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A 19-year-old woman presented to a headache specialty clinic complaining of a daily headache for 3 months. She awoke one morning with the headache and it had been continuous since that time. She denied any triggering event surrounding headache onset including no flu-like illness, stressful-life event or surgical procedure. The only possible trigger was a change in her birth control prescription 1 month prior. Her previous headache history was very minimal with infrequent tension-type headaches (TTHs), although she had several family members with probable migraine. The current headache was bifrontal and temporal in location. Average daily pain intensity was severe 7–8 out of 10 on VAS scale. The pain was sharp and throbbing in quality and was constant with no pain-free moments. There were no aura-type spells. Associated symptoms included nausea, vomiting, photophobia, phonophobia, osmophobia, and lightheadedness. She denied having any cranial autonomic symptoms with her headaches. There was a possible positional component to the headache being somewhat better while lying supine and in the morning hours but never any pain freedom. She also stated that over the past 3 months she had two spells of loss of consciousness in which the patient believed she fainted. There was no tongue biting or incontinence with these spells and she was unaware of any post-ictal confusion. However, there were no witnesses to these events. Her past medical history was significant for bipolar disorder. This was well controlled on carbamazepine. General and neurologic examinations were non-focal except for the presence of occipitonuchal and temporal tenderness to palpation bilaterally. Her mental status evaluation was normal. She had questionable cervical spine hypermobility on examination. She was taking ibuprofen on a daily basis without any benefit. She started the ibuprofen only after she developed the daily headaches. She had tried oral sumatriptan, but it was not effective. Prior to her evaluation at the headache clinic, she had a brain MRI with and without gadolinium which was read as a normal study; however, review of the imaging questioned loss of signal in the superior sagittal sinus. A diagnosis of probable NDPH was made, but further neuroimaging was suggested to rule out secondary mimics including an magnetic resonance venogram (MRV) and a magnetic resonance angiogram (MRA) of the intracranial and extracranial vessels. An EEG was also ordered for the loss of consciousness spells to make sure she was not having seizures. The patient was prescribed gabapentin as a preventive agent and was given salsalate, metoclopramide, baclofen and hydroxyzine as abortive agents. The following day she took gabapentin for the first time and added a metoclopramide tablet for nausea. Within minutes of taking these medications, the patient became giddy and after 10 min had a witnessed generalized tonic-clonic seizure lasting 8 min in duration with post-ictal confusion. She was brought to the hospital and there was seen by an epilepsy specialist. Further questioning of the patient and her mother revealed that for the past 3 months, the same time she was experiencing her daily headache, she had begun to have episodes of staring spells each lasting up to 10 s in duration and in which she would be non-responsive, have lip smacking and be picking at her clothes. These were occurring two times per week on average. In addition, she would experience occasional myoclonic jerks but these were very infrequent. Her EEG on hospital admission demonstrated 3–5 Hz generalized spike and polyspike wave discharges. MRV, MRA brain and neck were normal. Based on her clinical history of probable absence seizures, new generalized tonic-clonic seizures, myoclonic jerks and her EEG pattern, it was felt that this was most consistent with a diagnosis of JME. She was started on lamotrigine for the JME with a plan of tapering her off the carbamazepine which can worsen JME symptoms. On follow-up evaluation 3 weeks after hospital discharge and on a dose of lamotrigine 100 mg/day, she was both seizure free and headache free. She has now been followed for 9 months without any recurrent headaches or seizures remaining on the lamotrigine.
A 42-year-old female patient was admitted to our hospital on September 18, 2015. She had a 4-year history of recurrent oral ulcer, a 1-year history of right abdominal pain, a 6-month history of perineal ulcer, and a 4-month history of abdominal mass. She was diagnosed with an appendiceal abscess with cecum rupture and perforation in 2014 at a local county hospital. She received a partial cecum ileocecal resection and intestinal anastomosis. Then, an abscess incision and drainage were performed twice at that hospital. Fecal fluid discharge was observed during each procedure. Six months prior, she underwent an excision of a perineal ulcer at the local county hospital.\nA CT scan of the intestinal tract at our hospital indicated that her CD was multi-series. There were perforation of intestines and formation of a local abscess near the original surgical incision on the anterior abdominal wall. Her colonoscopy showed polypoid change. The pathologic biopsy indicated severe chronic active inflammation of mucosa.\nLaboratory tests revealed that HB was 99 g/L, ALB was 30.1 g/L, and ESR was 113 mm/h. Her admission diagnosis was CD with possible intestinal fistula.\nOn October 13, 2015, she underwent several surgical procedures at our hospital. These procedures included exploratory laparotomy, resection of the diseased small intestine, ileum end ostomy, and right lower abdominal sinus clean-up. She also received nutritional therapy during the perioperative period, which will be discussed below. Due to her poor economic status, 1 month after surgery, she had to choose azathioprine instead of infliximab (IFX).\nThis patient had a weight loss of 15 kg prior to this hospitalization. Her admission body weight was 34 kg and her body mass index (BMI) was 14.2 kg/m2. Our gastrointestinal surgeons removed her diseased small intestine and made a temporary ileostomy. This was done to remove the irritation of antigens from food and to optimize the “full rest” of the lower intestinal tract. It is worth mentioning that, even at this life-threatening point, the patient refused to use IFX and long-term enteral nutritional support for economic reasons. This laid the basis for the twists and turns of the rest of her treatment process.\nOn January 21, 2016, the patient presented to our hospital again and complained of CD (penetrating non-stenotic enterocolic active phase), ileostomy, and severe malnutrition. She was prescribed levofloxacin and fluconazole for anti-infection, diarrhea control, intestinal flora regulation, EN, and PN. However, pneumothorax occurred when the subclavian vein catheterization was performed, and a thoracic vent was placed. As the extra air gradually leaked out, the vent was pulled out. From her abdomen, yellow water-like stool was drained from the ileostomy.\nAt the second admission, the patient's weight had decreased to 27.5 kg (BMI 11.4 kg/m2). She also presented with hyponatremia, which could lead to sudden death at any time. There were multiple reasons for her severe malnutrition and hyponatremia. The primary reason was the considerable loss of electrolytes and water. The average drainage from her ileostomy was 500 and 1300 mL/day. In the early stage of post-operation, the daily excretion was as high as 1800 mL. When discharged, the patient was not well instructed on how to replenish enough water and electrolytes at home. The second reason was that the patient had severe dietary restrictions to prevent an ileostomy blockage caused by food. A fecal stone can form if the patient consumed food with insoluble fiber, which may block the proximal end of the foramen. This also resulted in a fear of eating, which deteriorated her nutritional status. This particular patient was severely malnourished and weighed only 27.5 kg. Thus, improving her nutritional status should be an integral part of treatment. However, PN administration failed because of pneumothorax. Therefore, in this second hospitalization, the patient only received drug therapy with azathioprine and cholestyramine, but she was still severely malnourished when discharged.\nOn May 31, 2016, the patient was admitted again because of a fistula rupture. At great risk, our gastrointestinal surgeon completed an operation consisting of small intestinal stoma closure, enterocolic anastomosis, enterostomy, jejunal nutrition tube implantation, and intestinal adhesion lysis on June 6, 2016. Because of her fistula rupture, the surgeons had to perform the small intestinal stoma closure, and they made a new small intestinal stoma (Fig. A). The jejunal nutrition tube provides another access for nutrition intake in which enteral nutrition could be injected. Normal food could still be ingested orally and then turned into food residue, which is discharged from the upper fistula bag. Therefore, after the third hospitalization, the patient gained two routes for feeding, oral and jejunal. This boosted her energy and also reduced exposure to antigens in the remaining vulnerable intestine. This time, she finally received IFX as her treatment option. On March 30, 2017, the patient's former fistula completely healed (Fig. B).\nTwo years later, on March 20, 2018, the patient came back to receive the stoma closure surgery. Her physicians and surgeons were surprised to see that she weighted 44 kg this time (Fig. C). We performed the operation on April 2, 2018, and she ran a fever because of an abscess on the abdominal wall. Thus, she was then prescribed anti-infective medication after the incision, and it was continued through the IFX treatment. There are several questions left to be answered about this change in the patient's status. What allowed her to gain so much weight, which allowed her to undergo the stoma closure surgery? What had changed in the past 2 years? Was it the selection of IFX or the administration of nutrition therapy? What played the most important role?\nAccording to the ESPEN guideline of clinical nutrition in inflammatory bowel disease,[ the energy requirements of patients with IBD are similar to those of the healthy population. However, the protein requirements are increased to 1.2 to 1.5 g/kg/day in adults with active IBD, which is higher than that of the general population. Therefore, the initial goal of nutritional therapy for this patient was set at 1500 kcal and 60 to 75 g protein. However, in the process of achieving this goal, the nutrition plan had undergone 8 adjustments. The details of the nutrition plan are shown in Table . During the second hospitalization, the patient received nutritional therapy only while in the hospital, which is a short period of time. A pneumothorax occurred when the subclavian vein catheterization was performed. We thought that the pneumothorax was related to the very low BMI of the patient. Fortunately, she continued the enteral nutrition after the second and third hospitalizations.\nA review and analyzation of the entire nutritional therapy for the patient, including the duration and details, is discussed below. Home enteral nutrition lasted for 2 years and 3 months, which accounted for a very large proportion of her total treatment course. This also happened to be the period of time in which she gained weight. Interestingly, we had unintentionally tried all methods of nutritional therapy, including PN via peripheral vein, PN via central venous catheterization, oral nutritional supplementation, nasogastric feeding tube, and jejunal feeding tube. The jejunal feeding tube lasted for 664 days, and it played a very important role in her treatment, providing 1200 to 1500 kcal/day. A significant reduction in inflammatory markers and an increase in body weight (Table ) was observed after 9 months of EN. However, we noticed an increase in her triglycerides at the fourth hospitalization.
A 51-year-old male weighing 131 kilograms (kg) presented to the emergency department (ED) via ambulance with altered mental status and slurred speech after undergoing cervical epidural injection with two milliliters (mL) of 2% lidocaine (40 milligrams [mg]) under fluoroscopic guidance in an ambulatory setting. He became unresponsive during the injection with subsequent brief convulsive activity for which he was given 2 mg of midazolam. Emergency medical services was called and found him obtunded with shallow breathing and low oxygen saturations requiring ventilation assistance. On arrival to the ED his breathing was spontaneous and erratic with low oxygen saturations. He remained somnolent with slurred speech, unable to answer questions appropriately or follow commands. Preparations were made for intubation given altered mental status and low oxygen saturations; however, oxygen saturations and mental status improved within the first 10 minutes of arrival and ultimately intubation was not required.\nOn cardiopulmonary monitor he was noted to have an irregularly irregular heart rhythm. Electrocardiogram showed atrial fibrillation with a rate of 82 beats per minute. Hemodynamically he was stable. He converted to normal sinus rhythm 20 minutes later. He reported no history of atrial fibrillation. Within 60 minutes of ED arrival the patient’s mental status was back to baseline without recollection of the events that had occurred after the start of the procedure. He only had chest wall pain, possibly from sternal rub or from any bystander chest compressions that may have been performed when he became unresponsive. Imaging studies obtained included the following: chest radiograph, computed tomography (CT) of the head, CT angiogram of the head and neck, and CT of the chest with intravenous contrast. No pertinent imaging abnormalities were identified. Serum/plasma levels of lidocaine and its primary active metabolite, monoethylglycinexylidide (MEGX) were obtained 15 minutes after patient arrival. Both levels returned undetectable. The patient was observed overnight in the hospital and remained asymptomatic and without any further dysrhythmia. He was discharged home the following day on aspirin 325 mg daily and with a referral to outpatient cardiology.
A 78-year-old man with end-stage renal disease presented to the ED with altered mental status. His daughter reported that he had two nights of hallucinations that progressed to whole-body shaking that morning. The patient’s baseline was alert and oriented to person, place, and time. He missed hemodialysis on the date of admission, but there were no other recent changes to his health.\nThe patient presented with a heart rate of 75 beats per minute, blood pressure of 131/95 millimeters of mercury, respiratory rate of 18 breaths per minute, oral temperature of 36.4 degrees centigrade, and an oxygen saturation of 99% on room air. A right-sided facial droop was noted. No other significant findings were recorded from the physical or neurological exams. His speech was unaffected, and he had no other focal neurological findings.\nA CT of the head without intravenous (IV) contrast was performed 30 minutes after arrival in the ED. The exam was normal, with no intracranial hemorrhage, midline shift, herniation, or hydrocephalus. There was mild to moderate atrophy and white matter attenuation, as well as intracranial vascular calcifications and trace fluid in the left mastoid air cells. The paranasal sinuses were clear, with a normal appearance of the surrounding bone and soft tissue.\nAfter consultation with neurology, a portable MRI brain scan was ordered without IV contrast and performed 6 hours after arrival. The scan revealed scattered subcortical and deep T2/fluid-attenuated inversion recovery (FLAIR) hyperintense foci, along with prominent sulci and ventricles (Figure ). Periventricular white matter changes were also evident on representative FLAIR and T2-weighted slices (Figures , ). The bony structures were intact, and the paranasal sinuses and mastoid air cells were clear. There was no significant change between the scan and a previous MRI from two years earlier. The diffusion-weighted imaging sequence was repeated twice, due to involuntary motion of the patient. After consultation with the radiologist, the patient was sedated and a 3T MRI brain without IV contrast was done 13.5 hours following arrival for higher imaging resolution.\nThe 3T scan was negative for acute infarction, midline shift, herniation, and hydrocephalus. T2/FLAIR hyperintensities in the central pons and white matter were noted, along with prominent ventricles (Figure ). Multiple foci (predominantly lobar punctate microhemorrhages) were evident in the right cerebral hemisphere and left basal ganglia. The major vascular flow voids were present, along with a trace amount of fluid in the left mastoid air cells. The paranasal sinuses were clear, and the surrounding osseous and soft tissues were unremarkable. There was no evidence of intracranial abnormalities or significant changes compared to the low-strength MRI exam.\nAfter the CT and MRI results were negative for structural causes, the patient was admitted to a neurology stepdown unit the same night for encephalopathy.
14 year old boy presented with deformity and shortening of his left lower extremity []. He had an initial motorbike accident 8 years ago. At that time he had experienced a traumatic open-knee arthrotomy with soft-tissue injury and putative physeal injury of the medial aspect of the knee and lower thigh. No fractures were noted on the initial radiographs []. The patient had a history of multiple previous surgeries including multiple femoral and tibial osteotomies [Figure and ] to address recurrent varus deformities, multiple skin grafts, and a knee arthrodesis [] at other institution. Physical examination demonstrated contracted skin grafts over his thigh and around the knee, which was fused in extension. Radiography [] confirmed 18-cm leg-length discrepancy (13 cm femoral + 5 cm tibial) in addition to a tibiofemoral varus deformity of 28°. His presentation was consistent with a missed medial Salter–Harris VI perichondrial injury, given the initial accident's scalping mechanism, and consequent growth arrest medially, resulting in recurrent varus deformity despite realignment osteotomies.\nAfter extensive counseling with the patient and his family, he was planned for a two-level osteotomy and gradual lengthening and deformity correction of the femur and tibia in external fixation. Due to poor tissue quality at the apex of deformity with thin skin grafts, the metadiaphyses of the femur and tibia were selected as sites of lengthening and deformity correction.\nIntraoperatively, multiple drill-hole osteotomies were created at the metadiaphyseal junctions of the distal femur and proximal tibia and the external fixator was applied [].\nThe patient was followed up regularly during the realignment phase and his correction schedule was adjusted accordingly. Ten months after application of the fixator, after demonstrating satisfactory healing and alignment [], the device was removed []. On recent followup radiographs, the femoral and tibial lengthening sites had consolidated, and the lower-limb alignment and leg-length discrepancy had been surgically corrected [].\nDisplacement of the perichondrial ring in Salter–Harris VI injuries permits bony bridging between the epiphysis and metaphysis, which may then cause progressive angular deformity. While classically associated with lawn-mower accidents, from Rang's work, a more recent case series demonstrated that these injuries are most commonly attributable to closed, minimally displaced injuries sustained via indirect forces, which may be treated nonoperatively with satisfactory results. However, cases of open Salter–Harris VI injuries, caused by a direct “scalping” mechanism, all associated with traffic accidents in the afore mentioned case series, require operative treatment. Operative treatment, in these cases, comprised of repeated debridement and skin grafting, as well as an anticipatory Langenskiöld procedure, utilizing free-fat interpositional graft. This procedure, when applied in acute management of such physeal injuries, may prevent growth arrest. These injuries are difficult to diagnose initially due to lack of visible fracture lines on radiographs, as was the case in our patient. Had the nature of the underlying perichondrial injury been diagnosed earlier, multiple prior surgeries and a stiff, deformed knee may have been prevented. Thus, the treating surgeon should have a high index of suspicion of a perichondrial injury based on the mechanism of injury (such as a scraping “road rash” injury) in a young child. Furthermore, these young patients should be followed up with serial radiographs during their growing years to ensure symmetric growth of the adjacent growth plates.\nWhile osteotomy at the apex of deformity would allow for angulation alone to realign the bone ends, geometry of the deformity does not dictate osteotomy level on its own. Proximity to the joint or physis, quality of bone, and (of particular relevance to our case) soft-tissue coverage must be considered in surgical planning. As Paley affirms, by selecting an osteotomy level or levels away from the deformity's apex, angulation in conjunction with translation are required to realign the bone ends (and, it follows, the mechanical axis).\nWhile the original classification includes five types of Salter–Harris fractures, this case demonstrates the presence of a distinct perichondrial Salter–Harris VI injury prone to subsequent growth arrest. These injuries can be missed often, due to low clinical suspicion and the absence of a fracture line on plain radiographs.
A 47-year-old woman referred to our center with a history of gradually worsening symptoms of headache, diplopia, and left-sided ptosis (eyelid drooping) for 1 year ago that has progressed intensely during the past 4 months.\nMagnetic resonance imagining that performed in another center revealed a localized midbrain lesion and referred to our center 9 months after her initial symptoms.\nThe neurological examination revealed left eye hypotropia and ptosis. The medial and upward gaze of the left eye was impaired and deviated slightly out and down in the primary position []. The pupil was unreactive and mildly dilated compare to the right eye. The rest of the physical examinations showed no significant findings.\nMultiplanar images at different MRI sequences with and without intravenous contrast showed small spherical tumor (10 × 5 × 5 mm) abutting anterolateral of the left cerebral peduncle. The tumor appeared isointense on T1-weighted images, hyperintense on T2-weighted images. Imaging confirmed a gadolinium-enhanced mass with no dural attachment, suggesting for Schwannoma [].\nAfter general anesthesia, lateral subfrontal craniotomy was performed. Dura opened on curly Lina Fashion. The carotid artery was found. By opening the liliequist membrane, the posterior communicating artery was followed to the interpeduncular fossa. Firm dark pink global tumor with a compressive effect on the mesencephalic area appeared. The third nerve ran into the tumor []. Attempts to separate the tumor from the nerve were seemingly impossible because the tumor was adhesive and appeared to be rising from the oculomotor nerve. Complete paralysis of the third nerve on preoperative examinations indicated neural, and tumor fibers had been intertwined. Therefore, the surgeon considered the complete resection of the tumor as the best option for the patient and the tumor, together with the involved part of the oculomotor nerve, was resected [].\nThe histopathological study of the 10 × 5 × 4 mm dark pink mass revealed round to oval centrally located nuclei with dispersed chromatin and eosinophilic cytoplasmic. Lobules of the tumor are separated from each other with collagen sheets and contained whorls and psammoma bodies. Necrosis was not a feature. The final diagnosis was meningothelial meningioma (WHO Grade I).[]\nOn postoperative examination, all preoperative signs were found as complete paralysis of the left oculomotor nerve without slight responses and reflexes, which was expected due to the tumor and nerve resection. At a 6-month follow-up, she continued to have diplopia and eyelid drooping. In the brain MRI finding, there was no evidence of recurrence during 1 year follow-up.
A 62-year-old woman presented to a hospital for health checkup in September 2009. Her physical examination revealed no abnormal findings, no hepatomegaly, no splenomegaly nor lymphadenopathy. The laboratory tests showed the following: RBC counts: 410 × 104, Hb level: 13.9 g/dL, and LDH level: 205 IU/L. Upper gastroduodenal endoscopy showed an elevated tumor with a diameter of 4 cm at the gastric body (Fig. ). The pathologic diagnosis was a diffuse large B cell non-Hodgkin lymphoma being CD20 and CD79a positive (Fig. ). Although the scattered normal lymphocytes showed restrictedly positive anti-CD3 antibody (T cell) localized outside large immunoblastic lymphoma B cells, the DLBCL cells were completely negative using the anti-CD3 antibody in immunohistology (Fig. ). For further treatment, the patient was consulted at our hospital in October 2009. Her physical examination and her blood test did not show any abnormal findings on admission to our hospital. Her PS was well (0). Abdominal CT and pulmonary CT were conducted for the determination of clinical stage; however, all tests were normal. Gastroduodenal endoscopy was retested, but the tumor in the stomach was not detected any more. Several biopsy specimens taken from the redness of the stomach did not show any findings of lymphoma at all. H. pylori infection was positive. We checked whether there was a sample exchange error at tissue sampling in the previous hospital; however, no mistakes were detected during the endoscopic examination. Because no lymphoma cells were demonstrated, the patient was followed up without treatment and gastroduodenal endoscopy was planned every 3 months for 1 year. During endoscopy performed at 3, 6, 9, and 12 months (Fig. ), no lymphoma cells were detected in the histology. During the following 3 years, endoscopy was performed every 6 months; however, without detection of lymphomas. Although gastroduodenal endoscopy and blood tests were performed every year, no DLBCL was detected, and she remains well without any evidence of recurrence at around 10 years after the initial diagnosis. Because H. pylori infection was positive and the histology showed intestinal metaplasia as well as gastric atrophy (Fig. ), H. pylori eradication treatment was started in June 2018 to reduce the risk of gastric cancer (Fig. ).
A previously healthy two-year-old male presented to a pediatric ophthalmologist with a history of acute proptosis of the left eye associated with fever. Examination showed that he could follow and fixate well with both eyes. He had prominent proptosis of the left eye and no palpable lymph node enlargement was noted. The rest of his ophthalmic examination was unremarkable. A computerized tomography (CT) scan of the orbits done at that time revealed a left retro-orbital soft tissue mass with minimal contrast enhancement. His white blood count was elevated (15.9 × 109/L).\nHe was diagnosed with orbital cellulitis and treated with a course of intravenous ciprofloxacin with little improvement. He was also non-responsive to a trial of oral dexamethasone. An orbital biopsy was performed via a lower lid approach. Histopathology showed tissue infiltrated by lymphocytes, plasma cells, hemosiderin-laden macrophages and histiocytes with mild stromal fibrosis. Immunohistochemical staining showed a mixture of B and T lymphocytes. A presumptive diagnosis of fibrosing orbital inflammatory disease was made and he prescribed oral prednisone (30 mg/day) 1 mg per kilogram with some improvement. Shortly afterwards, the patient was lost to follow-up.\nThree years later the patient presented to our unit with recurrent proptosis of the left eye and lid swelling, which could be felt as two firm palpable masses in the upper lid and one in the lower lid []. Best corrected visual acuity was 20/40 in both eyes and he had mild limitation of abduction of the left eye. Pupillary reactions were normal as were the anterior and posterior segments of both eyes. Magnetic resonance imaging (MRI) of the orbits showed extensive soft retro-orbital tissue mass, extending to the left cavernous sinus, iso-intense on T1 and T2 with diffuse post-contrast enhancement [].\nAnother orbital biopsy was performed through an upper and lower lid skin approach, along with an adenoidectomy and biopsy of the left inferior turbinate. Histopathological examination revealed an infiltrate of lymphocytes (both B and T), polyclonal plasma cells and histiocytes; however, no eosinophils were present. The histiocytes had large round nuclei, prominent nucleoli and vacuolated cytoplasm. Some of the histiocytes contained numerous intact mononuclear cells, plasma cells and erythrocytes in their cytoplasm. A few reactive lymphoid follicles were seen. No Reed-Sternberg cells, Dutcher bodies or micro-organisms (using Periodic-acid Schiff, Gram, Ziehl–Neelson and Wade–Fite stains) were seen. The biopsy was also negative for Epstein–Barr virus staining, Bcl-2 and CD1a. However, it was positive for S100 and weakly positive for CD68. All of the histopathological and immunohistochemistry characteristics ruled out possible malignancy or infection and the findings were consistent with RDD.\nDue to the large extension of the lesion and the cosmetic appearance, the child was referred to a pediatric oncologist for further evaluation and treatment. However, the family did not complete this follow-up.
A 51-year-old female presented to our outpatient department with bilateral knee pain and difficulty in ambulation without aid. On examination, she had bilateral medial joint line tender-ness and an antalgic gait with bilateral varus thrust. Plain radiographs confirmed bilateral Grade 4 osteoarthritis (-). Bilateral TKA was done on July 27, 2015, using CS Genesis II from Smith and Nephew. The patient had an uneventful intraoperative and post-operative course with physiotherapy and aided ambulation initiated on the 2nd day after the surgery. On follow-up, the patient was pain free, was doing active physiotherapy exercises with knee flexion range of 0–1100 bilaterally, and was walking without support.\nThree years later, she had a fall in her bathroom and presented with pain and swelling in the left knee. She complained of the presence of a swelling in the left popliteal area for 1 year, for which she was consulting a local medical practitioner; after the fall, she developed pain in the knee for which she was referred to the primary operating surgeon. On examination, a tender swelling was palpable in the popliteal fossa, extending up to the mid-calf (, ). Homan’s sign was negative and the knee flexion was restricted due to pain in the knee.\nAll blood tests to rule out infection were within the normal range. The ESR was 12 mm at 1st hour and the quantitative CRP was 6 mg/l. The total white blood cells count was 9500 and the D- dimer levels were normal. Radiographs showed no evidence of osteolysis, fracture, or implant loosening (-D). Color Doppler for DVT was negative. The USG of the cyst showed hypoechoic echoes in a Baker’s cyst-like collection ().\nAspiration under all aseptic conditions was done, and cytological findings were suggestive of hemorrhagic nature of the aspirate, without any evidence of polyethylene debris. The cultures were sterile.\nThe patient was advised rest, graduated physiotherapy, and minimal walking with stick. She became asymptomatic over the next 4 weeks; but a mild swelling persisted for 3 months. The patient has been asymptomatic with no complaints, and even at the last follow-up at 4-year post-TKR, there was no evidence of any implant-related issues.
A 13-year-old male was referred to the Dental Department at Krishna Devaraya College of Dental Health Sciences Center for definitive management of a right-sided maxillary lesion. Which was previously diagnosed as fibrosseous lesion with the help of CT 2 years back. A three-year history of a slow growing mass causing intermittent pain in the right midface was reported. The patient denied any visual disturbance. Physical examination revealed fullness of the right midface which was mildly tender to palpation. The overlying skin was not erythematous and he demonstrated no lymphadenopathy or trismus (). Intraoral examination revealed a firm, nontender swelling expanding the buccal cortex of the maxilla, extending from right lateral incisor to second molar, there was no mobility in the overlying teeth, but displacement of teeth was noted, measuring around 4 x 2 cm in diameter ().\nThe occlusal radiograph showed a large multilocular radiolucent area with a well-defined sclerotic margin extending from the right lateral incisor to the distal aspect of the right second molar, with ’spider web’ and ‘tennis racket’ pattern appearance, with which a preliminary diagnosis of OM was made (). A computed tomographic (CT) scan, axial and coronal views demonstrated an lytic lesion with expansion and thinning of the overlying buccal cortex with radiopaque foci spread throughout the lesion involving the right maxillary antrum ().\nAn incisional biopsy confirmed the diagnosis of odontogenic myxoma. The surgical management involved a combined intra- and extraoral approach. The tumor was resected with a margin of normal tissue. This involved a maxillary ostectomy (). Macroscopically, the surgical specimen consisted of a segment of complete right maxilla and antrum with gelatinous mass with glistening mucoid substance (). Microscopically, the tumor was composed of loosely arranged spindle cells with serpentine nuclei within a variably myxoid and fibrous stroma (). Postoperative recovery was uneventful. The patient has since been seen regularly for follow-up, and treatment planning for dental rehabilitation is currently underway. He will be monitored long-term for signs of recurrence clinically and radiographically.
A male Chinese patient aged 42 years with a height of 193 cm, weight of 95 kg and a history of chronic hepatitis B infection for >20 years. The patient presented with an obstructive liver lesion, as assessed by physical examination, and was admitted to hospital for 2 weeks. Computed tomography (CT) imaging evaluation identified a single tumor with diameter approximately 3 cm located in segments V and VIII of the liver without macrovascular invasion. The tumor was staged as TNM stage T1bN0M0 Ib, corresponding to Barcelona Clinic Liver Cancer (BCLC) Stage A. The patient had an Eastern Co-operative Oncology Group Performance Score (ECOG PS) of 0, no jaundice of the skin or sclera, and negative abdominal signs. Laboratory tests revealed that the patient had normal liver function (Child-Pugh Score 5 and Grade A) and coagulation, an AFP level 4.45 ng/mL, hepatitis B surface antigen titer of 1816, HBV DNA level of 3.45×104 IU/mL and Protein Induced by Vitamin K Absence or Antagonist-II (PIVKA-II) level 34 mAU/mL. Based on the single liver lesion, early stage of disease, good liver function and discussion with the patient, a hepatectomy was performed.\nAfter a successful hepatectomy with a surgical margin of 1 cm to the tumor, pathological examination revealed the tumor had a diameter of approximately 3 cm with a negative surgical margin consisting of moderately differentiated HCC and without tumor thrombus or satellite foci. After surgery, the patient received one cycle of TACE to identify residual lesions and confirm successful resection, and no residual tumor was observed. Post-surgery, the patient received thymosin to prevent the relapse of HCC, as well as oral entecavir (500 mg/day) for the management of hepatitis B. The patient experienced tumor recurrence 14 months after surgery with a single tumor of approximately 2 cm diameter located between the left and right hepatic pedicle of the first porta hepatis. The tumor was close to the portal vein and biliary tract without macrovascular involvement. The recurrent tumor was early stage (T1aN0M0 Ia) and based on the Milan criteria the patient was a potential candidate for liver transplantation. However, the patient refused to undergo liver transplantation and underwent a CT-guided microwave ablation procedure. Post-surgery TACE revealed complete tumor ablation without residual tumor. The patient continued to receive thymosin to prevent HCC recurrence, as well as oral entecavir for hepatitis B.\nAfter a further 11 months, the patient experienced a second recurrence characterized by multiple disseminated metastases in the left and right lobes of the liver and possible macrovascular invasion. No distant metastasis was identified and the patient had an ECOG PS of 0 with Child Pugh Stage 5 and Grade A. However, the tumor stage of the patient was relatively advanced beyond transplantation criteria (BCLC Stage B/C) (). Based on guideline recommendations, the patient was advised to initiate systemic therapy., Genetic testing indicated a low tumor mutation burden and immunohistochemical analysis was negative for programmed cell death ligand 1 (PD-L1) expression. A microsatellite stability test found no variation for MLH1, MSH2, MSH6, and PMS2, which indicated a low probability of benefit from immune therapy and this was communicated to the patient. The patient therefore initiated treatment with lenvatinib (Lenvima®, Eisai Co., Japan) 12 mg once daily and achieved a partial remission (mRECIST criteria) by imaging evaluation after 3 months of treatment (), and a reduction in PIVKA-II levels from 254 to 142 mAU/mL. Three months after initiation of lenvatinib, the patient decided to transfer to another hospital (Peking Union Medical College Hospital) with specialized expertise in the use of anti-programmed cell death 1 (PD-1) antibody therapy, at which they could begin additional treatment with anti-PD-1 antibodies (off label, toripalimab, Shanghai Junshi Biosciences, Shanghai, China) (240 mg). The patient experienced a severe immune reaction (Stevens-Johnson syndrome) 6 days after initiating immune therapy. Subsequently, the patient immediately terminated PD-1, and after returning to our institution received intravenous glucocorticoid therapy for one month, before switching from lenvatinib to apatinib monotherapy (off-label, Aitan®, Jiangsu Hengrui Medicine, China) (850 mg daily) for 10 days. The patient was then found to have progressive disease as evaluated by imaging examination (), accompanied by an increase in PIVKA-II level from 142 to 1019 mAU/mL. After multidisciplinary consultation, the patient was switched back to lenvatinib monotherapy.\nAfter one month of lenvatinib treatment, the patient was found to have again achieved a partial response (). In addition, the patient’s PIVKA-II level had decreased from 1019 to 324 mAU/mL and the tumor stage recovered to BCLC Stage A. Based on the Milan criteria the patient was suitable for a liver transplantation and was advised to make the most of this “window of opportunity”. However, the patient refused surgery. After being in remission for 4 months, the patient’s PIVKA-II level increased, which was defined as progressive disease. The tumor stage of the patient advanced to BCLC C and the patient missed the opportunity for radical hepatectomy. At this stage, the patient was switched from lenvatinib to regorafenib 80 mg daily. The patient was followed up for the last time on April 21, 2020, and PET-CT revealed bone and lung metastasis ().
A 73 year old right hand dominant active lady complained of bilateral shoulder pain for about two months and did not respond to two ultrasound guided subacromial subdeltoid corticosteroid injections. Her shoulder injury occurred whilst she had been caring for her husband and working on their farm. In the past she had recovered from non-Hodgkin’s lymphoma and currently was on antihypertensive medication (candesartan cilexetil once a day). She had led a physically active country life style prior to the presentation of the problem and had never smoked in her life.\nAt presentation there was painful limitation of right sided shoulder abduction to less than 90 degrees. Ultrasound documented a 9mm × 14mm partial width full thickness footprint tear of the anterior to mid right supraspinatus with tendinosis of most of the tendon and enthesopathy at the greater tuberosity of the humeral head (\n). A plain radiograph of the right shoulder showed a down-sloping Type 2 acromion. There was mild wasting of the right supraspinatus muscle. A diagnosis of a recent footprint tear superimposed on degenerative supraspinatus tendon with mild muscle atrophy was made. Following written informed consent from the patient, 8ml of autologous unclotted blood was venesected and centrifuged for 5 minutes at about 3000 rotations per minute in a special tube for PRP preparation (BCT, REGEN Labs, Switzerland). 4 to 5ml of liquid PRP was injected through a 22g 5cm long needle into the tear and its margins with simultaneous percutaneous tenotomy directed into the footprint of the anterior facet of the greater tuberosity under direct ultrasound imaging control (GE Logic 9, 9MHz probe). 5ml 1% lignocaine was injected into the superficial soft tissues, subacromial bursa and the supraspinatus tear for local anaesthetic purposes. The shoulder was placed in a sling with 90 degree elbow flexion for 7 days. Physiotherapy was commenced at 4 to 5 weeks post PRP with a home exercise program. At 8 weeks follow up post PRP the patient verbally reported a marked reduction in pain with improvement in shoulder movement. At the 7 and 10 month follow up there was complete relief from pain with full range of movement of the right shoulder and she was able to lift bags of potting mix in her farm. At the 10 month follow up, ultrasound (GE Logic 9, 9MHz probe) performed by the author showed a near complete echogenic infilling obliterating the tear defect. The lateral margin of the tear merged with this neotendon tissue with mild medial retraction (\n). She was completely pain free at a follow up 1 year after PRP injection.
A 47-year-old previously healthy Sinhala female's right foot was bitten by a snake near the back door of her home in the Kegalle district, Sri Lanka. Within seconds, she felt burning pain ascending along that limb, and there was heavy bleeding from the site of bite. Within a couple of minutes, she felt dizziness, nausea, and numbness of the whole body, had profuse sweating and frothy salivation, and was screaming in pain from the site of bite. On the way to the nearby hospital, she started to clench her jaw tightly and limbs became rigid; she was frothing and was not responding for about 5 minutes, indicating a generalized seizure. She arrived at the hospital within 30 minutes. The doctor at the outpatient department decided to administer ASV and directed the patient to an internal medicine ward for that. Physical examination findings at the ward were a pulse rate of 100/minute and blood pressure of 150/90 mmHg, and lungs were clear to auscultation bilaterally with an arterial oxygen saturation of 95% whilst breathing air with no neurological deficit. By this time, the killed snake was brought in and doctors identified it as a HNV; thus, antisnake venom (ASV) was not administered. Even though there was bleeding at the site of the bite even on admission to the hospital, her 20-minute whole blood clotting time, platelet count, prothrombin time and international normalized ratio, and activated partial thromboplastin time and liver function tests were all normal. Urine sample obtained via the catheter showed 50–55 red cells per high-power field, arterial blood gases indicated a compensated metabolic acidosis, and serum sodium and potassium levels were normal. Her urine output was <100 ml for the first 24 hours and serum creatinine rose from 80 μmol/l to 277 μmol/l. She was transferred to the Teaching Hospital, Kandy, on day 2 for further management.\nOn day 2, a bulla developed at the site of the bite, and there was an edema and warmth at the right foot. Complete (full) blood count demonstrated neutrophilic leucocytosis, and the CRP level of the following day was 261 mg/l. Intravenous antibiotics was started to cover the wound infection. Serum creatinine was 377 μmol/l with oliguria on day 2. Serum sodium and potassium levels remained within the normal range from day 1–5. On the day 5, creatine kinase was 75.1 U/l. Regular hemodialysis every other day from day 2 to day 24 and fluid management were started. Oral sodium bicarbonate was started, and management of her acute kidney injury with collaboration of nephrology team continued.\nOn day 3, her blood pressure rose to 160/90 mmHg, and it was controlled by prazosin and nifedipine SR; however, it generally remained on or above 140/90 mmHg until her discharge. She developed bilateral lung crepitations on day 3 that remained for 7 days. She developed bilateral parotid swelling and edema of the right leg on day 3, and it lasted 3 days. Edema below her right knee persisted another 10 days. Her blood picture on day 2 did not show hemolysis and was suggestive of bacterial infection but blood picture on day 5 showed evidence of microangiopathic hemolytic anemia (MAHA), and same changes were there in a blood film taken on day 11, as depicted in .\nHer day 2 hemoglobin level of 10.8 g/dl dropped to 8.4 g/dl on day 5. On day 2, her platelet count was 104 × 109/l and that dropped to nadir of 29 × 109/l in day 6 and was <150 × 109/l until day 20. A consultant in transfusion medicine has assessed her, and blood transfusion and plasmapheresis was performed on day 7. Another four cycles of plasmapheresis followed. Local edema at the site of the bite increased with necrosis (); thus, wound debridement was done on day 7 and followed up by regular wound toilets.\nWe did an electroencephalogram (EEG) on this patient on the earliest available day (day 11) and that was normal. The 2D echocardiogram done on day 17 was also normal.\nThe offending snake's carcass was taken to the Peradeniya University, and an expert on HNV, Dr. Kalana Maduwage, has confirmed it as a Hypnale hypnale. is a photo of the offending snake.\nAs her daily urine output improved to >1000 ml, she was discharged on day 30 and asked to come for a review in five days. She defaulted treatment and was on alternative medication. After developing progressive bilateral ankle edema and exertional dyspnea, she came back again on day 46, and hemodialysis and supportive therapy were restarted at the nephrology unit. On day 49, she had an anterolateral non-ST-elevation myocardial infarction (non-STEMI), and she was managed at the cardiology unit. She had progressive impaired vision of the left eye starting from a few days after the snakebite and could not count fingers held 30 cm in front of that eye on the 46th day. She was referred to the eye unit, there was bilateral optic disc edema more on the left, the patient was diagnosed of left anterior ischemic optic neuropathy (AION), and steroid therapy was started. Her erythrocyte sedimentation rate and contrast-enhanced computed tomography (CECT) brain done on day 53 were normal. is a photograph of fundi of this patient.\nShe had two episodes of seizures on day 76, and we suspected a possible relationship to her envenomation. The opinion of the neurology team regarding three seizures was obtained. Repeated EEG and CECT brain were normal. Despite being on calcium carbonate 500 mg plus 0.25 μg 1-alpha-hydroxycholecalciferol daily from day 46, her serum calcium level was low (1.8 mmol/l). Last two seizures were attributed to hypocalcemia due to chronic kidney disease following HNV envenomation, and daily calcium carbonate dose was increased to 500 mg thrice daily. After three months, she was diagnosed of end-stage renal disease by nephrology team and on hemodialysis once in four days and was searching for a kidney donor at six months.
We report a case of 35-year-old male presented to the outpatient department of Radiotherapy, CSSMU, Lucknow with chief complaints of sore throat and dysphagia for one month. On clinical examination, there was no palpable neck node. On oral cavity examination, an ulceroproliferative growth was visible over the left tonsil. On palpation, it was found to be extending up to the base of the tongue. On video laryngoscopy, extensive growth involving left tonsil, tonsil lingual sulcus extending to the base of tongue was seen. His routine hematological/biochemical investigations, blood sugar and X-ray of chest were normal. There was no history of smoking and recent trauma /surgery. Complete coagulation profile including the PT, aPTT, and international normalized ratio (INR) were found to be normal. After detailed workup, the disease was staged as carcinoma of left tonsil.\nAfter obtaining informed written consent for treatment, the patient was planned for radical dose of radiotherapy concurrently with weekly cetuximab therapy. The dose of cetuximab administered was 400 mg/m2 in the first week followed by 250 mg/m2 weekly thereafter.[] Radiotherapy was planned for a dose of 70Gy in 35# @200cGy/#; 5#/ week by parallel and opposing lateral fields to the whole neck and primary with sparing of cord after 46Gy. From the second week onwards, the patient developed skin reactions on the cheek. After receiving four doses of cetuximab and 40Gy of radiation, the patient developed sudden onset of pain in the left calf region which was found to radiate to the left foot. There was loss of sensation below the ankle joint. Color Doppler study of the left lower limb revealed complete thrombosis of lower superficial femoral, popliteal and proximal tibial arteries and veins with few collateral channels and no flow in anterior tibial artery and lower posterior tibial artery [Figure 1–]. The tests for antiphospholipid antibodies were negative. Emergency embolectomy was done and low molecular weight heparin was started and the patient was put under observation for 48 h. No improvement was observed after 48 h. There was a change in the color of the skin of the foot []. A repeat color Doppler examination showed complete thrombosis of lower superficial femoral, popliteal and proximal half of posterior tibial arteries and veins with few collateral channels and no flow in anterior tibial artery and lower posterior tibial artery. The patient had to ultimately undergo a left lower limb amputation. After three weeks of radiation therapy break, treatment was restarted with radiotherapy alone, omitting concurrent cetuximab. On first follow up, at six weeks after completion of radiotherapy, the patient was disease free at local site.
A previously healthy 6-year-old boy presented to a pediatric hospital with a 3-week history of torticollis. He had symptoms of an upper respiratory tract infection four weeks prior and had 2 days of documented fever at home during that time. He had been treated with a 7-day course of amoxicillin by the primary care physician for suspected streptococcal pharyngitis. Four days into the course of antibiotics, he woke up from sleep with pain on the left side of his neck. Despite taking ibuprofen and acetaminophen, he presented to the Emergency Department 3 weeks later due to persisting torticollis. Pain was worse with movement. There was no history of head/neck trauma. At the time of presentation, the infectious symptoms had resolved. Some fatigue was noted but he remained generally active, continuing to play hockey. There was no history of rash, peripheral joint pain, or weight loss. Past medical history and family history were unremarkable.\nOn examination, the patient was afebrile with normal blood pressure for age and a maximum heart rate of 110 beats per minute. The patient's head was tilted to the right with chin rotation to the left. No lymphadenopathy or masses were noted on palpation of the neck. There was no tenderness to palpation of bilateral sternocleidomastoid muscles. There was a limited range of motion in all planes of rotation of the neck secondary to pain, particularly in lateral flexion. Bilateral injected conjunctivas were present. The oropharynx was normal with no erythema or mucus membrane changes. Cardiovascular exam revealed normal peripheral pulses, a quiet precordium with normal heart sounds, and no murmur. Respiratory exam was normal. The abdomen was soft with no distension, tenderness, or hepatosplenomegaly. There were no bruits heard on auscultation of major vessel regions. There were no rashes or desquamation of the skin. Neurological exam was normal.\nAt the time of presentation, laboratory investigations revealed an elevated white blood cell count of 17.4 × 109/L with a neutrophil count of 14.1 × 109/L. Hemoglobin was normal for age at 110 g/L. Inflammatory markers were elevated including platelet count of 860 × 109/L and CRP of 38.5 mg/L. Renal function (BUN and creatinine) and liver function (ALP and ALT) were normal for age. Because of the unexplained elevated white blood cell count and evidence of inflammation, a chest X-ray was performed which revealed normal lung fields but an enlarged cardiac silhouette. X-ray of the cervical spine was normal with no atlantoaxial rotary subluxation demonstrated. Ultrasound of the neck revealed mild thickening of the left sternocleidomastoid muscle and no lymphadenopathy. Abdominal ultrasound with Doppler was normal.\nAdditional investigations included a normal throat swab for group A streptococci and a negative anti-streptolysin O antibody titer. High-sensitivity troponin was elevated to 176 ng/L. Creatinine kinase was normal. ANCA was normal. Electrocardiogram showed normal sinus rhythms without evidence of chamber hypertrophy. The patient underwent an echocardiogram to further characterize the enlarged cardiac silhouette identified on the chest X-ray. This revealed massive ectasia and aneurysmal dilatation of the right coronary artery, left main artery, left anterior descending artery, and circumflex arteries, as seen in . Left ventricular function was normal. The aortic arch was normal as were the proximal neck vessels.\nBecause of the dilated coronary aneurysms, the patient was diagnosed with KD. Despite lack of fever, given the evidence of ongoing inflammation and initial presence of bilateral nonsuppurative conjunctivitis, in addition to the coronary artery changes, the patient was treated with high-dose IVIG (2 g/kg) and started on daily low-dose aspirin. Low-molecular-weight heparin was started as antithrombotic therapy and once stabilized, daily atenolol was initiated. Activity was restricted as much as possible.\nInflammatory markers were followed. Platelets revealed a peak of 952 × 109/L and CRP a peak of 54.6 mg/L. After treatment, both platelet and CRP levels normalized.\nThe patient's neck pain and the limited range of movement resolved immediately after treatment, as did the bilateral conjunctivitis. The patient was stable and appeared well at time of discharge. His aspirin, low-molecular-weight heparin, and atenolol were continued. The CT angiogram performed after discharge revealed massively dilated and aneurysmal coronary arteries, as shown in .\nIn follow-up cardiology and rheumatology clinics, he has been doing well with no further neck pain or stiffness. He did not develop desquamation during follow-up, and the repeat echocardiogram one month after discharge was unchanged. He will continue long-term anticoagulation therapy with low-dose heparin with a target level greater than 0.5 IU/ml. He will also continue low dose aspirin and atenolol. His family was advised to have the annual influenza vaccine.
A 25-year-old man was referred by his general dental practitioner to the Department of Oral and Maxillofacial Radiology regarding a periapical lesion related to the apex of the upper right molar teeth and prosthetic management. Intraoral examination revealed that there were a few teeth with caries in different regions. Panoramic radiography revealed a well-defined radiolucency involving the root apices of the upper right maxillary first molar (). The borders exhibited dense radiopaque features. Provisional diagnosis of the radicular cyst was established, and root canal treatment was carried out. Endodontic retreatment was initiated by his dentist, but after a followup of several months, no changes were observed in the ovoid radiolucency, and the patient was referred for consultation to our department. The patient's medical history was unremarkable, included no trauma of the jaws, and he had not undergone any jaw surgery. He was not aware of the radiolucent area before his routine dental examination and had no sensory or motor deficiency. The tooth was asymptomatic and it also presented with a poor root canal filling. The overlying mucosa of the lesion was quite normal, and there was no sign of infection or fistula. The overall appearance of the lesion seemed to be that of a radicular cyst of the right maxillary first molar. On performance of vitality tests, all the teeth except the first molar- in the mentioned area appeared to be vital.\nTo reveal the exact location definition of the pathologic features, he was referred for a CBCT (NewTom 5G, QR, Verona, Italy) scan. After this, a CBCT was taken which revealed the maxillary sinus with its border extending but not involving the roots of the mentioned teeth, which is one of the anatomical variations of maxillary sinus (–). The dimensions of the defect were 13.2 × 14.2 × 9.3 mm depth (mesiodistal length, inferosuperior height, and buccolingual depth).
A 28-year-old Asian woman (G3P1) who had undergone emergency cesarean delivery owing to a compound presentation at full term was referred to our institution with a suspicion of abnormally located gestational sac. She had undergone laparoscopic cholecystectomy and open appendectomy previously. She did not have any medical, family, or psychosocial history. She had missed her menstrual period without any other symptom and visited a private obstetrical clinic to confirm the pregnancy. However, she was diagnosed as having an abnormal pregnancy such as cervical or CSP by USG.\nAt our institution, she reported that her last menstrual period was just 5 to 6 weeks prior. However, USG revealed a gestational sac in the anterior lower uterine segment with a fetus measuring 4.83 cm crown-rump length (CRL) with positive cardiac activity, corresponding to 11 weeks and 6 days of gestation. Color/power Doppler images depicted a hyperechoic rim of a choriodecidual reaction with excessive vascularity (Fig. ). Although we could observe a definitive abnormally located gestational sac, our patient did not have any pain during the physical examination. She admitted that her last menstrual period was different from her usual menstrual periods. Because CSP or cervical pregnancy was suspected, we performed computed tomography (CT) for a definitive diagnosis. The CT scan showed an intrauterine gestational sac in the lower uterine segment bulging through the anterior uterine wall at the site of the cesarean scar. No invasion of the urinary bladder was observed (Fig. ). On presentation, her β-human chorionic gonadotropin (β-hCG) level was 66,536.8 IU/L (Day 1). Initially, we injected 50 mg of methotrexate (MTX) mixed with 9 mL of normal saline in the amniotic sac through a 22-G needle transabdominally under USG guidance. Simultaneously, 2 ml of amniotic fluid was aspirated for termination of the pregnancy. However, fetal cardiac activity was still observed 2 days later (Day 3), without significant changes in the serum β-hCG levels (65,342.5 IU/L). We decided on laparotomy instead of laparoscopy because of the large CRL (Day 4). The intraoperative finding showed bloody amniotic fluid, blood clot, placenta, and a fetus at the lower segment of the uterus. A transverse uterine incision was made at the lower segment of the uterus (Fig. ). The gestational sac was removed, as well as most of the trophoblastic tissues that were adherent and invading the wall of the lower uterine segment. The fetus and placenta showed no definitive abnormalities (Fig. ). The estimated blood loss was 1.2 L at intra-operation, without immediate complication. The uterine defect was repaired into two layers by using 2–0 Vicryl sutures. Our patient received 3 units of packed red blood cells (PRBC) at the ward postoperatively. The serial β-hCG level was 1958 IU/L at 4 days after the surgery (Day 8). She was discharged in good condition 5 days after the operation (Day 9). After 1 month (Day 39), her β-hCG levels returned to normal (2.8 IU/L). She was very satisfied with the fact that she had recovered well without the need for intensive care or further treatment without the need for hysterectomy.
A 40-year-old woman with a mass in her breast and without any specific medical history underwent right muscle-preserving radical mastectomy and level III lymphadenectomy. A pathological study indicated that the mass was an infiltrating duct carcinoma and that there was extranodal invasion. Therefore, she was treated with adjuvant chemotherapy that included adriamycin, cyclophosphamide, paclitaxel, and herceptin; however, after the first course of chemotherapy via the left brachial vein, she experienced peripheral phlebitis of the left brachium and severe nausea. She underwent CVPC implantation surgery at the right subclavian vein using a percutaneous needle puncture approach based on anatomical landmarks without ultrasound guidance. Chemotherapy administration via CVPC was started two months after the implantation. However, infusion resistance and skin swelling on administration of the anticancer drugs via the CVPC appeared nine months after CVPC implantation.\nShe was asymptomatic; however, chest radiography revealed that the catheter had dislodged, and computed tomography confirmed that the catheter was stuck to the right pulmonary artery and indicated that it was straddling the right main pulmonary artery (). She was referred to our cardiovascular department for removal of the dislodged catheter percutaneously. She agreed to our percutaneous strategy and signed an informed consent form.\nWe initially inserted an 8-Fr sheath via the right femoral vein. We then administered 5,000 U of heparin after sheath insertion and maintained the activated clotting time between 200 and 300 s. As we performed the procedure immediately after the diagnosis, pre- and postadministration were not performed. Thereafter, we moved an 8-Fr Judkins Right guiding catheter (Mac1, Boston Scientific, Natick, MA, USA) to the right pulmonary artery with a Berman catheter (Harmac Medical Products, Buffalo, NY, USA) and performed pulmonary angiography (). One end of the dislodged catheter was stuck to the upper pulmonary artery branch, and the other end was in the lower pulmonary artery branch. Digital subtraction angiography showed no flow in the upper pulmonary artery branch, indicating that the dislodged catheter was completely stuck without any gap. We initially attempted to catch it with a single-loop snare (Amplatz gooseneck snare, Covidien, Dublin, Ireland) and triple-loop snare (EN Snare, Merit Medical Systems, South Jordan, UT, USA); however, we could not grasp the catheter because the acute tapering shape of the pulmonary artery caused difficulty in wire control and the complicated branching prevented the wires from entering the same artery. We then attempted removal with the entwined guidewire technique (crossing multiple guidewires in the same vessel and twisting together to entwine with the dislodged catheter), which is similar to the procedure for retrieving foreign bodies in the coronary artery; however, the procedure failed owing to the same reasons mentioned previously. We realized that selection of the correct distal pulmonary artery was very difficult and that we should use a proximal pulmonary artery. We decided to pull the end of the dislodged catheter from the distal pulmonary artery to the right main pulmonary artery and catch it with a gooseneck snare. We used a pigtail catheter to tug the dislodged catheter. We inserted an 8-Fr Judkins Right guiding catheter near the dislodged catheter, delivered the pigtail catheter straightened with a 0.035-inch guidewire to the distal pulmonary artery, rolled the pigtail catheter by drawing out the 0.035-inch guidewire, and pulled back the pigtail catheter and guiding catheter together. After several attempts, this procedure successfully freed the end of the dislodged catheter in the right main pulmonary artery (). Thereafter, we easily grasped the body of the dislodged catheter (). As we did not fix the grasping point considering unintended release, the dislodged catheter was replicated during pulling back in the vein. We were unable to place the catheter in the femoral sheath; therefore, we removed the catheter and sheath simultaneously. Hemostasis was easily achieved in a few minutes with manual compression, and no complications were noted.\nAfter removal of the dislodged catheter, the old central venous port was retrieved and a new port was implanted at the right jugular vein to avoid recurrence of catheter POS. The patient has been free from any adverse event for three months. She provided consent for the publication of this case report.
We present a clinical case report of a 34-years-old pregnant woman with a single gestation. An omphalocele enclosing mixed intestinal and hepatic content was diagnosed at 12th week (). Nuchal translucency (NT) was normal, and no other associated malformations were observed. Parents were counseled regarding ultrasound findings. A genetic study was performed including karyotype, 60K Array-CGH, and methylation-sensitive multiplex ligation probe analysis to analyze the presence of epigenetic and genetic changes related to BeckwithWiedemann Syndrome (BWS), all of them being negative. Parents were informed of the results, as well as the impossibility of ruling out BWS completely despite the negativity of the genetic study, since this disorder is caused by epigenetic defects and there are up to 20% of cases whose diagnosis is clinical and therefore postnatal. After counseling, the patient decided to continue with gestation. The 20th week anomaly scan showed no associated malformations, except a slight upward and leftward heart displacement due to diaphragm elevation. Cardiac function and ductal venosus flow were normal throughout gestation and the heart was structurally normal. At 21st week, the omphalocele content became only hepatic (). Ultrasound controls were scheduled every 4 weeks to rule out the early onset of fetal growth restriction, as well as other disorders. At 37th week the estimated bag size was 51 × 56 mm (). At 38.3 weeks, the patient was admitted because of mild but regular contractions. At 38.4 weeks, a caesarean section was performed with the coordination of the Obstetrics, Neonatology, Anesthesiology, and Pediatric Surgery teams. The newborn weighed 3540 g, Apgar Test 9-10, and pH 7.32 (). A thermal bag covering the whole baby's body was placed immediately after the birth to maintain body heat and reduce the risk of infection (). The baby was admitted in the Neonatal Ward for management and surgical preparation in the first hours, starting parenteral nutrition after childbirth. At 48 hours of age, he underwent surgery to repair the omphalocele. A primary closure of the herniation was the first choice, though mesh repairing using a synthetic patch was considered depending on the intra-abdominal pressure (IAP), which was monitored throughout the intervention. As IAP was 15 cm H2O, a primary closure of the defect was performed (Figures , , and ). The newborn evolved favorably. He remained relaxed with a rocuronium perfusion for 72 hours after surgery. Analgesia with fentanyl and midazolam perfusion was administered for 6 days after surgery. Intubation was removed 5 days after surgery. Oral feeding was introduced from the 6th day of the procedure. Parenteral nutrition could be removed after 15 days of life. Intestinal transit was normal on the 6th day of surgery. At 3 months of age, two inguinal hernias were diagnosed and repaired surgically (). During the exploration of the left herniated sac, the ileocecal junction and the appendix were observed inside. Due to this abnormal location, a prophylactic appendectomy was performed (). Currently, the baby is one year old and he is alive and well ().
An 18-year-old female was admitted to the surgical department with a two-week history of colicky epigastric abdominal pain, indigestion, and multiple episodes of emesis. Physical abdominal examination revealed tenderness of the epigastrium and a palpable epigastric mass which was misdiagnosed as an epigastric hernia. Patient's vital signs were normal. Rectal examination did not show any abnormal pathology. Laboratory blood tests revealed only a mild microcytic anemia and a mild leukocytosis. Multiple investigations were performed. Abdominal plain X-ray was not indicative and magnetic resonance imaging (MRI) showed two well-defined T2 hypointense and T1 isointense filling defects mass lesions within the stomach. The larger of these within the body of stomach measured approximately 100 × 50 mm and the smaller of these in the gastric fundus measured 40 mm, differentiating between gastric leiomyoma and a bezoar (). History was positive for previous trichophagia and negative for other eating disorders.\nAn upper GI gastroscopy test was recommended and the patient was transferred to a nearby upper gastrointestinal unit for further management where she was investigated additionally with Computed Tomography (CT) () and had a laparotomy and removal of the gastric trichobezoar through a gastrotomy. The patient was discharged 4 days post-op.\nTwo weeks postoperatively the patient presented with generalized cramping abdominal and intermittent back pain, nausea, and vomiting undigested bowel content all consistent with a small bowel obstruction.\nOn examination a distended abdomen was apparent with a palpable, hyporesonant mass in the right lower quadrant region. Abdominal X-ray was not indicative of bowel obstruction; however, CT scan imaging identified a high grade small bowel obstruction which was due to an intraluminal mass ().\nThe patient underwent an urgent laparotomy and a residual trichobezoar mass was found in the jejunum which was removed by enterotomy (). Careful examination of the whole bowel was performed to ensure that no other satellite bezoar masses remained. The patient was discharged 5 days postoperatively after an uncomplicated recovery.
A 39 year old man was admitted to the emergency department with paraesthesia in all limbs, diplopia, and nausea since two days. Within a few hours the patient developed weakness in both the lower and upper limbs. His neurologic examination revealed bilateral abducens nerve paralysis, facial diplegia, flaccid areflexic paralysis of the limbs (corresponding to Medical Research Council grade 3/5 in all muscles of the upper extremities and 2/5 in lower extremities) and bilateral flexor plantar responses. The bowel and bladder were not initially involved. The patient underwent electromyography examination with the possible diagnosis of GBS. The electromyogram confirmed an acute, acquired, disseminated polyneuropathy syndrome involving sensory, motor and autonomic fibers, with prolongation of the distal motor latency, prolongation of the F-wave, decreased sensory and motor nerve amplitude, disperse responses and reduced nerve conduction velocities. The cerebrospinal fluid revealed an elevated protein concentration with a normal cell count. Anti-ganglioside antibodies were negative. Intravenous immune globulin (IVIG) was then administrated at a dosage of 0.4 mg/kg/daily. The same day weakness progressed rapidly in his all extremities, rendering him unable to stand due to total loss of strength in all muscle groups. The 6th and 7th cranial nerves were involved in both sides, and respiratory muscle weakness appeared. This progressed to a respiratory failure that required mechanical ventilation. The patient remained in the intensive care unit for two months. IVIG was administrated again over 5 days for a total dose of 2 g/kg body weight, after five sessions plasmapheresis performed on every other day. Treatment with a booster IVIG (0.4 gr/kg/day) was continued every 15 days. Rehabilitation was also performed from the beginning of the disease. In addition oral prednisolone was administered at 1mg/kg/day, which was reduced by 5 mg every two weeks.\nTwo months after the onset of GBS, pain and decreased range of motion (ROM) emerged in both hips. NHO was diagnosed on the plain X rays of the pelvis. NHO was visible on the anteromedial and anterolateral aspects of both femurs (). The serum calcium was 10.5 mEq/L (normative range 8.6-10 mEg/L) and the serum alkaline phosphatase was 61 IU/L (40-129 IU normative range). Intravenous ibandronic acid 150 mg was given weekly until the patient began to take orally. IV ibandronic acid was discontinued and etidronate disodium was administered 10mg/kg for 10 weeks. ALP and Ca levels were measured periodically. The rehabilitative management continued, including passive and active - assistive ROM exercises for major joints, breathing exercises and electrotherapy for all upper and lower limb muscles. When the patient was discharged from the ICU after two months, the muscle strengths improved in all extremities (grade 3/5 in the upper and 2/5 in the lower extremities). The patient could stand up with support; however could not walk or sit in low position due to pain and limitation in the movements of hip joints. Passive ROM of both hips was restricted to 40º of flexion and 20º of internal and external rotation. Three-phase bone scan study showed prominent Tc99m-MDP uptake in the acetabulum, proximal femur and trochanter bilaterally (). Under computed tomography guidance, corticosteroid injection was made to the periarticular portions of both hips (). The pain decreased and a 10º degree flexion was achieved after injection. At 6 months follow up, his rehabilitation program continues. He can walk with a walker, and sit without support.
An 86-year-old gentleman was referred from his general practitioner (GP) with a computed tomography (CT) proven large right inguinal hernia containing small bowel loops. His past medical history included hypertension and chronic obstructive pulmonary disease (COPD) for which he was on inhalers. Following the appropriate clinical examination and proper discussion, he was added to the waiting list for an elective open mesh repair.\nDuring the hernia repair, widespread black spots were noted on the hernial sac (Figure ). The hernial sac was transfixed, excised, and sent for histopathological assessment. Microscopic examination reported patchy dark black pigmentation in the sub-mesothelial tissue of the hernial sac with no evidence of dysplasia or malignancy (Figure ). Upon viewing with high-power microscopic examination (Hematoxylin and Eosin x 20), a granular black pigment in the sub-mesothelial tissue was observed. The overlying mesothelial lining appeared irritated and mildly hyperplastic (Figure ). In addition, Melanin pigment was not detected with Mason Fontana stain (Figure ). Immunohistochemical studies using CD68 highlighted several macrophages containing black pigment (Figure ). Based on these results, the diagnosis of melanosis peritonei was concluded.\nIt was noted that the patient was being followed up by the colorectal team because of a 25 mm distal sigmoid polyp which was endoscopically resected four months prior to his hernia procedure. Histopathological assessment of the polyp reported a moderately differentiated adenocarcinoma with a <1mm clearance margin. A computed tomography (CT) scan of his chest, abdomen, and pelvis was performed as part of the staging of his sigmoid cancerous polyp and this showed no evidence of lung, liver, or bony metastases.\nThe colorectal multidisciplinary team earlier concluded that there was more than a 20% risk of having residual disease and that would favour the need for undertaking a sigmoid colectomy or high anterior resection depending on his fitness.\nDue to his age, comorbidities, and his baseline fitness, the patient eventually decided not to proceed with surgical resection. Therefore, a follow up was arranged in the form of regular carcinoembryonic antigen (CEA) levels, lower gastrointestinal (GI) endoscopy, and a repeat CT scan in six months.
A 56-year-old Caucasian woman was referred to our hospital after a chest X-ray showed a dense mass in the left middle lung (). The patient had been complaining of increasing dyspnea during exercise since one year and symptoms of chronic bronchitis for several weeks, which was resistant to therapy. No family history of cancer was reported and the patient had no history of smoking. Clinical examination was without any pathological findings except for dullness and reduced breath sounds over the right lung. Chest computed tomography (CT) showed a 3.0 × 5.0 cm intrathoracic tumor, which was surrounded by pneumatocele ( and ). This seemed most consistent by morphological criteria with an extrapulmonary, mediastinal benign solitary fibrous tumor. No metastases where found in lymph nodes or distant organs.\nBronchoscopic examination revealed a constriction of the left upper lobe but no endobronchial tumor. CT-guided percutaneous needle biopsy was performed and histological H&E examination at the Institute of Pathology Medical University Innsbruck showed a monomorphic cell rich spindle cell proliferation with mild nuclear atypia. The characteristic H&E morphology of the core biopsy together with homogenous vimentin positivity, bcl-2 positivity and focal keratin (CAM 5.2, CK7, CK19) as well as EMA positivity together with the absence of S-100 Protein (rules out malignant peripheral nerve sheath tumor with focal keratin expression), calretinin (rules out mesothelioma together with the clinical picture and the negative history for asbest exposure) and CD34 (rules out solitary fibrous tumor) led to the diagnosis of a monophasic synovial sarcoma.\nDiscussion at the multi-disciplinary bone and soft tissue tumor board (MUI) as well as in the lung and mediastinal tumor board (MUI) decided to do primary surgery as there was no spread of disease, no metastases on X-ray and CT. The tumor was surgically resected by thoracotomy (). The mediastinal mass was in close association with the phrenic nerve, which had to be resected together with a fragment of the diaphragm. The defect was closed with interrupted sutures. On gross examination it presented as a polycyclic well circumscribed with 7 cm in greatest diameter. The cut surface was whitish-grey and of soft consistency. Histological and immunohistochemical examination confirmed the diagnosis of synovial sarcoma ( and ). The final tumour was staged as pT2b N0 M0 R0. The patient recovered without any complications from surgery and received four cycles of adjuvant chemotherapy (100 mg Doxorubicin and 3000 mg Ifosfamid) and thoracic radiotherapy two months later. The patient is currently free of recurrence after a follow-up of 16 months.
A 40-year-old gravida five, para four woman presented for workup and management of abnormal uterine bleeding. Her past medical history was significant only for hypertension and anemia. On review of her social history, she admitted to drinking six packs of beer on the weekends but denied further substance use. She denied previous treatments for her bleeding including any previous intrauterine device usage.\nUltrasonography revealed a 7 cm fundal fibroid with otherwise normal pelvic anatomy. She was initially offered medical management of her bleeding. She declined any medical treatment and strongly desired definitive surgical treatment. She then underwent a total vaginal hysterectomy with adnexal conservation. Due to the large size of the uterus, a myomectomy was performed to facilitate vaginal removal. Her postoperative hospital course was relatively uncomplicated and she was discharged home on postoperative day three.\nOn postoperative day ten, she presented to the Emergency Department (ED) for fever, worsening abdominal pain, and new onset of nausea and vomiting. In the Emergency Department, she was tachycardic and tachypneic but afebrile. Her exam was significant for abdominal tenderness to minimal palpation, vaginal cuff erythema, and significant tenderness to palpation of the vaginal cuff. Lab work showed an elevated white blood cell count. She was admitted for management of presumed pelvic infection.\nA CT of the abdomen and pelvis was obtained and showed a 6.2 x 9.7 cm pelvic abscess adjacent to the vaginal cuff (Figures and ).\nInterventional Radiology placed a drain into the abscess and the patient was started on IV piperacillin/tazobactam. She was transitioned to oral amoxicillin/clavulanate potassium after four days on intravenous antibiotics and her drain was removed on hospital day 5. Vaginal wound cultures remained pending; however, due to continued clinical improvement on the oral antibiotic regimen, she was discharged home on hospital day 5 with a two-week course of amoxicillin/clavulanate potassium.\nThe patient then returned for her outpatient visit approximately one week later. The results of the vaginal wound cultures revealed a large growth of Actinomyces meyeri. The patient's case was discussed with an Infectious Disease (ID) specialist who recommended an additional two-week course of amoxicillin/clavulanate potassium.\nThe patient then returned to the ED on postoperative day 25 for pleuritic chest pain with mild cough but denied gynecologic complaints. She reported compliance with the oral amoxicillin/clavulanate potassium regimen. Exam and lab work were unremarkable. A chest X-ray showed left basilar heterogeneous opacities, likely subsegmental atelectasis. A CT angiogram was obtained due to concern for a possible pulmonary embolism (PE). The imaging was negative for a PE; however, it was concerning for possible pneumonia. The patient was discharged home with a five-day course of levofloxacin for treatment of pneumonia.\nOn postoperative day 27, the patient represented to the ED with worsening shortness of air and chest pain. Again, she reported compliance with her antibiotic prescriptions. Exam and lab work were again unremarkable. A repeat chest X-ray showed a slight progression of right basilar heterogeneous opacities thought to be infectious. Her antibiotic regimen was again discussed with ID specialists and an intravenous antibiotic regimen was felt preferable to an oral antibiotic course. She then completed an outpatient two-week course of IV ampicillin/sulbactam as recommended.\nOn postoperative day 37, a repeat CT of the abdomen and pelvis showed near complete resolution of the previous pelvic abscess. HIV testing was obtained and returned negative result. She reported significant improvement of her symptoms. She was placed on a six-month course of oral amoxicillin per ID recommendations with plans for continued follow-up in their clinic, as well as with gynecology. She has not shown any signs of recurrent infection after approximately 1 year of follow-up.
In August 2008, a 44-year-old female had undergone surgery for resection of a malignant melanoma in the right lower leg and a right inguinal metastatic lymph node (Fig. ), followed by chemotherapy with doxorubicin, adriamycin, vincristine, and interferon beta (DAV-feron). In March 2012, computed tomography (CT) revealed brain and lung metastases, so the patient began radiation therapy to treat these lesions.\nIn September 2012, the patient was admitted to our hospital for back pain. Abdominal CT and magnetic resonance imaging detected new multiple hepatic metastases of melanoma. A transcatheter arterial infusion of cisplatin was administered, and transcatheter arterial embolization (TAE) was performed. In October 2012, she began treatment with vemurafenib, based on the finding of a positive BRAF V600E mutation in the resected primary site of the skin, which was analyzed by direct sequencing analysis using DNA from the paraffin-embedded primary cutaneous melanoma. She tolerated the treatment remarkably well, and the size of the multiple hepatic and lung metastases decreased, while the size of the brain metastases did not. In addition, the serum concentration of 5-S-cysteinyldopa (5-S-CD), a biological marker of melanoma progression, was also decreased from 40.1 ng/mL to 5.2 ng/mL.\nIn December 2012, she suddenly developed severe abdominal pain. Abdominal CT revealed ruptured hepatic metastases accompanied by massive intra-peritoneal hemorrhage. A retrospective and sequential analysis of the CT images suggested that a part of the liver metastases had enlarged rapidly and then ruptured with intratumoral hemorrhage during vemurafenib treatment (Fig. ). An emergency TAE was performed by selective occlusion of the right hepatic artery using gelatin sponge particles. The postoperative course was uneventful for several days. However, on the 26th postoperative day, she developed hemorrhagic shock and died of renewed intra-abdominal bleeding.\nAn autopsy examination revealed hemoperitoneum due to rupture of the liver metastases. Metastases were also discovered in the brain and lungs as well as in the kidneys, adrenal gland, and lymph nodes, although these had not been detected on imaging while she was alive. There was also massive bloody ascites (1700 mL). The background liver was completely normal, whereas exposed necrotic tissue and intratumoral hemorrhage were observed at the site of tumor rupture (Fig. ). We concluded that the cause of death was hemorrhagic shock from ruptured hepatic metastases of malignant melanoma. Finally, for improved understanding of the mechanism of refractory metastasis, we conducted an immunohistochemical analysis of the signal transduction molecules, phosphorylated extracellular signal-regulated kinase (p-ERK), and phosphorylated Akt (p-Akt), as well as the melanocyte marker Melan-A and Ki-67 in tumor cells of the primary malignant melanoma obtained from the right lower leg and in hepatic and lymph node metastases obtained on autopsy (Fig. ). Our findings showed that hepatic and lymph node metastases were positive for p-ERK and negative for p-AKT, even though the primary tumor was negative for both.
A 48 year-old male of South Asian descent came to Emergency Room with pain and swelling of right RT leg and fever. He had suffered a road traffic accident 10 days ago and suffered trauma to RT leg. Patient was admitted in a General Hospital and treated. Above knee slab was applied. On the second day, patient developed blisters over the thigh, knee, and leg (). He was advised amputation of RT leg. He came to our hospital for further management. On physical examination, his RT leg was edematous with multiple blisters. Pulse oximetry showed 98% saturation. Doppler study showed normal flow. Radiographs were taken and patient was found to have proximal tibia fracture Schatzker’s type VI () Blood investigation showed marked increased in white blood cells, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP). There was increase in serum potassium andserum creatinine levels. Labroratory Risk Indicator for Necrotizing Fasciitis (LRINEC) score [] was found to be more than 8. Patient developed acute kidney injury and was required to undergo two cycles of dialysis. Patient was started on i.v. cefuroxime and gentamicin after culture sensitivity reports andrenal titration. Patient underwent surgical debridement and knee spanning external fixator (). Plastic surgery opinion was taken for soft tissue reconstruction. Thorough debridement was done. A second surgical debridement was done by the plastic surgeon after 2 weeks (). External fixator was removed as it was hindering with flap cover. Hence, two 4mm cannulated cancellous screws with washers were used to fix the articular fragments of proximal tibia and above knee slab was applied (). Patient was shifted to plastic surgery department for flap cover after 1month of admission (March 2019). We were advised to wait for 4 months for the flap to mature. Patient again came with pain, swelling in RT leg and fever. ESR and CRP was found to be raised. Flap was raised along its margin necrotic tissue was removed and antibiotic (vancomycin 1.5g) cement spacer was kept and limb reconstruction system (LRS) was applied in July 2019 (). LRS was applied spanning the joint as the joint was open. After 6 weeks patients ESR and CRP were repeated and found to be within normal limits. Antibiotic cement spacer was removed and bone grafting was done ( ). Patient was reviewed every month for clinical and radiological evaluation. In March 2020 patients X-rays showed consolidation of the graft radiologically, dynamization of LRS was done ( ). Patient was asked to partially weight bear with walker support. LRS removed in August 2020.At final follow–up, patient had an active knee flexion of 15 degrees.
A 32-year-old male attended the emergency eye clinic, complaining of the sudden reduction in vision, intense pain, watering, and swelling of the lids following blunt trauma to his right eye (RE) sustained 6–8 h before presentation. On examination, the vision in RE was a perception of light with good projection in all quadrants. Examination of the RE revealed lid edema, ecchymosis, subconjunctival hemorrhage, full thickness central cornea tear with the expulsion of the iris, and full chambered hyphema. The patient had corneal rupture [zone 1, open globe injury as per Birmingham Eye Trauma Terminology classification system] due to accidental slippage over the corner of a plastic table at home. There were no pathologic findings in the left eye and had best-corrected visual acuity of 20/20 at the time of presentation.\nInitial management of this patient included the administration of analgesia, intravenous antibiotics, and tetanus prophylaxis. An eye shield was used for protection to prevent further accidental trauma, and the patient was subsequently prepared for wound repair. Preoperative B-Scan ultrasonography was avoided in this patient with the fear of expulsion of intraocular contents. The risks and benefits of general anesthesia and alternatives of all anesthetic options explained clearly to the patient. The patient refused to do surgery under GA; therefore, the regional anesthesia was preferred in this patient. A high-risk consent explaining the extremely guarded visual prognosis was obtained from the patient before the primary repair of RE. Reconstruction of the right anterior segment was performed under local anesthesia. Incarcerated iris was dissected from the wound, and remaining iris was retroplaced into the anterior chamber. An anterior vitrectomy was performed and 10-0 nylon sutures were used for wound closure. Pupillary aphakia was noticed; therefore, posterior dislocation of crystalline lens was assumed intraoperatively. Postoperatively, intraocular pressure was persistently in the higher range despite the prompt use of systemic and topical antiglaucoma medication. The patient had severe corneal edema due to secondary glaucoma precluding fundal view and further management. B-scan ultrasonography of the RE revealed vitreous hemorrhages and total retinal detachment. The patient was treated with contact laser diode cyclophotocoagulation (DLCP) to the superior and inferior half of the ciliary body of the RE. Twenty-five diode laser burns of 2000 ms duration and using a power of 2000 mW were applied. The patient had persistently raised intraocular pressure, complaints of severe pain, discomfort, and the visual acuity of no perception of light even after DLCP. A diagnosis of a painful blind eye was made. Due to severe pain, discomfort, and nil visual prognosis in RE, the patient preferred enucleation with silicone ball implant. The patient was properly explained about the procedure, and proper informed consent was obtained prior of enucleation. The specimen was sent for histopathological examination. Left eye remained unremarkable with a best-corrected visual acuity of 20/20.\nOn gross examination, the enucleated eyeball measured 22.13 mm anteroposteriorly, 23.87 mm horizontally, and 21.73 mm vertically. The cornea was opaque with vascularization. The lens was opaque and migrated in subretinal space inferiorly with retinal vessels seen over the lens []. Adjoining retina was detached, and giant retinal tear was noted. This portion of the retina and the cataractous lens was dissected and seen under a compound microscope and documented for the subretinal migration of lens. Flat preparation of the retina showed nerve fiber layer hemorrhages [] and subretinal lens with the overlying retinal blood vessels []. High-resolution images under the compound microscope also revealed focal retinal pigment epithelial (RPE) atrophy overlying the crystalline lens [] and also showed a portion of the retinal tear, through which probably, migration of lens occurred []. The RPE layer was studied, which showed mostly healthy cells [] with an occasional atrophic changes near the subretinal migrated crystalline lens [].\nMicroscopic examination under hematoxylin and eosin stain revealed corneal stromal scarring with an occasional stromal vascularization []. The angle of anterior chamber showed cyclodialysis cleft []. Iris details revealed ectropion uvea and neovascularization []. Retina showed detachment with superficial nerve fiber layer hemorrhages [].
A 58-year-old man (fair skinned with a history of sun exposure) presented in 2016 for evaluation of newly appearing scaly red plaques on his left shoulder and left clavicle. His past medical history was significant for not only multiple primary cutaneous basal cell carcinomas, but also metastatic basal cell carcinoma with involvement of the brain, bone, liver, and lungs. The new skin lesions had appeared in the setting of near complete remission of his metastatic disease on continued nivolumab treatment; the successful management of his metastatic disease—prior to the onset of his recently developed cutaneous tumors—has previously been described []. This study was performed and consents obtained in accordance with UCSD IRB guidelines (NCT02478931).\nThe history of the present illness reveals that, in 2012, he developed a basal cell carcinoma on his left posterior shoulder. The tumor was excised and recurred. The subsequent postoperative wound margins were positive for carcinoma, and the site was treated with radiotherapy. Two years later, in 2014, an evaluation of back pain revealed metastatic basal cell carcinoma not only to his axial skeleton, but also to his liver and lungs. The diagnosis was confirmed with biopsies from the bone and liver. A soft tissue specimen of metastatic tumor also demonstrated basal cell carcinoma.\nIn August 2014, he started vismodegib. The treatment was discontinued after three months when he developed brain metastases. Following successful stereotactic radiosurgery, he began cisplatin and paclitaxel in November 2014. In March 2015, after four cycles of chemotherapy, the drugs were discontinued secondary to progressive bone and liver metastatic disease. Sonidegib combined with buparlisib (a pan-class I PIK3 inhibitor) was started in May 2015, and was discontinued six weeks later after disease progression in the liver. He received vismodegib and paclitaxel from July 2015 to September 2015; they were discontinued secondary to a lack of response.\nHybrid capture-based next generation sequencing (236 genes) was performed by Foundation Medicine on a liver tumor biopsy specimen from July 2015 (; Available online: ) []. The sequencing demonstrated a tumor mutational burden of 103 mutations per megabase (reference: >19 mutation per megabase = high tumor mutation burden) and multiple genomic alterations, including an amplification of PD-L1, PD-L2, and JAK2. In October 2015, based on the high tumor mutational burden and PD-L1 amplification, both of which are associated with a response to checkpoint inhibitors, he was started on the checkpoint inhibitor nivolumab at 240 mg intravenously every 2 weeks, with a remarkable and rapid improvement of performance status, and tumor shrinkage to near complete remission.\nHe presented with new erythematous plaques on his left anterior shoulder (6 mm × 6 mm) and left chest (8 mm × 6 mm) in June 2016 (). He was still receiving nivolumab, and the near complete remission of his metastatic basal cell carcinoma was ongoing. Biopsy specimens from both skin lesions showed similar pathologic changes, confirming the diagnosis of superficial basal cell carcinoma: superficial buds of basaloid tumor cells extending from the overlying epidermis into the dermis ().\nNext generation sequencing of the specimen from his left anterior shoulder primary cutaneous basal cell carcinoma was performed (). The sequencing demonstrated a tumor mutational burden of 45 mutations per megabase and eight characterized genomic alterations. In contrast to the metastatic basal cell carcinoma in his liver, the primary skin cancer did not demonstrate amplification of PD-L1, PD-L2 or JAK2.\nThe superficial basal cell carcinomas on his left anterior shoulder and left chest were each treated with electrodessication and curettage. A follow up examination in February 2017 showed complete healing of the treated skin cancer sites without tumor recurrence. He was still receiving nivolumab every other week, and his metastatic basal cell carcinoma continued to demonstrate over 95% regression on imaging.
We present a 77-year-old male with a past medical history of NHL/SLL diagnosed almost 10 years ago, who presented to the hospital with abdominal swelling, altered mental status, and difficulty in urinating associated with hematuria. On physical examination, diffuse bulky lymphadenopathy was found in the cervical, axillary, and inguinal areas. Detailed oncologic history and treatment regimens that were taken by the patient have been well explained in Table .\nLaboratory workup showed hypokalemia, hypophosphatemia, and elevated lactate dehydrogenase levels. During the hospitalization, computed tomography (CT) scan head was done that showed negative findings for any acute events. Due to altered mental status with underlying worsening of NHL and metabolic disturbances, the toxic and metabolic encephalopathy were the differentials under consideration. Metabolic derangements were corrected during hospitalization and that improved his mental status as well. Idelalisib treatment was discontinued abruptly a week prior to patient’s presentation to the hospital due to pancytopenia and a mixed response on the CT scan imaging. Upon admission, a repeat CT of the abdomen and pelvis showed diffuse bulky lymphadenopathy in the abdomen; one of the nodes in the anterior para-aortic region was measured about 5 cm × 5 cm × 8 cm (Figure ). Bilateral iliac, inguinal, and retroperitoneal lymphadenopathy was also significantly increased in size compared with prior CT scan. There was diffuse lymphadenopathy along with axillary and cervical regions as well (Figures -).\nBone marrow biopsy was done that showed a cluster of differentiation 5 (CD5) and CD23 positive B-cell population (37% of the lymphoid gate), lambda-restricted. Almost all of the B-cells showed immunophenotypic expression of CLL/SLL with lambda light chain restriction that was found in previous cases of this patient. Interestingly, a kappa light chain restricted population of monoclonal plasma cells co-expressed with CD56 (1.1% of total events) is also identified. Urology was consulted for urinary complaints of difficult voiding and hematuria. However, the patient was further diagnosed with paraphimosis and scheduled to have an elective circumcision that alleviated his urinary complaints later. Idelalisib treatment for SLL/NHL that patient took for almost one year was stopped a week prior to current clinical symptoms. Based on progressive SLL/NHL, the patient was started on venetoclax (B-cell lymphoma 2; BCL-2 inhibitor) 20 mg/day for seven days and 50 mg/day afterward. The patient did not develop any tumor lysis syndrome after starting the therapy and is tolerating the treatment well based on both imaging and clinical finding.
A 42-year-old male was referred to his ophthalmologist regarding the complaint of sudden blurriness of vision in his right eye. He was diagnosed five months ago with a case of ENKTCL, nasal type, stage IV-B with testicular and central nervous system involvement with 23% natural killer (NK) cells on cerebrospinal fluid (CSF) flow cytometry. He was on an active therapeutic plan consisting of cycles of chemotherapy (i.e., SMILE protocol: dexamethasone, methotrexate, ifosfamide, L-asparaginase, etoposide), to which there was a complete response in the form of negative flow cytometry and computed tomography (CT) of the chest, abdomen, and pelvis. Five months after the initial diagnosis, prior to the fourth cycle of treatment, a complaint of blurriness of right eye vision was made. Brain CT was done aiming to rule out ocular involvement, which turned out to be unremarkable. At the time of the complaint, visual acuity was 20/30 -2 in the right eye as opposed to 20/20 in the left eye. The right eye showed relative afferent pupillary defect. Extraocular muscle movements were competent in both eyes. On examination of the anterior chamber, +1 cells were visualized in the right eye only. On dilated fundus examination, there was vitritis in the right eye which obscured the vision. Left eye examination was insignificant. B scan ocular ultrasonography revealed retinal detachment in the right eye. An optic CT revealed vitritis in the right eye; an impression of disease infiltration of the eye was made. The therapeutic plan was a diagnostic vitrectomy followed by systemic therapy. A pars plana vitrectomy was made and the vitreous specimen was sampled revealing the presence of 10% viable lymphoid cells expressing CD2 and CD56 on immunohistochemistry stain. An orbital magnetic resonance imaging (MRI) was ordered revealing evidence of an enhancing retinal lesion centered on the optic disc with diffuse restriction consistent with lymphomatous infiltration; minimal proptosis of the right eye was noted (Figure ). Fundus photography of both eyes was performed postoperatively with the retina flat under the silicon in the right eye as seen in Figure and minimal disease involvement of the left eye which further confirmed the diagnosis (Figure ). In addition, CSF flow cytometry revealed immunophenotypic evidence of disease (75% mature T cells and 20% NK cells expressing CD2, CD16 and CD56). Follow-up examination revealed 6/9 vision in the left eye, as opposed to no light perception in the right eye. The patient received 35 Gray units of radiotherapy to the optic apparatus and posterior globe of both eyes and the entire cranium down to the third cervical spine region. Post-radiotherapy MRI revealed interval regression of the disease in the form of resolution of bilateral optic disc infiltrative nodule. Despite medical efforts, the patient’s condition deteriorated, and he passed away.
A 53-year-old woman with no significant past medical history presented to the emergency department with a ten-day history of progressive, severe right neck and shoulder/scapular pain that was worse with movement and radiated down her right arm to her fingers. NSAIDs, range of motion exercises, and trigger point injections had provided only minimal relief. She denied recent trauma, had no infectious symptoms, and reported no smoking history.\nRight shoulder radiographs revealed a right superior mediastinal mass (). Further imaging with contrast enhanced CT showed a 7.2 cm well demarcated, heterogeneously enhancing mass with areas of fat attenuation and some prominent internal vessels within the right superior, posterior mediastinum (). The mass anteriorly abutted the brachiocephalic artery and trachea but did not invade adjacent structures. A cervical spine MRI obtained for cervical radiculopathy symptoms partially included the mass, which was heterogeneous, hyperintense to muscle on both T1 and T2 weighted sequences with a few internal flow voids, and showed moderate loss of signal with fat saturation ().\nMesenchymal neoplasms, lymphoma, and teratoma were considered in the differential diagnosis based on imaging. Microscopically, the CT-guided biopsy showed multivacuolated and granular eosinophilic cells with small centrally located nuclei consistent with hibernoma. No lipoblasts or cytologic atypia was seen to suggest liposarcoma. Due to the unusual location, large size, and possible mass effect on adjacent structures, total excision was performed by a right video-assisted thoracoscopy. The surgeon described a dense, fatty, lobulated mass located in the posterior mediastinum with extension into the lower neck and without direct invasion of surrounding structures. The resection specimen measured 8 × 5 × 3 cm. Grossly, it was described as a circumscribed, yellow to tan, lobulated mass (). Histologic sections demonstrated cytologically bland brown fat cells with abundant granular cytoplasm identical to the preoperative biopsy (). Due to their classic histomorphologic features, most hibernomas can readily be diagnosed without the use of ancillary studies. Cytogenetic studies have demonstrated rearrangements of 11q13; however, amplification of MDM2 should prompt consideration of an atypical lipomatous tumor/well differentiated liposarcoma. The patient was discharged on the second postoperative day.\nOn follow-up, the patient had transient right vocal cord paralysis, likely due to the close proximity of the resected mass to the recurrent laryngeal nerve, with return of full vocal cord mobility about one month after surgery. No CT evidence of recurrence was seen on a chest CT four months after surgery, and a chest radiograph fourteen months after surgery was normal. Thirty-two months after surgery, the patient remains asymptomatic.
The patient is a 57-year-old nulligravid female who presented to clinic with symptoms of urinary urgency incontinence. She had a past medical history of endometriosis, autoimmune hepatitis, cirrhosis, and denied any pertinent mental/psychological history or trauma. Her initial symptoms included urgency, voiding up to 5 times an hour, and nocturia up to five times per night. She had no previous vaginal surgeries. Baseline sexual function evaluation was completed at intake with the Female Sexual Function Index (FSFI) questionnaire where the arousal domain equaled 0.9 (range 0-6, with 6 indicating maximal arousal) (). Pelvic examination was significant for vaginal atrophy but no notable clitoral or labial abnormalities were visualized. There were no signs of prolapse or pelvic floor musculature hypertonicity. She was asked to keep a voiding diary and then advised to attempt timed voiding upon its completion. She was also started on vaginal estrogen and a generic anticholinergic agent (oxybutynin 10 XL daily). After 4 weeks of treatment, she experienced significant worsening of anticholinergic side-effects and stopped the medication on her own. She was then started on a beta sympathomimetic (mirabegron 25mg daily). After 6 weeks of this therapy, she did not have any subjective improvement in her symptoms. The dose was increased to 50 mg daily and continued for an additional 4 weeks. Again, she did not have an adequate reduction in symptoms and was counseled on third tier treatment options. Ultimately the decision was made to proceed with SNM.\nThe sacral neuromodulator was implanted per manufacturer instructions after undergoing a peripheral nerve evaluation with >50% improvement in her urgency symptoms. At one-week follow-up, her incisional pain was minimal. She did not require oral analgesics and had reported marked improvement in urinary symptoms consistent with the test phase.\nHowever, near the six-month follow-up appointment, she expressed concerns about persistent arousal symptoms in the vaginal area overall with new onset hypersensitivity localized to the clitoris. She did not have these symptoms prior to or immediately postimplant, but she reported gradual development of arousal symptoms postoperatively over the six-month period. She had not initiated the use of any new medications or therapies during the same time period.\nPelvic examination did not demonstrate engorgement of the clitoris, change from the intake examination, or evidence of hypertonic pelvic floor muscle dysfunction based on digital assessment. To manage her arousal, the four programs that were programmed into the system were alternated with cycling activated. Behavioral modifications were suggested including loose clothing. Upon no change in symptoms, device deactivation was performed. This resulted in no notable improvement of the manifest arousal symptoms. However, her urgency symptoms immediately recurred upon the deactivation. At this point, the plan was to trial a new set of programs to see if her arousal symptoms could be eliminated using different settings. The patient agreed to trial all four new programs, each over at least a 10-day period and assess which one was associated with fewer arousal symptoms. The programming was done at sensory levels. Alternation of pulse width and frequency was performed as well. The patient was instructed to complete a 4-week diary indicating arousal and bladder activity. At follow-up, she had trialed each program and continued to experience sexual arousal symptoms. Her symptoms were present even when the device was turned off and intensified when the machine was on. The symptoms were also present without any clitoral contact by undergarments. It was becoming so bothersome that it was difficult for her to stay asleep at night and creating anxiety. Complete testing of the neuromodulator unit was reperformed with normal values noted for impedance. Reprogramming of the unit with a new set of programs was performed again with subsensory levels used at this point. Additionally, she was offered sexual counseling but it was declined.\nOne year after placement, she elected for removal of the generator and lead. The generator and lead, intact with tip, were successfully removed with no complications. At the 6-week follow-up after explant, she had recurrence of urinary urgency symptoms with persistence of hyper-arousability. One year later, she continues to be sexually active with mild discomfort due to vaginal atrophy (as noted on the pain domain in the FSFI). She reports that the clitoris remains hypersensitive with persistence of the arousal symptoms, although moderately improved from prior to SNM. At the follow-up visits, no changes in medications or new medical diagnoses were reported when compared to prior visits. Furthermore, she denied any new stressors or change in her personal life regarding the relationship with her husband.
A 19-year-old African American male with severe hemophilia A and history of persistent factor VIII inhibitor, despite multiple attempts at immune tolerance induction, and maintained on weekly emicizumab prophylaxis presented with severe muscle cramps in his anterior pectoral regions without a clear source, highlighted by imaging completed on admission (). The patient had required change in prophylactic medication from factor eight inhibitor bypassing activity to emicizumab a year before, as he continued to have many hospitalizations for hemarthrosis, resulting in contractures and discordant musculoskeletal exam. On-demand factor replacements did not occur since the initiation of emicizumab. The patient had a job at a restaurant that involved moderate exertion. However, he had been working at the restaurant for a year without any symptoms until the hospitalization. On examination, he had diffuse muscle tenderness of his anterior pectoral regions. Laboratory studies showed hyperCKemia (). Emicizumab was considered as a likely candidate causing symptomatic rhabdomyolysis. Laboratory testing did not demonstrate endocrine pathology, the complete blood counts and metabolic panel were normal, the history was not concordant with toxin-based insult, and the rheumatology workup was unremarkable. An extensive infectious disease workup done including Hepatitis panel, human immunodeficiency virus, Epstein-Barr virus, and cytomegalovirus studies, all of which were normal. CRP was not elevated; urinalysis did not demonstrate myoglobinuria. Coagulation studies showed no abnormalities, consistent with emicizumab in a hemophilia patient. He was managed with intravenous fluids and oral opioids around the clock for 24–48 h, and then changed to ‘as needed’ medication. He required two doses of oral opioids after this transition in care plan, due to retention of anterior pectoralis muscle tenderness during the remainder of his 5-day hospital course.\nA metabolic myopathy and rhabdomyolysis 60 gene panel next generation sequencing was performed through a commercial laboratory. The panel comprised genes associated with mitochondrial fatty acid oxidation defects, glycogen storage diseases, mitochondrial myopathy genes, channelopathies, etc. The patient was found to have two heterozygous variants of unknown significance in the ETFDH and the RYR1 genes, respectively. Pathogenic variants in the ETFDH cause autosomal recessive glutaric aciduria type 2C. The ETFDH variant seen in this patient is in the heterozygous form and two pathogenic variants are necessary to cause disease. His variant, denoted as c.1049G>A (p.Arg350Gln) is present in the African population at a frequency of 0.2% and in the total population at 0.02%. This means this variant is ten times more common in the African population and may be a likely benign variant.
A 47-year-old previously healthy Sinhala female's right foot was bitten by a snake near the back door of her home in the Kegalle district, Sri Lanka. Within seconds, she felt burning pain ascending along that limb, and there was heavy bleeding from the site of bite. Within a couple of minutes, she felt dizziness, nausea, and numbness of the whole body, had profuse sweating and frothy salivation, and was screaming in pain from the site of bite. On the way to the nearby hospital, she started to clench her jaw tightly and limbs became rigid; she was frothing and was not responding for about 5 minutes, indicating a generalized seizure. She arrived at the hospital within 30 minutes. The doctor at the outpatient department decided to administer ASV and directed the patient to an internal medicine ward for that. Physical examination findings at the ward were a pulse rate of 100/minute and blood pressure of 150/90 mmHg, and lungs were clear to auscultation bilaterally with an arterial oxygen saturation of 95% whilst breathing air with no neurological deficit. By this time, the killed snake was brought in and doctors identified it as a HNV; thus, antisnake venom (ASV) was not administered. Even though there was bleeding at the site of the bite even on admission to the hospital, her 20-minute whole blood clotting time, platelet count, prothrombin time and international normalized ratio, and activated partial thromboplastin time and liver function tests were all normal. Urine sample obtained via the catheter showed 50–55 red cells per high-power field, arterial blood gases indicated a compensated metabolic acidosis, and serum sodium and potassium levels were normal. Her urine output was <100 ml for the first 24 hours and serum creatinine rose from 80 μmol/l to 277 μmol/l. She was transferred to the Teaching Hospital, Kandy, on day 2 for further management.\nOn day 2, a bulla developed at the site of the bite, and there was an edema and warmth at the right foot. Complete (full) blood count demonstrated neutrophilic leucocytosis, and the CRP level of the following day was 261 mg/l. Intravenous antibiotics was started to cover the wound infection. Serum creatinine was 377 μmol/l with oliguria on day 2. Serum sodium and potassium levels remained within the normal range from day 1–5. On the day 5, creatine kinase was 75.1 U/l. Regular hemodialysis every other day from day 2 to day 24 and fluid management were started. Oral sodium bicarbonate was started, and management of her acute kidney injury with collaboration of nephrology team continued.\nOn day 3, her blood pressure rose to 160/90 mmHg, and it was controlled by prazosin and nifedipine SR; however, it generally remained on or above 140/90 mmHg until her discharge. She developed bilateral lung crepitations on day 3 that remained for 7 days. She developed bilateral parotid swelling and edema of the right leg on day 3, and it lasted 3 days. Edema below her right knee persisted another 10 days. Her blood picture on day 2 did not show hemolysis and was suggestive of bacterial infection but blood picture on day 5 showed evidence of microangiopathic hemolytic anemia (MAHA), and same changes were there in a blood film taken on day 11, as depicted in .\nHer day 2 hemoglobin level of 10.8 g/dl dropped to 8.4 g/dl on day 5. On day 2, her platelet count was 104 × 109/l and that dropped to nadir of 29 × 109/l in day 6 and was <150 × 109/l until day 20. A consultant in transfusion medicine has assessed her, and blood transfusion and plasmapheresis was performed on day 7. Another four cycles of plasmapheresis followed. Local edema at the site of the bite increased with necrosis (); thus, wound debridement was done on day 7 and followed up by regular wound toilets.\nWe did an electroencephalogram (EEG) on this patient on the earliest available day (day 11) and that was normal. The 2D echocardiogram done on day 17 was also normal.\nThe offending snake's carcass was taken to the Peradeniya University, and an expert on HNV, Dr. Kalana Maduwage, has confirmed it as a Hypnale hypnale. is a photo of the offending snake.\nAs her daily urine output improved to >1000 ml, she was discharged on day 30 and asked to come for a review in five days. She defaulted treatment and was on alternative medication. After developing progressive bilateral ankle edema and exertional dyspnea, she came back again on day 46, and hemodialysis and supportive therapy were restarted at the nephrology unit. On day 49, she had an anterolateral non-ST-elevation myocardial infarction (non-STEMI), and she was managed at the cardiology unit. She had progressive impaired vision of the left eye starting from a few days after the snakebite and could not count fingers held 30 cm in front of that eye on the 46th day. She was referred to the eye unit, there was bilateral optic disc edema more on the left, the patient was diagnosed of left anterior ischemic optic neuropathy (AION), and steroid therapy was started. Her erythrocyte sedimentation rate and contrast-enhanced computed tomography (CECT) brain done on day 53 were normal. is a photograph of fundi of this patient.\nShe had two episodes of seizures on day 76, and we suspected a possible relationship to her envenomation. The opinion of the neurology team regarding three seizures was obtained. Repeated EEG and CECT brain were normal. Despite being on calcium carbonate 500 mg plus 0.25 μg 1-alpha-hydroxycholecalciferol daily from day 46, her serum calcium level was low (1.8 mmol/l). Last two seizures were attributed to hypocalcemia due to chronic kidney disease following HNV envenomation, and daily calcium carbonate dose was increased to 500 mg thrice daily. After three months, she was diagnosed of end-stage renal disease by nephrology team and on hemodialysis once in four days and was searching for a kidney donor at six months.
A 23-year-old Caucasian man initially presented with new-onset generalized tonic-clonic seizures. He was found to have a non-enhancing lesion in the left frontal lobe and underwent resection which demonstrated a low-grade astrocytoma. Evaluation of IDH1/2, ATRX, TP53, and BRAF status was not performed, and this specimen is not currently available for our pathologic review and genetic analysis. Dermatologic evaluation did not demonstrate any café-au-lait macules or axillary or inguinal freckling but did reveal scattered melanocytic nevi. Additionally, no Lisch nodules or cutaneous neurofibromas were present. His mother is alive without a personal or significant family history of neoplasia. His father has a history of multifocal high-grade epithelial dysplasia of the oropharynx as well as resection of squamous cell carcinoma from the oral cavity. His sister died of glioblastoma at age 14, and his paternal grandfather had a history of cutaneous melanoma and died of brain cancer. Two paternal uncles are currently alive, one with a history of cutaneous melanoma and the other with a history of oral cancer. A pedigree of the paternal lineage is shown in .\nAfter resection, no additional adjuvant therapy was administered, and he was monitored by regular MR imaging of the brain over the next several years. Aside from suffering from occasional seizures associated with poor compliance to anticonvulsant medications, the patient remained otherwise neurologically intact. A surveillance scan 8 years after his initial surgery, now at 31 years of age, revealed a new area of nodular enhancement with associated hemorrhage adjacent to the prior resection cavity in the left frontal lobe (A, B, C). Additionally, a new T2- and fluid-attenuated inversion recovery (FLAIR) hyperintense mass lesion in the right cerebellar hemisphere was seen that did not enhance after contrast administration (A, B). The patient underwent a gross total resection of the enhancing nodule in the left frontal lobe. Pathology demonstrated a solid, non-infiltrative astrocytic neoplasm with marked nuclear pleomorphism, occasional xanthomatous tumor cells with foamy cytoplasm, and numerous eosinophilic granular bodies (D, E). The mitotic index was low, and high-grade histologic features including necrosis and microvascular proliferation were not identified. An immunostain for type IV collagen revealed abundant intercellular collagen deposition (F). Additional immunostains revealed that the tumor cells had intact/retained expression of ATRX protein and were negative for IDH1-R132H mutant protein. Ki67 labeling was seen in ~ 2% of tumor cells. A diagnosis of pleomorphic xanthoastrocytoma (PXA), WHO grade II, was rendered. Given this diagnosis and the potential for PXA to disseminate in the cerebrospinal fluid throughout the neuraxis, the right cerebellar lesion was considered worrisome for disseminated disease versus possibly representing a second primary tumor. To further evaluate the patient, MR imaging of the spinal cord was also performed, which demonstrated an expansile, contrast-enhancing mass within the right C6-7 neural foramen tracking along the C7 nerve root, consistent with a peripheral nerve sheath tumor (). A resection of the right cerebellar lesion was performed 1 month following the left frontal craniotomy. Pathology demonstrated a diffuse astrocytoma composed of neoplastic fibrillary astrocytes with elongate and irregular, hyperchromatic nuclei infiltrating through the subcortical white matter and internal granular layer of the cerebellum (C). The tumor cells demonstrated absence of ATRX immunostaining with intact staining in entrapped non-neoplastic neurons and endothelial cells, consistent with ATRX loss (D). The tumor cells were negative for IDH1-R132H and histone H3-K27M mutant proteins by immunohistochemistry. Ki67 labeling was present in ~ 5% of tumor cells. A diagnosis of diffuse astrocytoma, WHO grade II, was rendered. Given the significant family history and the presence of multiple histologically-distinct brain tumors and a peripheral nerve sheath tumor, genomic testing was recommended.\nAfter informed consent was obtained, targeted next-generation sequencing was performed on genomic DNA isolated from a peripheral blood sample and tumor tissue from the left frontal PXA and right cerebellar diffuse astrocytoma. This sequencing was performed on the UCSF500 Cancer Gene Panel as previously described, which utilizes capture-based next-generation sequencing targeting the coding regions of 479 cancer-associated genes along with select introns from 47 of these genes as well as the promoter region of the TERT gene [].\nThis analysis identified a focal heterozygous deletion on chromosome 9p21.3 spanning approximate coordinates chr9: g.21,700,000 – 22,800,000 (human genome assembly GRCh38) within the peripheral blood sample (). This deletion contains both the CDKN2A and CDKN2B tumor suppressor genes. The left frontal PXA demonstrated homozygous deletion of CDKN2A/B due to loss of the other copy of chromosome 9 containing the remaining intact CDKN2A/B alleles. This was accompanied by the p.V600E somatic hotspot mutation in BRAF (). Alterations involving IDH1, IDH2, TP53, and ATRX were not identified. The right cerebellar diffuse astrocytoma demonstrated homozygous deletion of CDKN2A/B due to copy-neutral loss of heterozygosity of chromosome 9p. This was accompanied by a hotspot activating missense mutation in PTPN11, two inactivating frameshift mutations in the NF1 tumor suppressor gene, and an inactivating frameshift mutation in the ATRX tumor suppressor gene. No alterations involving IDH1, IDH2, or TP53 were identified, nor was the BRAF p.V600E mutation found in the PXA. Apart from the copy number changes involving chromosome 9, no other chromosomal copy number aberrations were seen in either tumor.\nThe presence of germline CDKN2A/B inactivation together with the presence of multiple anatomically, histologically, and genetically distinct astrocytic neoplasms, both with accompanying somatic loss of the remaining CDKN2A/B alleles, is diagnostic of familial melanoma-astrocytoma syndrome. This genetic diagnosis is further supported by the significant family history of both glioblastoma and melanoma in the paternal lineage, suggesting that this is likely an inherited rather than de novo germline alteration in this patient. The patient and his family have been referred to a medical geneticist with expertise in cancer risk evaluation; however, no genetic evaluation of any family members has yet been performed. Given his newly-identified tumor predisposition syndrome, the patient has initiated the recommended cancer screening, including annual dermatologic, ophthalmologic, and dental examinations to monitor for the development of cutaneous, ocular, and mucosal melanoma. He will continue to undergo regular surveillance imaging of the brain. Additionally, imaging of the chest, abdomen, and pelvis was performed to rule out the presence of visceral malignancy, which was unrevealing except for the presence of the paraspinal peripheral nerve sheath tumor that remains asymptomatic and is being radiographically monitored at present.
A 55-year-old female with a history of renal cell carcinoma of the left kidney metastatic to the bony pelvis, lungs, mediastinum, and spleen presented to the emergency department with shortness of breath, pleuritic chest pain, and left scapular pain. She presented to the same emergency department one week prior with pleuritic chest pain but was discharged home after pulmonary embolism was ruled out.\nShe was diagnosed with renal cell carcinoma of the left kidney five years prior after presenting with gross hematuria. At that time, she underwent left radical nephrectomy. One year later, she developed a metastatic lesion in the bony pelvis for which she underwent radiation therapy. She as treated with pazopanib for two years with stable disease but stopped due to gastro-intestinal toxicity. Therapy was switched to nivolumab, which was discontinued after six months due to grade four pancreatitis and grade two rash. Eight months prior to her current presentation, she underwent radiation treatment to metastatic lesions in the left pubic symphysis and spleen. The patient initiated therapy with cabozantinib, a tyrosine-kinase-inhibitor used to treat renal cell carcinoma, three months prior to her current presentation.\nOn physical examination, she was wheezing in all lung fields and hypoxemic requiring supplemental oxygen. She had prior 12-pack-year smoking history but no formal diagnosis of chronic obstructive pulmonary disease (COPD). A chest x-ray revealed a small left pleural effusion and left basilar atelectasis. Laboratory workup, including complete blood count, renal and hepatic panels, and troponin, was unremarkable. An electrocardiogram (ECG) revealed sinus tachycardia without signs of ischemia. CT was not repeated due to her negative CT angiogram one-week prior. Given radicular and left scapular pain, an MRI of the spine was done, which revealed no pathologic metastases in the thoracic or lumbar spine but did reveal a new sacral lesion. Given her progressive stridor, she underwent laryngoscopy, which revealed a normal upper airway. A bronchoscopy showed significant trachea-bronchomalacia and thick purulent secretions in the left upper lobe, lingula, and right upper lobe.\nTwo days after admission, repeat chest X-ray revealed near complete opacification of left lung and large pleural effusion, a remarkably different radiograph from admission (Figure ).\nSubsequent CT chest revealed a large left pleural effusion with partial loculation as well as partial atelectasis of the left upper lobe and complete atelectasis of the left lower lobe. A right perihilar metastasis and perisplenic metastases were reported. The study was negative for pulmonary thromboembolism.\nThoracentesis revealed cloudy straw colored exudative effusion. A four French pigtail catheter was placed. Approximately 400 milliliters of yellow-green fluid was immediately drained. Pleural fluid studies revealed a white blood cell count of 33,000/μL (97% neutrophils), pH of 6.44, LDH of 4760 U/L, and an amylase of 394 U/L. She was started on vancomycin, cefepime, and metronidazole for presumed empyema. Pleural fluid cultures showed heavy growth of lactobacillus species, heavy growth of anaerobic gram negative cocci, and moderate growth of Candida krusei. Antimicrobial therapy was subsequently narrowed to ertapenem and anidulafungin. Given lack of improvement and continued significant chest tube output over the following week, further CT imaging was obtained, revealing a gastro-pleural fistula (via the left diaphragm and superior posterolateral stomach) with associated complex pleural effusion containing contrast material and gas (Figure ). This process abutted the known splenic metastases.\nAn esophagogastroduodenoscopy (EGD) revealed a 1.5-cm fistula in the posterolateral stomach that opened to the pleural space (Figure ).\nEndoscopic suturing was attempted to close the fistula with limited success (partial closure noted on imaging, with methylene blue dye taken via mouth visualized in the chest tube drainage catheter on water seal; Figure ).\nFor complete closure, the authors attempted a novel approach utilizing a venting gastrostomy tube and chest tube to water seal to facilitate closure of the fistula over the ensuing six weeks. Enteral feeding via jejunostomy tube to aid closure of the fistula was employed. The patient was continued on ertapenem and anidulafungin. She was also initiated on a proton pump inhibitor. She was discharged to a rehabilitation facility with plans to repeat imaging and methylene blue swallow in six weeks.\nUnfortunately, CT scans after six weeks showed that the fistula remained patent. A second attempt was made at endoscopic closure, which was again unsuccessful. One month later, during a hospitalization for electrolyte abnormalities, the patient decided to pursue elective surgical repair of the fistula in hopes of regaining the ability to resume normal oral intake. Four months after her initial presentation, she underwent laparoscopic surgery for fistula repair. The surgeon visualized extensive radiation fibrosis involving the stomach, spleen and retro-peritoneum. Given these findings and to avoid splenic bleeding, they pursued a conservative surgery whereby they stapled the stomach to ligate the gastro-pleural fistula anatomically. This approach is novel and was successful in our patient. A fluoroscopic upper GI series with oral contrast three days after surgery demonstrated no leakage of contrast outside of the GI tract or into the pleural space, and CT five days after surgery revealed no evidence of communication between the stomach and pleural space (Figure ).\nShe tolerated an oral diet. Gastrostomy tube, jejunostomy tube, and chest tube were removed without complication.
A 32-year-old G2P1 female presented at gestational week 41 for induction of labor. A labor epidural catheter was placed for pain management which was complicated by an inadvertent dural puncture. The Tuohy needle was immediately removed, followed by a second successful attempt at L3–4 interspace. An epidural catheter was threaded to 12 cm at the skin. A negative test dose of 3 mL of 1.5% lidocaine with 1:200 000 epinephrine, was followed by a continuous epidural infusion at a rate of 5 mL/hour using bupivacaine 0.1% and fentanyl 2 μg/mL. The epidural catheter was removed 2 hours after the delivery with no complications. On postdelivery day 2, the patient developed headache and stiff neck which was consistent with the diagnosis of PDPH. The patient was treated with bed rest, intravenous fluids, caffeine, and oral analgesics, but the symptoms were not relieved. Forty-eight hours later, the patient received an EBP with 20 mL autologous blood at L3–4 using a midline approach with loss of resistance to saline. She experienced neither back pain nor paresthesia during EBP procedure and achieved nearly immediate moderate relief of PDPH. Two days later, the patient developed recurrent severe headache with no associated neurological symptoms or fever. Her symptom was relieved with a second EBP of 20 mL autologous blood at L4–5 interspace. On postdelivery day 7, the patient complained of significantly worsening lower back pain that radiated to her buttocks bilaterally. She was afebrile and denied any motor, urinary, or gastrointestinal symptoms. A complete neurologic exam was performed, followed by an urgent lumbar spine magnetic resonance imaging (MRI) which demonstrated a small volume of layering blood within the intrathecal sac at the level of the sacrum (). A diagnosis of sacral radiculitis secondary to intrathecal hematoma was made. After consulting with neurological and neurosurgical teams, the patient was started on therapy of timed heating pads, transcutaneous electrical nerve stimulation unit, anti-inflammatory medication and acupuncture. She had a full recovery one month after delivery.
A 76-year-old lady with a history of RPC was admitted with hepatobiliary sepsis. She was deemed to be a high operative risk because of hepatitis B virus-induced Child A liver cirrhosis, hypothyroidism, hyperlipidaemia, and previous atrial fibrillation with a background of mild mitral, tricuspid, and aortic valve regurgitation. She had undergone laparoscopic cholecystectomy previously. She had also undergone multiple prior endoscopic and percutaneous biliary interventions to control sepsis and stone burden. The brushings and cytology from the intrahepatic ducts did not find malignancy. She was offered hepatic resection, but declined because of the high operative risk. She opted for indwelling percutaneous internal-external biliary drains with 3-monthly changes. In the last visit prior to this admission, she was discharged with a 12 Fr internal-external biliary drain (Navarre®; Bard Biopsy Systems, Tempe, AZ, USA) in the right hepatic duct and a 16 Fr drainage catheter (Cook Medical, Bloomington, IN, USA) in the left hepatic duct. The catheters were spigotted on discharge and the patient was taught to flush the catheters once a day with normal saline. However, she defaulted the follow up and was readmitted after 11 months with complaints of pain over the drain site and inability to flush the right catheter. The left drain site was normal. A plain abdominal x-ray revealed fractures of the right PTBD catheter with a normal left PTBD catheter ().\nThis result was further confirmed by a computed tomography (CT) scan, which showed a break in the right PTBD catheter near the liver capsule, with a 4 cm gap between the ends (), as well as a break at the distal segment. The left PTBD catheter was intact.\nThe catheter was fractured at two places with three fragments needing removal. The management of the catheter fracture was performed in stages. Firstly, radiologically guided percutaneous removal of the proximal fragment was performed. Further percutaneous attempt at removal of the middle and distal fragments was unsuccessful, because the strings of the catheter could not be pulled. A temporary 4 Fr Cobra angiographic catheter (Cook Medical, Bloomington, IN, USA) was inserted over the strings of the fractured catheter into the duodenum to drain the right hepatic duct and retain right biliary access. Second, endoscopic retrieval of the remaining fractured fragments () was done the next day. The intra-biliary catheter fragments were still tied to the duodenal components by the interlocking suture. Thus, all the catheter fragments could be successfully removed by the forceps. At this point, two catheters remained in place: the 4 Fr temporary right biliary access catheter and the 16Fr left biliary access catheter. Last, interventional radiology-guided exchange of the 4 Fr catheter to a 12F internal-external biliary drain (Navarre®; Bard Biopsy Systems, Tempe, AZ, USA) was then done the following day. The successful removal and insertion was confirmed by fluoroscopy ().
We report the case of a 51-year-old woman with a history of major depression after a suspected suicide attempt with olanzapine intoxication (maximum dose 160 × 7.5 mg of olanzapine; corresponding to 1200 mg). The legal representative of the patient signed informed consent for publication.\nAt first presentation in a primary care center, the patient was comatose and intubated. Activated charcoal was administered at an early point of time; however, no subsequent waking reaction occurred. An initial CT scan of the brain () revealed a left-hemispheric deep intracerebral hemorrhage (ICH). Upon admission, blood pressure was in normal range with no documented history of arterial hypertension. There was no medication history of anticoagulants nor was there a reported history of alcohol or drug abuse or any preexisting diseases such as arterial hypertension or liver insufficiency. Laboratory results showed a normal platelet cell count (218/μl), standard coagulation parameters (PTT: 26 s, INR: 0.9), and liver function (GPT: 30 U/l). Factor XIII was normal (85%). There were no signs of any preceding head trauma. Prior medication was olanzapine 5 mg daily and duloxetine 90 mg daily as well as lorazepam in an unknown daily dose.\nFor further therapy, the patient was transferred to our tertiary care center 12 hours later. At admission, we conducted a follow-up CT scan, which presented not only significant secondary enlargement of the left-hemispheric hemorrhage but also newly developed right-hemispheric ICH in the basal ganglia areas. In a CT-angiographic study, no source of the hemorrhage was detected. MRI at day 2 after admission confirmed bilateral intracerebral hemorrhage but neither showed any underlying cause of ICH (). Olanzapine serum levels measured approximately 24 hours after suspected intake were highly elevated (820 μg/l) confirming severe intoxication []. Corrected QT interval was 450 ms.\nTwo days after admission, the patient's conditions improved significantly. She regained consciousness, was able to speak, and did not show any severe focal deficits. However, within a few hours, the patient again lost her consciousness, a tetraparesis occurred, and reintubation was required. Another follow-up cerebral CT scan revealed massive progression of the bilateral hemorrhage and enlargement of space-occupying edema of the left hemisphere (). There was no documented hypertensive crisis in this period. Consequently, a left-hemispheric hemicraniectomy was conducted (). In the following days, hyperpyrexia, elevation of the creatine kinase (3885 U/l), moderate leukocytosis (13.5 g/l), and muscle rigidity occurred suggesting late-onset neuroleptic malignant syndrome. In spite of medication with dantrolen, fever was lowered insufficiently. At discharge to a rehabilitation center, lastly, olanzapine intoxication was discussed as the cause of bilateral ICH, since other causes were excluded.\nDuring a follow-up time of two months, the patient remained in a persistent vegetative state.
An 85-year-old man with a 1-month history of difficulty swallowing foods presented to our department and was diagnosed with AEBSCC, cT3N1M0, stage III, by thorough examination (Figs. , , , and ). The basaloid carcinoma extended from the upper thoracic esophagus to the middle thoracic esophagus based on imaging studies (Figs. , and ), endoscopy (Fig. ), positron emission tomography–computed tomography (Fig. ) and biopsy (Fig. ). Morphologically, the tumor was an elevated ulcerative area. Furthermore, the tumor was found to have spread into the submucosa (Figs. and ). Immunohistochemical staining showed the tumor to be negative for p16. The patient had been diagnosed with prostate cancer 10 years earlier and had received hormone therapy for 5 years. There had been no recurrence of the prostate cancer. His medical history also included pulmonary tuberculosis and spinal stenosis. He smoked 20 cigarettes per day for the prior 12 years and drank 2 go (approximately 361 mL) of alcohol daily. We initially recommended surgery as aggressive treatment because his general condition was good and the prognosis of AEBSCC is poor. However, he refused aggressive treatments including chemotherapy. We thus administered radiotherapy for symptom relief. The radiation field ranged from the supraclavicular lymph node region to the entire esophagus, and radiation was delivered at a dose of 60 Gy in 2-Gy fractions (Fig. ), allocated as 40 Gy to the regional field and 20 Gy to the boost field. To date, approximately 2 years have passed since radiotherapy completion. For follow-up of this patient with AEBSCC after radiation therapy, we obtained a detailed history and performed a full physical examination, computed tomography and upper gastrointestinal endoscopy every 3–6 months. The disease course has been good with neither recurrence nor metastasis and there were no adverse effects related to radiation therapy (Fig. ). There were no late adverse events related to radiation therapy.
A 68-year-old female with no significant past medical history presented with a rapidly progressing painless extrinsic irregular mass on her lateral aspect of right thigh for three-month duration (). She reported no trauma or previous surgery to that site. She noted occasional bleeding from this lesion. She denied weight loss, malena, constipation, hematuria, or abdominal pain. Her last colonoscopy was 10 years ago, the findings of the test were unremarkable. Her vital signs were stable. She had a biopsy of this cutaneous lesion by a local surgeon which revealed metastatic adenocarcinoma. An immune-peroxidase stain panel showed CAM5.2 positive, 34 BE12 positive, CDX-2 positive, CEA positive, CK-7 negative, CK-20 negative, and TTF-1 negative consistent with metastatic adenocarcinoma either bladder or gastrointestinal as primary. She presented herself to our institute for further care. Physical examination was only positive for a cutaneous lesion in the lateral aspect of her right thigh measuring 4 cm × 3 cm, a second lesion in the right upper posterior chest measuring 2 cm × 1.3 cm, and third lesion involving the right axilla measuring 1 cm × 1.5 cm. After initial evaluation by her medical oncologist she was referred to gastroenterologist for endoscopy. She had upper and lower endoscopy of the gastrointestinal tract. Upper endoscopy study was benign. Colonoscopy revealed multiple polyps in the transverse colon, descending colon, and rectum. Additionally, there was a large, friable, and infiltrative tumor which occupied 50 to 74% of the circumference of the ascending colon. It was not causing significant narrowing (). The tumor bled on contact. Multiple cold forceps biopsies were taken. The specimens were collected for pathology. The tumor measured approximately 6 cm × 3 cm in size. Biopsy of right thigh mass was done. The cecum and rest of the colon appeared to be normal. An immune-peroxidase stain panel done showed CAM5.2 positive, 34 BE12 positive, CDX-2 positive, CEA positive, CK-7 negative, and CK-20 negative, TTF-1 negative consistent with adenocarcinoma of colon (). A PET scan confirmed hypermetabolic activity only at ascending colon and in areas of cutaneous soft tissue masses. She was diagnosed to have stage 4 adenocarcinoma of the right colon with multiple cutaneous metastases and was started on combination chemotherapy with folinic acid, fluorouracil, and oxaliplatin (FOLFOX). Currently she is undergoing chemotherapy cycles.
A 47-year-old man underwent a combined SIT and renal transplant. He had short-gut syndrome with 35 cm of small bowel remaining after multiple resections for spontaneous volvulus. The native colon remained intact and functioning. He had end-stage renal failure due to oxalosis which had been demonstrated on pre-transplant renal biopsy. He received induction immunosuppression with pre-operative basiliximab 20 mg, with a second dose given on post-operative day 4. Early maintenance immunosuppression consisted of intravenous methylprednisolone, MMF 1000 mg BID, and tacrolimus titrated to a trough level of 10-12 ng/mL.\nProtocol endoscopy and biopsy of the SIT and native colon, accessed via a chimney ileostomy, were performed on day 13 post-transplant. As per our institutional protocol, the biopsies were interpreted independently by two experienced transplant pathologists. The donor ileum and native colon appeared macroscopically normal. Donor ileal biopsy showed a mixed inflammatory infiltrate with activated lymphocytes, eosinophils and plasma cells and evidence of crypt epithelial injury associated with > 6 apoptotic bodies per 10 consecutive crypts (Figure ). Native colonic biopsies were unremarkable at this time (Figure ). A diagnosis of mild ACR was made. This was treated with pulsed methylprednisolone, as per our hospital’s protocol. A subsequent biopsy performed 3 d later demonstrated resolution of the ACR, again with normal colonic biopsies.\nFurther protocol endoscopy and biopsy of the SIT and native colon was performed on day 23. The donor ileum had macroscopically flattened villi and the native colon appeared normal. Biopsy of the donor ileum, from both the chimney and the graft proximal to the colonic anastomosis, demonstrated focal villous blunting and flattening, with multifocal erosion, superficial ulceration with neutrophil clusters and inflamed granulation tissue. In areas there was marked degeneration and vacuolation of the surface epithelium with sloughing, but no viral inclusions were identified on immunohistochemistry. There was mixed mononuclear inflammation with foci of crypt degeneration, neutrophilic cryptitis, areas of crypt drop-out and up to 10 apoptotic bodies per 10 crypts, without confluent apoptosis (Figure ). In isolation these findings were concerning for at least moderately severe ACR, particularly in the setting of ACR only 10 d prior. An opinion was also sought from an international expert, who reviewed the biopsies, and felt that the changes in the small bowel were suspicious for moderate-severe ACR.\nImportantly however, the native colonic biopsies also demonstrated surface epithelial vacuolation associated with crypt injury with dilatation, goblet cell depletion, focal attenuation of the epithelium and focally increased basal apoptosis (Figure ). These new findings in the previously normal native colon suggested a non-graft specific pathological process and hence, in the absence of viral infection, or clinical features of graft-vs-host disease (GVHD), raised suspicion for MMF GI toxicity. We therefore chose to discontinue the MMF (substituted with azathioprine) and not give any specific treatment for rejection, pending an early repeat biopsy.\nFurther endoscopy and biopsy 4 d later (post-operative day 27) revealed significant improvement in histologic appearance with only low grade apoptosis, and by post-operative day 34 the endoscopic appearance was normal and histologic examination demonstrated normal villous architecture, regenerative crypts and 3-4 apoptotic bodies per 10 crypts. Native colonic biopsy showed evidence of healing injury and reduced apoptosis. Repeat biopsy on day 41 showed similar findings in the SIT and entirely resolved changes in the native colon. Viral inclusions were absent in all biopsy specimens.\nThe patient is now one year post transplant and has remained on azathioprine, tacrolimus and prednisolone. He currently has intestinal autonomy and a well-functioning renal graft and has had no further episodes of acute rejection.
A 55-year-old female presented to the emergency department with a history of fall on her outstretched hand after falling from a height of seven feet. She had complaints of pain over her left elbow joint and wrist joint. On physical examination, her left elbow was grossly deformed, and there was swelling over her left wrist. Tenderness was present over both her left elbow and wrist joints. The range of motion of her left wrist and left elbow joint were painfully restricted. No distal neurovascular deficit was noted.\nHer plain x-rays revealed an anterior elbow dislocation and distal radius fracture with intra-articular extension. There was an associated displaced fracture of the lateral epicondyle (Figure ).\nThe elbow joint was reduced under general anesthesia. A distal traction was given at the forearm. Since there was a fracture at the distal end of the radius as well, the traction could not be applied at the wrist. The traction was thus applied to the proximal forearm, and a posteriorly directed force was applied while the elbow was hyperflexed. Care was taken not to hyperextend the elbow joint as it could have put the anterior neurovascular bundle at a stretch. The elbow joint was unstable post reduction and, hence, was fixed with two ulnohumeral pins.\nThe displaced lateral epicondyle fracture was accessed through the lateral approach to the elbow and fixed with a partially threaded cannulated cancellous screw with a washer (Figure ).\nThe distal radius fracture was then reduced and pinned using five percutaneous K-wires. Two 1.5 mm K-wires were inserted from distal to proximal, and another pin was passed through the radial styloid. Two K-wires were added from the ulnar side to the radial side for prevention of supination and pronation (Figure ).\nAn above-elbow cast was applied for two weeks. Following this, the ulnohumeral pins along with the slab were removed, and a wrist brace was given. The patient was reviewed weekly for her pin site dressings, and elbow mobilization was encouraged. The wrist splint was then removed after four more weeks, and active elbow and wrist movements were started six weeks after trauma. At the three-month follow-up, the lateral epicondyle fracture had united, and the patient had regained flexion range of motion of zero degrees to 130 degrees at the elbow (Figure ).\nThe wrist had dorsiflexion of 70 degrees, palmar flexion of 40 degrees, and supination restriction of 10 degrees as compared to the normal side. At the final follow-up of one year, the patient was asymptomatic with both fractures united and a congruent elbow joint. The distal end radius fracture had malunited but was asymptomatic (Figure .)
A 70-year-old man presented to the emergency department (ED) after falling twice at home. While standing after eating a light meal, he had two separate episodes of brief loss of consciousness. On the second fall, the patient had hit his right shoulder, cheek, and foot, prompting the visit. The patient had received a colonoscopy earlier in the day for routine cancer screening. He had followed proper protocol regarding his bowel prep and had not had any immediate complications related to the procedure or anesthesia. Since the colonoscopy, he had continuous bouts of cramping abdominal pain. He had also felt dizzy throughout this period but attributed it to dehydration related to his bowel prep. The patient denied striking his head, chest pain, shortness of breath, nausea, vomiting, or blood in his stools. His past medical history was significant for coronary artery disease, hyperlipidemia, and hypertension. The patient took his benazepril, aspirin, and atorvastatin on the day prior to colonoscopy.\nOn physical exam, the patient was afebrile with a heart rate of 87 and blood pressure of 130/78. The patient's head, neck, chest, and neurological exams were entirely normal. His abdominal exam was remarkable for tenderness in the right and left lower quadrant with some voluntary guarding, but no masses or rebound was appreciated. His orthopedic exam demonstrated tenderness to his right acromioclavicular joint and ecchymosis of his right 5th metatarsal with no deformity and normal range of motion at all joints.\nOn laboratory evaluation, the patient's hemoglobin was 12.4 g/dL, with normal platelets and chemistry studies. Head computed tomography (CT) and shoulder and chest radiography were normal. Foot radiography demonstrated a nondisplaced 5th metatarsal fracture. CT of the abdomen and pelvis demonstrated moderate hemoperitoneum with splenic laceration (Figures , , and ).\nThe patient was admitted to the intensive care unit and underwent serial abdominal exams and every four-hour hemoglobin measurements. His hemoglobin fell to 8 g/dL at 48 hours after admission but subsequently stabilized. The patient was observed in the hospital and ultimately discharged home on hospital day 3 in good condition.
A 36-year-old woman (height, 147 cm; weight, 50 kg) with CIPA was scheduled for revision of left total hip arthroplasty. She was diagnosed as having CIPA because of recurrent episodes of unexplained fever, anhidrosis, burns, and bone fractures after birth. She had previously undergone 7 operations for spinal deformity and 1 operation of total hip arthroplasty in both the left and right sides. Although lack of general diaphoresis and thermal nociception were observed, the patient performed body surface cooling at her own discretion when she felt she was at a risk of hyperthermia, and her body temperature was kept approximately 36°C. No signs of mental retardation or orthostatic hypotension were observed. No abnormality was detected on chest radiographs and electrocardiograms. Blood biochemistry revealed no abnormality except mild anemia indicated by a hemoglobin level of 10.6 g/dl.\nNo premedication was administered. After the patient was brought into the operating room, routine monitoring and measurement of the bispectral index (BIS) were started. Body temperature was measured at 3 different sites (urinary bladder, esophagus, and precordial skin) and controlled by a hot-air-type heater. Propofol was administered at an effect-site concentration of 4 μg/ml by target-controlled infusion. After muscle relaxation had been achieved by administration of 50 mg of rocuronium, the trachea was intubated. Immediately after endotracheal intubation, systolic blood pressure increased from 130 to 145 mmHg, and heart rate increased from 60 to 95 beats per minute (bpm). Two minutes later, systolic blood pressure had decreased to 125 mmHg. Propofol was continuously infused intravenously at a target concentration of 2 to 4 μg/ml () and BIS levels were maintained between 40 and 60. After an arterial catheter had been placed, her position was changed from the supine to right lateral position. Surgery was then started.\nSince no circulatory change associated with pain occurred during surgery, opioids were not administered. Regarding hemodynamics, when 600 ml of blood was rapidly lost within 20 minutes, blood pressure decreased from 113/66 to 93/55 mmHg and heart rate increased from 55 to 70 bpm ( a). Similarly, when 850 ml of blood was lost within 30 minutes, systolic blood pressure decreased from 108/65 to 95/60 mmHg and heart rate increased from 66 to 74 bpm ( b). Administration of 0.1 mg of phenylephrine increased blood pressure from 87/55 to 117/76 mmHg and decreased heart rate from 70 to 65 bpm ( c).\nThe operative time was 6 hours and 49 minutes, and the duration of anesthesia was 8 hours and 41 minutes. The volume of blood loss was 3350 ml. Blood transfusion was performed with 1600 ml of preoperatively donated autologous blood, 900 ml of salvaged blood, and 720 ml of fresh frozen plasma. Intraoperative body temperature was controlled and kept between 36.0°C and 36.9°C at all 3 measurement points. After surgery had been completed, the patient was returned to the supine position and she was extubated. Since she did not complain of any pain after the surgery, no analgesic was administered. She was discharged at 6 weeks after the operation.\nBlood samples were collected 3 times: before anesthesia induction, after the start of surgery, and at the end of surgery. The levels of catecholamine fractions and cortisol were measured. Norepinephrine levels were below the normal range at all time points, and the levels of epinephrine and cortisol were within the normal ranges at all time points ().
A 49 year old female presented with a 4-week history of acute onset paracentral scotoma in her left eye. Best-corrected visual acuities were 85 and 71 letters in the right and left eyes respectively. Anterior segment examination was normal. Fundus examination showed a slightly reddish wedge-shaped lesion superior to the fovea in the left eye and this corresponded to a hypo-reflective lesion on NIR imaging. AF was normal. OCT B-scan revealed focal loss of IZ signal and attenuation of the EZ integrity in the region of the wedge-shape lesion (). There were no other obvious retinal or choroidal changes adjacent to these regions. Microperimetry demonstrated two loci with reduced retinal sensitivity in the supero-temporal fovea corresponding to the wedge-shaped lesion seen on NIR imaging. However, there were also other test loci infero-nasally and infero-temporally at 3° eccentricity with reduced retinal sensitivity. AO-FIO demonstrated loss of cone reflexes not only within the wedge-shaped lesion but also at other regions that coincided with relative scotoma (). En face OCT images of EZ and IZ showed low reflectivity profile that corresponded to the regions of cone reflex loss on AO-FIO. Hence, there were several lesions that were visible on the en face OCT image which were not on NIR.\nOn follow up at 6 months, serial OCT demonstrated partial recovery of the integrity of the EZ and IZ in the affected regions and improved visualization of cone tip reflexes on AO-FIO. There was improvement in retinal sensitivity from 7 to 27 dB and some of the defects seen on en face OCT images of EZ and IZ resolved. The main wedge-shaped lesion also reduced in size over the 6 months (). There was no significant difference in the en face OCT images generated by commercial and our custom software in illustrating the course of AMN recovery since retinal segmentation were almost identical.
Mr X is a 38-year-old, unemployed, single male with no children and with an elementary level of education. This was his index presentation with a 4-year history characterised by ongoing persecutory delusions, as well as auditory hallucinations. He was brought to the Emergency Department by ambulance because he was found to be bleeding profusely from his scrotum in the toilet of a petrol filling station. He alleged that he had cut open his scrotum to remove his testicles before his ‘tormentors’ could do so. He stated clearly that he did not want to die because he valued his life. This was therefore not an attempt at suicide.\nHe was initially admitted to the urology ward and then referred to psychiatry. The multi-disciplinary team diagnosed him with and treated him for schizophrenia. He responded well to haloperidol 2.5 mg orally in the morning and 5 mg orally at night. A long-acting injectable antipsychotic, flupenthixol decanoate 20 mg intramuscular was also prescribed. No adverse effects were reported. Lorazepam was titrated downwards from 1 mg orally twice daily to 1 mg orally at night, and then stopped before he was discharged. Lansoprazole 30 mg daily orally, tramadol 50 mg three times daily orally and paracetamol 1 g orally were also prescribed as needed.\nUpon discharge, on day 44 of the admission, the patient was symptom free with no psychotic or anxiety features.\nThe patient did not manifest any depressive symptoms throughout his hospitalisation, nor on his 4-week follow-up visit subsequent to discharge. He also demonstrated full and complete understanding that the voices, the self-conviction and his belief that people were coming to harm him were all part of his illness called schizophrenia. He also demonstrated full understanding that the belief of being harmed and people conspiring against him were also part of his schizophrenic illness that had been untreated for at least the past 4 years. With no negative emotion, he demonstrated intellectual understanding with unconditional acceptance of his illness. We emphasised to him that he must be consistent with medical check-ups at his local clinic as some other medical conditions can cause his illness to resurface. It was further emphasised to him that for as long as he took his treatment regularly and as prescribed the schizophrenia would be managed and controlled well. He agreed to stay away from all psychoactive substances. This user was amenable to following up with a clinical psychologist, an occupational therapist and a social worker.\nHe was followed up 1 month later and then referred to his local clinic for continuation of the prescribed treatment, appointments for continuation of psycho-education, counselling and relevant psycho-therapies. This patient responded well and remitted only on antipsychotic agents.
A 25-year-old Han Chinese woman presented to the emergency department of our hospital with abdominal pain, bloating, nausea, and emesis for 3 days. She could pass stool and flatus but with significant reduction. She denied bloody stools, fever, chills, or weight loss. In the past decade, she had experienced abdominal pain that would disappear after rest without any treatment. There was no known precipitating factor for the abdominal pain. She reported no other symptoms, such as constipation or diarrhea. There was no significant family history of genetic disease or cancer. Her antenatal history was unremarkable. Physical examination showed abdominal distension, tenderness, and rigidity. Routine laboratory values were unremarkable. After ultrasonography revealed an expanded intestinal lumen, we performed abdominal computed tomography (CT), which showed abnormal dilatation of the intestine (). A nasogastric tube was inserted, and approximately 300 mL of dark green fluid was drained.\nBecause of the peritoneal irritation and CT scan result, exploratory laparotomy was performed. The dilated intestine was intraoperatively identified as a duplicated tube volvulus of the distal part of the descending and proximal part of the sigmoid colon. No intestinal adhesions or coexistent congenital anomalies were found. The anomalous segment had separate intestinal walls. There was only one connection between the anomalous and normal lumens at one end of the duplicated colon; that is, a Y-shaped tubular duplication. Partial colectomy of the descending and sigmoid colon was performed, followed by end-to-end anastomosis without ostomy. Postoperative pathology findings confirmed colonic duplication (). Grossly, the duplicated colon was approximately 72 cm long and measured 15 cm in greatest diameter. Microscopically, the duplicated colonic segment showed muscular hypertrophy. No gastric heterotopy or adenocarcinoma was found; 10 lymph nodes were identified and all were unremarkable. The patient developed fever postoperatively, which was responsive to an antipyretic and was categorized as a grade I surgical complication according to the Clavien–Dindo classification system. She was discharged on postoperative day 14 and followed for 2 years without specific events or complications.\nThis report was reviewed and approved by the Peking Union Medical College Hospital Institutional Review Board. The patient provided written informed consent for publication of this case report.
A 38-year-old female was referred to the Outpatient Breast Clinic due to having a huge mass on the right breast for >2 years. On examination, a firm lobulated huge mass was observed with superficial veins on the breast being significantly bulging and a skin ulcer in the lower outer quadrant (). Neither clinically positive lymph nodes nor nipple discharge were found on clinical examination.\nThe most recent chest computed tomography indicated a swollen anterior chest wall with areas of low density, interpreted by radiologist as possible invasion of pectoralis major muscle. Preoperative examination tests did not prove any distant metastases. The patient underwent fine needle aspiration that revealed stromal cells, often accompanied by large, folded sheets of epithelium suggesting fibroepithelial lesion.\nTwo years ago, the patient had undergone a mammogram (), which had shown breast asymmetry due to a large lobulated mass with smooth and circumscribed margins.\nSurgery was scheduled after discussion with the patient who was explained that breast reconstruction might not be feasible due to the possible involvement of pectoralis major muscle. Informed consent was obtained from the patient. Wide mastectomy was performed. At this point, it has to be underlined that dissection was performed carefully since there were multiple bulging vessels. Trying to conserve pectoralis major muscle for reconstruction only a superficial layer of muscle fibers was resected.\nMacroscopically, surgical margins were free. Reconstruction with tissue expander was decided and it was inserted beneath pectoralis major. Skin flaps were closed under some degree of tension after the placement of surgical drains. Postoperatively, the patient recovered with no complications ().\nHistology revealed phyllodes tumor of 26 cm in maximum diameter with moderate cellularity and atypia, without invasive growth pattern. Heterogeneity was observed with regard to mitotic activity but not <5–7 mitoses/10 hpf were found so that the tumor was categorized as borderline phyllodes tumor according to WHO.
A 28-year-old Asian woman (G3P1) who had undergone emergency cesarean delivery owing to a compound presentation at full term was referred to our institution with a suspicion of abnormally located gestational sac. She had undergone laparoscopic cholecystectomy and open appendectomy previously. She did not have any medical, family, or psychosocial history. She had missed her menstrual period without any other symptom and visited a private obstetrical clinic to confirm the pregnancy. However, she was diagnosed as having an abnormal pregnancy such as cervical or CSP by USG.\nAt our institution, she reported that her last menstrual period was just 5 to 6 weeks prior. However, USG revealed a gestational sac in the anterior lower uterine segment with a fetus measuring 4.83 cm crown-rump length (CRL) with positive cardiac activity, corresponding to 11 weeks and 6 days of gestation. Color/power Doppler images depicted a hyperechoic rim of a choriodecidual reaction with excessive vascularity (Fig. ). Although we could observe a definitive abnormally located gestational sac, our patient did not have any pain during the physical examination. She admitted that her last menstrual period was different from her usual menstrual periods. Because CSP or cervical pregnancy was suspected, we performed computed tomography (CT) for a definitive diagnosis. The CT scan showed an intrauterine gestational sac in the lower uterine segment bulging through the anterior uterine wall at the site of the cesarean scar. No invasion of the urinary bladder was observed (Fig. ). On presentation, her β-human chorionic gonadotropin (β-hCG) level was 66,536.8 IU/L (Day 1). Initially, we injected 50 mg of methotrexate (MTX) mixed with 9 mL of normal saline in the amniotic sac through a 22-G needle transabdominally under USG guidance. Simultaneously, 2 ml of amniotic fluid was aspirated for termination of the pregnancy. However, fetal cardiac activity was still observed 2 days later (Day 3), without significant changes in the serum β-hCG levels (65,342.5 IU/L). We decided on laparotomy instead of laparoscopy because of the large CRL (Day 4). The intraoperative finding showed bloody amniotic fluid, blood clot, placenta, and a fetus at the lower segment of the uterus. A transverse uterine incision was made at the lower segment of the uterus (Fig. ). The gestational sac was removed, as well as most of the trophoblastic tissues that were adherent and invading the wall of the lower uterine segment. The fetus and placenta showed no definitive abnormalities (Fig. ). The estimated blood loss was 1.2 L at intra-operation, without immediate complication. The uterine defect was repaired into two layers by using 2–0 Vicryl sutures. Our patient received 3 units of packed red blood cells (PRBC) at the ward postoperatively. The serial β-hCG level was 1958 IU/L at 4 days after the surgery (Day 8). She was discharged in good condition 5 days after the operation (Day 9). After 1 month (Day 39), her β-hCG levels returned to normal (2.8 IU/L). She was very satisfied with the fact that she had recovered well without the need for intensive care or further treatment without the need for hysterectomy.
On January 31, 2015, a 43-year-old Asian man with a 3-year history of progressively invasive PC presented with pain in his right eye. Our patient, who had been staged T4N3M1(TNM classification), had also lost his vision more than a month earlier. He was diagnosed as having metastasis in the bilateral inguinal lymph nodes and ipsilateral iliac nodes before systemic metastasis to his liver and lungs. His medical history was remarkable due to his several surgeries. He denied any family history of inherited diseases and psychological illness.\nOn presentation, his best corrected visual acuity was no light perception in his right eye and 20/20 in his left eye. His intraocular pressures were 13.0 mmHg and 11.0 mmHg in his right and left eye, respectively. For the right eye, the pupil dilated to 5 mm, and then the pupillary reaction disappeared. An external examination revealed mild proptosis and ocular movement in all directions. A dilated fundus examination of his right eye showed post equatorial retinal detachment with a black eminence and a pale optic disk. There were no obvious abnormalities in his left eye.\nAn ophthalmic B-scan ultrasound showed retinal detachment with hemorrhage. Orbital MRI confirmed the thickening and strengthening of the right lateral wall, characteristics of metastatic carcinoma. The internal rectus and lateral rectus muscles were thickened and hardened, the 2-cm-long optic nerve was thickened, and its stump was invaded by the metastasis. The T1-weighted images of the MRI scans showed hyperintensity (Fig. ), whereas the T2-weighted images showed hypointensity (Fig. ). A contrast-enhanced MRI scan revealed inhomogeneous enhancement of the posterior wall (Fig. ). The presence of lesions was associated with invasion of the optic nerve, choroid, and sclera by the metastatic cells.\nThe deep layer, including the choroid, was infiltrated by cancerous tissue. Considering his severe eye pain and irreversible loss of vision, our patient had undergone right eyeball enucleation under general anesthesia on February 3, 2015. This type of procedure is indicated for patients who have had severe eye trauma and for those patients experiencing severe eye pain with unrecoverable vision. His complete eyeball was observed intraoperatively. Histopathological examination led to a diagnosis of metastatic moderately differentiated penile squamous cell carcinoma that infiltrated the sclera, choroid, retina, optic nerve, and external intraocular sites. Hematoxylin-and-eosin staining of the entire eyeball cellular neoplasm showed keratin pearls and infiltrative growth of keratinized cells. Intercellular bridges were seen in the nests of moderately differentiated squamous carcinoma cells (Figs. and ).\nOur patient received chemotherapy and radiotherapy during 6 months of follow-up, and then died due to brain metastasis.
A 14-year-old female patient reported with chief complaint of discoloration and pain in right upper front tooth since 10 days. Pain was dull and intermittent in nature. Past medical history was non-contributory. Past dental history revealed trauma to primary central incisors and right lateral incisor 10 years back, after fall from stairs. On reporting to local dentist, intrusion (grade I) was diagnosed for all 3 mentioned teeth. Patient was kept under observation. Later, these primary teeth exfoliated corresponding to their age.\nThe extra-oral examination of head and neck structures were within normal limits and corresponded to normal growth and development for that age. Intra-oral examination of hard tissues revealed the presence of abnormal, discolored, hypoplastic permanent right maxillary lateral incisor. Labial surface of crown was having notch-like structure in cervical third region of crown []. Palatal view revealed presence of cusp-like structure of 4-5 millimeters extending less than half the distance from CEJ [] and a developmental groove run deep between palatal aspect of lateral incisor and this additional cusp []. Tooth was tender on percussion, and electric pulp testing indicated negative response. The crown of the lateral incisor had normal mesio-distal width and was tilted palatally. Primary right maxillary canine was retained, and the tip of erupting permanent right maxillary canine was observed distal to crown of lateral incisor []. Intra-oral periapical radiograph revealed the presence of an extra root-like structure superimposing right lateral incisor. []. Periapical radiolucency was present with respect to same tooth. Maxillary permanent right canine was seen impacted above the retained right primary canine and distal to permanent right lateral incisor.\nTreatment was planned in order to prevent periodontal disease and development of dental caries on palatal groove and to correct the esthetic status of hypoplastic tooth. To achieve the desired outcome, treatment was done in 3 steps. In the first step, endodontic treatment was performed followed by orthodontic alignment of the tooth and finally, esthetic rehabilitation was done.\nThe tooth was isolated with rubber dam, and access preparation was done. Pulp tissues were removed with barbed broaches. An extra canal was observed palatally to main canal, which was quite smaller in dimension. Canal was negotiated, and pulp tissue was removed with the help of barbed broach. The cleaning and shaping for both the canals were achieved using K files (Mani, Japan) and simultaneously, irrigation with normal saline and 5.2% sodium hypochlorite was done. The final apical file used was 30 size (ISO 0.02 taper) for both the canals. The canals were finally flushed with normal saline and dried with absorbent paper points. The canals were filled with calcium hydroxide powder (Deepashree products, Ratnagiri, India), mixed with saline, and were kept under observation for 4 weeks. The periapical radiolucency remained unchanged after 1 month; however, the tooth was asymptomatic. Final obturation was done using sealer (Endoflux, Ammdent, Mohali, India) with gutta-percha (Dentsply, France, SAS) by lateral compaction technique []. Patient was recalled again after 3 months for check-up. Periapical lesion was healed. Patient was referred to department of orthodontics for correction of malposed canine.\nThe fixed orthodontic treatment was started on patient using MBT (Mclaughlin, Bennett, Trevisi) straight wire appliance with slot size of 0.022 × 0.028˝ dimensions. Brackets were bonded on the upper arch, but the malformed tooth was banded due to its convexity and hypoplasia.\nInitially, alignment was done to mildly de-rotate the canine using 0.016˝ NiTi wire (Libral Traders, USA) for 4 weeks, followed by 0.016˝ stainless steel wire with NiTi coil spring (light force) for distalization of canine. This procedure took 6 months to distalize. During distalization, the posterior anchorage was reinforced with Nance palatal arch. After distalization, a space of 1.5 mm was created between canine and lateral incisor. This space was closed with 0.018 × 0.025˝ stainless steel wire (Libral Traders, USA) using ‘T’ loop mechanics. This took approximately 8 weeks. The remaining distopalatal rotation of canine was finally aligned with NiTi wire (Libral Traders, USA). This alignment took 3 weeks followed by a stabilizing wire (3 months). The total fixed orthodontic procedure took 10 months.\nThe orthodontic treatment was followed by esthetic rehabilitation of lateral incisor by all ceramic crown [].
A 37-year-old male presented to our emergency department (ED) with bilateral, burning hand pain, specifically under his nail beds and at his fingertips. The patient was exposed to aluminum brightener while at work, involving both of his hands. The patient did not use gloves and had been working without any issues for several months before this presentation. The product had an HFA concentration of 10%.\nThe patient was hemodynamically stable on presentation, including initial vitals of heart rate of 86 beats per minute, respiratory rate of 18 breaths per minute, and a blood pressure of 138/78 mmHg. On physical examination, the patient had bilateral tenderness to both hands, but did not demonstrate any external findings such as erythema, necrosis, ulcerations, or discoloration. No significant neurological or vascular deficiencies were found for either extremity. The remainder of the physical examination was unremarkable.\nThe patient’s medical workup included an ECG, basic metabolic panel, complete blood count, and magnesium and calcium concentrations. The local poison control center was contacted, who agreed with the workup and recommended applying calcium gluconate (CaG) gel to the affected areas until the patient had complete relief of his pain.\nECG was negative for significant findings, including interval prolongations and arrhythmias. All lab results were within normal limits, including calcium and magnesium concentrations. The patient was treated with CaG gel, made in the ED by taking one gram of CaG and mixing it with standard lubricating gel in the ED. He received a total of three treatments of this gel before he had complete resolution of his pain.\nPer the poison control center, once the patient was asymptomatic, with a normal ECG and lab values, he could be discharged home. The patient was given instructions concerning return precautions and instructions on how to safely use HFA products, including the use of appropriate gloves. He was then discharged home.
A 38-year-old Ecuadorian woman with a past medical history of treated hepatitis C and a new presumed diagnosis of Crohn's disease presented as a transfer from an OSH in septic shock and respiratory failure due to complications after multiple abdominal surgeries for an uncontrolled GI bleed.\nApproximately three months prior to her transfer, she presented to an OSH with severe upper abdominal pain and bloody diarrhea for several weeks, prompting an endoscopic evaluation. She was diagnosed with Crohn's Disease and was discharged on mesalamine (1 g four times a day). Since her discharge, she was adherent with her medication but continued to have abdominal pain, nausea, and bloody diarrhea. She was readmitted one month later for a presumed Crohn's flare and then discharged on oral prednisone (40 mg daily for 2 weeks, followed by a slow taper) and mesalamine (1g four times a day). One month prior to her transfer, she presented again to the OSH for worsening emesis, bloody diarrhea, subjective fevers, and abdominal pain. She was noted to be septic with a maximum temperature of 103 °F (39.4 °C) and acutely anemic from an ongoing GI bleed that required multiple transfusions. Results of the computerized tomography (CT) scan were concerning for an intrabdominal abscess and empiric intravenous (IV) antibiotics (meropenem 1 g every 8 hours and vancomycin 1 g every 12 hours) were started. Endoscopic treatment of the GI bleed failed due to inability to localize it; thus, she underwent a total colectomy with a Brooke ileostomy. Due to ongoing abdominal pain, she underwent repeat abdominal CT, findings of which were concerning for a small bowel laceration. She subsequently underwent interventional radiologic (IR) drain placement and was continued on IV meropenem and vancomycin. Shortly thereafter, she developed septic shock and acute respiratory failure and was subsequently transferred to our institution, intubated and on vasopressors.\nUpon arrival to our hospital (day 0), she was tachycardic and febrile to 102 °F. Her laboratory findings were notable for leukocytosis of 12.4 × 103/μL, thrombocytopenia of 40 × 103/μL, anemia with hemoglobin of 10.9 g/dL, INR of 2.0, total bilirubin of 2.6 mg/dL, direct bilirubin of 1.2 mg/dL, and aspartate aminotransferase (AST) of 56 U/L. A repeat CT scan revealed bilateral pleural effusions and consolidations of the lungs, ascites, peritonitis with a loculated fluid and air collection concerning for an abscess, small adrenal lesions, bilateral hypoattenuating lesions within the kidney concerning for abscesses, and pneumoperitoneum. As a result, the patient underwent an exploratory laparotomy on day 1 with washout and enterotomy repair and right chest tube placement. Peritoneal cultures from the washout as well as blood cultures grew ESBL E. coli. From days 2–3, she was noted to have continuous blood loss in the ileostomy bag with worsening anemia nonresponsive to blood transfusions in the setting of disseminated intravascular coagulation (DIC). Due to the patient's borderline hemodynamic status and rapid blood loss, she was not deemed an endoscopic candidate and underwent an abdominal angiogram by IR on day 4. She was noted to have a brisk hemorrhage from a branch of the superior mesenteric artery (SMA) which required embolization with coils and gelfoam. Post embolization, her GI blood loss improved; however, subsequent serum analysis from days 4–5 continued to demonstrate down trending hemoglobin and platelet count and up trending liver function tests (LFTs). An ultrasound showed a thickened gallbladder with sludge but was devoid of calculi and possible common bile duct (CBD) dilation, prompting a magnetic resonance cholangiopancreatography (MRCP) of the abdomen which only showed mild pericholecystic fluid and a thickened gallbladder.\nPathology samples of the resected colon from the OSH were obtained on day 5 and demonstrated histoplasma-laden macrophages present in the vascular lumina throughout the colon consistent with severe histoplasma associated colitis [, , ]. Biopsies obtained during her initial esophagogastroduodenoscopy (EGD) and colonoscopy demonstrated diffuse inflammation with several noncaseating granulomata but were not stained for fungal entities. Due to the evidence of severe DH, the patient was started on IV liposomal amphotericin B (3 mg/kg daily) on day 5. DH was further confirmed with positive serum and urine histoplasma antigens and growth of histoplasma in blood cultures. Ophthalmologic evaluation was negative for any ocular involvement. Further workup was negative for other infections including human immunodeficiency virus (HIV), human T-lymphotropic virus (HTLV), Epstein-Barr virus (EBV), cytomegalovirus (CMV), hepatitis B, hepatitis A, Clostridioides difficile, and interferon gamma release assay.\nBy day 11, the patient was later found to have fecal matter oozing from her abdominal surgical sites and had another exploratory laparotomy with abdominal washout and drain placement. After the surgery, she was monitored closely and was assessed by physical therapy and nutritional services for her malnutrition and cachexia. Following these interventions, the patient improved clinically and was successfully extubated on day 19. A repeat CT of the abdomen showed resolution of the renal and peritoneal abscesses and the pleural effusions. Due to the severity of the histoplasma infection in conjunction with the multiple abdominal surgeries she underwent, and the resulting malabsorption, she was continued on IV liposomal amphotericin B for a total duration of three months and was then transitioned to oral itraconazole (200 mg three times a day for three days followed by 200 mg twice a day) to complete one year of antifungal therapy. She was eventually discharged to a subacute rehabilitation facility with close outpatient follow up and is now back to her baseline functional status.
A 40-year-old Korean woman was admitted to Samsung Medical Center for newly developed pulmonary nodules. The patient had no family history of malignancy. Three years prior, she had been diagnosed with cancer of the left breast and underwent left breast-conserving surgery with sentinel lymph node biopsy. Postoperative pathologic findings showed invasive ductal carcinoma and indicated that she was positive for both estrogen receptor (ER) and progesterone receptor. HER2 immunohistochemistry was 3+. The TNM stage was 1 (T1N0M0). At the same time, she was also diagnosed with papillary thyroid cancer (diffuse sclerosing variant [DSV], bilateral lobes) and underwent a total thyroidectomy with modified radical neck dissection. Since then, she has received radiation therapy for breast cancer and was given tamoxifen and goserelin as adjuvant therapy. Radioactive iodine therapy (50 mci×3) was administered three times for the thyroid cancer.\nHer chest computed tomography (CT) scan showed at least three well-defined nodules in the right upper and middle lobes, suggesting pulmonary metastases (). She underwent wedge resection of the RUL and RML using video-assisted thoracotomy for metastasectomy and a definitive diagnosis at the department of thoracic surgery. A nodule on the RUL and two nodules on the RML observed by chest CT scan were found operatively. The pathologic diagnosis was recurrent double primary cancer in both lobes.\nThere were two different components (metastatic ductal carcinoma from the breast and metastatic papillary carcinoma from the thyroid gland) in one pulmonary nodule in the RUL and RML. In the RML, a metastatic ductal carcinoma measuring 0.4 cm in size from the breast and metastatic papillary carcinoma measuring 0.15 cm in size were found. In the RUL, a metastatic ductal carcinoma measuring 0.9 cm in size from the breast and metastatic papillary carcinoma measuring 0.1 cm were found. We confirmed this diagnosis through thyroid transcription factor-1 (TTF-1) and ER immunohistochemical staining (). There were two components on each slide. In the TTF-1 stain, we observed positive signals in the papillary carcinoma component. The other tumor components showed ER-positive signals from the ductal carcinoma of the breast (). Intratumoral double primary carcinomas were found together in the three resected metastatic tumors.\nSince then, the patient received six cycles of docetaxel plus trastuzumab combination chemotherapy and additional radioactive iodine therapy. She is currently under treatment with trastuzumab and tamoxifen with stable disease.
We present a case of a 36-year-old male wrestler, presented with pain in his chest and in his left leg for the past two days. He also complained of pain in the big toe. There is no history of palpitation, dyspnea, sweating, or cough. No fever or headache. There is no history of trauma of lower limbs or any neurological deficit. There is no urinary or bowel trouble. There is no history of vomiting, diarrhea, or pain in abdomen. The patient is a chronic cigarette smoker with a history of two packets of cigarettes per week for the past 20 years. There is no history of recreational drug use.\nThe patient had a history of recurrent deep vein thrombosis, pulmonary embolism (PE), obstructive sleep apnea, insulin-dependent type ll diabetes mellitus, and candidal balanitis. There is a history of multiple surgeries in the past. The patient had undergone left elbow surgery, left knee surgery, right ankle surgery, and foot surgery, due to injuries caused by his profession. The patient has mild pitting edema in both feet.\nOn examination, the patient is extremely obese with a weight of 456 lbs., a height of 188 cm, and a BMI of 63.53 kg/m2. His blood pressure is 99/56 mm of Hg, a temperature of 98.6 F, respiratory rate of 16 per minute, and oxygen saturation of 98%. The patient is fully conscious and well oriented. There is no anemia, cyanosis, or jaundice.\nDoppler report of the lower limbs showed bilateral deep vein thrombosis (DVT) of popliteal veins and left common femoral vein, otherwise normal Doppler flow compressibility of profunda femoris and superficial femoral veins. Based on the clinical, laboratory, and the Doppler report, the patient was diagnosed with pulmonary embolism with bilateral DVT of popliteal veins and left common femoral vein (Table ).\nTreatment\nThe patient was admitted to the inpatient unit. After consulting cardiologist, IV heparin was started right away for DVT with pulmonary embolism.\nHe was also prescribed supportive therapy for controlling blood glucose and obesity, in consultation with an endocrinologist and dietician. The patient was advised for lifestyle modifications such as losing weight and staying active, to manage DVT, obesity and co-morbidities.
A 62-year-old woman underwent a periodic computed tomography (CT) examination for hepatitis B at another hospital, where she was diagnosed with significantly advanced gallbladder cancer. Her weight was 50.5 kg and her height was 153 cm, and a physical examination revealed no abnormalities. Laboratory tests showed slight elevation of the carbohydrate antigen (CA) 19-9 level at 57 U/ml; however, most of the other results were normal. Enhanced abdominal CT scans obtained on the first admission showed a large mass lesion located in the gallbladder that had invaded the anterior and medial segments of the liver. In addition, two annular enhanced masses in Couinaud’s hepatic segments 4 and 8 (S4 and S8; Figure a) were observed on CT examination. The patient was therefore diagnosed as having advanced gallbladder cancer with direct liver invasion and hematogenous metastasis to the liver, T4 N0 M1; stage IVb according to the American Joint Committee on Cancer tumor-node-metastasis (TNM) classification. The disease was judged to be inoperable, owing to the liver metastasis.After obtaining the patient’s informed consent, chemotherapy with gemcitabine plus S-1 was started at a dose of 1,200 mg of gemcitabine once a week for three weeks and 100 mg of S-1 every day for three weeks, followed by one week of rest. Because the patient experienced grade 3 appetite loss, according to the National Cancer Institute common toxicity criteria, the frequency of S-1 administration was reduced to every other day, starting with the second cycle of chemotherapy. This change allowed the patient to continue to receive the treatment as an outpatient. The serum CA19-9 level gradually decreased, and a follow-up CT scan performed after four cycles showed the main lesion to have dramatically reduced in size, while the two liver metastases had completely disappeared (Figure b). At that time, we were consulted regarding the feasibility of surgical treatment. We therefore recommended an additional two cycles of chemotherapy, hoping to obtain a further effect and confirm the absence of distant metastasis.\nAfter the two additional cycles, a CT scan showed that the tumor had further shrunk in size and was detected as only a slightly contrasted lesion in the liver bed. The serum CA19-9 level was 13 U/ml at that time. 18 F fluorodeoxyglucose-positron emission tomography (FDG-PET) showed no uptake of FDG in the main lesion or evidence of distant organ metastasis.One month after the final cycle of chemotherapy, we performed cholecystectomy and central bisegmentectomy of the liver with D2 lymph node dissection, which confirmed the absence of both peritoneal dissemination and liver metastasis, based on intraoperative macroscopic and ultrasound findings. Pathological examination demonstrated viable cancer cells (moderately differentiated adenocarcinoma) with granuloma formation and calcification on the free peritoneal side of the gallbladder. The cancer cells were found to have spread widely in the mucosal layer of the gallbladder, although no direct invasion to the liver was observed. Clusters of viable cancer cells were detected in the liver bed apart from the main lesion, diagnosed as liver metastases. In addition, three lymph node metastases were noted on the posterior surface of the pancreatic head. The areas of induration in the S8 segment showed microscopic regenerative changes without viable cancer cells; these findings were histologically compatible with a chemotherapeutic complete response (Figures and ).\nThe patient exhibited an uneventful postoperative course. The administration of gemcitabine plus S-1 was restarted as adjuvant chemotherapy and six cycles of the treatment were performed. One and a half years have passed since the operation, with no signs of recurrent disease.
A 46-year-old lady complained of awareness of breast mass. The mass was present for around 12 years and she was taking homeopathic medicines on and off for the same. There was history of waxing and waning in the size of the mass. There was a recent increase in the size of the mass and the surgeon referred the patient for a mammogram and FNA. On examination, the lump measured 3.5 cm, which was very firm to hard in consistency and had restricted mobility. The nipple was mildly retracted. No lymph nodes were palpable in the axilla. Mammography of left breast showed a high-density mass in the central region suspicious of malignancy; however, there was no evidence of microcalcifications. The lesion was classified as BI-RADS 4.\nFNAC was performed using a 23-gauge needle and smears were air-dried and stained using May Grünwald-Giemsa method as well as fixed in 95% ethanol and stained with hematoxylin and eosin. Lump was gritty on FNA and yielded particulate material. The smears were highly cellular and showed predominantly three-dimensional cohesive clusters and few scattered cells [Figure and ]. At places, the clusters showed acinar formation []. Occasional clusters of apocrine cells and singly scattered large cells with moderate nuclear pleomorphism and conspicuous nucleolus were also found []. Admixed benign cell clusters, few bipolar cells and hyalinized eosinophilic stroma were found in the background []. Considering the overall picture, a cytological diagnosis of ductal carcinoma was made, and she was advised surgical excision and histopathological examination to rule out invasion. Lumpectomy was performed, and the histopathological examination of the specimen showed a nodular lesion with expanded lobular units which showed extensive adenosis with marked sclerosis of intervening stroma. The proliferating glands were distorted in many areas and showed apocrine metaplasia at places. The lining cells showed moderate nuclear pleomorphism and atypia []. The individual glands were surrounded by myoepithelial cells which were confirmed by immunohistochemistry for smooth muscle actin (SMA) []. There was no evidence of any associated lobular or ductal intraepithelial neoplasia. The surrounding area showed features of fibrocystic disease. There was no evidence of an in situ or invasive malignancy. Based on these features, a diagnosis of nodular sclerosing adenosis was made.
An eight-year-old, male child, who was diagnosed as a case of Attention Deficient Hyperactive Disorder (ADHD) was treated in a private dental hospital for a fractured right central incisor (tooth 11). Considering the history of previous dental treatment that had failed and with further discussion with the child's pediatrician, it was decided that the best treatment option for the patient was to provide dental treatment using sedation. In the next dental visit the child was given a combination of Chloral hydrate 25 mg/kg, Hydroxyzine 1 mg/kg, and Meperidine 1 mg/kg for sedation. The patient was placed in a supine position and physically immobilized using a Papoose Board (Olympic Medical Corp, Seattle, Wash) before treatment was initiated. A mouth prop was inserted and access opening done in tooth 11, followed by placement of the calcium hydroxide intracanal medicament (Calcicure, Voco, Germany). During the next visit, the child was sedated using the same combination. As the patient exhibited slight discomfort with the placement of the rubber dam, the procedure was carried out without isolation. During the biomechanical preparation, the patient suddenly moved his head, due to which an endodontic instrument (Pro Taper hand file, Dentsply) slipped from the dentist's hand and the patient swallowed it. The procedure was stopped immediately and measures were taken to retrieve the file from the posterior region of oral cavity. The initial assessment by the dentist revealed that the patient was choking and had cough with no obvious signs of respiratory distress. The operator made a provisional diagnosis of foreign body aspiration.\nThe patient was immediately admitted to the Emergency Room for evaluation. A posteroanterior radiograph of the chest demonstrated the presence of a sharp foreign body at the level of the T4 vertebral body []. A diagnostic Computed Tomography scan (CT) was taken, which showed the endodontic instrument impacted on the left main bronchi []. Bronchoscopy done under General Anesthesia revealed that the endodontic instrument had pierced the mucosal folds tangentially, and was embedded in the wall of the left bronchial mucosa, buried up to the handle []. Using a bronchoscopic grasper, the handle of the endodontic instrument was grasped, gently pulling it out of the mucosal fold []. The site of penetration of the mucosa was inspected for bleeding and perforation. The endodontic instrument was then retrieved along with the withdrawal of the bronchoscope, under full visualization throughout the retrieval process. The patient was subsequently discharged with no complaints of discomfort on follow-up.
A 48-year-old Japanese man with a 5-year history of maintenance hemodialysis for diabetic nephropathy presented with spikes of fever every few months accompanied by the appearance and regression of multiple painful subcutaneous nodules over his legs and feet. These were diagnosed as erythema nodosum by a biopsy examination. Investigations showed had high levels of C-reactive protein (150 to 200 mg/L), a hemoglobin level of 146 g/L, a white blood cell count of 7.0 × 109/L, with 92% neutrophils and a platelet count of 2.2 × 1011/L, indicating a lack of anemia in spite of chronic inflammation. Administration of broad-spectrum antibiotics failed to generate a response. Peripheral lymph nodes were not palpable. The patient was also found to be negative for human immunodeficiency virus (HIV) infection. Sputum examination revealed no significant pathogenic bacteria, and an acid-fast stain test and a Mycobacterium tuberculosis polymerase chain reaction (PCR) test were both negative. Blood and urine cultures were also negative, and renal ultrasonography did not show renal cysts or masses. Several tests for collagen disorders including vasculitis were all negative.\nComputed tomography showed axillary lymph node enlargement (Figure A), which was corroborated by a gallium scan that revealed abnormally high gallium uptake in the axillary lymph nodes (Figure B). When administered the Mantoux test, the patient was found to be purified protein derivative, slightly positive at 10 mm, which points to tuberculosis infection. Tuberculosis was further indicated by a positive interferon-gamma release assay. Chest radiography revealed no abnormal shadowing in the lung fields, thus ruling out pulmonary tuberculosis. Based on these results, we suspected lymph node tuberculosis, and axillary lymphadenectomy was performed. Six lymph nodes were surgically removed and their histological examination revealed caseating granulomata in all of the lymph nodes (Figure A). Langhans giant cells were also observed in the granuloma. Further, the granuloma tested positive on an acid-fast bacteria stain (Figure B) and a M. tuberculosis PCR test. He was treated with rifampicin, isoniazid, and ethambutol.\nTo our surprise, lymphadenectomy was followed by a rapid decline in the patient’s hemoglobin levels from 144 to 105 g/L. Serum Epo levels also decreased from 223 mIU/mL to 10.7 mIU/mL (upper normal limit 23.7 mIU/mL for normal adult) by postoperative day 21. The patient received a weekly injection of 40 mg iron sucrose, and his serum ferritin level was maintained at 50–150 ng/mL. The patient at this stage needed Epo infusions to maintain hemoglobin levels (target hemoglobin between 100 and 120 g/L). The strong association between the excision of the tubercle and the drop in Epo levels made us examine the granuloma for Epo expression. The tubercle granuloma was found to be positive for Epo on in situ hybridization (Figure A). Methods of in situ hybridization are described in the Additional file . We further validated this by immunohistochemical staining, wherein strong cytoplasmic staining for Epo was found in these tubercle granuloma cells. The Epo-producing cells were positive for CD68 (monocyte/macrophage marker) but not for CD20 (B lymphocyte marker) or CD8 (cytotoxic T lymphocyte marker) (Figure B-D).
A 33-year-old woman was admitted to our hospital (Ali Ebne Abitaleb Hospital, Zahedan, Iran) due to prolonged fever, neck masses and the swelling of her left lower limb. She complained that fever had been prolonged for 3 months, being persistent during the whole 24 hour, without special pattern during days or nights. She also mentioned that she had spent a short period of productive cough 3 months ago, during which she found some small tender masses on her neck. Her productive coughs improved without medication, but fever and neck masses persisted. Two months later, she visited a surgeon for her persistent neck masses and fever and subsequently underwent an excisional neck mass biopsy. The pathologist reported a lymph node (7mm in diameter) infiltrated by normal shaped lymphocytes, suggestive for Toxoplasma infection and, to a lower probability, viral infections or lymphocyte predominant Hudgkin lymphoma. So the specimen was sent for immuno-histochemistric (IHC) evaluation.\nHer fever persisted, but she did not lose appetite or significant weight. She also did not have night sweats but was suffering from fatigue. Two weeks before admission, she noted the gradual swelling of her left lower limb, accompanied by dull pain. She visited a doctor and performed some laboratory tests, including complete blood count (CBC), renal and liver function tests which all showed normal results. She did not continue doing more evaluation for her problem and used no medication. Then, she felt the progression of fatigue as well as slight darkening of her urine, so came to our hospital.\nShe neither mentioned close contact with a known case of Tuberculosis, nor cats around her living place, but used unpasteurized milk and yoghurt. She was a house-wife and was uneducated. She had 2 children, being delivered by cesarean-section and had no history of abortion. She remembered that she had passed a course of DVT in her left leg after her first delivery, being 4 years ago, for which she had been under treatment for several months, but could not remember the name of her medications. She did not smoke, but her husband was smoker and IV-drug abuser.\nOn physical examination, she seemed ill, but was oriented. Her blood pressure was 120/80 mmHg. Pulse rate was 108/min and temperature was 39.5 °C (orally). She was pale. Her neck examination showed 5 non-tender, soft and mobile lymph nodes, with maximal diameter of 7-8mm bilaterally, both in anterior and posterior cervical lymph node chains. Her pharynx looked normal, but there were multiple small erosions on her soft palate. The patient was not aware of those erosions and felt no pain. The examination of heart, lungs and breasts were normal. The abdomen was soft, without organomegaly. Her left lower limb seemed edematous. The difference between the circumferential size of her left and right lower limbs were 4cm and 5cm for the legs and the thighs, respectively.\nThe primary laboratory data are shown in . The patient was also tested for Toxoplasma antibody (IgM & IgG), Wright test (due to consumption of unpasteurized dairy products), Purative Protein Derivative test (PPD) (due to the high prevalence of Tuberculosis in the region), and also HBS antigen, HCV antibody and HIV antibody owing to the presence of risk factors in her husband. Color Doppler ultra-sonogragphy showed the presence of thrombus in the left femoral and iliac veins. Heparin infusion and then warfarin (5mg-PO-daily) were started. Abdominal ultrasonography (US) reported a small (4-5mm) hyperecho lesion in the liver (The sonographist suggested that it must have been a hemangioma). No organomegaly or intra-abdominal lymphadenopathy was detected by US. Renal parenchymal echogenisity was slightly increased, with no hydronephrosis.\nAt the same day, the patient reported diarrhea (6 times per day with small volume). So stool specimen was sent for evaluation. Due to the presence of Anemia and Thrombocytopenia, the patient's peripheral blood smear (PBS) was observed, showing nucleated RBCs and multiple schistocytes and fragmented RBCs. No malignant or leukemic cell was observed on PBS. Furthermore, white blood cell (WBC) count on PBS was 2,750/ml (with 85% polymorpho-nuclear cells and 10% lymphocytes) and platelet count was 50,000/ml.\nAccording to the presence of microangiopathic hemolytic anemia, thrombocytopenia, fever and renal involvement, the diagnosis of TTP was assumed at the 2nd day of admission. Fresh frozen plasma (FFP) was administered (2 units every 8 hour) and the patient became a candidate for plasma exchange with FFP (2 liters daily, according to her weight).\nFollowing the diagnosis of TTP, the presence of anti-nuclear anti-body (ANA), anti double-stranded DNA (anti-dsDNA) were tested. Because of the previous history of DVT, the patient was also tested for anti-phospholipid and anti-cardiolipin antibodies (both IgM and IgG). At the time, the patient was retrospectively asked about photosensitivity and she gave positive answer. The result of stool examination showed Giardia lamblia trophozoites, so metronidazole was started (250mg PO every 8 hour).\nAt the 3rd day, the patient complained of right knee pain. Her right knee was warmer than the leg surface and the opposite knee. It had mild effusion and was painful during both active and passive movements, but had no tenderness or erythema. Other peripheral joints were normal on examination. The frequency of her defecations had decreased, but she was still febrile and did not seem to be well and even seemed worse and lethargic to some extent. Her blood pressure rose to 160/90 mmHg. Hydrocortisone (200mg IV as a stat dose) and then, dexamethasone (8mg IV every 6 hours) were administered and it got possible to initiate the 1st session of plasma exchange.\nAt the 4th day, fever had subsided; she seemed much better and had no articular complaint. The results of ANA and especially anti-dsDNA (76 E/ml, with normal range being less than 20 E/ml) were positive. Other tests including anti-phospholipid and anti-cardiolipin antibodies, PPD, Wright, HBs-Ag, HCV-Ab and HIV-Ab had negative results. In addition, IHC evaluation of the lymph node was negative for Hudgkin lymphoma. Additional urinalysis of the patient (3 days after discontinuation of menstrual bleeding) showed persistent proteinuria and hematuria, so urine sediment was observed, showing many RBCs with dysmorphic shapes (60% of total RBCs) as well as RBC casts. The 24-hour examination of the urine showed 14341mg protein and 760mg creatinine in 1700cc urine volume. Therefore, presence of 7 of 11 American College of Rheumatology (ACR) criteria (oral ulcers + photosensitivity + renal involvement + positive ANA and anti-dsDNA tests + hematologic abnormalities including leukopenia, lymphopenia and thrombocytopenia + arthritis) allowed the diagnosis of SLE.\nAfter 5 plasma exchange treatment procedures, serum creatinine decreased to 1.6 mg/dl, platelet count was 100,000 /ml and LDH was 582 U/l. Additional observation of the PBS showed significant decrease in fragmented RBC or schistocyte counts. Fever did not recur and the patient had the feeling of complete well-being, so that she did not accept remaining hospitalized and was self-discharged at the 8th day of admission, despite frequent explanation to the patient about the necessity for completing the plasma exchange treatment course and the need for more evaluation of her renal function status and her DVT. So dexamethasone was changed to prednisolone (50 mg PO daily) and she was advised not to change the dose or discontinue the drug without her physician consult. Warfarin (5mg daily) was continued and the patient was advised to check her International Normalized Ratio (INR) every week. She was also advised to visit a nephrologist, a rheumatologist and a hematologist during the coming week and of course, was advised to come back to the hospital as soon as possible, if not feeling well.\nOne week later, the patient returned to the hospital due to severe edema, headache, mild dyspnea (orthopnea) and delusion. She seemed ill and puffy. On examination, her blood pressure was 180/90 mmHg, respiratory rate was 18/min and body temperature was normal. Normal vesicular sound could not be heard on the base of her right lung and was dull on percussion. Heart sounds seemed normal with no bruits. Both upper and lower limbs were edematous. She was still taking prednisolone (50mg daily) and warfarin (5mg daily). Laboratory data are shown in .\nThere were many fragmented RBCs on PBS. Her chest X-ray showed right sided blunting of costophrenic angle. Her electrocardiogram showed low voltage complexes in all precordial parts. She went under echocardiography, but it was negative for pericardial effusion.\nPlasma exchange with FFP was started again and 3 methyl prednisolone pulse (lg IV daily, repeated for 3 days) and then a cyclophosphamide pulse (lg IV) was administered. The diuretic furosemide and Captopril were also started to control her blood pressure and to lower her edema. She became also a candidate for renal biopsy, so it was decided to stop warfarin for 1 week and to start heparin infusion during this period. After 6 courses of plasma exchange, the platelet count was 131,000 /ml and LDH was 505 U/l, so plasma exchange was tapered in volume and the interval durations got longer. She seemed well, her edema was decreased and her blood pressure was 140/80 mmHg and she did not complain of dyspnea anymore. Here, the patient refused renal biopsy.\nFinally, she was discharged home after 11 plasma exchange courses. She was advised to continue warfarin and check INR every other week and to take prednisolone 50mg daily for the following 2 weeks, then being tapered by a rheumatologist. She was also advised to receive cyclophosphamide pulses every month for 6 months, under the observation of a nephrologist. It must be mentioned that, we noted no delusions during her hospitalization course.
A 56-year-old woman came to our hospital because of epigastralgia and hepatomegaly about 15 years before developing a hepatic tumor. Enhanced computed tomography (CT) and angiography revealed hepatic vein and inferior vena cava (IVC) stenosis at the level of confluence, so she was diagnosed as BSC from congenital angiodysplasia or previous phlebitis and received IVC stent placement in our institution. Acetylsalicylic acid (100 mg/day) was prescribed after stent placement. Enhanced or unenhanced CT was performed once a year during her routine visit to our hospital for follow-up. The result of the hepatitis B and C virus test remained negative. There were no past history of drinking alcohol or chronic liver disease and no family history of hepatobiliary malignancy. Contrast-enhanced CT 13 years after stent placement showed a spot-like enhancement about 5 mm in diameter in the peripheral region of segment 8 of the liver (). The alpha-fetoprotein (AFP) level was below the reference value at that time. This enhancement was suspected to represent a small portal venous shunt or hepatic tumor such as HCC. At the time of CT, she showed an allergic reaction to the contrast material and developed facial edema. Intramuscular injection of epinephrine and an antihistamine drug resolved the symptom within hours. After the allergic episode, unenhanced CT and ultrasonography were performed for annual follow-ups of the IVC stent and nodular enhancement in the liver.\nThe hepatic lesion had increased to 1.6 cm in diameter on ultrasonography in the subsequent 2 years. In addition, laboratory data showed elevated AFP (70 ng/mL) and lens culinaris agglutinin-reactive fraction of AFP (AFP-L3, 85.5%), although data had been normal until then. Because growth of the hepatic tumor was strongly suspected from those findings, the primary physician decided on further imaging. The IVC stent was a handmade stainless-steel device and significant artifacts were anticipated on magnetic resonance imaging (MRI), so dynamic CT with contrast material under steroid preparation was performed for diagnosis. Dynamic CT revealed a well-circumscribed, low-density area (mean CT value, 42 Hounsfield unit [HU]) without pseudocapsule in the peripheral liver (). The nodule showed strong homogeneous enhancement (mean CT value, 145 HU) in the arterial phase (). Enhancement was prolonged to the portal phase and decreased to almost the same level as surrounding hepatic parenchyma in the equilibrium phase (mean CT values, 169 and 131 HU; , respectively). Based on these laboratory data, imaging findings, and the prevalence rate of HCC for BCS, the preoperative diagnosis of the lesion was HCC, clinical stage T1N0M0 in the union for international cancer control (UICC) staging system. Partial hepatic resection was performed for the tumor.\nIntraoperative ultrasonography was performed to define the cutting line of hepatic parenchyma, and resection was carried out under intermittent interruption of blood flow to liver with a cavitron ultrasonic surgical aspirator and bipolar electrosurgical unit. Intraoperative findings suggested slight fibrotic change to the background hepatic parenchyma. The tumor was completely resected with sufficient surgical margins. No complications were encountered intra- and perioperatively and the patient was discharged 10 days postoperatively. No sign of recurrence was seen during the follow-up and AFP was returned to normal level.\nHistopathological findings revealed a tumor comprising atypical cells with small, round nuclei and eosinophilic cytoplasm, forming ductal and alveolar structures (). No mucin production was observed. The tumor showed a replacing growth pattern without a hyalinized capsule and the border with the surrounding liver was vague. On immunostaining, the atypical cells were partially positive for hepatocyte specific antigen (HSA) and positive for cytokeratin (CK) 7 and CK19 (). Epithelial membrane antigen (EMA) was strongly positive in the tumor, mainly localized to the apical surface of the tubules. The tumor also has the component partially positive for AFP. The final diagnosis was C-HCC-CC with stem cell features, cholangiolocellular subtype. Inflammatory changes and slight fibrosis without distortion were observed in the surrounding hepatic parenchyma around the portal area. The liver damage was classified as A2F2 according to the new Inuyama classification, which was compatible with BCS-related hepatic damage. The patient had no other chronic hepatic disease, so the damage was supposed to be caused by congestion of hepatic vein even after IVC stent placement at the level of hepatic vein.
A 32 year-old woman was admitted due to intermittent abdominal pain which had persisted for one month. She had neither any remarkable past medical history nor family history. On physical examination, there was tenderness on the left upper quadrant of the abdomen; however, there was no palpable mass in the abdomen or no tenderness in both costovertebral angles. Laboratory data revealed that the lactate dehydrogenase (LDH) had risen to 1,114 IU/L. The others were within normal limits. Chest plain film showed streaky radiopaque densities on the right upper lobe, considered a scar of the inactive tuberculosis. Abdominal ultrasonogram showed a well-defined mixed echogenic mass on the left kidney. Abdominal computed tomography (CT) scan showed a well-defined mass with heterogenous enhancement on the lower pole of the left kidney with hilar infiltration, measured to be 12×10 cm in size (). Small-sized hilar lymph nodes and a thrombus in the left renal vein were observed. On day 23, a left radical nephrectomy was performed with left renal vein thrombectomy. A gross specimen was obtained from the left kidney of the patient, a 13×12×11 cm-sized mass on the lower pole of the kidney. The cut surface of the mass was grayish-white mixed with focal necrosis and hemorrhage ().\nA light microscopic finding revealed a highly cellular tumor composed of mitotically active (to the degree about 30 mitoses/10 high power field), atypical plump spindle cells, and indistinct cell borders arranged in short and intersecting fascicles, mainly involving the renal pelvis with extension to the renal cortex and capsule (). Malignant cells were not found in the dissected lymph nodes or in the renal vein.\nOn immunohistochemical stains, the tumor cells showed positive reaction to vimentin (), but showed negative reactions to cytokeratin, epithelial membrane Antigen (EMA), S-100 protein, and CD34. There were no extrarenal manifestations, and we diagnosed the tumor as spindle cell-type monophasic synovial sarcoma, which had originated from the kidney.\nFour months later, chest radiography showed a number of small nodular opacities considered as hematogenous metastasis. Chest CT scan showed variable sized, nodular opacities with vascular connection in the entire lobe of the right lung (). Percutaneous transthorasic needle biopsy (PTNB) on the right lower lung nodule was performed, and the pathologic finding was consistent with renal synovial sarcoma (). She had received combination chemotherapy with 28-day cycles of ifosfamide 2,500 mg/m2 on day 1 through 3 plus doxorubicin 60 mg/m2 on day 1. After the second cycle of chemotherapy, follow-up chest CT scan showed smaller-sized metastatic nodules on the right lung field, and the state of the response was partial remission. Toxicity was scored according NCl criteria. During chemotherpay, grade 2 neutropenia and grade 1 thrombocytopenia was developed. Non-hematologic toxocities were grade 1 nausea and grade 3 alopecia. Planed doses were administered without dose reduction. After 6 cycles of chemotherapy, complete remission was achieved ().
A premature, 34-week-old, 930 g, male neonate was born by cesarean section to a hypothyroid mother. The first one month was complicated by hypoglycemia, seizures and low hemoglobin. The patient was on breast milk and also supplements. He had physiological jaundice on day 3 of life which required phototherapy. After phototherapy, his bilirubin was 4.6 mg% out of which direct component was 1.2 mg%. At the age of one and half months, the patient passed clay-colored stool and was icteric. It was negative for stercobilinogen. His obstructive jaundice work up showed raised bilirubin of 9.5 mg% with direct bilirubin of 6.5 mg% and raised alkaline phosphatase 1020 IU. The ultrasonography had normal biliary tract with no dilatation of biliary radicals. Common bile duct diameter was 4 mm. At this point, surgical opinion was sought by the pediatrician. Hepatobiliary imino-diacetic acid (HIDA) scan was not conclusive as there was uptake by liver as well as early excretion, but there was no progress of dye in the intestine during 24 hours. So, exploratory laparotomy was done keeping in mind about the biliary hypoplasia or inspissated bile syndrome.\nThe patient's weight was 1.2 kg when he was taken for surgery. Gallbladder was distended and there was bile on aspiration. The extrahepatic biliary apparatus was normal. Intraoperative cholangiogram suggested dye was going in the pancreatic duct, but there was no dye in the intestine with terminal dilatation of CBD []. After kocherization, longitudinal duodenotomy was done in the second part of duodenum. On duodenotomy, there was choledochocele of size of less than 1 cm in diameter []. A vertical incision was taken on the cyst. There was gush of bile []. Because of very small choledochocele, we were not able to excise the mucosal lining of the cyst nor were we able to suture incised edges and take biopsy of the wall. Also, we could not identify opening of pancreatic duct. On table cholangiogram confirmed the presence of dye in the intestine []. Later, duodenotomy was closed. The postoperative period was uneventful. His bilirubin after 2 weeks of surgery was normal and stool was normal looking. A follow-up HIDA scan after 6 months had good uptake and free flow of dye in the intestine. At 12 months follow-up, the child was asymptomatic and thriving.
A 63-year-old African American woman presented to the Plastic Surgery clinic from her nursing home with a 2-year history of a painful distal forearm mass. The lesion was initially thought to be a keloid from a stab wound more than 20 years ago; however, she decided to have it evaluated because of a recent increase in size and intermittent bleeding. She denied any numbness, tingling, or weakness of the hand. Her neurovascular examination had normal findings. Clinically, the mass was located on the volar aspect of the mid-forearm, was pink and ulcerated, and 3 × 3 × 4 cm in size (). There was no associated supracondylar or axillary lymphadenopathy.\nBecause of the unique characteristics of the mass, we further evaluated the lesion with a magnetic resonance image of the right forearm. This image demonstrated a 3.8 × 2.1 × 4.1-cm mass in the subcutaneous tissue without invasion into the fascia or muscle (). The lesion was concerning for malignancy, so she was referred to a surgical oncologist for excisional biopsy. One month later, the mass was excised en bloc with careful dissection of the deep margin to maintain the fascia. We used a 6 × 4-cm elliptical incision, and this defect was closed primarily using suprafascial flaps for a tension-free closure.\nOn final pathology, the entire specimen was 6 × 4 × 0.8 cm. It was found to be an invasive nodular melanoma with positive, deep, and radial margins. This case was discussed at our multidisciplinary tumor board with plans of positron emission tomographic (PET) scan, reexcision, and sentinel lymph node biopsy.\nBefore her postoperative follow-up appointment, she underwent a PET scan, which unfortunately demonstrated a hyperactive node in the right axilla as well as a concerning left breast lesion. At her follow-up appointment, we offered a wide local excision with sentinel lymph node biopsy; however, the patient refused to have further surgery. On physical examination, there was a small, red, scaly lesion in the middle of the surgical site concerning for rapid recurrence. We referred her to a breast surgeon for workup of the left breast lesion, which ultimately was found to be ER/PR+ invasive lobular breast cancer. The patient was scheduled for close follow-up in both clinics but had not made her appointments. After reviewing the medical records, the patient refused to have any further surgical procedure at this time.
The patient, a 36-year-old Caucasian man, initially presented with altered mental status and a 5-day history of progressively slurred speech. The patient progressively became non-verbal and then developed fecal and urinary incontinence with watery diarrhea. This patient had no history of prior cerebral nervous system events or head trauma. In addition, there is no history of recent illnesses or any prior medical conditions. Pertinent social history includes an occupation focusing on outdoor pest and termite control for 13 years as well as an 8-year history of IV oxycodone use. There is no pertinent family history and the patient denies any outdoor hobbies. On physical exam, the patient remained afebrile, had delayed speech and communicated mainly through hand gestures. Comprehension was limited to single-step commands but strength was 5/5 bilaterally in both the upper and lower extremities. The remainder of the physical exam was unremarkable. The only laboratory abnormality discovered was a mild leukocytosis at 12 800 ul−1. His urine drug screen was negative, and both the patient and family denied any drug use. In addition, the urinalysis, chest x-ray and electrocardiogram were all unremarkable. The patient’s head computed tomography without contrast demonstrated the presence of possible bilateral infarcts or masses involving the basal ganglia and periventricular white matter greater on the right.\nThe next day neurology examined the patient and aside from new onset right facial diplegia, the physical exam findings remained unchanged from above. At that time, a magnetic resonance imaging (MRI) scan with and without contrast, a magnetic resonance angiography (MRA) scan of the head, a carotid ultrasound and an electroencephalogram (EEG) were all ordered. The EEG returned a finding of frontal intermittent rhythmic delta activity, a non-specific finding, often found in the setting of toxic or metabolic encephalopathies. The MRI results remained uncertain of whether the patient was presenting with bilateral masses or infarcts. Of note, paranasal sinuses and mastoid air cells were reportedly clear. The patient’s condition and prognosis at this time remained unclear and after several days his condition seemed to improve clinically according to family members. The patient’s improvement, perceived low suspicion for infection, and the current prevailing diagnosis of neurosarcoidosis prompted the initiation of a 5-day course of intravenous methylprednisolone 1 g/day. On day 3 of hospitalization, the patient continued to have diarrhea and reported a headache of unknown duration. Steroid administration led to a mild improvement in the patient’s condition, but the patient continued to remain mostly non-verbal.\nOn day 6 of hospitalization, the patient developed a new onset left hemiparesis, which prompted neurology to conduct a lumbar puncture showing a red blood cell (RBC) count of 35 µl−1, white blood cell (WBC) count of 590 µl−1 with 82 % lymphocytes, a glucose of 41 mg dl−1, and protein of 140 mg dl−1. The lumbar puncture was suggestive of an inflammatory process consistent with autoimmune, paraneoplastic or viral process. This was consistent with the initial course of treatment, however due to the lack of improvement, the etiology of this neurocognitive dysfunction was reassessed. On day 7 of hospitalization, the patient had a repeat MRI that demonstrated increased cerebral edema on the right side with the center of the mass demonstrating necrosis (see ). On day 8, the patient’s mental condition declined, his left hemiparesis worsened and the patient was no longer able to follow simple commands. Because of the prevailing thought of an autoimmune etiology, the patient was begun on dexamethasone 4 mg every 6 h. On hospitalization day 11, with recommendation from neurosurgery, the patient received a right basal ganglia and deep white matter stereotactic brain biopsy, which demonstrated non-caseating granulomatous inflammation with giant cells (see ). With this biopsy result, the infectious disease specialist recommended the addition of liposomal amphotericin B to cover the possibility of a fungal etiology. On day 14, the full pathology report returned with Gomori methenamine-silver stain revealing the presence of fungal hyphae favoring non-septate/pauciseptate hyphae (see ), which was consistent with an isolated cerebral mucormycosis infection. More specifically, PCR demonstrated Rhizopus oryzae fungus. Otolaryngology was consulted to identify a source of the mucormycosis infection; however, no point of entry could be ascertained as the paranasal sinuses were clear and no cutaneous manifestations were present. Due to the extent of the neurologic involvement, posaconazole was incorporated into treatment at the suggestion of the otolaryngologist. Of note, the otolaryngologist identified an otitis media but denied it as the source of the mucormycosis infection. After about 4 months of consistent antifungal therapy, the patient has improved significantly. He has regained much of his neurological functioning, including the ability to speak intelligibly and purposefully move his extremities. While the patient is still in recovery, he is slowly approaching a level commensurate with his pre-infection state.
A 74-year-old male presented in February 2016 with increasing dysphagia. There was no history of prior abdominal infection or surgery. On esophagogastroscopy, a necrotic and circumferential friable tumor was seen at 33 to 40 cm from the incisors, with an endoscopic appearance of involvement of gastroesophageal (GE) junction and the proximal 2 cm of the stomach. Biopsies of the distal esophageal tumor confirmed poorly differentiated adenocarcinoma. The patient was anemic with a hemoglobin of 89 g/L. Staging endoscopic ultrasound suggested a breach of muscularis propria and four enlarged paraesophageal nodes. Neoadjuvant chemoradiotherapy followed by esophagectomy was initially considered; however, a staging positron emission tomography (PET) scan demonstrated 18-fluorodeoxyglocose (FDG) uptake not only in the primary tumor, but also in the paraesophageal region near the GE junction and upper abdominal lymph nodes extending as far inferiorly as the right renal vessels, in a retrocaval location (Figure ).\nA radiation oncology consultation was sought regarding treatment options of such extensive lymphadenopathy. Palliative radiation therapy (RT) was recommended. The patient was also evaluated by a medical oncologist who advised that chemotherapy may be considered after assessing the response to palliative radiotherapy.\nFrom March 21, 2016 to April 5, 2016, the patient received palliative RT to the symptomatic primary tumor and closest adjacent nodes using a pair of anterior and posterior fields. A total dose of 30 Gray (Gy) was prescribed over 10 daily fractions. As the lymphadenopathy in the lower abdomen was not symptomatic, and would have contributed to increased toxicity, this region was deliberately excluded from the high dose RT volume (Figure ). Other than very mild odynophagia, the patient had no other RT-related side effects. On the first follow-up visit, one month following treatment completion, he had improved swallowing function and a weight gain of six pounds.\nFollow-up computed tomography (CT) scan was obtained on May 24, 2016 to evaluate for the suitability of chemotherapy and to serve as a baseline during systemic therapy. This demonstrated persistent thickening of the lower esophagus, with lymphadenopathy reported to have decreased in size and no significant retroperitoneal adenopathy. When given the option of receiving palliative chemotherapy, the patient declined and chose to continue on observation only. Further CT scans in August and October 2016 showed a complete response in the irradiated primary tumor and nodes, with a stable 10 mm lymph node at the right renal vein.\nIn January 2017, due to symptoms of increasing dysphagia, the patient was assessed by a thoracic surgeon for consideration of esophageal stent placement. Endoscopy on January 12, 2017 noted that there was a possible small amount of residual tumor at the GE junction, but there was no significant narrowing or stricture, and no biopsies were taken. A further CT scan on April 10, 2017 showed minor circumferential thickening of the distal esophagus, but unchanged from previous. Paraesophageal lymphadenopathy was reported to be unchanged. The PET-positive lymph node at the renal vein decreased from 10 mm to 5 mm.\nThe patient’s symptom of dysphagia resolved spontaneously, and an evaluation was made with a further PET scan on May 19, 2017 (Figure ). This demonstrated mild residual FDG activity within the distal esophagus, more likely inflammatory change rather than malignancy. The FDG activity within all the lymph nodes, both treated and untreated, had unexpectedly resolved.
An 83-year-old female with a past medical history of rheumatoid arthritis (on DMARD's), asthma, depression, gastroesophageal reflux disease (GERD), and lumbar spondylosis, as well as a past surgical history of right posterior total hip arthroplasty (1999), bilateral total knee arthroplasties (2003, 2012), and right shoulder hemiarthroplasty (2010), presented with five days of right hip pain and inability to ambulate after bending down. In the emergency department, initial radiographs revealed a right posterior hip dislocation, as well as chronic appearing fractures of the right greater trochanter and left inferior public rami (). Her right lower extremity was shortened, internally rotated, and adducted. A propofol-induced conscious sedation was performed by the emergency physician and closed reduction was attempted by an experienced orthopaedic resident. The reduction maneuver involved hip flexion, traction, adduction, and internal rotation followed by external rotation and abduction. After three attempts, post reduction radiographs were significant for a right inferior obturator hip dislocation (). The patient tolerated the procedure and was neurovascularly intact distal to her hip. Computed tomography (CT) was performed, which confirmed a persistently dislocated femoral head with intrapelvic migration through the right obturator foramen (Figures and ). Having failed three attempts at closed reduction, the patient was taken to the operating room for open reduction and revision arthroplasty.\nUsing a posterolateral approach, the femoral head was found to be locked inferior and posterior to the acetabulum. Manual traction was utilized to successfully extricate the femoral component from within the obturator ring. Both the femoral and acetabular components were stable; however, a large amount of posterior wear was noted on the liner, which was exchanged for a constrained component. A greater trochanteric hook plate with cerclage cables was then utilized for the fixation of the greater trochanteric fragment (). Excellent stability with a full range of motion was noted.\nPostoperatively, the patient was weight bearing as tolerated, with standard posterior hip precautions including an abduction pillow. Aspirin 325 mg BID was used for deep vein thrombosis (DVT) prophylaxis. Although the patient initially did very well, she developed urosepsis six months after the index procedure, leading to an acute right periprosthetic septic hip with Proteus mirabilis. Radiographs showed greater trochanteric escape from the hook plate (). She then underwent irrigation and debridement with greater trochanter excision and hook plate removal (). The patient was discharged with 6 weeks of ceftriaxone antibiotics via a peripherally inserted central catheter and has since been doing well with no further dislocations.
A 45 year old female with the diagnosis of 10 years' progression of definite () bilateral type I Menière's Disease, according to the Lopez-Escamez classification (), with recurrent vertigo and hearing loss attacks symptomatically controlled with sulpiride (50 mg) on demand, was referred to the cochlear implants unit of a tertiary hospital center as a possible candidate for cochlear implantation, because of profound hearing loss (1997 BIAP) in the left ear and fluctuating moderate-severe (1997 BIAP) hearing loss in the right ear. The most recent vertigo episode occurred 10 days prior to her visit to the cochlear implant unit. No visual impairment or other alterations were found on otoneurotologic examination; normal ocular movements, normal smooth pursuit, and saccadic movement, absence of ocular misalignment on skew deviation test and no spontaneous nystagmus were also found, despite the recency of the last vertigo attack. Other neurological examinations and standard cerebral MRI were also normal for this patient.\nIn addition to the same tests as for case I, because of the bilateral MD diagnosis, the patient had received 3 months prior a 3 Tesla MRI examination of the inner ear using the HYDROPS MRI sequence (): this sequence is based on the digital subtraction of images produced by the different time diffusion of gadolinium along the inner ear fluids. This MRI showed bilateral cochlear and vestibular endolymphatic hydrops with left side hydrops predominating, as can be observed in Figure .\nThe instrumental vestibular testing was performed by the same senior neurotologist using a vHIT ICS Impulse™ USB hardware version with software version 4.1: this device was a different unit from the device used in case I. Figure shows enhanced vHIT eye velocity responses for both sides, with an aVOR gain value of 1.14 on right horizontal canal function test and 1.05 for left side. In this case, the vHIT calibration was repeated four times with similar enhanced gain values obtained, and a fifth calibration was done with the default system calibration parameters, also yielding similar aVOR gain values. The VVOR test was also performed on this patient using the vHIT ICS Impulse™ device, finding an enhanced eye velocity response during the VVOR test with a measured VVOR gain () value of 1.39 for left side and 1.35 for right side.\nFor both cases presented in this paper, the patients gave written consent to publish the results obtained from their clinical examinations and instrumental tests.
A 5-year-old male child presented with left scrotal swelling and abdominal distension. His mother had noted a painless left scrotal swelling since birth and it was gradually increasing in size. She also noticed increasing abdominal girth for approximately 2 years. There was no history of trauma. Growth and development were otherwise normal, as were his appetite and feeding habits. Bowel and micturition habits were also normal. The family history was unremarkable. The patient was not on any medication and had no known allergy. On examination, his abdomen was hugely distended. A soft, cystic lump was palpable involving left and central part of the abdomen. Shifting dullness was absent. The scrotum was hugely enlarged and right testis could be felt separately from the swelling but not the left testis. It was not possible to get above the swelling on the left side. The swelling was oblong in shape, cystic, irreducible, and nontranslucent. Cross fluctuation with abdominal lump was absent.\nRoutine blood tests were within normal limits. An ultrasound examination was performed, and it showed septated ascites and left hydrocele. Contrast Enhanched Computed Tomography showed huge septated cystic lesion [] filling whole of the left and central part of the abdominal cavity pushing away the bowel anteriorly and posteriorly. The scrotal and abdominal swellings communicated in the left inguinal region. Both the testes were visible in the scrotum. With these clinical and imaging findings, a provisional diagnosis of abdominoscrotal lymphatic cyst was made.\nThe patient underwent laparotomy through an oblique left lower abdominal incision. Initially, the retroperitoneal space was explored. As we could not see the swelling, the peritoneum was opened to find a huge, soft cystic lesion [] arising from the greater omentum was identified. The cyst was opened and drained thin hemorrhagic fluid. It was multilocular and the lower pole of the cyst was seen going inside the left internal inguinal ring. With slight traction, the part of the cystic swelling lying in the scrotum could be delivered. Approximately, 900 mL of fluid was drained. The cyst was excised completely along with adjacent omentum. The internal inguinal ring was closed with a purse string suture. A drain was placed in the peritoneal cavity and the abdomen was closed in layers. Postoperative period was uneventful. The abdominal drain was removed on the 2nd postoperative day and he was discharged on the 3rd postoperative day. Histopathological examination of the cyst demonstrated features of chylolymphatic cyst [].
A 54-year-old man presented with severe claudication and coldness in both lower extremities several years ago. The ankle-brachial index of both lower extremities was not measurable. The lower extremity CT angiogram showed total occlusion from the infra-renal distal abdominal aorta to both external iliac arteries. There was a2013-05-06 well-developed inferior mesenteric artery (IMA) forming multiple collaterals at the distal abdominal aorta, but a definite angiographic stump of the distal aorta to the iliac arteries was not visible (). A CT angiogram was not enough to detect the stump of an occlusive lesion in the distal aorta and relationships to surrounding vessels. So we considered 3D full rotational angiography and spatio-temporal reconstruction for detecting the stump at the distal aorta and additional vascular structures.\nFirst, an aortography was performed via radial approach with a 5 Fr pigtail catheter. The aortography showed that there was total occlusion at the distal abdominal aorta below both renal arteries and total occlusion at the proximal bi-iliac arteries. Although there was a severe discrete concentric stenosis in the ostium (OS) of IMA, the entire vessel was well-developed which gave rise to profuse collaterals to the bi-iliac arteries from the distal to the occluded segment.\nVascular access was achieved via both femoral arteries and right brachial artery. 8 Fr short sheaths were inserted to both femoral arteries for a retrograde approach and an 8 Fr Vistabrite IG guiding sheath (Johnson & Johnson, Miami, FL, USA) was inserted to the right brachial artery for anterograde approach.\nA full rotational 3D angiography was performed and spatiotemporal reconstruction was carried out by Philips cine angiography FD 20 software (Philips, Amsterdam, the Netherlands). Mapping with 3D reconstructed images, an endoview showed a blunt stump in the distal aorta just distal to the IMA OS which was not shown on the CT or invasive diagnostic aortography ().\nThere was a suspicious micro-channel from the distal abdominal aorta to the right iliac artery from 3D rotational angiography (, transparent arrow). This micro-channel was not observed by previous CT angiography. A virtual line could be drawn from the suspicious stump in the distal abdominal aorta to the right iliac artery (, dot line). A successful guidewire passage was achieved through this micro-channel.\nUnder the 3D image guide, aggressive bilateral kissing wiring was performed using 035 soft long Terumo wire (0.035 Radiofocus® Guidewire M, Terumo Corp., Somerset, NJ, USA) under the 5 Fr multipurpose-1 catheter support from the distal aorta to the right iliac artery and from the right femoral artery to the right iliac artery by subintimal approach (). After successful 035 guidewire passage from the aorta to both iliac arteries, sequential predilation was done using Powerflex 6.0×80 mm (Johnson & Johnson, Miami, FL, USA) from the distal aorta to both external iliac arteries (). After the predilation of both entire iliac arteries to the distal aorta, simultaneous kissing stenting was performed using two Smart control stents {7.0×100 mm (aorta to right iliac artery)/6.0×100 mm (aorta to left iliac artery) ()}. Residual stenosis was approximately 40% and good distal flow was observed. To get optimal angiographic results, final kissing ballooning was carried out using two Powerflex 6.0×80 mm balloons. A final angiogram showed excellent angiographic outcomes (good distal run-off without flow limitation) (). The patient was stabilized and safely discharged for regular clinical follow up.
A 21-year-old man went to the emergency department of São Francisco Xavier Hospital (Lisbon) saying that he had appendicitis and needed an operation. He also said that his appendix and his liver were interfering with his voice. According to his mother, for the last three years the patient had displayed periods of behavioural changes, with aggressive behaviour and unwarranted laughter. Recently, he had been fired from several jobs for being late. The patient justified his behaviour by saying that he couldn't sleep at night, and described what seemed to be complex auditory hallucinations in the second and third persons with a depreciatory content.\nIn the previous two months the clinical picture had deteriorated, with disorganised thoughts and "periods in which he wasn't there", during which he did not answer any questions or initiate any conversation. According to the patient himself, at such times, he was perplexed because the words people said appeared to make no sense.\nDuring the mental state examination, the patient was alert and oriented in space and time. He displayed delusions with a hypochondriac theme that focused on concerns about the state of his liver and his appendix, and auditory/verbal hallucinations with a depreciatory content. The patient was euthymic, and his feelings were appropriate, with no blunting or flattening. He did not display any insight into his condition. The neurological exam did not reveal any changes and the Mini-Mental State Examination [] was normal (29/30).\nHis prior medical history included a head trauma at the age of 16 that had been caused by a motorcycle accident and had apparently not been serious. No cranial computer tomography (CT) had been done at that time. The patient admitted to a regular consumption of cannabis since the age of 13, together with alcohol abuse that had recently worsened. He also had a sporadic consumption of cocaine and methylenedioxymethamphetamine (MDMA).\nHis family history included a suicide attempt by his half-brother a few months before, which had not been associated with any psychotic condition.\nThe blood tests were normal except for the toxicological traces, which revealed the presence of cannabinoids in the urine sample (52 ng/ml).\nThe patient was compulsorily admitted to the hospital under the terms of the Portuguese Mental Health Law.\nDespite the fact that the cannabinoid levels became normal during the first few days of his stay in the hospital, the patient's psychotic symptoms persisted.\nA cranial CT revealed the presence of an arachnoid cyst at the level of the left Sylvian fissure, with a marked mass effect on the left temporal and frontal lobes and the left lateral ventricle. There was also an extensive pneumatisation of the left frontal sinus. A cranial nuclear magnetic resonance (NMR) was performed in order to get a more detailed image. It confirmed the nature of the lesion and revealed the existence of a left temporal lobe hypoplasia that was associated with the arachnoid cyst (Figures , , ).\nIt also showed that the left frontal sinus, which was more developed, was in contact with the arachnoid cyst. An EEG revealed unspecific changes in the median temporo-parietal zones, which were more widespread on the left side. A neuropsychological examination showed various alterations, with impairment of verbal memory, attention, ability to plan and increased impulsiveness with a tendency towards anti-social behaviour.\nThe patient started antipsychotic therapy with risperidone 2 mg tid. Due to the fact that no links between arachnoid cysts and psychotic symptoms have been clearly established and no focal or intracranial hypertension signs were observed, the neurosurgical department concluded that even tough there was a mass effect, the risk of operating was higher than the potential benefits.\nThe psychotic symptoms improved progressively during the stay, with amelioration of the psychomotor agitation and remission of the auditory/verbal hallucinations. Although it was clear that there was a significant improvement in relation to the delusional hypochondriac ideas, a complete remission of these symptoms was not achieved.\nThe patient was discharged after a 4-week inpatient stay and received follow-up outpatient care with psychiatric and neurosurgical appointments. Three months after discharge, the patient was working part-time and attending a technical course on computer hardware. He showed the same psychotic symptoms and remained without any insight into his condition.
The patient was a 40-year-old Moroccan man who had been vaccinated with bacillus Calmette–Guérin (BCG). He did not have any signs of previous TB infection or a history of sexually transmitted infections. The patient visited our hospital for a urology consultation and presented with dysuria, purulent discharge and a meatic penoscrotal fistula that had developed over a one-year period. The physical examination at admission found nodular lesions, urethral induration on the urethral path extending laterally to the cavernous bodies with multiple fistulas, and penoscrotal pus excretion (Figure ). The external genitalia examination, testis, epididymis and ductus deferens, were without remarkable signs. His prostate volume was normal with a soft consistency. His prostate volume was normal with a soft consistency.\nThe initial biological assessment revealed an inflammatory syndrome corresponding to a sedimentation speed of 80 associated with a urinary tract infection with Escherichia coli susceptible to fluoroquinolones. First, the patient received antibiotherapy based on ciprofloxacin and benefited from urinary drainage by suprapubic catheter. A retrograde and voiding urethrocystography (UCG) was then performed and revealed an extended narrowing of the whole anterior urethra associated with multiple fistulous portions toward the scrotum and perineum. The bladder control showed a bilateral secondary vesico-ureteric reflux (Figure ). At this stage, we reached a diagnosis of nonspecific sclero-inflammatory urethral stricture with complicating fistulas. After six weeks of urinary drainage and antibiotherapy, we decided to perform a urethroplasty enlargement to clear the narrowing urethral sinus tracts (Figure ).\nThe evolution was marked by delayed wound healing associated with the persistence of fistulas extending into the corpus cavernosum with purulent discharge. It was at this point of the treatment that we suspected TB and we carried out a biological assessment in this regard. His test result for Koch bacillus (BK) in the urine was negative. His tuberculin assessment result was positive. Multiple biopsies were then performed on the periurethral tissue and fistula tracts.\nThe histological examination confirmed urethral TB and showed the presence of giant cell epithelial lesions with caseous necrosis characteristic of TB. The treatment for TB was immediately established and marked by a localized, rapid and significant improvement. A second-stage urethroplasty was scheduled for two months after the start of his anti-TB treatment.
A 57-year-old man had suffered from low back and bilateral lower extremity pain for 3 years, and had been diagnosed with lumbar disc herniation by a general practitioner. He had a history of hyperlipemia and diabetes. However his symptoms were getting worse, and he developed additional symptoms: right upper extremity pain, and gait disturbance. He went to a nearby orthopedic clinic, and was soon referred to us for additional examination and treatment. His neurological examination revealed numbness in the right forearm and both thighs. Weakness of the interosseous muscle on the right side was detected, with grade of 4/5 recorded by manual muscle testing. His gait was very unstable, and he needed to be assisted by a walker. His right patellar tendon and Achilles tendon reflexes were hyperactive and the Hoffmann and Babinski reflex were positive. Laboratory values including full blood count, electrolytes, and inflammatory markers were all within normal limits.\nThe plain radiological findings of his cervical spine indicated an osseous protrusion in the right C6/7 foramen (Fig. ). Computed tomography (CT) scans showed an osseous tumor originating in the neighborhood of the right C6/7 facet joint and occupying a part of the spinal canal (Fig. ). Magnetic resonance images (MRI) revealed the compression of spinal cord at the C6/7 and spinal canal stenosis at the L3/4 level. The tumor was hypointense or isointense on T1-weighted and T2-weighted images.\nWe diagnosed him with cervical myelopathy caused by the compression of the spinal cord due to an intra-canal tumor, and performed a C6 laminectomy and a C7 partial laminectomy with posterior approach. The tumor did not show the adhesion to the dural membrane, and en bloc resection of the tumor was performed. The resected tumor was about 2 cm in diameter, and its surface was covered with cartilaginous tissue (Fig. ). The pathologic examination indicated mature trabecular bone with a cartilaginous cap, and we had a diagnosis of a benign osteochondroma (Fig. ). On the next day after the surgery, the pain on the right upper extremity was improved, but the symptoms on the lower extremities remained. Therefore, we performed a decompression of the spinal canal at L3/4 level 1 month later. And then the symptoms improved clearly. He was discharged from our hospital at 6 weeks after his first operation. Post-operative radiograph of the cervical spine did not show each segmental instability. Also, post-operative CT scan showed the removal of the lesion, and demonstrated that over 2/3 part of the facet was preserved (Fig. ). Recurrence was not observed at the time of examination 1 year after the first surgery.
The patient is a 49-year-old Caucasian male that initially presented with a three-to-four month history of unilateral tonsillar swelling. Computer tomography of the neck revealed bilateral tonsillar enlargement, right greater than left, with a right internal jugular chain lymph node measuring 1.5 cm by 1.5 cm. The patient underwent a bilateral tonsillectomy without complications. Pathology of the left tonsil was benign, but the right tonsil contained a 2.4 cm, poorly differentiated, squamous cell carcinoma. The remainder of his staging work-up was negative, and he was staged with T2N1M0, Stage III invasive squamous cell carcinoma of the right tonsil.\nCombined modality therapy with concurrent cisplatin chemotherapy and daily radiation therapy was initiated. The tumor bed and ipsilateral neck were treated with 200 centi-Gray (cGy) fractions to 7,000 cGy, while the contralateral neck received 170 cGy fractions to 5,600 cGy. The cisplatin therapy was administered at a dose of 100 mg per meter squared on days one, twenty-two and forty-three of his six weeks of radiation. The patient was able to complete his treatment as prescribed without the need for a treatment break. He developed grade II erythema within the radiation field on bilateral necks, dysgeusia, xerostomia, and mucositis symptoms but otherwise did well.\nFollowing completion of his treatment, the patient was able to return to work and displayed regular improvement during his scheduled follow-up visits. The erythema that developed during his treatment had completely resolved and the patient regularly reported feeling well. Approximately nine months after his last radiation or chemotherapy treatment, the patient reported to the clinic for routine followup. He noted a twenty-four hour history of a tender, hot, confluent rash on his right neck that began at his clavicle and extended superiorly to the mandibular angle (Figures and ). He reported an oral temperature of 102 F; his temperature in the clinic was 101.2 F. He also experienced some myalgias and arthralgias but denied constitutional symptoms of nausea, vomiting, weight loss, drenching night sweats, and fatigue. He denied any respiratory symptoms, cough, hemoptysis, or bone pain. The patient had received no interval chemotherapy and had not started any new medications since his last visit. Radiation recall dermatitis was suspected in this patient given the distribution of the rash and presenting symptoms. Due to the warmth of the tissue and the fever, there was some concern for cellulitis as well, which prompted treatment with doxycycline. The patient was seen several months after this clinical encounter for routine followup and reported that his rash resolved in the five to six days following the initial presentation. He continued to report experiencing xerostomia and dysgeusia; he denied any recurrence of his rash.
We report a case of a 42-year-old female who presented with complaints of palpitations accompanied by nausea and vomiting four years back. Her past medical history revealed that she had high blood pressure since four years. At that time she was investigated with radiological studies, serum markers and eventually diagnosed as a case of TA in accordance with the 1990 American College of Rheumatology criteria for TA []. An ophthalmological examination was non-contributory. The cardiovascular assessment showed normal carotid upstrokes with bilateral carotid bruits and soft right and left subclavian bruits with weak peripheral pulses. A computed tomography (CT) aortogram of the chest showed severe stenosis of bilateral subclavian arteries and mild stenosis of the right and left common carotid arteries at the origin (Figure ).\nA CT aortogram of the abdomen showed an occluded left renal artery, a very small left kidney, and mild narrowing of the abdominal aorta below the level of renal arteries (Figure ).\nShe was started on a combination regimen of glucocorticoids with azathioprine. Initially, her symptoms improved for six months, but later there was a clinical decline in her condition. She was switched to cyclophosphamide. During this period, her serum erythrocyte sedimentation rate (ESR) and C- reactive protein (CRP) were regularly followed, but as it can be seen in the graphical presentation, except the initial down bulging in 2012 on glucocorticoid and azathioprine regime, it didn’t show remissive response to any regime after 2012 (Figures -).\nAfter six months of follow-up, she was put on etanercept (TNF inhibitor). During her follow-up, her routine radiological imaging and other workup for systemic review was done, but in spite of aggressive biologic therapy, she continued to have active disease. Later in her disease process, she was also put on tocilizumab (humanized monoclonal antibody against the interleukin-6 receptor) and rituximab (chimeric monoclonal antibody against the protein CD20). All these regimens were nearly tried for six months or more but during her regular follow-up she didn’t respond to any of the regimens. However, the patient does not have any serious life-threatening associations with the disease process except chronic kidney disease (serum creatinine levels 3.38 mg/dL). During her treatment process, she was once hospitalized for acute gastroenteritis. Informed consent was obtained from the patient for this study.
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.
A 12-year-old female patient reported with a chief complaint of swelling and pain on the right lower 3rd region of the face []. The patient was moderately built. No significant medical and family history. On extraoral examination, a diffuse swelling was present on right lower 3rd of the face, measuring approximately 3 * 4 cm, extending anteroposteriorly from right commissure to right angle of mandible and superioinferiorly from 1 cm below the right alatragal line to right inferior border of mandible which on palpation was firm in consistency and tender with slight rise in temperature. Intraoral soft tissue inspection revealed a diffuse swelling measuring approximately 2 * 3 cm on right buccal mucosa obliterating the buccal vestibule extending from the distal aspect of 45 to the mesial aspect of 48. On palpation, there was buccal and lingual cortex expansion; the swelling was firm to hard in consistency and tender without pus or blood discharge when palpated apico-coronally. Intraoral hard tissue examination revealed root stumps in relation to 46. The provisional diagnosis of the radicular cyst was made and differential diagnosis as dentigerous cyst and ameloblastoma. OPG [] revealed a welldefined large unilocular radiolucency, measuring approximately 4 × 5 cm in size on the right body region of the mandible surrounded by a well defined sclerotic border, sparing 2 mm of the inferior border of the mandible. The lesion was extending mediolaterally from the distal aspect of 45 up to the mesial aspect of 48 and there was root resorption of 46 and 47. CBCT [] revealed the measurement of the lesion in all three dimensions, that is, anteroposteriorly (AP), mediolaterally (ML) and superioinferiorly (SI) as about 32.1 mm, 18.2 mm and 19.1 mm respectively. It revealed bicortical expansion with a displacement of inferior alveolar nerve. Radiographic diagnosis of the radicular cyst and differential diagnosis as unicystic ameloblastoma was given. Complete cyst enucleation was done under general anesthesia [] with aseptic precaution preserving the inferior alveolar nerve and keeping intact the right lower border of the mandible along with the extraction of 46, 47 and 48 followed by thorough curettage with betadine solution and wound closure done with 3-0 vicryl suture []. Postoperative medications were given for 7 days and Betadine gargles twice a day. The excised specimen measured appro × 5 cm [] was sent for histopathological examination, which revealed a cystic cavity lined by a non-keratinized epithelial lining of varying thickness and arcading pattern of proliferation. The connective tissue was infiltrated by the chronic inflammatory cell []. The lesion was 7 days healing without any discomfort []. Follow up after 1 month revealed complete healing of lesion.
A 59-year-old woman who visited with imbalance and ataxia symptoms. At first, neurologists were suspicion of ischemic stroke, but no sign of stroke was observed in MRI. Her laboratory tests were normal except mild leukocytosis (11200 cells/ml) and high BS (142 mg/dl). However, her family members did not agree with CSF examination. Gradually, her walking and memory were impaired; later, she started to have muscle and organ spasms, and reduced communication with her immediate surrounding. Her verbal communication decreased, and she became silent and uncommunicative. She was bedridden, and her swallowing disorder continued leading to aspiration attacks accompanied by trembling, spasms, and organ stiffness once. She was hospitalized on suspicion of central nervous system causes such as acute disseminated encephalomyelitis (ADEM). Computed tomography (CT) scan revealed no any abnormality. In brain MRI, the mild bilateral symmetrical restriction was observed at basal ganglia and insular cortex. (). After two weeks, second MRI was conducted that showed bilateral and symmetrical increased signal was in basal ganglia and cortex of frontal, temporal, parietal, as the well as insula lobes in Fluid-Attenuated Inversion Recovery (FLAIR) images and T2-weighted sequences. Increased restrictions are observed in these areas in DWI and ADC map sequences compared to the previous MRI (). MRI was done three weeks later showed bilateral and symmetric increased signal in the basal ganglia and cortex of the cerebral hemispheres, which are observed at FLAIR sequences (), and increased restriction was seen in the DWI and ADC map sequences (). The persistent restriction was the main imaging sign of CJD that was observed in this patient.It should be noted that this patient had various MRI results ranging from mild abnormality to severe one. EEG showed periodic changes in the form of periodic slow waves, which was one of her disease criteria. Finally, she died from aspiration pneumonia and sepsis one year after diagnosis.
The patient is a 50-year-old African American female with a history of bilateral breast reduction twelve years ago, iron deficiency anemia, and obesity, who presented to the surgeon's office complaining of tenderness of her right breast. The patient reported that recently she had been developing keloids along the scar of the right breast with some areas having a blue hue; her left breast was unremarkable. She noticed that after wearing a sports bra there was increased pressure and abrasions to the keloid, leading to cellulitis and edema. She was previously treated with two courses of antibiotics for what was presumed to be an infected keloidal scar of her right breast but with minimal improvement. On exam, she had a large 10 cm diameter keloidal region on the inferior and lateral aspect of the right breast with edema and cellulitis. The keloidal area had no palpable fluctuance; she exhibited no nipple discharge or palpable adenopathy of the right axilla ().\nThe patient had a benign-appearing mammogram 8 months prior, and all of her screening mammograms since her breast reduction have been without signs of malignancy. Another mammogram was ordered but was not performed due to patient discomfort. An ultrasound of the breast was preformed and suggested marked edema and skin thickening suggestive of infection but no definitive fluid collection or underlying suspicious mass was observed.\nThe patient underwent a right breast partial mastectomy for cosmesis and resection of the infected keloidal area. Intraoperatively, the mass was highly vascular, firm, but not fixed to the chest wall. Postoperatively, the pathology revealed a high-grade primary angiosarcoma of the breast with negative margins.\nPatient underwent a computed tomography of the chest, abdomen, and pelvis, which did not show any evidence of gross metastatic disease. The patient then underwent completion mastectomy and scheduled for adjuvant chemotherapy with combination gemcitabine and Taxotere, followed by radiation.
A 59-year-old European man crashed his car into a concrete dam (Fig. ). Bystanders attending to the accident found him in cardiac arrest and started cardiopulmonary resuscitation (CPR) immediately. Sufficient CPR efforts were continued until the emergency services had arrived. The first recorded heart rhythm was ventricular fibrillation (VF). On inspection, no signs of injury were immediately visible and no skid marks were found. CPR was continued by physician-staffed emergency medical services (EMS) according to the current advanced life support (ALS) guidelines []. Return of spontaneous circulation (ROSC) was achieved after 30 minutes. He remained unconscious without any sign of muscular activity. He was intubated, mechanically ventilated, and treated with catecholamines during and post CPR.\nAlthough the car was severely damaged, the prehospital physician deemed a traumatic cause for out-of-hospital cardiac arrest (OHCA) unlikely. Based on findings indicative of myocardial ischemia in a post-ROSC electrocardiogram (ECG), acute coronary syndrome was suspected as the etiology of cardiac arrest. After telephone consultation with the trauma leader of the regional trauma center, the patient was transported to the trauma center with percutaneous coronary intervention (PCI)-capability primarily within 120 minutes of the accident.\nOn arrival at the trauma center, the patient appeared clinically stable. His heart rate was 65 per minute, systolic blood pressure was 150 mmHg, oxygen saturation measured by pulse oximetry was 94%, and body temperature was 34.2 °C. Signs of myocardial ischemia were found in the ECG (Fig. ). His pupils were found to be equal, round, and reactive to light.\nAfter primary evaluation in the emergency room a whole-body CT scan revealed findings listed in Table . An MRI scan (Fig. ) of his head and neck was obtained immediately due to the severity of the CT findings. Additional findings in the MRI scan are summarized in Table .\nThe medical and social history of our patient were provided by his family. Subjective overall health assessment found the married man, who was a father and grandfather, to be in good health. He had suffered a fall leading to a fractured scapula 8 years before this accident, which was treated non-operatively. Two years ago, he was assessed for suspected coronary heart disease by a specialist in cardiology, who could not substantiate this suspicion.\nHe was transferred to the intensive care unit (ICU) for further treatment. Halo fixation was installed because only ligamentous structures were disrupted in this case. This procedure is common and adequate in AOD when no cervical spine fractures are present [].\nDue to several episodes of severe bradycardia, transient transvenous pacing was conducted. Cardiac diagnostics showed an ischemic cardiomyopathy with recurrent episodes of ventricular tachycardia. Assessment via echocardiography was performed in the trauma room, 3 weeks and 2 months after the accident and revealed akinesia of the left anterior descending coronary artery (LAD) region and hypokinesia of the inferior wall after a suspected myocardial infarction and VF. Early coronary angiography could not be performed due to severe brain injuries.\nAlthough he was initially assessed to have a poor neurological prognosis from the perspective of the neurologists and neurosurgeons because of his severe brain injuries, he could be discharged from the ICU after 23 days; he was responding to verbal contact and was able to move all his extremities.\nAfter 23 days of treatment at the trauma center he was transferred to a hospital close to his home. Further in-patient treatment was continued by local protocol for further 33 days (timeline in Table ).\nHe was discharged to a neurological rehabilitation facility, where care and rehabilitation efforts were continued with great success. Three months after the incident the tracheostomy was surgically closed.\nCoronary angiography was performed 4 months after the primary event and revealed no coronary artery disease. Subsequently, he had to wear a life vest due to arrhythmia. He was defibrillated once by the LifeVest® 3 months after the trauma during his stay at the neurological rehabilitation facility. Finally, 6 months after wearing the life vest an implantable cardioverter-defibrillator (ICD) was installed.\nSix months after the trauma, he was fully conscious, spontaneously breathing, independent of help in everyday life, and mobile with walking crutches. However, he was unable to swallow granular feed due incomplete bilateral paresis of the hypoglossal nerve. His neurologic status is continuously improving; treating neurologists attested a high potential of restitution.
An 82-year-old woman was referred to our department for incidental finding of pneumoperitoneum on a chest radiograph during a medical checkup at another hospital. Initial history taking revealed a prior surgical history of only a laparotomy secondary to acute appendicitis approximately 60 years before. During physical examination, the patient was afebrile with a pulse rate of 82 beats per minute and blood pressure of 136/86 mmHg. Abdominal examination revealed a slightly distended but soft, tympanic, and nontender abdomen, without signs of peritoneal irritation. There were no palpable masses. Urgent laboratory investigations showed a slightly low hemoglobin level with normal white blood cell count and C-reactive protein level (). A plain abdominal radiograph showed free air under the diaphragm bilaterally and small bowel dilatation (). Abdominal CT scan revealed massive free air and dilated small intestine with gas inside the bowel wall; however, there was no radiological evidence of a localized point of bowel obstruction and no apparent life-threatening acute causes of PCI (). Although autoimmune serological testing showed negative results for antinuclear, anti-Scl-70, and anticentromere antibodies, further detailed history taking revealed that the patient had suffered from Raynaud's phenomenon and was previously diagnosed with SSc approximately 30 years before. She recalled that she was treated and followed up for several years, albeit without detailed information regarding her treatment. A rheumatologist later confirmed that she indeed had limited cutaneous SSc, in view of the presence of sclerodactyly of the fingers, telangiectasia on her face, chest, and hands, and Raynaud's phenomenon, all of which met the criteria for SSc []. Her pneumoperitoneum was considered to be due to PCI. As the patient was stable with no life-threatening signs and symptoms, she was treated conservatively with bowel rest and maintenance of fluid levels and subsequently discharged home without surgical intervention. The patient remained well eighteen months after discharge, without any abdominal symptoms.
The patient is a 57-year-old nulligravid female who presented to clinic with symptoms of urinary urgency incontinence. She had a past medical history of endometriosis, autoimmune hepatitis, cirrhosis, and denied any pertinent mental/psychological history or trauma. Her initial symptoms included urgency, voiding up to 5 times an hour, and nocturia up to five times per night. She had no previous vaginal surgeries. Baseline sexual function evaluation was completed at intake with the Female Sexual Function Index (FSFI) questionnaire where the arousal domain equaled 0.9 (range 0-6, with 6 indicating maximal arousal) (). Pelvic examination was significant for vaginal atrophy but no notable clitoral or labial abnormalities were visualized. There were no signs of prolapse or pelvic floor musculature hypertonicity. She was asked to keep a voiding diary and then advised to attempt timed voiding upon its completion. She was also started on vaginal estrogen and a generic anticholinergic agent (oxybutynin 10 XL daily). After 4 weeks of treatment, she experienced significant worsening of anticholinergic side-effects and stopped the medication on her own. She was then started on a beta sympathomimetic (mirabegron 25mg daily). After 6 weeks of this therapy, she did not have any subjective improvement in her symptoms. The dose was increased to 50 mg daily and continued for an additional 4 weeks. Again, she did not have an adequate reduction in symptoms and was counseled on third tier treatment options. Ultimately the decision was made to proceed with SNM.\nThe sacral neuromodulator was implanted per manufacturer instructions after undergoing a peripheral nerve evaluation with >50% improvement in her urgency symptoms. At one-week follow-up, her incisional pain was minimal. She did not require oral analgesics and had reported marked improvement in urinary symptoms consistent with the test phase.\nHowever, near the six-month follow-up appointment, she expressed concerns about persistent arousal symptoms in the vaginal area overall with new onset hypersensitivity localized to the clitoris. She did not have these symptoms prior to or immediately postimplant, but she reported gradual development of arousal symptoms postoperatively over the six-month period. She had not initiated the use of any new medications or therapies during the same time period.\nPelvic examination did not demonstrate engorgement of the clitoris, change from the intake examination, or evidence of hypertonic pelvic floor muscle dysfunction based on digital assessment. To manage her arousal, the four programs that were programmed into the system were alternated with cycling activated. Behavioral modifications were suggested including loose clothing. Upon no change in symptoms, device deactivation was performed. This resulted in no notable improvement of the manifest arousal symptoms. However, her urgency symptoms immediately recurred upon the deactivation. At this point, the plan was to trial a new set of programs to see if her arousal symptoms could be eliminated using different settings. The patient agreed to trial all four new programs, each over at least a 10-day period and assess which one was associated with fewer arousal symptoms. The programming was done at sensory levels. Alternation of pulse width and frequency was performed as well. The patient was instructed to complete a 4-week diary indicating arousal and bladder activity. At follow-up, she had trialed each program and continued to experience sexual arousal symptoms. Her symptoms were present even when the device was turned off and intensified when the machine was on. The symptoms were also present without any clitoral contact by undergarments. It was becoming so bothersome that it was difficult for her to stay asleep at night and creating anxiety. Complete testing of the neuromodulator unit was reperformed with normal values noted for impedance. Reprogramming of the unit with a new set of programs was performed again with subsensory levels used at this point. Additionally, she was offered sexual counseling but it was declined.\nOne year after placement, she elected for removal of the generator and lead. The generator and lead, intact with tip, were successfully removed with no complications. At the 6-week follow-up after explant, she had recurrence of urinary urgency symptoms with persistence of hyper-arousability. One year later, she continues to be sexually active with mild discomfort due to vaginal atrophy (as noted on the pain domain in the FSFI). She reports that the clitoris remains hypersensitive with persistence of the arousal symptoms, although moderately improved from prior to SNM. At the follow-up visits, no changes in medications or new medical diagnoses were reported when compared to prior visits. Furthermore, she denied any new stressors or change in her personal life regarding the relationship with her husband.
Our patient is a 23-year-old gentleman who was referred to the rheumatology clinic for continual management of Juvenile Idiopathic Arthritis (JIA). He was diagnosed with polyarticular JIA when he was two years old. His presentation in the beginning consisted of symmetric arthritis involving fingers and wrists. As a consequence of arthritis, he underwent bilateral wrist fusion, which was done prior to him being seen at our clinic, hence we do not have the results of synovial biopsy. His initial treatment consisted of nonsteroidal anti-inflammatory (NSAIDs), later on methotrexate was added. He remained on methotrexate for about ten years; it was discontinued when the patient was diagnosed with lung problems, primarily bronchitis, the symptoms improved with increasing the dose of prednisone. Subsequently, he was started on azathioprine, remained on it for three years, but was discontinued due to liver function abnormalities. After this, he was started on hydroxychloroquine. About ten years ago, the patient was diagnosed with iritis, treated with prednisone, which was tapered off to a dose of 3 mg once a day. There was no family history of arthritis or other autoimmune conditions.\nAt the present clinic visit, he denied joint pain, swelling, redness, and morning stiffness, and had no eye complaints. The rest of the review of the system was also negative. On joint examination he had flexion contracture of fingers of both hands and bilateral wrist fusion. There was no active synovitis, and remainder of the systemic examination was unremarkable. Labs including completer blood count, blood chemistry, and markers of inflammation were within normal limits. In summary, a 23-year-old gentleman with polyarticular JIA primarily with joint and ocular manifestations, pulmonary and liver abnormalities attributed to drug exposure. His disease was considered stable, and it was recommended to continue hydroxychloroquine and prednisone, along with follow-up Rheumatology clinic visits.\nHe was also seen by gastroenterology for elevated liver enzymes, which had never normalized (Alkaline Phosphate: 716: (45–115), AST: 71 (8–48), ALT: 80 (7–55)). Patient denied nausea, vomiting, abdominal pain, itching, fever, and skin discoloration and physical examination did not show any skin findings of chronic liver disease, and the edge of the liver was palpable at the rib margin and was firm in texture. Autoimmune markers: anti-smooth muscle antibodies, anti-mitochondrial antibody, and hepatitis serologies for hepatitis B and C were negative. An MRI of liver which showed nodularity and fibrosis, and MR elastography showed modest elevation of liver stiffness: 4.5 (normal: 0–2.9). A liver biopsy, showed granulomatous hepatitis with periportal and early septal fibrosis. He was started on ursodiol, as his picture was primarily cholestatic and ursodiol has been shown to be beneficial in granulomatous bile duct disease. After treatment with ursodiol, there was some improvement in alkaline phosphatase (568, 538) and AST and ALT remained mildly elevated as before.\nSince his initial pulmonary involvement, he was followed up by his pulmonologist lung examination was unremarkable, pulmonary function test was read as FVC: 3.14 liters (63% of predicted), FEV1: 1.72 liters (41%) of predicted, uncorrected DLCO 20.61 (57% of predicted). Chest CT scan was done which showed micronodules throughout bilateral lung fields consistent with the small airway disease: bronchiolitis obliterans. Transbronchial lung biopsy showed granulomatous inflammation. These findings along with improvement of his pulmonary symptoms with steroids were attributed to existing rheumatologic diagnosis of arthritis. He also developed erythematous lesion of scalp, and skin biopsy was positive for granulomatous inflammation.\nIn the interim, he developed blurry vision and was referred to be evaluated by retina specialist. Examination by retina specialist showed multifocal choroiditis with panuveitis. Findings of multifocal choroiditis is very atypical of juvenile idiopathic arthritis, this led to a discussion between ophthalmologist and rheumatologist. His other systemic manifestations were also put together, which had previously showed granulomatous inflammation of liver, lungs, and skin. At this point, consideration was given to whether he has systemic granulomatous disease. DNA PCR and DNA sequencing for all twelve coding regions of NOD2 was done and it showed heterozygous mutation in Glu498Gly. Patient's family members are still considering genetic testing, as none of them are symptomatic, they have deferred it at present. He does not have children. Since we do not have information about mutation present in parents, hence, it is difficult to say whether the mutation is inherited or sporadic in nature.\nWith the above clinical presentation and gene mutation, patient was diagnosed with systemic granulomatous inflammation: Blau syndrome and he was started on Adalimumab. With the initiation of Adalimumab, there was marked improvement in Ophthalmologic symptoms. Alkaline phosphate continues to improve, (337, 259, 215), AST (49), and ALT (55) have also improved, though there has been fluctuations in AST and ALT with some values higher than the previous ones. MR elastography shows improvement in liver stiffness (3.5, 3.2) which is very close to the normal value of 2.9, and he has not developed any cholestatic symptoms. Repeated CT scans of chest have remained stable, with presence of micronodules. Pulmonary function tests are similar to before, consistent with obstructive pulmonary process (FVC: 3.11, 67% of predicted, FEV1: 1.81, 46% of predicted, FEV1/FVC 58, 60% of predicted, DLCO: 34.8, 54% of predicted), and there has been no reoccurrence of the scalp lesions.
One-month-old female baby presented with a circumferential diffuse swelling of entire right leg with reddish purple discoloration noticed since birth, which developed an abscess, ulceration and purulent discharge and sepsis. There was gross edema of right foot and thigh with inguinal adenitis []. Hematological investigations showed anemia and thrombocytopenia. A diagnosis of KMP was made and a biopsy was carried out after controlling the sepsis, which showed features of KHE. Immunohistochemistry was positive for Vimentin, CD31 and CD34, negative for Cytokeratin and GLUT 1 and Ki67 was 5-10% suggestive of KHE. X-ray of right lower limb did not show any bony involvement or osteomyelitis. MRI showed T2 hyperintense ill-defined circumferential mass involving the entire right leg and lower thigh. Underlying bones and joints were normal []. Doppler correlation of the lesion showed increased vascularity. Arterial feeders were from arteries around the knee joint and the venous drainage was into the tibial vein. The arteries from the external iliac to popliteal artery showed high resistance flow. Distal tibial vessels showed reduced but high resistance flow due to severe external compression. The baby was started on high dose Prednisolone regimen (40 mg orally on alternate days tapered to half the dose every 20 days till a dose of 2.5 mg on alternate days was completed).[] The KHE and KMP did not show any resolution and Propranolol 2 mg/kg daily orally in two divided doses was added.[] There was no improvement for five months since admission and the child required frequent hospitalizations for increasing size of the lesion, foul smelling discharge from the fungating ulcer over the lesion, the proximally spreading violaceous discoloration on to the thigh, increasing distal foot edema and life- and limb-threatening systemic and local sepsis. Hence, Vincristine was administered at weekly dose of 0.025 mg/kg[] for four weeks. The lesion resolved with local wound care and compression stockings gradually over the next 10 months []. The hematological changes due to KMP also got corrected. Currently, the child is on follow-up.
A 61-year-old male patient presented with headaches and numbness on the right-side of the face. Physical examination showed high blood pressure (210/90 mmHg). Neurological examination revealed nystagmus and decreased sensation on the right-side of the face. The cranial nerve and cerebellar examination were normal. Non-contrast computed tomography (CT) revealed no infarcted area but showed the aneurysms of the basilar and left vertebral artery. Absence of the petrous part of the left carotid canal was overlooked on the non-contrast CT examination. MR imaging showed two contiguous fusiform basilar artery and one left vertebral artery aneurysms, absence of normal flow-voids in the cavernous and petrous portion of the left ICA, and the petrous carotid canal was atretic. There was a small ischemic area in the left occipital lobe with abnormal diffusion restriction observed as hypointense on T1-weighted and as hyperintense on T2- weighted, FLAIR images [Figure ,]. On the non-contrast CT the ischemic area was not identified because the examination was applied in the first 24 hours after the onset of the symptoms.\nComputed tomography angiography revealed agenesis of the left ICA and left carotid canal []. The caliber of the left common carotid artery (CCA) was diminished and the left CCA terminated in the external carotid artery (ECA), with complete absence of the cervical, petrous, cavernous, and supraclinoid segments of the left ICA [Figure ,]. The right CCA was normal in caliber. Both CT and DSA showed two fusiform basilar artery and one left vertebral artery aneurysms. The left vertebral artery aneurysm was on the distal part of the V4 segment. Bilateral posterior inferior cerebellar arteries (PICA) were normal. One of the basilar artery aneurysm was encountered on the proximal part of the artery before the origin of the anterior inferior cerebellar arteries (AICA). The second aneurysm was found between the origins of the AICA's and superior cerebellar arteries. The AICA's were normal in caliber [Figure –].\nDSA examination demonstrated that the left anterior cerebral artery (ACA) was supplied through a patent anterior communicating artery (ACoA), and the left middle cerebral artery (MCA) was supplied by the basilar artery via the left posterior communicating artery (PoCoA). Collateral circulation between the left ECA and ICA was not encountered []. Transcranial Doppler sonography showed increased flow velocity in the left posterior cerebral artery [].
An 84-year-old woman was admitted to the GI ward of our hospital with dysphagia and chronic cough which had started from 1 month ago. Dysphagia was crescendo. At first, the patient had dysphagia to fluid but improved to any kind of meals and this complication forced her to induced vomiting. She noticed a surgical history of Esophagostomy and Colon interposition 40 years ago, that we do not know its indication. She had a history of myocardial infarction and she did not use any medication except Aspirin. Blood pressure was normal and heart rate was 125 and the saturation of O2 was 88%. The sclera was pale but not icteric. No lymphadenopathy was detected. Thyroid examination was normal. In the lung examination, the respiratory sounds was decreased at the both lower parts of lungs. Chest X-ray which showed in Fig. showed widening of mediastinum and consolidation in lower lobe of right lung. In abdomen, there was an old scar at midline that was for her past surgery. The bowel sound was normal. Abdominal X-ray was normal (Fig. ).\nComputed tomography (CT) of thorax showed a fistula between interposition colon and Trachea (Fig. ). Endoscopy was done and a mass was seen in the distal part of the interposed colon (Fig. ). Histopathology of a biopsy revealed an invasive well differentiated squamous cell carcinoma of colonic origin in reconstructed esophagus (Fig. ) and low grade dysplasia in gastric antral prominent mucosal fold. The SCC in the interposed colon is very rare as we searched in the literature.\nLaboratory test results showed was done. Hemoglobin was 12.4 g/dl, Hematocrit was 37%, mean corpuscular volume was 82 fl and liver function tests were all within normal limits.\nThe patient died 4 days after admission because of cardiac arrest and with asystole rhythm.\nEsophageal carcinoma, gastric carcinoma, benign esophageal lesions (polyp, esophagitis) and strictures are considered as the possible differential diagnosis. Although it is rare, but colonic polyp and carcinoma in the interposed segment may also be considered as a differential diagnosis.
A 46 years old man with end-stage hypertrophic cardiomyopathy and electrical storm underwent urgent orthotopic heart transplantation: the donor was a 54 years old woman, known for diabetes, nicotine consumption, morbid obesity, combined ventilation disorder with restriction due to obesity hypoventilation syndrome and suspected chronic obstructive pulmonary disease, and history of thrombophilia (activated protein C resistance) with recurrent deep vein thrombosis and pulmonary embolism in cerebral death due to brain hemorrhage. Pre-transplant cardiac workup showed a 90% stenosis in the middle right coronary artery (Fig. ), with normal LVEF, and no segmental wall-motion abnormalities or valvulopathy.\nThe technically uneventful transplant was completed with a venous coronary artery bypass graft (CABG) on the right coronary artery (RCA), with a total ischemic time of 191 min. After cross-clamp removal and appropriate induction therapy with methylprednisolone 500 mg IV, the heart showed severe global biventricular failure with severe functional mitral regurgitation. In the absence of preformed donor specific HLA antibodies in favor of an acute humoral rejection, primary graft failure was suspected, and mechanical hemodynamic support was immediately initiated with a central veno-arterial extracorporeal membrane oxygenation (ECMO) and intra-aortic balloon pump (IABP), and high dose of cathecolamines (Noradrenaline up to 30 mcg/min) A relatively low troponin release was observed during the first 24 h post-operative (peak at 2386 ng/l), favoring the hypothesis of myocardial stunning over necrosis.\nA cardiac tamponade on post-operative day 1 led to surgical revision. The intra-operative status was noteworthy for an occlusion of the venous CABG on the RCA. A coronary angiogram was urgently performed in attempt to treat the stenosis in the native vessel. Surprisingly, only a < 50% stenosis could be seen the mid RCA (Fig. ). It was then noticed that the angiogram performed in the donor had not been preceded by the administration of nitroglycerine, which retrospectively spoke for a severe localized vasospasm of the mid RCA at the site of an atherosclerotic plaque. No further intervention was performed and the ECMO could finally be weaned on day 9, after full recovery of the LV function and moderate persistent RV dysfunction.\nIn stable phase, during his fourth post-transplant week at the intermediate care unit, the patient developed sustained ventricular tachycardia at rest, with a heart rate of 150 bpm and no associated hemodynamic instability or even symptoms. Rapid cardioversion was easily achieved with a single administration of 2 mg magnesium sulfate IV. Nevertheless, the immediate post-cardioversion 12-lead ECG and echocardiography respectively showed severe ST-segment elevation in the inferior leads (Fig. and ) with inferior and inferolateral hypokinesia (Additional files , and ). Urgent cardiac catheterization showed a severe localized coronary vasospasm in the proximal RCA (Fig. ), which was rapidly reversed after intracoronary injection of 1 mg isosorbide dinitrate. The ECG and echocardiography quickly normalized after the acute event, without consecutive elevation of troponins. An endomyocardial biopsy excluded an acute cellular or humoral rejection. The patient was treated with diltiazem without further episodes of arrhythmias or ST-segment changes during his hospital stay.\nInterestingly, a few days before this coronary vasospasm, the patient complained from recurrent abdominal pain followed by melena. A colonoscopy showed ulcerations in the distal ileum, correlated with reversible signs of bowel ischemia in two consecutive CT scans, without evidence of occlusion or embolization in the mesenteric vessels. We could speculate that systemic factors in the recipient might favor vasospastic events. Because of a previously reported case of segmental mesenteric ischemia related to mycophenolate mofetil [], this drug was replaced by azathioprine without any further abdominal pain or bleeding episodes.
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.
A 75-year-old man was referred to our hospital for abdominal fullness and nausea since 2 months. He had a medical history of hypertension and hyperlipidemia and a surgical history of the right inguinal hernia. The patient's laboratory findings were within normal limits. Abdominal computed tomography (CT) revealed a well-demarcated oval isodensity mass of 25 mm at the tip of his appendix. Contrast-enhanced CT revealed a lesion with gradual homogeneous contrast enhancement from the arterial phase to the equilibrium phase (). No abnormal findings were found in the root to the middle of the appendix. Abdominal ultrasonography (US) revealed a well-demarcated hypoechoic tumor. The tumor size was 22 mm × 18 mm × 18 mm, with some cystic area and blood flow (). Colonoscopy findings were normal. The patient's symptoms naturally alleviated during examination period.\nPreoperative diagnosis indicated appendiceal neuroendocrine tumor (NET) G1 or gastrointestinal mesenchymal tumors, such as GIST. Malignancy could not be ruled out; therefore, laparoscopic ileocecal resection with D3 lymph node dissection was recommended. Intraoperative findings revealed a well-demarcated tumor at the tip of the appendix, with no invasion into the surrounding tissue. This observation was similar to the preoperative imaging findings. According to another intraoperative finding, dissecting the adhesion between the terminal ileum and the peritoneum, which was the effect of the past herniorrhaphy, was necessary. The operation time was 167 min, and the amount of blood loss was 100 ml.\nPathological findings revealed a well-demarcated tumor originating from the muscular layer at the tip of the appendix and spindle-shaped heterotypic cells proliferating in a bundle. Vascular invasion and lymph duct invasion were not detected. No tumor cells were found in the dissected lymph node. Immunohistochemical studies revealed negative values for KIT and CD34 and positive values for S-100 protein (), which confirmed the schwannoma of the appendix. The patient was discharged on the 9th day after surgery without any complication requiring medical treatment. The patient is presently doing well without any evidence of recurrence at 3 months after surgery.
62-year-old Caucasian female with a history of hyperlipidemia and osteopenia presented to an outpatient family medicine clinic after suffering a wild canine bite. The patient received standard rabies prophylaxis and subsequently underwent treatment with trimethoprim-sulfamethoxazole. 19 days later, the patient presented to an outside emergency room complaining of fatigue, dizziness, and multiple falls related to balance difficulty. After admission, the peripheral blood count revealed a white blood cell count of 1.2, suggesting a diagnosis of aplastic anemia. The bone marrow revealed hypoplastic bone marrow and hypoproliferation consistent with severe aplastic anemia. No metastatic carcinoma, viral inclusions, or granulomas were seen.\nCT head revealed a 3.7 cm × 6.6 mm left frontal subdural hematoma, a 7.0 × 1.7 cm left temporal epidural hematoma, and left cerebral edema without midline shift (). Upon neurosurgical consultation, the patient's examination showed the patient awake and fully oriented without any clinical neurological deficits. The patient was transferred to the intensive care unit for close monitoring. Platelets were transfused while hematology panels were sent for analysis. The patient's studies were negative for parvovirus, iron deficiency, and paroxysmal nocturnal hematuria but were notable for vitamin B12 deficiency. She was initiated on dexamethasone to induce demargination of cell lines, and was started on vitamin B12 replacement, and her antibiotic was changed from trimethoprim-sulfamethoxazole to vancomycin. Her platelets never reached the 100,000 benchmark despite transfusions, but her other cell lines improved. She was transferred out of the ICU and later discharged home in stable condition with a white count of 5.6, hemoglobin of 11.0, hematocrit of 32.7, and a platelet count of 52 K.\nFollow-up visit two days later revealed white count of 12.0, hemoglobin of 12.7, hematocrit of 37.0, and a platelet count of 117 K. She had no neurological deterioration and follow-up CT scan revealed reduction in the size of the epidural hematoma to 6.3 × 1.5 cm and the subdural hematoma to 2.5 cm × 4.4 mm (). Final blood work was negative for HIV, hepatitis A, B, or C, bacterial infection, lupus, rheumatoid arthritis, and antiplatelet antibodies. The anemia was ultimately attributed to her trimethoprim-sulfamethoxazole use.
A 63-year-old African American woman presented to the Plastic Surgery clinic from her nursing home with a 2-year history of a painful distal forearm mass. The lesion was initially thought to be a keloid from a stab wound more than 20 years ago; however, she decided to have it evaluated because of a recent increase in size and intermittent bleeding. She denied any numbness, tingling, or weakness of the hand. Her neurovascular examination had normal findings. Clinically, the mass was located on the volar aspect of the mid-forearm, was pink and ulcerated, and 3 × 3 × 4 cm in size (). There was no associated supracondylar or axillary lymphadenopathy.\nBecause of the unique characteristics of the mass, we further evaluated the lesion with a magnetic resonance image of the right forearm. This image demonstrated a 3.8 × 2.1 × 4.1-cm mass in the subcutaneous tissue without invasion into the fascia or muscle (). The lesion was concerning for malignancy, so she was referred to a surgical oncologist for excisional biopsy. One month later, the mass was excised en bloc with careful dissection of the deep margin to maintain the fascia. We used a 6 × 4-cm elliptical incision, and this defect was closed primarily using suprafascial flaps for a tension-free closure.\nOn final pathology, the entire specimen was 6 × 4 × 0.8 cm. It was found to be an invasive nodular melanoma with positive, deep, and radial margins. This case was discussed at our multidisciplinary tumor board with plans of positron emission tomographic (PET) scan, reexcision, and sentinel lymph node biopsy.\nBefore her postoperative follow-up appointment, she underwent a PET scan, which unfortunately demonstrated a hyperactive node in the right axilla as well as a concerning left breast lesion. At her follow-up appointment, we offered a wide local excision with sentinel lymph node biopsy; however, the patient refused to have further surgery. On physical examination, there was a small, red, scaly lesion in the middle of the surgical site concerning for rapid recurrence. We referred her to a breast surgeon for workup of the left breast lesion, which ultimately was found to be ER/PR+ invasive lobular breast cancer. The patient was scheduled for close follow-up in both clinics but had not made her appointments. After reviewing the medical records, the patient refused to have any further surgical procedure at this time.
The subject is a 13-year-old female patient with a history of arterial hypertension, tachycardia, and cardiomegaly. In the clinical evaluation a palpable lump was found in the hypogastrium. Full laboratory tests were requested and the following relevant data were found: adrenaline and noradrenaline elevated in blood and urine. An ultrasound was carried out revealing an ovoid, heterogeneous solid mass of 4.4 × 3 cm in contact with the vagina and below the uterus, causing an effect of mass on the bladder. In order to determine the nature, characteristics, location, and relationships of this mass, magnetic resonance imaging (MRI) was requested and this reported an image of 4 × 6 × 4 cm, located in the retrovesical and right vaginal parauterine region in contact with the posteroinferior wall of the bladder, which it deforms (). It was decided to perform a surgical biopsy of the lesion, obtaining the following anatomopathological results: paraganglioma. To assess functionality, the existence of lesions in other locations and the presence of metastases, a metamyodobenzylguanidine (MIBG) scan was requested, which was negative for neuroendocrine tumour metastasis. It was decided to perform surgical resection prior to pharmacological treatment. A laparotomy was performed, revealing a tumour in the retrovesical, parauterine, and right paravaginal region in contact with the vaginal vault and lower back part of the wall of the bladder. The lesion was dissected and resected respecting the vagina, uterus, and bladder (). Coinciding with the surgical manipulation of the lesion, the patient presented fluctuation of arterial pressure that required pharmacological management. The anatomopathological study of both the biopsy and the resection revealed a proliferation of neoplastic cells that were predominantly present in solid nests separated by fibroconnective vascular stroma. The cells showed eosinophilic cytoplasm and mild nuclear pleomorphism ( and ). Immunostaining techniques were performed for chromogranin A and synaptophysin, both markers being positive ( and ). With these findings, the diagnosis of extra-adrenal pelvic paraganglioma was reached. The patient evolved for three years with local recurrences and metastases in intrapelvic lymph nodes due to the neoplasia described. These results were obtained due to the studies of positron emission tomography that revealed three pelvic hypermetabolic nodules (right side: 14.2 mm, left side: 14, 9 mm and 10.3 mm), with a hypermetabolic focus on the floor of the pelvis relative to the vagina, to the right of the midline, adjacent to the metallic suture clip ( and ).
A 56 year old female presented to her primary care physician with a painless finger mass. She first noticed the mass about 7 years ago and it had been growing slowly since that time. Her former primary care physician had removed the mass about 5 years prior. She recalled being told at that time that it was a “ganglion cyst.” The mass had since returned and grown back to its former size. It was not frankly painful, though it did feel tight and often got in the way of daily tasks. She denied any numbness or tingling in her finger, and denied any warmth, redness or swelling in her other joints. Her past medical and family histories were non-contributory, including no history of gout, autoimmune disease, or cancer. Physical examination revealed a non-tender, soft, well circumscribed mass on the extensor aspect of the right fifth digit, just proximal to the base of her fingernail (). The mass was approximately 2cm x 2cm and did not transilluminate (). The mass was fluctuant and encapsulated. There was no swelling or effusion in the hand, fingers, or joints. Neurologic exam showed no sensory or motor deficits.\nThe radiograph showed a non-invasive soft tissue mass on the dorsal aspect of the fifth distal phalanx (). Incisional biopsy was performed. The mass was found to be composed of non-encapsulated fatty tissue, and extended to the extensor tendon sheath. The patient was referred to hand surgery for a definitive excision.\nGross examination of the specimen revealed multiple fragments of lobulated and rubbery tissue which were mostly white to grey with focal yellow areas. Histological examination of the specimen stained with hematoxylin-eosin revealed an encapsulated benign neoplasm. The mass was composed of synovial-like mononuclear cells, with a variable number of multinucleate osteoclast-like cells (), and foam cells (). Both the mononuclear cells and the giant cells showed strong reactivity for CD68 (). These findings are consistent with a diagnosis of tenosynovial giant cell tumor, localized type (giant cell tumor of tendon sheath).
A 55-year-old woman presented with recurrent invasive lobular carcinoma of the right breast following previous lumpectomy and partial irradiation for invasive lobular carcinoma. Ductal carcinoma in situ of the opposite left breast also had been treated in the past with lumpectomy. Given the patient history and the recurrence of disease in the right breast, bilateral skin- and nipple-sparing mastectomies with right axillary sentinel lymph node biopsy were planned. These would be followed by immediate bilateral autologous DIEP flap-based breast reconstructions.\nPreoperatively, a computerized tomography angiogram was obtained to evaluate the perforator vascular anatomy of the anterior abdominal wall. This study showed a 2.5–3.0-cm left adrenal mass. Subsequent work-up diagnosed a pheochromocytoma. The patient was offered a laparoscopic adrenalectomy after alpha-blockade in addition to a genetic evaluation for hereditary causes of pheochromocytoma. She was advised to undergo an adrenalectomy first before moving forward with oncologic and reconstructive breast surgery. However, given her concern for breast cancer progression, the patient preferred to proceed first with the breast cancer surgery and reconstruction to be followed with later surgery to address the adrenal tumor.\nUnder consultation with the patient’s Endocrinologist, alpha-blockade (phenoxybenzamine) was initiated 2 weeks before surgery to prevent pheochromocytoma crisis and beta blockage (propanolol) was started 1 week later for heart rate control. Subsequently, bilateral skin- and nipple-sparing mastectomies were initiated with simultaneous abdominal tissue harvest for the reconstruction. Figure a Doppler probe was used to identify the dominant perforators of the abdominal wall on each side of the midline. As the skin and soft tissue flap was developed, dissection on the right side in the suprafascial plane showed the perforating vessels to be small, measuring less than 1.0-mm in diameter. To avoid injury to these vessels, the customary transmuscular dissection of the vessels was changed to include in the flap a 4 × 3-cm portion of the right rectus abdominis muscle and 4 × 2-cm portion of rectus fascia to surround and protect the vessels. On the left side, the perforating vessels to the flap also were small, but only a small cuff of rectus abdominis muscle and fascia was included in the flap. On both sides, only the deep inferior epigastric vessels were used for blood supply.\nFollowing completion of the bilateral mastectomies, the internal mammary arteries and veins were isolated by removing a short segment of the third costal cartilage. The deep inferior epigastric vessels of the abdominal flaps were divided and microvascular anastomoses were performed between the deep inferior epigastric artery and vein and the internal mammary artery and vein on each side. With strong flow through the anastomoses, the flaps were deepithelialized (with the exception of a small skin paddle along the inframammary fold for postoperative monitoring), and inset in the mastectomy defects. The abdominal wall donor sites were then closed. Because the 4 × 3 cm segment of rectus abdominis muscle and fascia had been included on the right side to protect the vessels, the fascial closure was reinforced with a 4 × 6-cm piece of SeriScaffold® mesh (knitted, multi-filament, bioengineered silk) []. The mesh was placed as an underlay deep to the rectus fascia secured with 1 PDS mattress sutures, and the fascial edges were then approximated over the mesh with 0 Ethibond figure-of-eight sutures. On the left side, the rectus abdominis fascial closure was completed with 0 Ethibond and 1 PDS sutures. In order to prevent bulging in the upper abdomen where tissue had not been removed, the fascia was tightened by placating with an 0 PDS running suture. The remaining abdominal skin was advanced and closed in layers with exteriorization of the umbilicus through the skin in the midline (Fig. ).\nThree months later, the patient was brought to the Operating Room for a laparoscopic left adrenalectomy for the pheochromocytoma. Figure with the patient in position and with complete pharmacologic abdominal relaxation, a Veress needle was placed for insufflation. However, the abdomen proved too tight to accommodate sufficient pneumoperitoneum. Given that inadequate abdominal wall distensibility was responsible for inadequate insufflation, rather than difficulty with Veress needle placement, open trocar placement was not undertaken. A laparoscopic retroperitoneal approach was not attempted because it also relies on creating working space through insufflation-mediated abdominal wall expansion [–]. Thus, the laparoscopy was aborted without incision. The adrenalectomy was not converted to an open procedure for several considerations: the patient’s reluctance to proceed with an open intervention; because pharmacological management of pheochromocytoma via alpha blockade rendered the procedure non-emergent [, ]; and the belief that primary closure would be troublesome.\nWhile kept on pharmacological management of the pheochromocytoma, the patient was evaluated in the outpatient setting for assessment of abdominal wall compliance at regular intervals. Five months later, the patient’s abdomen was felt to be compliant and she was taken back to the Operating Room for a laparoscopic left adrenalectomy. Pneumoperitoneum was established by Veress needle placement at Palmer’s point and the abdomen accommodated 3 l of pneumoperitoneum. The left adrenalectomy proceeded routinely and the patient was discharged home the following day.
We present the case of a 13-year-old female patient, known with severe visual field loss, who referred for another opinion regarding the ophthalmological diagnosis.\nAnamnesis at presentation revealed that at the age of 9 years and 3 months, on a routine ophthalmological examination, papillary calcification and retinal hemorrhage were discovered in the left eye. At that moment, the suspicion of intracranial calcifications was raised. The patient underwent clinical neurological examination, EEG, and cerebral MRI, all of them revealing a normal aspect. The patient was also recommended fluorescein angiography, which showed papillary autofluorescence. The diagnosis established then was papillary drusen in both eyes, buried in the right eye and mixed in the left eye and the patient was recommended to keep it under observation, together with a periodical examination of the visual field.\nThe patient had had multiple examinations of the visual field over the time.\nThe first visual field examination of the right eye showed an arcuate nasal defect, in the superior nasal quadrant, sketching an aspect of nasal step, structure of the sensitivity defect that in 3 years time evolved into a quadranopsia.\nIn the left eye, the first visual field examination showed inferior nasal quadranopsia, extended superiorly with a nasal arcuate defect respecting 20° centrally, which after three years evolved into a paracentral diffuse defect with an island of central vision of 5°.\nHowever, over the time, the examination of the visual field was made with different types of machines, and no correlation of the modifications could be made objectively.\nThe patient received several different diagnoses from several different ophthalmologists, among which optic nerve drusen; papillary oedema and hamartoma have to be mentioned.\nAt presentation, the patient’s visual acuity was 20/ 20 with correction for the RE and 20/ 20 without correction for the LE, with a refraction ROD: -1 DSf<> -0.75 DCyl, 179* and ROS: +0.50 DSf<>-0.75 DCyl, 167* and a cycloplegic refraction: OD: -0.75 DSf<> -1 DCyl, 168, OS: +0.75 DSf<> -1 DCyl, 170.\nThe intraocular pressure was 19 mmHg GAT in the right eye and 13 mmHg GAT in the left eye.\nSlit lamp examination of the anterior segment revealed no pathological changes for both eyes, and the red-discrimination test was also normal.\nFundoscopy presented only with papillary pathological modifications.\nThe optic disc in the right eye was elevated, with relatively clear margins, pink color, and the absence of cupping. At 5 o’clock meridian, a nodular yellow mass, with irregular outline, could be noticed ().\nIn the left eye, the optic disc was also elevated, pale, of irregular outline, and the absence of cupping was noticed. Nodular, yellow, reflective protrusions, with irregular contour and brambleberry shape could be noticed ().\nThe retinal vessels, the macula, and the retinal periphery presented no pathological changes in either of the eyes.\nThe clinical examination suggested the diagnosis of optic nerve drusen in both eyes. B-scan ultrasonography and optical coherence tomography (OCT) examinations were used for the confirmation of the diagnosis.\nB-scan ultrasonography is considered the gold standard method for the detection of optic disc drusen. In this patient’s case, it showed round, hyperechoic structures, observed at the optic nerves of both eyes. The A-scan mode, which was overlapped on the structure only for the left eye, showed hyperreflectivity at the anterior side of the optic nerve, of supraretinal intensity.\nOptical coherence tomography is a useful examination in the assessment of the structure and the anatomical shape of the drusen, and in the analysis of retinal nerve fiber layer (RNFL) and GCL-IPL complex.\nFor patients under 18 years old, however, there is no normative database regarding the normal values of the analyzed parameters, therefore these analyses are useful only for patient’s follow-ups.\nThe OCT scan of the optic nerve showed a prominent aspect of the optic disc, with a lower value of average RNFL thickness in the left eye compared to the right eye ().\nMacula was structurally normal, with an asymmetry of macular thickness, thinner in the left eye, compared to the right eye ().\nThere was also an asymmetry of thickness regarding the GCL-IPL complex, which was thinner in the left eye compared to the right eye ().\nThe investigations confirmed the diagnosis of optic nerve drusen in both eyes.\nThe differential diagnosis in the case of this patient took into consideration the following pathologies:\n• Papilloedema – excluded by B-scan ultrasound;\n• The existence of an intracranial expansive process – excluded by clinical and imagistic examinations;\n• Optic nerve tumors\no Astrocytic hamartoma – the proliferation of astrocytic cells occurs above the optic disc, whereas optic disc drusen is located in the substance of the optic nerve.\no Optic nerve sheath meningioma – excluded by clinical and imagistic examinations.\n• Leber optic neuropathy – it typically presents with severe loss of central vision.\n• Infiltration of the optic nerve (leukemia, lymphoma) – excluded by normal laboratory tests.\nThe patient’s visual field examination at presentation revealed a superior nasal altitudinal scotoma at the right eye (), and at the left eye an important constriction of the visual field, with the preservation of a small 15* island of temporal paracentral vision ().\nThe patient was not recommended any treatment, but only periodical follow-up with visual field examination at every 4-6 months, and annual OCT.\nThe patient came back a year later for follow-up. At examination, there was no progression of the visual field alterations (,), but the intraocular pressure was at the superior level of the normal range, 21 mmHg GAT for the right eye and 20 mmHg GAT for the left eye.\nTherefore, the patient was recommended the treatment with a prostaglandin analogue to prevent the exacerbation of the visual field loss in order to attenuate the mechanical compression on the ganglion cells axons and to improve the blood flow to the optic nerve head.
A 28-year-old female presented to us in July 2015 with complaints of pain and restricted movement of the right ankle joint since 1 month. The pain aggravated on walking. There was no history of trauma and was not associated with fever, loss of weight, or any other constitutional symptoms. History and family history were non-contributory. Clinical examination revealed no inspectory findings. Palpation revealed tenderness over the anterior aspect of the distal tibia with no local rise of temperature. Range of motion of the right ankle joint was painful and restricted. X-rays revealed a well-defined expansile predominantly lytic lesion in the distal epi-metaphyseal region of the right tibia with minimal periosteal reaction seen along the medial margin suggestive of malignant tumor of the bone (). Magnetic resonance imaging (MRI) of the right ankle joint suggested an ill-defined eccentric expansile lesion measuring 4.3 (CC) × 3.0 (transverse) × 2.9 (anteroposterior) involving epi-metaphyseal end of the lower end of tibia causing cortical breach and having extra-osseous tissue component with the abnormal signal in flexor and extensor group of muscles with the possibility of GCT (). According to the radiological classification of campanacci the tumor was classified as a grade 3 lesions []. Laboratory investigations were within normal limits and surgery in the form of intralesional curettage with bone cement was planned. Anterior incision was taken over the distal end of the tibia. Bone was exposed, a cortical window of 3 cm × 2 cm was made (), the tumor was excised (), and curettage was done. The specimen was sent to histopathology for further examination. A thorough wash was given, and the defect was treated with 5% phenol. The cavity was filled with three layers consisting of bone graft, gel foam, and cement mixed with vancomycin, respectively (). The tumor was sent for histopathological examination which confirmed the diagnosis of GCT (). X-rays were taken at regular follow-up to confirm the union and the cast was removed after 3 months postoperatively, and weight-bearing was started. The patient was followed up regularly with no signs of recurrence.
A 14-year-old male with DMD has been on daily oral steroid since 9 years of age. He weighed 53 kg (47th percentile) and was 147 cm tall (less than 3rd percentile). He became nonambulatory at 12 years of age. Forced vital capacity (FVC) was 2.37 L or 80% predicted. A polysomnogram was completed and revealed moderate obstructive sleep apnea. His echocardiogram (ECHO) was normal with cardiac magnetic resonance imaging (MRI) demonstrating normal left ventricular ejection fraction (LVEF). He participated in many school activities including wheelchair soccer.\nHe presented to the emergency department with confusion, tachycardia, tachypnea, and fever up to 39.2 degrees Celsius a few hours following soccer practice. During practice, he had been transferred from his wheelchair and his right leg had brushed the ground causing him to have to be placed on the ground until more help was obtained to put him back in his wheelchair. Brain computed tomography (CT) was negative for any acute intracranial process contributing to his current state. Chest computed tomography (CT) scan was negative for pulmonary embolism but showed patchy diffuse nodular airspace opacities seen scattered throughout both lung fields (). He had progressive acute hypoxic respiratory failure requiring mechanical ventilation. Shortly after intubation, he suffered cardiac arrest requiring 3 minutes of cardiopulmonary resuscitation (CPR) before return of spontaneous circulation. ECHOs were consistent with increased pulmonary vascular resistance including moderate right ventricle dysfunction and elevated pulmonary arterial pressure. Duplex ultrasound evaluation of lower extremities was without evidence of deep venous thrombosis bilaterally. X-rays of lower extremities revealed cortical step off at the proximal left femoral neck and nondisplaced fracture at the distal right tibial metaphysis and fibular metaphysis (). Ophthalmologic exam revealed Purtscher-like retinopathy. The patient was treated with 48 hours of antibiotics that were discontinued with negative blood, urine, and mini bronchoalveolar lavage (BAL) culture. The patient was treated with mechanical ventilation and right ventricular afterload reduction with milrinone and inhaled nitric oxide for pulmonary hypertension. These therapies were able to be weaned off, and the patient was able to be extubated on day five of admission. He was later transferred to the pediatric floor and later discharged home without any residual effects from the entire event.
The patient was a 25-year-old, right-handed woman who presented two months after the sudden onset of lower extremity paresthesias and sensory loss that extended into the abdomen and lower chest and one episode of urinary urgency and subsequent incontinence. Her examination was notable for brisk lower extremity deep tendon reflexes but no other abnormalities. MRI of the cervical and thoracic spine revealed both cervical and midthoracic plaques consistent with demyelination. A subsequent brain MRI revealed five scattered, nonenhancing, white-matter lesions; including a large (approximately 5 mm in diameter) lesion involving the corpus callosum and two lesions perpendicular to the left lateral ventricle. Her B12 level, HTLV-1 antibodies, vitamin E level, RPR, ANA, and Lyme titre were all within normal limits She was started on glatiramer acetate following her clinically isolated syndrome of partial transverse myelitis and MRI findings confirming dissemination in space. Two and a half years after her initial presentation, she developed right hemiparesis that resolved over a few days following treatment with intravenous steroids. She had a subsequent similar episode of left hemiparesis and hemisensory deficit two months after that, confirming a diagnosis of clinically definite multiple sclerosis.\nOne year after her initial presentation, she developed a sudden onset of discomfort in her legs associated with an urge to move that was worse at night and impaired sleep. The symptom resolved while walking and had been present for only 3-4 days at the time of presentation. Her last menstrual period was two weeks prior to this presentation, and she was faithfully using an oral contraceptive. Her iron level was 108 mcg/dl, iron saturation was 28.6%, and ferritin was 94 ng/ml. A brain MRI did not reveal any new lesions. Her symptoms resolved a few days after a course of intravenous methylprednisolone (1 g daily for three days), and she had no recurrence over the next three years.
A 56-yr-old woman was transferred to emergency room of our hospital because of suspected PTE on October fifth, 2010. When she arrived, mental state was clear and she complained dyspnea and chest discomfort. Initial systolic blood pressure (BP) was 90mmHg and heart rate was 116 bpm. Chest X-ray revealed no remarkable finding. Transthoracic echocardiography demonstrated decreased contractility of dilated right ventricle (RV) with D-shaped left ventricle on parasternal short axis view (). Also it showed a huge echogenic mass in dilated pulmonary trunk and a whip-like moving mass in right atrium (RA) (). The right atrial mass moved through tricuspid valve to right ventricle. Estimated pulmonary artery systolic pressure was about 70 mmHg. Computed tomography (CT) finding also showed heavy thrombus in main pulmonary artery which extended into lobar branches of pulmonary system and deep vein thrombosis of left external iliac vein ().We diagnosed PTE with huge thrombus on RA clinically. Because the patient had stable vital sign, we selected thrombolytic therapy with tissue plasminogen activator (t-PA) infusion (100 mg over 2 hr). After one hour from t-PA infusion, the patient suddenly became unconscious. Her peripheral oxygen saturation was 76%, systolic BP 50 mmHg, and heart rate decreased to 33 bpm. Shortly afterward, the patient arrested and we started cardiopulmonary resuscitation (CPR). During CPR, our team made a critical decision to apply percutaneous cardiopulmonary support (PCPS) system for hemodynamic stabilization before surgical embolectomy. PCPS was inserted via right femoral artery and left femoral vein under fluoroscopic guidance and local anesthesia. The Capiox (Terumo, Tokyo, Japan) emergent bypass system was used. Thereafter, hemodynamic state of patient was stabilized at the PCPS flow rate of 3-3.5 L/min. We revised our plan to continue thrombolytic therapy and anticoagulation with PCPS support in consideration of stable vital sign and high bleeding risk of surgery during thrombolytic therapy. Heparin was administered intravenously and warfarin was overlapped after 5 days. Activated coagulation time was maintained within target range of 150-200 sec and activated partial thromboplastin time (aPTT) was controlled at approximately 80 sec during the PCPS support. She was decannulated sixty-two hours after PCPS insertion, when repeat echocardiography noted that RV function was much improved compared with initial echocardiography and estimated pulmonary artery systolic pressure decreased below 50mmHg even though mass remained on main pulmonary artery.\nIn sequence, ventilator was weaned and she transferred to general ward. General condition of the patient was improved gradually and she discharged without complication. After discharge, warfarin was continued at target international normalized ratio (INR) of 3. One month later from discharge, we followed up chest CT. The CT showed thrombus on main pulmonary artery and deep vein thrombus of left external iliac artery were clearly disappeared (). For two years follow up, she has been doing well without any complication and recurrence of thromboembolism.

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