of well circumscribed margins led us to give a provisional diagnosis of a benign lesion. Based on the positive clinical features in our extra- and intraoral assessment of the patient, a provisional diagnosis of a neurofibroma was considered, taking into consideration the fact that the patient revealed pathognomonic signs of neurofibromatosis type 1 extraorally.\nConsidering the clinical presentation and localization of the lesion, we included a neurofibroma, schwannoma, neurilemmoma, granular cell tumour, reactive lesions like a giant cell fibroma or focal fibrous hyperplasia, leiomyoma, rhabdomyoma, hemangioma, lymphangioma, lipoma, and benign salivary gland tumours among differential diagnosis taking into account the peripheral exophytic nature of the lesion.\nRoutine haematological examination revealed a normal blood profile and no other imaging findings for soft tissue analysis like ultrasonography or MRI were performed considering the miniscule proportions of the lesion and its benign presentation. Patient was also referred to the adjacent medical college to rule out the possibility of any internal lesions. Excisional biopsy of the tumour mass on the tongue was performed in toto and primary closure achieved with a single interrupted Vicryl suture. The patient was prescribed an NSAID medication and asked to use povidone-iodine mouth rinse.\nHistopathological examination which is the investigation of choice in peripheral exophytic lesions involving the tongue revealed a section with densely collagenous stroma with proliferation of spindle cells as fascicles with thin wavy nuclei in irregular pattern. Numerous plump fibroblasts were present and there were many vascular channels. Connective tissue is lined by stratified squamous epithelium (). The specimen further underwent immunohistochemical analysis, which can be considered a gold standard, and was found to be immunoreactive for S-100 stain which was positive for the spindle cells, thereby, signifying its origin from neural crest tissue and confirming our diagnosis of a neurofibroma (). The lesion on the shoulder underwent an incisional biopsy and on histopathological examination revealed numerous elongated rete ridges with melanin pigmentation of the basal layer with no increase in the number of melanocytes. The specimen tested negative for S-100 protein ().\nThe postoperative healing was uneventful and patient was followed up after two weeks and subsequently after 6 months. The patient was also advised to report for periodic follow-up visits due to the potential for neurofibromas to undergo malignant transformation. |
During routine cadaveric dissection for teaching undergraduate students, it was found that in a 52-year-old male cadaver, the celiac artery originated as a ventral branch of the abdominal aorta. The left inferior phrenic artery arose from the main trunk of the celiac artery at a distance of 2 cm from the origin of the celiac trunk. It went on to supply the left dome of the diaphragm. The right inferior phrenic artery had a normal origin from the abdominal aorta, and it followed its usual course. The celiac artery then gave a hepatogastric trunk at a distance of 2.5 cm from its origin. The hepatogastric trunk gave three branches: the left gastric artery (LGA) at a distance of 3 cm from the origin, esophageal branch at a distance of 4 cm from the origin, and thereafter it continued as the left hepatic artery. Further, from the celiac trunk arose the splenic artery and a common hepatic artery. The common hepatic artery trifurcated into three terminal branches: a middle hepatic artery, the right gastric artery, and the gastroduodenal artery []. The gastroduodenal artery traversed behind the first part of duodenum and bifurcated into the right gastroepiploic artery and the superior pancreaticoduodenal artery.\nThe right hepatic artery arose as a branch of the superior mesenteric artery at a distance of 3 cm from the origin of superior mesenteric artery. It gave origin to the cystic artery at a distance of 8 cm from its origin. On exploration of the three hepatic arteries, we found that the middle hepatic artery supplied segment I or the caudate lobe, the right hepatic artery supplied segment V, VI, VII, and VIII and the left hepatic artery supplied segment II, III, and IV of the liver. In addition, the middle hepatic artery had a relatively smaller diameter in comparison to the left and right hepatic arteries. |
A 60-year-old male presented to the ENT clinic in November 2008 with a two-month history of dysphonia. There were no other symptoms apart from lethargy. His past medical history included mild chronic obstructive airway disease which required no treatment. He was a cigarette smoker: 40 pack years and consumed approximately 20 units of alcohol per week mostly in the form of spirits.\nOn examination, he appeared systemically well, with no peripheral stigmata of chronic disease. Examination of the neck revealed bilateral cervical lymphadenopathy. There were multiple small (<2 cm), firm, well-circumscribed masses bilaterally which were mostly distributed within levels II-III of the neck. Flexible nasendoscopy performed in the clinic showed a fixed left hemilarynx. Examination under anaesthesia confirmed the findings of the flexible nasendoscopy, a large tumour of the left vocal cord crossing the anterior commissure to the right side along with slight subglottic extension.\nA staging CT scan of the head and neck was undertaken which showed a transglottic mass with significant local invasion and confirmed the bilateral enlarged cervical lymph nodes. No distant metastases were noted, including clear lung fields ().\nTwo weeks after presentation, total laryngectomy and bilateral modified radical neck dissections were performed. The histology revealed a 3.5 cm moderately differentiated (grade 2) SCC with microvascular, perineural, and thyroid cartilage invasion (T4). In addition, bilateral lymph nodes were positive for metastatic SCC with extracapsular spread evident on the left side (<6 cm greatest dimension). This cancer was therefore staged as T4N2c.\nIn January 2009 (2 months postoperatively) the patient attended a routine follow-up appointment. He was well and had no symptoms of local recurrence or distant metastatic spread; however, on examination a small parastomal lesion was seen. Prompt local excision of this was performed and histology showed a parastomal recurrence. As a consequence, chemoradiotherapy was started.\nSix months after the initial presentation, the patient returned with a 5-week history of a mild swelling in the extensor compartment of his right forearm (May 2009). The forearm was swollen and mildly tender, but there was no erythema, and nothing sinister was suspected (). A short course of amoxicillin was started with no reduction in the size of the swelling. Orthopaedic review in July 2009 reported no loss of range of movement or injury to the elbow. During this time, the swelling had enlarged and was reported to be 10 × 7 cm. Subsequent fine needle aspiration of the lesion revealed metastatic SCC with similar morphology to the laryngeal primary cancer. An MRI of the forearm showed that the lesion was confined to the muscle. The patient was managed palliatively and unfortunately died 6 months later. |
At 34 weeks of gestation, a 31-year-old gravida 3 para 1 woman was referred to our emergency department from a local clinic owing to progressive epigastric pain associated with nausea and vomiting for 3 days. She had no significant medical or surgical history and no history suggestive of thromboembolism. She had never used any oral contraceptives or other hormonal preparations. Upon arrival, her vital signs were stable without fever (BP 120/78 mmHg, pulse rate 88/min, respiratory rate 19/min, and body temperature 37.1°C). The hematologic examination revealed marked leukocytosis (WBC 24,200/CMM) and relative intravascular depletion (hematocrit 44.5%). Coagulation profile and biological tests were within normal limits. Obstetric ultrasound showed a normal male fetus compatible with his gestational age. In addition, a mild fatty change in the liver and a moderate amount of ascites were also noted. The fetal monitor showed that the uterus contracts every 10 minutes. She was initially kept for conservative treatment (fasting, nasogastric suction) and absolute bed rest under the suspicion of acute gastroenteritis and preterm labor. However, signs of acute peritonitis gradually developed within 6 hours after admission. Thus, an emergency exploration was performed to find a segmental gangrene of the small intestine with 1,500 mL of serosanguineous peritoneal fluid, but no frank obstruction and perforation was identified (). The premature baby, at 1,850 grams, was then delivered via cesarean section, with an Apgar score of 4 at one minute and 6 at five minutes. The gangrenous small intestine was resected, and a primary anastomosis was performed. Her postoperative course was uncomplicated, and she was discharged one week after surgery. The outcome of the baby was also excellent without any sequela after intensive care. The pathologic examination revealed mucosal denudation, submucosal edema and hemorrhage, and inflammatory infiltration in the muscularis propria. The intramural and mesenteric vessels in the specimen were patent with blood stasis (). Screens for inherited thrombotic disorders (protein S, protein C, antithrombin III deficiency, and factor V Leiden mutation) for the patient were done after surgery to demonstrate negative results. Four years later, the patient was pregnant again and delivered at term without any thromboembolic event throughout the course. Till now she has been recurrence-free for 7 years without anticoagulant therapy. |
A 5-year-old boy presented to a tertiary hospital with 1-week history of pyrexia and cough. He was diagnosed with PCD at 1 month of age, having been noted to have both dextrocardia and persistent neonatal respiratory distress. Diagnostic investigations demonstrated static cilia throughout the sample on high-speed video microscopy analysis and an outer dynein arm defect on ciliary ultrastructure analysis by transmission electron microscopy. Subsequent testing showed he had extremely low levels of nasal nitric oxide (nNO) at 25 parts per billion (8.33 nl/min) and genetic tests demonstrated that he is heterozygous for two known disease-causing mutations in the dynein axonemal intermediate chain 1 (DNAI1) gene.\nIt is often challenging in children with PCD to identify respiratory exacerbations as, even when well, they have a persistent wet cough []. However, in the three months prior to presentation, he had received 2 courses of oral antibiotics for respiratory exacerbations. These had presented with increased productive cough, wheeze and fever. Both courses of antibiotics led to his symptoms temporarily improving to his baseline daily cough but deteriorating soon after the course was complete.\nOn admission, he had bilateral wheeze and crepitations at lung bases, which were more evident on the right side. His chest X-ray showed extensive collapse of his right side lower lobe with volume loss (). He was initially treated with aggressive airway clearance physiotherapy before administration of hypertonic 7% saline nebulisers and intravenous (IV) antibiotics (Cefuroxime 50 mg/kg, four times daily). However, his clinical signs did not improve and hence he went on to have a flexible bronchoscopy under general anaesthetic. This demonstrated mucus plugging of the right-sided lower bronchial division, which was suctioned out. At the time of his bronchoscopy, a peripherally inserted central catheter (PICC) line was placed to facilitate a prolonged course of IV antibiotics.\nA week after bronchoscopy, with ongoing use of airway clearance physiotherapy, mucolytic agents (hypertonic saline and Dornase alfa (recombinant human deoxyribonuclease (DNase) nebulisers) and IV antibiotics, a further chest X-ray demonstrated re-expansion of the right lower lobe (), accompanied by an alleviation of his respiratory symptoms. He was discharged two weeks after admission with ongoing hypertonic 7% saline nebulisers, to be given twice daily prior to physiotherapy, and 2.5 mg DNase nebulizer, to be given once daily after physiotherapy. |
A previously healthy 19-year-old Chinese man presented with weakness of his right limbs that rapidly worsened over a short interval. He had initially felt a weakness of his right lower limb six months prior to presentation. An X-ray of his right ankle at that time had been normal and no treatment was given. Two months ago, our patient felt a weakness of his right upper limb and the symptoms of his right lower limb worsened. At the same time, he developed fever (highest temperature, 39.5°C), blurred vision in his left eye and a mild episodic headache lasting for several minutes on each occasion. No nausea or vomiting occurred.\nComputed tomography at an outside hospital showed numerous low-density cysts and calcifications scattered throughout both sides of his brain (Figure A). The boundary of each cyst was clear with a high-density ring, sometimes calcified. Brain magnetic resonance imaging demonstrated extensive cerebral white matter leukoencephalopathy. Numerous cysts of various sizes were scattered throughout his hemispheres, thalamus, basal ganglia and left ventricle (Figure B-F). The boundaries of the cysts were hyperintense on both T1- and T2-weighted images and gave a low signal on the fluid-attenuated inversion recovery image. Enhancement was observed in the lining of the cyst wall. The cystic content was heterointense in the T1-weighted and fluid-attenuated inversion recovery images.\nA physical examination showed that his vision was 0.15 in his left eye and 0.5 in his right eye. He had grade-4 strength in his right limbs and a positive Babinski sign on his right. The suspected diagnosis included parasitic infection, glioma and leukoencephalopathy. To make a correct diagnosis, we performed a large excisional biopsy of the left frontal cyst, including the adjacent brain tissue. Analysis of the cyst fluid did not suggest malignancy or infection (parasitic in particular). The biopsy specimen of his white matter and cyst wall revealed a pronounced reactive gliosis with conspicuous formation of Rosenthal fibers. In addition, focal hemosiderin deposits, which indicate previous hemorrhage, and microcalcifications were observed. Many ectatic vessels and angiomatous changes with cellulose-like degeneration were observed (Figure ). A neuro-ophthalmologic examination was performed, but no evidence of Coats retinopathy was found (Figure A, B). A visual field examination showed irregular visual field defects of both eyes (Figure C). Visual-evoked potentials disclosed a mild prolongation of P100 latency of his left eye and an increased amplitude of both eyes (Figure D). The results of serological and immunological tests were within the normal range. We diagnosed our patient with LCC. |
The patient is an otherwise healthy, 18-year-old male with a body mass index of 37 kg/m2, who presented with chronic bilateral axillary, bilateral thigh, and posterior neck HS lesions. He had failed medical treatment including injectable tumor necrosis factor antibody therapy and presented for wide radical excision of his axillary lesion with a plan for later skin grafting by plastic surgery.\nHe was previously treated with adalimumab (Humira), a tumor necrosis factor antagonist, without resolution. He was brought to the operating room for excision of his right axillary lesions. General anesthesia was induced with 4 mg midazolam, 150 μg fentanyl, 80 mg lidocaine, 200 mg propofol, and 50 mg rocuronium. An 8.0 mm endotracheal tube was inserted through the vocal cords. Anesthesia was maintained using oxygen, air, and sevoflurane. Additional 400 mg propofol, 20 μg dexmedetomidine, and 100 μg fentanyl were administered in total during the surgery. Patient received 1 g intravenous acetaminophen, 30 mg ketorolac, and 1 mg hydromorphone for pain control. Four milligrams of ondansetron and 4 mg of dexamethasone were administered, and the patient was extubated successfully after reversal of paralytic with 4 mg of neostigmine and 0.8 mg of glycopyrrolate. Surgery involved excision of skin and subcutaneous tissue of the axilla and placement of a negative pressure wound vacuum.\nThe surgery team requested the regional anesthesia team to perform a block to control postoperative pain, as axillary resection of the soft tissue was extensive. Prior to placement of ESB, patient was awakened from general anesthesia and he recovered in the post-anesthesia care unit (PACU). He was evaluated by the regional anesthesia team 37 minutes after his arrival to the PACU, at which time he was fully awake and oriented to person, place, and time, and his self-reported pain score was 8 on the numeric rating scale.\nWe performed an ESB in the PACU, with the patient lying in left lateral decubitus position. The patient’s back was palpated for landmarks; the seventh cervical spinous process (C7) was identified and marked, and used to count caudally to thoracic vertebra 2 (T2). The T2 spinous process was identified and marked. A high-frequency linear transducer (HFL50; 15–6 MHz) was placed on the back, slightly to the right of midline in a coronal plane. The transducer was translated medially until the transverse processes (TPs) were identified, and then cephalad to identify the T2 TP, lateral to the marked T2 spinous process. Skin was then sterilized with ChloraPrep™ skin prep solution (2% w/v chlorhexidine gluconate in 70% v/v isopropyl alcohol; BD, Franklin Lakes, NJ, USA), and a 20-gauge, blunt-tipped, 100 mm echogenic block needle (B Braun, Bethlehem, PA, USA) was advanced in-plane from caudal to cranial to make osseous contact with the T2 TP. At this point, a small volume of saline was injected to assess for satisfactory spread of fluid underneath the erector spinae muscle (ESM) and above the TP. A volume of 30 mL of 0.25% bupivacaine hydrochloride (75 mg) with 3 mg of preservative-free dexamethasone was then injected under direct ultrasound visualization (Sonosite X-Porte; SonoSite, Bothell, WA, USA). Craniocaudal spread was noted below the ESM ().\nFollowing block placement, he had a marked decrease in axillary pain, from the aforementioned pain score of 8 to 0 on the numeric rating scale, 25 minutes after completion of the ESB. He did not require any narcotics or adjuncts for pain in the recovery room. He was maintained on scheduled acetaminophen 1g every 6 hours, celecoxib 200mg every 12 hours, and gabapentin 300mg every 8 hours. Tramadol 100mg was given every 6 hours as needed for breakthrough pain. Neurological assessment to pin prick was performed, and diminished sensation was noted on the right side over the shoulder, scapula, axilla, and anterolateral chest wall ( and ). Handgrip, flexion and extension at wrist and elbow, and abduction and adduction at the shoulder were intact.\nThe patient underwent two more debridements of the axilla and placement of negative pressure wound vacuum at days 3 and 6 after his initial surgery under general anesthesia.\nHe requested the regional team perform the same block, as he was satisfied with the analgesia. ESB was performed in a similar fashion as described above with similar analgesic efficacy. Average time to first narcotic consumption after each T2 ESB was 39.3 hours.\nThis patient was receiving chemical venous thromboembolic (VTE) prophylaxis, beginning after his first surgery. He was receiving scheduled enoxaparin 40 mg once daily at the time of the second and third ESB. |
Patient B was a 37-year old male of Turkish descent referred for immunological investigation by the treating nephrologists because of IgM deficiency. Asymptomatic renal insufficiency was detected at the age of 28 years when a cirrhosis of the left kidney and mild hydronephrosis of the right kidney were found. Serum creatinine was 3.2 mg/dl (normal range 0.6–1.2 mg/dl), proteinuria was 2.5 g/d. He reported no increased susceptibility to infections, and his chronic renal insufficiency caused only mild clinical symptoms (development of fatigue and tachycardia upon physical strain).\nThe mRNAs encoding the membrane-bound and secreted immunoglobulin heavy chains are produced from identical primary transcripts, which are differently processed at their 3′ ends. Regulation of membrane-bound vs. secreted forms of the immunoglobulin heavy chains depends on the competition of 2 mutual cleavage polyadenylation sites (pAs/pAm) (). In mice targeted deletion of the mu heavy chain cleavage polyadenylation site pAs leads to deficiency of secreted IgM with intact expression of surface IgM and normal secretion of other immunoglobulin isotypes (). Therefore, we sequenced mu heavy chain gene including the polyadenylation sites in both patients with sIgMD and found no alterations (data not shown). Both patients' B cells were able to express precursor, secreted and membrane IgM mRNA (Figure ). Furthermore protein expression of monomeric and native pentameric IgM (Figure ) and surface expression of IgM on the B cell membrane (data not shown) was comparable to healthy controls.\nTo elucidate the genetic basis of the patients' selective IgM deficiency we used a targeted resequencing approach to sequence potential candidate genes. In both patients, we identified defects within the intrinsic B-cell receptor signaling pathway. Patient A harbored a c615G > T missense mutation in exon 8 in the tyrosine kinase BTK. The G > T transition resulted in a glutamic acid to aspartic acid substitution at position 205 within the highly conserved proline-rich (PRR) region located at the C-terminus of the TEC homology (TH) domain (Figures ). Proline rich regions are involved in protein-protein interactions, including interactions with G proteins and intramolecular association with the SH3 domain (). Mutations within the proline rich regions have been shown to abolish SH3 domain binding and result in functional impairment of BTK, pointing toward a potential biologic relevance of the BTK mutation found in patient A (). |
A 12-year-old female reported to the maxillofacial surgery clinic with the complaint of a painless mass in the left buccal area of the maxilla. Furthermore, the patient complained of absence of the maxillary canine and first premolar tooth on the same side since 2 years.\nOn extraoral examination, there was a swelling on the left side of the face causing obvious asymmetry with obliteration of nasolabial sulcus. Intraorally, there was an expansion in all dimensions measuring about 5 cm × 5 cm in anteroposterior and superior inferior direction. On palpation, the swelling was hard, nonlobulated; nontender, not fixed to the overlying skin and local temperature was not raised. The growth had expanded the palatal, alveolar and buccal aspect to such an extent that the overlying mucosa had indentation marks from the lower teeth causing discomfort to the patient during speech and mastication [].\nThe orthopantomography (OPG) revealed [] a radiolucent lesion with irregular borders located between the apices of the left maxillary lateral incisor and the left maxillary second premolar. The canine was displaced toward the apices of the incisors, and the first premolar tooth was present within the lesion. The coronal computed tomography (CT) of the maxilla revealed a well-circumscribed radiolucent mass with calcific bodies which were multiple and interspersed throughout the lesion with prominent sclerotic margins. Maxillary left canine was impacted without being involved in the lesion. The axial CT confirmed that the left maxillary sinus and the nasal cavity were not encroached. Maxillary left first premolar was present within the lesion [].\nUnder premedication (i.e. injection atropine, 0.6 mg IM, 60 min before surgery and injection diazepam 5–10 mg IM) and local anesthesia, an adequate window was created and the tumor mass was enucleated along with the enclosed 24 tooth [Figures and ]. The mass was partially solid with partial cystic degeneration, and a gritty sensation could be elicited on examination. The remaining cavity was found to be clean without any tissue tags hence chemical or mechanical curettage was not done. The wound was sutured with 3–0 silk with an open packing. The packs were changed after weekly intervals for 6 weeks and the patient was instructed to keep the area clean.\nThe histopathological picture [] of the biopsy sample revealed epithelium and underlying connective tissue stoma. The tumor cells were seen arranged in various patterns firstly, duct-like structures of varying sizes which were lined by cuboidal cells with a lumen lined by an eosinophilic rim of varying thickness. Secondly, rosettes like structures lined by tall columnar cells with hyperchromatic polarized nuclei were seen []. The connective tissue also showed the presence of multiple calcified structures along with amorphous eosinophilic material at a few places. The histopathologic diagnosis confirmed the lesion to be a follicular AOT.\nDuring the 1-year follow-up period both clinically and radiographically, we observed a gradual reduction in the size of the deformity and adequate healing of the defect. The displaced maxillary left canine also moved on the eruption path and axis []. |
A 50-year-old Japanese woman fell from a height of approximately 10 m and was brought to our hospital by an ambulance. She had no remarkable medical and family history, was a social drinker, and a non-smoker of tobacco. Her marital status was stable with her husband. Her consciousness levels were 14 points (E4, V4, M6) according to the Glasgow Coma Scale at initial arrival. She complained of respiratory discomfort and low back pain, and was in an unrestful state. Her respiratory rate was 24 breaths/minute, blood oxygen saturation (SpO2) was 95% under 10 L/minute oxygenation, subcutaneous emphysema was recognized in the left side of her chest, and her breathing sounded weak. A hemoperitoneum was not detected on focused assessment with ultrasonography for trauma. Her pulse rate was 90 beats per minute (bpm) and her blood pressure was 180/120 mmHg as measured by an automatic blood pressure monitor. However, her capillary refill time was 3 seconds and radial artery pulsation was feeble with cold sweat in her extremities; hence, she was recognized as being in a state of shock. Her face swelled, and bleeding from her nasal and the oral cavities continued. A subcutaneous hematoma was found in her lumbar and her left femoral region. Her left elbow was deformed and swollen. Because she was in a restless state, accurate neurological assessments were difficult, but coarse paralysis of limbs was not observed.\nFirst, a 28-French chest drain was inserted in her left thoracic cavity for the diagnosis of tension pneumothorax. Hemothorax was not recognized. A pelvis X-ray showed unstable pelvic fracture and a contrast-enhanced computed tomography (CT) pan scan was additionally performed. In the contrast CT, retroperitoneal bleeding with the extravasation of contrast media was recognized (Fig. ). Other injuries and laboratory data on initial arrival are shown in Tables and respectively.\nThe injury severity score in this case was 66 and the probability of survival was calculated as 59.3%. Multiple rib fractures were seen, even though active bleeding from intercostal artery injury and pulmonary laceration were not noted at the initial visit. She had deteriorated during CT (blood pressure 67/42 mmHg, pulse rate 75 bpm), and blood transfusion was therefore started and intubation was performed. Transcatheter arterial embolization was performed for hemorrhagic shock with the pelvic fracture as the main bleeding source. While starting angiography, she did not respond to the massive blood transfusion and her systolic blood pressure was maintained at around 60 mmHg. Therefore, first, a 7-French aortic occlusion catheter (RESCUE BALLOON®, Tokai Medical Products, Aichi, Japan) was inserted from her left femoral artery. Her hemodynamics had improved and her systolic blood pressure was 90 mmHg due to REBOA (30 mL saline inflation into the balloon) at the first lumbar vertebra level. Next, transcatheter arterial embolization was started using the sheath inserted in her right femoral artery. Her bilateral internal iliac arteries were embolized from the origin portion with a gelatin sponge. Furthermore, we embolized the extravasation of contrast media of her middle sacral artery and lumbar artery with n-butyl-2-cyanoacrylate. Her blood pressure had been monitored by continuous invasive arterial measurement from her upper right brachial artery during interventional radiology. The systolic blood pressure under REBOA was intended to be controlled under 100 mmHg (so called permissive hypotension); however, it was actually in the range of 90 to 160 mmHg. After 18 minutes of occlusion, the balloon was deflated and the aortic occlusion catheter was removed. However, she was still hemodynamically unstable. The internal iliac artery angiography was performed again, and the site that had just been embolized, as described above, presented recanalization. Since coagulopathy was recognized as a complication, we interpreted that it would be difficult to restrain the hemorrhage with the gelatin sponge. Therefore, it was re-embolized from the origin portion of her bilateral internal iliac arteries by n-butyl-2-cyanoacrylate (Fig. ). After embolization, retroperitoneal gauze packing and pelvic external skeletal fixation were additionally performed. Further, a repeat CT pan scan was performed, and a massive hemothorax had appeared in her left pleural cavity, which had not been captured at the first arrival (Fig. ). As the inserted chest drain at the first arrival was detained between lobes, the amount of bleeding in the pleural cavity had not been reflected precisely. Thoracotomy hemostasis was performed and a hemothorax of approximately 2500 ml was aspirated to search for the source of the bleeding. However, clear active bleeding was not captured and only extremely minute bleeding from the rib fracture site, chest wall, and pulmonary contusion area was detected. Therefore, special hemostasis treatment was not needed. After a massive hematoma was removed, a chest drain was placed again and her chest was closed. Continuous blood transfusion was not needed before or after the operation and her postoperative hemodynamics became stable. The timeline of the initial treatment in our emergency department is shown in Table .\nThe retroperitoneal packing gauze was removed on day 3. On day 24, the chest drain was removed without increasing the hemothorax. Complications accompanied by internal iliac artery occlusion, such as gluteal muscle necrosis, did not appear. She regained the ability to walk, and was moved to another hospital on day 154 for rehabilitation. She was discharged from the rehabilitation hospital 6 months after the injury, and her activities of daily living were almost independent. |
An 8 days old male neonate was born to an Asian mother through vaginal delivery at 37 weeks of gestation, weighed 2,380 g, and had APGAR scores of 9 and 10 at 1 and 5 min, respectively. He was admitted to our hospital with a 2 days history of fever of up to 39°C but did not have respiratory or gastrointestinal symptoms. The infant's family denied any medical history and TB contact. His physical examination at admission documented smooth respiration, clear breathing sound, and no hepatosplenomegaly. The complete blood count indicated a total white blood cell count of 17,500/μL with 69% neutrophils, 20% lymphocytes, 9% monocytes, and 2% eosinophils. The C-reactive protein level was 7.3 mg/dL. The findings of the cerebrospinal fluid (CSF) analysis were normal. Bacterial cultures of the blood, urine, and CSF were negative. Intravenous antibiotics, namely cefotaxime and ampicillin, were administered after admission on the basis of suspicion of neonatal fever. Despite the administration of the antimicrobial combination therapy, the fever persisted and the neonate developed abdominal distension when he was 12 days old. Abdominal radiography exhibited nonspecific dilated bowel loops. Because no improvement in the condition of the patient was observed after changing antibiotics, infection caused by some virus and other atypical pathogen, including Mycobacterium tuberculosis, was considered. Tests for herpes simplex virus, Epstein–Barr virus, cytomegalovirus, hepatitis B virus, rubella, Chlamydia trachomatis, and Toxoplasma gondii were all negative. The repeat C-reactive protein level was elevated to 14.4 mg/dL. Coagulopathy with 323.7 μg/mL of abnormal fibrin degradation product and more than 20 mg/L of D-dimer were also noted. Antibiotics were switched to vancomycin and ceftazidime empirically. Chest radiography displayed only increased right lung field infiltration when the infant was 12 days old (), and chest computed tomography (CT) imaging exhibited a large amount of right pleural effusion with mild inflammatory changes in the right lower lobe when the infant was 15 days old (). Pleural effusion drainage was suggested but refused by his parents at that time. Gastric lavages for acid-fast staining and culture were examined when the infant was 20 days old after his parents agreed to further testing, and one of the three acid-fast stains of gastric lavages yielded few acid-fast bacilli. Repeat chest and abdomen CT imaging performed when the infant was 24 days old indicated patchy consolidation in the right upper lung, multiple new nodules in both the lungs, moderate pleural effusion, and multiple low-density nodules in the spleen and hepatic hilar region without hepatomegaly (). Subsequently, pigtail catheter insertion for pleural effusion drainage was performed. The findings of pleural fluid analysis indicated a total white blood cell count of 10,800/μL with 6% neutrophils, 57% lymphocytes, and 37% mesothelial cells; a total protein level of 4.6 g/dL, a lactic dehydrogenase level of 250 IU/L, and a glucose level of 164 mg/dL. TB infection was strongly suspected. The neonate was administered isoniazid (15 mg/kg/day), rifampicin (15 mg/kg/day), and pyrazinamide (20 mg/kg/day) when he was 24 days old. After initiating anti-TB treatment, the neonate's symptoms and signs subsided gradually. Finally, both gastric lavage and pleural effusion cultures showed M. tuberculosis complex.\nThe neonate's mother was 33 years old, gravida 1, para 1. Her Group B streptococcus test was negative. She had been healthy with no previous medical history and TB contact history; however, she developed a mild dry cough 1 week after delivery, experienced persistent general weakness, and was admitted to our medical intensive care unit because of altered mental status 24 days postpartum. Laboratory examinations indicated leukocytosis, thrombocytopenia, coagulopathy, acute hepatic failure, and acute renal failure. The HIV serology test was negative. A chest X-ray exhibited a miliary TB pattern (). A chest CT image displayed diffuse interlobular and intralobular septal thickening with ground-glass opacities (). Because her neonate was highly suspected to have TB infection at that time, acid-fast staining and TB polymerase chain reaction (PCR) of the sputum were performed. Both tests were strongly positive. The mother was administered anti-TB therapy immediately, but she died 3 days after hospitalization. M. tuberculosis infection was confirmed through sputum culture. |
A 24-year-old female patient was initially referred to our vascular department for investigation of bilateral lymphedema. Her echocardiogram showed mildly impaired LV with evidence of segmental wall motion abnormality and hypertrabeculation in the apex suggestive of noncompaction. Further questioning revealed a history of sudden death in two of her brothers. One of the deaths was related to an unknown cardiomyopathy. The history of the patient started at birth when she had difficulty swallowing and several delayed milestones such as walking and talking at the age of 5. Due to socioeconomic issues, the parents did not seek medical assistance. As the patient matured, there was an additional history of palpitations, which were occasionally associated with syncopal attacks. The patient had generalized, but progressive weakness throughout the years. Her weakness increased with activity and decreased with a rest. The weakness was mainly proximal, worse in the upper limbs. Due to constant fatigability, she dropped out of school.\nHer vital signs were normal on physical examination, she demonstrated normal heart sounds. Lower limbs bilateral nonpitting edema up to the knees, proximal bilateral upper and lower limbs weakness with intact sensation, waddling gait, and easy fatigability with few steps were noted.\nComputed tomography coronary angiogram was performed to exclude coronary artery disease. Results showed segmental wall motion abnormality and revealed normal coronaries, LV dilatation, and lateral wall and apical noncompaction with the noncompacted myocardium to compacted myocardium ratio in the range of 2.5–3.5 []. CMRI was done for further evaluation of LV function and volume. Her CMRI showed mildly dilated LV with mild global hypokinesis and a marked decrease in longitudinal shortening. End-diastolic volume was 120 mL, end-systolic volume was 61 mL and the ejection fraction was 50%. In addition, multiple areas of noncompacted myocardium, particularly in the apex and most of the lateral wall extending from apical lateral wall to the mid-to-basal segment were seen. The ratio of maximum thickness of the noncompacted myocardium to compacted myocardium was more than 2.5 in multiple areas []. Mildly dilated left atrium and mild mitral regurgitation were seen. Right ventricle (RV) has a prominent trabeculation, but normal systolic function.\nFurther investigation showed elevated creatine kinase and electromyography findings consistent with myopathy. Left deltoid biopsy showed advanced dystrophic changes of undetermined type. Brain magnetic resonance imaging (MRI) was negative.\nThus, the clinical impression was proximal myopathy mostly mitochondrial or congenital, congenital lymphedema, LV noncompaction with LV ejection fraction of 40%, and normal RV function. As her LV impairment is mild, the decision was made to keep her on beta-blockers and on angiotensin-converting enzyme inhibitor in the form of metoprolol and lisinopril, respectively. On subsequent follow-up visits, she reported a history of interval improvement apart of occasional palpitations with no history of shortness of breath, syncope or dizziness. She has been advised to do regular physiotherapy and to wear compressive stockings for her lymphedema. |
A 42-year-old man presented with the complaint of painful swelling in the right lower limb for 5 months. Also, he noted a serous discharge from the scar of the previous surgery, over the last 5 months. His medical history included an internal fixation with a metal rod implant for femoral fracture treatment (). Roughly two months after the initial symptoms, the implant was removed (). The computed tomography (CT) detected a huge right proximal thigh lesion as well as multiple soft tissue lesions. These soft tissue lesions included the subcutaneous tissue of the face and abdominal wall, left perinephric and posterior pararenal space, left gluteal region, right second rib, and the right iliac bone. The Magnetic resonance imaging (MRI) scan of the right thigh showed an ill-defined, lobulated, expansile, intramedullary lytic lesion, which involved the metaphyseal and diaphyseal region of the femur. It also showed thickening and multi-focal breach of the cortex with associated extraosseous soft tissue component involving the skeletal muscle tissue. The positron emission tomography (PET) showed increased FDG avid uptake in the right thigh, nodal and extranodal tissue, intramuscular and subcutaneous region, renal, bowel, skeletal, omental, and peritoneal region, indicating the possibility of a disseminated malignancy ().\nA biopsy of the femoral lytic region was performed, and the patient underwent a prophylactic internal fixation of the right femur with nails.\nThe biopsy’s histology revealed large, atypical cells in solid sheets with a round to oval irregular nuclei and nuclear indentations (). The chromatin was coarse to vesicular with prominent nucleoli and moderate basophilic to eosinophilic cytoplasm. Amidst these large cells, the characteristic hallmark cells with kidney-shaped nuclei were also present. Mitotic activity was brisk. These neoplastic cells were invading and replacing the surrounding bone trabeculae, indicated by dead bony fragments, collection of giant osteoclast cells, and reactive bone formation. Besides, the skeletal muscle and fibro-adipose tissue involvement hinted at the extraosseous presence. On immunohistochemistry (- and , ), the Leukocyte common antigen (LCA) showed membranous positivity of variable intensity, and the CD20 immunostain was negative. CD3 showed weak membranous positivity, and CD30 showed diffuse, strong, and uniform immunoreactivity in the large cells in the membrane and Golgi zone. ALK immunostain revealed diffuse nuclear as well as cytoplasmic positivity. MUM1 depicted strong, diffuse, nuclear immunostaining. CD2, CD4, CD5, CD7, CD8, CD138, c-MYC, P63 and pan-cytokeratin were negative. In-situ hybridization for Epstein Barr virus was negative. On histopathology, a diagnosis of anaplastic large cell lymphoma, ALK-positive, was entertained. Taken together with the clinical presentation, imaging details and histopathological findings, this case seems more to be systemic ALCL with widespread dissemination with secondary involvement of the metal rod implant.\nThe patient received one cycle of chemotherapy in the form of CHOEP (Cyclophosphamide, Doxorubicin, Vincristine, Etoposide, and Prednisone) regime following the diagnosis. Unfortunately, he died as a result of therapy-related complications. |
A 60-year-old woman underwent transurethral resection of bladder tumor (TURBT) at our institute in 2004; her pathological diagnosis was a high-grade UC with adenocarcinomatous differentiation (pT2a, G2>G3). Radical cystectomy was conducted. Only carcinoma in situ (CIS) was found in the surgical specimen, and the surgical margin was negative. There was no cancer cell infiltration in the resected uterus or anterior wall of the vagina, and no lymph node involvement was detected. The patient developed continuous pain and bleeding from the residual vagina in 2010, and a tumor was found in the residual vagina; magnetic resonance imaging (MRI) showed it to be located on the anterior wall (). A biopsy of the tumor revealed a pathological diagnosis of adenocarcinoma (). Computed tomography (CT) and bone scintigraphy revealed no metastasis. Based on a preoperative diagnosis of a primary adenocarcinoma occurring on the residual vagina, tumor resection was performed (). The sigmoid colon was partially resected as it was strongly adherent to the tumor. On pathological examination, adenocarcinoma and SCC were detected (); on immunohistochemistry, sections of the tumor were positive for the SCC markers CD56, chromogranin A, and synaptophysin and were negative for the urothelial carcinoma markers GATA-3, p63, uroplakin, thrombomodulin, and 34βE12. We then reexamined the original TURBT specimen and confirmed the presence of SCC (). Adenocarcinoma and SCC were mostly present in the superficial layer of the TURBT specimen, while high-grade UC was found in the deeper layers where muscle invasion was present. Based on these findings, the tumor was diagnosed as a recurring bladder tumor. Local recurrence and pelvic bone metastasis were detected via MRI 3 months after the patient underwent surgical resection of the vaginal recurrence, whereupon she underwent radiation therapy (52 Gy, 26 fractions). She developed ileus in January 2011 and underwent release surgery. Subsequently, multiple lung metastases and local recurrence in the pelvis developed in June, and she died of disease progression the following month. |
The patient is a 15-year-old female whose medical history did not reveal any previous incidence of severe illness. Six days prior to admission, the patient had experienced high fever (highest temperature of 39.5°C) and mild signs of upper respiratory infection. At the time of admission, she was experiencing episodes of unconsciousness.\nThe patient manifested altered mental status and seizures. She was unable to response to sound and had lost control of her eye reflexes. Moreover, the patient's limbs twitched accompanied with trismus and she experienced nausea and vomiting, but not urinary incontinence. The earlier mentioned symptoms lasted for 1 to 3 minutes and disappeared spontaneously and reoccurred in episodes. A computed tomography scan, which showed symmetrical brain lesions in the cerebellum and thalami, was performed on the patient at the local hospital before being admitted to the department of our hospital.\nThe blood pressure of the patient was 115/75 mm Hg and she displayed a positive left Kerning sign. Other physical examination results were unremarkable. Hematologic data for the patient are presented in Table . Analysis of the cerebrospinal fluid (CSF) following lumbar puncture found a clear, colorless fluid with an opening pressure of 310 cmH2O. Other results are summarized in Table .\nBrain MRI revealed multiple symmetrical brain lesions. Prolonged T1- and T2-relaxation time signals were evident in the right regions of the frontal and parietal lobes, bilateral dorsal areas of the thalami, dorsal caps of the pontine, bilateral cerebellar hemispheres, and the cauda cerebelli. High signal intensities and slightly low central signals were demonstrated on diffusion-weighted imaging (DWI). After enhancement, the lesions of the bilateral dorsal thalamus, bilateral cerebellar hemisphere, and right parietal lobe exhibited low-level circular enhancement (Fig. ).\nBased on the characteristic MRI findings, clinical presentations, the history of upper respiratory tract infection, and the lack of other prominent etiologies, a diagnosis of virus-associated ANE was made. In addition to maintaining sufficient hydration, oseltamivir (150 mg twice daily for 7 days), methylprednisolone (450 mg once daily for 5 days), and acyclovir (500 mg thrice daily for 10 days) were administered to the patient intravenously. Magnesium isoglycyrrhizinate, mannitol, and other supporting treatments were also given to the patient as needed. On the 7th day of hospitalization, the patient regained consciousness and was responsive to various cues. MRI scans at this timepoint revealed that the size of lesions in the bilateral dorsal thalamus were slightly reduced, the internal signals were obviously uneven and surrounded by short T1 signals (Fig. ). The dose of oseltamivir was then reduced to 75 mg for a further 7 days, and methylprednisolone was gradually reduced to 80 mg daily. Prednisone acetate (1.5 mg/kg/d for 3 months, dose reduced week by week) was then given to the patient in decreasing doses until completely withdrawal of the medication. This treatment strategy led to a near complete recovery from ANE as confirmed by MRI findings 15 days after diagnosis of the disease (Fig. ). The size of lesions in the bilateral dorsal regions of the thalami, dorsal caps of pontine, and bilateral cerebellar hemispheres were significantly reduced and surrounded by short T2 signals.\nThe GE Signa Excite 1.5T superconducting MRI system and orthogonal coils were used in this study. The patient underwent both plain and enhanced MRI scanning. The scanning parameters employed include a layer thickness of 6 mm, interval of 2 mm, and field of view of 18.0 × 18.0 cm. Others are horizontal spin-echo T1-weighted imaging (SET1WI) (TR: repetition time 300–500 milliseconds, TE: echo time 8–12 milliseconds), fast spin echo T2-weighted imaging (FSET2WI) (TR: 2500–5000 milliseconds, TE: 90–102 milliseconds), fluid-attenuated inversion recovery (FLAIR) (TR: 8002 milliseconds, TE: 104 milliseconds) and diffusion-weighted echo-planar imaging (TR: 7100 milliseconds, TE: 129 milliseconds). Additional parameters include sagittal SET1WI (TR: 300–500 milliseconds, TE: 8–12 milliseconds), and partial coronal scanning FSET2WI (TR: 2500–5000 milliseconds, TE: 90–102 milliseconds). For the enhanced MRI scanning, the contrast agent gadolinium-diethylenetriamine pentaacetic acid 0.1 mL/kg was administered intravenously. The transverse, sagittal, and coronal positions were SETWI (TR: 300–500 milliseconds, TE: 8–12 milliseconds). |
A 55-year-old non-diabetic, non-hypertensive male with a history of recurrent colicky left lumbar pain presented with acute urinary retention. Catheterization was attempted; however, it was unsuccessful. Ultrasound revealed an overdistended urinary bladder with a normal-sized prostate and scarring and focal caliectasis in the left kidney (). The right kidney was normal and no calculi were seen on either side on ultrasound. Serum electrolyte, renal and liver functions were normal. The haemogram revealed neutrophilia. The prostate-specific antigen was within normal limits. A rigid urethroscopy was performed owing to suspicion of a left urethral calculus and a 11-mm size calculus was removed from the posterior urethra. The patient was discharged and had no difficulty with micturition for 2 weeks thereafter. The patient subsequently developed burning micturition with hesitancy and induration in the perineal region. The urine was turbid and microscopy revealed the presence of Escherichia coli. A perineal ultrasound revealed an abscess in the perineum, which extended to the proximal parts of the corpus spongiosum (). This abscess was drained under saddle block. The patient subsequently developed a discharging sinus at the operative site () leaking purulent fluid. A retrograde urography revealed periurethral extravasation of the injected contrast material with a lytic lesion in the left pubic ramus (). A repeat perineal ultrasound revealed a linear hypoechoic tract leading from the skin surface to the corpus spongiosum (). A retrograde CT urography was performed to look for the extent and ramifications of the abscess. On the non-contrast CT scan, a lytic lesion was seen involving the left inferior pubic ramus (). The pubic symphysis and bodies of both pubic bones were normal. A proximal femoral nail was noted in situ on the left side, which was inserted 10 years before for fracture of the proximal shaft of the femur owing to accidental trauma. On injecting iodinated contrast into the urethra, there was extravasation of the contrast in the periurethral region in the soft tissues surrounding the bulbar and the posterior penile urethra. An extension of the contrast through the external anal sphincter into the intersphincteric plane () with inflammatory stranding in the ischioanal fossae was seen. The contrast also extended into the lytic lesion present in the left inferior pubic ramus (). The contrast also extravasated through the cutaneous opening in the perineum and the natal cleft (). E. coli were isolated on pus culture. The patient was treated with intravenous antibiotics and suprapubic cystostomy was performed. The patient is presently being considered for elective urethroplasty. |
A 54-year-old woman with poorly controlled diabetes (Glycated hemoglobin [HbA1C] of 10.5%) and grade 4 chronic kidney disease (creatinine of 3.2 mg/dL) presented to the clinic with a history of productive cough and dyspnoea of grade 4 Modified Medical Research Council scale for a period of one week. She had a history of high-grade squamous intraepithelial lesion on her pap smear a year back for which she has not consulted further. Her physical examination revealed tachypnoea and grossly reduced breath sounds on the left side. Her blood gas analysis on admission showed respiratory alkalosis with partial pressure of Oxygen (PaO2) of 64.7 mm Hg and saturation of 94% in room air. Her chest X-ray revealed a complete homogenous opacity on the left side (Fig. a). High-resolution computed tomography of the thorax showed a complete collapse consolidation of the left lung with abrupt left main bronchial cut off and a mild left pleural effusion. Her cardiac status was normal and the ultrasonogram of the abdomen was normal except for a simple renal cortical cyst in the right kidney. The pleural fluid analysis revealed a sterile effusion, negative for malignancy. Her sputum culture grew methicillin-resistant Staphylococcus aureus for which she was treated with linezolid. Bronchoscopy revealed a fleshy vascular growth completely occluding the left main bronchus (Fig. b). Bronchial carcinoid or a bronchogenic carcinoma was suspected. The biopsy was sent for histopathological examination that showed bronchial mucosa and fragments of necrotic tissue with many broad aseptate hyphae invading the stroma and the vessel wall occluding the vascular lumen suggestive for invasive mucormycosis (Fig. ). Bronchial lavage cytology also revealed aseptate hyphae and was negative for any malignant cells. Histopathology confirmed mucormycosis and so a fungal culture was not performed. The patient and family opted for medical management considering the high risks associated with the surgery. We treated her with oral posaconazole based on her renal functions. She died due to worsening renal failure. |
A 70-year-old man with hypertension was transferred to our center after a traffic accident. He was in drowsy mental state with a Glasgow Coma Scale of 14. The patient was showing symptoms of chest pain and shortness of breath. Respiratory rate of 21 breaths per minute with an oxygen saturation of 97%, a regular pulse of 101 beats per minute, and blood pressure of 101/61 mmHg was recorded at the moment.\nComputed tomography (CT) after the accident showed a small amount of hemopericardium, but no other vital organ injuries (). Based on these findings, BCI was strongly suspected. Transthoracic echocardiography (TTE) was performed by an experienced cardiologist, which revealed a small pericardial effusion, but the site of injury could not be determined.\nUnder general anesthesia, exploratory median sternotomy was performed, and initial vital signs in the operating room were stable. The hematoma in the pericardium and over the RV was removed. In an attempt to visualize the LA and the apex of the left ventricle (LV), sudden massive arterial bleeding occurred. Subsequently, cardiac arrest ensued. While manual compression to the heart was being performed, CPB was established immediately with ascending aortic perfusion and two-staged right atrial venous drainage. CPB circuit priming and cannulation took approximately 20 minutes and open cardiac massage was continued. After achieving cardioplegic arrest, optimal visualization of the operative field was achieved.\nCareful inspection of the heart revealed a 2-cm laceration of the left upper PV at its junction with the left atrium and the tear was repaired using a Peri-Guard Repair-Patch (Lamed GmbH, Berlin, Germany) and reinforced with 6-0 prolene (). Thorough inspection was performed again under CPB and no further injury was found. However, CPB weaning was difficult due to akinesia of the left anterior descending artery territory. We performed emergency coronary artery bypass surgery and subsequently weaned the patient off CPB. However, he remained in a semi-coma state after cardiac exploration and suffered low cardiac output syndrome (LCOS). Patient’s condition worsened and the patient was pronounced dead on postoperative day (POD) 2. Total aortic cross-clamp and CPB times were 112 and 208 minutes, respectively. |
A 66-year-old Japanese man who had no past medical or medication history complained of gross hematuria and visited a nearby hospital in October 2013. He had no habit of drinking alcohol or smoking tobacco. He was diagnosed as having a right renal tumor and underwent right nephrectomy laparoscopically. The pathological diagnosis was right renal cell carcinoma (RCC), clear cell carcinoma, pT1bN0M0, v1 (Fig. ). One and half years later, lymph node swelling was detected at hepatic portal region and he underwent lymphadenectomy. The pathological diagnosis was a metastasis from RCC. Two years after diagnosis, he was suspected of lung metastases and started treatment with interferon α. Three years later, the multiple lung metastases grew larger and were determined as progression despite interferon α therapy. At this point, he was referred to our hospital in October 2016. There were no abnormalities on physical examination and his vital signs were normal. He started treatment with sunitinib 50 mg/day on a schedule of 4 weeks on treatment and 2 weeks off; however, adverse events including grade 3 thrombocytopenia (platelet count, 49,000/μL), gum swelling, and hoarseness became intolerable 2 weeks after starting sunitinib. Four weeks after cessation of sunitinib 50 mg/day, he was started on a dose of sunitinib 25 mg/day on a schedule of 2 weeks on and 1 week off. Computed tomography (CT) findings in January 2017 revealed that his lung metastases had shrunk; however, he continued to experience the same adverse events. Therefore, the dose of sunitinib was further reduced to 12.5 mg/day on a schedule of 2 weeks on and 1 week off. CT findings in May 2017 revealed new metastases in the pleura, diaphragm, and the right paracolic gutter (Fig. a, b). As a result, the treatment was changed from sunitinib to axitinib and he started treatment with axitinib at 10 mg/day; however, adverse events including gum swelling, dysphonia, hypertension, diarrhea, and thrombocytopenia became intolerable (Fig. ). Two weeks after cessation of the drug, the dose of axitinib was gradually reduced from 6 mg/day to 4 mg/day. CT findings in September 2017 revealed the metastases had diminished in size and lung metastases were maintained at a diminished size (Fig. c, d); however, the adverse events could not be controlled and he discontinued axitinib treatment. His adverse events improved after discontinuation of axitinib; however, CT findings in December 2017 revealed the size of metastases had increased again (Fig. e, f). Consequently, he was started on fourth-line therapy with nivolumab (3 mg/kg every 2 weeks) and did not experience any adverse events. However, after he had received eight cycles of nivolumab, his metastatic lesions had grown, peritoneal dissemination appeared in his pelvic region, and pleural effusion appeared (Fig. g, h), so nivolumab was discontinued. After giving a detailed explanation of treatment options to our patient, he decided to rechallenge with axitinib 4 mg/day. However, adverse events including gum swelling and dysphonia became intolerable. After that, the dose of axitinib was reduced to 2 mg/day, and he experienced relief of adverse symptoms except for hoarseness. CT findings in August 2018 revealed metastases in lungs, pleura, diaphragm, and the right paracolic gutter had diminished in size (Fig. i, j). He has been continuously receiving a low dose of axitinib at 2 mg/day for 10 months with metastases maintained at reduced size. |
A 60-year-old male judge with hypertension and diabetes describes a single episode of monocular transient visual loss OD. A dark curtain descended from superior to inferior OD, lasted 10 minutes and then spontaneously resolved. The patient was seen by an outside ophthalmologist and a diagnosis of “retinal migraine” was made. The next day the patient suffers two more episodes of similar visual loss and calls the ophthalmologist back and is given a referral for a two week appointment to neurology for “migraine”. That night the patient suffers a hemispheric stroke with hemiparesis and aphasia. A carotid study shows near occlusion of the right internal carotid artery.\nThe diagnosis of “retinal migraine” is one of exclusion. In elderly patients the diagnosis should be made with caution in the absence of headache history, prior attacks without residual or sequelae, and in patients who have vasculopathic risk factors. The International Headache Society (IHS) criteria for the diagnosis include: “≥ 2 attacks of “fully reversible” monocular visual loss with migraine headache”. The presumptive vasospasm mechanism for migraine headache and visual aura has fallen out of favor and spreading depression of cortical neuronal activity is a more plausible explanation for the visual symptoms in migraine. Unfortunately there is no diagnostic test for the migraine aura and a convincing history is required for the diagnosis. The role of the ophthalmologist is to exclude permanent visual loss after the event by performing formal visual field, documenting that there is no permanent residual loss, and ruling out alternative etiologies or eye markers for ischemic disease (e.g., central or branch retinal artery occlusion, Hollenhorst plaque). The most characteristic historical features for migraine are positive (e.g., scintillation or fortification scotoma) rather than negative (e.g., a black curtain) visual phenomenon; march and build up across the visual field in a nonvascular distribution, stereotyped quality with repeated events without permanent sequelae; and preferably the headache follows the aura after a few minutes duration. The most worrisome historical features for ischemia as the cause for amaurosis fugax include older aged, vasculopathic patient with first onset of an altitudinal (e.g., curtain over my vision) onset and disappearance, duration of minutes (1-10”), and seconds in onset. I would consider ordering a work up in transient monocular visual loss for patients with altitudinal history (e.g., curtain of visual loss), visible embolus, residual visual field defect, vasculopathic risk factors, and an ischemic time course. I would expedite the evaluation if the course was one of an increasing crescendo of events. On the other hand, I often defer work up for visual loss symptoms which have been present for years (e.g., hundreds of events with no residual loss) or have longer duration (e.g., 1-2 hours to days); are non-altitudinal events, the patient has no vasculopathic risk factors, or gives only a vague history of blurring. The clinician should also try to elicit a history suggestive of fluctuating rather than transient visual loss that might suggest the more common benign etiologies like ocular surface disorders or dry eyes. I will give these latter patients a pinhole to try during the events and if it alleviates the problem then it can be safely assumed to be ocular surface or refractive in nature. I also ask the patients to keep a diary of their events to establish some of the historical details above and I often give an empiric artificial tear trial. |
A 47-year-old woman presented to our clinic and complained of a left upper eyelid lesion that had increased slowly in size over the past three years. The lesion was 1.0 cm in size. It was round shaped, circumscribed elevated and had brown pigmented color (Figure 1 ). The appearance was typical of a seborrheic keratosis. Her visual acuity and eyelid movements were normal.\nThe procedure is performed under local anesthesia with intravenous sedation and magnification. Upper eyelid tumor is marked with 2 mm margin. A line is drawn on the eyelid at the level of the lid crease. Then, the advancement flap of the anterior lamella is outlined with two Burow’s triangles marked for excision, one triangle medial or lateral to the defect and the second diagonal to the first, above the lid crease (Figure 2 ). An incision is then made through the skin and the subcutaneous tissue of the lesion. The lesion was excised with a 2 mm free margin. The triangles’ boundaries are cut with a scalpel, dissected, and mobilized with blunt scissors (Figure 3A ). The subcutaneous tissue at the edges of the defect is undermined in the subdermal plane to minimize the tension at the suture lines. An advancement flap of the skin and orbicularis of the upper eyelid was undermined, elevated, and advanced inferiorly over the defect (Figure 3B ). Interrupted buried 6/0 nylon sutures are used to approximate the dermis and subcutaneous tissue and close the defect completely (Figure 4 ). Topical antibiotic ointment is applied twice daily for 7 days. The sutures are removed in 10 days. Histopathological examination of the tumor revealed seborrheic keratosis and confirmed that the margin was free of tumor. The patient has been followed up for six months with no evidence of recurrence and has no concerns with eyelid function. Moreover, this treatment produces good aesthetic results (Figure 5 ) and increased patient satisfaction. |
Our patient is a 54-year-old female with a past medical history of hypothyroidism and very severe obesity (BMI 48 kg/m2). She underwent laparoscopic gastric sleeve surgery in the year 2012. Results were non-satisfactory in terms of weight loss with a difference of 6 kg/m2 in BMI post-procedure. So after six years, she underwent a laparoscopic biliopancreatic diversion with a duodenal switch. She had an uneventful postoperative recovery period. An upper gastrointestinal (GI) study contrast post-procedure did not reveal any evidence of obstruction or leak. The patient was discharged home two days after the procedure. A few days later, she started experiencing three episodes of nausea with brown-colored vomitus. She was found to be septic, with a heart rate of 110 beats per minute and temperature of 100.2oF. Her white blood cells count was 12/mm3.The source of infection was presumed to be intraabdominal considering her symptoms. Computed tomography (CT) of the abdomen and pelvis showed mildly dilated proximal small bowel loops. The patient was started on empiric antibiotic therapy with ceftriaxone 1 gm intravenous (IV) daily and metronidazole 500 mg IV every eight hours. Symptoms did not improve, so she was taken back to the operating room for diagnostic laparoscopy. Partial small bowel obstruction was noted along with ischemia of a segment of the ileum that was part of the duodenoileostomy due to mesenteric dissection. She underwent an open revision of the small bowel anastomosis with resection and anastomosis for the obstruction revision of the duodenoileostomy. Her hospital stay post-surgery remained uneventful. Diet was advanced gradually throughout the hospital course and a week later, the patient was discharged home with outpatient follow-up. Three weeks after that procedure, she noticed a productive cough with thick, yellow, foul-smelling phlegm and shortness of breath. She saw her primary care physician. A chest X-ray performed showed a right lung infiltrate with a right-sided pleural effusion. She was started on treatment with augmentin 500 mg/125 mg every eight hours. Her symptoms became worse so she came to the emergency room. Her vitals showed blood pressure 129/79 mmHg, heart rate 86 beats per minute, respiratory rate 20 breaths per minute, and temperature 98.6oF. Pulse oxygen saturation was 97% on room air. Mild leukocytosis was evident (white blood cells count 11.4/mm3 with no bands or left shift). A chest CT showed loculated, right-sided hydropneumothorax with almost total collapse of the right lung (Figure ).\nThere was a fistulous connection evident, extending from the surgical anastomosis in the stomach/bowel in the right upper quadrant through the right hemidiaphragm to the right hemithorax. These CT scan findings were new as compared to a CT scan obtained for this patient six months prior to the duodenal switch when she presented to the emergency department for non-specific left-sided chest pain. To analyze the anatomy of the fistula further, an upper gastrointestinal fluoroscopic contrast study was performed that showed a large fistula from the distal stomach prior to the duodenal bulb opening to the right pleural cavity (Figure ).\nConsultations from gastroenterology and cardiothoracic surgery teams were obtained. Chest tube drains were placed with the plan of eventually performing a video-assisted thoracoscopic surgical decortication. Post-procedure CT showed patent chest tubes draining the right pleural cavity. The drained fluid was exudative in nature as per Light’s criteria (fluid lactate dehydrogenase > 12,000 u/L and total protein ratio = 0.7) and culture from the right lung empyema grew Escherichia coli, Klebsiella pneumoniae, Pseudomonas aeruginosa, and Candida albicans. An infectious disease consultation was placed at this time. The patient was started on levofloxacin 750 mg IV daily for two weeks as per the sensitivity result obtained for Escherichia coli, Klebsiella pneumoniae, and Pseudomonas aeruginosa. Micafungin 100 mg IV daily was started for the infection with Candida albicans. This was later switched to Diflucan 400 mg IV daily for a total of two weeks. Repeat cultures from the draining fluid were negative toward the end of the second week.\nFor treatment of the fistula, the patient was transferred to another facility for esophagogastroduodenoscopy (EGD) and possible clipping of the fistula due to the unavailability of that particular service in our hospital. As a result, there was a delay of 16 days from admission to the treatment of the fistula. When the EGD was performed, it showed that there was no anastomotic leak from the previous surgery. No evidence of any stricture was identified at the previous anastomosis. A small fistulous tract was noted in the distal part of the antrum likely secondary to ulcer formation that was noted in very close proximation to the fistula tract. An endoscopic clipping was performed. A post-procedure contrast study performed on the same day revealed complete closure of the fistulous opening. The patient was transferred back to our facility after the procedure. An upper GI contrast study performed two days later showed residual leakage from the distal stomach to the right upper quadrant. A repeat EGD was performed along with fulguration of a fistulous opening with argon beam coagulation and repeat orthoscopic clip application with complete obliteration of the fistula tract. This was confirmed by an upper gastrointestinal contrast study showing no persistent fistulous communication between the post-bulbar duodenum and pleural space (Figure ).\nAfter confirmation of fistula obliteration, a video-assisted thoracoscopic surgery was performed followed by the washout of the right pleural space with the placement of a right-sided chest tube. The patient was discharged home after the procedure and has been followed by multidisciplinary teams on an outpatient basis. |
A 51-year-old female with a history of rheumatoid arthritis and a 10.5-pack-year smoking history presented with an aspergilloma in her right lung. After failing medical management, she was treated with a right pneumonectomy at an outside institution. This was complicated by BPF and empyema of the pneumonectomy cavity. She underwent two additional thoracotomies requiring rib resection, and placement of serratus anterior and later latissimus dorsi flap to close the fistula. Seven months following her last operation, she presented to us with stridor, persistent cough, and dysphagia, concerning for postpneumonectomy syndrome. Review of last computed tomography (CT) imaging from three months after the pneumonectomy revealed a multiloculated pleural space, with air fluid levels in the pneumonectomy cavity. An updated CT scan showed interval progressive rightward mediastinal shift with nearly complete obliteration of the pneumonectomy cavity by the heart (Fig. ). A bronchoscopy was performed, which demonstrated narrowing of the left mainstem bronchus (Fig. a) and stenosis of the lower lobe bronchial orifice due to external compression of the airways. Results of a previous complex right-sided BPF with two areas of disrupted bronchial staple line were noted (Fig. b).\nThe patient elected to proceed with operative correction of her postpneumonectomy syndrome. A thoracotomy in the fifth intercostal space was performed and dense adhesions in the chest with rotation of mediastinal structures were faced. Upon entering the pleural space, a small loculated serous fluid collection was encountered. To rule out an infected field, the pleural rind and fluid samples were sent for intraoperative gram stains, which returned negative. Cultures were also submitted. The mediastinum was mobilized from the chest wall, taking care to avoid damage to the muscle flaps, which had previously sealed the BPF. As a result, only the mid and inferior portion of the mediastinum was mobilized. A saline immersion test was performed to ensure the integrity of the muscle flap seal over the right mainstem bronchus. The implants were sized based on measuring the amount of saline instilled in the chest, and close hemodynamic monitoring of arterial and central venous pressures. Before placing the implants, the thoracotomy was closed temporarily after placement of implant sizers, monitoring hemodynamics to ensure there was no right heart compression. Two implants (250 mL and 100 mL) were placed into the pleural cavity, and the thoracotomy was closed. The postoperative recovery was uneventful. The patient was discharged on post-operative day 5. She noted complete resolution of her stridor, cough, as well as dysphagia four weeks post-operatively. Her post-operative chest radiograph showed partial medialization of the inferior mediastinum with persistent rightward deviation of the proximal trachea (Fig. ). At time preparation of this manuscript, the patient continues to have full resolution of symptoms at fourteen months following surgery. |
A 50 year-old male, with a history of coronary arterial bypass grafting 14 years back, presented with shortness of breath and dry cough. An X-ray revealed a large mass in the left hemithorax adjacent to the heart silhouette. A chest CT demonstrated the presence of a mass with smooth edges, in middle mediastinum next to the heart and partially intrapericardial (Fig. ). The mass was of heterogeneous density and of 11 cm size. Presence of atelectasis at the left lower lobe abating the mass was clearly seen. Based on clinical and radiologic evidence, we did proceed with CT guided FNA of the mass. The cytology findings revealed inflammatory lesion. Laboratory tests were normal. Based on patient symptoms, history and the presence of a mass potentially compressing the cardiopulmonary structures in vicinity, we decided to offer exploratory surgery for diagnosis and treatment.\nStandard hemodynamic monitoring and general anesthesia were followed by positioning, prepping and draping patient in left lateral decubitus position. An anterolateral left thoracotomy was carried out and entrance in the hemithorax was made without any challenge. The mass was assessed and found to be leaning medially on the surface of the lateral wall of the left ventricle, including the pericardial layer and had smooth edges which didn’t infiltrate the lung (Fig. ). We started dissecting the mass from its smooth capsule, making it through all its layers. An old and degraded piece of surgical swap was visualized (Fig. ). The surgical swap was removed along with the capsular layer of this mass. Patient tolerated the procedure very well and blood loss was minimal. A chest tube was inserted in the left hemithorax and chest wall was closed following standard procedures.\nIn the immediate post-operative phase, patient improved steadily and on day four was discharged home symptom-free. In the long-term follow -up, patient was found to remain without symptoms. |
A 69-year-old woman was admitted to hospital due to sternotomy site pain that had exacerbated 2 days prior. She had a 15-year history of hypertension and diabetes mellitus. The patient had undergone coronary artery bypass graft (CABG) surgery for triple vessel disease 2 months prior to presentation. She was discharged 9 days after the CABG surgery without complication. One week prior to the admission for the worsening sternotomy site pain, the patient presented in the outpatient clinic due to CABG operation site pain, but the pain was not relieved by opioids. Two days prior to admission, she developed redness, tenderness, and pus on the operation site, as well as wound dehiscence. At the time of admission, the patient was afebrile and complained of operation site pain with a slightly elevated C-reactive protein (CRP) level of 1.74 mg/dL. After obtaining specimens for ordinary bacterial, fungal, and mycobacterial culture, empirical therapy with vancomycin and cefotetan was initiated. The Gram stain showed many white blood cells, few epithelial cells, and only rare Gram-positive rods. Chest computed tomography on the second day of admission demonstrated cellulitis and focal osteomyelitis around the sternotomy site (), as well as focal fibrosis and linear atelectasis in the right-upper lobe, which were thought to be inflammatory sequelae. Her CRP level was normalized to 0.62 mg/dL on the sixth hospital day. On the eighth hospital day, wound closure was performed for wound dehiscence.\nThe wound culture on blood agar showed small, orange colored convex colonies without aerial hyphae after 5 days of incubation (). The organism was catalase-positive, oxidase-negative, and urease-positive gram-positive rod. It was preliminarily identified as an actinomycete, and as the treating physician believed the organism to be an anaerobic actinomycete, the antibiotics were changed to penicillin G for empirical therapy. The isolate was identified as a Rhodococcus species by a commercial identification system (API CORYNE, bioMérieux SA, Mercy l'Etoile, France) with the API code 1111004 (98% identification percentage), but the CAMP test was negative, demonstrating that the equi factor was not produced, and the Gram stain at 4 hours and 24 hours did not match the Rhodococcus species. The organism was identified as G. bronchialis DSM 43247T with a log score value of 1.808 by the MALDI-TOF mass spectrometry (Bruker Daltonics Inc., Billerica, MA, USA). A secondary match was indicated for Mycobacterium chlorophnolicum DSM 43826T with a log score value of 1.341. 16S rRNA gene sequencing of the organism was performed for further identification. PCR was performed according to previously published methods [,,]. The purified PCR product was identified as G. bronchialis by 16S rRNA gene sequencing analysis, with 100% identity (1,324/1,324 bp) for G. bronchialis DSM 43247T (GenBank accession number CP001802). For secondary matches, the sequence of the isolate exhibited 98.6% (1,305/1,323) and 98.6% (1,304/1,322) identity for Gordonia rubripertincta DSM 43197T (GenBank accession number X80632) and Gordonia rhizosphera IFO 16247T (GenBank accession number AB023368), respectively (). In addition, the hsp65 sequence of the organism showed 99.8% (421/422) homology with the corresponding sequence of G. bronchialis strain DSM 43247T and 93.1% homology with that of Gordonia species KTR9 (GenBank accession number CP002907) []. In line with the Clinical and Laboratory Standards Institute (CLSI) guideline [] the organism was identified as G. bronchialis. The patient was subsequently transitioned to imipenem administration. In follow-up wound culture on the 11th day, G. bronchialis was also grown after 5 days of incubation by pure culture. On the 18th day of admission, the patient had surgical wound debridement and curettage. A surgical specimen was drawn for modified acid-fast stain and Gram stain, which were negative, and a final wound culture was also negative.\nAn antibiotic susceptibility test was performed using the broth microdilution method, with a Sensititre RAPMYCO (Trek Diagnostic Systems, Part of Thermo Fisher Scientific, Oakwood Village, OH, USA) panel and interpreted using CLSI guideline M24-A2 []. The organism was susceptible to all antibiotics tested with minimum inhibitory concentrations (MICs) of trimethoprim/sulfamethoxazole ≤ 0.25/4.75 µg/mL, ciprofloxacin ≤ 0.12 µg/mL, moxifloxacin ≤ 0.25 µg/mL, amikacin ≤ 2 µg/mL, doxycycline ≤ 0.5 µg/mL, clarithromycin ≤ 2 µg/mL, linezolid ≤ 1 µg/mL, imipenem ≤ 2 µg/mL, cefepime ≤ 1 µg/mL, amoxicillin/clavulanate ≤ 2/1 µg/mL, ceftriaxone ≤ 4 µg/mL, minocycline ≤ 1 µg/mL, cefoxitin ≤ 8 µg/mL, tigecycline ≤ 0.5 µg/mL, and tobramycin ≤ 1 µg/mL.\nFor a few days before and then after undergoing CABG, the patient stayed in her daughter's house, where a dog was kept as a pet. We cultured specimens from the dog to determine the source of the organism, but these were all negative for G. bronchialis. After an 8 week course of intravenous imipenem, the wound healed completely and the patient was discharged symptom free. |
A 42-year-old man with history of HIV, on highly active antiretroviral therapy, and 7.5 pack-years of smoking, initially presented for perianal mass excision. The perianal lesion was verrucous, circumferential, and extensive throughout the anorectal perineum, causing high clinical suspicion for carcinoma. The mass was excised and pathology diagnosis confirmed that the specimen was positive for squamous cell carcinoma. HPV molecular testing was not performed at that time.\nSubsequently, two months after excision, the patient presented to the emergency room with severe perianal pain accompanied by serosanguinous to purulent discharge. Physical exam revealed ulceration along the prior excision site and a prominent right inguinal lymph node that had enlarged over the preceding weeks. In addition, computerized tomography (CT) of the pelvis suggested multiple areas of deep fluid collection and abscess formation. The patient was admitted to the hospital for intravenous antibiotic treatment and monitoring of infection in the setting of AIDS (absolute T cell count 239/mm3 with 8% T helper cells; viral load 116 copies per mL). He was also diagnosed with active hepatitis B during this admission (viral load >501,000,000 IU/ mL). Fine needle aspiration of the enlarged right inguinal lymph node demonstrated malignant cells, consistent with metastatic squamous cell carcinoma. The patient began treatment with chemotherapy (mitomycin/capecitabine) and radiation therapy and was discharged home with a plan to continue outpatient therapy.\nSix months after the excision procedure, the patient returned to the emergency room with shortness of breath and was found to have new deep vein thrombosis on Doppler imaging. CT angiography of the chest ruled out pulmonary embolism but instead revealed eleven pulmonary nodules ranging from 3mm to 12mm in greatest dimension, scattered throughout both lungs and raising clinical concern for distant metastasis (A,C,E). The patient eventually agreed to interventional radiology-guided biopsy. Multiple nodules were noted to have increased in size over a one-month time interval (B,D,F), including the largest nodule, which had increased to 24mm from 6mm; this nodule was biopsied and diagnosed as squamous cell carcinoma. Over the next few weeks, the patient was noted to have new thrombi occluding the inferior vena cava, involving the right common iliac vein, and manifesting as a subsegmental pulmonary embolism. Ultimately, one month after lung biopsy, the patient passed away due to massive thrombosis within the heart. The family did not consent to autopsy. |
A 2.5-year-old boy presented to the authors’ clinic with his parents in July 2005, with a 3-month history of a firm lump and bowing deformity on his right forearm. The parents reported that the appearance of the boy’s right forearm was asymmetrical compared with the left one, and the deformity had gradually progressed during the past 3 months. No notable pain was noted. The boy had a normal physical development and denied any traumatic and medical history. The physical examination revealed the middle forearm was tumid and bowing on the ulnar and dorsal side. The patient had full range of motion on his shoulders, elbows, wrists and fingers except for a limited rotation motion on the right forearm which was 80 degrees of supination and 45 degrees of pronation. There was no tenderness to palpation over the right ulna, and the neurovascular examination was normal. The child appeared in good health on other systems.\nRadiographs of his right forearm showed a large expansile lucent lesion in the diaphysis of the ulna, extending to the proximal and distal metaphysis (Figure A). The lesion presented as fusiform-shaped and typical “ground-glass” appearance with a size of 5.7 cm in length and 1.9 cm in maximal width. The computed tomography (CT) scan indicated it had a well defined circumscription and no calcification and bone septums were seen. The cortex showed marked expansion and thinning without periosteal reaction. The density in the area of lesion was homogeneous and the CT value was an average 123 Hu, which was similar to the density of the cancellous bone, but without the visible trabecula of bone (Figure B,C). No distinct soft tissue mass was exhibited on the radiographic or CT images. The laboratory tests, including complete blood cell count, thyroid function, liver and renal functions, were within normal limits.\nBased on the clinical and typical image presentations, a diagnosis of monostotic fibrous dysplasia was established without performing an incisional biopsy. In view of the deformity progressing and limited rotation motion on the right forearm, an en bloc resection of the lesion and autogenous fibular graft was carried out. At surgery, the right ulna was exposed subperiosteally, and the lesion was excised en bloc with preservation of the proximal and distal healthy metaphysis. Then the bone defect was reconstructed using the free autogenous fibula of the ipsilateral lower leg and the intramedullary fixation was achieved with a 2.0 mm Kirschner wire. A postoperative cast was employed to immobilize the elbow and forearm in a neutral position. The excised gross sample revealed the cortical bone was thin and firm except for a small perforation on the upper pole. The medullary cavity of the ulna was replaced by a solid grey white expansile mass. The histological examination confirmed the diagnosis of fibrous dysplasia (Figure ).\nSix weeks after surgery, the patient had a well grafted union and the pin and cast were removed (Figure A). No early complications were seen. At 3-month follow up, he had regained the full range of rotation movement on his right forearm. However, 4 years postoperatively, the patient returned to the authors’ clinic for a reappeared deformity on his right forearm. A subsequent radiograph was taken and demonstrated a long, expansile lytic lesion in the middle and proximal ulna surrounded by an irregular thin bone shell, indicating recurrence of the tumor (Figure B). The lesion was enlarged and extended proximally to the coronal process level. The bowing deformity in the ulna, as well as in the radius was more severe than the initial presentations. On being informed of the natural history of fibrous dysplasia, the parents refused further treatment and determined to observe. At the last follow-up, 8 years postoperatively, the lesion and the deformity in the ulna had been further progressive, but there was no evidence of malignant transformation (Figure C). |
An 82-year-old woman with past medical history of hypertension and hyperlipidemia, presented with a right cheek nodule in December 2015. Biopsy result was consistent with ulcerated malignant melanoma, nodular type, Breslow depth of at least 2.7 mm. The nodule was locally resected, pathology results were consistent with extensive residual malignant melanoma, Breslow depth of 6.05 mm with multiple microsatellite metastases present, angiolymphatic invasion was identified and one sentinel lymph node was negative for melanoma (0/1). Melanoma in situ extended to the superior margin and medial and lateral specimen tips, therefore procedure was followed with Mohs surgery which showed no residual disease. Six months later, patient presented with two new nodules on her right cheek below the area of her prior excision. At that time, she was also noted to have right submandibular lymphadenopathy. Excisional biopsy of the nodule and FNA of submandibular lymph node showed recurrent malignant melanoma. Staging CT chest/abdomen/pelvis and brain MRI showed no evidence of distant metastatic disease. Multidisciplinary teams agreed on deferring surgery and proceeding with systemic therapy. Patient was subsequently started on anti-PD-1 therapy with Pembrolizumab at a fixed dose of 200 mg every 3 weeks. Patient continued to show a positive response until fourteen months of therapy when restaging CT scans demonstrated a left upper lobe nodule concerning for metastatic disease. PET scan demonstrated a high SUV uptake of 15, also consistent with metastatic disease. A left lung needle biopsy was subsequently completed which was consistent with metastatic malignant melanoma. Mutational analysis revealed double KIT mutations at V559 and N822I, therefore, Imatinib; a tyrosine kinase inhibitor known as Gleevec, was started at a dose of 400 mg daily. This was given in combination with Pembrolizumab at 200 mg every three weeks. Shortly after, patient developed a pruritic rash on her back and shoulders in addition to Grade 2 transaminitis. Imatinib was held for approximately 3 weeks until her symptoms and transaminitis resolved. No further intervention was needed. She then restarted Imatinib at half dose of 200 mg daily. One month later, patient developed another episode of grade 2 transaminitis. Imatinib was held for approximately three weeks and once her LFTs normalized, Imatinib was restarted at 400 mg 3 times a week (Monday/Wednesday/Friday). Restaging scans completed after 3 months of combination therapy showed significant decrease in size of lung nodule. In 6 months, restaging scans showed complete remission with no radiographical evidence of metastatic disease. Patient continued on combination therapy of Pembrolizumab and Imatinib, with no evidence of disease for almost 12 months. However, she ultimately developed recurrent disease with brain metastases after a total of 2 years on combination therapy. She was eventually transitioned to hospice care. Patient's health care proxy has provided informed consent for publication of the case |
A 24-year-old unmarried woman was admitted to the Department of Neurology, The Second Hospital of Hebei Medical University, Shijiazhuang, Hebei Province, China because of severe unresponsiveness and twitching in October 2019. Subsequent computed tomography of the skull showed low-density shadows of the left temporal lobe and bilateral frontal lobe. Magnetic resonance imaging (MRI) of the skull and intracranial blood vessels showed direct enhancement of the cranial veins and brain. Diffusion imaging of the brain showed multiple filling defects, abnormal enhancement and diffuse signals at the distal end and branch of the superior sagittal sinus, the left transverse sinus-sigmoid sinus-internal jugular vein and the right transverse sinus. The patient’s prothrombin time was 13.4 s, prothrombin activity was 77% and D-dimer was 0.88 μg/ml. The patient was considered to have CVST with brain parenchymal damage (cerebral oedema and venous infarction). Therefore, the patient underwent whole cerebral angiography via femoral artery catheterization and catheterization thrombolysis. After the operation, the patient was given comprehensive treatments, such as thrombolysis, sedation and lowering of intracranial pressure.\nThe patient’s bone marrow aspiration result was verified to be normal. To identify the cause of CVST in such a young woman, a thorough medical history was taken and the patient reported a history of increased menstrual flow for 6 months, increased vaginal discharge and a peculiar smell for 1 month. Prior routine examination of leucorrhoea in the local hospital showed inflammatory changes. After taking oral anti-inflammatory drugs for 1 month, the condition did not improve. Then, the patient was given an urgent examination with uterine accessory ultrasound to prompt the following question: is this condition uterine disease? MRI of the pelvic cavity demonstrated an enlarged cervix with an irregular shape. The neurologist asked the gynaecologist for two consultations in total. The first physical examination found that there was redness and swelling at the vaginal opening and that the tumour was limited to the vaginal opening, and then a biopsy was performed. Five days later, the second physical examination showed that there was a mass of approximately 9 cm in diameter in the vagina, covered with pus and stained with blood, and the pedicle could not be touched (). After the patient's neurological symptoms improved, she was transferred to the Department of Gynaecology, The Second Hospital of Hebei Medical University, Shijiazhuang, Hebei Province, China for gynaecological treatment in November 2019.\nAfter being transferred to the Department of Gynaecology, a gynaecologist (L.M.) performed two physical examinations and found that the tumour was progressively prolapsed. The diameter had increased from 11 cm to 14 cm, of which the pedicle could not be touched (). The patient's cancer antigen (CA) 125 level was 36.4 U/ml. The pathological examination of the vaginal mass confirmed a tumour. Therefore, the patient was given laparoscopic total hysterectomy and bilateral salpingo-oophorectomy. During the operation, it was found that the patient's cervical tumour was approximately 15 × 15 × 15 cm, touching the thick pedicle, which was approximately 2.5 cm in diameter. What was confusing for the physicians was that the patient's uterus was found to be completely inverted and adhered to the left ovary and the front wall of the rectum, but there was no mass in the uterus. The two fallopian tubes were stuck together and the appearance of both ovaries was normal (). As there was no evidence of extrauterine spread of the tumour at the time of surgery, the tumour was staged as clinical group I based on the Intergroup Rhabdomyosarcoma Study (IRS)-I. Finally, postoperative histology confirmed embryonic cervical rhabdomyosarcoma with a tumour size of 18 × 16 × 7 cm, which invaded the whole layer of the cervical wall, and a tumour thrombus was seen in the vessel (). Immunohistochemistry revealed the following: CD10 (++), CD117 (–), CD34 (–), CD68 (+), CKpan (−), desmin (++), DOG1 (–), Ki-67 (90%+), LCA (–), lysozyme (–), MyoD1 (–), myogenin (+), S-100 (–), SMA (–) and vimentin (+). However, the patient declined further chemotherapy and radiotherapy, and then she was discharged.\nTwo months later in January 2020, the patient quickly showed signs of relapse. A physical examination revealed a mass of approximately 8 × 8 × 5 cm in the vaginal stump that was crumbly and bled when touched (). Based on the IRS treatment scheme, adjuvant chemotherapy with vincristine, actinomycin-D and cytoxan was administered (2 mg vincristine on day 1 + 0.4 mg actinomycin-D on days 2–6 + 0.4 g cytoxan on days 2–6; intravenous infusion). As the patient’s condition progressed rapidly, the vaginal mass progressively increased to 13.53 × 9.92 × 10.74 cm in size () and there were lung metastases and para-aortic lymph node metastases during the second chemotherapy session. The patient refused subsequent chemotherapy and was discharged. Two months later, the patient died of complications. The reporting of this study conforms to CARE guidelines.Written informed consent was obtained from the patient to publish this case report and the patient was reassured that all of her information would be deidentified. |
A 38-year-old male patient who was a smoker (5 pack-years) and had a history of unknown arrhythmia without medical treatment was admitted to the emergency room due to right hemiplegia. During the initial examination, the patient was stuporous with a Glasgow Coma Scale score of 7. He was diagnosed with infarction of the left middle cerebral artery (MCA) territory () with occlusion of the M1 segment of the left MCA (). He had atrial fibrillation, heart failure with a left ventricular ejection fraction (LVEF) of 27% (), pulmonary edema, pleural effusion, and mild cardiomegaly (). He was admitted to the intensive care unit of the Department of Cardiology. He received a tracheostomy, mechanical ventilation, and additional medical treatment. He showed improvement in the LVEF from 27% to 47% on echocardiography and normal sinus rhythm on electrocardiogram (ECG) after treatment.\nOn day 37 after admission, the patient was transferred to the Department of Rehabilitation Medicine, where he was provided with stroke rehabilitation and CR. The Mini-Mental State Evaluation score denoting the cognitive function was 30. In the manual muscle test, the right upper extremity was graded as good in the proximal portion and fair in the distal portion. The right lower extremity was graded as good. The functional ambulation category (FAC) score was 4 and the Berg balance scale (BBS) score was 53. The modified Barthel index (MBI) score was 77. Speech evaluation showed anomic aphasia with an aphasia quotient (AQ) of 79. On day 38 after admission, the nasogastric tube was removed, and oral feeding was started after the videofluoroscopic swallowing study. The tracheostomy tube was also removed on day 43 after confirming that the patient had sufficient strength for coughing and sputum expectoration. He received physical therapy, occupational therapy, and speech therapy for stroke rehabilitation.\nThe first symptom-limited exercise tolerance test (ETT) was conducted on day 45 after admission (). After 14 minutes and 48 seconds, the ETT was terminated upon patient’s request due to leg discomfort. Using the Fitness Registry and the Importance of Exercise National Database (FRIEND) equation, the predicted VO2 max, which reflected age and weight, was 42.15 mL/kg/min []. The measured VO2 max was 21.7 mL/kg/min (51.5% of predicted value), and there was no abnormality in exercise ECG and hemodynamic response. The patient participated in a CR program for 2 weeks (1-hour sessions five times per week) (). An ECG-monitored exercise training with 4.6 metabolic equivalents (METs) was started, and the intensity was gradually increased. After 2 weeks, a follow-up ETT was performed, and the test was stopped after 15 minutes and 53 seconds upon patient’s request. The VO2 max improved from 21.7 to 27.3 mL/kg/min (from 51.5% to 64.8% of the predicted value) (). The patient also received an educational program about risk factor management, including smoking cessation and nutrition. Upon discharge (day 60 after admission), the BBS score changed from 53 to 56 (smaller than the minimal clinically important difference [MCID] of 12.5) [], MBI score from 77 to 98 (larger than MCID of 20.1) [], and AQ from 79 to 88.2.\nThe patient underwent a home-based CR program three times a week after discharge from the hospital for 8 weeks. After 8 weeks, a follow-up ETT was performed (). The test was stopped after 18 minutes and 30 seconds upon patient’s request, and the VO2 max showed further improvement (31.3 mL/kg/min, 74.3% of predicted value). Based on the guidelines of the American Association of Cardiovascular and Pulmonary Rehabilitation (AACVPR), risk stratification of the patient was changed from moderate risk at the first test to low risk at the follow-up ETT []. |
A 14-year-old boy was admitted to our emergency department (ED) in status epilepticus. Four weeks previously, he was seen at a neurology department because of generalized convulsive status epilepticus (GCSE), myoclonus, and hallucinations. The seizure frequency was about once 10 days, and the onset duration varied from 20 minutes to 60 minutes. He was hospitalized, but no seizures occurred during the hospitalization. Magnetic resonance imaging (MRI) of the brain revealed no obvious abnormalities. Electroencephalogram (EEG) monitoring for 24 hours indicated no epilepsy discharge waves. He was discharged on sodium valproate for seizure control. Twenty-four hours before admission to our ED, he was again seen at the other hospital for GCSE. He had experienced 4 episodes, with an onset duration that varied from 10 minutes to 20 minutes. Sodium valproate, carbamazepine, and diazepam were administrated; however, the status epilepticus was not suspended. He was thus transferred to our ED. This study was approved by the institutional review board (IRB) of the First People's Hospital of Lianyungang City. Written informed consent was obtained from the patient. Written informed consent was obtained from the patient's father for the publication of any potentially identifiable images or data included in this article.\nOn examination in our ED, he was alert and his general examination was without abnormalities. Neurological evaluation only showed a bilateral Babinski sign, and he appeared to have normal cognitive development. He appeared to be of normal weight and height for his age, and his nutritional status appeared sufficient. He had no history of recent infections or flu-like symptoms, injury, toxicosis, or academic achievement decline. He had a history of syncope several years prior, but the details of the event could not be obtained.\nRoutine laboratory tests, including a complete blood count (CBC), erythrocyte sedimentation rate, platelet count, tests of kidney (creatinine, urea nitrogen) and liver function (alanine aminotransferase, aspartate aminotransferase, albumin, direct bilirubin, indirect bilirubin, total bilirubin), and protein, lactate, and ammonia levels were normal. Urine toxicology was unremarkable. However, his glucose was slightly below the lower limit of normal (3.76 mmol/L, reference range: 3.9-6.1 mmol/L). Testing for specific antibodies associated with autoimmune encephalitis including N-methyl-D-aspartate (NMDA) receptor antibody, voltage-gated potassium channel (VGKC) antibody, anti-leucine-rich glioma-inactivated (LGI1) antibody, anti-contactin-associated protein-like 2 (Caspr2) antibody, anti-Hu antibody, and anti-CRMP5/CV2 antibody was negative.\nThyroid function tests revealed a low thyroid-stimulating hormone (TSH) level (0.06 mIU/L, reference range: 0.34-5.60 mIU/L), but normal free triiodothyronine (FT3, 4.5 pmol/L, normal range: 3.8-6.47) and free thyroxin (FT4, 1.36 pmol / L, normal range: 7.9-17) levels. However, serum anti-thyroid antibody levels were elevated. His anti-TPO antibody level was 76.7 mIU/L (reference range: 0.25-34 mIU/L), and his anti-TG antibody level was 237.1 mIU/L (reference range: 0-4 mIU/L).\nCerebral computed tomography (CT) showed no signs of increased intracranial pressure, hemorrhage, or a space-occupying lesion. MRI, including T1, T2, and diffusion-weighted imaging (DWI) with apparent diffusion coefficient (ADC) mapping, and fluid-attenuated inversion recovery (FLAIR) sequences, and contrast-enhanced angiography of the brain also revealed no abnormalities, especially no signs of encephalitis. EEG showed a marked slowing of the background rhythm, suggestive of encephalopathy, and spike waves corresponding with seizure activity (Figure ).\nBased on new-onset seizures, an EEG suggestive of encephalopathy, elevated anti-TPO and anti-TG antibody levels, and otherwise extensive and negative workup, a diagnosis of HE was considered.\nThe patient was started methylprednisolone, 500 mg for 5 days, which was then gradually switched to oral prednisone, 60 mg/day during which time his psychiatric symptoms such as hallucinations and agitation were resolved by haloperidol and olanzapine, respectively. Diazepam, midazolam, sodium valproate, chloral hydrate, and phenobarbital were ineffective at controlling his seizures. His seizures were finally controlled with a combination of sodium valproate 2,400 mg/day, clonazepam 4 mg/day, topiramate 350 mg/day, and levetiracetam 1,800 mg/day.\nAt his 18-month follow-up, the patient has remained seizure-free and in good physical condition. |
An 11-year-old Sudanese boy presented with frequent bleeding diathesis (ecchymosis, epistaxis, and gingival bleeds) (Fig. ). The most commonly encountered type of hemorrhage was extensive bleeding after trauma followed by subcutaneous bleeding. The patient had a history of delayed bleeding episodes since the day of circumcision, which took approximately 1 month to stop. Repeated episodes of epistaxis were also reported. He did not receive blood or blood product transfusion. He reported bleeding in the subcutaneous tissue following trauma when he was playing with his friends. Hemorrhage into joints also occurred after trauma. Spontaneous joint bleeding was not reported. Pain and slight swelling of muscles sometimes persisted for months. Looking into the family history, all family members were normal except his mother. The mother had a history of severe interval bleeding diathesis (menorrhagia). The patient had no known chronic sickness, drug allergies, or any developmental abnormality.\nOn examination, the patient was neither icteric nor cyanosed, and was not dysmorphic. There was no noticeable change when checking the ear, nose, and throat. The cardiopulmonary examination was normal. The patient’s central nervous system examination showed good sensation and reflexes. He had no skin rash or organomegaly. The liver function tests and complete blood count were all normal. Viral screening for human immunodeficiency virus (HIV), hepatitis B virus (HBV), and hepatitis C virus (HCV) were negative (Table ).\nThe coagulation tests were performed when he was asymptomatic. The main abnormal investigations were as follows: fast lysis of whole blood clots and shortened euglobulin lysis time. The other tests including clotting time, prothrombin time (PT), partial thromboplastin time (PTT), and thrombin time (TT) were all within the normal values. This suggests that there were no abnormal changes in blood coagulation. This was further confirmed by the testing of coagulation factor activity. Platelet count, bleeding time, and von Willebrand factor were also normal (Table ).\nConcerning the clot lysis test, the blood was collected and promptly placed in a glass test tube and incubated at 37°C. The clot was formed and retracted normally, but then underwent lysis. Following a few hours of incubation (5–8 hours with interval assessment), the clot was hardly visible, and it was just a small strand of fibrin. The fibrinogen and d-dimer concentration were within normal limits. To determine the status of fibrinolysis, euglobulin lysis time was calculated, and the result indicated shortened lysis time (Table ). To further investigate, we treated the patient’s plasma with a pooled normal plasma and repeated the euglobulin lysis time, which returned to normal. However, in such cases, it is preferable to use thromboelastometry to verify the diagnosis. Unfortunately, due to a lack of facilities, this technique was not performed. The defect seems to be inherited as an autosomal recessive trait (Fig. ). |
The proband was a 34 year-old right-handed man. From the age of 26 years, involuntary movements of the bilateral lower limbs, associated with dysarthria, grinding teeth and drooling, appeared and gradually worsened. At 31, he suffered from epileptic seizures, which were considered to be generalized tonic-clonic seizure, but antiepileptic drugs had never been administered. One year later, involuntary movements spread to his upper limbs and orofacial automatisms including abnormal tic-like facial movements, tongue protrusion and biting his lips appeared. Then he was treated with haloperidol (2 mg three times a day) and baclofen (10 mg three times a day) for 2 years for his choreic and dystonic problems, but he responded poorly to drug treatments. At age of 34, his involuntary movements gradually spread to his whole body and epileptic seizures increased in frequency. Since the disease onset, the patient had never suffer from psychiatric problems. Neurological examination revealed poor muscle tone and absent deep tendon reflexes in all limbs. Additionally, right positive babinski sign was elicited. Laboratory data revealed elevated creatine kinase level in the peripheral blood. Acanthocytes were found in 4% of cells on the peripheral blood smear test. Doppler ultrasound examination revealed splenomegaly. Brain magnetic resonance imaging (MRI) showed progressive, symmetrical, mild atrophy of the caudate heads (Figure ). His 24-h continuous electroencephalography (EEG) showed generalized asynchronous theta and epileptiform activity, which mostly originated from the right temporal lobe. A nerve conduction study showed a polyneuropathy, which revealed the right peroneal nerve, right median nerve and bilateral ulnar nerves were partly damaged. His score of Mini Mental Status Examination (MMSE) was 27. The father of the proband did not show any neurological abnormalities and died from pneumonia at 65 years old (Figure ). The mother of the proband (II-3), a 65-year-old woman, showed mild involuntary movements in her limbs since the age of 45 years (Figure ). The proband's uncle (II-5), a 52-year-old man, showed mild cognitive impairment (MMSE 24), characterized by memory impairment and had seizures history of 31 years, which were simple partial seizures and treated with antiepileptic drugs (Figure ). His another uncle (II-1) and two sisters (III-1, III-2) had no neurological clinical symptoms. Brain MRI and peripheral blood smears of the proband's mother, his uncles and two sisters are normal. The clinical picture of the proband was suggestive for ChAc, but the inheritance mode of this family seems to be autosomal dominant.\nAll patients were of Han nationality from Hunan province, China. Blood specimens and genomic DNA were obtained from family members and 100 control subjects after informed consent. The 73 exons and flanking intronic splice consensus sequences of VPS13A were amplified by polymerase chain reaction (PCR) (, ). By sequencing, we identified a novel homozygous nonsense mutation c.8823C > G (p. Tyr2941*) in exon 65 of VPS13A in the proband (Figure ). Five members of the family including the proband's mother (II-3) and his uncle (II-5) were detected to be heterozygous for mutation c.8823C > G (Figures ). The homozygous nonsense mutation c.8823C > G (p. Tyr2941*) causes the loss of TPR10 domain of the vacuolar protein sorting 13A protein. This homozygous nonsense mutation c.8823C > G was not detected in 100 healthy controls, thus representing a novel etiology in an ChAc Chinese family. Besides, the mutations in genes responsible for Huntington's disease and McLeod disease were screened and the results were negative. |
A 49-year-old man presented with a soft and fixed lump in the left hypothenar area (Fig. ). The mass was not tender, but it was associated with symptoms of tingling sensation and paresthesia in the left ring and little fingers that had lasted for 4 years. Preoperative image studies revealed an encapsulated and multilobulated mass compatible with a lipoma, which measured 8 cm × 5 cm × 2 cm (Fig. ).\nUnder general anesthesia, the mass was operated by a T-shaped skin incision (Fig. ). The mass was mainly located in the subcutaneous layer, however, deep extensions were seen reaching into the carpal tunnel, the hypothenar muscles, and intertendinous spaces between the left index and little fingers. The palmar digital branches of the ulnar nerve for the ring and little fingers passed through the mass and the branch for the little finger was firmly attached to it (Fig. ). To enable a complete excision of the mass, the branch for the little finger was temporarily transected. After complete excision of the mass, the transected branch was coapted again under microscopy (Fig. ).\nWith the exception of temporarily reduced sensation in the left ring and little fingers immediately after surgery, no particular complications were noticed. Basic histologic examination revealed mature white adipose tissue without atypia. Furthermore, fibrous septa were observed, while mitotic figures, necrosis, or lipoblasts were not detected. Immunohistochemical staining showed no cyclin-dependent kinase 4 (CDK4) or mouse double minute 2 homolog (MDM2) expression. These results ruled out the possibility of malignancy (Fig. ). Complete sensory recovery was achieved 6 months after surgery, without any sign of recurrence (Fig. ).\nWe obtained the patient's clinical and radiologic data. Informed written consent was obtained from the patient for publication of this case report and accompanying images. This study was approved by the Institutional Review Board of Chonnam National University Hospital (CNUH-2018-308) and was conducted in accordance with the principles of the Helsinki Declaration II. |
A 59-year-old male visited the rehabilitation department because of ongoing difficulty swallowing. Two months earlier, a 15-kg piece of iron was dropped on his neck from a height of 10 m. He was admitted and diagnosed with whiplash injury and a compression fracture of the fifth thoracic vertebra. He was treated conservatively, and the neck pain subsided. As the swallowing difficulty continued, he visited the rehabilitation department.\nOn physical examination, there was slight atrophy of the right sternocleidomastoid and upper trapezius muscles. The tongue was deviated to the right, and the gag reflex was impaired but the uvula was not significantly deviated. He complained of mild dysarthria and a hoarse voice. Examination of the upper and lower extremities revealed no weakness, but the power of right scapula elevation, head tilt to the right, and neck rotation to the left were decreased. The sensory examination was normal.\nIn a motor nerve conduction study, the compound muscle action potential of the right accessory nerve was not evoked. Needle electromyography revealed abnormal spontaneous activity in the right trapezius and sternocleidomastoid muscles. Injury of the accessory nerve was confirmed, although the other cranial nerves were not checked because the patient would not cooperate.\nA videofluoroscopic swallowing study (VFSS) revealed that lip closure and jaw movement were normal, but the soft palate and tongue movements were impaired. There were decreased epiglottic inversion, laryngeal elevation and movement of pharyngeal wall and delayed pharyngeal transit time. There was no penetration or aspiration of liquid, semisolid, or solid, but a large amount of material was retained in the right vallecula and pyriform sinus ().\nTo identify the cause of the dysphagia, brain and cervical magnetic resonance imaging (MRI) were performed. Brain MRI showed no abnormal findings in the brain parenchyma, and therefore, a brain lesion was excluded. Cervical MRI showed medullary compression and disruption of ligaments (). The sagittal section of the cervical computed tomography (CT) clearly showed a posteriorly displaced basion with respect to the tip of the dense of axis, and therefore, a posterior atlanto-occipital dislocation was diagnosed. And in the axial view, bony erosion of the atlas on the right side was shown (). For the treatment of the posterior atlanto-occipital dislocation, surgery was recommended, but the patient refused. Consequently, he underwent occupational therapy for the dysphagia. We educated the patient on a compensatory strategy when swallowing (rotation of the neck to the right side while swallowing food) and advised him to have repeated swallows to reduce pharyngeal residues. And he was also encouraged to perform resistance and range of motion exercises for the oral tongue, tongue base, neck, and shoulders to improve swallowing function. We have followed him for one year, and his dysphagia symptoms (choking during liquid swallowing, sensation of retained residual after swallowing, and food leakage into the nasal cavity) have improved. But, the degree of atlanto-occipital dislocation which was evaluated by X-ray was not changed. |
In January 2007, a 9-month-old boy with recurrent anaplastic astrocytoma of occipital lobe was transferred to our hospital for further management. On referral, he looked chronically ill but was adequate development for his age. Neurologically, he showed grade IV right sided weakness. He was born at 40 weeks of gestational age by normal vaginal delivery and the baby weighed 3.1 kg at birth. An obstetric ultrasound examination showed a huge mass in occipital lobe (64×46 mm) at the gestational age of 38 weeks. At birth, he had no other physical abnormalities (head circumference 34 cm, 50-75 percentile). A brain magnetic resonance imaging (MRI) () at 1 week of age revealed a huge lobulated, soft tissue mass on parietooccipital lobe. Subtotal resection of the mass done at 2 weeks after birth confirmed anaplastic astrocytoma (). At 1 month of age, he was treated with chemotherapy as per the Korean Society for Pediatric Neuro-Oncology (KSPNO) infant brain tumor protocol (vincristine, etoposide, cisplatin, cyclophosphamide, carboplatin, ifosfamide) for 4 months until a follow-up brain computed tomography (CT) showed progressive tumor of 35×34 mm-sized mass in the occipital lobe. At 5 months of age, he underwent 2nd tumor resection. The tumor histology was the same as before. Within 2 weeks after the surgery, he was placed on temozolomide with no avail. At 7 months of age, chemotherapy was changed to irinotecan, celecoxib, etoposide and thalidomide. At 9 months of age, upon showing further progression of tumor on brain MRI, he was finally referred for further management.\nUpon arrival, he underwent the 3rd gross tumor removal at the age of 11 months and had post-operative recovery for two months. He was able to walk holding with right side weakness (grade IV), make eye contact and have control over his head at that time. And treatment strategy was followed by proton therapy to tumor bed for a total dose 40 cobalt gray equivalent (CGE)/20 fractions (Fx) in 4 weeks (). At 20 months of age, he was able to walk without assistance. Neurologically, he continued to have limping gait on the right. Neurocognitive function test by Korean-Wechsler Preschool and Primary Scale of Intelligence (K-WPPSI) at 38 months of age showed verbal intelligence 109, performance intelligence 71 and total intelligence 88 and he showed adequate development and at 51 months of age showed verbal intelligence 133, performance intelligence 108 and total intelligence 125. Now at 53 months of age, he remains disease-free with adequate head size for his age and the height of 50 percentile. The most recent follow up of brain MRI (3 yr post-proton therapy) showed a large surgical defect at left parietooccipital operative bed without evidence of tumor recurrence (). |
A 58-year-old Asian woman was admitted to our hospital for multiple nodular legions in the pancreas. She had undergone right nephrectomy for RCC 20 years earlier. Since then, she had undergone soft tissue resection of the right shoulder (2005), partial left nephrectomy (2006), and partial chest wall resection (2007) for metastases from RCC, and she was started on interferon therapy in 2007. In 2008, during routine follow-up, abdominal computed tomography (CT) revealed multiple space-occupying legions in the pancreas, but she had no subjective symptoms. Her carcinoembryonic antigen and carbohydrate antigen 19-9 levels were within normal limits. Contrast-enhanced abdominal CT revealed multiple stained nodules in the pancreas (Fig. ). 18F-2-fluoro-2-deoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) showed FDG accumulation in the tail of the pancreas (standardized uptake value, 2.5) (Fig. ), but no other accumulations of FDG elsewhere in her body. Abdominal magnetic resonance imaging (MRI) showed three stained nodular legions (one each in the head, tail, and body of the pancreas), but no evidence of dilatation of the main pancreatic duct or bile duct (Fig. ). Preoperative differential diagnoses included pancreatic endocrine tumor and metastatic carcinoma. On the basis of these findings and her previous medical history, she was diagnosed with multiple isolated metastases to the pancreas from RCC.\nAs metastases occurred while the patient was being treated with interferon, surgery was indicated. Intraoperative ultrasonography showed more than four nodules in the pancreas from the head to the tail, but there was no evidence of lymph node swelling or peritoneal dissemination. She underwent PPTP with splenectomy. Because all blood supply to the stomach comes from the left gastric artery via intramural vessels and all blood drains from the stomach through the left gastric vein, close attention was paid to preservation of these vessels. Her pancreas contained ten macroscopic and more than eleven microscopic metastatic lesions (Figs. and ). Their pathological diagnosis was compatible with metastatic clear cell RCC, similar to the primary RCC resected 20 years earlier (Fig. ).\nTwo weeks after PPTP, the patient fell during a hypoglycemic episode and broke her right femur. Subsequently, however, her control of blood sugar was generally satisfactory. Adjuvant therapy consisted of interleukin (IL)-2 (70,000 U/week) for 2 years, after which IL-2 therapy was discontinued because the patient was experiencing severe nausea and vomiting. There was no evidence of RCC recurrence 28 months after PPTP, but she changed hospitals thereafter. We were informed by a local hospital that she died as a result of gastrointestinal bleeding 35 months after pancreatic resection. |
Case 1: Ms. K, a 70-year-old woman who immigrated to the US at the age of 53, began complaining of watery eyes, chest pain, lower back and joint pain, leg cramps, and weakness. She harbored delusions of being afflicted with high blood pressure, uterine cancer, blood cancer with bone metastasis, brain cancer with extensive metastasis, and believed that her brain was "shrinking."\nShe first visited a cardiologist in 2013, complaining of intermittent episodes of chest pain over six months. An electrocardiogram (EKG) at the time showed bradycardia, a first-degree atrioventricular (AV) block and a left bundle branch block. At her sixth visit with the cardiologist, she mentioned non-specific somatic complaints, which she said were because of a "hematological problem." Five months later, she was evaluated for “renal hypertension” and imaging studies showed a renal cyst. While she did not follow up with the nephrologist, she continued to make hospital visits for persistent chest pain. A full medical workup was completed and found to be normal at every ER visit. Medical records from a prior ER visit revealed that she had made claims that the Russian military entered her residence and stole her urine, resulting in the disappearance of her kidneys.\nMs. K was brought to the ER by the police after she showed up with a can of gasoline and matches at her primary doctor's office and threatened to burn it down. She was irate and claimed that all of her doctors, in the US and in her home country, were concealing the fact that she had oncological issues. She vehemently denied any psychiatric illness, stating that these diagnoses appeared on her records as a result of a rumor started by an envious former colleague. She explained that because she had been a former practicing neurologist in her home country, she was confident that she had cancer. Upon repeated questioning, she admitted that in a final bid to receive the medical attention that she was rightfully due, she had devised the plan to burn down the doctor’s office.\nWhile in the psychiatric inpatient unit, she remained somatically preoccupied and reported abdominal pain, lower back pain, and weakness, which she attributed to the metastatic spread of uterine cancer to her spine. Radiological imaging confirmed no evidence of uterine cancer, though a thickened endometrium was reported with recommendations for further testing by tissue sampling. Because Ms. K’s ability to make rational and reasonable decisions about her psychiatric and medical treatment was compromised by her delusions, the team sought and was granted a court order allowing them to treat her over her objection. |
A 50-year-old Hispanic woman with no significant past medical history presented to our emergency department with a 1-week history of left eye pain and 1-day history of decreased vision in her left eye. She had visited another hospital 2 days prior to presentation, where she was diagnosed with acute dacryocystitis and preseptal cellulitis, based on her clinical exam and orbital imaging (). She was discharged home on oral amoxicillin/clavulanic acid and asked to follow up with ophthalmology as an outpatient. Despite compliance with the prescribed oral antibiotics, her symptoms worsened significantly, thus prompting presentation to our institution.\nOn evaluation, visual acuity was 20/25 OD and no light perception OS. The left pupil was 4 mm and nonreactive with a left relative afferent pupillary defect. She had left proptosis with complete ophthalmoplegia. She also had left upper and lower eyelid edema and erythema and significant resistance to retropulsion of the left globe (). Intraocular pressure was 80 mm Hg OS.\nComputed tomography (CT) of the orbits with contrast showed an enlarged left lacrimal sac, a large complex fluid collection in the inferomedial left orbit, and severe tenting of the posterior globe (). There was an interval decrease in the size of the left lacrimal sac compared to prior CT, suggestive of posterior rupture into the orbit with subsequent decompression of the sac.\nThe patient was started promptly on intravenous vancomycin and piperacillin-tazobactam and taken emergently to the operating room. Significant purulent material was drained from the lacrimal sac, which was noted to have ruptured posteriorly into the orbit. After drainage of the lacrimal sac and medial aspect of the orbit, no additional purulence was noted. A lateral canthotomy and inferior cantholysis was performed.\nPostoperatively, the patient had significant reduction in proptosis, resistance to retropulsion, eyelid edema, and erythema. As there was no improvement in motility after admission day 4, intravenous methylprednisolone was administered with subsequent significant improvement in motility. Unfortunately, her visual acuity remained at no light perception.\nIntraoperative cultures were positive for Staphylococcus epidermidis. She was discharged on postoperative day 6 with a peripherally inserted central catheter and a 2-week course of intravenous ceftaroline and oral metronidazole. Three months after discharge, she underwent uncomplicated left external dacryocystorhinostomy and lateral canthoplasty. Crawford tubes were removed 12 weeks postoperatively. At 6 months of follow-up, she was without tearing. |
A 38-year-old man presented to the emergency department (ED) complaining of pain in his left knee and inability to ambulate after being tackled to the ground. He stated that he had been drinking alcohol that day and got into a physical altercation that ended when police tackled him to the ground. His past medical history consisted of schizophrenia, for which he was compliant with his medications.\nThe vital signs were all within normal limits, and the Glasgow Coma scale (GCS) was 15. The patient appeared intoxicated and was uncooperative during certain procedures of the physical exam. His left knee demonstrated a large effusion with ecchymosis over the anterior, superior portion of his knee. Bony landmarks of the knee were difficult to palpate because of the large effusion. Distal peripheral pulses were palpable. The patient was resistant to allow both active and passive range of motion of the knee, and refused assessment for ligamentous instability. The remainder of his physical exam was unremarkable.\nStandard 3-view radiography of the knee was performed, which did not reveal any foreign bodies, fracture or subluxation. Given their diagnostic uncertainty, the authors performed a bedside ultrasound of the knee using a 13-6 MHz linear transducer (Sono-Site M-Turbo, Bothell, WA). Ultrasound examination demonstrated an anechoic area at the distal end of the quadriceps tendon at its attachment to the superior pole of the patella, suggestive of a quadriceps tendon rupture []. A comparison view of the right knee revealed no such abnormality [].\nAfter the diagnosis was made, the patient was placed in a knee immobilizer; the leg was elevated; ice and analgesia were given; and the orthopedic surgeons were notified. The patient was admitted to the orthopedic service and underwent operative repair of a complete quadriceps tendon rupture the following day. The patient was later discharged to a rehab facility and had an uneventful recovery. |
A 51-year-old male with a permanent IVC filter that had been inserted approximately 20 years ago when the patient developed a DVT during a hospitalization for severe non-ischaemic cardiomyopathy, was transferred to our medical intensive care unit for shock and acute renal failure. Following the IVC insertion, he had been treated with warfarin for one year and had been on anti-platelet therapy since.\nHe had been admitted to the hospital three days prior to transfer after presenting with progressive bilateral lower extremity pain and decreased sensation in his gluteal region. Acute bilateral DVTs involving the common femoral and popliteal veins were diagnosed. Over 48 h, despite receiving unfractionated heparin, he developed anuric renal failure and shock. Placement of a right internal jugular dialysis catheter was complicated by airway compromise due to a retropharyngeal haematoma necessitating endotracheal intubation. The heparin infusion was discontinued and the patient was transferred to our hospital.\nOn arrival, his mean arterial pressure was 71 (104/53) mmHg while on norepinephrine, vasopressin, and phenylephrine. Arterial blood gas analysis showed a pH of 7.06, partial pressure of carbon dioxide (PaCO2) of 28 mmHg, partial pressure of oxygen (PaO2) of 312 mmHg, and a lactate of 16 mmol/L. The platelet count was 31 K/μL. Examination was notable for tense bilateral lower extremity oedema. Dorsalis pedis pulses were detectable with Doppler ultrasound. An abdominal computed tomography (CT) showed dilation of the distal IVC suggesting thrombosis (Fig. A). Transthoracic echocardiography showed a 25% ejection fraction with no right ventricular dilation or strain. The IVC was collapsible proximal to the hepatic veins. Lower extremity ultrasound confirmed acute bilateral DVTs involving the external iliac and femoral veins. Laboratory evaluations excluded thrombophilia, heparin-induced thrombocytopenia and thrombotic thrombocytopenic purpura. Infusion of 5 L of isotonic fluid and continuous renal replacement therapy led to a reduction in the vasopressor requirement, a reduction in lactate to 2.0 mmol/L, and pH/PaCO2 normalization. However, the lower extremity oedema progressed with development of bullae and purple skin discolouration (Fig. B). Dorsalis pedis pulses became undetectable, consistent with compartment syndrome due to PCD.\nCatheter-directed thrombolysis, surgical thrombectomy, and fasciotomy were deemed to be contraindicated due to ongoing shock, severe cardiomyopathy, the retropharyngeal haematoma, and persistent thrombocytopenia thought to be the consequence of platelet consumption. Unfractionated heparin was restarted and, within 24 h, lower extremity pulses were again palpable. However, there was a progressive rise in creatinine phosphokinase to 44,000 IU/L and an increase in lactate to 5.8 mmol/L despite continued vasopressor support and continuous dialysis. His family decided to pursue palliation and withdrawal of life-supportive measures. Post-mortem examination confirmed an occluding thrombus at the level of the IVC filter with extension to the internal and external iliac veins (Fig A, B). The autopsy did not identify an underlying malignancy. |
A 21-year-old Hispanic male presented initially with increased pain in the middle back that radiated to both legs. A thorough neurologic exam was otherwise normal and a complete review of systems was negative. An magnetic resonance imaging (MRI) scan of the lumbar spine without contrast showed a well-defined lesion extending from the middle of the L1 vertebrae to the top of the L3 vertebrae as shown in . A laminectomy of the inferior portion of L1, all of L2, and the superior portion of the L3 lamina was performed. Upon opening the dura, unencapsulated tumor was found. Superficial tumor was removed and a deep dissection was performed revealing a deep tumor capsule that was removed in its entirety. Intraoperative pathology confirmed the diagnosis of myxopapillary ependymoma. Deep to the encapsulated portion, further unencapsulated tumor was found surrounding several nerve roots. A careful dissection was performed with intermittent, but not prolonged, firing of the gastrocnemius and anal sphincter noted on free-run electromyography (EMG). All visible tumor was eventually removed and a gross total resection (GTR) was presumed. Further histologic analysis showed no mitotic figures and no necrosis in the sample. After an uncomplicated post-operative course, the patient was discharged from the hospital on post-operative day 2.\nGiven that the piecemeal rather than en bloc GTR increased our concern for microscopic residual tumor, as well as the reported benefits of radiotherapy for MPE (discussed below), the patient was offered adjuvant radiotherapy. He was prescribed a total of 52 Gy in 26 fractions, using a dose painting approach in which the clinical target volume (CTV, defined as the postoperative cavity/pre-operative cranial-caudal extent of tumor) was prescribed 52 Gy in 26 fractions, and the planning target volume (PTV, defined as the CTV plus a 2 cm margin which encompassed the thecal sac from L1 to L3) was prescribed 49.4 Gy in 26 fractions. Radiotherapy was planned and delivered with the Tomotherapy® system (Accuray Inc., Sunnyvale, CA, USA) which allows the planning and delivery of intensity modulated radiotherapy (IMRT) via a helical delivery of megavoltage (MV) energy radiation, akin to a standard computed tomography (CT) scanner expect that therapeutic megavoltage radiation is delivered as opposed to kilovoltage diagnostic radiation, using a hydraulically controlled collimator to achieve modulation of the beam. This system also allows a MV CT scan to be performed prior to treatment to optimize accuracy of patient setup. The external beam radiotherapy (EBRT) was completed within 3 months following the initial surgery. Follow-up MRI scans of the lumbosacral spine were obtained 1, 6, 10, and 14 months postoperative. No scans showed any sign of recurrent or metastatic disease.\nNineteen months after the initial operation, the patient presented for routine follow-up with no complaints, a negative review of systems, and no abnormal physical exam findings. Spinal MRI with contrast showed the development of an enhancing lesion at the S1-S2 level at the tip of the thecal sac consistent with drop metastases; also noted was stable appearance of a linear enhancing lesion at the L2-L3 level as shown in . The last spinal MRI was five months prior and showed no evidence of this lesion. This new lesion developed far inferior to the previous radiation field.\nThe patient underwent a lumbosacral laminectomy from L5-S2. Following removal of the lamina between L5-S2 and the ligamentum flavum between L5 and S1, a firm tumor nodule was palpated between S1 and S2. An attempt to dissect the tumor was made, however, the tumor capsule had significant arachnoid adhesions involving multiple sacral nerve roots. This complicated the resection. Alternative angles of dissection were pursued, however sacral nerve adhesions were again found. The tumor was debulked, however, the capsule was unable to be removed due unacceptably high levels of intraoperative free-run EMG activity in the sphincter muscle. The dura was closed with visual tumor left behind. Pathology showed small bland cells forming perivascular pseudorosettes with intervening myxoid material, shown in . The morphology was similar when compared to previous resection material and determined to be consistent with recurrence. A Ki-67 stain showed a tumor proliferative index of 4-9 percent. The patient remained in the hospital for three days and at the time of discharge had no neurological deficits.\nOne month following the operation, the patient started his second course of EBRT; at that time, he felt well, with absolutely no symptoms and no neurological deficits on physical examination. He was again treated with Tomotherapy®; the CTV was prescribed a dose of 54.0 Gy in 27 fractions, while the PTV was prescribed a dose of 50.5 Gy. The PTV extended from inferior lumbar region to the superior two-thirds of the sacrum, with the superior extent of the PTV at the middle of the L4 vertebral body. IMRT was used to minimize dose to nearby sacral nerve roots that could potentially result in bladder incontinence, impotence, or bowel incontinence. During EBRT he developed nausea, as an expected complication, that was treated with prochlorperazine. Otherwise treatment was well tolerated and he completed as planned.\nFive months following the completion of radiotherapy, the patient followed up with radiation oncology. The patient had no complaints and a negative review of systems, including no bowel or bladder incontinence reported. A physical exam showed no abnormalities. An MRI with contrast noted the initial laminectomy at L2-L3 was unchanged, with a linear intradural enhancement seen consistent with MRI taken prior to surgery and radiotherapy. Fatty marrow changes were seen in lumbar vertebrae and attributed to post-radiation change. Evidence of the L5-S2 laminectomy was noted with an irregular heterogeneous enhancement seen at the surgical site. No definite residual mass was seen at the S1-S2 level. The patient is now over 48 months since initial diagnosis and 27 months since his second surgery and has no radiological or symptomatic evidence of tumor progression on continued follow-up. |
A 75-year-old woman with a medical history significant for hereditary pancreatitis and type II diabetes mellitus presented with an episode of acute pancreatitis and recent weight loss. The patient suffered recurrent episodes of pancreatitis sporadically for the past 30 years, which were generally mild and resolved with bowel rest, pain management, and hydration. The PRSS1 mutation was present in this patient. Similarly, multiple members of the patient's family tested positive for the hereditary pancreatitis mutation PRSS1 and had repeated pancreatitis necessitating pancreatectomy.\nLaboratory workup revealed an elevated lipase of 973 (NL: 0–160 U/L). Computed tomography (CT) imaging demonstrated findings of acute pancreatitis, including an edematous pancreas, peripancreatic edema, and fat stranding []. Pancreatic parenchymal calcifications which would be expected with a chronic process were absent. Abdominal magnetic resonance imaging (MRI) showed a dilated pancreatic duct as well as thrombosis of the portal vein, splenic vein, superior mesenteric vein, and the majority of the intrahepatic portal vein branches []. Magnetic resonance cholangiopancreatography (MRCP) further demonstrated abnormal fluid signal intensity in the portal vein and splenoportal confluence. An adjacent fluid collection was also noted consistent with a pseudocyst, with the suggestion of fistulous communication between the pseudocyst, the portal vein, and the main pancreatic duct at the head region raising suspicion for a PPVF [].\nEndoscopic retrograde cholangiopancreatography (ERCP) was subsequently performed to evaluate the pancreatic duct. A pancreatic duct leak was identified, but the guidewire could not be passed to traverse the leak, and therefore, no intervention was performed. The ERCP revealed contrast filling the small collection after cannulation of the main pancreatic duct at the ampulla. However, there was also opacification of the portal vein in the same injection of contrast, confirming the MRI suspicion of a PPVF [].\nThe patient underwent supportive medical management for symptom control and anticoagulation for the portal thrombosis for 2 months. The anticoagulant used was Warfarin 4 mg daily which was titrated appropriately to achieve a therapeutic international normalized ratio (2-3). Over the next 4 months, the patient's symptoms improved and anticoagulation was subsequently stopped once collateral circulation was identified on follow-up imaging []. |
A 20-year-old Chinese man presented with a 4-year history of scalp pruritus and hair loss. At the age of 16, he started to suffer from a gradual loss of hair, initially from the vertex and occipital areas, but eventually extending to the entire scalp during subsequent 1 month after the onset of symptoms. He reported no family history of a similar entity or trauma. Physical examination revealed alopecia of the entire scalp and the remaining hair was short and less than 2 cm, as shown in Fig. a. Upon palpation, the scalp felt mildly tender and had a boggy spongy consistency. The scalp could be easily pressed down to the underneath bone, but immediately restored to original shape. Computer tomography (CT) scan demonstrated diffuse thickening of the subcutaneous tissues in the occipital region, which was measured as approximately 15 mm (Fig. b).\nChemical panel, complete blood count, thyroid function test, and antinuclear antibody titers yielded normal outcomes. Trichoscopy revealed no yellow dots, black dots, or exclamation mark hair. Hair pull test was negative. No hair loss was observed in other body parts. Skin biopsy showed increased subcutaneous adipose tissue and mild perivascular mononuclear infiltration into the superficial dermis (Fig. a). Scant perifollicular mononuclear infiltration accompanied by perifollicular fibrosis was equally observed in Fig. b. The count of hair follicle was decreased. Adipocyte disruption and mucin deposition were also demonstrated, as illustrated in Fig. c, d.\nAccording to the findings and outcomes, he was eventually diagnosed with LA. After the diagnosis was validated, the patient was administered with prednisone at a daily dosage of 30 mg in combination with acupuncture therapy for consecutive 6 months. Acupuncture therapy has been reported to accelerate the blood circulation and stimulate the acupoint. However, the symptoms of hair loss and the thickening of subcutaneous fat tissue were not significantly mitigated after this combined therapy. |
Herein, we report a case of a 71-year-old female patient hospitalized for anemia, motor incoordination, gait disturbance, weakness, and decline of her functional state. She was asymptomatic eight months prior to the day she was hospitalized, when she had asthenia, adynamia, hyporexia, symptoms of dizziness, and vertigo. Six months prior to admission, the symptoms increased, with onset of loss of balance, and postural instability. The patient also developed deterioration for walking, with repeated falls, which led her to stop walking for fear of falling, and limited her physical daily living activities to simply transferring from the chair to the bed. Likewise, it was noted that upon flexing her neck, she reported feeling an electrical discharge irradiating from her back to her legs. Two months before hospital admission, she noted lesions on the tongue, which produced a burning sensation and pain when swallowing foods - leading her to decreased intake of food and a loss of 5 kg coupled with increased feeling of weakness in her general state. She consulted with her local hospital, where she was diagnosed with paraparesis and anemia to continue studies.\nAs personal antecedents, she revealed hypertension managed with captopril 50 mg every 12 h. She did not report alcohol consumption, vegetarian nutritional habits, or other personal or family antecedents of importance.\nUpon physical exam for admission, she was in poor general state, pale, and marked loss of muscle mass. Blood pressure was 130/80 mm/Hg, without orthostasis, respiratory rate 17 per min and heart rate 85 per min. Her weight before developing the diseasewas 70 kg; her current weight is 60 kg, height 1.65 m. The oral cavity showed smooth, shiny, reddish depapillated tongue with ulceration at lateral level ).\nNeurological exam revealed that she was alert and oriented in all three spheres, no cranial nerve involvement, or abnormal movements. She had muscle weakness grade 3/5 and spasticity in all four limbs, tone and tropism diminished. The superficial sensitivity, touch, pain, and temperature systems were normal. Deep sensitivity was altered, with lack of vibratory sensation of the sense of position in upper and lower limbs, numbness in hands and feet, and positive Lhermitte's sign. Gait was characteristic of sensory ataxia with postural instability, broad support polygon, positive Romberg's sign, diminished osteotendinous reflexes especially in lower limbs and bilateral flexor plantar reflex present. Different para clinical studies were carried out (). Urinalysis, serology, and direct Coombs test were also performed under normality parameters. Additionally, extended blood test was conducted, revealing red cell anisocytosis, macrocytosis, and poikilocytosis. White series with hypersegmented neutrophils and platelet series without alterations were found. Upper gastrointestinal endoscopy was performed with the biopsy showing chronic atrophic gastritis positive for Helicobacter pylori.\nGeriatric assessment scales were applied, showing: mini-mental exam (28/30), geriatric depression scale (4/15), physical aspect of Barthel's scale of activities of daily living (60/100), JH Downton's scale of risk of falling in the elderly (3 points), and assessment of nutritional state through the mini nutritional assessment (16/30 points).\nVitamin B12 deficit diagnosis was performed with hematological manifestations given by macrocytic anemia, neurological manifestations by ataxic gait, and in skin and mucosa by Hunter's glossitis. Because of the neurological alterations in association with ataxic gait and low levels of vitamin B12, nuclear magnetic resonance of the cervical and thoracic spine was requested, which documented images compatible with sub-acute combined degeneration of the spine (.\nReplacement with Cbl was begun, showing after three weeks increased Hemoglobin values with decreased levels of lactate dehydrogenase indicating improvement in ineffective erythropoiesis. Glossitis was resolved after a month of establishing Cbl reposition (C and D). After three months of treatment, gait improved with the patient walking independently; sensitive symptomatology also improved. |
A previously healthy 44-year-old woman presented to the emergency room with early satiety, abdominal bloating, and epigastric discomfort that had started about a week ago. A right upper quadrant ultrasound was done followed by a CT of the abdomen and pelvis. The CT scan showed a partially visualized large pericardial effusion. There was no lymphadenopathy. She then underwent an echocardiogram that showed a hyperdynamic left ventricle systolic function with large circumferential pericardial effusion with tamponade physiology. She was taken to the cath lab where a pericardiocentesis was performed and a total of 730 cc of sanguineous fluid was drained and a pericardial drain was placed. The pericardial fluid contained 65,000 WBCs with the differential showing 6% polys, 1% lymphocytes, 1% monocyte, and 92% highly atypical lymphocytes with moderate basophilic cytoplasm, rare punched-out vacuoles. Most of the large atypical cells had a vesicular nuclear chromatin, some with large nucleolus and marginated chromatin. A hematoxylin and eosin (H&E) stained cell block preparation is shown in . This in addition shows numerous apoptotic debris and histiocytes with ingested debris. In tissue sections of BL, the histiocytes and apoptotic debris constitute the starry-sky appearance. Similar findings were seen in this cell block preparation, providing morphological evidence of a highly proliferative lymphoma. Flow cytometry further confirmed the presence of a large B cell population comprising 94% of lymphoid gated events. They were kappa light chain restricted (strong expression of light chains) and were CD19 and CD20 positive. They aberrantly expressed CD10. They did not express CD5 and CD23. FISH was positive for the t(8;14). Overall, the diagnosis was consistent with BL. Diagnosing BL is a challenging process, especially in bodily fluids. However, as shown before [], the presence of atypical lymphocytes with characteristic morphologic features and confirmatory flow cytometry is very helpful in establishing the diagnosis. FISH for t(8;14) is virtually confirmatory of BL.\nFor staging she underwent a bone marrow biopsy that was negative for lymphomatous involvement and a CT of the torso that showed a decreased, now small pericardial effusion, stable small bilateral pleural effusions, a 2.6 cm lobulated soft-tissue lesion in the right atrium, and no evidence of lymphadenopathy. A cardiac MRI revealed on T1- and T2-weighted images a broad base, isointense structure measuring 3 cm × 1 cm arising from the lateral wall adjacent to the atrioventricular junction of the right atrium (). Connected to this broad based mass, there was an isodense, circular, well-circumscribed mass measuring 1 cm in diameter protruding from the broad based mass into the right atrium. Late gadolinium enhancement images showed heterogeneous enhancement of the broad base mass and no enhancement of the circular mass. Her LDH at presentation was normal and an HIV test was negative. A lumbar puncture revealed no evidence of lymphoma cells in the CSF. The patient was treated with four alternating cycles of modified CODOX-M and IVAC in combination with rituximab [] and achieved a complete remission. A follow-up cardiac MRI showed no evidence of the right atrial mass. She remains in remission to this date, more than 5 years since her initial presentation. |
A 73-year-old Japanese man on PD presented with progressive worsening of abdominal pain and cloudy peritoneal fluid. He had high blood pressure, and he started continuous ambulatory peritoneal dialysis (CAPD) because of hypertensive nephrosclerosis 8 years previously. A PD catheter was primarily inserted at the right abdomen, but it was removed and inserted at the left abdomen because of exit site and tunnel infection 5 years previously. He had no past medical history of diabetes mellitus and major abdominal surgery. In the peritoneal equilibration test, his result was high. Bloody ascites was not evident. One year previously, he had been hospitalized for PD-associated peritonitis caused by touch contamination that was treated with intraperitoneal cephazoline and cephtazidime. Bowel adhesion was not noted 5 years previously; however, local bowel adhesions and agglomeration of the intestine were detected by computed tomography (CT) after the identification of PD-associated peritonitis (Fig. , ). The major findings of EPS, such as peritoneal thickening and calcification, were not noted on CT.\nOn physical examination, his blood pressure was 134/74 mmHg, pulse rate was 76 beats/min, and temperature was 99.7 ° F. He complained of severe pain in the right upper quadrant of the abdomen, and this area was tender on palpation. The exit site was clear. Laboratory tests revealed mild inflammation, with a white blood cell count of 10,100 /μL and C-reactive protein level of 0.9 mg/dL. The peritoneal fluid cell count was increased at 980 /mL. Based on these findings, PD-associated peritonitis was diagnosed. CT showed localized dilation of the intestine, which suggested adhesive small bowel obstruction (Fig. ). As we suspected that the peritonitis might be associated with bacterial translocation from the dilated intestine, he was advised to stop eating and was switched from CAPD to hemodialysis. Additionally, he was treated with intravenous vancomycin and cephtazidime. The PD catheter was flushed once a day to prevent catheter obstruction with fibrin, and the characteristics of the peritoneal fluid were monitored. His abdominal pain was resolved and peritoneal fluid cell count decreased to < 30/mL, and thus, he resumed oral intake on day 8.\nAfter resumption of oral intake, his abdominal pain worsened and his peritoneal fluid cell count dramatically increased to 9600/mL on day 15. The peritoneal fluid became cloudy with a high amount of fibrin and white blood cells (Fig. ). Although he stopped eating again, his abdominal pain did not improve, and fecal material with foul smell was identified from the PD catheter on day 23 (Fig. ). Culture of peritoneal dialysate on admission was negative; however, culture of peritoneal dialysate on hospital day 23 was positive for Enterococcus faecalis and Bacteroides caccae. On CT, the intestinal contents disappeared and the dilated intestine collapsed, indicating that the intestinal contents had leaked into the abdominal cavity (Fig. ). Considering these facts, intestinal perforation was diagnosed, and he underwent ileocecal resection with colostomy creation. Although intra-abdominal adhesion was severe, fibrinous encapsulation of the bowel, which would suggest EPS, was not detected macroscopically during surgery (Fig. ). As indicators of EPS were not evident, the PD catheter was removed. The perforation site was located at the adhesive intestine. The tip of the peritoneal catheter was located in Douglas’ pouch, and it did not injure the adhesive intestine. Pathological examination of the resected specimen revealed inflammatory cells associatet with the peritonitis in the intestinal wall. Intestinal fibrosis, arterial alteration, and tissue calcification were not evident pathologically (Fig. , ). Although his serum beta-2 microglobulin (B2M) level was high (41.05 mg/L), amyloidosis and deposition of B2M were not observed (Fig. -). The postoperative course was uneventful and left arteriovenous fistula surgery was performed on day 42. Since then, he has been on maintenance hemodialysis with no recurrence of peritonitis. |
The patient was a 63-year-old male former smoker with a history of insulin dependent diabetes mellitus and hypothyroidism. He reported having a “sensitive stomach” for three years with cyclical episodes of nausea, vomiting, diarrhea, constipation, and poor oral intake over the course of three months, with progressive weakness and a 40 lb weight loss. Initially his symptoms were treated conservatively based on imaging demonstrating a diffusely distended GI tract concerning for enterocolitis or ileus. Conservative management was unsuccessful, with the patient presenting to the emergency department due to the sudden onset of intractable severe vomiting that lasted for hours, coupled with severe abdominal distention. The patient had never previously had a colonoscopy or esophagogastroduodenoscopy.\nPhysical examination revealed a cachectic man (BMI 16.69 kg/m2, Glasgow prognostic score 1 with intermediate prognosis) with a mildly distended and thinned abdominal wall. The abdomen was nontender with normal bowel sounds and without rebound, guarding, or evidence of peritonitis. Colonoscopy demonstrated a dilated colon with friable mucosa but no evidence of ischemia. The distal transverse colon demonstrated luminal narrowing with the suggestion of extrinsic compression. A CT abdomen and pelvis with contrast showed a large bowel obstruction with a transition at the splenic flexure with focal wall thickening. Radiographically this was considered to likely be secondary to a pancreatic tail cancer with local malignant extension into the colon. Additionally, possible infiltration of the omentum in the right hemiabdomen was concerning for omental carcinomatosis. Diffuse heterogeneous bone densities raised concern for metastatic disease versus patchy osteopenia.\nThe patient underwent a subtotal colectomy with ileostomy. Intraoperatively it was noted that the patient was cachectic with minimal body fat. The transverse colon was congested, distended, cyanotic, and densely adherent to the retroperitoneum in the area of the pancreatic body and tail. Firm, white tissue was identified outside the pancreas with likely invasion of the middle colic vessels. Palliative subtotal colectomy and end ileostomy were performed for symptomatic relief.\nOn macroscopic examination, a 5.5 cm constricted area was identified in the transverse colon with associated serosal puckering. Sectioning of the puckered area revealed a firm yellow-tan cut surface and numerous submucosal cystic spaces ranging from 0.1 to 0.5 cm in greatest dimension. Sparse focally adherent pericolic fat with scattered fibrinous adhesions and palpable lymph node candidates were identified.\nHistologic examination demonstrated a moderately differentiated adenocarcinoma invading externally into the colon at the splenic flexure with associated colonic stricture, ulceration, and mural fibrosis (). The pericolonic tissue, mesentery, subserosa, and lymph nodes demonstrated signet ring-like cells without typical adipocytes (Figures and ). The signet ring-like cells demonstrated a round to oval shaped nucleus which was pushed to the side of the cell by a cleared out cytoplasmic vacuole, resembling a signet ring. The cytoplasm was variably clear to eosinophilic. These signet ring-like cells were smaller in comparison to typical mature adipocytes and showed variation in size with a thickened cell membrane. No cellular atypia or mitotic figures were observed.\nThe signet ring-like cells were surrounded and separated by a mucoid to myxoid fibrovascular stroma. In the omentum, the signet ring-like cells were organized in lobulated aggregates separated by fibrous tissue. In the subserosal region, focal areas of signet ring-like cells were identified surrounding nerves, raising the concern for perineural invasion. Additionally, one pericolonic lymph node demonstrated signet ring-like cells mimicking the appearance of metastatic involvement ().\nImmunohistochemical staining of the signet ring cells demonstrated these cells to be negative for AE1/AE3, CD138, or Kreyberg staining while being positive for S-100 staining (Figures and ); the immunoprofile was most consistent with atrophic adipocytes rather than signet ring cell carcinoma. |
A 65-year-old Japanese woman was admitted to our hospital with a 1-month history of vomiting. She had undergone no medical examination for 10 years prior to admission. Nine months before admission, she was hospitalized at another hospital with dyspnea. She was diagnosed with acute heart failure and three-vessel coronary arteriosclerotic lesions by coronary angiography. She subsequently underwent coronary artery bypass grafting surgery. At that time, computed tomography (CT) revealed irregular soft tissue mass lesions with calcification in the abdominal aortic retroperitoneum and mesentery (Figures and ). Positron emission tomography (PET) showed that 18F-fluorodeoxy glucose mildly accumulated only in the irregular soft tissue mass lesions (standardized uptake value 3.35). Based on the PET result, the soft tissue mass lesions were considered unlikely to be cancerous, and she was followed up by regular imaging evaluation.\nOne month before admission to our hospital, the patient was readmitted to the previous hospital with persistent vomiting. Abdominal CT revealed enlargement of the abdominal aortic retroperitoneal and mesenteric soft tissue tumor masses. The masses were compressing her duodenum, leading to symptoms of bowel obstruction. She was clinically diagnosed with retroperitoneal fibrosis, and prednisolone was administered at a dose of 40 mg/day. After 3 weeks of treatment, however, the tumor masses did not reduce in size and her symptoms persisted.\nShe was transferred to our hospital at this point, and on admission she was afebrile (35.3°C), her body weight was 45.1 kg (no weight loss), and she had a performance status of 3. The palpebral conjunctiva appeared anemic, although we observed no marked hair loss, oral ulcers, tongue swelling, thyroid gland and lymph node enlargement, skin rash, arthritis, or Raynaud's phenomenon. The soft tissue masses were not palpable on the abdomen. In addition, there were no findings of syncope, dizziness on standing up, movement disorders, or sensory impairment. The results of laboratory examinations were as follows: white blood cell count increased to 14,750/μL, hemoglobin level decreased to 9.5 g/dL, platelet count was normal 302,000/μL, and erythrocyte sedimentation rate elevated to 21 mm/h. Her serum total protein and albumin concentrations decreased to 5.4 g/dL and 3.1 g/dL, respectively. Her serum protein fraction revealed that her albumin level was decreased to 57.0%, α1-globulin increased to 5.0%, and α2-globulin (13.6%), β-globulin (8.7%), and γ-globulin (15.7%) were all normal. Her serum IgG (1,512 mg/dL) and IgG4 (10 mg/dL) were normal. Her serum IgA decreased to 68 mg/dL. Her serum concentrations of IgM (70 mg/dL), C-reactive protein (0.1 mg/dL), serum creatinine (81.3 μmol/L), calcium (2.32 mmol/L), glycosylated hemoglobin (5.7%), and total cholesterol (188 mg/dL) were normal. She was positive for antinuclear antibody with a titer of 1 : 40, and her serum soluble interleukin-2 receptor level was increased to 662 U/mL. Urinalysis was normal with no Bence Jones protein. Electrocardiography revealed no abnormalities including axis deviation, bundle branch block, or low voltage. In addition, there was no granular brightness in the ventricular wall on cardiac ultrasound. No osteolytic lesion and compression fracture of the skull, femoral neck, or thoracolumbar spine were observed by whole-body CT.\nAfter being transferred to our hospital, the patient continued to take prednisolone at a dose of 40 mg/day. However, the masses still did not reduce in their size and her symptoms were still persistent. Therefore, on the 40th day of hospitalization, we performed mesenteric biopsy for histopathology and decided to attempt gastrointestinal bypass surgery using abdominal laparotomy. The intraoperative findings showed that the mesentery at the root of the superior mesenteric artery was very hard and thick with white coloration and was compressing the third portion of the duodenum. In addition, two fibrotic masses approximately 1 cm in diameter were found in the mesenteric lesion (). The fibrotic mass was resected and dissected to yield a histopathological and immunohistological specimen. Unfortunately, gastrointestinal bypass surgery could not be performed because her hemodynamics were unstable during the operation. At that time, postoperative finding was mesenteric panniculitis.\nThe result of a biopsy of the resected mesenteric lesion indicated poor cellular components and hyalinization and degeneration of tissue with a small amount of fat. No evidence of the infiltration of inflammatory or tumor cells was present (). Congo red staining demonstrated amyloid deposition (). In addition, immunohistological staining [] was positive for lambda light chains () but negative for kappa or transthyretin (Figures and ). In addition, electrophoresis of serum and urine proteins showed a monoclonal spike in the gamma region, which indicated IgG and Bence Jones protein lambda light chains. A gastric mucosal biopsy and random skin biopsy showed no evidence of amyloid deposition. There were no symptoms or physical, laboratory, or imaging findings of systemic amyloidosis. Accordingly, we finally diagnosed the patient with AL lambda amyloidoma in the abdominal aortic retroperitoneum and mesentery. After laparotomy, her general condition worsened due to complications of pneumonia and deep vein thrombosis. We scheduled bone marrow aspiration as soon as possible, but she experienced sudden acute myocardial infarction and died 5 months after admission. |
A 50-year-old woman with unremarkable family history was referred to our epilepsy clinic for recurrent episodes of abnormal behaviors lasting for 6 months. Her family described that she showed stereotyped behaviors and blank expression for the first time. The patient did not respond for up to 20 minutes, and then recovered spontaneously. The attacks were often followed by convulsions with involuntary micturition and accompanied with prolonged period of drowsiness and confused state continuing for up to one hour. The episodes occurred every 3 weeks in the first two months. Later the frequency of attacks gradually increased up to once a week.\nThe physical and neurological examinations were all normal. Laboratory examinations including fasting blood glucose and glycated hemoglobin (HbA1C) showed no abnormality. While epilepsy-dedicated brain MRI showed no abnormal findings, interictal EEG revealed occasional spikes on the left temporal area (). Under the diagnosis of left temporal epilepsy, oxcarbazepine 300 mg/day was introduced. However, her seizure was not controlled even after higher dosage of oxcarbazepine nor co-treatment with other antiepileptic drugs such as lamotrigine, valoproic acid, levetiracetam, pregabalin and topiramate. During the medical treatment, the longest period for symptom free that she experienced was only two weeks. Four months later, the attacks occurred almost every day. The patient was hospitalized to perform video-EEG monitoring for the further characterization of the attacks and the possible presurgical evaluation. Habitual convulsion was observed at 3 AM, but the EEG showed only diffuse slow activity during the attack (). However, a finger stick glucose level during the attack was only 36 mg/dL. On further questioning, it was apparent that her previous symptoms tended to occur in the early morning or several hours after meals, and that the post-attack confusion could be shortened if she drinks glucose-containing beverages. The subsequent endocrine evaluation suggested insulinoma, and contrast-enhanced Abdominal CT scan revealed an enhanced mass in the head of the pancreas (). After surgical removal of the tumor, the blood glucose level turned to normal. A benign insulinoma was also confirmed by histopathological evaluation. No abnormality was found in the follow-up EEG after six months, and the patient remained seizure-free without medication during the two year follow-up. |
A 44 year old female patient, cotton spinner by occupation, presented with pain and a hard mass in the right upper arm which had gradually increased in size in the past 2 months. Physical examination revealed a painless oval soft-tissue mass within the biceps muscle of the right upper arm. There was no upper limb length discrepancy, cafe au lait spot or any endocrine problem. A plain x-ray of the right humerus showed a typical “ground-glass-like” lesion without obvious soft tissue swelling and periosteal reaction. However, the bone cortex seemed to have defects near the fibrous dysplasia of the proximal humerus []. Computed tomography (CT) examination showed multiloculated radiolucent endosteal scalloping involving the full length of the right humeral shaft, combined with a solitary hypo-dense soft-tissue mass. It seemed that some continuity did exist between fibrous dysplasia of the proximal humerus with soft-tissue mass []. Clinicoradiological diagnosis was suspected soft-tissue sarcoma secondary to fibrous dysplasia of the humerus. Radiotherapy and chemotherapy were suggested along with wide excision surrounding the mass or amputation of the right upper limb and patient was transferred to this institute.\nIn order to get more clues for further diagnosis, magnetic resonance imaging (MRI) examination of the right upper arm was performed [Figure and ]. The results showed that the mass appeared a defined oval intramuscular (biceps muscle) type which was low signal intensity on T1-weighted images (T1WI), while homogeneous fluid-like high signal intensity on T2-weighted images (T2WI). Changes in intensity of the humerus were consistent with fibrous dysplasia. However, the bone cortex of the humerus was intact demonstrating that continuity with bone and soft tissue lesions was not apparent [Figure and ]. These findings did not correlate with the initial diagnosis of previous hospital but implied some lesion like ganglion cyst (composed of gelatinous mucoid).\nThe radiological findings made the diagnosis more confusing. A further 99mTc-methylene diphosphonate (MDP) whole-body bone scan was performed and the result revealed no concentration at the site of the mass in the right biceps, but increased uptake in the right humerus. These changes were interpreted as monomelic fibrous dysplasia. Due to the difficulty of making diagnosis, biopsy of both soft-tissue mass (total excision) and adjacent humerus (local curettage and allografting) was performed. The histological findings were described as intramuscular myxoma of soft-tissue mass and fibrous dysplasia of the humerus lesions, both without evidence of sarcomatous degeneration [Figure -]. A final diagnosis of Mazabraud syndrome (one atypical monomelic type) was established by the histological results. No recurrence of myxoma and sarcomatous degeneration of fibrous dysplasia were observed in 3 years followup. |
A 51-year-old male with a permanent IVC filter that had been inserted approximately 20 years ago when the patient developed a DVT during a hospitalization for severe non-ischaemic cardiomyopathy, was transferred to our medical intensive care unit for shock and acute renal failure. Following the IVC insertion, he had been treated with warfarin for one year and had been on anti-platelet therapy since.\nHe had been admitted to the hospital three days prior to transfer after presenting with progressive bilateral lower extremity pain and decreased sensation in his gluteal region. Acute bilateral DVTs involving the common femoral and popliteal veins were diagnosed. Over 48 h, despite receiving unfractionated heparin, he developed anuric renal failure and shock. Placement of a right internal jugular dialysis catheter was complicated by airway compromise due to a retropharyngeal haematoma necessitating endotracheal intubation. The heparin infusion was discontinued and the patient was transferred to our hospital.\nOn arrival, his mean arterial pressure was 71 (104/53) mmHg while on norepinephrine, vasopressin, and phenylephrine. Arterial blood gas analysis showed a pH of 7.06, partial pressure of carbon dioxide (PaCO2) of 28 mmHg, partial pressure of oxygen (PaO2) of 312 mmHg, and a lactate of 16 mmol/L. The platelet count was 31 K/μL. Examination was notable for tense bilateral lower extremity oedema. Dorsalis pedis pulses were detectable with Doppler ultrasound. An abdominal computed tomography (CT) showed dilation of the distal IVC suggesting thrombosis (Fig. A). Transthoracic echocardiography showed a 25% ejection fraction with no right ventricular dilation or strain. The IVC was collapsible proximal to the hepatic veins. Lower extremity ultrasound confirmed acute bilateral DVTs involving the external iliac and femoral veins. Laboratory evaluations excluded thrombophilia, heparin-induced thrombocytopenia and thrombotic thrombocytopenic purpura. Infusion of 5 L of isotonic fluid and continuous renal replacement therapy led to a reduction in the vasopressor requirement, a reduction in lactate to 2.0 mmol/L, and pH/PaCO2 normalization. However, the lower extremity oedema progressed with development of bullae and purple skin discolouration (Fig. B). Dorsalis pedis pulses became undetectable, consistent with compartment syndrome due to PCD.\nCatheter-directed thrombolysis, surgical thrombectomy, and fasciotomy were deemed to be contraindicated due to ongoing shock, severe cardiomyopathy, the retropharyngeal haematoma, and persistent thrombocytopenia thought to be the consequence of platelet consumption. Unfractionated heparin was restarted and, within 24 h, lower extremity pulses were again palpable. However, there was a progressive rise in creatinine phosphokinase to 44,000 IU/L and an increase in lactate to 5.8 mmol/L despite continued vasopressor support and continuous dialysis. His family decided to pursue palliation and withdrawal of life-supportive measures. Post-mortem examination confirmed an occluding thrombus at the level of the IVC filter with extension to the internal and external iliac veins (Fig A, B). The autopsy did not identify an underlying malignancy. |
A 54-year-old female presented for laparoscopic adrenalectomy, with a diagnosis of a vascular nonfunctioning adrenal tumour.\nShe had originally presented about 18 months previously with an episode of abdominal pain, dizziness, and vomiting. Blood pressure on admission to hospital was normal, although it was noted in the referral letter from her family doctor to have been 240/60 mmHg with a heart rate of 90 beats per minute.\nHer other medical histories included hypercholesterolaemia, polycystic ovarian syndrome, and morbid obesity treated with gastric banding 8 months before the current admission: this had resulted in a weight reduction from 117 kg (Body Mass Index, BMI 46.3) to 64.5 kg (BMI 25). She had diabetes mellitus, which had previously been controlled with insulin but since her bariatric surgery had been controlled with metformin. Her other drug treatment comprised fenofibrate, citalopram, vitamin B12, folic acid, and multivitamins.\nFollowing abdominal ultrasound on admission, she underwent abdominal CT on the same day, followed by a succession of other imaging modalities because of diagnostic uncertainty. She also had biochemical tests for phaeochromocytoma, Cushing's syndrome, and Conn's syndrome. Results of imaging and diagnostic tests are summarised in .\nThe anaesthetic technique for the laparoscopic adrenalectomy consisted of induction with midazolam 2 mg, alfentanil 1 mg and propofol 100 mg, neuromuscular blockade with rocuronium 50 mg, and maintenance of anaesthesia with oxygen/air/sevoflurane. Fentanyl was given to a total dose of 400 micrograms, and further rocuronium titrated against the response to a peripheral nerve stimulator. Blood pressure monitoring was with an automatic noninvasive technique, and no central venous pressure monitoring was used.\nDuring mobilisation of the tumour, blood pressure rose to 266/124 mmHg. Initially this was treated with increased inspired concentration of sevoflurane and increments of fentanyl. Similar episodes occurred on two further occasions, and an esmolol infusion was given during these episodes (total dose 200 mg). Following removal of the tumour which took approximately three hours altogether, there was no further recurrence of hypertension and no hypotension. The patient was extubated at the end of the procedure and returned to a general surgical ward. She remained well until her discharge from hospital two days later.\nHistology of the adrenal gland, reported six days later, showed that about half of it consisted of a distinct greyish-red lesion. Histology of this lesion showed an arrangement of small cells with hyaline globules, together with sustentacular cells. This is characteristic of a phaeochromocytoma and is known as a zellballen. The diagnosis was made by a pathologist experienced in the histology of adrenal glands. |
A 52-year-old female presented with anorexia and abdominal pain since 2 years. Patient gave history of undergoing hysterectomy eight years back, for endometrial carcinoma. Postoperatively radiotherapy was advised; but due to extreme morbidity, she could receive only 10 fractions. Details of this treatment were not available as she was treated at a different hospital. Clinically, the patient's vital organs were stable. On per abdomen examination, a 12 × 10 cm firm mobile lump was palpable in the left lumbar region. Her hemoglobin and counts were normal. Renal functions were also normal. Abdominal USG revealed an echogenic mass, 8 × 7 cm, showing posterior acoustic shadowing, probably inspissated fecal matter. A repeat USG performed 4 days later after giving water enema showed the mass unchanged in size and echotexture. CT scan indicated a mass with peripheral calcification [], but was unable to differentiate whether it was in colon or small bowel loops. There was no evidence of any recurrence or metastatic lesion of the endometrial primary. Barium enema revealed an entero-colic fistula with an enterolith at the site of the fistula []. Colonoscopy showed a fistulous opening in region of sigmoid colon. A clinical and radiological diagnosis of enterolith with entero-colic fistula was made. Patient underwent an exploratory laprotomy under general anesthesia after bowel preparation. At laprotomy a fistula between mid-ileum and sigmoid colon was seen, containing a hard nonmobile mass. Rest of the small bowel and colon was normal, no diverticuli were detected. Gall bladder and common bile duct were normal. Segmental enterectomy with sigmoidectomy [] was done and patient had an uneventful recovery. Cut-section of the specimen showed presence of an enterolith obliterating the lumen of the fistulous connection between the two bowel loops. The mucosa appeared normal. Histopathology was unremarkable, showing congestion and acute on chronic inflammation. The presence of enlarged nuclei was the only change that could be attributed to radiotherapy. Chemical analysis of the enterolith revealed calcium carbonate in a matrix of amorphous material. |
A 33-year old Caucasian woman was diagnosed with adenocarcinoma of the right breast in 1976 following the discovery of a breast lump. She underwent a right-sided mastectomy without adjuvant therapy. Two years later at age 35, back pain prompted a bone scan which revealed a 'hot spot' in the region of the lumbar spine. This was thought to represent spinal metastases and the patient received local radiotherapy. After counselling for risk-reducing surgery, the patient chose to undergo a bilateral salpingo-oophorectomy, but declined a proposed hysterectomy.\nThere was a strong family history of breast disease with both her mother and maternal grandmother diagnosed with breast cancer at 34 years and 41 years, respectively. There were no other known cancers in her family. In 2004, aged 62 years, the patient underwent genetic testing, which uncovered a BRCA1 mutation (185delAG). A prophylactic left-sided mastectomy was subsequently performed.\nAged 64 years, the patient presented complaining of change in bowel habit, faecal leakage, urgency and left-sided abdominal discomfort. Examination revealed a tender, palpable fullness in the left iliac fossa. Initial routine blood tests showed haemoglobin of 11.7 g/dl with a normal mean cell volume and no abnormalities of electrolytes, liver or kidney function. Tumour markers revealed carcinoembryonic antigen (CEA) 3 ug/L and CA125 3 Ku/L (both within normal limits). At colonoscopy, a necrotic lesion was seen in the proximal sigmoid colon. Biopsies confirmed a very poorly differentiated papillary adenocarcinoma of the sigmoid, the immunoprofile of which favoured ovarian origin rather than colonic; being highly positive to CK7, while negative to CK20 (Figures and ). In addition, there was some faint staining for ER but PR staining was negative. A staging CT scan excluded macroscopic metastases and reported a large bowel-related mass measuring 68 mm by 67 mm, contiguous with the left lateral aspect of the bladder with no associated pathological lymphadenopathy. The radiological appearance suggested a malignancy of colonic origin.\nLaparotomy findings included a bulky tumour adherent to the left pelvic brim and pelvic side-wall, as well as two separate peritoneal deposits, one within the pelvis and one at the terminal ileum. There was no further pathology noted. The patient underwent an en bloc resection of sigmoid colon, bladder, omentum, left pelvic side wall and uterus. The histology of the surgical specimens revealed peritoneal nodules of poorly differentiated papillary adenocarcinoma (Figures and ), a normal atrophic uterus with no evidence of tumour nor Fallopian tube remnants, and a poorly differentiated papillary adenocarcinoma of the large bowel which penetrated the bowel wall and had invaded adherent bladder (Figures and ). There was associated extramural venous invasion but no metastatic lymph nodes (total of 3 found). When the specimens were reviewed in a multidisciplinary setting, incorporating colorectal surgeons and gynaecologists, it was concluded that they represented a primary peritoneal carcinoma of high grade. A post-operative CA125 titre performed within 2 weeks of the operation was 3 Ku/L and subsequent titres over the following 4 months were between 3 and 4 Ku/L. She is currently receiving chemotherapy treatment. |
A 36-year-old woman was admitted for evaluation due to recurrent anaphylaxis. Three days before her hospital visit, the patient drank about 2,000 mL beer because of heavy stress. Normally she drank 1,500-2,000 mL beer twice a week. Two days later, she ate fried chicken for lunch and after 1-2 hours generalized urticaria and lip swelling developed, accompanied by dyspnea and light-headedness. However, there was no loss of consciousness. The patient recovered after a few hours of rest, although pruritus and general weakness remained. The following day, the patient underwent blood tests at a local hospital and marked elevations in her aspartate aminotransferase (AST) and alanine aminotransferase (ALT) levels were noted. When the patient arrived at the hospital, her blood pressure was 110/70 mmHg and urticaria with pruritus observed. She was treated with an antihistamine and steroids and referred to the emergency department at our hospital for further evaluation.\nThe patient had experienced five similar episodes in the past. The first episode occurred 10 years previously. At that time, the patient was suffering from stress brought on by overwork. The next day, 3 or 4 hours after eating black bean sauce with noodles, the patient developed generalized urticaria, lip and periorbital swelling, and nausea. At the same time, her throat felt constricted and she was short of breath. She recovered after treatment in the emergency department; however, it was difficult to determine the exact therapeutic regimen. The second episode occurred 3 years later. Generalized urticaria, lip swelling, and dyspnea developed after eating chicken stew. When the patient visited the emergency department of another hospital, her serum aminotransferase levels were between 1,000 and 2,000 IU/L and she was admitted. She was negative for hepatitis virus markers, and an abdominal ultrasound detected no abnormal findings. Her serum aminotransferase levels quickly dropped below 200 IU/L over the following week and she was discharged. About a month later, at a follow-up visit, her serum aminotransferase levels had returned to normal values. The third and fourth episodes, which occurred 3 years ago and 7 months ago, respectively, were similar except that neither was associated with specific foods. Worthy of note is that during the fourth episode, which was treated at another hospital, it was initially suspected that her blood pressure was low. In both the third and fourth episodes, her serum aminotransferase values rose to 5,000 IU/L and then improved rapidly. The fifth episode occurred 6 months ago after the patient ate noodles. These episodes had increased in frequency over the past year from approximately once every 3 or 4 years to twice a year.\nThe patient had no history of bronchial asthma or drug or food allergies. She also had not been taking any medicine or herbal supplements for several months. Her family history was unremarkable. The patient was a married housewife who had a regular menstrual cycle. She had been living in urban areas and had never had pets.\nAt the present admission, the patient was alert. Her blood pressure was 145/87 mmHg, and her other vital signs were normal. Neither urticaria nor angioedema was present because they had already resolved. Her abdomen was soft, without tenderness, distension, or organomegaly. Laboratory testing revealed that her complete blood count and differential were normal without peripheral eosinophilia (white blood cell, 9,400/µL; total eosinophil count, 47/µL). Her serum creatinine level was normal, and her blood glucose concentration was mildly elevated (177 mg/dL). In addition, her AST and ALT levels were markedly elevated (681 and 1,337 IU/L, respectively). Her levels of alkaline phosphatase, gamma-glutamyl transpeptidase, and lactate dehydrogenase were also elevated to 122, 129, and 339 IU/L, respectively; however, there was no hyperbilirubinemia. Serological analyses for hepatitis A, B, and C viruses and tests for autoantibodies such as anti-nuclear antibody, anti-mitochondrial antibody, and anti-liver kidney microsomal type 1 antibody were all negative. Furthermore, her serum ceruloplasmin level was normal. Serum tryptase and histamine or its urinary metabolites were not measured. Testing for metanephrine or catecholamines and other hormonal studies was not performed because of a low probability of pheochromocytoma or carcinoid syndrome. Abdominal computed tomography demonstrated mild periportal edema of the liver. There was no evidence of abnormal focal lesions in the liver or biliary dilatation. A liver biopsy was not performed.\nInitially, we suspected food-induced anaphylaxis, specifically wheat-induced anaphylaxis, because the intake of wheat-containing foods was a common feature in several episodes. Although her total serum IgE levels were 297 kU/L (reference range ≤100), wheat-specific IgE antibodies were not detected. Moreover, a skin prick test with a large panel of commercial extracts of food allergens, including wheat, returned negative results. The skin panel also contained extracts of apple, banana, grape, orange, peach, pineapple, tomato, beef, cabbage, carrot, celery, chicken, chicken egg, milk, codfish, crab, lobster, shrimp, oyster, peanut, walnut, hazelnut, and mushroom (Allergopharma, Reinbek, Germany). The skin test results were confirmed by a multiple allergen simultaneous test (Advansure Allergy Screen, LG Life Sciences, Seoul, Korea). In addition, when an oral provocation test with noodles was performed under controlled conditions and monitoring, no reaction was observed over the course of 1 hour. The patient then underwent a 20-minute exercise challenge test. The patient experienced no symptoms either during or after exercise, which suggested that the attacks were not caused by wheat or exercise. No association between the previous episodes and foods, medications, or other triggers could be identified.\nHer AST and ALT values improved rapidly without specific treatment, and on day 5 of her hospital stay her values were 40 and 258 IU/L, respectively (). Self-injectable epinephrine was prescribed and the patient was discharged. |
A fifty-seven-year-old male with a past medical history significant for asthma and hypertension presented with a chief complaint of distal left lower extremity pain. At the age of twenty-one he was struck by a motor vehicle and sustained multiple fractures as well as a significant degloving injury to his distal left lower extremity. He underwent multiple extensive surgeries involving bone and skin grafts. Approximately six years prior to presentation at our clinic, he experienced a considerable exacerbation of his pain symptoms, was diagnosed with a sural neuroma, and eventually underwent surgical excision of the neuroma. The sural nerve endings were buried deep into the muscle just above the periosteum.\nUnfortunately, his pain returned which he described as burning, shooting, and located over his left lateral leg distal to the knee extending down to the ankle. The pain was exacerbated with palpation, eversion of the foot, and prolonged standing. He was treated with physical therapy, gabapentin, amitriptyline, and TENS unit with minimal benefit. At the time of presentation, he was taking desipramine 25 milligrams (mg) nightly and hydrocodone-acetaminophen 5 mg/500 mg as needed, which alleviated only a portion of his pain. On examination, he was a well-appearing man in no apparent distress. His left lower extremity distal to the knee revealed skin grafts, scarring, and hyperpigmentation (). Hyperalgesia was present over the left lateral leg approximately halfway between the knee and ankle. This region had a positive Tinel's sign over a portion of the scar. There was no distinct palpable mass. The rest of the standard neurological examination was within normal limits.\nIn March of 2007, the patient received an injection of the sural neuroma site with local anesthetic and steroid. He experienced significant relief for only the duration of the local anesthetic effect. He was then scheduled for cryoneurolysis of the sural neuroma. Throughout a period of 3 years in our clinic, he reported excellent relief from cryoneurolysis for 3 months following each serial treatment. In fact, he reports that for a period of approximately 10 weeks following the procedure, “I forget that I even have a pain in my leg.”\nBecause of imaging limitations with fluoroscopy, ultrasound was used to better characterize the relationship of the cryoprobe in soft tissues relative to the fibula. The patient was placed in the right lateral decubitus position, and sterile prep and drape were performed using chlorhexidine/alcohol. Three milliliters (mL) of 1% lidocaine was infiltrated subcutaneously using a 27-gauge needle at the entry site for the cryoprobe located approximately 2 centimeters (cm) proximal to the skin grafts (). Using an 11 blade, a 3-millimeter (mm) stab skin incision was made through which a 12-gauge angiocatherer was inserted into the subcutaneous tissue with the aid of ultrasound guidance (General Electric Venue 40 ultrasound system and 12 L linear probe). Ultrasonographic examination of the corresponding painful site did not allow visualization of an apparent sural neuroma. A 14-gauge cryoprobe (Wallach WA5000 Painblocker) was inserted through the 12-gauge angiocatheter upon removal of the needle. Proper sensory stimulation was used to confirm proper placement of the cryoprobe at 125 Hertz (Hz) to reproduce paresthesias over the patient's typical location of pain. The cryotherapy session consisted of 6 individual cryolesions, each of 4 minute duration, with a 40-second thaw period between each lesion.\nDuring the beginning of the procedure, the cryolesion and cryoprobe were imaged using ultrasound both in-plane and out-of-plane view. The proximity of the cryoprobe to the fibula was easily visualized prior to beginning cryotherapy (). Any apparent vascular structures were readily avoided during cryoprobe insertion under direct in-plane ultrasonographic guidance and doppler examination. The spacial magnitude of the cryolesion relative to the cryoprobe was easily visualized under real-time ultrasound. The lesion appeared as an echo-opaque hemisphere with a hyperechoic rim with a posterior acoustic shadowing (). The lesion was easily appreciated as increasing in size radially throughout the 4-minute period as well as decreasing radially during the 40-second thaw period between lesions. The diameters (in cm) of the lesions were 0.80, 0.87, 1.01, and 1.12 at 1.5, 2, 3, and 4 minutes, respectively. The short axis view confirmed the spherical nature of the lesion at the tip. The largest ultrasonographically measured cryolesion was an area of 0.85 cm2 and a circumference of 3.28 cm, at 4 minutes into freezing cycle (). It must be taken into consideration that because of the posterior acoustic shadowing cast by the cryolesion, as its radius increases the dimensions of the posterior portion of the cryolesion must be approximated for measurement purposes. The cryoprobe was positioned so that the posterior portion of the cryolesion would include the periosteum of the lateral mid-fibula where the symptomatic neuroma was believed to lay. After a 4-minute cryolesion, the tissue changes were visualized at 50 seconds but then normalized at 2 minutes and 20 seconds (). The patient tolerated the procedure well without apparent complications. Sequential freezing and thawing pictures as presented in Figures –. |
A 52-year-old male presented to our hospital with a chief complaint of sputum. Productive cough with yellowish sputum for two months, four pulmonary nodule in both lungs which was detected in chest computed tomography (CT).\nUsing inhalers (tiopropium, salmeterol/fluticasone) because of chronic obstructive pulmonary disease (COPD) which was diagnosed 15 years ago. Seven years ago, he received occasionally a intravenous steroids because of COPD flare-ups which occurs one or two time. Pulmonary tuberculosis had been treated for three-years medical care in 13 years ago. Hepatitis C was diagnosed in 10 years ago.\nHis vital signs were within the normal range. There was no lymphadenopathy in the cervical, axillary, or inguinal regions. His had barrel chest. On auscultation of the chest, a coarse breathing sound with wheezing in left upper lung fields was heard.\nLaboratory data were within the normal range. Blood glucose was 116 mg/dL. HbA1c was 6.1%. Hepatitis C virus antibody test was positive. Human immunodeficiency virus antigen and antibody were negative, Paragonimus antibody and Aspergillus antigen were negative.\nA chest radiography showed an inactive tuberculosis scar in right upper lobe and two nodules in left lower lobe (). A CT scan of the chest showed three nodules in left lower lobe, a nodule in right lower lobe, and emphysema ().\nWe confirmed no endobronchial lesion by bronchoscopy. The bronchoscopic washing specimen was negative for the acid-fast bacilli smear and culture test, and also for the microbial and fungal culture test. A transcutaneous needle biopsy of the nodule at left lower lobe showed thick fungal hyphe with right-angle branching, highly suggestive for mucormycosis (). Because the patient was immunocompetent, we screened other affected lesion. However brain magnetic resonance imaging and nasal endoscopy showed no apparent lesion.\nThe patient was started on amphotericin B. Surgical resection of the affected lobe was not possible treatment option, because pulmonary function test showed that forced expiratory volume in one second of the patient was 45%. Amphotericin B was used during 1 week and discontinued after discharge. Posaconazole oral suspension was started for home treatment. Total treatment duration is 3 months. After treatment, the chest CT in the follow-up showed that the size of the nodules was reduced (). After the end of the treatment for 3 months, chest radiography revealed no definitive relapse of mucormycosis (). |
An 18 year old woman was referred to our outpatient clinic of Endocrinology, University-Hospital of Naples Federico II because of hypothyroidism due to Hashimoto's thyroiditis. Blood samples showed high levels of thyroperoxidase and thyroglobulin antibodies and normal calcitonin serum levels. The patient was euthyroid with normal serum levels of thyroid-stimulating hormone (TSH), free triiodothyronine (FT3), and free thyroxine (FT4) during replacement therapy with L-T4. At physical examination, a palpable nodule of ~2 cm in size was detected in the isthmus of the thyroid. There were no palpable cervical lymph-nodes. An US evaluation confirmed an isolated lesion located in the isthmus, showing an isoechoic solid nodule with smooth margins; its size was 18 × 13 × 6 mm with intra and perilesional vascularity (Figure ). Therefore, a FNA was performed and cytological results revealed a TIR3A lesion. The cytological specimen showed an increased cellularity with some microfollicular structures in the background of scant colloid (Figure ). Thus, we assessed the risk factors associated with the isolated TIR 3A nodule of our patient. According to the ATA guidelines we repeated the FNA which confirmed the same result (TIR3A). The second US (after 6 months) showed that there were no clear signs suggesting malignancy such as microcalcifications or taller than wide-shaped nodules. However, we found a small hypoechoic cranial component in the nodule with blurred margins and elastography revealed an increased stiffness in this cranial component. No nodules were detected in the contralateral lobes by US; cervical lymph nodes were normal. Among the possible risk factors, our patient referred a familial history of thyroid cancer. Her mother was submitted to total thyroidectomy for a follicular variant of PTC twenty years ago; our subsequent evaluation showed that she was disease free at the moment.\nOn this basis, we decided that a surgical treatment was indicated for our patient and assessed the risk/benefit of total thyroidectomy vs. isthmusectomy. |
This 27-year-old male NF1 patient was being followed in the NF clinic with known plexiform neurofibromas in the left submandibular region and right pelvis/hip girdle. He developed symptoms of lumbosacral plexopathy with right sciatica and ankle weakness and intermittent urinary retention. His previously stable pelvic tumor was reimaged and found to have increased in size from 15 × 12 cm2 to 19 × 15 cm2 (Figure ).\nOn PET imaging, it had a central area of necrosis with a surrounding high SUV region suggestive of an MPNST (Figure ).\nA biopsy confirmed a grade 3/3 MPNST. Given its size and location, neoadjuvant treatment was recommended prior to attempted surgical resection. Just prior to his scheduled admission for chemotherapy, he developed severe, acute right hip pain. Imaging revealed a fracture-dislocation of the hip (Figure ).\nThe initial concern was for a pathologic fracture, but on review of new and old imaging, it was felt to be due to chronic changes from the plexiform neurofibroma combined with hip girdle weakness from his MPNST-related lumbosacral plexopathy.\nHe was placed in tibial pin traction and subsequent hip abduction bracing. He was given two cycles of chemotherapy (doxorubicin/ifosfamide) followed by six weeks of inpatient radiation therapy (57.5 Gy, IMRT) to the pelvic region with sparing of the remainder of the plexiform neurofibroma. He had a complicated hospital course but eventually was discharged to a rehabilitation center. One month later, he underwent operative intervention targeting complete resection of the malignant, internal pelvic component of the tumor with sparing of the benign neurofibroma where it exited the pelvis. His postoperative course was complicated by a pelvic hematoma that resolved without intervention and worsening of his lumbosacral plexopathy with a complete foot drop.\nHe was transferred back to the rehabilitation center but suffered multiple hip dislocations. This limited his recovery, and after discussion of treatment options, the patient underwent a Girdlestone procedure, which is an excision arthroplasty of the hip, two months after his MPNST resection. He recovered well and ultimately was able to ambulate with a walker. There was no evidence of malignancy in the hip specimen.\nUnfortunately, 17 months after the Girdlestone procedure and 2 years after the initial MPNST diagnosis, he developed lung metastases. He failed treatment with a combination of gemcitabine and docetaxel with pancytopenia and the disease progressed. Currently, 2.5 years after the initial MPNST diagnosis, he is enrolled in a clinical trial with an MDM2 inhibitor and pembrolizumab. |
A 31-year-old male patient presented with pain and decreased vision in the right eye that had begun 1 year earlier. Because all symptoms resolved with rest, so the patient did not seek medical attention. One month before presenting to our clinic, symptoms had dramatically worsened and were occasionally accompanied by nausea and vomiting. A review of the patient's medical history revealed that he had been diagnosed with a retinal detachment in the left eye at another eye clinic 2 years earlier. The patient chose not to undergo surgical repair of the retinal detachment because he was afraid to undergo surgery. Between that time and the first visit to our clinic, vision in the left eye had painlessly decreased to no light perception (NLP). The patient did not use antiglaucoma or anti-inflammatory medications and had no history of systemic disease.\nAt the time of presentation, best-corrected visual acuity (BCVA) was 20/200 (refractive correction of −4.75D) in the right eye and NLP in the left eye. The IOP was 60 mm Hg in the right eye and 9 mm Hg in the left eye. Ocular examination revealed mild corneal edema, clear lens, and a normal deep anterior chamber with a wide angle in the right eye and a normal anterior chamber and clear lens in the left eye. Fundus examination showed a vertical optic disc cup-to-disc ratio of 0.9 in the right eye and a long-standing retinal detachment associated with proliferative retinopathy in the left eye. Humphrey visual field testing (30-2) showed predominantly temporal inferior and superior visual field defects in the right eye. A diagnosis of POAG was given to the right eye based on clinical signs and symptoms.\nThe patient was admitted to the hospital and topical (travoprost, once a day; timolo, twice a day; brinzolamide, twice a day), oral (vinegaramin, 25 mg, 3 times a day), and intravenous (Mannitol 20%, twice a day) antiglaucoma agents were administered. Unfortunately, IOP in the right eye remained above 40 mm Hg despite administration of the maximum amount of medical therapy. Therefore, canaloplasty was performed on the right eye in a routine manner. After applying topical anesthesia, an incision was made in the conjunctiva and Tenon capsule and a 4 mm × 5 mm superficial scleral flap was created at the limbus at 12 o’clock. A deep flap was then created within the superficial flap margin. A temporal paracentesis was performed before SC was unroofed and a trabeculo-Descemet's window was formed. A flexible microcatheter (iTrack 250A; iScience Interventional, MenloPark, CA) was used to dilate the full circumference of the canal by injecting sodium hyaluronate 1.4% (Healon GV) during catheterization. Following complete circumferential dilation of SC, a 10-0 polypropylene tensioning suture was placed. The deep flap was excised and the superficial flap and conjunctiva were closed in a watertight manner. High-resolution ultrasound biomicroscopy (UBM) was used to assess SC and anterior segment angle morphology (Figure ).\nOne day after surgery, BCVA in the right eye was 20/100, the cornea was clear, and IOP was 7 mm Hg. Fundus examination revealed scattered white-centered retinal hemorrhages in the periphery and posterior pole (Figure ). A mild choroidal detachment was detected in the right eye using UBM. Therefore, the patient was administered tobramycin and dexamethasone ophthalmic suspension every 2 hours. One week after surgery, the choroidal detachment had completely resolved, IOP had improved to 18 mm Hg, and retinal hemorrhages began to resolve. Three months after surgery, the BCVA in the right eye was 20/80 and IOP remained in control at 16 mm Hg with topical latanoprost 0.005% (1 drop per day, Pfizer Inc, New York, NY). Fundus examination showed complete resolution of all retinal hemorrhages. |
A 34-year-old female with a history of cerebrovascular accident 1 month back with left-sided hemiparesis presented with a history of severe right leg pain for 8–10 days, progressive blackish discoloration of the right lower limb for 3 days. Echocardiographic evaluation showed floating, pedunculated, soft thrombus in the cardiac chambers, attached to left atrial appendage, mitral apparatus, right atrial cavity, and at right ventricle apex (). CT angiography showed a common filling defect within the femoral bifurcations extending to the superficial femoral artery. The proximal and mid-superficial femoral artery showed absent filling. Reconstitution of flow seen in the distal femoral artery. Popliteal artery was not opacified suggestive of occlusion (). The patient underwent emergency right sided superficial femoral arterial thromboembolectomy (for SFA long segment thrombosis) and below knee amputation. Infective endocarditis with distal embolization was suspected, and the patient was started on empirical antibiotics. Post procedure she was started on heparin infusion along with antiplatelets.\nMultiple sets of blood cultures were sent, which were reported as negative. Prothrombotic workup was sent including anti-nuclear acid test, antiphospholipid antibody workup, and homocysteine levels. All the tests were negative. On the seventh day of hospital stay, the patient was noted to have newly onset dysarthria and delirium. MRI brain showed new acute infarct in the left parietal region. Hyperacute left middle cerebral artery (MCA)/posterior cerebral artery (PCA) borderline infarct. Old MCA infarct right sided with embolic infarcts in right MCA territory; focal thrombosis in right internal carotid artery (ICA) and absence of flow in the distal ICA and wall thickening in right common carotid artery; and focal irregularity in right PCA. CT chest and abdomen showed left upper and lower lobe pulmonary veins thrombus. Mediastinal lymphadenopathy and few lung nodules were present. A few airspace opacities and patchy consolidation were seen in the left upper lobe. Both kidneys (right more than the left) showed segmental wedge-shaped nonenhancing area suggestive of infarcts. Main renal arteries were patent. Other findings included right internal iliac artery occlusion, right common femoral artery occlusion extending to the superficial femoral artery, nonopacified popliteal artery, suggesting occlusion and few collaterals in the thigh.\nHistopathological examination (HPE) of the clot showed metastatic squamous cells ( and ). EBUS with mediastinal lymph node biopsy showed metastatic squamous cells (). The family reported a history of hysterectomy 1 year back with no histopathology reports available. Hence, it was decided to send Pap smear from the vault, which was suggestive of high grade squamous intra epithelial neoplasia ( and ). Oncology opinion was taken and a diagnosis of metastatic squamous cell carcinoma with vaginal vault as most likely primary with endocardial secondaries was made and initiated on palliative chemotherapy with paclitaxel and carboplatin. Bronchioalveolar lavage was done and the culture showed Pseudomonas aeruginosa. Antibiotic was adjusted according to the culture and sensitivity report. Patient was gradually bridged with warfarin and discharged home. |
A 53-year-old man, who denied any underlying disease before, underwent colonoscopy for routine health examination. Several hours later, sudden onset of abdominal pain around left upper quarter was mentioned. He came to our emergency department with chief complaints of abdominal pain and left shoulder pain. On arrival, his vital signs were as following: body temperature 36°C, pulse rate 82 beats per minute, respiratory rate 22 breaths per minute, and blood pressure 110/76 mmHg. The physical examination revealed pink conjunctiva with unremarkable cardiovascular and pulmonary findings. On palpation, tenderness over the epigastric and left upper quadrants of his abdomen was noted. Involuntary muscle guarding was also noted.\nLaboratory examination showed 17000/uL white blood cell (WBC) and normal hemoglobin (14.3 gm/dL). His ECG showed sinus rhythm with RBBB and his chest radiograph was normal without evidence of free air. During the observation period at ED, suddenly his systolic blood pressure was dropped to 90 mmHg with sinus tachycardia. Ongoing severe abdominal pain was mentioned. He received abdominal computed tomography (CT) under the initial impression of hollow organ perforation with septic shock. However, CT scan showed massive hyperdense ascites surrounded around the spleen without free air (Figures and ). Grade II of spleen laceration was found on this CT scan. Therefore, spleen laceration with hemoperitoneum as the complication of colonoscopy was impressed. General Surgeon was consulted at the same time. Due to the fact that severity of spleen laceration is grade II, conservative treatment was suggested. He received blood transfusion with PRBC 2U; then, he was admitted to ward. After admission, his hemodynamic status was stable with improving symptoms. So, he was discharged from our hospital 3 days later. However, LUQ pain was noted on the same day of discharge, so he came back to our ED immediately. Abdominal and pelvic CT was arranged again with progressing ascites compared to previous study. Grade III of spleen laceration was noted. General surgeon was consulted and splenectomy was indicated. He received splenectomy after admission. According to the procedural record of his colonoscopy, it was performed smoothly without any significant technical difficulty. Splenic injury was found few hours later on the day of colonoscopy. It might result from the extra tension between the spleen and splenic flexure which varies from different positions of patients.\nThe patient was discharged under the stable vital signs with improving symptoms. However, the follow-up abdominal CT scan showed progressed hemorrhage. In the end, splenectomy was arranged in order to prevent further worsening outcome. Whether routine follow-up CT scan is indicated even in stable asymptomatic patient may warrant further studies to have conclusion. |
A 71-year-old Japanese woman was referred to our hospital for evaluation of her elevation of serum gamma glutamyl transferase levels to 135 IU/L (reference value 16–73 IU/L), which were incidentally found in a biochemical test in medical health check. She had no symptoms of epigastralgia or jaundice. Her physical examination and past medical history was unremarkable. She did not smoke or drink. The remainder of her serum chemistries, complete blood count, and coagulation profile were all normal. Serum levels of tumor marker such as carcinoembryonic antigen (CEA) and carbohydrate antigen 19-9 (CA19-9) were within normal limits.\nAbdominal ultrasonography (US) and computed tomography (CT) demonstrated a cystic lesion measuring 2.0 cm in maximal diameter at the left lateral segment of the liver with peripheral left lateral anterior subsegmental bile duct (B3) dilation (Figure , and ). Magnetic resonance (MR) imaging also confirmed a cystic lesion at the left lateral segment of the liver, but the presence of a mural nodule in the cystic lesion or mucin was not confirmed (Figure ). MR cholangiography showed a cystic lesion at the left lobe of the liver, but a filling defect in the bile duct and a communication between the cystic lesion and bile duct could not be defined (Figure ). Duodenoscopy showed a widely patent papillary orifice with extruded mucoid material, and endoscopic ultrasonography (EUS) could not detect a tumor component in the bile duct or cystic lesion (Figure ). Endoscopic retrograde cholangiogram (ERC) demonstrated amorphous filling defects in the common bile duct corresponding to mucin, and percutaneous transhepatic cholangiograms (PTC) revealed communication between the hepatic cyst and the major bile duct (Figure ). The cytological examinations through both ERC and PTC routes were negative for malignancy.\nLeft and caudate lobectomy of the liver with extrahepatic bile duct resection and reconstruction was performed because the lesion seemed to be a potential malignancy. Gross examination of the resected specimens revealed no obvious mass protruding into the lumen, and only a markedly dilated bile duct was observed at a glance (Figure ). Microscopically, the cystically dilated bile duct was lined by tall columnar epithelium with micropapillary features and mucin hypersecretion, but the tall papillary growth with fibrovascular cores or villous structures showing mass component was not present (Figure ). These neoplastic cells with hyperchromatic nuclei and loss of cell polarity, lacking stromal invasion was observed (Figure ). Ovarian-like stroma typically seen in hepatobiliary cystadenomas was not observed in the wall. On immunohistochemistry using standard streptavidin-biotin-peroxidase method, meoplastic cells were positive for MUC2 and MUC5AC, but negative for MUC1, CK20. The patient has been well without any evidence of recurrence for 38 months since her operation. |
A 73-year-old Asian woman with an underlying anxiety disorder, functional headache, and hypertension was prescribed escitalopram and lorazepam when she presented with progressively worsening headaches to her primary care doctor. Her symptoms did not improve with the medications, and she was unable to eat well and required bed rest. She was transported to our hospital 4 days later after developing chest and back pain with altered consciousness. She was a housekeeper, had no allergies, and had no alcohol or tobacco smoking history. On arrival, her Glasgow Coma Scale score was 3/15 (E1V1M1); both pupils were approximately 4 mm in diameter and reactive. Her blood pressure was too low to be measured, her carotid artery pulse was palpable, her heart rate was 112 beats/minute, and her respiratory rate was 30 breaths/minute. Her conjunctiva was pale. An auscultation of breath sounds did not reveal upper and lower airway obstructions and was within normal limits. Her abdomen was soft and flat without tenderness. She had no skin abnormalities (such as rash). Both legs had no edema. Echocardiography on arrival was performed as point of care ultrasound and revealed a hypercontractile left ventricle with an eliminated left ventricular cavity and a collapsed inferior vena cava without right ventricular dilation. There was no pericardial effusion or obvious large regurgitant jet observed on color Doppler. In response, we immediately inserted a peripheral venous catheter and began introducing fluid resuscitation; however, she developed PEA. Conventional CPR according to the adult advanced cardiovascular life support guidelines (including adrenaline) was initiated and a return of spontaneous circulation (ROSC) occurred. However, her blood pressure was unstable and PEA returned, prompting repeated CPR with immediate administration of fluids and three adrenaline injections. Venoarterial extracorporeal membrane oxygenation (VA-ECMO) was initiated for refractory PEA. Whole-body contrast-enhanced computed tomography was unremarkable, and the admission laboratory results were also unremarkable, except for anemia (Table ). Her hemoglobin level decreased from 7.1 g/dL to 3.5 g/dL 1 hour later without obvious signs of gastrointestinal hemorrhage. Therefore, 8 units of packed red blood cells were transfused for 1 day, after which her hemodynamic status stabilized. She was in a coma without sedatives; thus, targeted temperature management at 34 °C was initiated on admission to an intensive care unit. Echocardiography in the intensive care unit showed a thickened interventricular septum (which was 12.8 mm), prolonged anterior mitral valve, and contact between the bodies of the anterior and posterior mitral valves, suggesting that the left ventricular obstruction could have potentially occurred through this redundant anterior mitral valve. VA-ECMO was terminated on day 3, and after stabilizing her hemodynamics, transthoracic echocardiography showed a sigmoid septum with normal left ventricular function (ejection fraction, 75%) (Fig. ). On day 26, dobutamine-infused (30 μg/kg per minute) Doppler echocardiography revealed a significant outflow gradient (236 mmHg) accompanied with chest pain (Fig. ) and intermittent systolic anterior motion (SAM) of the mitral valve; continuous monitoring during Doppler echocardiography showed a Brockenbrough–Braunwald sign (Fig. ), which is a fall of arterial blood pressure after premature ventricular contraction; these findings confirmed a diagnosis of latent LVOTO due to a sigmoid septum. The significant LVOTO was not dependent on SAM but might have occurred due to the greatly thickened interventricular septum. As a result, carvedilol was initiated with gradual increment up to 10 mg/day on day 35. In addition, verapamil (120 mg/day) was administered on day 29. A follow-up dobutamine-infused Doppler echocardiography on day 40 showed a reduction of the outflow gradient to 14 mmHg, indicating a successful medical therapy.\nThe worsening anemia that was identified at admission was suspected to be hemolytic anemia (HA) based on results of the blood test. The results of the laboratory examination, including total bilirubin, direct bilirubin, lactate dehydrogenase, reticulocyte count, and haptoglobin, and a direct Coombs test on day 1 are presented in Table . Her drug lymphocyte stimulation test was positive, and agglutination occurred when her serum reacted with lorazepam. Therefore, lorazepam-induced immunological HA was diagnosed. Her anemia improved following discontinuation of lorazepam. Her hemoglobin level was 11.9 g/dL on day 64, and she was eventually discharged on day 68 without any sequelae. |
A 70-year-old Chinese lady attended the glaucoma clinic during routine follow up in November 2007. She had undergone trabeculectomies with 5FU OU in 1998 for primary open angle glaucoma and phacoemulsification OD with foldable IOL implantation (SA60AT) through a 2.8-mm corneal tunnel in 2003. In 2005, she had a repeat trabeculectomy with MMC and a needling procedure OD. In her previous visits, the bleb in OD was thin, minimally vascular with slight elevation, and the intraocular pressure (IOP) was maintained at 12–15 mmHg (applanation tonometer), the iris was normal, and the best-corrected visual acuity was 20/30. The cupping in OD was noted to be 0.65 with inferior neuroretinal rim thinning. Fast retinal nerve fiber thickness analysis by optical coherence tomography showed similar inferior thinning with thickness in other quadrants in normal range. The Humphrey field analysis (24-2) showed a superior nasal step. When she presented in November 2007, the best-corrected visual acuity was 20/40 in OD and 20/30 in OS and the IOP was 26 mmHg and 16 mmHg OD and OS, respectively. The cornea was strikingly clear, the corneal wound appeared intact, and the chamber was deep. Surprisingly, the iris was completely absent and the posterior chamber intraocular lens with the opacified intact capsular bag was visible in its entire extent []. Few remnants of iris tissue were stuck to the nasal anterior capsulotomy margin. The bleb was almost flat with some vascularization, and the underlying sclera was pigmented and the pigmentation extended posteriorly upto the fornix []. On questioning about possible trauma, the patient revealed that she sustained injury OD 4 months ago by hitting against the edge of a table back in China. She had pain and decreased vision following the injury for which topical medications where started but could not provide further details although no intervention was done. Gonioscopy showed no signs of angle recession; faint pigments were found at the second sclerostomy opening/fistula and none at the previous fistula. Dilated fundus examination revealed 0.8 cup with superior and inferior thin rims but healthy retina and macula OD. The field defect had increased to a superior arcuate scotoma. There was a functioning bleb with normal iris OS. She was started on latanoprost eye drops OD, but since adequate control was not achieved, Baerveldt tube surgery was subsequently performed. |
The patient was a 60-year-old man who had been suffering from isolated chronic non-productive cough for about 1 year. He discontinued smoking 9 months before the first visit following development of this cough and had never taken an ACE-I. Although he did not complain of heartburn and other symptoms suggestive of GERD, endoscopic assessment of the esophagus revealed reflux esophagitis (Los Angeles classification Grade B). He had had no respiratory infections during the 8-week period preceding the first visit. No abnormal shadows were noted on chest or paranasal sinus X-rays and chest CT scan. Cutoff points in testing of bronchial hyperresponsiveness and cough reflex hypersensitivity were set at below 10000 μg/ml [] and 3.9 μM []. Airway reversibility to inhaled β2 agonist was 6.5%, and testing for bronchial responsiveness to methacholine and cough reflex sensitivity revealed no hyperresponsiveness (29053 μg/ml) and no hypersensitivity (500 μM). Cell fractionation of bronchoalveolar lavage fluid revealed percentages of macrophages, lymphocytes, neutrophils, and eosinophils of 91%, 7%, 1.7%, and 0.3%, respectively. Cough was evaluated based on frequency and intensity as follows: 10 = cough level at the first visit, 5 = half the level at the first visit, 0 = none. Neither bronchodilator therapy nor anti-inflammatory therapy improved the cough. PPI was given after discontinuing bronchodilator and anti-inflammatory therapy. The cough was markedly improved 2 weeks after initiation of PPI (cough level 1), but returned nearly to pretreatment level 3 weeks after discontinuation of PPI (cough level 7, cough sensitivity 62.5 μM). On 24-h esophageal pH monitoring performed prior to re-initiation of PPI to determine the reason cough improved with PPI, the probe was positioned in the lower esophagus 5 cm above the upper border of the lower esophageal sphincter. Acid reflux in the esophagus was considered present if pH was 4 or less []. Some cough and acid reflux were observed, little cough-related acid reflux was noted (Figure , *; cough, #; acid reflux, $; cough-related acid reflux). Following re-initiation of PPI, the cough disappeared (cough level 1, cough reflex sensitivity 62.5 μM). |
In September 2015 a 74-year-old white woman presented with abdominal pain, pyrosis, and weight loss. Her medical history included cholecystectomy for cholelithiasis in 2010. Her blood analyses were normal. A physical examination was unremarkable. A computed tomography (CT) scan showed an intrahepatic neoplastic lesion of 38 mm localized in the gallbladder bed associated with multiple spleen, pulmonary, and peritoneal metastases. Serum levels of CA19-9 were increased (1100 U/mL; reference range less than 37 U/mL). She was then referred to our oncology division. The Eastern Cooperative Oncology Group (ECOG) performance status (PS) was equal to 0; a physical examination found only mild abdominal tenderness. In October 2015 an ultrasound-guided biopsy was performed on the intrahepatic lesion. Pathological analysis revealed the presence of bile duct adenocarcinoma cells; immunohistochemistry showed strong expression for CK19. A pulmonary biopsy on one of the secondary lesions confirmed the diagnosis of lung metastasis. From November 2015 to January 2016 she underwent first-line chemotherapy with cisplatin (25 mg per square meter of body surface area) followed by gemcitabine (1000 mg per square meter), each administered on days 1 and 8 every 3 weeks []. After three courses of treatment, a CT scan showed a condition of stable disease assessed by Response Evaluation Criteria in Solid Tumors (RECIST) evaluation criteria. Her serum levels of CA19-9 were slightly decreased to 576 U/mL. No signs of bone marrow toxicity were observed; grade 1 of gastrointestinal toxicity was recorded (nausea and vomiting). She was then treated with an additional three courses of cisplatin-gemcitabine. A following CT scan revealed a dimensional increase of lung metastases with a 10% increase by RECIST criteria, whereas no changes were observed in the other lesions; her CA19-9 serum levels were increased to 753 U/mL. Grade 4 thrombocytopenia was observed soon after the fourth cycle of chemotherapy; the doses were then reduced by 10%. Grade 1 constipation and nausea were reported for the remaining courses. Her PS had not deteriorated; her liver and renal functions were normal. We then decided to treat her with gemcitabine alone as maintenance therapy starting in April 2016, with the following schedule: 1000 mg per square meter, administered on days 1, 8, and 15 every 4 weeks. After the second cycle, grade 3 thrombocytopenia and lower limb lymphedema were observed and the doses were reduced by 20%. In August 2016 a CT scan revealed a significant dimensional regression of lung lesions (50% reduction as assessed by RECIST evaluation criteria) and a complete regression of spleen lesions (Fig. a–c); her CA19-9 serum levels were decreased to 536 U/mL. She continued maintenance therapy with gemcitabine alone for 10 months until February 2017 when a CT scan showed lung disease progression and new liver lesions (Fig. d). At the same time, her CA19.9 serum level increased to 1265 U/mL (Fig. e). |
A 26-year-old male presented to Accident and Emergency following assault with a baseball bat. His past medical history consisted of longstanding Hepatitis C infection only, he was on no regular medications nor had any known allergies.\nOn admission, the patient had complained of bilateral pleuritic pain on inspiration with visible, tender bruising to the posterior thoracic region bilaterally. There were no urinary, bowel or neurological symptoms reported and no open wounds were visible on examination. The anterior abdomen was soft and non-tender to palpation.\nA CT scan of chest (performed at 30s post i.v. injection) showed acute fractures of left 10th/11thand right 8th/9th ribs, but no evidence of pneumothorax or lung contusion. A splenic laceration was not suspected clinically and the timing of the scan, optimized for the chest, only showed subtle splenic changes which were not identified by the original reporter ().\nLater that day, the patient complained that their pain had spread from the chest down to the right and left flanks. This prompted a CT scan of abdomen/pelvis (performed at 70 s), which demonstrated a contained splenic laceration with no evidence of capsular breach (). Both the rib fractures and splenic injury were treated conservatively with bed rest and analgesia.\nThe patient abruptly deteriorated becoming hypotensive and tachycardic in keeping with hypovolaemic shock. A further CT scan showed splenic capsular rupture, active bleeding and large-volume intraperitoneal haemorrhage ().\nThis was managed urgently with endovascular embolisation. While the patient gave informed consent for the procedure he indicated that he was unlikely to be compliant with some aspects of his aftercare and likely to self-discharge very soon after completion of the procedure.\nFollowing local anaesthesia an ultrasound guided puncture of the right common femoral artery was performed and the splenic artery selectively catheterized with a Sim 1 catheter (a reverse angled catheter). Angiography revealed three pseudoaneurysms, two arising from a second order upper pole branch and one from a third order equatorial branch (). These were then superselectively catheterized and embolized with a series of 3, 4 and 5 mm microcoils (Nester & Vortex, Nester = Cook Medical, Bloomington, IN, USA, Vortex = Boston Scientific, Watertown, MA, USA), delivered through a microcatheter. This abolished filling of the pseudoaneurysms but there was more devascularisation of the upper half of the spleen than envisaged when the decision to perform superselective embolisation was made. The right common femoral artery was then closed with a Starclose vascular closure device (Abbott Medical).\nThe decision to perform superselective embolisation was made with the intention of achieving definitive haemostasis in a patient who was likely to leave hospital at the earliest possible opportunity and not comply well with the procedural aftercare (). There was no further bleeding.\nDay 1 post-procedure, the patient developed a post-embolisation syndrome (PES) characterized by fever, nausea and left abdominal pain. A fourth CT scan was performed. This showed splenic necrosis, parenchymal and intravascular gas, which is an expected appearance post-embolisation. No fluid collection was demonstrated. Conservative resuscitation was initiated with i.v. fluid and antibiotic administration.\nThere was no indication for surgical drainage at this point as the CT scan showed only splenic necrosis, with no evidence of abscess formation. The patient’s temperature and clinical features were also settling, further reassuring stabilisation of his condition ().\nThe patient recovered from this episode a few days later, and deemed medically fit, was subsequently discharged home.\n3 weeks later, the patient presented to Accident & Emergency once again; this time with episodes of frank haemoptysis. He had an associated fever, and left upper quadrant (LUQ) pain.\nBlood tests showed elevated inflammatory markers, suggesting an infection, thought to be of likely respiratory origin at this time. |
A 36-year-old woman at 19+4 weeks of gestation was referred to Xinjiang Medical University affiliated First Hospital for abnormal thyroid function in August 2017. She was tested for thyroid function a week ago because of excessive sweating. There was no history of fatiguability, weakness, proptosis or diplopia, palpitations, unusual bowel habits, or recent changes in weight. Upon physical examination: height 152 cm, body weight 60 kg, normal development, moderate skin temperature and elasticity, negative eye symptoms; a diffuse goiter with several small nodules, no tenderness, tremor or vascular murmur; heart rate 88 beats/min, no arrhythmia; no hands shaking or edema in lower extremities. Laboratory findings: thyroid function is listed in Table . Thyroid ultrasound showed a normal thyroid gland with some nonspecific nodules. The patient refused to undergo magnetic resonance imaging of the pituitary. The results of complete blood count, routine urinalysis, blood glucose, and renal function were all within normal ranges. She had no family history of thyroid disease. Past history showed that the patient had been diagnosed with hyperthyroidism for 2 years. Two years ago, she visited the hospital for lower abdominal pain and was diagnosed with hyperthyroidism after related inspections. Previous medical records showed abnormal thyroid function indicated by increased serum FT3 and FT4 levels, whereas TSH levels remained normal or mild elevated (Table ). The radioiodine131 uptake test was completed in October 2015, which was 4.1% at 3 hours and 20.5% at 24 hours. A thyroid ultrasound showed a normal thyroid gland with several nonspecific nodules. She was treated with methimazole orally once per day for 10 mg. The proband visited the hospital several times and the methimazole dosage had been repeatedly adjusted (10–30 mg/d). However, thyroid function was not restored and medication was discontinued by herself because of pregnancy. During hospitalization, she was diagnosed with THRS based on genetic analysis and no medication was given.\nThree generations of the pedigree are presented in Fig. . The family had no history of consanguineous marriage. Neither the proband's parents nor her sibling had any history or symptoms of thyroid dysfunction, but their thyroid function and genotype were not available. Her eldest son (III:1) and daughter (III:2), aged 18 and 16 years, respectively, did not have any symptoms of hyperthyroidism or hypothyroidism, but they were shorter than their peers. The eldest son had a height of 167 cm (approximately –1SD on standard growth charts) and the daughter had a height of 150 cm (approximately –2SD on standard growth charts), while both had low academic performance. Their thyroid functions suggested that serum TT3, FT3, TT4, and FT4 levels were increased, while TSH levels were mildly elevated. The proband's youngest son was 49 cm tall weighted 3.6 kg of an uneventful 40-week gestation and normal vaginal delivery. His thyroid function was normal. No abnormal signs on physical examination were found in these 3 children. The thyroid function results are shown in Table . During a 2-year follow-up period, 3 children had no complaints and their physical exams were normal. |
A 59-year-old female patient presented to a plastic surgery clinic with a lump on her right medial thigh. She noticed the lump eight months ago and it was gradually increasing in size. It was painless initially; however, the lump gradually became tender. The patient was otherwise fit and well with no co-existing morbidities. Closer examination of the right thigh revealed a high consistency mass, with reduced mobility. There was no right inguinal lymphadenopathy or lymph nodes enlargement.\nLaboratory blood tests were unremarkable. An MRI scan was requested to further assess the mass, which was irregular but well-defined in the medial compartment of the thigh, measuring 8.2 x 6.6 x 4.3 cm in size. The mass showed an intermediate signal on both T1 and T2-weighted imaging, appearing hyperintense compared to the adjacent muscular tissue, which also persisted on fat-suppressed imaging. The mass invaded the intramuscular fat planes and caused compression and displacement of the adductor muscles. There was no evidence of intra-muscular invasion. Normal cortical outline and medullary signal intensity of the femur were seen in the right thigh. There was no evidence of bone contusion, marrow oedema, fracture line, or cortical discontinuity. Other muscles of the thigh had a normal outline and signal intensity with no evidence of focal or diffuse oedema. Neurovascular structures were unremarkable. Overall, MRI findings were suggestive of soft tissue neoplasm, with a possibility of a neurogenic tumour. A wide excisional biopsy was then performed to further assess the tissue histologically by the pathologist and confirm the diagnosis. The removed mass measuring 8.5 x 6.5 x 5.4 cm and is shown in Figure . The mass had a smooth outer surface and a soft consistency. The cut surface showed a homogenous tan-white appearance with some slit-like spaces and occasional haemorrhagic spots. Multiple sections were processed for histopathological examination from different planes.\nHistopathology of the mass revealed a tumour arranged in sheets and fascicles composed of round to oval cells along with variably sized adipocytes (Figure ). Numerous interspersed lipoblasts with indented nuclei were also seen. Moreover, severe nuclear atypia was noted including bizarre cells (Figure and Figure ). An area of necrosis was seen with a few areas, which showed malignant and fibrous histiocytoma-like features. Furthermore, a few thin-walled dilated and congested blood vessels were also noted. Also, brisk mitosis was noted (35-38 per 10 HPF) (Figure ). Immunohistochemical staining (IHC) was also performed and the tumour cells were diffusely positive for vimentin and focally positive for S-100; while negative for creatine kinase (CK), smooth muscle antigen (SMA), desmin, CD34 and MyoD1. Overall, the pathological findings are all suggestive features of a high-grade sarcoma, favouring the epithelioid variant of PLS.\nThe patient was also referred to the Department of Nuclear Medicine & Molecular Imaging for post-surgical restaging and assessment for possible metastases using 18F-fluorodeoxyglucose positron emission tomography-computed tomography (18F-FDG PET-CT) scan. The abdominopelvic section showed the liver measuring within normal limits; however, the scan revealed an ill-defined hypermetabolic hypodense lesion seen involving segment VI measuring approximately 2.8 x 1.8 cm suggestive of hepatic metastases with concurrent fatty liver. The scan was also remarkable for the musculoskeletal system, revealing multiple hypermetabolic osseous lesions involving the skull base, right iliac bone, and few vertebrae (predominantly T7-L1); which was suggestive of skeletal metastases. A non-hypermetabolic pulmonary nodule in the right lung field was also detected; it was considered to be likely benign, but interval scanning is suggested to monitor further progress. Finally, there was also evidence of diffuse subtle hypermetabolism at the site of surgery which is suggestive of postsurgical inflammatory sequelae; however, microscopic residual disease cannot be completely ruled out. These multi-organ lesions were due to be biopsied to examine their immunohistochemical findings to confirm metastatic lesions of primary pleiomorphic liposarcoma. However, the patient did not attend the follow-up appointments. |
A 39-year old African American woman known to have hemoglobin (Hb) SC disease was admitted to the hospital with the diagnosis of VOC. The pain involved the left shoulder and the upper/lower back and was constant, sharp and throbbing in nature with an intensity score of 10 on a scale from 0 (no pain) to 10 (most severe pain). Past medical history was significant for VOCs at a rate of 2–3 VOCs per year with no pain between VOCs, avascular necrosis (AVN) of the left humeral joint, pneumonia, urinary tract infection, and a remote history of allergy to morphine. In the emergency department (ED), she was given meperidine 125 mg intravenously (IV) every two hours. She did not achieve adequate pain relief after receiving 3 doses of meperidine and, hence, was admitted to the hospital. The attending provider decided to start her on patient-controlled analgesia (PCA) pump using morphine lockout dose 1 mg, lockout interval 10 minutes, and a one-hour dose limit of 8 mg morphine. The patient indicated that she is allergic to morphine and usually receives meperidine for pain, but she could not give details about the nature of the allergy to morphine. About 8 hours after starting the PCA, she experienced hallucinations, disorientation, fever 103° F, chest oppression, and difficulty breathing with pulse Oximetry of 86%. She was transferred to the intensive care unit and intubated. The chest x-ray was normal. She recovered within 24 hours, and management of pain was resumed with meperidine and was discharged 7 days after admission.\nManagement of patients with sickle cell pain should be individualized. Patients with SCD are authorities on their disease. They know what helps them most. Accordingly, providers should listen, believe, and respect patients unless proven otherwise. The selection of a specific opioid and its dose should be based on the patient’s previous experience. No opioid or a specific dose of an opioid applies to all patients all the time. Opioids are ligands that bind to receptors and slow the transmission of painful stimuli along the central nervous system pathways. The binding to and activation of a specific receptor by an opioid vary considerably among patients. Opioid receptors are G protein-coupled with exogenous and endogenous opioids as ligands. Recent studies– have revealed a helical structure of the opioid receptors, which forms pockets in which the corresponding ligand (opioid) fits snugly. Not all opioids fit snugly into the same receptor’s pocket. This explains why some patients may have better analgesia with a certain opioid but not with another opioid. |
Our patient is a 72-year-old gentleman with a past medical history of paroxysmal atrial fibrillation, hypertension, and diabetes mellitus, who was recently diagnosed with interstitial lung disease. He was originally from Pakistan, immigrated to the United States in 2006 with notable travel history to the United Arab Emirates several years back. Two months prior to this admission, he was sent to the emergency room from an urgent care clinic when he was found to be in new-onset atrial fibrillation. Workup at that time included a chest X-ray which was abnormal, and hence a CT scan of the chest followed which showed interstitial pneumonia and a round soft tissue density in the right upper lobe surrounded by honeycombing reported as possibly a mycetoma with prominent mediastinal lymph nodes. An elective video-assisted thoracoscopic surgery (VATS) lung biopsy was done, and histopathology showed features consistent with usual interstitial pneumonia (Figures , ). He was tested for HIV and tuberculosis, which were negative. He followed with his pulmonologist and was started on nintedanib therapy as an outpatient.\nHe subsequently presented to our hospital with dyspnea on exertion that had gradually worsened over two weeks. He was noted to have a significant increase in oxygen requirement. He was hemodynamically stable and afebrile. Physical examination was significant for respiratory distress and diffuse crackles bilaterally. Initial labs were significant for hemoglobin of 11.9 g/dL with a hematocrit of 36.4%, a white blood cell count of 11,400 cells/mm3, and a CD4 cell count of 513 cells/mm3. Repeat CT scan of his chest without contrast showed findings consistent with interstitial lung disease, now including both microcystic and macrocystic honeycombing more pronounced in the lung bases. A rounded soft tissue density was noted within one of these macrocystic changes in the right upper lobe, measuring up to 1.8 cm (Figure ), slightly increased from prior where it measured up to 1.3 cm. Superimposed on chronic findings, there was evidence of new bibasilar airspace disease, left greater than right without evidence of pleural effusion or pneumothorax. Two of the fungal cultures sent from his VATS biopsy were growing Talaromyces sp. at this time. The patient was started on vancomycin, cefepime, azithromycin, and liposomal amphotericin while premedicating with normal saline and acetaminophen. However, after receiving the amphotericin, the patient developed a hypersensitivity reaction twice despite premedication and had to be switched to voriconazole. His respiratory status continued to worsen with high oxygen requirement necessitating non-invasive positive pressure ventilation, and subsequent intubation. He developed shock with multiorgan failure, and he was transitioned to comfort measures. |
Case 4: Retropharyngeal abscess
An 8-month-old boy was treated for an abscessed lymph node of the neck, which was incised. Staphylococcus aureus was isolated, and he received the appropriate antibiotic therapy, after which he was asymptomatic and discharged home. His parents returned a month later after noticing the boy's tachypnea (40-50/min) and stridor. The physical exam revealed diffuse rales from the upper respiratory tract, without an obstructive component. Inflammatory parameters were elevated -- (CRP 20 mg/L, ESR 48 mm/h, leukocytes 28 x 109/L), but without neutrophilia or a left shift. He was afebrile. There was no palpable lymphadenopathy. Generally, the boy was lively and had a good appetite. Chest radiograph and abdominal US did not show signs of an infection. When searching for a possible focus of the infection an US examination of the neck revealed a partly cystic partly solid change, with the size of 4.5 cm x 3 cm x 5 cm to the left of the trachea (Figure ).
After contrast application, the finding enhanced peripherally -- a 1 cm thick band of tissue became apparent (Figure ).
An MRI of the neck was performed and it confirmed a retropharyngeal abscess, which was spreading downwards along the prevertebral muscle (Figure ).
Due to poor access to the abscess for an incision (small child with a very short neck), the ENT physicians decided for a conservative approach with a prolonged seven-week antibiotic treatment. The therapy led to a complete regression of the abscess and resolution of the breathing problems. The changes regressed quickly and were no longer visible with US; thus, MRI was used for the subsequent following. Due to two severe infections in such a short period, the immunological tests were done but were negative.
In this case, the visualization of the neck was hindered because of the patient's constitution and short neck. Therefore, we were |
This 44-year-old Saudi female was not known to have any medical illness, presented to the emergency department with a history of epigastric pain three days earlier to admission. The pain was continuous, started suddenly, and was 9/10 in severity according to the patient with no diurnal variation. Pain radiated to the back, stretching in nature, aggravated by movement, and relieved by rest. The pain was associated with nausea and vomiting (gastric content). There was no history of change in bowel habits, urine, or cardiopulmonary symptoms. The patient was neither a non-smoker nor an alcoholic. The patient denied any history of abdominal trauma, scorpion bites, recent medication use, or previous similar complain. Family history was insignificant for autoimmune disease, inherited diseases, or similar complaints. The surgical history was clear. The patient was known to have morbid obesity, for which she underwent a minimally invasive procedure; an IGB was inserted three weeks ago. She lost 20 kg, from 130 kg to 110 kg. Eight months ago, the patient was COVID-19 positive, for which she was managed in the intensive care unit for three days.\nOn examination, the patient was hemodynamically and vitally stable. The abdominal examination revealed severe epigastric tenderness, normal bowel sounds, and soft lax consistency in other regions. No abnormalities were detected on systemic examination.\nOn investigation, laboratory, complete blood count showed leucocytosis, renal and liver function tests were within normal limits. Amylase was above the average level values 291 (see Table ). Imaging showed ultrasound was nil for gallbladder stones or common bile duct dilatation. CT scan of the abdomen showed acute interstitial edematous pancreatitis with compression of the pancreas by balloon. Furthermore, there was dislodgment of the catheter into the duodenum (Figure ).\nThe patient was treated conservatively by nothing per oral, intravenous fluids, antiemetics, and analgesia. The patient improved clinically and was discharged after three days of hospitalization without the removal of IGB. However, after two weeks of discharge, the patient presented with a similar picture in a more severe form, indicating the removal of IGB for improvement. |
A 38-year-old Afro-Caribbean female presented to the otolaryngology outpatient clinic with bleeding from an ulcerating left fungating parotid tumour. She first noted a pea-sized mass 5 years prior. A fine-needle aspiration cytology of the mass at that time showed ACC. The patient refused surgery and was lost to follow-up. The tumour slowly increased in size but within the last year underwent rapid growth.\nShe appeared malnourished and focused examination revealed a 14 by 12 cm fungating parotid mass with areas of necrosis and haemorrhage []. The motor function of the facial nerve could not be assessed properly due to the mass effect of the tumour.\nOn admission, her hemoglobin was 9.3 g/L and albumin level <10 g/L. Other blood tests including electrolytes, liver function tests, and coagulation profile were normal.\nThe patient was admitted for nutritional support, hemostasis, and further management of her parotid malignancy.\nOn the second day of admission, she developed acute confusion and became agitated. Computed tomography of the brain surprisingly showed a thrombosis of the contralateral transverse sinus extending into the proximal part of the internal jugular vein []. A Doppler ultrasound scan of the lower limbs revealed thrombosis of the distal part of the right common femoral vein. Following the advice from neurologists and hematologists, the patient was started on warfarin for anticoagulation. An inferior vena caval filter was also inserted to prevent thromboembolism from the femoral vein thrombosis.\nOn the 12th day of admission, the International Normalized Ratio (INR) value went up to 8.0, and the patient bled from the tumour site. Warfarin was stopped, and the patient was given Vitamin K and fresh-frozen plasma. Within the next 3 days, the INR came down to 2.0. Due to difficulty maintaining INR values within the therapeutic range, warfarin was switched to low-molecular-weight heparin (LMWH).\nOn the 28th day of admission, 8 days after starting heparin, the platelet count dropped from 500 to 15 × 109/L. The serum was positive for platelet factor-4 antibodies and a diagnosis of Type II heparin-induced thrombocytopenia (HIT) was made. The heparin was therefore switched to lepirudin, a direct thrombin inhibitor. The platelets returned to the normal level after 5 days of stopping heparin.\nThree days later, the patient's condition deteriorated rapidly due to septicemia. She was transferred to the intensive care unit and started on broad-spectrum antibiotics. The patient's nutritional status got worse since her hospital admission as she was not having adequate per oral intake and refused nasogastric feeding.\nBecause of the patient's worsening prognosis despite maximal medical management, the multidisciplinary team recommended the surgery as a possible solution in addressing the primary issue, the tumour.\nShe underwent a radical left-sided parotidectomy and type-1 modified radical neck dissection, as the carcinoma was staged as T4, which has a high risk of having lymph node metastasis. Moreover, the neck dissection was necessary for facilitation of the latissimus dorsi pedicled muscle flap reconstruction. The facial nerve was sacrificed as the tumour engulfed all branches of the nerve, but the accessory nerve and internal jugular vein were preserved. A part of the zygomatic arch was also removed as the tumour involved it. Intraoperatively, the tumour was found herniating into the oral cavity through the parotid duct opening (Stensen's duct). The oral mucosa around the tumour was excised and closed primarily. A latissimus dorsi pedicled muscle flap was raised to reconstruct the facial defect and a split skin graft was used to cover the muscle flap.\nThe histology confirmed moderately differentiated ACC of the parotid with clear margins but noted vascular invasion. None of the 61 lymph nodes harvested were positive for malignancy. The disease was staged as pT4pN0M0.\nThe postoperative complications included hematoma formation at the latissimus dorsi flap donor site, which was drained. The patient also developed an episode of septicemia due to an infected central venous catheter, which was treated with antibiotics. Part of the split skin graft had to be re-grafted due to a local wound infection.\nAfter being an inpatient for 65 days, she was discharged from the hospital with a normalized serum albumin level and underwent adjuvant postoperative radiotherapy. There were no signs of any local or regional disease after her treatment []. Unfortunately, she died 19 months after the surgery due to lung metastasis. |
A 31-year-old woman, gravida 2 para 1, presented for a prenatal ultrasonographic examination at 36 gestational weeks owing to a suspicion of a fetal thoracic wall defect. Her personal history revealed a spontaneous abortion and no consanguinity. She underwent routine ultrasonographic examinations at 13, 22, and 30 gestational weeks at a regional hospital; however, at 35 gestational weeks, ultrasonography revealed an abnormal fetal thoracic wall.\nPrenatal ultrasonography revealed a fetal thoracoabdominal wall defect with partial displacement of the left ventricle and the liver associated with rotation and elongation of the heart and a high index of clinical suspicion for intracardiac malformations such as tricuspid atresia, a ventricular septal defect, and pulmonary artery hypoplasia (Figures –).\nBased on the aforementioned findings, she was admitted to the Obstetrics and Gynecology Clinic in Târgu Mure at 39 gestational weeks, where she underwent a cesarean section. The male newborn weighed 3,100 g with an APGAR score of 7. Clinically, he demonstrated a superior abdominal wall defect, a partial extrathoracic displacement of the heart, and a partially herniated liver (these structures being covered by a very thin skin layer), and also a diastasis of the sagittal suture (Figure ). The newborn was intubated, and we applied a saline-soaked gauze pad on the thoracoabdominal and cranial defects to maintain humidity.\nPostnatal echocardiography confirmed the prenatal diagnosis and also showed a partial extrathoracic and extra-abdominal displacement of the heart and liver, a large ventricular septal defect, great arteries originating from the left ventricle with the aorta situated anteriorly, a posterior deviation of the outlet septum causing severe subpulmonary stenosis, hypoplasia of the pulmonary artery, and a large hourglass-shaped left ventricle secondary to narrowing of the heart at the level of its extrathoracic displacement.\nWe also performed thoracoabdominal CT-angiography, which showed complex cardiac malformations consisting of large ventricular and atrial septal defects, an increased left ventricular volume, with apical extrathoracic aneurysmal dilatation below the xiphoid process at the level of the abdominal midline, hypoplasia of the right ventricle, and a reduced caliber of the pulmonary trunk artery. Abdominal CT revealed partial transparietal herniation of the left hepatic lobe adjacent to a left ventricular diverticulum, and an increase in the size of the right adrenal gland with hyperdense contents suggesting an adrenal hematoma. Cranial CT revealed a diastasis of the sagittal suture causing subcutaneous herniation of the venous sagittal sinus.\nFollowing admission to the Neonatal Intensive Care Unit, the newborn was administered ampicillin and amikacin, fluconazole, prostaglandin E, and phenobarbital (because he presented with multiple seizures), and also received daily dressing changes. During the first week of life, he showed multiple episodes of bradycardia and low oxygen saturation despite undergoing orotracheal intubation; therefore, surgical intervention was postponed until he was hemodynamically stable. He underwent surgical intervention at 14 days of age, consisting in the replacement of the heart inside the thorax via a systemico-pulmonary shunt procedure, with vascular prosthesis, the ligature of both persistent arterial canal and pulmonary artery trunk, and repair of the diaphragm defect. The abdominal wall defect was also sutured, but the thorax remained open. The surgical procedure was performed in extracorporeal circulation, and lasted 4 h and 15 min.\nPostoperatively, the newborn developed multiple episodes of tachyarrhythmia and low cardiac output suggesting an inability of the heart to adjust to the intrathoracic pressure. Unfortunately, the newborn died 5 h postoperatively secondary to progressive hemodynamic deterioration, metabolic acidosis, and hypoxia. |
A 47-year-old Hispanic male presented to our emergency department with severe neck pain. He has had intermittent episodes of neck pain with radiation down his spine and lower extremities for the past year. It was described as a sharp and sometimes cramping, 8–10/10 in intensity, intermittent and was triggered by activity like lifting heavy objects. Two weeks prior to admission he was evaluated at a local clinic for the same complaints, treated with ibuprofen and referred to our rheumatology clinic where a workup was in progress.\nHis pain became more intense prompting him to seek medical attention and was admitted to our hospital for evaluation. He noted that he had been more fatigued lately with generalized malaise. He had difficulty climbing stairs and moving from a sitting to a standing position but was able to eat with a fork/spoon and comb his hair without any difficulty. He reported that his fingers turned blue on exposure to cold weather. He occasionally had joint pains with swelling and redness of multiple joints, associated with morning stiffness lasting at least 30 min. He also reported noticing frothy urine for the past year.\nHis past medical history was significant for hypothyroidism and hypertension. His medications consisted of lisinopril, aspirin and levothyroxine. He had no allergies. He denied any family history of thyroid, autoimmune or rheumatological diseases. He was a landscaper by profession. He denied smoking cigarettes, alcohol use or illicit drug use.\nOn examination, he was a young Hispanic male in some distress from the neck pain. He had unremarkable vital signs with a BMI of 25. His physical examination was normal. X-rays of his chest and spine were normal. His blood work revealed anemia with hemoglobin of 11 g/dl, marked hypoalbuminemia of 1.7 g/dl and a total protein of 3.6 mg/dl. He had a blood urea nitrogen of 33 mg/dl and serum creatinine of 1.0 mg/dl. His triglycerides were elevated at 265 mg/dl, cholesterol of 209 mg/dl and a calculated LDL of 132 mg/dl. His previous blood work done a few days ago at our rheumatology clinic showed an elevated erythrocyte sedimentation rate of 53 mm/h, TSH 10.6 and free T4 1.27 ng/ml. A urinalysis showed 1+ blood with 9 RBC, hyaline and granular casts but no RBC casts, protein of greater than 600 mg/dl with a urine protein-creatinine ratio of 4 grams/gram of creatinine. HIV, hepatitis B and C serologies were negative. Antinuclear antibody was positive with a titer of 1:640 and a peripheral pattern.\nA nephrologist and a rheumatologist were consulted. Given normal renal function, microscopic hematuria and massive proteinuria, membranous nephropathy secondary to SLE was suspected. Further workup revealed a low C3 of 76 mg/dl, a normal C4 of 36 mg/dl and the anti-dsDNA titer was positive at 29 IU/ml. Renal biopsy was done and it revealed immune-complex-mediated diffuse segmental proliferative and membranous nephritis with features of focal cellular and fibrocellular crescents (8–10%). The segmental active glomerular lesions were in approximately 79–83% of sampled glomeruli, which were characterized by segmental endocapillary hypercellularity, hyaline ‘pseudothrombi’ (intraluminal immune aggregates), duplication of glomerular basement membrane (GBM) and, cellular and fibrocellular crescent. There was no significant tubulointerstitial chronic injury. The biopsy was diagnostic for lupus nephritis class IV-S (A/C) and V by ISN/RPS 2004 classification (). He was started on prednisone, hydroxychloroquine and mycophenolate mofetil. Four months after initiating therapy he started to show improvement in his proteinuria. He continues to have a normal renal function (). |
A 35-year-old Chinese male patient with a 1-year history of left arm swelling from unknown causes was investigated in February 2010. Written informed patient consent was obtained from the patient. Histological investigation on multiple biopsies revealed tumor tissue composed of nodular proliferation that was predominantly eosinophilic, with a few spindle cells and large areas of central necrosis and hemorrhage. The epithelial cells exhibited abundant eosinophilic cytoplasm with large vesicular nuclei and prominent cell nuclei. No vascular invasion was observed and the patient had a multifocal disease that extended along the span of the left arm in addition to the disease in the left axillaries, left clavicular and right upper planar region. Immunohistochemical analysis revealed positivity for cytokeratin (CK), EMA, vimentin and CD34, but was negative for S100, desmin, smooth muscle actin (SMA), melanin, CD68 and HMB45. The results were consistent between the left forearm and left arm epithelioid sarcoma. The patient sought advice from the sarcoma team at the Peter MacCallum Cancer Institute (East Melbourne, Australia), who advised that the patient undergo neoadjuvant chemotherapy with ifosfamide and Adriamycin. After 6 cycles, positron emission tomography scan images showed a partial response in the soft tissue density of the left upper extremity and axilla. Computed tomography scanning showed a reduction in the size of the planar base mass. After much deliberation, the patient opted to undergo autologous immune enhancement therapy (AIET).\nAs a therapeutic alternative, the patient received 7 infusions of AIET. The patient initially underwent an induction phase, receiving 6 harvestings of peripheral blood every 2 weeks. After a 5 month follow-up, the patient volunteered for consecutive AIET infusions. Peripheral blood (60 ml) was collected from the patient each time and peripheral blood mononuclear cells (PBMCs) were isolated from the blood as per the protocol described by Terunuma et al(). Isolated PBMCs were seeded onto anti-CD3 and anti-CD16-coated flasks and incubated overnight at 39°C with 5% CO2. The cultures were then incubated at 37°C and maintained for 14–16 days with routine media nourishment using the patient’s own plasma separated from the peripheral blood. Interleukin-2 was added as a supplement during the NK and T cell seeding. After an optimal expansion to the desired cell population, cells were harvested by centrifugation and washed three times using phosphate-buffered saline. The retrieved cell number was measured using the Trypan blue dye exclusion test and the cells were then re-suspended in 100–200 ml of normal saline with human albumin for intravenous infusion. |
A 56 years-old female was referred to our clinic because of a 6-months history of intermittent dysesthesia and numbness in the anterior part of the thigh. She reported increasing pain during walking, but it was present even at rest. Over the weeks, the walking distance became progressively reduced. There was no history of rheumatoid arthritis, metabolic or inflammatory disease, preceding pelvic trauma, or coagulation disorder. Laboratory investigations including red and white blood count with differential count, erythrocyte sedimentation rate, C-reactive protein, rheumatoid factor, liver and renal function tests, and tumours markers (CEA, CA19-9) were normal. On examination, the range of movement of the hip was complete and a tender, nonpulsatile mass was palpable in the proximal third of the right thigh. Conventional radiographs of the hip showed minor findings of degenerative osteoarthritis of the right hip. An abdominal ultrasonography was also performed because she had lower abdominal pain finding no abdominal abnormalities in the major organs (uterus, ovary, urinary bladder, kidney, liver, gallbladder, spleen, and pancreas). An MRI scan confirmed the presence of a cystic lesion very close to the femoral nerve that appears dislocated and compressed as well as femoral vein and artery. The cyst seems to be connected and originate from the hip joint. However, a ColorDoppler examination revealed no pathologic compression or blood-flow reduction of femoral vessels and the pulses in the distal leg were normal. The patient underwent ultrasound-guided aspiration of the cyst and injection of triamcinolone and bupivacaine. We confirmed a progressive reduction in size and a replacement of the femoral vessels and nerve in anatomic position. Up to 40 ml of fluid have been removed and analyzed. The examination of the fluid was unremarkable. After 6 months, there was no evidence of significant fluid re-accumulation with complete resolution of the symptoms. The patient was pain-free and able to return at her normal lifestyle. |
A 63-year-old female with no past medical or surgical history presented to the clinic with complaints of progressively worsening weakness and pain in the upper extremities bilaterally. MRI of the cervical spine demonstrated multilevel cervical degenerative disc disease with reversal of normal lordosis and a kyphotic deformity in addition to varying levels of moderate to severe central canal stenosis from C4-T1 and moderate to severe bilateral foraminal stenosis at almost every level, especially on the right side (Figure ).\nWe discussed the various conservative and surgical options with the patient, and it was determined that surgery would provide the best chance to stop the progression of her neurological symptoms. Due to her cervical kyphosis, we opted for an anterior approach to restore vertebral column height and lordotic curvature. The anterior fusion would supplement with posterior instrumentation given the number of levels requiring fusion.\nDescription of the surgery\nThe left-sided approach was chosen to theoretically decrease the risk of recurrent laryngeal nerve injury. A transverse incision was created extending from medical sternocleidomastoid to midline in existing neck crease, planned using fluoroscopic localization overlying the C5-6 interspace. Standard neck dissection was performed. Omohyoid muscle was divided sharply (and reapproximated at the end of the case). Discectomy and graft placement proceeded superiorly to inferiorly with Caspar pin retractors for retraction two levels at a time, C3-5, C5-7, then C7-T1. The posterior osteophyte complexes were removed to decompress the spinal cord and exiting nerve roots. Six-degree lordotic cages were used at each level to restore natural cervical lordosis. Following the anterior portion of the case, the patient was turned prone on a Jackson frame, and her head was immobilized in a neutral position with a Mayfield head holder. Subsequently, posterolateral screw and rod instrumentation in a standard fashion was placed utilizing stereotactic navigation with lateral mass screws from C3-6 and pedicle screws at T1 (Figure ).\nThe patient tolerated the procedure without complication, was discharged to the rehabilitation center on day three. She followed up at nine weeks, at which time a CT scan of the cervical spine was performed showing good bony fusion (Figure ), and her hard cervical collar was discontinued. At nine weeks post-surgery, her symptoms have significantly improved. |
A girl aged 13 years reported to the out-patient clinic, Department of Pedodontics and Preventive Dentistry, with the complaint of pain and swelling in her upper left front tooth. She gave a history of a traumatic injury, about 1 year prior to the time of reporting. She mentioned that she had consulted a dentist after having a traumatic episode, and she was told that she needed to undergo a root canal treatment after the closure of the root end. She however, failed to report back to the dentist and did not pursue the treatment until the tooth begun to pain. She had a noncontributory medical history. She expressed her concerns that she will not be able to pursue treatment for multiple appointments. Examination revealed a sinus tract in the labial vestibule of the maxillary left central incisor. Radiographic examination revealed radiolucency in the periradicular area of the upper left central incisor []. Clinical and radiological examination indicated pulp necrosis with acute exacerbation of chronic apical abscess in relation to the upper left central incisor. The patient was given a detailed explanation concerning the treatment and prognosis. Considering her inability to make frequent appointments, apexification with MTA was considered after the disinfection of the periradicular area.\nIn the first appointment, the already existing access cavity was refined with a safe-end tapered bur (Endo-Z; Dentsply/Maillefer, Ballaigues, Switzerland) under rubber dam isolation. The root canal was irrigated with a 5.25% sodium hypochlorite solution and the final irrigation was done with a normal saline solution []. After drying, the root canal was filled with a mixture of calcium hydroxide powder and saline. The access cavity was sealed with IRM, analgesics and antibiotics were prescribed, and the patient was recalled in 1 week []. One week later, the access cavity was reopened and the canal was thoroughly irrigated with the 5.25% sodium hypochlorite solution and the canal was refilled with calcium hydroxide which was changed once within the interval of 1 week. At the third appointment, 1 week later, the sinus tract had resolved and the tooth was asymptomatic. Biomechanical preparation of the root canal was done using standard hand instruments (K-files and H-files) and the canal was enlarged up to a size of instrument number 80. All appointments were carried under rubber dam isolation. The canal was dried with paper points and MTA was dispensed and mixed according to the manufacturer's instructions. The canal was filled up to half its length with MTA and condensed with hand plugger and back-end of a paper point. The thickness of the MTA plug was almost twice that of the recommended 3–4 mm as the tooth was compromised with acute exacerbation. A moist cotton pellet was inserted into the canal to aid in setting and was left undisturbed for 15 min according to the manufacturer's instructions [].\nLateral compaction technique was used to obturate the canal with gutta-percha and zinc oxide eugenol sealer. The access cavity was restored with glass ionomer cement []. A follow-up radiograph after 4 months showed that the lesion had begun to resolve []. The patient was given a composite resin crown to show improved esthetics which will be replaced by a ceramic crown later. The preoperative and postoperative appraisal is evident from the clinical picture []. |
A 44-year-old man consulted an internist due to abnormalities on an upper gastrointestinal series. He had no relevant medical history. On examination, he appeared comfortable, with a temperature of 36.9 °C, blood pressure 152/97 mm Hg, and pulse 79 beats/min. The abdomen was soft, without tenderness or distension. The remainder of the examination was unremarkable. The laboratory data on admission showed a slightly decreased hemoglobin level of 13.3 g/dL, but the tumor marker levels (CEA, CA19-9) were almost within the normal limits. Upper gastrointestinal endoscopy showed an elevated lesion with central depression, bridging hold, and no abnormalities of the gastric mucosa in the greater curvature of the middle part of the stomach (Fig. ). Endoscopic ultrasonography showed that the well-demarcated, heterogeneous echo-poor mass originated from the muscle layer of the stomach (Fig. ). An upper gastrointestinal series showed an elevated lesion with a central depression in the greater curvature of the middle part of the stomach (Fig. ). Computed tomography showed a 22-mm solid mass with a smooth margin and hypervascularity in the gastric wall at the upper part of the stomach. Integrated PET/CT showed an intense tracer uptake by the tumor (SUVmax = 6.8) (Fig. ). Based on these findings, a gastrointestinal stromal tumor was suspected. Although the size of the tumor measured less than 5 cm and did not have clear malignant views, we judged this case to be a candidate for surgery in consideration of an intense tracer uptake by PET/CT. Laparoscopic endoscopic cooperative surgery was performed. First, the tumor location was confirmed at the greater curvature of the upper part of the stomach, after which the blood vessels in the excision area around the tumor were minimally ligated using an ultrasonically activated device. Next, endoscopic submucosal resection using endoscopic submucosal dissection (ESD) was performed around the tumor, and the seromuscular layer was intentionally perforated after three-quarters of the circumference of the excision had been finished. The tip of an ultrasonically activated device was then inserted into the perforation, and seromuscular dissection around the tumor was performed. After the tumor had been resected and removed, the incision line was closed using laparoscopic stapling devices. The operative time was 119 min, and blood loss was 5 mL.\nThe resected specimen showed a tumor measuring 20 × 18 mm in diameter, and its divided face was solid and poorly marginated. A histopathological examination showed lymphoplasmacytic infiltration and fibrosis (Fig. ), and an immunohistochemical analysis showed the infiltration of IgG4-positive lymphoplasmacytic cells (Fig. ). The patient was diagnosed with probable IgG4-related sclerosing disease of the stomach.\nThe patient made an uneventful recovery and was discharged on postoperative day 7. He was examined in a clinic 7 weeks after surgery and was found to have made a full recovery. Subsequent blood tests revealed that his serum IgG4 level was 98.1 mg/dL (reference range 4.8–105 mg/dL); however, the levels had not been examined prior to resection as an IgG4-related sclerosing disease had not been suspected.\nThe gastric manifestations of IgG4-related sclerosing disease are rare, and PET/CT is a useful imaging technique for their diagnosis and follow-up. The organ that is most commonly involved in IgG4-related sclerosing disease is the pancreas; such involvement is termed autoimmune pancreatitis (AIP) []. AIP is a specific type of pancreatitis that is thought to have an autoimmune etiology, and which typically shows the infiltration of IgG4-positive plasma cells and increased levels of serum IgG4. IgG4-positive plasma cells may involve not only the pancreas but also other organs, including the bile duct, gallbladder, salivary gland, thyroid gland, lungs, gastrointestinal tract, liver, retroperitoneum, kidney, prostate, and lymph nodes []. In 2003, Kamisawa et al. proposed a new clinicopathological entity, IgG4-related sclerosing disease, and suggested that AIP is a pancreatic lesion that reflects this systemic disease [].\nA diagnosis of IgG4-related disease is definitive in patients with the following findings: (1) organ enlargement, mass or nodular lesions, or organ dysfunction; (2) a serum IgG4 concentration of >135 mg/dL; and (3) histopathological findings of >10 IgG4 cells/HPF and an IgG4+/IgG+ cell ratio of >40 %. A diagnosis of IgG4-related disease is possible in patients who fulfill criteria (1) and (2), but with negative results on histopathology or without a histopathologic examination, whereas a diagnosis of IgG4-related disease is probable in patients with organ involvement (1) and in whom the histopathologic criteria are fulfilled, without an increase in serum IgG4 concentration (2) []. Our patient fulfilled criteria (1) and (3); thus, he was diagnosed with probable IgG4-related sclerosing disease. Although the patient’s serum IgG4 levels were normal after the resection of the submucosal tumor, they could have been elevated prior to surgery.\nThere are few reports of IgG4-related sclerosing disease presenting as a gastric lesion. Rollins et al. reported a gastric midbody mass which was histologically characterized as an IgG4-related autoimmune fibrosclerosing pseudotumor []. Fujita et al. reported an IgG4-related gastric ulcer without the main manifestation of autoimmune pancreatitis []. Only one case of IgG4-related gastric submucosal tumor was described by Murakawa et al. [], in a patient who underwent laparoscopic endoscopic cooperative surgery. Thus, a gastric submucosal tumor of IgG4-related sclerosing disease is a rare manifestation.\nSome reports have shown the usefulness of PET/CT imaging in IgG4-related sclerosing disease [, ]. PET/CT detects the various sclerosing lesions of the whole body; it is therefore effective for the diagnosis and follow-up of IgG4-related sclerosing disease. This disease is characterized by frequent temporal and spatial recurrence and requires a long-term follow-up. A continuous whole body search by PET/CT is therefore useful for IgG4-related sclerosing disease patients.\nAs corticosteroid therapy is effective in IgG4-related sclerosing disease, it may be considered the first treatment option. However, a therapeutic diagnosis with corticosteroid dosage is not recommended when differentiation with a malignant tumor is difficult []. Therefore, low invasive surgery such as LECS may be an effective therapy option for patients who are diagnosed with IgG4-related sclerosing disease presenting as a gastric submucosal tumor, such as the present case, in consideration of the side effects of corticosteroid dosages.\nIgG4-related sclerosing disease is a relatively new disease concept, and its prognosis has not been well described. Additional studies of its long-term prognosis are needed. |
We present the case of a 65-year-old male, a known case of diabetes mellitus, who presented to the outpatient department with generalized weakness, low mood, and lack of interest in daily activities for the last 6 to 8 months. Prior to the onset of his symptoms, he had been working abroad for the last 40 years. He was considered a model employee and was trusted by his superiors in decision-making and financial matters. However, in the 6 months prior to his admission, he had been cheated out of his life savings and had also lost his home. He had been dismissed from his job as well for poor performance and incorrect accounting. At that time, he had been diagnosed as suffering from depressive disorder and had been prescribed anti-depressants, with minimal improvement in symptoms.\nOn presentation to our hospital, he was vitally stable. His general physical examination yielded no significant findings. In his neurological examination, he was conscious and oriented. His Mini-Mental State Examination score was 25/30 with impaired registration and recall. Examination of his motor, sensory, and cerebellar systems was unremarkable. Mental state examination yielded poor eye contact, low mood, and ideas of worthlessness. No abnormal beliefs or perceptions were elicited.\nAll routine laboratory investigations were normal. His random blood sugar was 140 mg/dL, fasting blood sugar was 98 mg/dL, and glycated hemoglobin (HbA1C) was 6.0%. His fasting lipid profile, serum vitamin B12, serum folate levels, and thyroid function tests were also normal. Screening for viral hepatitis and human immunodeficiency virus (HIV) was negative. A computed tomography (CT) scan of the brain was performed that showed ischemic changes. The most striking finding was an old left frontal infarct with a compensatory dilation of the anterior horn of the left lateral ventricle (Figure ).\nHe was treated with aspirin, statins, cognitive behavioral therapy, and sertraline, a selective serotonin re-uptake inhibitor (SSRI). His mood and cognition improved. His recollection and recent memory, which had previously been impaired, also showed mild improvement. He began to take interest in self-care and hygiene and also started to participate in community activities. He was, however, still unable to perform complex calculations like he had done previously as part of his job.\nHe has been called for follow-up after 6 months, with a plan to repeat imaging of the brain. |
A 57-year-old man took part in a research trial at the Clinical Research and Imaging Centre, Bristol, United Kingdom. The man was screened for pre-existing conditions including musculoskeletal pain, cardiovascular, respiratory, psychiatric, immunological, oncological, urogenital, gastrointestinal, endocrine, neurological, rheumatologic and ophthalmic disorders. Importantly, the medical history was only taken with respect to on-going diagnoses with the exception of oncological disorders. In addition, a 12-lead electrocardiogram (ECG), clinic and ambulatory blood pressure and full-screen sensory testing on the forearm was performed for research purposes, which were all confirmed to be within the normal range.\nDuring the research MRI scan, a T1-weighted magnetization-prepared rapid gradient echo (T1-MPRAGE) of the whole head was obtained (Figure ). This showed confluent low intensity in the right parietal white matter containing a focal cystic change. The preliminary neuroradiology report noted generally reduced volume compared to that expected for the patient’s age.\nA less prominent hypointensity was observed in the left hemisphere (Figure ).\nIn addition to the main focal lesion in the parietal white matter, at least three smaller focal lesions were seen in the frontal lobe white matter, two in the right hemisphere and one at a similar lateral and frontal position in the left hemisphere (Figure ).\nBased on a preliminary report of the T1-MPRAGE, it was recommended that the patient be followed up in the Stroke Clinic as the suspected lesion was thought to represent small vessel ischaemia possibly with lacunar infarct.\nThe patient was invited for a clinical MR scan through a stroke clinic in the South of England. This scan showed bilateral susceptibility artefact lateral to the third ventricle, bilateral signal change adjacent the posterior horn of the lateral ventricles, prominent lateral ventricles, and linear signal voids extending to the cortical surface, with overlying cortical defects. The clinician noted that this was likely the site of previous craniotomy or intervention.\nThe patient was followed up by consultation, which revealed that he had suffered tubercular ventriculitis at age 16 and had been admitted to the Midland Centre for Neurosurgery and Neurology. At the time, pneumoencephalography was performed to assess the patient, which resulted in a coma lasting approximately one week, after which the patient recovered with anti-TB treatment. |
A 51-year-old woman underwent hysterectomy and pelvic lymph node dissection for uterine cancer when she was 48 years old, and lymphedema developed in the left leg soon after the operation. She had one episode of cellulitis. Despite wearing elastic stockings, lymphedema worsened, and she visited our institution at the age of 49 years. She was diagnosed with lymphedema based on lymphoscintigraphic finding. There was a development of collateral lymphatic vessels and dermal backflow in bilateral lower leg (See figure, Supplemental Digital Content 1, which displays lymphoscintigraphic findings. Collateral lymphatic vessels were observed in the bilateral lower legs. Lymphatic function in the left thigh was impaired, ). She had no allergies or other pertinent medical histories.\nLVA was performed at the age of 50 years. The postoperative course was uneventful, and lymphedema improved. However, lymphedema worsened again at 1 year postoperatively after taking a long flight, although she wears elastic stockings daily. A second LVA was planned (Fig. ).\nPreoperative indocyanine green (ICG) lymphography showed a linear pattern in the right leg. Dermal backflow was observed in the left thigh and lower leg. There was no linear pattern in the area (left lower leg) where the lymphatic thrombus was found afterward. Preoperative echography showed 2 hypoechoic circles measuring about 0.5 mm in diameter that did not collapse with pressure from the probe, although the veins collapsed with pressure (Fig. ). Compared with lymphatic vessels, veins usually collapse more easily under pressure, because the inner pressure of the lymphatic vessels is higher than that of the veins. In this case, the 2 circles did not collapse under pressure, and we surmised that the inner pressure prevented collapse. The hypoechoic circles extended proximal and distally and did not have flow with on color Doppler mode.\nDuring LVA, we identified 2 parallel white vessels beneath the superficial fascia. Two vessels were in close contact. We diagnosed these as lymphatic vessels because of the location and appearance and the fact that they ran in parallel, which is not usually observed with other vessels or nerves. When we incised the vessels, white material was extruded (Fig. ). A diagnosis of lymphatic thrombosis was made, and we concluded that the vessels did not collapse with pressure from the probe during echography because of thrombus. Intraoperative echography revealed the same findings, that is, a hypoplastic circle without collapse by probe pressure, 15 cm distal from the incision, which indicated that there was lymphatic thrombus in at least 15 cm. We ligated the lymphatic vessels, closed the wound at this site, and performed LVA at other sites (4 sites in the left and 1 site in the right leg). Though the postoperative course was uneventful, the patient’s lymphedema did not improve postoperatively. This may be partially because the patient gained weight after LVA, and there is another possibility that postoperative thrombus formed within the anastomosis site had harmful effect for lymphedema, although it is difficult to confirm.\nHistopathological examination showed a thickened smooth muscle layer (tunica media) in the lymphatic vessels (See figure, Supplemental Digital Content 2, which displays histopathological findings of the lymphatic vessel and the lymphatic thrombus. (a) The lymphatic vessel (a) and the lymphatic thrombus (b) (×20, H&E). Thickened smooth muscle layer (tunica media) in the lymphatic vessels is observed. (c) The lymphatic vessel (×100, D2-40). The endothelial cells of the vessel were negative for D2-40, ). Fibrous thickening of the tunica intima was observed, and the inner lumen was narrow. The inner layer of the vessel was negative for D2-40, which is a marker to stain the lymphatic endothelial cells. In the thrombus, hyperplasty of fibroblasts and organization were found (Fig. ). We did not observe hyperplasty of the lymphatic endothelial cells, which are positive for D2-40 within the thrombus. |
A 26-year-old female patient was transferred to the Samsung Medical Center from an outside institution due to severe refractory cardiogenic shock. The patient was diagnosed with non-Hodgkin’s lymphoma 2 years prior and underwent 6 cycles of chemotherapy, which included doxorubicin. After chemotherapy treatments were completed, the patient suffered from orthopnea and dyspnea, and an echocardiography revealed a severely depressed left ventricular (LV) ejection fraction (25%) and dilated LV. Due to complications from cancer treatments, she had an episode of acute decompensated heart failure and was referred to Samsung Medical Center.\nOn initial presentation, vital signs included arterial blood pressure of 79/27 mmHg, heart rate of 133 beats/min, respiratory rate of 28 breaths/min, and body temperature of 35.9°C. She had pulmonary edema with bilateral pulmonary congestion () and multiorgan failure with liver and kidney involvement. Despite the use of inotropes and vasopressors, we were unable to achieve stable vital signs.\nPercutaneous extracorporeal life support (P-ECLS) was then placed using the left femoral artery and vein. However, the left leg became ischemic after several hours, and we decided to shift the cannulas to the right groin. The patient was brought to a hybrid operating room, and the groin was opened bilaterally. First, we removed the left femoral arterial cannula and performed a thrombectomy. Then, the arterial and venous cannulas were inserted through the common femoral artery and vein, and the catheter for the superficial femoral artery was inserted at the same time.\nThe following day, transthoracic echocardiography revealed a distended left ventricle, and the chest X-ray showed worsening pulmonary edema. In this case, we determined that an atrial septostomy was not a viable option since both the femoral veins were already cannulated or had been surgically repaired. Instead, we performed an emergency standard median sternotomy and inserted a vent catheter (malleable 20 Fr.) with a tip-cut into the right upper pulmonary vein and passed it into the left ventricle. Then, the inserted vent catheter was connected to the venous line of the P-ECLS circuit via a Y-shaped connector (). A few hours later after the insertion of the vent catheter, the pump flow was maintained at 2.4 L/min/m2, obtaining a mean systemic pressure of 60 mmHg and central venous pressure of 8 mmHg. The flow through the vent catheter was measured to be 1 to 2 L/min.\nThe patient was on P-ECLS and left heart venting for 5 days. During this time, the pulmonary edema was resolved (). Transthoracic echocardiography revealed improved right and LV function and decreased chamber size. The LV ejection fraction was 30% to 40%, when the pump was off. When the function of the right heart and the lung improved, the drainage from the femoral vein was gradually reduced by progressively clamping the venous line and lowering the r.p.m. In this way, the system was modified from P-ECLS to paracorporeal LV assist device (LVAD) by the complete clamping of the femoral venous drainage catheter. Thus, the femoral venous cannula and the oxygenator were removed on postoperative day 5. The reasons for our decision were complete LV support, possible longer-term support due to the absence of the oxygenator and the low level of anticoagulation, and the prevention of recurrent LV distension.\nOn postoperative day 8, we were able to wean the patient from LVAD and remove all cannulas from her chest and groin. She was extubated and was finally discharged home on hospital day 28. During follow-up, the LV ejection fraction worsened from 40% to 20%, and the symptoms of dyspnea became worse than before hospital discharge. Currently, this patient is on the heart transplant list and is waiting for transplantation. |
A 36-year-old man with no specific past illnesses was referred for evaluation of his tachycardia and dyspnea for a week. After admission, physical examination revealed grade 2/6 systolic murmur at the left sternal borders and decreased breathing sounds with both basal rales. The initial electrocardiogram (ECG) revealed wide QRS tachycardia (), and the chest X-rays showed marked cardiomegaly with pulmonary congestion. A routine blood profile, including myocardial markers and inflammatory markers, did not reveal any specific abnormalities. The wide QRS tachycardia observed in the ECG showed RBBB, left axis deviation and atrioventricular dissociation. Echocardiography showed 25% of left ventricular ejection fraction and enlargement of the left ventricle and left atrium without a regional wall motion abnormality. Intra-venous and oral verapamil was administered under the suspicion of tachycardia-mediated cardiomyopathy resulting from idiopathic posterior fascicular VT. However, continuous telemetry monitoring demonstrated incessant VT without any response to the treatment. Therefore, electrophysiological studies and radiofrequency catheter ablation (RF-CA) were carried out.\nDuring the electrophysiological recordings, intermittent sinus capture beats were observed, nevertheless, VT continued, and it could not be terminated with 12 mg of adenosine or right ventricle overdrive pacing (pacing cycle length 260-200 ms). Ablation was started from the distal area, where the earliest Purkinje potential was found (). Entrainment at ablation site did not meet the classical criteria, and pace-mapping at ablation site was similar but not exactly matched with clinical VT. It might be due to fusion of pacing beats with incessant VT. If the VT failed to stop within 20 seconds of RF-CA application, the ablation catheter was moved more proximally, and the RF-CA application was restarted. VT was terminated within about four seconds of RF-CA application at the LV mid septal area during 4th RF energy application. Afterwards, a few ventricular beats were observed for 1-2 seconds before disappearing completely. Two more RF-CA applications were delivered at the same location. After the final RF-CA application, VT was not induced either by the programmed stimuli or an isoproterenol infusion of up to 20 µg/min.\nAfter the procedure, there was no left bundle branch block (LBBB) or intraventricular conduction delay on the ECG. Telemetry monitoring revealed that VT did not return, and that normal sinus rhythm was maintained without a single premature ventricular contraction. The patient's symptoms were improved, and he was discharged two days after the procedure. Follow up echocardiography performed five months after catheter ablation showed 49% of left ventricular ejection fraction and shrinked LV. Chest X-rays performed in an outpatient department six months after discharge showed a decreased cardiac silhouette () without symptoms. Follow-up observations are ongoing, and neither VT nor any cardiac symptoms have recurred. |
A 42-year-old Hispanic male without significant past medical history presented with a 4-week history of slow mentation, difficulty concentrating, and change in personality. He complained of bilateral lower extremity weakness, as well as a presyncopal episode and sudden onset of numbness involving his right body side 3 days before admission. Other symptoms included intermittent nausea and vomiting. Physical examination included slow mentation, impaired concentration, facial weakness of the right lower half, dysarthria, and mild weakness in the lower extremities without sensory level or sphincter dysfunction. Full strength in his upper extremities, diminished reflexes throughout, and intact sensation were noted. No cerebellar dysfunction, nystagmus, or Babinski signs were present. Laboratory tests for systemic autoimmune, inflammatory, and infectious diseases, and neoplasm were all negative.\nInitial unenhanced head computed tomography (CT) showed hypodense lesions in the body and genu of the corpus callosum and right basal ganglia. A subsequent brain magnet-resonance-imaging revealed multiple supratentorial lesions involving the rostrum of the corpus callosum, genu of the right internal capsule and basal ganglia and subcortical cingulate white matter, with variable degrees of enhancement associated with mass effect (). Initial differential diagnoses included an acute demyelinating process versus primary CNS lymphoma, glioma, or acute/subacute ischemic infarct.\nCerebrospinal fluid analysis (CSF) showed mild increase of total protein and elevated CSF levels of myelin basic protein, without oligoclonal bands. High doses of intravenous methylprednisolone (1 gm/day) were administered; 72 hours after admission, the patient developed altered level of consciousness, aphasia, left gaze deviation, and right hemiplegia.\nCerebral angiography () demonstrated bilateral intracranial arteriopathy with occlusion of the supraclinoid segment of the left intracranial internal carotid artery distal to the takeoff of the left anterior choroidal artery, occlusion of the right anterior cerebral artery, and severe disease in the proximal right middle cerebral artery. The distal branches of the right middle and anterior cerebral arteries were mainly fed by collateral anastomoses from the anterior and posterior choroidal arteries, producing arterial blush (puff of smoke) consistent with an angiographic Moyamoya pattern.\nWithin few days the patient developed a massive infarction involving almost the entire left hemisphere and the bilateral anterior cerebral arterial territories, associated cerebral edema, midline shift complicated by brain herniation, and eventually death 11 days after admission. |
A 74-year-old man with no significant medical history presented with his left clavicular pain. A chest X-ray showed a neoplastic fracture in the left clavicle. Abdominal and chest CT examination revealed the renal mass with early enhancement in right kidney with 35 mm diameter (Fig. ). In addition, CT scan showed osteolytic mass in the left clavicle, left iliac crest, and small nodule in middle lobe of the right lung (Fig. ). It is the basic policy of our institution to recommend radical nephrectomy for clinical stage T1 and T2, and subsequently he underwent laparoscopic radical nephrectomy. The pathological examination showed clear cell RCC (Fuhrman grade 2) with tumor invasion of perirenal fat, leading to the diagnosis of stage pT3aN0M1. With a diagnosis of left clavicle and iliac bone metastasis, the patient received the treatment with zoledronic acid 4 mg intravenously every 4 weeks.\nAccording to the International Metastatic RCC Database Consortium score, the patient was classified as poor risk (<1 year from time of diagnosis to systemic therapy, Karnofsky Performance Status 70, low hemoglobin value) and began to receive nivolumab plus ipilimumab combination therapy for his multiple metastatic sites.\nAfter one cycle of combination therapy, he felt listlessness of the right forearm and difficulty in speaking clearly. The brain magnetic resonance imaging showed a brain metastasis and cyber knife therapy was performed for brain metastasis (Fig. ). In parallel with radiotherapy, the patient received the second course of the combination therapy. Two weeks later, he complained of grade 3 nausea and anorexia. He also experienced grade 3 diarrhea, grade 2 liver dysfunction, and grade 1 rash, leading to discontinuation of third course of combination therapy (Fig. ). We suspected severe irAEs due to various symptoms, but we were unable to exclude the possibility of bacterial enteritis and started the administration of 40 mg (1 mg/kg per day) PSL for the patient. With the negative test result of fecal culture, the dose of PSL was increased to 80 mg and irAEs gradually improved within next 7 weeks. Finally, the dose of PSL was tapered to 7.5 mg. Metastatic sites maintained partial response for 30 weeks after the discontinuation of ICIs without any additional therapy (Fig. ). However, follow-up CT examination showed the new lung metastatic lesion after 41 weeks after discontinuation (Fig. ) and we decided to start readministration of nivolumab monotherapy (Fig. ). As a result, he experienced only grade 1 diarrhea after three cycles of nivolumab, which can be controlled with the temporary increasing dose of PSL. Importantly, any other irAEs such as nausea, anorexia, liver dysfunction, and rash did not appear after the introduction of nivolumab monotherapy.\nFollow-up CT scan at 12 weeks after the introduction of nivolumab showed the increase in lung and iliac bone metastasis, leading to withdrawal of nivolumab and subsequent axitinib therapy. |
A 55 years old male patient, affected by severe morbid obesity (weight: 193 kg, height 1.75 m, BMI: 63 kg/m2) referred to our Institution for a multidisciplinary evaluation for bariatric surgery. Medical history revealed hypertension, type II diabetes mellitus, severe gastro-esophageal reflux disease (GERD) with hiatal hernia and a family history of gastric cancer (father and uncle). No previous abdominal surgery was reported. A preoperative upper endoscopy (UE) showed antral gastritis with extensive intestinal metaplasia (the helicobacter pylori test was negative); a medium size hiatal hernia was confirmed, as well as grade B esophagitis (Los Angeles classification). Echography revealed hepatic steatosis with no biliary tract alteration. Psychiatric evaluation did not find any contraindication for a bariatric procedure, suggesting a restrictive procedure. After a multidisciplinary counseling, we finally indicated a restrictive procedure for the patient. Being aware of the potential worsening of GERD related to sleeve gastrectomy, and particularly worried for the impossibility of endoscopic surveillance of his intestinal metaplasia in case of standard RYGBP, the patient agreed to undergo RYGBP with remnant gastrectomy, aware of the increased operative risk. A standard 6 trocar access was performed (). Greater gastric curvature was firstly dissected, medially to laterally, and the fundus completely mobilized, dividing the short gastric vessels. Right gastro epiploic and right gastric vessels were divided close to the gastric wall, and the duodenum was then divided using a 60 mm endoscopic linear stapler. Gastric resection was completed stapling the stomach on a 37-French calibration tube, creating a gastric pouch of 40 cc. An antecolic antegastric RYGBP was finally performed (). The remnant was extracted by an enlargement of a midline trocar and two drains were left in place. Because of the important omental adhesions and impressive visceral adiposity, the intervention was technically demanding (287 min). Postoperative course required a 3 days ventilation support in Intensive Care Unit, and was otherwise surgically regular. The patient was discharged on the 8th postoperative day. At 6-month follow-up the patient presented an excess weight loss of 43%, diabetes and hypertension were resolved and he did not complain of any GERD symptom. |
The deceased donor was a 67-year-old man with a kidney Doppler ultrasound (DUS) that was negative for any nodular lesion. As part of the routine postoperative follow-up management, the recipient underwent DUS to assess the patency of the graft on postoperative day 1. The DUS finding was suspicious for an acute arterial thrombosis but did not reveal any focal irregularities. Consequently, a computed tomography (CT) scan was urgently obtained but it did not show any arterial complications. However, it serendipitously revealed a 2.4-cm lesion on the upper pole of the renal allograft which was not detected during the back-table or ultrasonography monitoring. A biopsy of the lesion was performed, and its histology revealed an epithelial proliferation of large cells with finely granular cytoplasm and medium round nucleus vesicular acidophilus, arranged tubules, and alveoli and cords immersed in a connective tissue stroma. This picture was consistent with oncocytoma. However, because the eosinophilic variant of chromophobe renal cell carcinoma (RCC) may morphologically resemble renal oncocytoma, immunohistochemical staining was performed using Ki-67 antibodies and RCC antigens. The results were negative, ruling out chromophobe RCC. The therapeutic options and potential related outcomes were clearly discussed with the patient. Given the low risk of malignant transformation in an oncocytoma [], we found no reason for resection of the lesion or an allograft nephrectomy. Consequently, we opted for active surveillance of the benign tumor with ultrasonography, every 2 months, for the first year and, then, with magnetic resonance imaging (MRI), every year (Fig. ). The patient received mycophenolate-mofetil, tacrolimus, and prednisone throughout the 5-year follow-up period and the regimen for immunosuppression was not changed despite the presence of the renal mass. After 60 months of active surveillance, we report that radiological studies have shown no growth, regression, or any other interim morphological changes to the lesion, and the patient is alive and well (Fig. ). |
A 66-year-old male patient arrived at the emergency room of Kangwon National University Hospital. He was rescued after being jammed between an excavator basket and a wall with some stacked material for 5 minutes. When the patient arrived, the vital signs were stable except the respiratory rate: blood pressure 121/71 mmHg, heart rate 90 beats per minute (bpm), respiratory rate 30 breaths per minute, body temperature 36.9°C, and O2 saturation 94%. The patient complained of severe pain on the whole left side of his body down to the waist and on the right chest wall. According to the plain radiography and the computed tomography performed in the emergency room, the patient had multiple rib fractures (right, 7th and 8th; left, 3rd to 12th), hemothorax on both sides (left dominant), left scapular fracture, liver laceration, retro-peritoneal hematoma, and transverse process fracture of the thoracic and lumbar spine. He was initially admitted to the intensive care unit after closed thoracostomy (). There was no special complication during the chest tube insertion, and the initial drainage was approximately 200 mL. There was bloody drainage during the chest tube insertion; however, it became serous and the patient was transferred to the general ward on the next day. On the 4th post-trauma day, the patient complained of acute pain radiating in the left rear direction. About 400 mL of bloody drainage was found at the collecting bottle at the same time. When the patient complained of pain, he became drowsy as the blood pressure decreased to 70/50 mmHg; however, the blood pressure became 100/70 mmHg after the infusion of 300 mL of isotonic saline. Although the vital signs became stable with a heart rate of 71 beats per minute, respiratory rate of 24 breaths per minute, body temperature of 36.6°C, and O2 saturation of 99%, bloody drainage of 100 mL or more hourly continued. A fluid resuscitation was done through the intravenous infusion of vitamin K1 and tranexamic acid. Then, plain radiography and computed tomography were performed. From the results of plain radiography and chest computed tomography, we observed that the left hemothorax had increased. At the abdominal computed tomography, a portion of laceration at the liver improved; however, it was decided to perform further exploration on the basis of the judgment that there was a possibility of major bleeding because the retro-peritoneal hematoma increased (). At 0:00 (midnight) on the 5th post-trauma day, the patient was transferred to an operating room. After the insertion of the right radial arterial line and the right internal jugular venous central line under general anesthesia, a drape was performed while the patient was in the right lateral decubitus position. When a thoracoscope was inserted after removing the existing chest tube, a huge amount of pleural hematoma was found. The 2nd port was made at the intersection of the 4th intercostal space and the anterior axillary line, and the 3rd port was made at the intersection of the 8th intercostal space and the posterior axillary line. The 3rd port was elongated by 8 cm for bleeding control and hematoma evacuation during one-lung ventilation. When the hematoma was removed through the 3rd port under the assistance of a thoracoscope, it was possible to find that a fractured fragment of the 7th rib remained in the intra-thoracic cavity after penetrating the parietal pleura with its sharp edge. The descending thoracic aorta was just next to it. It was believed that the bleeding originated from the damage of the aorta adventitia inflicted by the fractured rib segment. The sharpness of the rib was dulled by a bone file, and the aorta was repaired by a 2-point interrupted suture using 4-0 prolene (). Other than this, there was no conspicuous damage at the pleura, diaphragm, lung, or parenchyma. After irrigation by 5 L of warm isotonic saline, two 28-Fr. chest tubes were inserted at the 1st and the 2nd ports, and the operation was completed with routine closure. The patient was checked for stable vital signs and chest tube drain status in the recovery room. Around 4:30 a.m., the patient was transferred to the general ward after extubation. |
A 30-year-old woman with a noncontributory medical history was referred to the endodontic specialist clinic at PSMMC complaining of pain and localized intraoral swelling in the left first mandibular molar. One year earlier, the tooth had undergone root canal treatment with cementation of the post and core and placement of a permanent crown. The clinical examination revealed localized swelling in the gingival sulcus. Periodontal probing through the furcation showed increased probing values with a grade II defect [] (). The radiographic examination revealed a large post in the distal canal and a large furcal lesion related to the distal root opposite the post placement. Iatrogenic root perforation was suspected.\nAfter the administration of local anesthesia (1.8 mL of lidocaine with 1 : 100,000 epinephrine), the crown was removed with a crown removal instrument, and the post was removed with an ultrasonic instrument using a light brush and cutting motion to break up the cement around the post. A paper point was used to check for the presence of blood spots to determine the location and size of the perforation. The perforation site was irrigated with 2.5% sodium hypochlorite followed by drying of the canal and sealing of the perforation with mineral trioxide aggregate (MTA; Dentsply Tulsa Dental) mixed with saline and placed with a microapical placement system (Dentsply Tulsa Dental) and condensed with a paper point (). A wet cotton pellet was then placed on the MTA material. The tooth was temporized with glass-ionomer cement and cementation of the crown. At the second visit, the cotton pellet was removed and the MTA setting was checked.\nThe patient was referred to the prosthodontics department for permanent crown placement. Follow-up appointments at 3 months, 6 months, 9 months, and up to 4 years showed complete healing of the soft tissue and bone lesions and a normal pocket depth of 3 mm. |
A 72-year-old male patient visited SMG-SNU Boramae Medical Center with exertional chest pain, which had been aggravated during the most recent 3 months. The initial electrocardiogram revealed sinus bradycardia with a heart rate of 50 beat per minute, and there was no ST segment elevation. The serum cardiac enzyme level was within the normal range, and there were no abnormal findings except mild functional mitral regurgitation in transthoracic echocardiography, which showed a left ventricular ejection fraction of 67% and no regional wall motion abnormality. Myocardial single-photon emission computed tomography showed a reversible perfusion decrease at the basal inferior wall. Preoperative coronary angiography showed 70% to 80% stenosis of the left anterior descending coronary artery (LAD), 90% stenosis of the proximal ramus artery, 50% stenosis of the proximal left circumflex artery, 95% stenosis of the distal right coronary artery, and 90% stenosis of the posterolateral branch artery (PLB). According to the patient’s past medical history, he had undergone right upper lobectomy for squamous lung cancer 3 years ago. In addition, arteriosclerosis obliterans of both the lower extremities had been treated medically for 6 years.\nDiagnosed with unstable angina with three-vessel disease, the patient underwent off-pump coronary artery bypass graft surgery (CABG). Under general anesthesia, an advanced venous access catheter and a Swan-Ganz (SG) catheter were inserted via the right internal jugular vein by the anesthesiologist. Several premature ventricular contractions (PVCs) occurred during the insertion of the SG catheter. We used the left internal mammary artery as a pedicled graft and anastomosed it to the mid-LAD. We also harvested the saphenous vein from the left lower leg and anastomosed it to the left internal mammary artery to make a Y-shaped composite graft. Subsequently, the vein graft was anastomosed to the ramus artery and PLB sequentially. During the operation, there were no events, although frequent PVC occurred.\nAs soon as the patient was transferred to the intensive care unit, frequent ventricular tachyarrhythmia began to occur and the hemodynamics became unstable. We tried our best to control the unstable ventricular arrhythmia with the administration of antiarrhythmic drugs like lidocaine and amiodar-one as well as a meticulous correction of the fluid and electrolyte imbalance, but none of the medical measures worked. Furthermore, ventricular fibrillation (VF) occurred three times at intervals of about 1 hour ().\nConsidering reperfusion injury to be the cause of VF, we inserted an intra-aortic balloon pump to provide hemodynamic support, but failed to stop recurrent VF, and the patient’s condition worsened considerably. In fact, we did not notice the severely angulated SG catheter demonstrated by the serial chest radiographs until around 6 hours after the patient was brought to the intensive care unit. We found that the SG catheter was located too deep in the right ventricle and relocated the catheter to remove the acute angulation (). However, the catheter still seemed to readily stimulate the ventricle; thus, we decided to remove the catheter. During the removal of the SG catheter, VF occurred once again. The removal of the catheter finally resulted in prompt and complete cessation of recurrent ventricular arrhythmia. There were, in total, 5 events of VF and defibrillation was performed 9 times. Unfortunately, the delayed removal of the problematic catheter led to considerable myocardial damage. The myocardial contractility, which was shown to be good by trans-esophageal echocardiography at the end of the operation, worsened and led to a left ventricular ejection fraction of 43%. The serum troponin-I level was also markedly elevated from an immediate postoperative value of 2.0 ng/mL to a peak value of 45.7 ng/mL on postoperative day 1.\nDespite this event, the patient recovered and was transferred to the general ward on postoperative day 6 without any significant complications. Myocardial function was fully recovered without any regional wall motion abnormality, but the saphenous vein harvest site was dehisced and the wound problem had to be addressed. The patient was discharged in a good condition 21 days after the operation. |
A 43-year-old female with a past medical history of severe allergy-induced asthma and chronic nasal drip presented to the clinic with worsening cough and chest tightness for 10 days and was treated for bronchitis with steroids and antibiotics. She then presented to the emergency department with severe and acutely worsening shortness of breath, sudden onset pleuritic chest pain, and sharp radiating pain between both shoulder blades with deep inspiration. At time of presentation, the patient was afebrile with vital signs within the normal limits. Lab work was insignificant with negative leukocyte count and negative cardiac enzymes. A chest X-ray demonstrated an abnormal right cardiomediastinal silhouette with large opacity over the right mediastinum adjacent to the right atrial border. A follow-up CT scan revealed a large right-sided mass adjacent to the right atrium and extending into the right chest measuring 5.1 cm × 9 cm × 4.3 cm (). Her last imaging study was a fluoroscopy study 10 years ago that showed no indications for a mediastinal mass. An echocardiogram revealed a normal ejection fraction (55–59%), no wall motion abnormalities, and a cyst near the right atrium. The patient had continued pleuritic chest pain and difficulty breathing and the decision was made to perform video-assisted thoracoscopic surgery (VATS) for pericardial cyst removal. The patient underwent general anesthesia with a 37 French left-sided double-lumen tube. Standard ASA monitors were applied. Two large-bore peripheral IVs and an arterial line were placed for continuous blood pressure monitoring. Intraoperatively, a large cystic lesion was adherent to the pericardium (). There was no solid component and no obvious communication with the pericardium. Part of the cyst wall was left on the phrenic nerve to preserve it. The patient tolerated the procedure well, had no postoperative complications, and was discharged home on postoperative day number two. The final pathology report revealed benign, acute inflammatory pericardial cyst. |
A 3-year-old girl was referred to a tertiary metabolic bone disease unit for premature loss of primary teeth with roots intact and low serum ALP activity (123 IU/L; reference range: 230–700 IU/L) []. Routine genetic testing revealed compound heterozygosity (c.350A > G, p.Y117C, c.400_401AC > CA, p.T134H) for different TNSALP missense mutations in exon 5 of the ALPL gene, confirming the diagnosis of HPP. On presentation, radiologic assessment of the left hand and arm showed tongue-like lucencies projecting into the metaphyses consistent with childhood HPP. She did not have any clinical features of skeletal involvement of the lower limbs and no motor developmental delay except for a mild waddling gait as a younger child. The patient had a relatively asymptomatic clinical course until she presented at age 11 years with swelling and tenderness of the left ankle that was nonresponsive to paracetamol or ibuprofen. An MRI scan of the ankle suggested a diagnosis of chronic recurrent multifocal osteomyelitis, which was subsequently confirmed by biopsy. The symptoms of pain and swelling of the lower limb joints showed spontaneous transient improvement at age 13 years. Recurring at age 14 years, the symptoms fluctuated and caused significant pain and disability. These symptoms eventually stabilized when the patient was transitioned to adult care at age 17 years. At age 18 years, she successfully underwent radiofrequency ablation for Wolff-Parkinson-White Syndrome, a cardiac disorder unrelated to HPP. The patient is now 27 years of age and has experienced an episode of metatarsal stress fracture; she also suffers from generalized aches and pain.\nOver 22 years, this patient was hospitalized 3 times for a total of 19 days (Table ). Only 1 hospitalization exceeded 3 days, when the patient was admitted for 14 days to receive intravenous antibiotics for suspected osteomyelitis (Table ).\nOutpatient specialist visits, outpatient procedures, and day case procedures represent the majority of healthcare resources used by this patient (Fig. ). Seven specialists provided care for the patient; a pediatric dentist was seen on 40 occasions. Dental procedures, including restorative dentistry (performed on 3 occasions), were the most common of these. This patient was also seen by a pediatric rheumatologist and psychiatrist (Table ). |
A 73-year-old man presented with a 24-hour history of vomiting and severe, constant epigastric pain. In the preceding three months he had noticed intermittent, dull epigastric pain worse on lying flat. He suffered from type II diabetes, hypertension, asthma and intermittent claudication. He had previously undergone treatment for a transitional cell carcinoma (TCC) of the bladder and repair of umbilical and inguinal hernias. He was a retired electrician with previous asbestos exposure and he was an ex-smoker. On examination, he was tachycardic and pyrexial with a distended, generally tender abdomen but no signs of peritonitis. Respiratory examination was unremarkable and his blood tests showed a raised white cell count (16.1 × 109/L) along with raised serum creatinine (189 μmol/L) and urea (11.9 mmol/L). Chest x-ray showed an unusual air fluid level in the lower chest (fig ). Under fluoroscopy, a fine bore tube was inserted and a gastrografin® (Schering AG, Germany) study was done (fig , ). This showed a gastric volvulus with complete obstruction within a Morgagni hernia.\nAn emergency laparoscopy was done via open insertion of a 10 mm port at the umbilicus and subsequent insertion of two 5 mm ports. The stomach, transverse colon and omentum were trapped in the hernia (fig ). They were reduced with difficulty after incising the neck of the sac and the edge of the diaphragmatic defect (fig ). There was a large section of necrotic omentum, which was resected and placed into a retrieval bag and subsequently removed. The transverse colon and stomach were carefully examined and found to be viable. The falciform ligament formed part of the wall of the sac and this was reduced along with the sac and excised (fig ). The edges of the defect were widely cleared of peritoneum and fat to expose the muscle and fascia of the diaphragm. The defect was large and was not suitable for primary closure. Polypropelene mesh was used, with approximately 2 cm of overlap over the edges of the defect and was secured in place using 5 mm titanium tacks (Protack, Autosuture, Tyco Healthcare USA) (fig ). The patient recovered well and was eating normally by day three although discharge was delayed due to a lower respiratory tract infection.\nHe presented jaundiced six weeks later and an ERCP was done to treat a bile duct stone. Unfortunately two months later he presented with shortness of breath and was diagnosed with left pleural mesothelioma from which he subsequently died. There was no evidence of the mesothelioma on either the preoperative chest xray or at the time of operation (although the pleural cavities were not opened). Interestingly, on review of his medical records he had been noted to have a small Morgagni hernia on routine chest x-ray 21 years before however the patient was not informed and no action had been taken. |
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