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The patient was an unemployed 65-year-old female patient with height and weight of 158 cm and 48 kg, respectively. Her nationality and race were Korean and Mongoloid, respectively. She visited the outpatient clinic for pain in her knee and hip joints on both sides. The knee pain had developed seven years ago and was more severe on the right than on the left and caused a lot of disruption in her daily life. The patient also had been experiencing knee instability since adolescence, but no special diagnosis or management had been performed. The instability was aggravated by sitting, standing up or pivoting motions. The patient complained of a 3-year history of pain in the hip joints of both sides without any special trauma, which made it difficult to walk more than 500 m without walking aids. The patient had no past or family history of diseases that would be accompanied by multiple joint pain.\nUpon physical examination, the right knee presented with slight joint effusion and no marked anterior or posterior laxity (negative Lachman and pivot shift tests and anterior/posterior drawer tests), but the patient had an instability on the frontal plane (laxity at the valgus: grade 3, laxity at the valgus; grade 3). The left knee also showed no marked anterior or posterior laxity (negative Lachman and pivot shift tests and anterior/posterior drawer tests), but instability on the frontal plane (laxity at the valgus: grade 3, laxity at the valgus; grade 3) was demonstrated. There was no limitation in the range of motion of either knee. Both hip joints were positive on the Patrick test and rolling test and both joints showed a limited range of motion (right: flexion 90°, extension 10°, abduction 25°, adduction 15°, internal rotation 10°, external rotation 20°; left: flexion 95°, extension 10°, abduction 20°, adduction 15°, internal rotation 15°, external rotation 20°).\nBlood tests showed that the whole blood cell test, as well as the liver, kidney, thyroid, parathyroid, and adrenal function tests, were all within normal limits. Tests related to autoimmune diseases (rheumatoid factor, antinuclear antibody, anti-cyclic citrullinated peptide, and HLA B27) were all negative.\nA lower extremity scannogram showed more valgus anatomic axes (right: 12°, left: 10°) than normal (reference value: 6° ± 3°) (A). Both knee standing radiographs showed joint space narrowing of the lateral compartment with osteoarthritis (right: Kellgren-Lawrence grade 3, left: Kellgren-Lawrence grade 3) and hypoplasia of the tibial intercondylar eminence and flat trochlea femoralis were also seen (B). Physician-applied varus/valgus stress radiographs revealed lateral and medial compartment joint openings in both knees (). Magnetic resonance imaging (MRI) showed reduction of the cartilage thickness in the weight-bearing area of the lateral femoral condyle, a scarcely pronounced intercondylar notch and absence of the ACL (). On both hip anteroposterior views, both hip joints showed severe osteoarthritis (right: K–L grade 4, left: K–L grade 4) ().\nOn the basis of the above clinical and image findings, we diagnosed congenital aplasia of the ACL with secondary knee and hip osteoarthritis.\nThe patient complained of more pain in the right knee and right hip than in the left side, so right total knee and hip arthroplasty (TKA, THA) were performed simultaneously. About one year after the last surgery, the patient presented with more aggravated pain and instability of the left knee and hip joint. She underwent TKR and THR simultaneously (). As a result of the surgeries, the patient's symptoms improved and she was satisfied with the results. After the surgeries and follow-up for more than a year (right 24 months, left 12 months), both her knee and hip were pain-free without evidence of instability on physical examination.
A 71-year-old male with a medical history of CHF with an ejection fraction of 10%, pulmonary hypertension with right ventricular systolic pressure (RVSP) of 59, coronary artery disease status post coronary artery bypass graft (CABG) ×6 in 2013, sleep apnea, chronic kidney disease stage 3, and chronic obstructive pulmonary disease presented to the hospital with worsening abdominal distention, shortness of breath, and increased lower extremities edema within the last couple of days. He uses 3 L of oxygen at home with BiPAP (bilevel positive airway pressure) at night. The patient admitted to orthopnea and paroxysmal dyspnea. He notes adherence to a strict oral fluid intake and takes lasix 40 mg daily in addition to bumex 2 mg twice a day. He first developed ascites and pleural effusion in 2019 after he presented with worsening shortness of breath and bloating with abdominal ultrasound showing ascites. He underwent diagnostic paracentesis with drainage of straw-colored fluid. Computed tomography (CT) abdomen from June 2019 showed small pleural effusion and was not drained. On further review, he required monthly paracentesis for his ascites with drainage of straw colored or light green fluid of 5 to 9 L each time. With his recurrent ascites, he had at least 5 different surgeons performing his therapeutic paracentesis for varying record of either straw colored or cloudy fluid.\nOn physical examination, the positive findings noted were normal respiratory effort with crackles noted bilaterally with diminished breath sounds in the right base and jugular venous distension noted. His cardiac examination was unremarkable. He did have abdominal distension with dullness to percussion with a positive fluid wave. There was also +2 pitting edema to bilateral lower extremities. Vitals were stable. Laboratory tests done on admission was unremarkable with blood urea nitrogen of 41 mg/dL and creatinine of 1.59 mg/dL, which is his baseline. His serum lactate dehydrogenase was 152 IU/L, albumin was 3.0 g/dL, and total protein of 7.2 g/dL. His chest X-ray showed moderate pleural effusion with atelectasis or infiltrate in the right lower lobe. He then had a chest CT that showed moderate right pleural effusion with compression atelectasis, no infiltrate, and moderate ascites. Review of his abdominal CT scan done in December 2019 had shown mild hepatomegaly with possible hepatocellular disease, which could be secondary to congestive hepatomegaly. There were no masses seen in either the chest or abdomen CT. Bedside ultrasound of the lung showed moderate pleural effusion.\nFor this visit, he had a diagnostic paracentesis in which 9.2 L of cloudy milky fluid was drained. His peritoneal fluid analysis workup as seen in was unremarkable with negative culture. His serum ascites albumin gradient was 1.6 g/dL. However, he does not have a history of any cirrhosis. His peritoneal fluid sent for flow cytometry was negative for any B- or T-cell lymphoma. He had a trial of intravenous laxis, but the next day, he still had a moderate-sized pleural effusion as seen on ultrasound. He then elected for a therapeutic thoracentesis in which 1.1 L of milky fluid was drained. Due to the milky appearance of the fluid, the decision was made to test the pleural fluid for triglycerides, which came back as 280 mg/dL and cholesterol of 140 mg/dL. His pleural fluid analysis as seen in had shown a total protein of 2.8 g/dL, lactate dehydrogenase (LDH) of 82 IU/L, and lymphocytic predominance. Based on the Light criteria, it was a transudative pleural effusion. After thoracentesis and paracentesis, his dyspnea improved to where he was discharged home.\nAfter his hospitalization, he had further workup for chylothorax. He did need another paracentesis 2 months later in which the peritoneal fluid was tested for triglycerides, which was 671 mg/dL confirming chylous ascites. He had a lipid panel done which showed triglycerides of 125, total cholesterol of 143, low-density lipoprotein of 97, and high-density lipoprotein of 21. A liver ultrasound showed no focal abnormality with no dilated ductal structures visible. Repeat echocardiogram was obtained which showed a severely dilated left ventricle with an ejection fraction <15% with severely reduced left ventricular systolic function. There was also severe global hypokinesis of the left ventricle. Right ventricle (RV) was moderately dilated with severely reduced function with TAPSE of 9.7 mm and RVSP of 55 indicative of moderate pulmonary hypertension.\nHe was recommended to adapt to a medium chain triglyceride diet in which our dietitian here educated him on. Unfortunately, no centers close to us have lymphoscintigraphy available. We did find a center that was able to do the test; however, the patient has no means of transportation to get that far, so he had elected to not pursue this option at this time. Liver cirrhosis was ruled out, and due to the low percentage rate of thoracic surgery causing chylothorax and with CABG in 2013 and his ascites and pleural effusion started in 2019, we determined that his biventricular failure was likely contributing to his chylous ascites and chylothorax. He is currently on conservative management with monthly paracentesis, medium chain triglyceride diet, lasix, and bumex for fluid diuresis. Even though his blood pressure, which stays on the low side and history of chronic kidney disease, is limiting his diuresis management, he is maintaining an overall negative balance daily.
An 89-day old female infant, presented to our clinic with absent nasal columella since birth (). The patient was a product of a normal vaginal delivery (NVD) of a preterm (27 weeks) pregnancy, with birth weight of 1.1 kg. The patient was admitted to the NICU for prematurity and respiratory distress, and was discharged after 70 days. Currently, she has no difficulty in breathing nor feeding. The patient has family history of congenital heart disease of her uncle who is currently 10 years old and is doing well and both of her elder brother and sister are medically free. Moreover, the mother denied radiation exposure or utilization of any medications during pregnancy or breast feeding. The patient has no previous history of trauma, malignancies or infections, and there was no consanguinity between the parents.\nThe physical examination revealed that her growth chart (weight, height, and head circumference) is at the 30th percentile according to the CDC growth chart for females below 36 months. A total absence of the nasal columella from the nasal tip down to the root of the philtrum, involving the medial crura of the ala cartilage. Surrounding structures such as the septum, nose, and upper lip are normal. The rest of her physical examination was entirely normal.\nThe laboratory investigations, chest x-ray, echocardiogram, and ultrasound of the abdomen were all unremarkable. After discussing the treatment options with the patient’s parents, they preferred the option of having the newly introduced 2-stage reconstruction of the columella described by Pan et al. [] after the age of one year. The first stage will involve bilateral nasal sill flaps that will be elevated and mobilized medially to create the new columella, and in the second stage we will insert an auricular composite graft to provide support where a portion of the caudal septum was missing, and to complete the new columellar reconstruction [].
A 74-year-old female who lived alone was found dead in her home. The estimated time of death was evaluated to be of 48 hours. According to the relatives’ information, she was diagnosed with hypertension and was under a diagnostic workup of an ovarian cyst. The judicial authority required an autopsy, which was performed 2 days after the discovery of the corpse.\nThe external examination revealed an apparent well-preserved corpse with a body mass index of 34.8, without any sign of external injuries. The examination of the head and neck was unremarkable. However, the examination of the lung depicted an extensive bilateral thrombosis of the main pulmonary arteries until their segmental subdivision. The large thrombus entirely occluded the arterial lumen, reproducing the shape of the vessel as a “cast”, coated by the intimal surface, characterizing a bilateral massive pulmonary thromboembolism.\nAccording to the morphological characteristics, a thromboembolic nature was macroscopically confirmed ( and ).\nAt the opening of the abdominal and pelvic cavities, the left uterine adnexa were represented by a smooth cystic tumor, weighing 15 kg () and measuring 33 cm in its longest axis. At the cut surface, the cyst was multiloculated and drained a yellowish mucinous material. No papillary excrescences were seen, but a partly necrotic and solid nodule of 8.5 cm was found adhered to the cystic wall.\nThe examination of the contralateral ovary and uterus was unremarkable, the latter showing an atrophic endometrium. The bilateral dissection of the deep vessels of the lower limbs failed to show thrombosis, while the exploration of the veins of the pudendal plexus (ovarian and periuterine veins) showed the presence of extensive thrombosis ().\nNo other noteworthy finding was detected. The cause of death was identified as massive pulmonary thromboembolism in a woman with a large ovarian neoplasm. During the autopsy, different organs were sampled (uterus, ovarian neoplasm, pudendal plexuses and thrombotic formations) for histopathologic investigation.\nMoreover, samples of biological fluids (heart and femoral blood, urine, bile and gastric content) were taken for toxicological analyses. The search for drugs and/or alcohol was performed and results were negative.\nMicroscopically, the massive ovarian neoplasm was assessed as “atypical proliferating mucinous tumor (borderline)” () and the thrombotic nature of the occluding material in the periuterine veins was confirmed, with secondary thromboembolism in the pulmonary arteries.\nTherefore, the cause of death was identified as pulmonary thromboembolism due to pelvic thrombosis, concomitant with a giant ovarian neoplasm. According to the evidence provided by the autopsy and the histological findings, the compression effect of the mass on the pudendal venous plexus enabled the formation of intravascular thrombi, whose detachment led to pulmonary embolism and death.
A 59-year-old man presented to our hospital with a chief complaint of progressive swelling of his right lower extremity associated with pain. He had previously undergone partial resection of a soft-tissue mass in the right thigh during early childhood and his thirties at another hospital. However, the residual mass had gradually increased in size, eventually causing discomfort and pain. The details of the previous surgical treatment were not available. The patient had no other significant medical or family history except laparoscopic cholecystectomy for gallstones 16 years ago. A physical examination revealed a diffuse, compressible swelling extending from the right proximal thigh to the back of the knee (). The swelling was massive, especially in the frontal aspect of the thigh, and a blue discoloration of the skin suggested an invasion of vascular lesions into the skin. No limb-length discrepancy was observed. Laboratory data revealed elevated D-dimer (7.52 µg/mL) and fibrin degradation product (FDP; 15.3 µg/mL) levels. Other laboratory data were within the normal ranges. Short inversion time inversion recovery (STIR) T2-weighted magnetic resonance imaging (MRI) revealed an area of high signal intensity in the subcutaneous tissue of the right lower extremity, indicating a large VM (). The lesion was well-circumscribed, and the structures below the muscle fascia were normal. Right femoropopliteal arteriography revealed no evidence of abnormal hypervascular lesions or rapid arteriovenous shunting (). Based on these findings, a clinical diagnosis of a slow-flow VM was made. After discussing the case among the relevant departments, complete surgical resection was indicated because the lesion was located in the subcutaneous tissue and was well-circumscribed. The operation was performed under general anesthesia. A skin incision was made over the lesion in a zig-zag manner to prevent scar contracture. The subcutaneous lesion, including the involved skin, was excised. Some perforating veins were ligated, and macroscopic complete resection was achieved. Two subcutaneous drainage tubes were placed, and the skin incision was closed with simple interrupted sutures. A histopathological examination of the specimen revealed dilated capillary and venous channels with sparse smooth muscle cells, which were compatible with the findings of VM (). During the postoperative course, minor skin necrosis at the incision line was observed. After wound care with surgical debridement, split-thickness skin grafting was applied on postoperative day 35 for coverage of the wound. No functional impairment associated with the operation was observed. The patient was discharged 52 days after surgery. He was followed up with clinical and ultrasonographic examinations. At 8-month follow up, he was free of symptoms and satisfied with the results ().
We report the case of Mr X, a fit and healthy 46-year-old Chinese gentleman with no previous medical or surgical history who presented to the acute surgical assessment unit complaining of a 6-month history of vague abdominal discomfort. On further questioning he revealed his pain was localized predominantly to the left upper quadrant (LUQ), was dull in nature and associated with nausea, dyspepsia and early satiety. On physical exam there was a palpable mass in the LUQ which was smooth and non tender. Laboratory investigations were all within normal limits.\nMr X underwent an esophagogastroduodenoscopy which did not identify any mass or abnormality. He later received a CT abdomen which displayed a well circumscribed 5.5 cm × 5.5 cm × 5.6 cm mass projecting from the mesenteric fat and lying adjacent to the transverse colon with no identifiable cleavage plane between them (Fig. ). There were no pathologically enlarged lymph nodes identified. Mr X subsequently underwent colonoscopy which was normal.\nHis case was discussed at a gastrointestinal multi-disciplinary meeting with differential diagnoses of primary colorectal cancer, large gastrointestinal stromal tumour (GIST) and DT all considered. It was elected that Mr X undergo laparotomy with excision of this mesenteric mass.\nOn opening the abdomen the mass was found to be infiltrating surrounding structures including the small bowel and had encased itself around the superior mesenteric artery and vein. Blood supply to the small bowel was compromised and the mass was adherent to the cecum necessitating an extended right hemicolectomy, small bowel resection, jejuno-jejunal anastomosis and a ileo-colic anastomosis.\nUnfortunately it was not possible to excise the mass intraoperatively and a sample was sent for histology.\nHistological examination identified hypocellular spindle cells which can be seen to infiltrate between adjacent adipocytes and which stained positive for beta-catenin but stained negative for the GIST markers CD117 and DOG-1 (Fig. ) supporting the diagnosis of DT.\nHis lesion is currently inoperable and systemic treatment options are being considered whilst his mass is kept under radiological surveillance.
A 64-year-old man with recent inferior wall myocardial infarction was transferred to the emergency department. He had chest pain for 3 days about two weeks previously and underwent coronary angiography at another hospital. The coronary angiography showed total occlusion of mid-RCA and 70% long segment stenosis at mid LAD. PTCA was tried to open RCA but failed.\nOn admission to our hospital, he was hemodynamically unstable with severe pulmonary congestion, respiratory distress and hypotension requiring vasopressor support. His blood pressure was 90/60 mmHg with infusion of Dobutamine 10 μg/Kg/min, pulse rate was 100/min and respiration rate was 25/min. On physical examination, systolic murmur was audible at the cardiac apex and coarse breath sounds were audible, with rales and wheezing over both lower lung fields. The electrocardiogram showed sinus tachycardia with the rate of 113/min and Q wave in II, III, aVF that was compatible with old inferior wall myocardial infarction. The chest X-ray showed cardiomegaly with pulmonary congestion. The transthoracic echocardiogram showed akinesis of inferior wall with moderately reduced left ventricular systolic function with estimated ejection fraction of 40% and moderate to severe mitral regurgitation with anterior mitral leaflet prolapse. The eccentric mitral regurgitant jet was directed to the posterolateral side of the left atrial chamber in color flow mapping, and pulsed Doppler interrogation of the right upper pulmonary vein demonstrated holosystolic flow reversal suggesting severe mitral regurgitation. The transthoracic echocardiogram could not detect papillary muscle rupture. Initially, he was treated medically with intravenous dobutamine, digitalis, ACE inhibitor and diuretics for 3 days, but the cardiogenic shock and pulmonary edema state continued without improvement. So, he underwent transesophageal echocardiography for further evaluation of mitral regurgitation. In mid-esophageal two-chamber view, grade III/IV mitral regurgitation with anterior mitral leaflet prolapse was identified (). In mid-esophageal four-chamber view, flail motion of anterior mitral valve leaflet was visualized which was highly suspicious of the ruptured chordae tendinae (). In transgastric two-chamber view, we could identify the ruptured head of papillary muscle as a separated mobile mass attached to chordae tendinae of anterior mitral valve leaflet and the stump of the ruptured posteromedial papillary muscle (, ). During diastole, the two ruptured parts moved freely within the left ventricular cavity but, during systole, each ruptured end of the papillary muscle parts approaced each other and we could clearly visualize the stump of the ruptured posteromedial papillary muscle within the left ventricle (, ). With these findings, he was transferred to cardiac surgery. At the operation field, the ruptured parts of the posteromedial papillary muscle were identified but repair looked impossible, so the valve was removed and mitral valve replacement, as well as LIMA to LAD bypass surgery, was successfully performed. The post-operative course was uneventful and the patient survived.
A 19-year-old man was brought to the emergency department following a road traffic accident after his motorcycle skidded and hit the road divider. His Glasgow Coma Scale (GSC) on initial examination was 12/15. He sustained laceration of his upper lip and tongue, comminuted fracture of the right mandible parasymphysis, and avulsed teeth 11, 12, 41, 42, 43, and 44 (). He was intubated immediately for airway protection. An emergency head CT scan showed that he also sustained depressed fracture of the frontal bone with subdural and epidural hemorrhage. CT scan also showed right parasymphysis mandible fracture and dislocated left condyle (). The left condyle was dislocated anteriorly and superiorly into the infratemporal fossa medial to the zygomatic arch. There were no fractures of the condyle and zygomatic arch.\nHe underwent emergency craniotomy with evacuation of blood clot by the neurosurgical team. In the same setting, the facial laceration injury was sutured and an arch bar with intraosseous wiring was placed to stabilize the fractured mandible. Condyle dislocation reduction was also attempted. Due to the orotracheal intubation tube, the occlusion was not assessed following reduction. The patient was then transferred to the intensive care unit (ICU) subsequently with the orotracheal intubation kept in place. Following extubation 5 days later, it was noted that the patient kept his mouth open without any closure movement. There was also excessive drooling of saliva due to the inability to close his mouth. On examination, his mandible movement appeared restricted and the mandible was unable to move in any direction. He was not obeying instruction well. Multiple manual reduction attempts at bedside were unsuccessful.\nAn open reduction and internal fixation was planned for the right parasymphysis of mandible fracture, and it was planned to perform reduction of the dislocated condyle on the left side. Owing to the patient's neurological injury, the surgery could only be done 2 weeks after the injuries were sustained. In view of the ASD condyle and the prolonged period of dislocation, we anticipated difficult reduction. This was discussed with the patient and his family, and it was decided if the need for open or surgical reduction arises, they prefer surgical approach to be done intraorally. During the surgery under general anesthesia with muscle relaxation, initial attempts were made to reduce the left condylar dislocation by using manual traction by Hippocratic method and then with the assistance of a mouth gag but proved to be unsuccessful. Our next attempt was to release the intraosseous wiring at the parasymphysis fracture site effectively rendering the mandible in two separate pieces to simulate mandibulotomy-assisted reduction as described by previous clinicians [, ]. Once this was not successful, we went ahead with an intraoral condylotomy on the left side by piezoelectric surgery. First, a coronoidectomy was done to get access to the condyle. Then, the condylar neck was osteotomized using the piezosurgery, and the mandible was then able to be pushed back into occlusion. Finally, open reduction and fixation of the right parasymphysis fracture was performed and stable occlusion was achieved.\nPostoperative CT scan confirmed the reduction of the dislocation (). There was a slight deviation of the jaw to the left and mouth opening was 19 mm. Occlusion was acceptable and elastics were placed for 6 weeks. Jaw exercises were encouraged, and review after 2 months postoperatively showed improvement in mouth opening at 40 mm with stable occlusion. The patient was then referred to a prosthodontist for further rehabilitation and treatment of the missing teeth.\nOn 1-year follow-up, the patient presented with no complaint. Clinically, there was no tenderness at the joint or muscle of mastication on palpation or during movement. However, there was mild asymmetry of the jaw with the chin deviated 2 mm to the left (). Mouth opening was maintained at 40 mm with deviation to the left on opening (). Occlusion was good with upper and lower dentures in place. CT scan shows union between the condyle head and the condylar process stump (). Its position remains as seen immediately after surgery. The stump of the condylar process meanwhile has remodeled to form a neocondyle.
A 65-year-old female patient reported to the outpatient department of Terna Dental College, Navi Mumbai, complaining of swelling on the left side of the jaw for 1 year and on the right side over the past month. She was apparently fine 1 year ago. Her past dental history revealed extraction of teeth numbers 36 and 37 1 year ago, following which she noticed swelling in the left mandibular posterior region, which gradually enlarged to the present size. Her past medical history was noncontributory and a general physical examination revealed no abnormalities.\nExtra orally, diffuse bilateral swellings were noted over the lower third of the patient’s face. The overlying skin appeared to be normal. On the left side, swelling extended superior-inferiorly from 2 cm below the ala tragus line to the lower border of the mandible, and anteroposteriorly from the corner of the mouth to the anterior border of the masseter. On the right side, swelling extended from the ala tragus line to the lower border of the mandible and from the corner of the mouth to the ascending ramus (). The swelling was firm and non-tender. The left submandibular lymph nodes were palpable, firm, and mobile.\nOn intraoral examination, a soft, painless, proliferating growth was seen extending posteriorly from the distal aspect of 35 to the retromolar region measuring approximately 4.5 cm × 2.5 cm. Teeth numbers 36 and 37 were missing and a soft tissue mass was found to be covering the crown of 38. Buccal vestibular obliteration with cortical expansion was observed (). Swelling was soft and non-tender, and showed surface ulceration because of indentations of the opposing dentition.\nA right-side lesion presented as a gingival/alveolar mass on the buccal aspect of 45 to 47 measuring approximately 2 cm × 1.5 cm. The involved teeth were vital and free of any carious lesion. Growth was soft to firm in consistency with well-defined margins and an ulcerated surface.\nAn orthopantomograph revealed the presence of bilateral ill-defined radiolucent lesions. The left-side lesion showed the involvement of the entire mandible extending from tooth number 31 to the ramus. Root resorption of involved teeth was noted. The right side showed a radiolucent lesion around the periapical area of 46 and the interdental area of 45 and 46 (). Cone-beam Computed Tomography (CBCT) revealed expansile lytic lesions with irregular borders. The periapical area in relation to 41 and 42 was spared ().\nBased on the clinical and radiographic features, the provisional and differential diagnoses considered were of intraosseous carcinoma, ameloblastoma, central giant cell lesions, and lymphoma.\nHistopathological examination of the incisional biopsy from both right and left sides showed diffuse sheets of uniformly appearing plasma cells with eccentric nuclei and pale cytoplasm (). Nuclear hyperchromatism and pleomorphism of cells were suggestive of a malignancy (). Connective tissue stroma showed interstitial amorphous eosinophilic amyloid-like material in abundance, which was later confirmed with Congo red staining (). Occasional giant cells were also noted. Immunohistochemistry (IHC) was performed, which revealed strong positivity for CD138 and lambda light chain; kappa chain was negative ( and ).\nRoutine hematological and biochemical investigations revealed the presence of anemia, hypocalcemia, and raised parathyroid hormone (PTH) levels. Protein electrophoresis of serum showed elevated levels of total serum protein and globulin suggestive of a characteristic M band in the gamma globulin region and hypoalbuminemia. The values of serum free light chain assay were well within normal limits. Based on these findings, the diagnosis of bilateral SBP of the mandible was made. The patient was referred to the oncology center for further systemic work-up and radiographic survey to rule out MM. Unfortunately, the patient did not survive to complete the investigations.
A 55-year-old female came to dermatology outpatient department with a single ulcerated mass measuring 5 × 4 cm, involving left nasolabial fold and cheek. The lesion had developed 3 years back. Base of the ulcer was covered with granulation tissue. She also had multiple asymptomatic, small, firm, skin-colored papules and nodules all over the face and neck since 25 years []. General physical and systemic examination revealed no abnormality. Routine laboratory investigations were within normal limits. There was no history of consanguinity in the family. A skin biopsy was performed from both the ulcerated mass and one of the papules present on the neck.\nHistopathological examination of the ulcerated mass showed focal ulceration of epidermis with underlying tumor composed of basaloid cells in nests, cords and solid sheets in a myxoid background []. Focal peripheral palisading and retraction artifact was also seen []. The cells were small, monomorphic with round to oval hyperchromatic nuclei and scant cytoplasm. Squamous differentiation was seen within some of the cell nests []. Frequent mitosis was present with focal areas of necrosis []. Intervening stroma showed acute and chronic inflammatory cells. On immunohistochemistry (IHC), basaloid cells were positive for Bcl2 (diffuse, ) and CD10 []. CD34 was negative in the stromal as well as the epithelial cells [] while an epithelial membrane antigen (EMA) was positive in the squamous cells []. Based on these findings, a diagnosis of basal cell carcinoma with squamous differentiation was rendered.\nA skin biopsy from a papule on the neck showed a circumscribed tumor in dermis composed of multiple keratin-filled horn cysts surrounded by basaloid cells and solid aggregates with peripheral palisading surrounded by proliferating fibroblasts. No mitosis was seen. Overlying epidermis showed mild thinning. A diagnosis of TE was made [].\nHer son who is 36 year old presented with similar multiple papulonodular lesions all over the face since childhood []. A skin biopsy from the lesion showed features of TE [].
An otherwise fit and healthy 28-year-old man with no previous hand injuries presented to the accident and emergency department after midnight with a retained bolt in his little finger. While resisting police arrest, the police had to use TASER guns to detain him. The patient was awake with no other significant injuries and reported that he was hit with bolts from a distance of approximately two meters. Police officers were able to remove all bolts from his forearms and thighs except for this one in his left little finger, which was fixed to the middle phalanx. After primary and secondary surveys where no other injuries were noted, the patient was referred to the Plastic Surgery Trauma Service. Clinical examination revealed that the bolt entered the dorsum of the middle phalanx with no exit wound. Capillary refill was normal at the tip of the little finger, he had normal sensations in both digital nerves distribution, and the flexor and the extensor tendons were intact.\nHis X-ray (Figure ) showed the bolt penetrating both the cortices of the middle phalanx from dorsal to volar with the tip ending in volar soft tissues. From a clinical point of view, the main concern was that the deep, pointed part is barbed at right angles, which would suggest difficult retrieval through the same entry point (similar concept to fish hook barbs). The patient refused overnight stay, as he was in police custody and preferred to return to receive his treatment the following day. He was discharged with oral amoxicillin/clavulanate 625 mg three times a day.\nUnder regional block and arm tourniquet control, the surgeon retrieved the entire bolt through the entry point and confirmed the complete removal using fluoroscopy. The wound was explored dorsally by a lazy S incision, and the extensor tendon was split longitudinally at zone II. A Bruner’s type incision was used to explore the volar aspect. A hematoma was identified within the flexor sheath, but the flexor tendon was found to be intact. The injury was distal to the Flexor Digitorum Superficialis insertion. Both neurovascular bundles were intact. The flexor sheath, as well as the bony tract, were thoroughly washed out with normal saline. No structural repair was required. The patient was later discharged with oral amoxicillin/clavulanate 625 mg for five more days. No further complications were encountered in the immediate post-operative period.
A 46-year-old woman presented with shortness of breath, food intolerance, and inability to sleep in the supine position for 1.5 years. The patient was known to have an elevated left hemidiaphragm diagnosed as diaphragmatic eventration since her childhood, which was confirmed by chest radiograph and computed tomography (CT) scan. Diaphragmatic plication was performed with a 10-cm low lateral thoracotomy incision at the ninth intercostal space. The postoperative course was uneventful. The patient presented 1 year later, complaining of pain in the left lateral chest wall and left abdominal upper quadrant along with a sensation of fullness in the epigastric region which started 4 weeks after the surgery. The pain was described as sharp and stabbing exacerbated by movement and local touch, which was rated as 7/10 on visual analogue scale (VAS) on the lateral chest wall. The sensation of fullness in the epigastric region was aggravated by the upright posture, food intake and bending forwards rather than coughing or deep breathing. The nature of the pain was described as a pulling pain, rated as 8/10 on the VAS. There was no history of shortness of breath, cough or chest pain. On examination, the patient had significant hyperalgesia and hyperesthesia covering a large area of the lateral left chest wall from the mid-axillary line to the left upper quadrant in the distribution of the thoracotomy scar. The incision scar was clean and well healed. No tenderness or mass was elicited at the site of epigastrium. Chest radiograph and upper gastrointestinal endoscopy were obtained to exclude the possibility of recurrence of the eventration and injury to the abdominal viscera. Radiologic imaging was unremarkable and showed the repair to be intact, with no visceral injury or neuromas identified at the incision site. The patient had earlier received an intercostal block which provided partial pain relief. Prior medical treatment included non-steroidal anti-inflammatory drugs (NSAIDS) and gabapentin which provided only partial pain relief.\nA diagnosis of PTPS was made and left-sided diagnostic intercostal nerve blocks at the ninth to 11th thoracic intercostal spaces were performed. Pain was relieved only in the thoracotomy scar area, and the patient complained of persistent epigastric discomfort. The patient returned to her home country, but presented with the same complaint 1 year later. A repeat left diagnostic intercostal nerve block was performed in preparation for a possible intercostal neurolysis. Again, the thoracic wall pain improved but the epigastric discomfort persisted. As the earlier blocks proved ineffective in eliminating the epigastric discomfort and multiple investigations were unremarkable, involvement of the phrenic nerve was postulated as the cause of the discomfort. With a history of left diaphragmatic paralysis, a diagnostic left phrenic nerve block was planned. Under ultrasound guidance, the left phrenic nerve was identified under the left sternocleidomastoid muscle and above scalenus anterior muscle. Using a 22-gauge echogenic needle, a total of 10 mL of 0.5% ropivacaine was injected to anesthetize the left phrenic nerve. There was significant improvement of the abdominal pain and fullness following the left phrenic nerve block. The patient still had some residual pain along the chest wall radiating to lateral and anterior abdominal wall. It was postulated that the pain was mediated by two separate sites, including the intercostal nerves due to intercostal neuralgia at the incision site, and due to traction of the peritoneal ligaments of the stomach on the site of repair at the diaphragm mediated by the left phrenic nerve. A combined ultrasound-guided left intercostal neurolytic block for left intercostal neuralgia and local anesthetic phrenic nerve block for her epigastric pain was planned. Under ultrasound guidance with real time imaging, 15 mL of 0.6% phenol was injected in the ninth to 11th thoracic intercostal spaces. Ultrasound-guided phrenic nerve blockade was also performed with 5 mL of 0.5% ropivacaine and methylprednisolone acetate (40 mg). As there is a paucity of literature on phrenic nerve neurolysis, a corticosteroid was added to the local anesthetic for the blockade with the intent to increase the duration of block. The patient experienced significant pain relief with a decrease of the VAS from 8/10 to 3/10. The pain relief lasted for 4 - 6 h. The result was confirmed with a repeat phrenic nerve block at a later date that also resulted in a complete pain relief for a similar duration. In both instances, the patient had a transient partial sensorimotor blockade of the left shoulder, which is an expected consequence of the phrenic nerve block.
A 19-year-old man was brought to the emergency department following a road traffic accident after his motorcycle skidded and hit the road divider. His Glasgow Coma Scale (GSC) on initial examination was 12/15. He sustained laceration of his upper lip and tongue, comminuted fracture of the right mandible parasymphysis, and avulsed teeth 11, 12, 41, 42, 43, and 44 (). He was intubated immediately for airway protection. An emergency head CT scan showed that he also sustained depressed fracture of the frontal bone with subdural and epidural hemorrhage. CT scan also showed right parasymphysis mandible fracture and dislocated left condyle (). The left condyle was dislocated anteriorly and superiorly into the infratemporal fossa medial to the zygomatic arch. There were no fractures of the condyle and zygomatic arch.\nHe underwent emergency craniotomy with evacuation of blood clot by the neurosurgical team. In the same setting, the facial laceration injury was sutured and an arch bar with intraosseous wiring was placed to stabilize the fractured mandible. Condyle dislocation reduction was also attempted. Due to the orotracheal intubation tube, the occlusion was not assessed following reduction. The patient was then transferred to the intensive care unit (ICU) subsequently with the orotracheal intubation kept in place. Following extubation 5 days later, it was noted that the patient kept his mouth open without any closure movement. There was also excessive drooling of saliva due to the inability to close his mouth. On examination, his mandible movement appeared restricted and the mandible was unable to move in any direction. He was not obeying instruction well. Multiple manual reduction attempts at bedside were unsuccessful.\nAn open reduction and internal fixation was planned for the right parasymphysis of mandible fracture, and it was planned to perform reduction of the dislocated condyle on the left side. Owing to the patient's neurological injury, the surgery could only be done 2 weeks after the injuries were sustained. In view of the ASD condyle and the prolonged period of dislocation, we anticipated difficult reduction. This was discussed with the patient and his family, and it was decided if the need for open or surgical reduction arises, they prefer surgical approach to be done intraorally. During the surgery under general anesthesia with muscle relaxation, initial attempts were made to reduce the left condylar dislocation by using manual traction by Hippocratic method and then with the assistance of a mouth gag but proved to be unsuccessful. Our next attempt was to release the intraosseous wiring at the parasymphysis fracture site effectively rendering the mandible in two separate pieces to simulate mandibulotomy-assisted reduction as described by previous clinicians [, ]. Once this was not successful, we went ahead with an intraoral condylotomy on the left side by piezoelectric surgery. First, a coronoidectomy was done to get access to the condyle. Then, the condylar neck was osteotomized using the piezosurgery, and the mandible was then able to be pushed back into occlusion. Finally, open reduction and fixation of the right parasymphysis fracture was performed and stable occlusion was achieved.\nPostoperative CT scan confirmed the reduction of the dislocation (). There was a slight deviation of the jaw to the left and mouth opening was 19 mm. Occlusion was acceptable and elastics were placed for 6 weeks. Jaw exercises were encouraged, and review after 2 months postoperatively showed improvement in mouth opening at 40 mm with stable occlusion. The patient was then referred to a prosthodontist for further rehabilitation and treatment of the missing teeth.\nOn 1-year follow-up, the patient presented with no complaint. Clinically, there was no tenderness at the joint or muscle of mastication on palpation or during movement. However, there was mild asymmetry of the jaw with the chin deviated 2 mm to the left (). Mouth opening was maintained at 40 mm with deviation to the left on opening (). Occlusion was good with upper and lower dentures in place. CT scan shows union between the condyle head and the condylar process stump (). Its position remains as seen immediately after surgery. The stump of the condylar process meanwhile has remodeled to form a neocondyle.
A 33-year-old woman (gravida 2 para 1) with a history of amenorrhea for 8 weeks and 4 days presented with two consecutive failed surgical abortions. She had a previous history of preterm delivery by cesarean section at gestational age of 33 weeks under the diagnosis of breech presentation in 2011. A uterine anomaly was found incidentally during surgery. On May 3, 2014, pregnancy was confirmed by detecting gestational sac in the endometrial cavity using ultrasonography at 5 weeks and 2 days of gestation. One week later, the crown-lump length (CRL) was consistent with an age of 6+0 weeks without a fetal heartbeat. At 8 weeks of gestation, a fetal heartbeat was still not observed and CRL had not increased. Under the diagnosis of missed abortion, she planned to undergo D&E. Ultrasound-guided D&E was attempted, but the suction cannula could not reach the uterine cavity with the gestational sac at a local clinic and a secondary referral hospital.\nAfter the failed surgical abortion, she was referred to our hospital. A pelvic examination revealed a single uterine cervical opening without vaginal septum. Ultrasonography revealed two separate uterine cavities with a 2.8-cm gestational sac in the left-sided cavity but no definite fetal pole. A septum dividing the uterine cavity was observed, but fundal indentation was not definite (). For pregnancy termination, ultrasound- and/or operative hysteroscopy-guided surgical abortion was planned. The day before the operation, misoprostol 400 µg was inserted intravaginally to soften the uterine cervix. After adequate dilatation of the uterine cervix, a curette was inserted into the uterine cavity under ultrasound guidance; however, it did not reach the left-sided uterine cavity containing the gestational sac. When a hysteroscope was inserted into the uterine cavity, it passed easily into the empty right-sided uterine cavity but could not be passed into the left-sided cavity. During the operation, slight communication to left-sided cavity was noted just above the cervical internal os. After the communication was widened using hysteroscopic resection of the uterine septum, the hysteroscope could enter the left-sided cavity with the conceptual tissue (). After further resection of the uterine septum, conceptual tissue was successfully removed by curettage and vacuum aspiration under ultrasound guidance. Because the placental tissue was within the uterine septum, we did not perform a septectomy. The patient was discharged without complications on the same day. Two weeks later, magnetic resonance imaging (MRI) was performed to enable a more accurate diagnosis of the Müllerian anomaly. MRI revealed that the septum extended nearly to the external cervical os without fundal indentation, suggesting complete septate uterus with cervical septum. There were no specific findings in the endometrial cavity or either ovary. There was no urologic anomaly (). According to the European Society for Human Reproduction and Embryology/European Society of Gynecological Endoscopy (ESHRE/ESGE) classification system, the female genital anomaly was classified as U2bC1V0.\nBecause the patient experienced adverse obstetrical events, including preterm delivery and miscarriage, an operative hysteroscopic septectomy was planned for the next pregnancy. Upon entry to the endometrial cavity, diffuse endometrial polyps and a uterine septum were noted (). However, a cervical septum was not found. Operative hysteroscopic uterine septectomy and endometrial polypectomy were performed on September 5, 2014. She was discharged uneventfully later the same day. After the operation, an intrauterine Foley catheter was inserted for 1 week, and high-dose continuous cyclic estrogen-progestin therapy with conjugated equine estrogen 1.25 mg was taken PO bid for 28 days, followed by medroxyprogesterone acetate 10 mg qd for 14 days for 2 months. The pathology finding of the uterine septum was “endomyometrial tissue showing proliferative phase.”
A 76-year-old woman was admitted to our hospital for the treatment of a right common iliac artery aneurysm. The patient had been previously diagnosed with a right-sided aortic arch with ALSA originating from KD. On physical examination, we found that her blood pressure was 112/60 mmHg in both arms, and her heart rate was regular at 90 beats per minute. Furthermore, no carotid bruits or any other abnormal findings were observed. Concomitantly, the laboratory examinations were unremarkable. Chest X-ray revealed a tortuous aorta with the absence of the left first aortic arch. While transthoracic echocardiography demonstrated no congenital anomaly, computed tomography (CT) revealed the right-sided aortic arch and KD with the maximal diameter 33 mm (). Three-dimensional CT scanning showed the proximal-to-distal order of the branches of the aortic arch to be as follows: the left common carotid artery, right common carotid artery, right subclavian artery, and ALSA originating from the KD (). Moreover, a right common iliac artery aneurysm with a maximal diameter of 48 mm could be recognized.\nOn the day before the scheduled surgery for iliac aneurysm, the subject complained of sudden back pain and numbness of the left arm. CT analysis revealed a retrograde type A aortic dissection and occlusion of ALSA. The false lumen had thrombosed, and an intimal tear was found in the KD (). The ascending aorta was 39 mm in maximal diameter, and the thrombosed false lumen was 10 mm thick (). ALSA originating from the KD was obstructed due to compression of the false lumen. Axillo-axillary bypass was immediately performed with an 8-mm ringed expanded polytetrafluoroethylene (ePTFE) graft, which caused the blood flow to the left arm to resume. After antihypertensive therapy for 2 months, CT showed an occlusion of the axillo-axillary bypass graft, but the false lumen was significantly diminished in size, and ALSA was well enhanced by increased antegrade flow in the true lumen. CT showed no abnormalities of the cervical and intracranial arteries and the circle of Willis. Because there was a possibility that the KD could rupture or recanalize to the false lumen, endovascular treatment was chosen to exclude the KD, including the intimal tear of the dissection. The endovascular procedure with debranching of ALSA was performed under general anesthesia in the operating room 2 months after the onset of aortic dissection. Two GORE TAG Thoracic Endoprostheses (WL Gore and Associates, Flagstaff, AZ, USA) were inserted through the left femoral artery and deployed in the descending aorta according to the diameter at the proximal and distal landing zone. A 34-mm-diameter, 200-mm-long GORE TAG® stent graft was first deployed at the distal descending thoracic aorta. Second, a 37-mm-diameter, 150-mm-long GORE TAG® stent graft was deployed at the level of the ALSA coverage. Percutaneous coil embolization of the proximal segment of ALSA was then performed to ensure exclusion of the KD through the left brachial artery. After deployment of the devices, angiography showed successful exclusion of the KD and a small type 2 endoleak. Finally, a blood pressure gradient of 13 mmHg was observed between the arms.\nThe patient’s post-procedure course was uneventful. Neither left arm claudication nor subclavian steal syndrome occurred. Although CT scanning revealed a small endoleak, the stent graft was correctly positioned, and the KD had been successfully excluded (). The patient was successfully discharged from the hospital on the seventh day after the treatment. Three months later, endovascular repair of the right common iliac artery was performed. At that time, the endoleak remained. However, 1 year later, follow-up CT showed that the endoleak had disappeared. The most recent scan was performed 5 years after the operation and showed neither any endoleak nor any dilatation of the KD.
A 10-year-old girl with a 2-year history of intermittent hematuria was referred to the Urology department of a tertiary hospital in Johannesburg. She had received multiple courses of antibiotics for the same at the local clinic. Urine microscopy done at the local clinics suggested urinary tract infections. No parasite eggs were seen on urine microscopy. The child described the hematuria to be terminal, with an initial clear urine stream followed by bloody urine toward the end of urination. This raised suspicion that the source of the bleed might be in the lower urinary tract. The mother gave history of travel to rural parts of the KwaZulu-Natal province of South Africa and admitted that her child went for swims in the rivers.\nOn examination, the child appeared clinically well and comfortable. No signs of malnutrition nor pallor. Systemic examination was unremarkable. A bedside ultrasound was done which showed normal kidneys, but a suspicious posterior wall bladder mass seen on ultrasound was concerning. Blood tests showed normal renal function and normal full blood count. The child was admitted for a cystoscopy under general anesthesia. On cystoscopy, multiple hemorrhagic polypoidal lesions were seen on the posterior wall of the bladder to the dome of the bladder (Figure ). Adjacent bladder mucosa showed a granular pattern known as “sandy patches” which indicate areas of healed schistosomiasis. The ureteric orifices, trigone, and urethra were normal. Multiple biopsies were taken to rule out malignancy.\nThe histology revealed multiple viable Schistosoma ova on a background of dense inflammatory infiltrate, with a marked predominance of eosinophils (Figure ). The child was treated with Praziquantel 40 mg/kg orally as a single dose. The child responded well to treatment and the hematuria resolved permanently. To rule out upper urogenital tract disease involvement, a CT intravenous pyelogram (IVP) was performed, the result of which was normal.\nAt 6 months follow–up, the child was doing well with no complaints. A surveillance cystoscopy was performed which showed a normal bladder. Renal function tests and renal ultrasound were normal. The child was discharged after being counseled about warning signs of recurrence and preventive measures to avoid new contact with the pathogen.
A 28-year-old nulliparous pregnant Japanese woman was referred for prenatal evaluation at 30 weeks of gestation because of abnormal ultrasound findings of fetal congenital heart defects. The family history of the parents was unremarkable. Fetal ultrasonography at 30 weeks and 5 days of gestation showed vascular ring, Ebstein's anomaly, ventricular septal defect, and single umbilical artery. The estimated fetal body weight corresponded to the Japanese standard for the gestational age. Fetal ultrasonography showed vertex presentation of the moving fetus and the fetal stomach appeared to be normally dilated (). The pregnant woman had not felt any fetal movements since 31 weeks of gestation. At 32 weeks and 5 days of gestation, fetal ultrasonography showed absence of fetal movement with breech presentation, polyhydramnios, absent filling of stomach, and fetal growth restriction (FGR) (). However, abnormal Doppler findings regarding the fetal middle cerebral artery, umbilical cord artery, and ductus venous were not observed. Clinical diagnosis of fetal akinesia was made at this point. At 32 weeks and 6 days of gestation, amniocentesis was performed to assess the possibility of chromosomal aberrations. Interphase fluorescence in situ hybridization (FISH) analysis on uncultured amniocytes for chromosome 13, 18, and 21 revealed two signals, respectively. At 34 weeks and 6 days of gestation, progression of polyhydramnios with maternal respiratory compromise occurred () and 2300 mL of amniotic fluid was removed. G-banding analysis on cultured amniocytes revealed a karyotype of 46,XY,22pstk+ (). After discussion with the parents about the prognosis of the fetus based on ultrasound findings, including fetal akinesia since 31 weeks of gestation, FGR, congenital heart defects, and left-sided pleural effusion that indicated severe phenotype with prenatal onset of genetic disorders, perinatal palliative care was chosen. At 36 weeks and 3 days of gestation, fetal ultrasonography showed further progression of polyhydramnios with maternal compromise (), and 2000 mL of amniotic fluid was removed and labor was induced with oxytocin. The breech neonate was delivered vaginally at 36 weeks and 4 days of gestation with an Apgar score of 1 at 1 min and 1 at 5 min. Birth weight was 1839 g, length 45.5 cm, head circumference 31.8 cm, and chest circumference 23.5 cm. External examination revealed marked muscular hypoplasia of upper and lower extremities, extremely thin transverse palmar creases, joint contractures of lower extremities, hypertelorism, and deep-set ears. The neonate died within 2 h after birth due to respiratory failure. Therefore, we could not assess developmental profile after birth. In addition, permission for neonatal autopsy was not obtained from the parents. Clinical features of the neonate were not sufficient to diagnose a specific disease but suggested the possibility of genetic disorders, including diseases caused by either a single gene or a chromosomal defect. After genetic counseling and obtaining written consent from the parents, whole exome sequencing (WES) was performed with genomic DNA extracted from the placenta using the eXome Hidden Markov Model v1.0 (XHMM). Although the causative gene mutations related to the phenotype of the neonate were not identified, a 3 Mb deletion of chromosome 1p was suspected (). The suspected deleted region by the exome analysis using XHMM was further validated by chromosomal microarray (CMA). CMA analysis demonstrated monoallelic deletion located from positions 849466 to 3347420 on chromosome 1p36.33–p36.32 (Chr1:849466–3347420) including 76 genes, which is known as chromosome 1p36 deletion syndrome (). Among 76 genes, the gene SKI, which is known to contribute to the phenotype of chromosome 1p36 deletion syndrome, is included [, ]. These results were consistent with the prenatal sonographic findings and the neonate was diagnosed with chromosome 1p36 deletion syndrome. In addition, CMA analysis revealed no additional copy number variations (CNVs), which suggested de novo deletion rather than inheritance from the parents. After genetic counseling for future pregnancies, the parents decided against genetic carrier screening. Postnatal sub-telomeric FISH analysis on cultured amniocytes revealed a terminal deletion of chromosome 1p ().
In November 2007, a 14 year old female patient presented to our dental office. She had an open bite and had experienced a traumatic dental injury at nine years of age. Tooth 21 was avulsed and had been replanted and tooth 11 was intruded. Both teeth had ankylosed, were infraoccluded, and demonstrated a stone percussion sound. Her complaints were the aesthetic appearance of these teeth and the fact that she had used an orthodontic appliance for a long time (since nine years of age). Clinical and radiographic findings presented a face with a convex profile, open bite, and the presence of an orthodontic appliance (). On radiographic evaluation, tooth 11 showed pulpal canal obliteration and tooth 21 had received root canal treatment. Inflammatory root resorption and replacement root resorption were present in both teeth, and tooth 12 demonstrated apical root resorption ().\nAfter evaluation, orthodontic treatment was recommended, with extraction of the four first premolars and autotransplantation of the mandibular premolars to the site of the upper central incisors. The treatment plan consisted of removing the orthodontic appliance, study for orthodontic treatment, extraction of teeth 14 and 24, extraction of teeth 11 and 21 and preparation of the sockets, extraction of teeth 34 and 44 and transplantation to the sockets of teeth 11 and 21, reshaping the transplanted teeth, placement of a full fixed appliance, removal of the orthodontic appliance, and the placement of an upper Hawley retainer and lower flexible lingual bonded retainer on the six anterior teeth. The patient's parents accepted the proposed treatment plan.\nArticaine hydrochloride anhydrous was used as a local anesthetic, with 1:200,000 adrenaline tartrate (Meganest 1:200,000, Clarben, S.A., Madrid, Spain). Teeth 11 and 21 were extracted after an intrasulcular incision to allow for better gingival fiber reinsertion. The recipient sites were evaluated clinically and on the radiographs (). The donor teeth were measured with periapical radiography and acrylic model teeth were made with the same shape and dimensions of the donor teeth (). These models were used in the preparation of the alveolar recipient sites.\nThe recipient bed was prepared for immediate transplantation to the site of tooth 11, whereas transplantation was deferred by ten days for tooth 21 (). The teeth chosen for transplantation were cleaned with a polishing brush and toothpaste, and disinfection of the oral cavity was carried out with a solution of 0.1% chlorhexidine digluconate and 0.1% clorbutanol hemihydrate (Eludril, Pierre Fabre Dermo-Cosmetique, Soual, France). The teeth chosen for transplantation were atraumatically extracted and gently inserted into the prepared alveolar socket. The extraalveolar time of the transplanted teeth was minimal (less than five minutes), in order to maintain the vitality of periodontal ligament cells.\nAfter transplantation, the teeth were splinted during a period of ten days using 3-0 silk sutures crossing the occlusal face of the tooth, and then were fixed with composite (). The occlusion was checked and controlled with blue/red 60 µm interarticular paper (D-50769, Bausch KG, Koln, Germany). An antibiotic was administered, starting 24 hours before the intervention (one gram amoxicillin tablets, one tablet every 12 hours), and continuing for six days after the procedure.\nInstructions were given for analgesic treatment (paracetamol, 500 mg tablets as necessary, no more than 4 tablets in 24 hours). An ice pack was applied to the face, protected with fabric or paper. A soft diet was advised for 48 hours after the transplantation, and chewing with the transplanted teeth was prohibited for a period of seven days. Careful oral hygiene was also recommended, followed by a mouthwash with 1 mg/mL chlorhexidine digluconate (Eludril), diluted in 5 mL of water. The sutures were removed 10 days after the operation. The day after the transplantation, the patient was observed and questioned regarding the occurrence of pain, swelling, and bleeding. Follow-up examinations were performed 10 days, one month, three months, six months, and annually after the transplantation. In each follow-up examination, a radiographic examination and sensitivity testing to cold and percussion were performed, as well as observations of the gums, the marginal groove, and dental mobility.\nOrthodontic treatment began three months after the transplantation, because orthodontic movement after periodontal healing could prevent the ankylosis of autotransplanted teeth. After a temporary resin-based composite build-up on the natural premolar crowns, they were shaped into upper central incisors (). Orthodontic treatment was performed and transplanted teeth were clinically evaluated through percussion, mobility tests, and radiography. Due to the young age of the patient, a period of time was allowed to pass with monthly vitality checks, in the hope that revascularization would occur. However, revascularization did not take place. The transplanted teeth developed pulp necrosis, and root canal treatment was performed (). The periodontal ligament healed and healthy periradicular tissues were maintained.\nA satisfactory aesthetic result and functional occlusion were obtained. The 7 year follow-up showed maintenance of aesthetic improvements, adequate function of the transplanted and reshaped teeth, and radiographic evidence of periradicular tissue health ().
A 53-year-old African-American woman with ESRD was transferred from dialysis clinic to the emergency room (ER) for evaluation of non-radiating and dull epigastric pain for two weeks associated with fever and chills during hemodialysis (HD). Three months ago, she was hospitalized and treated for Streptococcus pneumoniae and Enterobacter cloacae bacteremia. A year ago she was treated for S. maltophilia bacteremia secondary to an infected dialysis catheter. Past medical history was also significant for hypertension, atherosclerotic vascular disease pending elective coronary artery bypass graft (CABG) surgery, and sudden cardiac arrest followed by ICD placement. With the current presentation, both blood and catheter cultures obtained at the dialysis clinic were positive for S. maltophilia, prompting her subsequent arrival to the emergency room. She presented with a continuation of fever and chills, as well as tachycardia and episodic hypotension. She was noted to have mild epigastric tenderness. There was no surrounding erythema, discharge, or tenderness noted around the tunneled dialysis catheter on the right anterior chest. Initial workup showed elevated troponin and procalcitonin. Chest X-ray findings were suggestive for left lower lobe pneumonia. Within the ER, the patient's hypotension resolved with fluid resuscitation and intravenous levofloxacin therapy was started with blood cultures drawn.\nCardiology was consulted for persistent elevation of troponins and it was presumed secondary to impaired clearance in ESRD. Transthoracic echocardiography (TTE) was done to evaluate for endocarditis given the presentation of bacteremia and fevers. TTE revealed artifact noted on an abandoned ICD lead in the right heart concerning for possible vegetation. Repeat blood cultures were positive for S. maltophilia and the patient was continued on levofloxacin. Infectious Diseases was consulted and as per their recommendation the infected tunneled dialysis catheter was removed on the 2nd day of the hospitalization (DOH). The patient continued to be febrile despite levofloxacin therapy and a transesophageal echocardiogram (TEE) was performed to look for a cardiac source. Subsequent blood cultures were negative at this time but the patient continued to be symptomatic. The TEE was done on the 4th DOH and showed a 1 x 0.5 cm echodensity attached to an abandoned right ventricular (RV) ICD lead in the superior vena cava (SVC) as it entered into the right atrium (RA). The echodensity was concerning for a vegetation due to IE. ID consultation recommended removal of the abandoned lead with culture of the probable vegetation that may have served as a nidus for recurrent bacteremia. Levofloxacin therapy was continued and a new dialysis catheter was placed on the 5th DOH with HD restarted. At this time, the patient became afebrile and reported symptomatic improvement. Despite a strong suspicion for IE by Duke criteria, fluorodeoxyglucose positron emission tomography (FDG PET) on the 6th DOH was non-diagnostic for differentiating infective etiology from thrombotic. Cardiothoracic surgery was consulted for removal of the abandoned lead for culture and planned to do so in coordination with her pending elective CABG procedure. In the interim, repeat blood cultures remained negative on the 7th DOH and the patient was stable and discharged on the 12th DOH with instructions to transition to oral levofloxacin until her CABG procedure, scheduled 18 days from discharge.\nUnfortunately, the patient expired due to complications from cardiac arrest secondary to severe hypokalemia in the postoperative period after removal of the infected ICD lead and successful CABG. No vegetation could be appreciated on gross inspection of the removed ICD-lead and subsequent culture was negative for any growth, indicating a resolution of the IE over the one-month course of levofloxacin treatment since presentation.
The index patient, a 38 year old female, was born after an uneventful pregnancy and delivery. Shortly after delivery she was found to have an ectopic 3rd kidney and a patent ductus arteriosus which required surgical closure at 18 months of age. Her development was normal with no history of seizures, meningitis, encephalitis or major head trauma. She was noted to have thrombocytopenia at age 23 years after complaints of unexplained weight loss. Bone marrow aspiration did not reveal any abnormalities. She continues to have easy bruising and prolonged bleeding time. She had her first seizure at 24 years with no evidence of any precipitating factors. Her neurologic examination was normal. Brain magnetic resonance imaging (MRI) revealed bilateral multiple and diffuse PNH (Figure \n). She has episodic headache with photophobia and phonophobia. An echocardiography at age 32 years revealed a mildly dilated aortic root and ascending aorta with mild aortic regurgitation.The second patient, a younger sister of the index case, is now 36 years of age. Her medical history is relevant for migraine headaches with visual aura. Her neurologic examination was normal and her EEG did not show any interictal abnormalities. An MRI of the brain shows heterotopic gray matter of both hemispheres (Figure \n). Her echocardiogram was normal.\nThe parents are a healthy nonconsanguineous couple. The mother is now 56 years. She is of English, Irish, and Belgian ethnic background. Her past medical history is unremarkable. Her brain MRI does not show evidence of PNH. The father is now 58 years. He is of Ukrainian and Polish ethnic background. His past medical history is unremarkable. His brain MRI does not show evidence of PNH.\nDNA sequencing revealed both sisters to be heterozygous for a c.2002C > T transition in exon 13 of the FLNA gene resulting in a p.Gln668Ter mutation. This nonsense mutation has previously been reported as a FLNA disease-causing mutation in a patient with PNH\n[]. Analysis of lymphocyte extracted DNA from the mother and father did not reveal the FLNA exon 13 mutation. Parental relationships were verified molecularly using the Identifier kit (Applied Biosystems).
A 36-year-old male with a medical history of HIV presented to our institution with behavioral changes characterized by decreased responsiveness. He had been diagnosed with HIV after routine screening 14 years prior. His HAART regimen consisted of abacavir-lamivudine, atazanavir, and ritonavir. At baseline, he was able to ambulate and converse without difficulty. Four months before presentation, the patient was noted to have stiffening of his neck and back. During this time, the patient also developed decreased verbal output. When the patient's family first noticed these symptoms, the patient was transferred from his previous residence in a group home into his parents’ house. While residing with his parents, the patient stopped taking his HAART. His mother reported that his symptoms initially improved, but he later developed further worsening of his speech and gait. His mother restarted his HAART, however, the patient continued to worsen to the point of multiple falls and virtually no verbal output. This prompted his family to bring the patient to the emergency room. When admitted to an internal medicine service, he was found to have minimal verbal output, minimal responsiveness to verbal stimuli, and decreased movements. The patient was continued on his HAART. Empiric acyclovir was started to cover possible viral encephalitis. Psychiatry was consulted for possible catatonia. He was given lorazepam without improvement. After failure of this benzodiazepine challenge, neurology was consulted.\nOn our neurology team's initial assessment, the patient was awake and alert but was minimally verbally or physically responsive. He would mouth words, without audible response. He had rigidity in all extremities, more severe in his lower extremities. There was no significant tremor. He also had diffuse hyperreflexia and sustained bilateral ankle clonus. He required assistance to stand, had difficulty initiating movements, had stooped posture, and had a magnetic gait.\nMagnetic resonance imaging (MRI) brain showed extensive leukoencephalopathy with periventricular involvement and subcortical extension in the bilateral frontal and parietal lobes []. Given the symmetry of leukoencephalopathy, degree of global cerebral atrophy, and lack of gadolinium enhancement, this likely represented sequela of HIV encephalopathy. The patient also had subtle mineralization of bilateral basal ganglia. MRI spine was unremarkable. His absolute CD4 count was 208 cells/mcL, and HIV viral load was 36378 copies/mL. Serum analysis revealed a borderline thiamine level at 64 nmol/L. Otherwise serum analysis was unremarkable, including normal ammonia, Vitamin-B12, and folate level. Serum toxoplasma antibodies and rapid plasma reagin were negative. Blood bacterial cultures and mycobacterial cultures were negative.\nCerebral spinal fluid (CSF) analysis revealed 94 mg/dL protein, 58 mg/dL glucose, 2 white blood cells/mcL, and <1 red blood cell/mcL. Serum and CSF cryptococcal antigen were negative. CSF herpes simplex virus, varicella virus, and John Cunningham virus polymerase chain reactions were negative. CSF West Nile virus IgM and IgG were negative. CSF cultures were negative, including bacterial, mycobacterial, and fungal.\nGiven the patient's clinical presentation and radiographic findings, the patient was started on carbidopa-levodopa. This was initiated at 12.5–50 mg TID with gradual titration up to 87.5–350 mg TID (total levodopa 1050 mg daily). With titration of levodopa to 1050 mg daily, the patient had improved extrapyramidal signs and symptoms. He had increased facial expression and improved fluidity and spontaneity of speech. His bradykinesia and gait apraxia also improved significantly. He was also started on amantadine 100 mg twice per day as recommended by the physical medicine and rehabilitation physicians to optimize participation with rehabilitation. Before initiation of amantadine, the patient had already reached the optimal carbidopa-levodopa dose (total levodopa 1050 mg daily) and had shown significant symptomatic benefit. The patient was discharged on HAART, amantadine, trimethoprim-sulfamethoxazole prophylaxis, thiamine, and carbidopa-levodopa 87.5–350 mg TID.\nThe patient was evaluated in clinic 3 months after discharge. He was compliant with the carbidopa-levodopa regimen. He retained the level of functionality he had regained at discharge. Furthermore, he did not display any adverse effects, specifically no dyskinesias or any adverse psychological reactions.
A 27-year-old Thai female presented with a rapidly growing mass over her right thigh for 3 months. She had no underlying or previous surgery. She had pain when she walked. She had slightly limited her right hip function on flexion and abduction. Physical examination revealed a firm nonpulsatile mass over the right thigh measuring ~8 × 6 cm. in diameter (). No notable grossly skin involvement and the mass appears not fixed to bony structure. No sensory deficit was identified. Inguinal lymph node cannot be palpated.\nThe magnetic resonance imaging (MRI) scan demonstrated a 7.8 × 5.8 × 5.7 cm, in vertical × transverse × AP diameter of well-defined, thin rim, enhancing simple cyst with vascularity at the right anterior intermuscular fascia of the upper thigh, just inferior to the right inguinal region. The cyst was located between the pectineus muscle and sartorius muscle, accompanied with anterior bulging to subcutaneous fat. The right common femoral artery and vein and superficial artery and vein were displaced posteriorly ().\nPercutaneous aspiration of cyst revealed clear yellowish fluid about 60 ml., and the cytological examination showed hypocellularity consisting of few small lymphocytes and foamy macrophages in background of few erythrocytes and concluded cystic fluid. The cyst disappeared for a few days and presented in the same size within one week.\nThe operation was performed on supine position under general anesthesia. Vertical incision was performed; the cyst was identified and found dense adherance to femoral artery. Carefully, dissection was done. Complete cystic removal with femoral artery preservation was successful with minimal leakage of cyst wall (). Cystic content had shown the clear, yellow fluid ().\nMicroscopic examination was demonstrated as . Hematoxylin-eosin staining (H&E staining) indicated that the wall of the cyst consisted of a single flattened cell lining. The cell lining was histologically supposed to be derived from endothelium or mesothelium by routine H&E staining (Figures and ). Immunohistochemical studies for D2-40, CD31, CD34, and calretinin were performed to define the nature/origin of the cystic cell lining. Immunohistochemical studies revealed the cell lining of the cyst was positive for CD34, CD31 (), and D2-40 () and negative for calretinin. The constellation of histology and immunohistochemistry demonstrated that the wall of the cyst was not derived from the mesothelium but from the lymphatic vessels. The final pathological diagnosis was a benign lymphatic endothelial-lining cyst of soft tissue. The cystic fluid was sent for cytology and revealed no malignancy. No postoperative complication was found.
The patient, a 24-year-old, right-handed, Caucasian female, assistant nurse, with no previously diagnosed impairment of glucose metabolism was admitted to our Center for Eating Disorders due to her AN, which she suffered from 13 years of age. Current hospitalization was her 2nd stay in the department. She had been comorbidly diagnosed with posttraumatic stress disorder according to the ICD-10 (also valid under DSM-V). There was no history of type 1 diabetes in the family. Type 2 diabetes developed only in her grandmother (on mother's side) during the later stages of her life and was compensated on an insulin regimen. The patient was an everyday smoker of 2–3 cigarettes and an occasional drinker of 2 glasses of wine or 1 shot of hard liquor 2 times a week.\nUpon admission, she was fully oriented in all spheres, with no signs of neurological deficit and unremarkable blood work (see for the initial lab values). She had a history of chronic bronchial asthma, gastritis and secondary amenorrhea for 6 months due to her eating disorder. Her medication consisted of 300 mg of trazodone (used irregularly due to her low compliance) and albuterol sulfate inhaler for her asthma. The initial BMI was 17.4 (h = 176 cm; w = 53.9 kg).\nHer medication had been switched to 10 mg of escitalopram, 2.5 mg of olanzapine and her sleep had been augmented by 2 mg of bisulepin. She had been undergoing a specialized regime psychotherapeutic eating disorder programme with a positive weight gain of 1.8 kg in 2 weeks.\nDuring the third week of hospitalization, she signed an informed consent and was enrolled in the randomized, double-blinded study focused on possible effects of tDCS on self-perceived body image. In the study we included patients between 18 and 65 years with a diagnosis of AN according to the ICD-10, we excluded patients with a history of brain injury, epileptic paroxysm, migraines or with metallic object within the neurocranium. We used Anamorphic Micro computer application to objectify the patient's perceived body image distortion. The patient initially overestimated her bodily size by 122% and desired a size of 101% compared to her objective proportions (objective dissatisfaction), which constituted 83% of her perceived size (subjective dissatisfaction) suggesting a gross distortion of body image congruent with the diagnosis and possibly leading to her rehospitalization.\nShe had been scheduled to undergo 10 sessions of 30min, 2mA anodal stimulation of the left DLPFC (F3, Current Density of 0.571 A/m2) with cathode over the right orbitofrontal region (Fp2) (). We used the HDCstim portable programmable direct current stimulator (manufactured by Newronika s.r.l.) with conductive silicone electrodes (anode 5x5 cm, cathode 6x8,5 cm) covered by hydratable holding bags in plant cellulose soaked in saline (0.9%).\nOn the second day of stimulation, the patient started referring to blurred vision, but she attributed it to her fatigue. The physical and neurological exam was unremarkable, and the patient did not wish to pursue any further examination.\nOn the 6th day of stimulation, she complained of recurring problems with eye accommodation and increasing fatigue, an onset of polydipsia resulting in polyuria was noticed. These symptoms were considered a part of her eating disorder. Nevertheless, an ophthalmological appointment was set up. At the beginning of the 7th day of stimulation, the research team was fully informed about the extent of the aforementioned symptoms and the principal investigator decided to stop any further application until the patient ´s state gets thoroughly examined and resolved, concurrently it was decided to unblind the study. We found out, that a real (non-sham) stimulation was used in the patient.\nOn the 8th day the symptoms of polyuria, polydipsia, and blurred vision had worsened, the ophthalmologist prescribed corrective lenses and suspected the unusual findings to be of psychogenic etiology.\nExtended blood work and an internal exam were ordered showing a fasting glucose level of 39 mmol/l (702 mg/dl). The patient became disoriented, confused and subdelirious, nauseous and referring to abdominal pain. She was immediately rushed to the acute internal ward to properly manage the hyperglycemic crisis.\nFurther diagnostic tests showed markedly decreased C-peptide levels and negative DMAP (Diabetes Mellitus Autoantibody Panel), which met the ICD-10 criteria for a diagnosis of DM1. Her olanzapine medication had been immediately discontinued and switched to quetiapine. The ultrasound exam ruled out any gross pancreatic pathology and in the next week the blood glucose level normalized on an intensified insulin regimen of 3 units of aspart 3 times a day and 2–4 units of glargine overnight.\nDuring our follow-up the perceived size constituted 123.5% compared to reality, the objective dissatisfaction reached 116% and the subjective dissatisfaction was 94%.
A 36-year-old female presented with a 6-month history of blisters on the dorsal aspects of her hands bilaterally with associated fragility and photosensitivity. This was also associated with a 2-year history of increased hair growth on her cheeks. She had been previously treated with oral antibiotics and topical steroid with no obvious improvement.\nThe patient had a medical background of asthma for which she was using twice daily fluticasone/salmeterol inhalations and salbutamol inhalations as required. Her only other medication was the oral contraceptive pill, which she had been taking long-term. She had no family history of PCT or haemochromatosis. The patient consumed approximately 15 alcoholic standard drinks per week.\nOn examination, the patient had multiple blisters and erosions distributed over the dorsa of her hands with a number of milia (fig. ). A punch biopsy of the right hand index finger and a perilesional biopsy of the left hand were performed. Histopathology showed a partly regenerated subepidermal blister with a heavy inflammatory cell infiltrate between the old roof and the regenerating base (fig. ). A small amount of festooning was evident. Immunofluorescence was strongly positive for IgG present along the basement membrane. The pattern was recognized as the ‘U-serrated pattern’, typical of antibodies to type VII collagen. Based on these findings, EBA was diagnosed and salt split skin preparation was not performed.\nAlthough the initial attending doctor was dissuaded from a diagnosis of PCT on the basis of histology findings, subsequent review favoured PCT clinically and porphyrin studies later confirmed this (table ). Further blood results demonstrated normal full blood count, renal function and electrolytes. The patient had deranged liver function tests, but her liver screen did not demonstrate any abnormalities, including negative hepatitis B/C and HIV serologies and normal iron studies and alpha fetoprotein levels. A liver ultrasound demonstrated findings of fatty liver. Desmoglein 1 and 3 reactivity and subsequent collagen VII reactivity testing were all negative, excluding a concomitant diagnosis of pemphigus or EBA.\nThe patient was commenced on hydroxychloroquine 200 mg twice weekly and was given advice on mechanical and photoprotection. She was also commenced on regular venesections, aiming for a ferritin level of 50 μg/l to improve PCT symptoms.
A 48-year-old heterosexual African man presented as an emergency with a progressive disabling anal pain for three days on a background one-year history of a chronic anal ulcer. He has not been on highly active antiretroviral therapy (HAART) since being diagnosed human immunodeficiency virus (HIV 2)-positive 11 years ago. The exquisite anal pain was constant, exacerbated by bowel motions and not relieved by opiate analgesia. There was no altered bowel habit but the fear of defaecation made him constipated and exacerbated the symptoms. There was no anal discharge or rectal bleeding on defaecation. He had no fever, night sweats, or anorexia but had lost weight significantly. He has a long history of severe anal pruritus which he believes precipitated the anal ulcer. Physical examination revealed a wasted man in great anal distress but with normal vital signs. Chest, cardiovascular and abdominal examinations were unremarkable. There was no soilage on inspection of the perineum and underclothes but perianal examination revealed excoriation and ichthycosis indicating long-standing pruritus. There was superficial scarring with macular patches from probable chronic herpetic simplex lesions. There was no characteristic visible posterior anal fissure or reactive perianal skin tag. A digital rectal examination was not done because of severe anal pain and spasm. He consented to examination under anaesthesia and appropriate surgical treatment. His haemoglobin level was 9 g/L but white blood cell and CD4 counts were not available. Under general anaesthesia, in the lithotomy position a digital rectal examination revealed a posterior broad based anal ulcer. Proctoscopy showed the ulcer commencing above the dentate line in the lower rectum and ending below at the anoderm just distal to the internal anal sphincter. It was associated with an induration over a swelling which expressed copious pus and faeces on pressure into the intersphincteric space. There was no external opening into the anal skin. With the aid of the Eisenhamer retractor, the ulcer cavity was demonstrated as secondarily extending in a horse-shoe manner in the intersphincteric and submucosal planes consistent with an HIV ulcer (). The differential diagnosis included an idiopathic anal fissure with a complicating abscess in an HIV-positive patient, an sexually transmitted disease (STD)–associated anogenital ulcer, anorectal abscess from an acutely infected intersphincteric anal gland and anal malignancy (carcinoma, lymphoma or Kaposi sarcoma). In a resourced area, biopsy would identify the treatable aetiology. The cavity was deroofed, debrided and irrigated with normal saline until brisk bleeding ensued. A minimal lateral internal sphincterotomy using the open method relieved the ensuing anal spasm. Apart from postoperative bleeding and the continuing discharge of pus that settled, he had instant pain relief. Because of a possible herpetic aetiology of the ulcer, he was commenced on acyclovir 200 mg bd for two weeks. He was discharged on the fifth postoperative day and was advised sitz baths and good perianal hygiene. Follow-up at six weeks showed an appreciative patient with a minimally painful healing anal ulcer and satisfactory anal continence.
A 21-year-old female patient presented with progressive exertional dyspnea and irregular palpitations for 3 months. She had past surgical history significant for excision of a cutaneous myxoma in her left arm. Physical examination revealed a high jugular venous pressure and a diastolic murmur. An electrocardiogram showed atrial fibrillation. Laboratory investigations were within normal limits except for a low TSH and elevated free T3 and T4. Transthoracic echocardiography (TTE) showed a large echogenic mobile mass with central constriction attached to the interventricular septum (IVS), occupying the entire right atrium and right ventricle (RV) and obstructing the flow of the tricuspid valve. There were two other masses of the same echogenicity: one was occupying the left ventricle (LV) and the other was in the left atrium attached to the interatrial septum at the site of fossa ovalis (, Videos ). The left ventricular dimensions and function were normal. Cardiac magnetic resonance showed similar findings with no septal invasion and tissue characterization suggestive of multiple myxomas (). Computed tomography of the chest, abdomen, and pelvis revealed the same findings () in addition to an enlarged thyroid nodule and a left adrenal cyst that measures 65 × 57 mm (). Ultrasonography of the thyroid gland revealed a markedly enlarged right lobe of the thyroid with normal vascularity. Serum aldosterone, dexamethasone suppression test, dehydroepiandrosterone sulfate, and 24-hour urine metanephrines were within normal limits.\nThe patient underwent surgery where all three masses were excised. However, the tricuspid valve was inseparable from the RV mass; hence, it was replaced with a tissue prosthesis. The masses were grossly reddish grey in color, fleshy, and gelatinous in consistency (). The histopathological examination of the excised masses revealed myxomatous cellular proliferations with sparse collagen fibers consistent with multiple myxomas (Figures and ). The patient was followed up on 6-month intervals. After 2 years of follow-up, the adrenal cyst was stable in size; however, TTE showed a mass in the left ventricular outflow tract (Figures and , Videos ). The newly developed mass was surgically excised, and histopathology revealed myxomatous tissue.
The patient was a 1.5-year-old boy who was admitted to the hospital with a primary complaint of fever, diarrhea, and vomiting. At the beginning of the hospitalization, the patient had a generalized clonic-tonic seizure. The patient was dispatched to our intensive care unit on the fourth day of admission due to reduced consciousness.\nPatient is the first child of a family and is resident of the village. He had a history of pneumonia at 6 months of age. There was a history of seizure of fever in his family. From the outset, he was treated with ceftriaxone, vancomycin, phenytoin, and acyclovir. The growth and development were normal.\nThe patient had tachypnea and tachycardia, and fever was 39°C. Percent oxygen saturation without getting oxygen was 98%. It was toxic and had GCS = 8. The patient had a mild tenderness on the right side of the abdomen with a predominance in the RLQ (right lower quadrant). The rectal examination was normal. The amount of urine output was normal. Patients were treated with liquid therapy, and intravenous antibiotics were changed to moropenem and vancomycin. Patient tests: serum electrolytes (sodium and potassium), blood gas analysis, coagulation tests, liver and kidney function tests, vidal test and Albumin were normal.\nSome of the patient's laboratory results were as follows:\nIn addition, he had hypocalcemia and Hypomagnesemia and were treated with sepsis as a common symptom. Due to biliary secretion from the stomach tube, bloody diarrhea, tenderness and abdominal distension, abdominal ultrasonography was performed with suspicion of obstructive problems such as enuresis and acute appendicitis.\nUltrasound findings favored the diagnosis of acute appendicitis: insignificant free fluid in the space between the interleuk and a non-compressible appendix of 65 mm in the lower right quadrant of the abdomen was evident. The patient underwent appendectomy (Figure ). One day after the surgery, the child was alert (Glasgow Coma Scale /Score GCS = 12), but the fever continued. Metronidazole and intraperitoneal ciprofloxacin were administered. Two days after the commencement of these antibiotics and appendectomy, the patient's alertness became normal, and the general condition was satisfactory with erythrocyte sedimentation reaction (ESR) = 32, and platelet count and white blood cell count increased. A pathological report of acute puffiness appendicitis with peri appendicitis confirmed. After two weeks, the patient was discharged with a good general condition.
A 30-year-old male patient presented with a low backache since 1-month, radiation of pain to the right lower limb and weakness of the right lower limb since 20 days. Neurological examination revealed, muscle strength grade III proximally and grade IV distally according to Medical Research Council (MRC) in the right lower limb and grade V in the left lower limb with impaired fine touch and pain sensation in L5 and S1 dermatome, with absent knee and ankle reflexes in the right lower limb and plantar reflex was mute on both sides.\nAn anatomical localization of thoracic spinal cord lesion was made. Magnetic resonance imaging (MRI) of spine revealed bulky edematous spinal cord from D4 vertebral body to D12 vertebral body along with a intramedullary ring enhancing lesion of size 1.1 cm × 0.9 cm located above the conus medullaris at the level of D11 vertebrae lesion was hypointense on T1 and hyperintense on T2 with ring enhancement on contrast []. Scolex could not be identified. MRI of the brain was normal.\nDifferential diagnosis of spinal NCC and tuberculoma was considered. Diagnostic cerebrospinal fluid analysis by nucleic acid amplification test was positive for anti-cysticercal antibodies; a final diagnosis of spinal neurocysticercosis was made. Peripheral smear revealed neutrophilic leukocytosis and stool examination was negative for ova and cyst. The patient had no history of consumption of pork.\nIn view of the small size of the lesion, extensive cord edema, and demyelination; the patient was managed conservatively, and surgery was deferred. The patient was initially treated with dexamethasone followed by addition of albendazole therapy (400 mg OD). At the time of discharge patient had significant neurological improvement and MRC grade IV in the left lower limb with improved sensations in L5 and S1 dermatome. At follow-up after 6 weeks of albendazole therapy, there was a significant reduction in symptoms with improved MRC grade V. Follow-up MRI showed a reduction in the size of lesion and edema of cord [].
A 54-year-old female patient came to dental office to improve her smile. There were no relevant medical history and no contraindication to dental treatment.\nThe extraoral examination indicated a symmetrical and mesognathic facial pattern with a convex profile. At rest, there were about 2 mm of incisal edge visible with slightly incompetent lips.\nOn the intraoral objective examination, the patient presented an all-ceramic crown on the element 16, two crowns screwed onto implants at the sites of the elements 35 and 36, and Miller's I class gingival recessions on the teeth 11, 21, 37, 34, and 45. The patient's biotype was thick, and there were no periodontal problems (). The movements of laterality and protrusion had no working and nonworking precontacts. The overbite was 3.5 mm and the overjet 1 mm.\nThe patient presented a canine and molar 1st dental class. The lower midline coincided with the facial median while the upper midline was displaced 2 mm to the right. The smile line was medium, the incisal trend compared to the lower lip was convex, the lip was 1 mm apart, the dental exposure was 8 teeth (from 14 to 24), and the labial corridor was normal both on the right and on the left.\nFrom the esthetic analysis of the profile, an adequate support of the upper lip was observed for the correct inclination of the upper incisors. At the dental esthetic analysis, the inversion of the dental axis of teeth 12 and 22 was found. Problems were present at the level of the gingival margins and at the embrasures lines. There were diastemas between all maxillary teeth with the exception of the space between 11 and 21.\nA surface of wear was found on the incisal edge of tooth 21, and there was the presence of white spots on 12, 11, 21, 22, and 23.\nSubsequently, posterior radiographs were performed to assess the presence of interproximal carious lesions, photographs were taken, and dental impressions were taken in order to make a diagnostic wax-up. At the time of the reevaluation, the presence of carious and periodontal lesions was not found. The diagnosis included the presence of diastemas between the upper front teeth, the incorrect position of the elements 12 and 22, the presence of white spots, the nonideal proportion of the elements 11 and 21, and the noncoincidence of the facial midline with the upper dental midline.\nThe treatment of the white spots could have been performed using infiltrating resin (Icon, DMG) resin with a superficial [] or deep [] treatment. However, the patient wanted a change not only in the color and surface texture of his teeth but also in morphology.\nIt was decided to treat the upper anterior teeth with a minimally invasive multidisciplinary orthodontic-prosthetic approach. The proposed treatment plan was to move the upper teeth to redistribute the diastemas between 14 and 24, restore a correct surface texture, and restore the position of the upper dental midline and the proportion of the teeth using feldspathic ceramic veneers.\nOn the study models originated by preliminary impressions, the technician performed an initial wax-up () on which he fabricated a silicone template (Vestige 70 shore, Trayart) to print the preliminary mock-up in the patient's mouth.\nThe flowable composite resin is then dispensed in to the silicone template and positioned in the patient mouth (). The previsualization provided a true copy of the planned wax-up allowing for an immediate evaluation of shape, volume, occlusion, and relationship with the surrounding tissue. The patient thus accepted the treatment plan. Impressions in polyvinylsiloxane (Aquasil, Dentsply) were taken, and a teleradiography and an orthopantomography were performed. X-rays and impressions were sent to the Invisalign® (Align Technology) center together with the orthodontic treatment goals.\nThe cephalometric radiography study has allowed to appreciate the following: severely brachifacial growth type; 1st skeletal and dental class; poor sagittal development of the maxilla; and increased mandibular development.\nThe orthodontic treatment of the upper arch alone was carried out using transparent aligners (), in order to redistribute the diastemas present in the superior frontal group so as to uniform the spaces, center the medians, and facilitate the subsequent prosthetic rehabilitation of the same. In addition to aligning the upper frontal group from 13 to 23, derotation of the 23 and correction of the distal tipping were performed.\nAfter six months, the orthodontic treatment was completed (), alginate impressions of the dental situation with the redistributed diastemas were taken. The technician developed class IV plaster models and made the final wax-up on them (), making a silicone template for the definitive mock-up. The mock-up was printed in the patient mouth after performing computerized anesthesia. The preparation of teeth 13, 12, 11, 21, 22, and 23 was performed through the mock-up so that they could be minimally invasive and preserve the greatest possible amount of enamel () []. In the mesial parts of the elements 14 and 24, two direct composite resin restorations were performed. Once the teeth were prepared, the retractor fibers (Retraction Cord #00, Ultrapak™) were positioned, and an impression in polyether (Impregum Penta, 3M ESPE) was taken. The mock-up was printed again on the prepared teeth, and it was finished and left in the patient's mouth as a temporary (). The dental technician received the impressions, developed the model, and stratified sintered feldspathic veneers from tooth 13 to tooth 23.\nAfter 7 days, ceramic veneers were made, and the patient returned at the dental office. The feldspathic ceramic veneers were etched with 9% hydrofluoric acid from 60 to 90 seconds, rinsed, and then cleaned with pure alcohol for 5 minutes. The silane was placed on them, and then the adhesive was brushed on without light curing. The prepared teeth were etched with 35% orthophosphoric acid for 30 seconds and rinsed for 30 seconds, and then the primer and the bonding were applied on without light curing. Resinous composite cement (Variolink Esthetic, Ivoclar Vivadent) was applied, the veneers were positioned on the teeth, and the cement excesses were removed.\nEach veneer was light-cured for 120 seconds.\nFinishing was carried out with fine-grain diamond burs and decreasing abrasiveness rubber burs. The patient returned at the office every six months for maintenance calls, and 3 years after the cementation, the control photographs and radiographs were taken ().
A 24-year-old woman presented to our outpatient department with complains of severe mid back pain since 2 months. Patient was four months postpartum and lactating. The pain was so incapacitating that she was not able to sit or stand. There was no history of any recent or previous injury or fall, and no history of a fever or constitutional symptoms. She was of short stature, thinly built, and kyphotic posture. There was tenderness on palpation of lower dorsal and lumbar vertebrae and movements were painfully restricted. There was no sensory or motor deficit. There was difficulty in carrying out routine activities, which compelled the patient to seek advice.\nRadiographs () showed kyphoscoliosis and multiple vertebral fractures. An MRI of the spine () showed vertebral compression fractures of D8, D9, D11, L2, and L3 vertebral bodies. Intervertebral discs and the spinal cord appeared to be normal. A detailed history was taken rule out cause of multiple pathological fractures. She was a multigravida (second delivery), having had her first child eight years ago. The first pregnancy was normal without similar complaints.\nA complete metabolic workup was done which was normal except for a mild increase in serum alkaline phosphatase (160; normal range, 20-140 IU/L). A PET scan was performed to rule out secondaries in view of raised alkaline phosphatase (), and it did not reveal any abnormalities. A dual energy X-ray absorptiometry (DEXA) scan of the spine and femur 45 region showed a T score of -4.5 at the lumbar spine and -2.8 at the hip region.\nShe was diagnosed with severe osteoporosis with multiple vertebral fractures and medical management and weaning was advised. She was reluctant to immediately wean her infant, so she was treated with total contact orthosis and vitamin D and calcium. At eight months postpartum, the pain subsided but DEXA scan showed no improvement in density scores. There were no new fractures detected on radiograph (). Lactation was stopped and she was started on teriparatide therapy along with vitamin D and calcium supplementation. At her one year follow-up, the patient was symptom free and DEXA scan showed improvement with T scores in the lumbar and hip region of -1.0 and -0.5, respectively.
A 61-year-old man presented with a history of dull aching pain in his lower spine, rectal pain, and increased urgency of defecation lasting for three weeks. Abdominal examination and digital rectal examination were unremarkable. A barium enema showed extrinsic compression of the lower rectum from a presumed low pelvic mass and two persistent filling defects within the descending colon. Colonoscopy showed a pedunculated polyp at 50 cm. Biopsies of this polyp confirmed it to be a tubulovillous adenoma with mild dysplasia. The rectal mucosa was normal. CT-guided biopsy of the presacral mass initially suggested a prostate cancer. However, on review of the histology and scan, a carcinoid tumour of the presacral space was diagnosed. An MRI of the pelvis carried out showed a loculated mass 6 × 4.3 × 4.8 cm, proximal to the lower rectum. The rectal mucosa was found to be intact whilst the serosal surface was inferiorly breached and in direct contact with the mass. The mass displaced the inferior aspect of levator ani laterally and there was evidence of direct extension into muscle on the left side. Staging CT scan did not show any evidence of distant metastases. The patient underwent abdominoperineal excision of the rectum.\nMicroscopy of the CT-guided biopsies of the presacral mass showed fibrous tissue extensively infiltrated by an epithelial neoplasm. The tumour was moderately to poorly differentiated and had a focally cribriform growth pattern. Despite being described as moderately to poorly differentiated, the tumour had a low Ki-67 index with no necrosis or other features of poor prognosis. Immunohistochemical staining of the tumour cells strongly expressed prostatic acid phosphatase and showed weak patchy CD56 positivity. Furthermore, it revealed strong and diffuse positivity for neuroendocrine markers: synaptophysin and chromogranin. The tumour cells also coexpressed the cytokeratin MNF116 and showed patchy AE1/3 positivity.\nMacroscopically, the surgical resection specimen showed a tumour mass measuring 59 × 42 × 27 mm which was attached posteriorly to the rectum in the midline, close to the distal margin. The tumour surface had a grey-white appearance, and yellow discoloured areas were present (). Distally, there were areas of cystic changes within the tumour mass. The tumour involved the muscle of the anal canal but not the mucosa. Microscopic sections of the tumour revealed a homogenous neoplasm that was made up of a uniform population of round cells that had slightly granular nuclei (). The cells were arranged in trabeculae and islands with a prominent vascular connective tissue stroma. In some areas, the tumour intermingled with smooth muscle bundles from around the anal canal. The morphological features suggested a neuroendocrine (carcinoid) tumour (). This was confirmed by immunohistochemistry which showed that neoplastic cells expressed both chromogranin and synaptophysin. The cysts described inferiorly were lined by columnar mucinous epithelium, which strongly expressed cytokeratins 7 and 19 but was negative for cytokeratin 20. There was a subpopulation of neuroendocrine cells within the cyst lining showing expression of chromogranin and synaptophysin and thus confirming the diagnosis of a carcinoid tumour arising from a retrorectal cystic hamartoma (tailgut cyst). There was no cytological evidence of high grade malignancy; however, the tumour did reach circumferential margin at many points. Proximal lymph nodes from the mesorectum showed no evidence of malignancy. A NM MIBG scan also ruled out distant metastasis.
A 64-year-old man who underwent a right upper lobectomy in our institute, involving an extended resection of the posterior chest wall to treat a stage IIIA lung cancer. The dorsal portion of the third to fifth rib was additionally cut during the procedure, and the defect of chest wall was covered using Gore-Tex® Dual Mesh (Japan Gore-Tex Inc., Tokyo, Japan), which is a pure and unique expanded polytetrafluoroethylene (ePTFE) prosthesis, consists of two functionally distinct surfaces [].\nPostoperative acute course was uneventful. However, at postoperative Day 6, he experienced acute severe pain in the right shoulder. The slightly purulent drainage through drainage tube positioned on the mesh was also found. Pus culture from the drain discharge isolated Corynebacterium atrium. Computed tomography (CT) revealed that when the symptoms appeared, abnormal position of the right scapula with the inferior angle of the scapula was caught inside the top of the sixth rib (Fig. ). The conservative treatment failed to improve the scapular dislocation. We performed a redo surgery. We found that all suture threads which attached Mesh to sixth rib were cut, and the dislocation of the right scapula with the inferior angle of the scapula protruding into the right intrathoracic cavity though his thoracotomy defect (Fig. ). We also found a local infectious change at the head side of the Mesh (not the site of scapular dislocation), and found no macroscopic intrathoracic infectious changes. A removal of mesh was carried out. After improving the scapular dislocation and removal of mash, washing with 10 L of physiologic saline was carried out. We did not want to use synthetic materials because of infection. In this case, however, the infection was localized and titanium plate is reported to be resistance to infection [], we performed titanium plate fifth rib fixation to avoid the recurrent dislocation of the scapula (Fig. ). After the redo surgery, continuous lavages with physiologic saline of the thoracic cavity and antibiotic therapies were also performed for 21 days. The repeated culture of the drainage showed no bacterial isolation. After removal of drainage tube, there was no recurrence of infection and scapular dislocation. He had full range of motion of his right shoulder without pain. Patient is now doing well with free from recurrences of cancer, infection and scapular dislocation, 16 months after the redo surgery.
We present the case of a 28-year-old male who suffered a high-energy motorcycle accident. At admission, the patient was conscious, Glasgow coma scale (GCS) 15, hemodynamically stable, and presenting superficial excoriations on the trunk and lower limbs. However, there was a wound of approximately 20 cm on the lateral aspect of the right hip at the level of the greater trochanter, exposing the entire proximal end of the femur (Figure ).\nAfter a clinical evaluation and imaging tests that excluded cranial or abdominal disorders, we prioritized the neurovascular examination of the affected limb, which did not present complications, and the protection of the femoral head with the use of moistened gauze and saline solution. Radiographs in the anteroposterior view of the right hip showed a hip dislocation with a greater trochanter fracture (Figure ).\nAn exhaustive irrigation of the acetabular cavity and the exposed femur was performed, using 10 liters of saline solution at 9% when the patient was in the surgical room. The procedure happened under sedation and spinal anesthesia. A large debridement of muscle, fascia, and bone tissues was required to remove all the devitalized tissue, considered viable only when active bleeding and the clean appearance of the open wound was observed through direct vision by the surgeons.\nThe fractured fragment of the greater trochanter was fixed with two 6.5 mm cancellous screws and washers at the proximal end of the femur (Figure ). After a revision of the debridement sites and radioscopic control of the hip reduction and fixation, the wound was closed (Figure ).\nAfter the first 48 hours of surgery, the wound was releasing a significant amount of secretion, bloody and serum like, and a strong odor was observed, with no laboratory exams indicating infection. At this time, a new surgical procedure (second look) with greater aggressiveness was obtained, removing all devitalized tissue and bad-in-appearance cutaneous cover, which was not necrotic but had an unhealthy appearance (Figure ). A vacuum-assisted closure was used (Figure ).\nThe vacuum-assisted closure was changed every week for four weeks until the appearance of granulation tissue at the surface of the surgical wound (Figure ). During this period, the patient's laboratory exams showed a drop in the hemoglobin level, resulting in a 7.1 g/dl result, which was corrected with a transfusion of 600 ml of red blood cell (RBC) concentrate. As a rehabilitation procedure, we started daily physiotherapy with passive limb mobility and activity, within pain limits, with no load on the right hip.\nIn the fifth week, a new surgical procedure was performed for skin grafting. It was performed with no major occurrences and implantation of the graft was successful. Shortly after the removal of the stitches from the graft surgery, in about eight weeks, partial weight bearing on the affected limb was initiated with the use of two crutches.\nAfter a one-year follow-up, the patient had good mobility of the affected limb (Figure ) without significant pain during mobilization and examination of the joint, with a Harris Hip Score of 93 points. The radiograph showed a decrease of the articular space in the right hip (Figure ) but the magnetic resonance imaging (MRI) showed no necrosis of the femoral head. (Figure ). He was able to ride a bicycle, run, and do squats.
A 42-year-old female patient presented to our orthopedics department with right hip pain which was exacerbated after active movement for 3 hours. There was a history of sprain when she missed a step during walking down the stairs. The physical examinations revealed slight swelling of the affected hip joint, tenderness in the lateral part of the right hip, and limited movement of the hip due to the pain. The AP view of the X-ray showed incomplete fracture of the right femoral neck and the classification was Garden type I (Fig. A). Further computed tomography (CT) and magnetic resonance imaging (MRI) were used to confirm the diagnosis of this type fracture, which showed that the superior cortex of femoral neck was discontinuous and the inferior cortex remained intact (Fig. B, C). The patient was given conservative treatment including additional immobilization devices (cast of right lower limb) of the affected lower limb and strict bed rest during the first 3 weeks after injury. Analgesic was used when necessary during the early stage. Then bed-to-wheelchair transfer training and half-to-full weight bearing mobilization supported by a walker began in the following 6 weeks. In case of the secondary displacement, Routine X-ray of the hip joint was performed for the 1st week after injury. Treatment outcomes in patient were assessed by multiple imaging techniques including X-ray and CT scanning as well as functional recovery Harris hip score (HHS) and visual analog scale (VAS).[ There was no secondary displacement, signal of osteonecrosis of the femoral head or other complications of conservative treatment observed during the 24-month follow-up. Fracture union was achieved at the time of 3-month follow-up. The HHS of this case was improved from 72 to 95 at the time of 3-month follow-up after treatment. The VAS score of the hip pain declined to 0 at the time of 3-month follow-up while the VAS score was 6 at the 1st visit.
Index patient, a 34-year-old female, hailing from a middle socioeconomic status, was brought to the clinic with marked behavioral symptoms. History revealed that about 10 days back, she had an abrupt onset of blurring of vision, with tingling sensation on the left half of her body. This subsequently improved within 12 h after being given benzodiazepines. Prominent behavioral changes followed this. She became exceedingly talkative, started repeating the same phrases many times, and would often ask for food after eating. She was unable to remember the particulars of her son like which class he was studying in. Reportedly, she was unaware of date and day, which started improving after about 7 days of onset of her illness. Biological functions were within physiological limit. She was unable to perform her household activities during the period, though she was taking care of her basic personal care under supervision. There was no history of psychoactive substance use. History of past medical or surgical or psychiatric illness revealed hypertension and occasional headache but without any psychiatric illness. Her medication history revealed she was on oral contraceptive pills and antihypertensive. There was no past history of any mental illness. Family history revealed dementia in her paternal grandfather. On physical examination, no focal neurodeficit was observed. She was cooperative toward the examiner, yet appeared anxious, restless and had stereotyped repetition of certain phrases. Speech output was increased in volume, and she was answering the queries of the examiner in an occasional irrelevant manner. No psychotic symptom was elicited. Judgment and insight was maintained. Her hematological and biochemical profile on the day of visit was unremarkable []. She was started on divalproex sodium 500 mg at night. Considering her acute onset blurring of vision, a neurological opinion was sought. She was advised for neuroimaging and multiplanar magnetic resonance images of brain through T2-weighted and fluid-attenuated inversion recovery sequences revealed acute nonhemorrhagic infarct in bilateral temporo-occipital region (PCA territory) []. At follow-up after 3 weeks, she was still more talkative and irritable, and there had been occasional aggressive outbursts without any new physical complaints. She was complaining of headache at times and was sleeping less than usual.
A 75-year-old man presented with progressive left eyelid swelling that started four weeks previously. His medical history included proximal gastrectomy because of a moderately differentiated gastric adenocarcinoma five years previously. The tumor cells expressed cytoplasmic cytokeratin- 7 (CK7) but not cytokeratin-20 (CK20). The eyelid swelling improved with prednisone administration. However, leg weakness and swelling developed one week prior, and the patient was consequently transferred to the oncology department of our hospital with a suspected spinal or vertebral metastasis. Orbital magnetic resonance imaging (MRI) revealed exophthalmos and a T2-weighted high-signal-intensity lesion accompanying fatty stranding on the preseptal and septal areas. The lesion was mostly enhanced and spread to the extraconal area; the inferior rectus muscle was also thickened without any definite mass. There was no specific finding showing an acute infarct on brain MRI. The overall imaging findings were suggestive of preseptal and septal cellulitis. Therefore, teicoplanin and ceftriaxone were administered because of concern about the potential for clinical complications of orbital cellulitis including visual disturbances and central nervous system manifestations.\nUpon physical examination at our clinic, the patient presented with localized relatively ill-demarcated erythematous swelling and indurations at the left upper and lower eyelid (). Histopathological examination revealed diffuse infiltration with tumor cells that formed strands or clusters between the collagen bundles throughout the upper dermis (). The infiltrating tumor cells were round and polygonal and of variable size. Cytoplasm was eosinophilic, while the nuclei of the tumor cells were pleomorphic with conspicuous nucleoli with mitotic figures. Signet ring cells with intracytoplasmic mucinous vacuoles that exhibited nuclei laterally were also observed. Immunohistochemical staining showed the tumor cells expressed cytoplasmic epithelial membrane antigen (EMA) () and CK7 () but neither CK20 nor carcinoembryonic antigen (CEA). Therefore, a diagnosis of cutaneous metastasis of poorly differentiated gastric adenocarcinoma with signet ring cells was made. The patient's condition deteriorated rapidly, and he died two weeks later.
A 42-year-old man with a past medical history of schizophrenia was found outside a behavioral health facility and appeared groggy with slurring of speech. The patient was given naloxone by emergency medical services without improvement in his symptoms and was transported to the emergency department for further evaluation. On presentation, the patient was afebrile and hemodynamically stable. He was alert and oriented to person, place, and time; however, exhibited slurred speech and an inability to stay awake despite repeated stimulation. Lab work did not show electrolyte abnormalities, leukocytosis, or a deranged thyroid-stimulating hormone level (Table ). Urine toxicology was negative for barbiturates, cannabinoids, cocaine, benzodiazepines, amphetamine, and opioids. His serum ethanol level was negative and his acetaminophen level was less than 5 µg/mL. His urinalysis was not indicative of infection. Clozapine level was noted to be 172 ng/mL (reference range: 350-650 ng/mL). Chest X-ray showed no acute cardiopulmonary abnormality. The patient reported that he was in his usual state of health the prior night when he restarted clozapine after approximately six weeks of medication noncompliance due to the inability to see his psychiatrist for a prescription. He stated that he took 300 mg of clozapine at the full dose the previous evening, and did not restart it with a taper. Medication history indicated that the patient had been prescribed haloperidol 5 mg daily and clozapine 100 mg in the morning and 300 mg in the evening for at least three years. He denied using any opioids or alcohol. He also reported no history of recent infection, shortness of breath, chest pain, or trauma. The following day, the patient was alert and oriented to person, place, and time, and was able to converse normally with no drowsiness or somnolence. He was evaluated by the psychiatry service, and his altered mental status was attributed to the resumption of clozapine at full dose after a period of medication noncompliance. The patient was restarted on clozapine at 25 mg twice daily with plans to increase slowly to therapeutic doses in the outpatient setting.
A 49-year-old female patient was admitted to our department with intermittent dyspnea, palpitation, and dizziness. She was diagnosed with uterine leiomyoma four years ago and she had myomectomy operation because she had not wanted total hysterectomy. Nine months ago she was diagnosed with iliac vein thrombosis and receiving already warfarin sodium treatment. Physical examination was almost normal except bilateral pretibial edema. Chest X ray was normal. On magnetic resonance venography (MRV), there was intravenous mass, which was originated from right internal iliac vein and extended into the inferior vena cava (). After MRV further examinations such as transthoracic/transesophageal echocardiography (TTE/TEE) and thorax MR were planned.\nTTE revealed a mass extending from the inferior vena cava through the right atrium. After TTE we performed TEE and MR. TEE revealed two little membrane-like attachments of the tumor to the right atrial endocardium. Thoracic, abdominal, and pelvic MR showed an intravascular mass, which was concordant with leiomyomatosis. The existing mass was extending from right iliac vein throughout to the right atrium with an obvious venous occlusion (). Oral anticoagulation therapy was discontinued, and low molecular weight heparin therapy was started.\nSurgery was performed through median sternotomy. Beating heart surgery was performed with cardiopulmonary bypass at normothermic range. Cardiopulmonary bypass was established by cannulating the ascending aorta and left femoral vein. Superior vena cava cannulation was performed after pulling out the mass. After right atrial oblique incision, tumor was detached from right atrium. The mass was not attached strongly to the VCI endothelium. During extraction of mass, vena cava superior was clamped. The entire tumor in the IVC and right atrium was extracted from the right atrium (see Supplemental file 1 in the Supplementary Material available online at ). Operative specimen with intracardiac and intracaval components was 25 cm length, 186 gr weight, and grey-white and rubbery color (). Histopathologic assessment was compatible with intravenous leiomyoma.\nThe postoperative course was uneventful. The patient was discharged on the seventh postoperative day without any problem. Before discharge, we performed cardiac MR and there was no residual tumor. Six months after operation, thoracoabdominal MR was reperformed and there was no residual tumor either. However, there was a relative collapse at VCI, which was depending on the extraction of huge mass from intravascular cavity (). The patient's follow-up is still ongoing, and we did not observe any recurrence within the past 14 months.
Patient 4 is a 73-year-old man who was diagnosed with metastatic melanoma to the peritoneum and lungs at the age of 72. At diagnosis he was treated with 13 cycles of atezolizumab (anti-PD-L1) and cobimetinib (MEK inhibitor) as well as radiation therapy to the abdomen. He tolerated these therapies well with no adverse events however his disease continued to progress. Next, he was treated with ipilimumab and nivolumab (Table ). Eleven days after receiving his first cycle, he developed worsening abdominal pain, nausea, vomiting and decreased oral intake. He underwent an upper endoscopy that demonstrated active gastritis and active duodenitis with villous injury. A flexible sigmoidoscopy showed mucosal ulceration with biopsies demonstrating severe active colitis with ulceration. He was then treated with prednisone 60 mg daily with difficulty tapering due to symptom recurrence. Further doses of ipilimumab and nivolumab were held for a total of 2.5 months. Restaging scans were obtained at the time and showed ongoing progression of disease burden in the abdomen. The severity of his irEC and the risk of colonic perforation prompted the decision to initiate concurrent treatment with infliximab. The patient received three more cycles of ipilimumab and nivolumab with infliximab (5 mg/kg every 4 weeks, Table ). He had no further diarrhea, abdominal pain, nausea, vomiting, or other manifestation of irAEs. Follow-up upper endoscopy after two doses of concurrent therapy showed chronic inactive gastritis and a normal duodenal mucosa, and a flexible sigmoidoscopy showed a mucosal ulcer that demonstrated crypt architectural disarray but with improvement from previously seen active colitis. A follow up colonoscopy after completion of a total of three doses of concurrent therapy showed scattered crypt epithelial apoptosis and minimal crypt architectural distortion. Staging scans after completion of a total of 4 cycles of ipilimumab and nivolumab (3 of which were on concurrent therapy) demonstrated interval progression of his metastatic disease burden in the chest, abdomen, and pelvis. He is being considered for surgical debulking to reduce the metastatic disease burden in the abdomen.
A 66-year-old female was admitted to Kagawa University Hospital (Kagawa, Japan) with the complaint of intermittent epigastric pain that was exacerbated by fasting. The patient had a history of hypertension and obstructive sleep apnea syndrome. Physical examination upon admission revealed no anemia (via conjunctival pallor examination), jaundice or pulmonary abnormalities. On palpation, the abdomen of the patient was soft and flat, with no areas of tenderness. Furthermore, pretibial edema was not observed and superficial lymph nodes were not palpable. Serum concentrations of the tumor markers, carcinoembryonic antigen and carbohydrate antigen 19-9, were within the normal ranges (<5 ng/ml and 0–37 U/ml, respectively). However, X-ray examination indicated reduced gastric distension, as well as deformation of the stomach, which exhibited a leather bottle-like appearance (). In addition, the lower gastric body demonstrated luminal narrowing and increased rigidity, with a depressed lesion (longest diameter, 20 mm) at the posterior wall of the gastric antrum and abdominal computed tomography revealed thickening of the antrum. No lymphadenopathy was observed. Additionally, endoscopy revealed an ulcerative lesion covered by a white necrotic substance on the posterior wall of the antrum () and severe luminal narrowing, with poor distension of the lower gastric body. The upper gastric body, however, demonstrated good extension when compared with the middle and lower gastric bodies. The folds of the gastric antrum were flexible, stretched smoothly, and crossed one another, resulting in a waffle-like appearance on the greater curvature of the upper gastric body (). Analysis of biopsy specimens from the ulcerative lesion revealed a poorly differentiated adenocarcinoma containing signet ring cells, however, adenocarinoma was absent from biopsy specimens obtained from the abnormally crossed folds. Due to the morphological changes that occured in the gastric folds, creating the waffle-like appearance, it was determined that cancer cell invasion of the upper gastric body was likely, and a total gastrectomy was performed. The resected specimen revealed the wall thickening and crossing folds of the gastric body () that were previously observed by endoscopy. Microscopic examination revealed that cancer cells had spread throughout the upper gastric body and had infiltrated the vessels in the submucosa, predominately into the muscularis propria, and marginally into the serosa (). Immunohistochemical examination revealed positive staining for MUC5AC and MUC6 (gastric marker mucins) and negative staining for MUC2 and CD10 (intestinal marker mucins), indicating gastric-type mucin expression. The final diagnosis, according to the Japanese Classification of Gastric Carcinoma (), was T4aN3aM0, clinical stage IIIC advanced gastric cancer. The patient was discharged 17 days after surgery without complications and commenced three cycles of S-1 adjuvant chemotherapy (80 mg/day, days 1–28) for 16 weeks. Following three courses of chemotherapy for 16 weeks, treatment was terminated due to patient fatigue. The patient has survived and is without disease recurrance 14 months after surgery.
A 48-year-old right handed male presented to us in the orthopaedics out patient department with chief complaints of deformity in the left forearm and loss of function of left forearm for the last two months. Patient had been earlier operated for bifocal fracture shaft of radius and fracture shaft of ulna by open reduction and internal fixation with square nails 4 years back. According to the patient, the square nail for radius was removed one year after primary surgery because of impingement but no record was available with the patient. Patient, a bus driver by occupation complained of progressive increase in forearm deformity that got aggravated for the last two months prior to seeking consultation in our institution.\nOn examination, there was gross deformity of left forearm with marked wasting and old healed surgical scar marks on dorsum and ulnar border on inspection ( and ). On palpation, there was painless abnormal mobility at all three fracture sites (two in radius and one in ulna). There was no distal neurovascular deficit.\nRadiographs revealed bifocal atrophic non-union of radius with non-union of ulnar shaft fracture with broken square nail for ulna in situ (). Patient was subsequently planned for the surgery after the complete workup. Under regional block, ulna was approached through direct subcutaneous approach. Broken square nail was removed followed by freshening of fracture ends and fixation with 7 Hole 3.5 mm Locking Compression Plate with bone grafting using blocks of cortico-cancellous graft harvested from the iliac crest. Using Thompson’s approach, radial non-unions were exposed, fracture ends at both the non-union sites were freshened followed by fixation with single 10 Hole 3.5 mm Locking Compression Plate after filling the gaps in between fracture ends with blocks of cortico-cancellous bone taken from the iliac crest. Wounds were closed on negative suction drains and above elbow back slab was applied (Figures and ). Patient was closely observed for the signs of compartment syndrome but the post operative period was uneventful.\nAfter two weeks, skin staples and back slab was removed and patient was put on physiotherapy in the form of wrist and elbow range of motion exercises.\nAt 6 weeks follow up, fracture started showing signs ofunion and patient was allowed limited activity. At 12 weeks post operatively, full activities were allowed as fracture had united.\nAt one year follow up, the patient who could not hold anything in his left hand at the time of presenting to us, had resumed his occupation as a bus driver and was now holding steering wheel of bus using same left hand and had good functional and cosmetic results (Figures , and ), with radiographs showing good consolidation of the fractures ().
The patient was a 28-year-old woman, diagnosed with arterial hypertension 2 months before and medicated with lisinopril and an oral contraceptive. She was admitted to the emergency room with a generalized and intense (8/10) abdominal pain associated with nausea, vomiting and a single episode of watery stool. The patient did not report any fever, labial edema, dyspnea or similar.\nIn the objective examination, she was hypertensive (blood pressure of 200/140 mm Hg) without evidence of cardiac dysfunction or fever and her abdomen was distended and painful to superficial and deep palpation. No other changes were found, namely in the oropharynx and in the cardiopulmonary, anorectal and neurological systems.\nThe clinical analysis revealed leukocytosis (20,590/µL) with neutrophils predominance and negative C-reactive protein. There were no other significant abnormalities. The patient was also submitted to an abdominal ultrasound which revealed a small volume of ascites scattered throughout the four quadrants. This was confirmed by an abdominal computed tomography (CT) scan that also showed a parietal thickening of a 20 cm segment in the middle ileum.\nAccording to the initial findings, the main hypothesis was IBD with subocclusive presentation associated with a hypertensive urgency. Therefore, the patient was admitted to an intermediate care unit and treated with a beta-blocker and a calcium channel blocker. At the same time, she stopped taking the ACEI. Given the condition severity and the clinical suspicion, the patient was medicated with antibiotics and corticoids empirically. This unsuccessful strategy was suspended 48 h later, since there was no support for maintaining it: the microbiological and autoimmune studies came back all negative. Thus, other diagnoses were considered, such as intestinal angioedema caused by ACEIs. This hypothesis was also supported by the clinical evolution of the case: fast clinical recovery, continuous apyrexia, no inflammatory syndrome associated, quick resolution of the imagiological findings and negative results for the microbiological studies, serologies, autoimmune parameters and hereditary angioedema.\nThe patient was discharged and has been followed as an outpatient, asymptomatic and without a record of new episodes. She has normal blood pressure, does not take ACEI anymore and the study pending secondary causes for hypertension came back negative.
A 23yr old male presented with cyanosis and easy fatigability from early childhood. He was thinly built, deeply cyanosed [TcO2 saturation 70%] and clubbed. Further examination included a single second heart sound, and a grade 2/6 ejection systolic murmur at the upper left parasternal area. Twelve lead electrocardiography confirmed sinus rhythm, a QRS axis of +120, right ventricular dominance with early transition of the QRS complex in V2. The chest X-ray was usual for TOF, except that there was an indentation of the tracheal shadow on both sides, more prominent on the right ().\nEchocardiography revealed the usual findings of TOF with confluent normal sized pulmonary arteries. Suprasternal short axis view demonstrated two “circles” one on each side of the trachea, suggesting a double aortic arch ().\nTo define the anatomy further, high-resolution CT, and cardiac catheterization were carried out which revealed a double aortic arch, with the right arch being smaller and more cranially situated ().\nTwo neck vessels arose from each arch, and there was no pull-back pressure gradient on either arch. The characteristic features of the TOF with adequately sized pulmonary arteries were shown. Chromosomal analysis excluded a 22q11 deletion. Having reviewed these investigations the patient admitted to direct questioning that he had suffered swallowing difficulties especially for solid foods for many years but attributed this to his cyanotic heart disease. He denied any respiratory symptoms at any time. The surgical plan was to perform single stage correction of the TOF through a midline sternotomy, together with relief of the vascular ring, by division of the minor arch. At operation, the ventricular septal defect (VSD) was closed, the infundibular muscle resected with trans annular enlargement of the right ventricular (RV) outflow, together with insertion of a pulmonary valve prosthesis (23mm Epic valve). As visualization of the aortic arches from the midline approach was difficult because of the size of the ascending aorta, relief of the DAA was deferred.\nFollowing uneventful recovery from this initial procedure, on the 7th post operative day via a right thoracotomy, the minor (right) component of the double arch was ligated and divided distal to the origin of 2nd arch branch. This arch was non dominant, higher, and more posteriorly positioned, clearly compressing the oesophagus. Recovery continued uneventfully. At the six month postoperative review, he was doing well with complete disappearance of the dysphagia. Echocardiography confirmed no residual postoperative intracardiac issues and normal flow in the remaining left arch.
A 79-year-old man was referred to our clinic with a 6-month history of a rapidly growing mass in the left gluteal region. His main complain was pain, radiating down, to the posterior thigh muscles, while in a seated position. He had no previous history of any significant underlying illness, except for gastritis, treated with PPIs and diet. He had also undergone prostatectomy for benign prostatic hyperplasia 2 months before presenting to our clinic. No previous history of trauma in the gluteal or pelvic area was mentioned. At the time of presentation, there was no weight loss, anorexia, fever or other associated symptoms. Physical examination revealed a large, firm, tender mass. Due to rapid growth, the clinical impression was of a malignant neoplasm. Laboratory findings, including lactate dehydrogenase and alkaline phosphatase, were normal. Imaging studies were performed, including pelvic, abdominal and chest computed tomography (CT) and pelvic magnetic resonance imaging (MRI). The CT and MRI examination of the pelvis demonstrated a 7 × 4.4 cm sized soft-tissue mass in the region of left gluteus maximus and gluteus medius muscle. The lesion extended to the surface of the left iliac bone, without infiltrating it (Fig ). The chest and abdominal CT were performed for detecting metastasis prior to surgery and a CT-guided fine needle aspiration (FNA) biopsy of the mass was performed to determine if the tumor was benign or malignant. The CT was negative for metastasis and the FNA result was inconclusive.\nAt surgery, an incision was performed parallel to the gluteus maximus muscle. As the tumor remained deep within the gluteus maximus muscle (Fig. ), a wide excision of a big portion of the underlying gluteus maximus and gluteus medius with a 3–4 cm margin was performed (Figs and ). Exploration of the sciatic nerve revealed that it was not infiltrated by the tumor.\nOn gross examination, the tumor was poorly defined, pale yellow on cut surface with a maximum diameter of 7.6 cm.\nMicroscopically, the tumor was poorly circumscribed and diffusely infiltrating. Neoplastic cells were large, polygonal with eosinophilic granular cytoplasm and small round to ovoid nuclei. They were separated by sclerotic collagenous tissue (Fig. ). Few mitotic figures were noticed. Atypia, spindling, high nuclear to cytoplasmic ratio, pleomorphism or areas of necrosis were not identified.\nImmunohistochemical study was positive for S-100, CD-68, Inhibin-A, Calretinin, CD-57, NSE and negative for AE-1/AE-3 and Desmin. Ki-67 showed positivity in less than 1% of tumor cells. The tumor was diagnosed as morphologically and immunohistochemically consistent with granular cell tumor.\nOn postoperative evaluation, 6 months after surgery, there are no signs of recurrence or distant metastasis. The tumor board recommended that the patient was to be closely followed-up, every 6 months, for the following 2 years.
A 72-year-old woman presented with a large mass in the right frontal scalp and left sided-motor weakness. The mass was progressively enlarged over 4 years, and motor weakness has been developed since 6 months before admission. Scalp tumor was a well-defined, subcutaneous firm mass, and located in the right frontal area adjacent to the midline. The enhanced computed tomography (CT) scans showed the well-enhanced mass, sized 6.2×2.6×5.1 cm in the right frontal scalp, and diffuse growth of intracranial tumor accompanying the hyperostotic change of the skull bone (). Brain magnetic resonance images revealed the well-enhanced tumors with bony infiltration in the right frontal region, diffuse meningeal thickening with multiple cystic changes in the right hemisphere (). Main feeder of tumor was the right external carotid artery, and anterior part of the superior sagittal sinus was compressed by tumor. She underwent a total removal of the scalp tumor and bifrontal craniectomy under impression of extracranial extension of intracranial meningioma. In consideration of the patient's age and tumor location adjacent to the eloquent area, intracranial tumor with thickened dura mater was partially removed, and followed by duroplasty with cranioplasty. The outer table of skull was rough, and tumor was firm and tan-gray color intraoperatively. The tumor was diffusely adhere to the brain surface was observed and it was not possible to undergo total resection without cortical injury.\nHistopathological examination of the tumor specimen demonstrated a mass composed of geographic necrosis, increased cellularity, patternless or sheet like pattern and invasion, mild to moderate pleomorphism. Four or more mitotic cells per 10 high-power fields were seen, but mitotic numbers were not over twenty (). Immunohistochemical staining was positive for vimentin and epithelial membrane antigen, and negative for cytokeratins and S-100 protein. Conclusively, these findings were compatible with an atypical meningioma. Postoperative adjuvant radiotherapy was performed immediately and she received a total dose of 44.8 Gy in 28 fractions for 6 weeks. After adjuvant radiation therapy, she complained of aggravation of motor weakness in the left arm. But, her motor weakness was transient and she was uneventful at discharge.
Our patient is a 24 year old male who was involved in a motor vehicle accident. He was an unrestrained occupant in the front seat of a truck when he sustained the injury. Upon arrival to our hospital, he was tachycardic and tachypneic and was managed accordingly with ATLS protocol.\nHe had severe pain over the pelvis and gross deformity over the the right leg with distal neurovascular structures intact on examination. He had degloving soft tissue injury over the right heel for which debridement was done at the hospital where he was referred from.\nPelvis x-ray and CT scan was taken and showed right side overlapping symphysis pubis associated with ipsilateral transverse acetabular fracture type (). The x-ray of the right leg showed a closed tibial shaft fracture.\nThe patient was unable to maintain his O2 saturation with atmospheric breathing at the emergency room. He was put on intranasal O2 support for a lung contusion injury he had. He had no urinary complaint or blood at the urethral meatus, therefore a Foley catheter was inserted with no need for cystourethrography. He was taken to the operating theater 10 days after the injury. Closed reduction of the locked symphysis was not attempted inorder to avoid further displacment of the ipsilateral acetabular fracture. The patient was positioned supine on the operating table and his pelvis and both legs were prepped and draped free. The symphysis pubis was approached through a Pfannenstiel incision 2 cm above the pubic tubercle with about a 10 cm incision width. The rectus abdominis was found to be partially contused and was further surgically splitted at the midline vertically to retract it laterally. The retropubic space was accessed with blunt dissection to expose the overlapped pubis symphysis and the rami ().\nThen 3.5 cortical screws were applied on the opposing pubic bone bodies with an anterior to posterior direction, approximately 2 cm lateral from the center using them as a joystick to apply a reduction clamp. Using the Jung Bluth clamp on the inserted screws, rotational and disimpacting force was applied and reduction of the symphysis pubis was achieved. After achieving reduction, 3.5 mm size and 6-hole precontoured reconstruction plate was applied on the superior aspect of the symphysis. The plate was secured with three cortical screws on each side.\nFor the percutaneous fixation of the acetabulum, an entry point was identified laterally on the supra-acetabular area with the help of a fluoroscopy image aiming to the symphysis and a stab incision was made for a guide wire insertion. The guide wire was directed from the entry point towards the symphysis using the pelvic inlet and obturator oblique outlet views under fluoroscopy. A 4.5 cannulated drill bit was used to create a path for the screw. Then the transverse column acetabular fracture was fixed percutaneously, using a 6.5 mm size and 100 mm length partially threaded cannulated screw. A post-operative x-ray image was taken the day after surgery ().\nPost-operatively, the patient was stable and followed in the ward with 72 hours of post-operative broad spectrum intravenous antibiotics and adequate analgesics. Prophylactic unfractionated heparin 7,500 IU subcutaneous twice a day was continued according to the hospital’s protocol.\nThe patient was discharged after 5 days of hospital stay, shifting the UFH to oral rivaroxaban 10 mg per day for 1 month and additional adequate analgesics. He had no access for center-based physiotherapy, hence was advised on home physiotherapy with hip and knee passive range of motion and non-weight bearing status of 6 weeks period due to the associated tibial fracture he had. On the 2nd week of his visit to the OPD, the surgical wound was in good condition and the stitches were removed. He was on bilateral axillary crutch support for bed to chair and home mobilization during the first 6 weeks of his post-operation period. At his 2-month follow-up, the patient had no pelvic pain and was able to partially weight bear with the help of a crutch. He had no urinary symptoms or suprapubic pain. At 6-months follow-up he was back to his normal pre-injury activity. Pelvic radiograph showed the hardware in a good position with fracture and dislocations staying reduced ().
Case 3: 34-year-old man, who presented within 3 hours of injury to the right hand, sustained while grinding the sugarcane pulp.\n[: a) Pre-operative image of Injured hand b) Post-operative image of the injured hand c) Image at 4 weeks follow-up]\nThe two of the three patients sustained injuries to the non-dominant hand. The injury characteristically involved four fingers of the hand. The thumb being shorter than all the fingers is rarely involved in the cascade of the injury. All injured fingers had macerations along the margins of the skin, possibly due to the roller blades being wet at the time of injury. There were longitudinal lacerations over the fingers, both on the dorsal and volar sides, with the nail complex involved sparingly. The lacerations, however, were more extensive on the dorsal side. The extensor expansion had vertical slits running along its length till the metacarpophalangeal joints. The proximal interphalangeal joints were exposed with the avulsion of the central slips and loss of continuity of the covering capsules, whereas, the flexor tendons were in continuity with loss of the flexor tendon sheath in the region of the volar skin laceration. The digital nerves and vessels were not damaged, in any of the cases. The bone continuity was lost only amongst two of the three patients. These injuries were managed with debridement under anesthesia. On receiving the patients, wounds were washed and covered with sterile dressings, with broad spectrum antibiotics being started.\nWithin the operation room, the principles ofwound care were followed with an attempt at restoring the bony stability and soft tissue coverage to allow early mobilization of the fingers. Wound debridement and lavage with 6 liters of normal saline was carried out under regional block. The vertical midline slits in the extensor expansion were stitched using interrupted 4-0 nylon sutures. The skin was approximated over tendons to provide them with a biological coverage using sutures i.e. nylon 3-0.\nThe fractures, sustained by case II were fixed using K-wires, whereas, mini fixators were used in case III. Two of these patients required split thickness skin grafting over the dorsum of hand, which was undertaken after the three days of sustaining the injury. The hand was immobilized in using a dorsal plaster slab for pain relief, and the patients were allowed to flex their fingers within the slab. Limb elevation was maintained post-operatively to decrease soft tissue edema. The patients were administered analgesics to reduce the pain and promote active flexion of fingers. The slab was removed in the second week and the K-wires in case 2 were removed after confirming union at three weeks. The patient is assessed for the post-traumatic stiffness, which is clinically apparent in inter-phalyngeal, meta-phalyngeal and wrist joints. The patient undergoes rehabilitation under the supervision of a physiotherapist, where he practices passive, active-assisted and active stretching of the involved joint. The aim is to restore the function to normal or near normal dexterity. The patients were assessed using mini dash score at the end of 6 months. The score was 0 in two patients and 1 patient (case 3) was lost to follow-up.
A 17-year-old adolescent nulligravid girl whose last menstrual period was four days back referred with the diagnosis of low lying myoma after being transfused with four units of blood. She presented to the Gynecology Outpatient Department on 22 July 2018, with history of irregular vaginal bleeding of 2-year duration.\nShe gives history of lower abdominal swelling which was small initially and progressively enlarged to attain the current size for the last 13 months. She is sexually active but not married. She has no history of abdominal pain, urinary, or bowel complaint, has no bleeding from other sites, and has no significant past medical and surgical history noted.\nOn examination, patient was conscious, coherent with blood pressure of 100/60 mmHg, pulse 80 /min, and temperature normal and has pale conjunctiva, cardiovascular, and respiratory systems normal. Abdominal examination revealed 20-week sized nontender, firm, irregular, fixed, and lower border unreachable mass. On vaginal examination, a firm huge nontender growth attached to the anterior vaginal wall. It was difficult to reach at the cervix. A clinical diagnosis of cervical myoma was made.\nHer blood investigations on arrival showed hemoglobin of 9.7 gm/dL and were transfused one unite blood and the hemoglobin elevated to 10.7 gm/dL. Ultrasonography showed empty uterus with normal size echotexture and pushed up in to the abdomen. There is 10 by 12 cm hypoechoic mass arising from the cervix filling the vaginal canal with these the diagnosis of cervical myoma was made. The diagnosis necessitated Computed Tomography (CT) scan of the abdomen and pelvis which helped to know the extent of the mass and reported hypodense contrast enhancing mass seen on the uterus arising from the cervical region measures about 10.3 cm by 14.4 cm. The mass grows down in to the vagina and concluded with the diagnosis of contrast enhancing cervical mass likely myoma ().\nUnderstanding the complexity of her surgery and possible postoperative morbidity and mortality, surgical, anesthesiology, nursing, and recovery room teams was assembled with gynecologic oncology team. She was prepared for elective laparotomy the day before the surgery. On 18 August 2018, the patient was taken to operating room, after the general anesthesia given; she was positioned in a supine position. The abdomen cleaned with povidone iodine and draped with sterile towels and midline vertical incision was made. Intraoperatively, bilateral fallopian tubes and ovaries, the uterus, and urinary bladder grossly look normal; there is 10 cm by 15 cm sized firm mass between the vagina and the lower uterine segment (). Vesicouterine peritoneum incised the bladder reflected away from the lower uterine segment and upper vaginal wall, about 4 cm vertical incision was made on proximal anterior vaginal wall, and dissection of the mass away from the anterior vaginal wall was tried but it was difficult to have a clear cleavage line to excise the whole mass. Then another 6 cm longitudinal incision was made on the posterior vaginal wall; sharp and blunt dissection were made to separate the bulk of mass away from the vaginal wall; finally excision of the mass from the base within 1 cm of normal vaginal tissue was performed to carefully remove a 15 × 10 × 7 cm encapsulated mass (). The anterior vaginal wall, extension of incision on the cervix, and posterior vaginal wall closed separately with vicryl number 0 in two layers (). Hemostasis was secured, correct count was reported, the fascia and skin were closed using delayed absorbable stitch. The surgery was completed after 2hrs and 20 minutes.\nThe excised mass was sent for histopathological examination; the patient recovered completely and discharged on 6th postoperative day. The final pathological diagnosis of the mass was vaginal angiofibroma ().
After a multidisciplinary evaluation, at the end of November 2019, a 13-year-old girl attended the Pain Therapy Clinic of the Ospedale Pediatrico Bambino Gesù in Rome, where acupuncture is also practiced as an analgesic technique. She reported pain in the left wrist and hand, with intensity 10 on the Numeric Pain Rating Scale (NRS), pulsating, and always present, thus preventing any movement. Marked hypersensitivity and allodynia were present at the level of the left fingers, wrist and hand. Pain was present in both flexion and extension of the wrist and caused a marked reduction in strength in the left wrist and hand, making a handshake impossible for the girl. There was no redness but swelling and sweating at the level of the fingers of the left hand. The pain was so intense that it interfered with her regular attendance at school, generating social withdrawal phenomena, which are unfortunately very frequent in patients with chronic pain.\nPain began three months before the consultation, after an accidental fall with trauma to the left wrist. It gradually increased and did not respond to either NSAIDs or limb immobilization. The diagnostic tests performed at the time (X-ray, Doppler ultrasound and magnetic resonance imaging) were negative, as were the blood chemistry tests ( and ).\nFrom the age of 8, the girl was followed by a pediatrician in our hospital for a history of cramps and pain in the lower limbs, especially in the ankle and left knee, both in the absence of trauma or caused by frequent falls. In the following years, clinicians found bilateral flatfoot (with subsequent surgery), mild ligamentous hyperlaxity and vitamin D deficiency. In addition, borderline cognitive level with motor coordination disorder and executive function deficit, as well as stuttering, were then diagnosed. Elements of anxiety emerged from the administration of Self Administered Psychiatric Scales for Children and Adolescents (SAFA-A, D and S) questionnaires, as highlighted in particular by the subscales “Separation anxiety” and “Generalized anxiety” [,]. Concern for one’s own health was observed, with experiences of herself as being ill; the scores indicate a propensity to somatize. There was a tone of mood oriented in a deflected sense, and insecurity. The results of the Lie scale were: 8; T: 65 [,]. In addition to pulsating headache with phono and photophobia, frontal epilepsy was also diagnosed, which could explain the frequent falls, and moreover had excluded hyperbaric oxygen therapy as a feasible regimen in this case (due to a cost/benefit evaluation, and increased exposure to oxygen toxicity during the treatment itself). Episodes of dizziness with difficulty in maintaining an upright position lasting a few hours were also observed, and on two occasions she also had an episode of unconsciousness lasting about 2 min.\nDuring the first visit, as a consequence of the mother’s need to have time to convince the recalcitrant daughter, in the meantime it was recommended to administer oral tramadol (100 mg/mL), 5 gtt in the morning and 5 gtt in order to reduce musculoskeletal pain. The reason for choosing a drug such as tramadol rather than any other pharmacological option lies in the fact that the pain was so intense that it affected the patient’s relationships []. We therefore opted for a drug that would have an immediate effect, so as to be able to undertake the acupuncture course. Subsequently, in the first two sessions, after careful disinfection of the skin with 2% chlorhexidine, and using the appropriate needles for length and diameter based on the type of acupuncture and the selected points, we used the following acupoints: TE 4 (Yang Pool), TE 5 (Waiguan), LI 5 (Yang Xi) and SI 4 (Wan Gu), all on the left side. These points were chosen on the basis of a pathology which, according to traditional Chinese medicine, was caused by cold wind. These points produced heat and dissipated the wind []. The needles were kept for 30′, with stimulation every 10′. In the third session, the girl reported a slight improvement in the painful symptoms but at the same time unbearable pain in the affected limb during the previous sessions due to the insertion and maintenance of the needles in the affected area. We decided to change strategy and to use abdominal acupuncture and stimulation of the points CV 4 (Guan Yuan), CV 12 (Zhong Wan), CV 16 (Zhong Ting), CV 17 (Shan Zong), ST 24 bilateral (Huaroumen) and KI 17 bilateral (Shang Qu), with appropriate needles and maintaining the same interspeed and time of stimulation. The needles were inserted to a depth of 0.2 cun. These new points (CV 4, CV 12, CV 16, CV 17 and KI 17), in addition to heating and dissipating the wind, re-established the correct circulation of qi []. In fact, used in combination, their purpose was to move the qi from the kidney to the extremity of the upper limb passing through the shoulder [,]. ST 24 was used to calm the patient and increase her compliance with acupuncture [].\nAuriculotherapy was also associated with Vaccaria seeds on the Wrist, Hand and Shenmen points, with the recommendation to stimulate them at home for 10 min, 4 times a day until the next session. Vaccaria seeds are used to stimulate certain points in auriculotherapy due to their almost spherical shape, and the absence of pharmacological properties []. The Shenmen point was chosen for its anxiolytic effect, given the patient’s psychological difficulties [].\nAfter eight sessions (two months) of abdominal acupuncture the pain completely disappeared (Numeric Pain Rating Scale value 0) and the girl regained full functional capacity of the arm and a normal life. Follow-up at three months, six months and one year demonstrated complete remission of symptoms, with constant values of NRS equal to 0. The reduction in pain, and subsequently its total disappearance, allowed the patient to resume normal school attendance, and therefore to resume a life of normal, balanced relationships.
A 56-year-old previously healthy man with heavy smoking history and sedentary lifestyle presented to the emergency department with acute paraplegia while he was attempting to lift a truck tire weighted about 75 kg off the ground. Accompanying symptoms included urinary retention, severe burning sensation in the low back with radiation to the entire back and anterior torso, and decreased sensation over both legs and the genital region. Physical examination revealed paraplegia, decreased sensation to both pinprick and light touch distal to the T10 dermatome, absent deep tendon reflexes of both lower limbs, and bilaterally non-palpable dorsalis pedis artery pulsations. Proprioception and vibratory sensation were normal. The anal tone was normal, and the deep anal pressure sensation, anocutaneous reflex, bulbocavernosus reflex, and voluntary anal contraction were all present. Detailed neurologic examination according to the American Spinal Injury Association Impairment scale (AIS) is summarized in Table . The patient was classified as T10 AIS C incomplete spinal cord injury. Computed tomographic (CT) angiography of the aorta for possible abdominal aortic dissection or ruptured aneurysm showed aortoiliac occlusive disease distal to the subphrenic aorta with prominent collaterals (Fig. ). Magnetic resonance imaging (MRI) of the spine (Fig. ) for suspected myelopathy revealed symmetric hyperintensities in the anterior part of spinal cord at the T9 to T12 level on T2-weighted images and diffusion-weighted images, indicative of acute spinal cord infarct. For aortoiliac occlusive disease with spinal cord infarction, intravenous hydration and anticoagulation with heparin were instituted. The patient opted for medical management for aortoiliac occlusive disease after discussion with vascular surgeon considering the high operative risk, prominent collaterals without imaging evidence of compromised perfusion of visceral organs on CT angiography, and the lack of signs of ischemia in the legs such as chronic ulcer. Anticoagulation was later bridged to warfarin, with the international normalized ratio maintained between 2 and 3. For study of aortoiliac occlusive disease, blood tests were reviewed and showed polycythemia, hemoglobin level 19.6 g/dL, hematocrit level 53.5%, and decreased erythropoietin level. The diagnosis of PV was made after the patient was tested positive for Janus kinase 2 V617F mutation. Exhaustive workup for vasculitis, autoimmune, and coagulation disorders were all negative. Phlebotomy and cytoreduction therapy with hydroxyurea were instituted for high-risk PV, targeting a hematocrit level of <45%. Detrusor hypoactivity and increased urethral pressure were disclosed on urodynamic examination for evaluation of neurogenic bladder, which was then managed with clean intermittent catheterization and medications including terazosin and bethanechol. The patient became catheter-free 5 months after disease onset. Neurological condition and function improved after inpatient and outpatient rehabilitation. Fourteen months after disease onset, the patient could ambulate with a walker. Muscle power by the Medical Research Council Muscle scale improved to grade 4 in the right leg and 3+ in the left leg. The AIS also changed from C to D. No recurrent thrombotic event or deterioration of neurologic function developed during the 14-month period of follow-up. The clinical course is summarized in a timeline (see Table, Supplemental Content which summarizes the clinical course).\nInformed consent was obtained from the patient. Ethical approval was not necessary for this case report under the regulations of institutional review board of the National Cheng Kung University Hospital.
A 45-year-old man was referred to the department of Oral Medicine of the Isfahan University of Medical Sciences, Isfahan, Iran. He was complaining of exophytic painless mass in dorsal surface of his tongue. He expressed that the lesion had appeared 2 years ago and lasting ever since. The mass had negligible growth rate when he first noticed, but had grown rapidly over the past 2 months to attain the present size. The patient's chief complaint was his fear of speaking because it might make the relatives shocked and wonder and also his phobia of transmission to others or whether it might have a cancerous nature.\nMedical history of the patient showed history of epilepsy begining at age 10, taking anti-seizure medications since childhood, and his dental history showed multiple chronic oral ulcers diagnosed as drug-induced lichenoid reaction which were presumed to be related to anti-seizure medications. The list of medications included phenytoin sodium (300 mg/d) and carbamazepine (600 mg/d). It seemed that the patient was of a low socioeconomic status and had not followed his treatment and was taking his medicaments irregularly. Evaluating his laboratory tests revealed anemia, leucopenia, transient thrombocytopenia, folic acid deficiency, and increased liver enzymes. In the study of peripheral blood smear and bone marrow aspiration, mild relative erythroid hyperplasia was diagnosed for him. Symptoms of hirsutism due to phenytoin were evident in his head and face. Despite of long-term phenytoin consumption, there was no gingival hyperplasia.\nClinical examination revealed a reddish-yellow exophytic, pedunculated mass which centered on the dorsal surface of tongue, measuring 4 × 3 × 1 cm3 in size. The lesion was lobulated with a smooth surface. The base of the lesion was so tightly stuck to the bed that the interface was always slightly bloody []. Neither any traumatic factor in the oral cavity, nor palpable cervical lymph nodes were detected. The diagnostic hypotheses were hemangioma, angiosarcoma, inflammatory hyperplasia, and PG.\nAn excisional biopsy of one of the masses was taken. Hematoxylin-eosin stained section showed [] hyperplastic stratified squamous parakeratinized epithelium covered with a serofibrinous membrane. The underlying fibrovascular stroma showed a large number of budding capillaries, plump fibroblasts, and areas of extravasated blood and a dense acute and chronic inflammatory cell infiltrate. These findings were consistent with a histopathological diagnosis of PG.\nAlthough the lesion had been removed excisionally, it relapsed in 3 months. At first, we referred the patient to a hematologist for evaluating the problems of his medical history. According to the hematologist, his main blood problems and increased hepatic enzymes were side effects of his medications. We searched for the side effects of his medications and found PG as a possible carbamazepine side effect in very rare cases.[] Then, he was referred to a neurologist for changing of his medications. Finally, Gabapentine took the place of carbamazepine.\nRegarding the recurrence of the lesion, we preferred intralesional injection of corticosteroids. A solution was prepared by diluting 0.1 ml of triamcinolone 40 mg/ml with 0.5 ml of 0.5% lidocaine, and 0.1 ml of the mixture was injected into the lesion. The injections were given weekly for 16 weeks. Local antifungal treatment was prescribed simultaneously to prevent fungal superinfection, Significant improvement of the lesion at each visit was noted. At 20 week follow up, the lesion was 90% resolved, with some residual erythema and swelling []. The residual lesion was removed by cryosurgery. The patient was monitored for a year and no relapse was noted [].
A 67-year-old Caucasian woman presented to her primary care physician with a 1-year history of epigastric abdominal pain, occasional nausea, and emesis, and an unintentional 10 pound weight loss. An abdominal CT scan showed a 4.5 × 4.1 cm periampullary mass arising from the pancreas without evidence of metastatic disease. Interestingly, the CT scan also showed the colon to be in a dorsal position in relation to the SMA (). Laboratory analysis revealed normal liver function tests and serum CEA level but an elevated CA 19-9 level. At operative exploration, the entire transverse colon was found to be retroperitoneal and lies dorsal to the SMA and SMV. The duodenum coursed anterior rather than posterior to the mesenteric vessels. The SMA lies further to the right than normal and the SMV–portal vein confluence lies further to the left than usual. Inspection of the duodenum and head of the pancreas revealed a firm mass in the head of the pancreas. A classic pancreaticoduodenectomy was performed. The anterior placement of the duodenum made division of the jejunum beyond the ligament of Treitz relatively easy. The tumor dissected nicely away from the SMA with no uncinate process of the pancreas present. Our standard procedure is to bring the jejunal limb for reconstruction up in a retrocolic position, through a defect made in the transverse mesocolon to the right of the middle colic vessels. This limb is used to create the pancreaticojejunostomy, hepaticojejunostomy, and gastrojejunostomy. Due to the retroperitoneal position of the transverse colon, we brought the jejunal limb up in an antecolic position. Pathology revealed a moderately differentiated PDA with all surgical margins being free of tumor and 1 out of 11 specimen lymph nodes was positive for metastatic disease. The patient tolerated the procedure well and was discharged to home on postoperative day 5. The patient eventually developed recurrent disease and died 34 months postresection.
A 4-month-old male infant, after an uncomplicated pregnancy and delivery, weighed 3000 g at birth; there were no complications during pregnancy and no family history. The infant had symptoms of mild respiratory distress for 2 months, without fever or cough. He was diagnosed with patent foramen ovale by echocardiography at the primary hospital and received no treatments. Later, the symptoms were aggravated, and his parents sought care, again at the primary hospital, 9 days later. Ultrasonography of the abdomen showed features suggestive of hemangioendothelioma of liver. The hospital suggested that to the infant should be transferred to our hospital for treatment.\nExamination revealed an irritable infant with cough, fever, breathlessness and cyanosis; the infant weighed 6.8 kg and was in respiratory distress with a relative ratio of approximately 130/min. The infant had multiple skin hemangiomas of varying sizes on the head, neck, right index finger, and right shank. His physical examination was remarkable for bilateral wheezy phlegm in both lung bases. The physical examination showed enlarged cardiac dullness, but there were no significant heart murmurs. Abdominal distention was noted. The liver was soft and located 8 cm below the costal margin at the right mid-clavicular line.\nEchocardiography showed right atrial and right ventricular dilatation with a thickened right ventricular anterior wall and interventricular septum. The normal structure of the cardiac chamber and continuous atrioventricular septum were observed. The infant was diagnosed as having severe pulmonary artery hypertension with mild tricuspid regurgitation and trivial mitral regurgitation. Ultrasonography of the abdomen showed densely hypodense lesions, increased volume of liver lobes, with a clear boundary and a maximum diameter of 4.1 cm. The liver lobes had a heterogeneous internal echo and enhanced peripheral echo. Visible blood vessels were observed in the hypodense lesions. There were only a few normal liver parenchymas.\nA computed tomography (CT) scan of the abdomen showed an enlarged, irregularly shaped liver with multiple low-density intrahepatic masses, and its CT value was approximately 43 HU. Contrast-enhanced abdomen CT showed significant enhancement in the periphery of tumor, uneven enhancement in the center part of tumor during the arterial phase, centripetal enhancement in portal vein phase and even enhancement in late phase of tumor, which was more enhanced than normal liver. The lesions were more uniformly strengthened, and their degree of strengthening was higher than that of normal liver tissue in the delayed period (Fig. ).\nThe laboratory examinations showed normal values for creatine kinase, creatine kinase-MB, alanine aminotransferase, aspartate aminotransferase, unconjugated bilirubin, serum total bilirubin, conjugated bilirubin, creatinine, serum urea, and coagulant activity. However, thyroid-stimulating hormone (TSH) was 19.5 uIU/mL (NL = 1.36–8.8 uIU/mL), free triiodothyronine (fT3) was 3.21 pmol/L (NL = 4.5–10.5 pmol/L), and free thyroxine was normal. Meanwhile, alpha-fetoprotein was 15434.76 Ug/L (NL = 0–25 Ug/L).\nAfter we discussed the patient's condition, the infant was diagnosed with hepatomegaly, pulmonary artery hypertension, cardiac insufficiency, pneumonia, hepatic hemangioendothelioma, hypothyroidism, and multiple hemangiomas. Then, PAH and cardiac insufficiency were managed with fluid restriction, oxygen, diuretics, and inotropic support. In addition, the pneumonia was managed with antibiotics. Because fT3 was mildly decreased, we suggested that the infant should be regularly seen for endocrinology consultation. Meanwhile, propranolol was given at a dosage of 1 mg/kg every 12 hours.\nEight days after admission, the patient underwent transcatheter arterial embolization under general anesthesia. Unfortunately, because the diagnosis was relatively clear and the condition of the infant was worsening, his parents agreed to allow the patient to undergo TAE and refused a digital subtraction angiography-guided percutaneous biopsy after we informed them of the clinical risk. In addition, the TAE was approved by the ethics committee of Ji’nan Children's Hospital. The perineum was disinfected and draped with the patient in the supine position. The femoral artery was punctured by the Seldinger technique, and 100 IU/kg of heparin was administered to avoid thrombosis. The celiac artery was catheterized with a 4-F PIG angiographic catheter (Cordis, MI) under X-ray guidance. We observed that the blood supply to the IHH was derived from the proper hepatic artery branches, including the left hepatic artery and right hepatic artery, by digital subtraction angiography. The celiac artery was catheterized again with a 4-F Cobra guide catheter (Terumo, Tokyo, Japan) under X-ray guidance. Then, the 2.6 F microcatheter (Asahi, Nagoya, Japan) was used for superselective catheterization of the feeding artery. During the procedure, a pingyangmycin-lipiodol emulsion was injected slowly through the catheter until the periphery of the hemangioma was completely surrounded. Gelatin sponge particle (350–560 μm) embolization of the feeding artery was performed if the blood supply artery was faster, as shown by angiography. Selective celiac arteriography was performed once again if necessary to judge the degree of embolism of the supplying arteries. The injection was stopped when a small branch of the portal vein around the tumor was developed or the total volume was administered. At the conclusion of the embolization, the microcatheter was withdrawn, and the sheath was removed. Hemostasis of the femoral artery was then achieved by manual compression for 10 to 15 minutes.\nPostoperatively, meticulous nursing care was given to the patient, and symptomatic relief and supportive treatment were continued. Thirty-five days after admission, the PAH decreased, the symptoms of cardiac insufficiency were alleviated, and the severe pneumonia was cured. On the third month after the TAE, the abdominal distention was alleviated. The liver was soft and located 2 cm below the costal margin at the right mid-clavicular line. A CT scan of the abdomen still showed an irregularly shaped liver with multiple significantly strengthened intrahepatic masses, and the size of the largest mass was 1.6 cm. Echocardiography showed normal pulmonary artery pressure and EF. The patient underwent a second TAE under general anesthesia. There were no serious complications after the operation. At the 1-year follow-up, the infant had steadily gained weight, the liver volume and size of the mass decreased considerably (Fig. ), and the hypothyroidism returned to normal.
A 33-year old, previously fit and well, Caucasian woman presented to a UK acute medical unit with 2 days of gradual onset pressure-like headache, fever, neck stiffness and vomiting. There was no travel history of note or recent ill contacts. On examination, she was febrile at 38.6 °C and had photophobia with nuchal rigidity, however with a normal conscious level, and unremarkable systemic and neurological examinations. Initial tests included a normal serum white cell count and a C-reactive protein of < 1 mg/L, normal creatinine and electrolyte levels, and a normal chest radiograph. A Computed Tomography (CT) of her brain was reported as normal with no masses, haemorrhage or hydrocephalus. Cerebrospinal fluid (CSF) analysis revealed an elevated white cell count of 172 × 106/L, with complete lymphocytosis and elevated CSF pressure of 32 cm H20 (Table ). A working diagnosis of viral meningitis was made. Following a lumbar puncture, she was treated empirically with intravenous acyclovir 10 mg/kg three times a day pending her CSF virology polymerase chain reaction (PCR) screen result which came back negative after 3 days at which point the acyclovir was stopped (Table ). She also received paracetamol as an analgesic and antipyretic but did not receive steroids. Her headache improved, she was afebrile for 48 h and she was discharged home after 5 days.\nA week later, the clinical phenotype had evolved considerably from that of pure meningitis, to meningoencephalitis. On representing to the Emergency Department, she had additional clinical features of confusion with impaired concentration, memory deficits and mild dysarthria. She remained febrile. She rapidly became increasingly agitated, and developed visual hallucinations and a generalised tonic-clonic seizure. Over 5 days, she developed a reduced conscious level alternating with agitation and mutism. She was intubated and ventilated, and treated empirically with intravenous ceftriaxone 2 g a day and acyclovir 10 mg/kg three times a day for presumed meningoencephalitis. She was subsequently transferred to our hospital for further investigations and care. During this period, despite ongoing antimicrobial therapy for a week, she had ongoing pyrexia and a persistently reduced Glasgow Coma Scale (GCS) requiring critical care support. She later developed marked autonomic instability, along with new orofacial and upper limb dyskinetic movements.\nShe underwent detailed investigations for infective and autoimmune causes of encephalitis and meningitis. Her Magnetic Resonance Imaging (MRI) brain with contrast showed a few tiny non-specific supratentorial white matter T2-weighted high signal foci which were not thought to be of clinical relevance. Otherwise the appearance of the brain parenchyma was normal with no abnormal enhancement post-contrast. A summary of her serum and CSF investigations is shown in Table . CSF repeatedly demonstrated marked lymphocytosis with elevated protein levels, along with an elevated opening pressure on one occasion and a reduced CSF/serum glucose ratio on two occasions. Investigations for a number of infections were negative. An electroencephalogram showed generalized brain wave slowing indicative of diffuse cerebral dysfunction. Serum live cell-based assay for NMDA-receptor (NMDAR) antibodies from day 14 of illness was positive. CT of the pelvis demonstrated a left ovarian lesion. Subsequent excision confirmed a stage 1A G1 immature ovarian teratoma on histopathological examination. A diagnosis of anti-NMDAR encephalitis related to ovarian teratoma was made. Due to a sampling error, a CSF NMDAR antibody result was not available.\nShe was treated with high-dose intravenous methylprednisolone 1 g a day for 5 days on day 28 of illness followed by five cycles of plasma exchange, and subsequently started on prednisolone at a dose of 60 mg once daily via a nasogastric tube. She was extubated after 3 weeks and she continued to demonstrate improvement in her concentration, memory and language ability. After leaving intensive care, neuropsychiatric assessment documented executive dysfunction with concrete thinking and anxiety, however these rapidly improved. She underwent a left salpingo-oophorectomy 2 months following her initial presentation. She was discharged from hospital after 3 months at which point she was independent in her mobility and self-care. Steroids were slowly reduced and stopped over 3 months as an outpatient. Further immunotherapy was not required. Because of the clear clinical diagnosis, supported by immunological assay and clinical improvement, repeat CSF tests were not clinically indicated.\nOverall, she made an excellent recovery. She has remained well and returned to work as a receptionist. Further assessments revealed only subclinical executive dysfunction with no major impact on her activities of daily living. Serial gynaecological oncology reviews confirmed complete excision of teratoma and absence of contralateral disease. Oncological prognosis is considered to be good. We consider the likelihood of recurrence of encephalitis to be low.
Our patient is a 62-year-old woman with a history of anemia, deep vein thrombosis, anxiety, and Devic's disease. After an initial misdiagnosis of MS, she was found to have Devic's disease when she tested positive for the NMO-IgG biomarker in her serum. She has required plasmapheresis as her primary treatment for her Devic's disease due to the failure of corticosteroid treatment. She received plasmapheresis via a left arm brachial-axillary PTFE AVG that has been in place for 10 years and used without incident.\nIn May 2017, the patient developed acute thrombosis of her AVG. Thrombolysis was completed, but the graft clotted off again within 1 week. She was seen and evaluated in our clinic for placement of a new dialysis access in June 2017, and we elected to create a new right arm AVG using an immediately cannulable graft (Acuseal, W.L. Gore & Associates, Flagstaff, AZ, USA) []. This was placed in a brachial-axillary configuration with the brachial artery connected to the axillary vein via a trilayer PTFE graft. There were no issues during the operation, and Doppler ultrasound confirmed a widely patent graft at the conclusion of the case. The patient was able to use this graft for plasmapheresis immediately after surgery.\nThe patient presented to our clinic 2 weeks after surgery for a routine postoperative visit. During this visit, it was noted that the graft was thrombosed, and there was now an absence of a radial and ulnar pulse. The patient was asymptomatic, and thus elective open thrombectomy of the graft was scheduled. This procedure was completed in late June 2017; during this procedure, we were unable to pass the Fogarty thrombectomy catheter past either the arterial or venous anastomoses. Open exploration of both anastomotic sites was then performed and severe myointimal fibrosis was noted within both the brachial artery and the axillary vein. While the axillary vein was completely obliterated by this reaction, a portion of the brachial artery remained patent. A patch angioplasty was completed using a native vein, and pulsatile flow to the radial and ulnar arteries was obtained. The graft was excised as it was no longer functional.\nAn open exploration of her prior left arm AVG was completed to determine whether this graft could be utilized for dialysis. A similar myointimal fibrotic reaction was seen here as well, affecting both the left brachial artery and axillary vein. A portion of this tissue was sent to pathology for evaluation, which revealed the presence of a myointimal fibrotic reaction. A temporary dialysis access was then obtained. The patient was started on a 5-day steroid taper during this period, and the patch angioplasty of the brachial artery remained patent 3 months after the procedure.\nThe patient was brought back to the operating room 2 weeks later for the establishment of a new left arm brachiocephalic arteriovenous fistula. This fistula matured successfully and is now being used for hemodialysis without any issues.
A 38-year-old married female without any past or family history of psychiatric or neurological illness and with good premorbid functioning. She was harboring delusion of infidelity against her husband along with disturbed socio-occupational and biological functioning for the past 6 years. There was no history of substance use disorders, except excessive caffeine use, or any high risk sexual behavior. Organic psychiatric disorder or symptoms of schizophrenia were not evident. Detailed general physical examination including neurological evaluation did not reveal any abnormality except mild pallor. She reported regular menstruation and the last childbirth was 10 years back. Mental status examination revealed that she was having an uncooperative and aggressive attitude, delusion of infidelity, impaired judgment, and lack of insight. So, she was admitted to psychiatry ward with a diagnosis of persistent delusional disorder as per International Classification of Diseases, 10th edition. She was started on intramuscular olanzapine 10 mg/day. On investigation, her complete hemogram revealed a Hb of 11.5 gms/dL and a serum ferritin level of 30.2 ngm/ml (normal value for females is 10-291 ng/mL). Other routine hematological and biochemical parameters including blood sugar, renal, liver, and thyroid function were within normal limits. We also planned a vitamin B12 and folic acid estimation which could not be done due to lack of resources.\nWithin 4-5 days of starting olanzapine, there was a decrease in her aggression and she became cooperative for interviewing. However, she would report that she was having sleeping difficulty due to an itching sensation in both upper and lower limbs at night. She would rub her legs against bedclothes, tie pieces of cloth around her limbs, and ask for limb massage to get relief from such a discomfort. She would have such symptoms for hours after lying down at night. During daytime, she would, however, feel comfortable, relaxed, and was able to sleep. She was evaluated for sleep disorder-before this she never had any sleep complaint, neither was there any family history of sleep disorder. During sleep, there was no history of snoring, no abnormal limb or breathing movements. Ear, nose, throat examination was also noncontributory. She was having a thin built and a body mass index of 15.56. As she became cooperative, she was shifted to oral psychotropics (olanzapine 15 mg/day + clonazepam 1 mg at bedtime). Also oral iron supplementation in the form of ferrous fumarate 350 mg, vitamin B12, and ascorbic acid was started. To improve her sleep, general sleep hygiene practices were initiated, she was prohibited from taking any caffeine beverages at night and the clonazepam was further increased up to 2 mg at bedtime. But there was no improvement in her nighttime symptoms.\nIn view of her above symptoms and investigations, she was diagnosed to have RLS with an International Restless Legs Scale Score (IRLS) of 24 indicating high severity and olanzapine was considered to be the probable cause. Hence, the olanzapine was decreased to 10 mg/day and oral risperidone 2 mg was added at bedtime. On the next day, the severity of symptoms decreased to a RLS score of 12. No other change in medication was done during this period. So, after 2 days, olanzapine was stopped and risperidone was increased to 6 mg at bedtime. Thereafter, her troublesome symptoms ceased completely. Though she still harbored the delusions yet the conviction decreased and she became more cooperative with her husband and the treating clinician. Then after about a week of uneventful ward stay, she was discharged free of her sleep-related difficulties on risperidone 6 mg and clonazepam 1 mg at bedtime with the plan to continue her antipsychotic drugs and iron supplementation on an outpatient basis.
A 58-year-old female presented with a 3 cm mass in the lower inner quadrant of the left breast. Ultrasound and mammography revealed a Breast Imaging Reporting and Data System [] (BI-RADS) 4 lesion. Core biopsy of the lesion showed a grade 2 invasive ductal adenocarcinoma. The patient had wide local excision of the lesion along with axillary lymph node dissection.\nPathological examination revealed a T2 N0 M0 grade 1 invasive ductal carcinoma with 14 examined lymph nodes showing no evidence of metastases. The patient subsequently refused adjuvant radiation and chemotherapy.\nFive years after her surgery, the patient presented with a painless, mobile, 15 cm mass involving the upper and lower outer quadrants of the left breast. No evidence of nipple retraction or discharge was observed. Mammography revealed a BI-RADS 5 lesion. A core biopsy showed breast tissue containing areas of bone formation with partially calcified osteoid material surrounded by stellate and spindle-shaped stromal cells. There was also osteoclast-like giant cells present.\nContrast-enhanced computed tomography (CT) of the chest and abdomen revealed a large lobulated left breast mass with cystic and calcific foci, measuring 7.1 × 10.7 × 11.6 cm with no evidence of invasion into the chest wall or skin. No pulmonary, hepatic, or bony lesions were identified.\nThe patient refused mastectomy and chose to have a wide local excision of the breast mass (see ).\nPathologic examination of the mass showed osteogenic sarcoma with malignant cells admixed with neoplastic woven bone and frequent mitotic figures (see ). No evidence of infiltrating ductal carcinoma or ductal carcinoma in situ was observed. The tumour was noted to involve all margins. A completion mastectomy was subsequently performed, which showed no evidence of residual disease on pathological examination.\nA technetium-99 methylene diphosphonate bone scan was undertaken, and no evidence of primary osteosarcoma arising from bone was detected, indicating that the breast lesion was primary osteosarcoma.
A 24-year-old married Arab woman had been admitted to a local health center 2 months prior to referral to our urology department. She had been hospitalized there four times in 1 year for acute pyelonephritis. The fourth episode raised the suspicion for an underlying problem and justified her referral to our urology department after management of the acute pyelonephritis.\nOn admission, she complained of ascending left-sided flank pain during micturition but did not have dysuria or hematuria. She also had a history of frequent urinary tract infections (UTIs) as a young adult.\nShe was perfectly asymptomatic on the right side. A physical examination was normal. Her temperature was 37.4 °C, her blood pressure was 128/84 mmHg, and her pulse rate was regular at 76 beats per minute. Laboratory tests were normal; in particular, a urine examination showed no leukocyturia or bacteriuria.\nShe underwent an abdominal ultrasound which showed an asymmetric size of the kidneys and a bilateral chronic pyelonephritis aspect. Her right kidney measured 10 cm while the left measured 12 cm.\nA voiding cystourethrography (VCUG) was performed and showed grade IV VUR on the left side and grade I VUR on the right (Figs. and ).\nAn abdominal and pelvic computed tomography (CT) scan detected a left completely duplicated collecting system with hydroureteronephrosis and poor opacification of the upper pole moiety. In addition, the parenchyma of the upper pole moiety was atrophied with secretory and excretory delay. In association with VCUG findings, it appeared that the refluxing ureter was the one that drains the upper pole moiety and inserts lower into the bladder. On the right, a duplex collecting system was detected with hypotonic calyces, pelvis, and ureter of the upper pole moiety. An atrophic parenchyma and poor opacification of the upper pole moiety was also detected (Figs. and ). Renal scintigraphy was not available.\nWe carried out a left heminephrectomy because of the poor functioning of the upper pole moiety based on imaging findings associated with recurrent UTIs (Fig. ). On the right side she underwent dextranomer/hyaluronic acid (Deflux®) injections. Dextranomer/hyaluronic acid (Deflux®) was injected submucosally below the ureteral orifice at the 6 o’clock position to create a prominent bulge and raise the distal ureter and ureteral orifice.\nA year after the surgery she has no complaints. The symptoms are completely resolved. Biological and radiological follow-up is unremarkable. A timeline of the case is presented in Figure .
A 71-year-old woman complained of syncope and was transferred to a neighboring hospital. She had a medical history of appendectomy and hypertension, and no persisting respiratory symptoms or dysphagia was documented. Endotracheal intubation was performed because of her hemodynamic instability and altered consciousness. Thereafter, a non-enhanced CT revealed an ATA-AD with a DAA accompanied by a massive pericardial effusion (). The false lumen was not extending to the descending aorta (). She was then referred to our hospital. On her arrival, her systolic blood pressure was 50 mmHg and pulse rate was 88 beats per min with a high dose of inotropic support. Her Glasgow Coma Scale score was E3VTM6. Neither paralysis nor difference in limb blood pressure was observed. Laboratory test showed severe thrombocytopenia with a platelet count of 23×109/L. Enhanced CT was not performed because of her hemodynamic instability, and she was rushed to the operating theater after an immediate pericardiocentesis at the emergency bay to prevent sudden cardiac arrest. After induction of general anesthesia, median sternotomy was performed. Transesophageal echocardiography revealed no cardiac anomalies, a good left ventricular contraction and a competent aortic valve. Cardiopulmonary bypass was established by cannulation to the left femoral artery and bicaval venous drainage, and systemic cooling was initiated subsequently. Intraoperatively, we found that the left aortic arch was free from dissection, but the right aortic arch (RAA) was affected (). We feared that malperfusion to the right common carotid artery branching off from the RAA might occur if we clamped the RAA; thus, we added another arterial perfusion to the right axillary artery branching off separately from the RAA. After clamping both aortic arches at a bladder temperature of 28°C, significant decline in neither direct measurement of blood pressure of the left radial and the right dorsalis pedis arteries nor cerebral oxygenation monitored by near-infrared spectroscopic oximetry (NIRO-200, Hamamatsu, Herrsching, Germany) was observed. Confirming that malperfusion was avoided through these findings, we decided to avoid deep hypothermic circulatory arrest (DHCA) with retrograde cerebral perfusion. Systemic cooling was also discontinued. During the resection of the ascending aorta without opening both aortic arches, systemic perfusion was not interrupted. We found an intimal tear in the distal end of the ascending aorta () and the distal anastomosis with a 26-mm J-graft (Japan Lifeline, Tokyo, Japan) was performed excluding the intimal tear. Then, we removed the femoral arterial cannulation and resumed antegrade reperfusion through an epiaortic branch of the graft followed by ascending aorta replacement. After extubation on postoperative day (POD) 4, no neurological deficiencies were noted, but she developed respiratory dysfunction accompanied by a severe strider, which demanded transit support of non-invasive positive pressure ventilation (NPPV) for 5 days. She was discharged on POD 60. A new communicating dissection from the RAA to the descending aorta was shown on postoperative CT on POD 11. An intimal tear was found near the distal anastomosis () and the preoperative diameter of 32 mm () of the RAA increased to 35 mm ().
Patient 2 was a 31-year-old woman of Salvadoran descent, who delivered three children. At 4 years of age, the patient first presented episodes of seizures and abnormal neurological findings and was diagnosed with HHH syndrome. The patient had developmental disability, manifested by expressive language and attention problems determined upon neuropsychiatric evaluation, and significant myopia. The patient was prescribed a diet consisting of protein restriction to 1.5 g/kg/d during childhood. The patient's weight was at the 25th percentile with height less than the 3rd percentile.\nThe patient's first pregnancy occurred at 18 years old. She had episodes of nausea and dizziness. She was maintained on a low-protein diet in the first trimester, but mild hyperammonemia was detected during the 11th and 12th week of pregnancy; as a result, protein intake was further restricted to 40 g/d. It was unclear if the patient was compliant or not. At 22 weeks gestation, the patient developed seizures, and was initiated with carbamazapine. Brain imaging showed multiple small calcifications of unreported cause, with normal cerebral angiography. The child was born at full term, via Cesarean-section. The baby girl had intrauterine growth restriction (weight <3rd percentile). Although the baby girl remained small, follow-up recorded a normal development at her 2 years of age.\nVery little is described for the course of her second and third pregnancy. The second child was born via Caesarian section. A moderately elevated ornithine concentration (302 μM/L) was measured in the cord blood, but the levels decreased to normal in the newborn's blood 24 hours after birth. The patient's blood ammonia levels increased 24 hours postpartum and were given a treatment consisting of oral sodium benzoate and intravenous arginine. The patient responded well to the treatment. Follow-up showed normal growth and development for the child at 10 months of age.\nThe third child was born via repeat Cesarean section and had transient respiratory distress which required mechanical ventilation. No further information was given regarding the outcome of this child.\nUpon follow-up, the patient was noted to have been prescribed more anticonvulsants, and had significant weight loss from 95 to 50 lb. The patient died suddenly at the age of 31 years and 9 months, with an autopsy revealing two firm nodules in the brain, one each in the left parietal and frontal lobes with no determined cause of death.
A 57-year-old female was diagnosed at an outside institution 3 years previously with PTC with invasion into the recurrent laryngeal nerve, strap muscle, esophagus, and trachea, as well as multiple lymphadenopathy (T4aN1bM0). The patient was obese (body mass index: 35.0) and had diabetes. Her performance status was 0 according to the Eastern Cooperative Oncology Group system, and she did not smoke or drink.\nThe patient was referred to our department for treatment. She underwent total thyroidectomy and paratracheal and selective neck dissections (D2a), with resectional management of the recurrent laryngeal nerve, trachea, and esophagus. She also underwent concurrent reconstruction of the recurrent laryngeal nerve. Three months later, her tracheostomy stoma was closed with a local flap, and radioiodine treatment was performed. After that, she received follow-up care at another institution. Three years after her initial treatment of PTC, fluorodeoxyglucose positron emission tomography demonstrated multiple foci of increased fluorodeoxyglucose uptake in the left RPN and upper mediastinal lymph nodes (Fig. ). The patient was referred to our department for treatment of lymph node metastases. She complained of no symptoms associated with the retropharyngeal or upper mediastinal lymph nodes. Contrast-enhanced T1-weighted magnetic resonance imaging of the neck showed lymph node enlargement (18 × 14 mm) in the poststyloid component of the parapharyngeal space without invasion of the carotid artery, as well as in the upper mediastinum up to the innominate artery without invasion of the adjacent tissue (Fig. ). Cytology of the upper mediastinal lymph nodes revealed papillary adenocarcinoma. No cranial nerve deficits were observed. The patient was diagnosed with left retropharyngeal and upper mediastinal lymph node metastases from PTC. She underwent upper mediastinal dissection and left retropharyngeal dissection concurrently. The upper mediastinal nodes were resected successfully through the cervical approach with partial resection of the clavicle, though it was difficult to extend the neck due to a previous postoperative neck scar, severe obesity, and short neck. The retropharyngeal mass was then resected transorally.\nTransoral videolaryngoscopic surgery was performed according to the procedure reported by Tomifuji et al. []. The patient was intubated orotracheally with supine positioning. An FK-WO TORS Laryngo-Pharyngoscope Retractor (Olympus, Tokyo) was positioned so as to provide sufficient working space for RPN dissection. The retractor was then suspended with a holder. A flexible endoscope capable of angulation in 4 directions (Visera LTF-type VP, Olympus) was inserted through the oral cavity while an assistant held and manipulated the endoscope for viewing the surgical field.\nTo identify RPN, we performed intraoperative ultrasonography with the Aloca Arietta 70 (Hitachi, Tokyo), a flexible laparoscopic transducer manipulated with forceps. We confirmed that the RPN was located in the deep portion of the posterior palatine arch, lateral to the constrictor muscle. The RPN was sharply marginated. Ultrasound showed that the carotid artery was located adjacent to RPN (Fig. ). Transoral ultrasound helped determine the mobility of RPN relative to the carotid artery, through differential application of pressure to the retropharyngeal mass.\nWe cut the pharyngeal mucosa and submucosa away from posterior palatine arch and could visualize the constrictor muscle. After severing this muscle, the RPN was visible (Fig. ). During our procedure, we used ultrasound continually to confirm that the surgical approach to the tumor was appropriate. The transducer is compact in size, and its cable is thin and flexible. Therefore, even in a narrow working space such as the intraoral cavity, we could perform the intraoperative ultrasonography without the need to undock it.\nThe operator employed a single-use electrosurgical knife with radiofrequency alternating current (KD-600, Olympus) to resect the tumor by transoral videolaryngoscopic surgery. This knife has several characteristics, as follows: a disposable needle, malleable shaft, and adjustable needle length from 2 to 4 mm. We used the Swift mode (output power 30, effect 4) of the high-frequency surgical system (ERVE VIO300D). This procedure could be completed without the need for intraoperative conversion to an open surgery procedure. The transoral videolaryngoscopy surgery lasted 2 h, with little blood loss and no need for blood transfusion, and no tracheostomy was performed either pre- or postoperatively.\nNo serious hemorrhage or emergent airway compromise was observed during the patient's hospital stay. There were no serious adverse events that required further intervention. The patient began eating food with a paste-like consistency on postoperative day 2, and resumed a regular diet on postoperative day 5. The nasogastric tube was removed on postoperative day 2. Postoperative swallowing function was normal with a functional outcome swallowing scale (FOSS) of grade 0. A postoperative videofluoroscopic swallowing study (VFSS) showed no aspiration or penetration. The retropharyngeal tumor in this case was diagnosed pathologically as papillary adenocarcinoma without extranodal spread.\nThe patient underwent 131I therapy postoperatively. She is alive and has remained disease free 20 months after treatment.
A 64-year-old woman was presented to us with a two-year history of poly arthritis, most marked in the knees and both hand proximal interphalangial joints (PIP) and associated with left ankle pain, in October 1998. Past medical history revealed a history of hypertension and intracerebral hemorrhage. Rheumatoid arthritis was diagnosed according to 1987 ACR revised criteria, based on a symmetric polyarthritis involving the PIP, shoulders, left ankle and knees, morning stiffness and the presence of rheumatoid factor.\nShe was treated with hydroxychloroquine and methotrexate, but an exacerbation occurred involving the right knee in January 1999. Also, she had numbness of the right sole. At radiologic evaluation of the knees, narrowing of the medial joint space and joint effusion were noted, but no erosion was seen. Treatment with local steroid injection was resumed with increments of methotrexate doses.\nWithin a few weeks of local steroid injection in the knee joint, however, right knee pain worsened and numbness of the sole continued. She gave no history of prior injury or other paraesthesia.\nOn physical examination, she revealed pain and increased sensation along the plantar aspect of the right foot and the mass was palpable on the posterior aspect of the right knee. Also, she showed weak flexion of the big toe and decreased right ankle jerk.\nA lobulated 4×4×3 cm3 cystic lesion with inhomogeneous signal intensity was seen on ultrasound. The needle aspiration of the right knee joint was unproductive, and magnetic resonance imaging of the right knee and popliteal fossa was obtained and revealed a Baker’s cyst(). The cyst extended towards the popliteal tendon and lateral collateral ligament and there was no evidence of deep vein thrombosis.\nOn electromyography, 3+ and 2+fibrillation and positive sharp waves potentials were observed in the abductor hallucis and tibialis posterior showing abnormal spontaneous response. During contraction, polyphasic motor unit was observed in the abductor hallucis and tibialis posterior.\nOn nerve conduction studies, proximal latency and amplitude in the right tibial nerve was 15.4ms and 0.1mV, showing low amplitude and delayed proximal latency of right tibial compound muscle action potential.\nRepeated needle aspiration was unproductive, and open surgical procedure was performed. At operation, a lobulated cystic lesion displacing neurovascular structures was released, but neurovascular invasion by a cyst was not found(). The cavity contained many folds and measured 4×4×3 cm3, and extended anteriorly toward the posterior cortex of the tibia. The communication between the synovial compartment and the cavity was present near the popliteal hiatus. The biopsy finding was compatible with Baker’s cyst due to rheumatoid arthritis(). Her pain resolved following surgical removal of Baker’s cyst, but mild degree of numbness remained.
A 71-year old, female patient with a previous history of non-Hodgkin lymphoma and transient ischemic attack came to the emergency room of our University Hospital for sudden onset of right hemifacial paraesthesia, edema of the lower lip (Fig. ) and accentuation of an already present tinnitus. The current presentation had been preceded by a few blisters similar to those usually observed in herpes labialis, and no aphthous ulcer was detected on mouth inspection. Background therapy included aspirin and betahistine, with no personal and family history of adverse drug reactions, atopy, contact dermatitis, urticaria, angioedema, cranial nerve palsy, granulomatous or inflammatory diseases. After symptomatic treatment by intravenous steroids and antihistamines, the patient was discharged with prescription of a short-course therapy with oral prednisone and cetirizine []. This resulted in partial remission of symptoms, but 1 week later the patient was readmitted to ER for symptom recurrence and worsening of lip edema without detectable oral cavity and tongue alterations. Due to the apparent involvement of the 5th cranial nerve, a varicella-zoster virus (VZV) infection was hypothesized and therapy with valaciclovir initiated. On occasion, a blood sample was drawn showing evidence of anti-VZV IgG with undetectable IgM. One month later, on further admission at the ER for the same clinical picture associated with swelling over the left zygomatic region, an angioedema of unknown origin was suspected. Thus, a course of twice daily dose of 10 mg cetirizine was prescribed [–]. However, this approach was ineffective and also the subsequent replacement of aspirin with clopidrogel and temporary withdrawal of betahistine resulted in no improvement. IgM and eosinophil count, as well as plasma levels of angiotensin converting enzyme were in the normal range, thus helping to exclude the hypothesis of Gleich syndrome or sarcoidosis. Patch testing for dental materials was also negative, and complement screening was then performed with evidence of normal levels of circulating C1q (143 mg/L) and both antigen (302 mg/L) and functional (109%) C1-Inhibitor (C1-INH). On the contrary, C4 was low (0.03–0.04 g/L; NR 0.09–0.36 g/L) and C3 fluctuated around the lowest levels of the referral range (0.93–0.82 g/L; NR 0.9–1.8 g/L) on repeated assessments. These findings ruled out the possibility of acquired AE due C1-INH deficiency [–], prompting us to explore the (auto)immune-inflammatory state: antineutrophil cytoplasmic antibody tested negative, whilst low titer (1:160) anti-nuclear antibodies (ANA) were found along with antiphospholipid antibodies (lupus anticoagulants; anti-cardiolipin, anti-β2-glycoprotein IgM), possibly related to complement consumption [].\nAs ultrasound scan detected only a subcutaneous, hypoechogenic thickening of the inferior lip and we did not find any further clinical or laboratory sign of systemic inflammation, recurrent swelling was interpreted as a form of idiopathic angioedema and treated with tranexamic acid after a thrombophilia screen testing negative for further risk factors [, ]. Both this antifibrinolytic drug and a following, therapeutic course with the leukotriene receptor antagonist montelukast [] failed to solve the edema. Finally, clinical picture, blood analyses, and lack of response to any of the previous therapies suggested the possibility of Melkersson–Rosenthal syndrome []. Thus, the patient was referred to the Dental Clinic, where a mucosal biopsy of the affected lower lip was performed (Fig. ). Histopathological examination showed non caseating granulomas (Miescher’s cheilitis), consistent with a diagnosis of MRS. The two aggregates of non-caseating granulomatous inflammation consisted of lymphocytes and epithelioid histiocytes, and few multinucleated giant cells, clustered around scattered vessels (Fig. ). Special staining for identification of fungal microorganisms and acid-fast bacteria were negative. No foreign material could be detected even at polarization. One month of oral steroid (prednisone, 25 mg qd, gradually tapered to 5 mg) resulted in remission of lip swelling but not in definitive recovery. However, since then patient’s perception of both symptoms and aesthetic relevance decreased, and at present she undertakes a few-days regimen of prednisone only when feeling a relapse of edema.
A 7-year-old male patient reported to the Department of Oral Medicine and Radiology with the chief complaint of swelling in the upper front tooth region since 25 days. Swelling was small initially, which gradually increased to the present size. Swelling was associated with pain, which was continuous, dull, and mild in nature. Patient gave history of trauma to the upper front tooth region 2 years back. There was no history of pus discharge and ulceration associated with the swelling. On examination, extraorally diffuse swelling in the right maxillary anterior region in the middle third of the face was noted with upward displacement of nares (). The swelling was tender on palpation, hard in consistency with no rise in local temperature. Intraorally swelling extended from labial frenum to distal aspect of maxillary right first deciduous molar (tooth number 54) with obliteration of labial and buccal sulcus. Discoloration and proximal caries was seen in relation to maxillary right deciduous central incisor (tooth number 51). Also, maxillary right permanent central incisor (tooth number 11) was clinically missing and maxillary left permanent central incisor (tooth number 21) was erupted (). Upon palpation, intraorally swelling was hard in consistency and tender. Mobility in relation to 51 and 52 was also observed. Based on the history and clinical findings, a provisional diagnosis of radicular cyst of 51 and a differential diagnosis of dentigerous cyst in relation to clinically missing 11 was given.\nPatient was then subjected for radiographic investigation. Intraoral periapical (IOPA) radiograph and occlusal radiograph revealed proximal caries involving enamel and dentin without pulpal involvement in relation to 51, well-defined radiolucency in the periapical regions of 51 and 52, and this radiolucency was enveloping the coronal portion of 11 and 12. Root resorption of 51 and 52 were noted. Occlusal radiograph also revealed the presence of buccal cortical plate expansion ().\nCone beam computed tomography revealed osteolytic lesion with sclerotic margin in the periapical region of 51, measuring about 1.8 × 2.5 × 2.5 cm in dimension. The tooth 11 was displaced apically, root resorption of 51 and 52 was seen. Thinning of nasal floor with buccal cortical plate expansion was present ( and ). Based on CBCT features, radiographic diagnosis of radicular cyst of 51 was given. Cyst was enucleated with extraction of 51 and 52 and preservation of 11. Histopathologically the specimen revealed cystic epithelium and fibrous connective tissue capsule. The epithelium showed arcad-ing pattern, with dense collagen fibers in the connective tissue. These histopathological features confirmed the diagnosis of radicular cyst in relation to 51.
The proband was a 5 year old girl suffering from muscle weakness soon after birth. She was the first child of a healthy non-consanguineous couple and was vaginally delivered at full-term with normal weight and Apgar scores. She was found to have ptosis of both eyelids soon after birth, rarely showed limb movements, and exhibited weakness in chewing and swallowing. She was unable to erect her head until she was 6 months old and was unable to crawl until she was 10 months old. She was unable to sit until she was 1.5 years old and has never been able to stand, even at the conclusion of the present study. She was unable to bilaterally move her upper arms or hold objects steadily in both hands. She had retardation of her language development; she started babbling at 1.2 years old and, at the time of the present study, was only able to speak at a low rate and with poor articulation. A former gene panel test showed negative results for spinal muscular atrophy and peroneal muscular atrophy.\nPhysical examination confirmed the following: physical retardation (height, 97 cm; body weight, 16 kg); bilateral ptosis; hyperextension of ankle and carpal joints; foot dropping; amyotrophy in bilateral proximal lower limbs; hypotonia in all four limbs; no elicited tendon reflexes; low muscle strength [Medical Research Council (MRC) scale grade 3 in cervical muscle, grade 2 in bilateral proximal upper limbs, grade 3 in distal upper limbs, grade 1 in bilateral proximal lower limbs, and grade 2 in distal lower limbs]; normal sensation, and normal cutaneous plantar reflex. She also had a high-arched palate, enamel hypoplasia, and a small jaw; she did not exhibit nystagmus (). Thoracolumbar scoliosis and right acetabular dysplasia were revealed by X ray (). Her serum CK level (118.9 U/L) was normal and she was negative for anti-AChR and anti-MuSK antibodies. Her neostigmine test showed a negative result. Her EMG () presented spontaneous potentials (in the form of positive sharp waves and fibrillations) as well as a reduction in motor unit recruitment for skeletal muscles of the limbs. Her motor unit potential (MUP) revealed an increased time course (14.2 ms of left extensoris digitorum communis and 14.4 ms of right tibialis anterior) but a normal amplitude. The conduction velocities of both her sensory and motor nerves were decreased. The amplitudes of both CMAP and sensory nerve action potential (SNAP) were decreased, whereas their peak latencies were prolonged. H-reflex waveforms were not elicited. Unfortunately, the patient did not cooperate with a repeated nerve stimulation examination. Electroencephalography (EEG) showed extensive 3–4.5 Hz, θ and δ waves mixed with non-sustained discharges of a small amount of low-amplitude spike/sharp waves during shallow sleep (). Assessment via the Wechsler Intelligence Scale revealed a low verbal intelligence quotient of 52, whereas the intelligence quotient could not be determined due to the patient's inability to perform bilateral hand movements. No abnormalities were found via blood-urinary metabolic screening, electrocardiography, visual/auditory evoked potentials, or magnetic resonance imaging of the head and spinal cord. The patient's parents refused muscle biopsies to further confirm the patient's diagnosis. To identify the ultimate cause, whole-exome sequencing (WES) was performed. It was approved by the ethics committee of the Second Xiangya Hospital of Central South University (approval No.: XY-LL20180408), and informed consent was obtained from the patient's parents.
A 37-year-old male immigrant patient was admitted to our hospital with recurrent hemoptysis about 50 mL per day. His thoracic computed tomography (CT) scan showed lymphadenopathy around right lower lobe bronchus; however, there was no explanation for hemoptysis on CT and conservative treatment was initiated (Figure ). After few days, he presented again with the same symptoms and his detailed medical history revealed two massive hemoptysis episodes previously in his country. Thoracic CT scan was repeated and the same images were obtained. Fiberoptic bronchoscopy was planned to find the possible pathology. A written informed consent was obtained from the patient.\nFiberoptic bronchoscopy was performed under local anesthesia. Larynx, trachea, carina, and left bronchial system were all found to be normal. Bronchoscopy showed mucosal infiltrations and a 2 to 3-mm ridge in the lateral wall of the right lower lobe. Also, a clot was observed inside the right lower lobe, suggesting that the reason for hemoptysis was mucosal irregularity. Therefore, punch biopsy was performed. Following punch biopsy, massive bleeding occurred. Early intervention could not control the bleeding, and intubation was performed urgently. After 1,500 mL bleeding and 30-min cardiopulmonary resuscitation, bleeding was taken under control and intubation tube was gently replaced by a double-lumen tube. Meanwhile, CT scans were retrospectively checked and suspected that it was a bronchial system pathology. Thus, the patient was operated. During thoracotomy, the first, right main bronchus was taken under control and later exploration was started. There was no mass around the intermediate lobe and its distal segments. Since the ridge which bled was located at the orifice of right lower lobe, bilobectomy was performed in a usual manner. Specimens were examined for bleeding site, ex vivo. In the mucosal ridge, a 1-mm ostium opening to the lower lobe artery was observed. Using a mosquito clamp, the bronchovascular fistula was detected (Figure ).\nIn the early postoperative period, the patient reported ocular symptoms, genital ulcerations, and arthralgia within the previous year, suggestive of Behçet"s disease. On postoperative Day 4, high-dose steroid and cyclophosphamide treatment were prescribed to the patient to prevent Behçet's disease exacerbation, in addition to azathioprine maintenance treatment for 18 months. He was uneventfully discharged on postoperative Day 6.\nPathological examination revealed no specific agents with Ehrlich-Ziehl-Neelsen, Gram, and Grocott staining. No lesions were seen, except for a yellow, irregular area of 22x18x5.5 cm in size, 2 cm away from the bronchial surgical margin in the lower lobe of the right lower lobectomy specimen. The intraparenchymal contours were unable to be fully distinguished and a hepatized appearance was visible due to intense bleeding in the middle and lower lobes. Microscopic examination revealed necrotic nodules and vasculitic changes, compatible with clinically suspected Behçet's disease (Figure ).
A 12-year-old female patient reported to the department with a chief complaint of forwardly placed upper front teeth. The patient cited esthetics as the main reason for desiring orthodontic treatment.\nOn extraoral examination, she had a mesocephalic, mesoproscopic face, convex profile, posterior divergence, and incompetent lips. The patient displayed 100% upper incisor crown exposure, increased buccal corridor space, and a nonconsonant smile arc. Intraoral examination revealed a constricted maxilla, reduced intercanine width of 30 mm, and intermolar width of 42 mm and mild crowding in the lower anterior segment. The patient exhibited Class II molar relation bilaterally, over jet of 9 mm and overbite of 6 mm []. Ashley Howe's analysis showed an increased premolar basal arch width compared to premolar diameter concluding that arch expansion is possible. The cephalometric tracing confirmed that the patient had a moderate Class II skeletal pattern with ANB value of 6 degrees. The patient had average growth pattern and proclined maxillary and mandibular incisors [ and ].\nThe objectives of treatment in the sagittal plane were to achieve ideal overjet and overbite by mandibular advancement, in the vertical plane to correct lower anterior facial height, in the transverse plane to achieve expansion of maxilla and overall to attain a pleasing soft tissue profile.\nThe initial treatment plan decided for the patient was to opt either for twin block or the Frankel appliance. However, according to our treatment objectives, it was essential to achieve maxillary expansion complemented with an ideal overjet, overbite, and improvement in soft-tissue profile. Thus, this lead to the discovery of Twinkle R 3D appliance which was fabricated with a construction bite made by sagittal advancement of 6 mm and vertical opening of 4 mm. The patient was asked to wear the appliance for 14 h in a day. Activation of jackscrew was done by slow maxillary expansion protocol,[] that is quarter turn every 2 days for 3 weeks. After the desired expansion was attained, activation was discontinued. The patient was asked to follow-up every 3 weeks and was instructed to wear the appliance for a period of 8 months [].\nFollowing 8 months of treatment, the patient's profile and smile greatly improved due to expansion of the maxillary arch complemented by mandibular advancement. Overjet and overbite were improved due to the postural forward positioning of the mandible. The midlines were coincident with each other and with the face. In addition, a good buccal occlusion and Class I canine relation were achieved []. End of treatment panoramic tomogram showed good root parallelism and healthy appearance of supporting tissues []. Superimposition and cephalometric analysis showed 4 mm of increase in mandibular length [ and ].
The patient's mother was 36 years old, with a total of 5 pregnancies and 1 live birth. Eleven years ago, she gave birth to a healthy boy via cesarean section. She previously had 2 artificial abortions and 1 spontaneous abortion, with no family history of hereditary diseases. Ultrasound test result was normal at gestational week 6. At gestational week 12, nuchal translucency of 1.3 mm was observed, with no positive findings. Non-invasive DNA analysis showed low trisomy 21 risk, low trisomy 18 risk, and low trisomy 13 risk. At gestational week 24+5, ultrasound examination showed abnormal development of the long bones of the limbs (the length of the long bones was shorter than 1% controlled to the same gestational week of normal fetus), thick metaphysis in the right lower limb, irregular vertebral arrangement, and a narrow and small thorax (Fig. A–D). The patient's parents decided to terminate the pregnancy at 27 weeks of gestation considering this as a lethal skeletal dysplasia.\nAfter the termination of pregnancy at week 27, the gross examination of the fetus showed a flat face and nose bridge, short limbs, asymmetric short lower limbs, and bilateral clubfeet with bilateral ankle joint contracture (Fig. A, B). The autopsy report indicated that the left humerus bone was 2.5 cm long; the right humerus bone, 2.0 cm; the left femur, 3.0 cm; and the right femur, 2.2 cm; all were significantly shorter than 4SD measured at the same gestational age. In addition, the shapes of T3 to L3 were abnormal, the vertebral bodies appeared fused, the thorax was small with a maximum circumference of 16 cm, and the abdominal circumference was 26 cm. The autopsy also found subcutaneous edema in the head and face and thick metaphysis in the lower limbs. X-ray imaging showed short femur and humerus bones, a narrow and small thorax, thick metaphysis with a thick “splashed paint”’ pattern, and asymmetric short lower limbs (Fig. A–C).\nThe karyotype of the fetus was 46, XX. No unusual single-nucleotide polymorphisms was detected. Whole exome analysis showed that the fetus was heterozygous for the EBP mutation (NM_006579.2; C.440G>A p.Arg147His), which must have occurred de novo because the parents were non-carriers (Fig. ). Thus, CDPX2 was confirmed.
A 73-year-old male with edentulous maxilla was referred to the University Department of Oral and Maxillofacial Surgery of the “Evaggelismοs” general hospital due to a migrated implant into the right maxillary sinus. The patient suffered from chronic obstructive pulmonary disease and therefore he had quitted smoking. Five years ago he underwent dental rehabilitation with the placement of six implants in the maxilla and four in the mandible. In less than 2 months after initial placement, all implants had failed to osseointegrate. A year and a half later the patient underwent guided bone regeneration with bovine-derived xenograft and 7 months after this point, another 10 implants were inserted into both the maxilla and mandible. Two months later, all implants had once again failed to osseointegrate. During the attempt of removal by the dentist, one of the implants was displaced into the maxillary sinus, without the dentist being able to retrieve it. The patient then visited another dentist, who could not either remove the migrated implant.\nUpon arrival to our clinic, a full medical and dental record was retrieved from the patient, and he was scheduled for surgical removal of the implant. Prior to operation, the patient underwent a radiographic examination with water’s X-ray (Fig. ), as well as CBCT examination (Fig. ) which confirmed implant migration and revealed its exact position inside the maxillary sinus.\nSurgical procedure initiated with local anesthesia by injecting xylocaine 1% andepinephrine 1:100,000 solution in the soft tissues involving the right half of the maxilla. After a crestal incision, a full-thickness mucoperiosteal flap was raised, exposing the anterior-lateral wall of the maxilla in an area extending from canine to molar region. Using a high-speed rotary instrument under sterile saline solution irrigation, a rectangular window was created in the anterior-lateral maxillary wall. The implant was detected through the bony window and captured by a mosquito forceps (Fig. ). The mucoperiosteal flap was then placed back at its initial position and was anchored with 4.0 resorbable sutures (Fig. ). Amoxicillin (1 g twice daily) was prescribed for 1 week with analgesic treatment. Sutures were removed 2 weeks after surgery. The patient was advised to follow a soft diet plan for 4 weeks and was provided with proper oral hygiene instructions. He underwent scheduled visits on a monthly basis to check the course of healing for the following 6 months.
This is a 26-year-old man who experienced right lower limb weakness for 2 years and the weakness exacerbated in last half year. He visited the second affiliate hospital of the Zhejiang University School of Medicine. A physical examination indicated the result of the straight leg raising test was positive and also muscle atrophy. The muscle power of the right lower limb had decreased to grade 3. There was no sensory impairment. Magnetic resonance imaging revealed an intraspinal extradural tumor over T10 to L3 (Fig. ). It appeared to be a spinal extradural arachnoid cyst (SEAC). To confirm whether the fistula existed between the subarachnoid space and arachnoid cyst, and to localize the position of the fistula, we performed a real-time technique. First, we injected contrast medium into the cyst under fluoroscopy. After 1 h the follow-up computed tomography (CT) was administered, and it revealed there was no contrast-infiltration into the subarachnoid space (Fig. ). We then extracted about 20 mL of fluid from the cyst. The follow-up magnetic resonance imaging on the same day indicated the cyst did not become smaller (Fig. ). A “one-way valve” fistula was suspected such that cerebral spinal fluid could pass into the cystic space from the subarachnoid space but could not flow in the opposite manner. Therefore we designed a procedure to localize the fistula. We penetrated two needles into the cyst and subarachnoid space separately in the L3/L4 level under digital subtraction angiography (Fig. ). Pending the fluid drained through those two needles, we injected 10 mL of contrast medium slowly into the subarachnoid space and a little contrast medium infiltrated into the cystic space horizontally at the T12/L1 level. We then administered high resolution computed tomography (HRCT) immediately to confirm the position in the axial plane. The HRCT revealed a funnel-shaped enhancement between the lower edges of the T12 body and the left nerve root, and this is the accurate position of the fistula (Fig. ).\nAfter general anesthesia and endotracheal intubation, the patient was placed in prone position. A fluoroscopy was used to determine the correct operative level, and a longitudinal incision was made 5 cm off midline at the T12 level. Muscle was dissected layer by layer, and a spinal process and left lamina were exposed. The left lamina was partially ground by cutting burr and then removed along with ligamentum flavum by Kerrison punch. After that, the cyst was exposed. Under a microscope, the cystic wall was fenestrated and it then drained off clear cystic fluid (cerebral spinal fluid). After partial excision of the cyst and evacuation of cystic fluid, a spinal endoscope (SPINENDOS, Germany) was maneuvered into the space and the fistula was detected (Figs. , ). The fistula was detached from the arachnoid membrane and was ligated with a 7–0 Vicryl purse string suture. Regional leakage from the repaired site was noted. An anastoclip was then used to close the fistula (Fig. ). Pulmonary pressure was elevated by ventilator (valsalva maneuver) to check the leakage and there was no more leakage. Hemostatic matrix and gel [Fibrin sealant kit (human), (Shanghai RAAS Blood Products Co, Ltd., Shanghai, China)] were utilized at the local region. The postoperative diagnosis was a thoracolumbar extradural arachnoid cyst. The pathological report revealed an arachnoid cyst (Fig. ). The symptoms improved on postoperative day 2. His lower limbs regained strength with limited dorsiflexion of the right foot.\nThrough the 3-month outpatient department follow-up, there was no more numbness or weakness of his right lower limb. The motion of dorsiflexion also improved. The 3-month follow-up magnetic resonance imaging revealed no recurrence of the previous lesion and no spinal cord compression (Fig. ).
A 34-year-old woman with no significant past medical history presented with the chief complaint of left facial numbness, left ear pain and decreased hearing in the left ear of three years duration. The patient had also been experiencing sharp and shooting pain in different areas of her left thigh. The pain was not associated with any weakness, tingling or numbness. Besides mild fatigue she denied having any fevers, night sweats or weight loss. Neurological physical examination was grossly intact except for sensory loss in the V2 (maxillary) and V3 (mandibular) distribution of trigeminal nerve (cranial nerve V). Abdominal exam was without evidence of lymphadenopathy and hepatosplenomegaly. Given deficits in the sensory distribution of trigeminal nerve, she was initially evaluated by ear, nose and throat (ENT) and underwent two sequential minimally invasive surgeries for nasal polyps without significant resolution of her symptoms. She was subsequently referred to a neurologist and had magnetic resonance imaging (MRI) of the brain performed. Brain MRI revealed a soft tissue mass with expansion in the left Meckel’s cave, measuring 22 x 16 x 12 mm (Figure ), raising concerns for a trigeminal schwannoma. She was evaluated by the neurosurgery and underwent an orbital zygomatic craniotomy and left trigeminal schwannoma resection. Pathology revealed deposition of abundant hypocellular eosinophilic material on light microscopy examination (Figure ). Congo red staining demonstrated characteristic 'apple-green birefringence' upon polarization (Figure ), consistent with diagnosis of trigeminal nerve amyloidoma. Unfortunately, no immune fluorescence or electron microscopy was done on the pathology specimen to determine the amyloid subtype. Postoperatively, the patient was referred to oncology to rule out systemic amyloid deposition. Basic workup including complete blood count was unremarkable except for hemoglobin of 12 g/dL with mean corpuscular volume (MCV) of 76 fL per cell. White blood cell and platelet counts were within normal limits. Comprehensive metabolic panel did not reveal any liver or renal abnormalities. Coagulation profile including prothrombin time (PT), activated partial thromboplastin time (APTT), international normalised ratio (INR) was normal. The patient had both serum and urine protein electrophoresis with immunofixation done which did not reveal any monoclonal protein. Serum free light chains were normal with kappa free light chain 16 mg/dL, lambda free light chain 12 mg/dL and kappa lambda free light chain ratio of 1.36. Urinalysis was without evidence of hematuria or proteinuria. Electrocardiogram (EKG) revealed normal sinus rhythm. Two-dimensional (2D) echocardiogram revealed ejection fraction 60% and normal ventricular wall thickness. Abdominal fat pad biopsy as well as a bone marrow biopsy was performed and both were without evidence of amyloidosis or other plasma cell dyscrasia or lymphoproliferative disorder. The patient was not offered any more localized and systemic therapy with follow-up brain MRI three months after surgical resection without evidence of recurrent amyloidoma.
A 72-year-old male patient visited the hospital due to a right maxillary central incisor fracture. A periapical lesion was not found, and his periodontal condition was favorable. On March 6, 2007, a flapless atraumatic extraction was performed and the socket was probed. According to the probing results, the existing labial bone destruction was severe. Mucoperiosteal flap elevation was performed before implant placement. Bio-Oss was grafted to restore the labial dehiscence defect. A collagen membrane (Ossix) was then covered, and the wound was sutured. Flap undermining was not performed to maintain the labial soft tissue contour. The membrane where the upper part of the implant was covered was intentionally left exposed. The exposed membrane was absorbed over time, leading to favorable secondary healing. After 6 months, the secondary surgery was performed. The final prosthesis was installed on November 29, 2007. This prosthesis remained stable even after 100 months of function.\nSocket sealing surgery is performed for extraction socket preservation when an implant is placed immediately following tooth extraction. Because a mucoperiosteal flap is not formed, this surgery is favorable because the alveolar bone and its surrounding soft tissues can be preserved as much as possible. In this study, an open membrane technique and a palatal gingival graft technique were used, with the aim of preserving as much keratinized gingival tissue as possible. However, the benefits of keratinized gingival preservation remain controversial. Some studies have stated that there is insufficient evidence for the importance of keratinized gingival preservation []. However, other studies have indicated that preservation of the vestibule and its associated keratinized gingiva can improve oral hygiene and minimize the risk of bleeding on probing, recession, plaque-induced peri-implantitis in implant, and restoration in the future [–]. As an attempt to cover the grafted extraction socket and to prevent bacterial colonization from salivary contamination, free gingival grafts were introduced in 1994 for socket sealing [] However, the reported failure rate was 50% or higher (26% for total necrosis and 31% for partial necrosis) []. For this reason, a new procedure using a combined epithelialized-subepithelial graft was introduced []. The free gingival graft used in this study demonstrated that wound dehiscence occurred in two cases, but that healing was successful in all cases. All assessed grafts functioned successfully until the final prosthesis was loaded.\nWilson et al. presented clinical results for cases where a connective tissue membrane was used for immediate implant placement. They reported excellent osseointegration results with immediate implant placement in horizontal bone defects of 4 mm or higher []. Recently, Stimmelmayr et al. reported bone grafting to rebuild buccal alveolar defects at the same time that the tooth is extracted, combined with a soft tissue graft to seal the socket, showed promising results and could be an alternative treatment to delayed hard tissue grafting [].\nZubillanga et al. attempted to use primary sutures for ridge preservation in all cases. They found that though 45% of the membrane was eventually exposed, infections or other clinical complications were not observed in any cases []. Furthermore, Engler-Hamm et al. compared the use of primary sutures made of resorbable membranes with intentional exposure during ridge preservation. They reported that the discomfort and swelling following surgery was less severe when the membrane was intentionally exposed without flap dissection than when the primary suture was performed through flap dissection. They also found that the results were more favorable when keratinized gingival tissues were preserved, and no differences in bone resorption were observed []. In this study, the resorbable collagen membrane was intentionally exposed to allow for socket sealing. All of the cases assessed here had successful results with minimal observed complications. Unlike previous studies that showed the resorbable membrane as a reservoir of bacterial propagation resulting in infection and eventually failure of bone graft, there was not a single case of failure due to infection when membranes were intentionally exposed. This means that appropriate antibiotic therapy and disinfection could sufficiently reduce the risk of infection, and extraction socket sealing surgery using resorbable collagen membrane can show clinical results similar to those of palatal gingival graft.\nIn this study, socket sealing surgery was performed to prevent soft tissue retraction in regions of esthetic importance, such as the maxillary anterior region or the premolar region. In the average observation period of 78.7 ± 31.4 months, the post-extraction loss of marginal bone was found to be 1.21 ± 0.13 mm on average, indicating that a stable state was maintained. In conclusion, palatal gingival grafts and open membrane techniques using resorbable membranes can be used to produce clinically favorable results in terms of soft tissue preservation in regions of esthetic importance (Additional file ).
A 24-year-old gravida 2 para 1 presented at 28 weeks of gestational age with nausea, vomiting, and inability to tolerate oral intake. Her past medical history was significant for chronic constipation and need for intermittent self-catheterization as a child. Her prior pregnancy was complicated by a primary cesarean with postoperative ileus and clostridium difficile infection requiring bowel resection. She had a total of 5 prior surgeries on her intestine and colon throughout her life. Repeated laparotomy for abdominal pain is common to CIPO []. Her family history was significant for a brother with megacystis who had bladder surgery as a child and required catheterization as a toddler. Her prior child was alive and well with no medical issues.\nShe was hospitalized for a total of 5 weeks during her second pregnancy beginning at 28-week gestational age and required nasojejunal feeds for nutrition. General surgery was consulted and computed tomography demonstrated ileus pattern with no obvious evidence of obstruction. While hospitalized, an ultrasound at 31 weeks of gestation revealed a fetus measuring greater than the 95th percentile, polyhydramnios, and severe megacystis (). She eventually delivered at 35 weeks of gestational age due to persistent intolerance of oral intake. Repeat cesarean delivery was performed.\nAt birth, her infant was noted to have an enlarged bladder, microcolon, and poor tolerance of oral intake (). He required catheterization to drain his bladder. A colonic biopsy was performed which revealed ganglion cells were present, ruling out Hirschsprung's disease. He eventually required partial colonic resection with colostomy. He had a second abdominal surgery in his first 12 months of life with additional resection of bowel. Since this last abdominal surgery, he has had improved weight gain and tolerance of oral intake.\nGenetic testing was performed on the mother and the infant, and they were both confirmed to have a novel heterozygous mutation in the ACTG2 gene (C632G>A, p.R211Q) on chromosome 2p13.1.
A 50-year-old Thai male presented with three episodes of generalized seizures and right-sided hemiparesis for 6 h before arrival. He had no previous seizures. He had a history of well-controlled diabetes mellitus and hypertension for 20 years and took metformin 1000 mg/day and diltiazem 60 mg/day. His past medical history revealed progressive slowness in thinking and walking, memory impairment, sleep-wake disturbance and mood disorder, which had slowly progressed for the past 20 years; however, it had rapidly worsened during last year of his life. He was diagnosed with organic mood disorder 5 years before this presentation and treated with risperidone 0.5 mg/day, sertraline 50 mg/day, and trihexyphenidyl 1 mg/day. Even with this treatment, his symptoms had been progressively worsening for the past 1 year to the point that he could not perform daily living activities, such as taking the correct medications. He was the fifth of seven children. His sister had a history of unexplained hearing loss, cognitive decline, and slowness of movement starting at the age of 20. When she was 40 years old, she developed visual and auditory hallucinations as well as recurrent transient ischaemic attacks with full recovery. The patient’s father and mother died at the ages of 70 and 78, respectively, and had no history of cognitive impairment or stroke. A mental status examination showed a good level of consciousness; however, he was mute and slow to respond to commands. A motor examination showed right-sided weakness (grade 2/5 for arm and grade 0/5 for leg) and generalized hyperreflexia except for right leg hyporeflexia and no sensory impairment. There was also mild right facial weakness. A CT of the brain showed diffuse white matter abnormalities, old multiple lacunar infarctions in the bilateral basal ganglia, thalamus, and left pons. The initial diagnosis was acute ischaemic stroke with seizures. He was prescribed 300 mg/day of aspirin and usual stroke care. Phenytoin was prescribed for seizure control. An MRI of the brain was performed on day 12 after admission. The results showed acute infarction of the left pons (Fig. ), several old lacunar infarcts surrounded by minimal gliosis in the bilateral putamen and thalamus as well as few scattered small, old infarcts surrounded by minimal gliosis in the bilateral frontal-parietal periventricular white matter without anterior temporal lobe lesion (Fig. a, b, and c). Surprisingly, a large number of microbleeds were found throughout the brain (Fig. d, e and f). The total numbers of cerebral microbleeds was 214 and 136 in lobar areas according to the Microbleeds Anatomical Rating Scale (MARS) []. After a comprehensive review of vascular risk factors, his HbA1C was 5.5%, and his serum LDL was 79 mg/dL. His blood pressure was well-controlled. None of these risk factors explained his symptoms and MRI findings. He had a history of unexplained cognitive impairment and mood disorder. In addition, his sister had a history of cognitive decline, psychiatric symptoms and transient ischaemic attack; therefore, a genetic condition, such as CADASIL, was suspected and confirmed by molecular genetic testing, which revealed a homozygous known pathologic variant, c.1672C > T (p. Arg558Cys), in the NOTCH3 gene. His clinical symptoms deteriorated, and he died of tracheobronchitis with secretion obstruction.
A 25-year-old Korean male was admitted due to 3 days′ history of hematochezia. Two weeks earlier, he had visited the outpatient clinic due to intermittent dull left lower quadrant pain and loose stool of 3 months′ duration. He had mild exertional dyspnea. Faint pansystolic murmur was audible along his left sternal border. A firm tender mass of 3–4 cm in size was palpable at his left lower abdomen. Colonoscopy revealed a small pedunculated polyp on the cecum and another small sessile polyp on the sigmoid colon The two polyps were removed with polypectomy. The mucosal folds were hypertrophic and the lumen was slightly narrow over a long segment of the sigmoid colon. Histologic examination of the removed polyps revealed lymphoid polyps. Mucosal biopsy from the narrow segment of the sigmoid colon disclosed only mild infiltration of chronic inflammatory cells.\nOn admission, his vital signs were stable. Hemoglobin was 11.0g/dl. Upper GI endoscopy was unremarkable. Colonoscopy revealed a small shallow ulceration at the previous polypectomy site on the cecum, and any evidence of recent bleeding could not be noted. Four days later, he experienced mild hematochezia again without any significant change in blood pressure and heart rate. Urgent sigmoidoscopy revealed fresh blood up to the splenic flexure, but the bleeding point was not clear.\nSelective inferior mesenteric arteriography demonstrated abnormal vasculature involving the sigmoid and distal descending colon without active bleeding (). Echocardiography and right cardiac catheterization demonstrated a ventricular septal defect with Qp/Qs ratio of 1.99. On the next day of the cardiac catheterization, he experienced a sudden massive lower GI bleeding and became hypotensive. On urgent laparotomy, a long abnormally hard thickened colonic segment with dilated tortuous vasculature involving the distal descending colon, sigmoid colon and upper rectum was found. Segmental colectomy, including the involved segment, was done. Post-resection barium angiography was done and the finding was similar to that of the preoperative angiography and confirmed the completeness of the resection. Barium enema was done with the resected specimen showing markedly thickened bowel wall (). The cut surface of the bowel wall demonstrated multiple ectatic blood vessels throughout the entire thickness of the wall (). Histology revealed abnormal ectaticvasculature () and a bleeding point could be identified ().\nHis postoperative course was uneventful. A cardiac surgeon recommended open heart surgery for his ventricular septal defect, but he refused because he felt that his dyspnea was tolerable. He was discharged in a relatively good general condition.
A 37-year-old Thai woman was referred to our institution for fixation of multiple recurring pathological fractures of both upper and lower extremities. Five years ago, she had sustained fractures of the right shoulder, left elbow, and right femoral shaft after falling on the ground. She received internal fixation of those fractures, as illustrated in Supplementary 1. One month previously, she had been admitted to a local hospital because of fractures of the right distal humerus and left tibial shaft without history of trauma. While staying at that hospital, a new fracture occurred at the left femoral shaft. Since complicated orthopedic procedures for fixation of her fractures were anticipated, she was subsequently transferred to our hospital.\nShe reported urinary frequency, with more than 10 nocturia episodes per night, accompanied by increased thirst. She was a non-smoker and did not have a history of diabetes, hypertension, or other chronic diseases. She denied a family history of any metabolic bone diseases or genetic disorders.\nA musculoskeletal examination showed slightly varus malalignment of the right elbow and shortening with external rotation deformity of the left leg, and painful swelling of the left tibia. A neck examination did not reveal thyroid gland enlargement, thyroid nodules, or palpable neck masses.\nA review of plain radiographs revealed multiple fractures with diffuse osteopenia. Brown tumors were also observed at multiple sites (the shaft of the right humerus, the proximal shaft of the right radius and ulnar, the proximal shaft of the left femur, and the shaft of the left tibia; Fig. ). Moreover, a bone survey showed a salt-and-pepper appearance of the skull, while a rugger-jersey appearance was observed in a lateral spine radiograph (Fig. ). These findings were consistent with osteitis fibrosa cystica, prompting further laboratory investigations for PHPT.\nThe patient’s initial laboratory investigations are listed in Table . She had elevated serum calcium (13.6 mg/dL); low phosphorus (2.2 mg/dL) with high bone turnover, giving markedly elevated alkaline phosphatase (1482 U/L); and very high serum PTH (3850 pg/mL).\nThe patient was diagnosed with PHPT. Because her serum calcium was high, it was decided that a parathyroidectomy procedure would be performed prior to operative fixation of the fractures. The fractures were temporarily stabilized with a long leg back-slab on the left leg and a U-slab on the left arm. Further investigation with a parathyroid scan (using 99mTc-Sestamibi and single photon emission computed tomography) showed a parathyroid lesion at the inferior pole of the left thyroid lobe. The patient underwent a left lower parathyroidectomy; a 2.5-cm mass that was removed was subsequently histologically confirmed as a parathyroid adenoma.\nAfter removal of the tumor, the patient’s PTH level decreased to 21 pg/mL on postoperative Day 3. Immediately after the surgery, calcium carbonate, alfacalcidol, and vitamin D2 (ergocalciferol) supplementation were prescribed as part of a prophylactic protocol to prevent postsurgical hypocalcemia. Despite receiving those supplementations, hungry bone syndrome developed approximately 1 week after the parathyroidectomy, and her serum calcium and phosphate levels decreased to 8.1 and 1.1 mg/dL, respectively. As a result of the syndrome, intravenous calcium gluconate was given; the oral calcium carbonate dosage was raised to 4800 mg per day, while the alfacalcidol was increased to 3 μg per day to maintain a normal serum calcium level. The calcium and vitamin D supplements were gradually decreased over time, and the hungry bone syndrome had resolved by 10 months later.\nAfter the serum calcium level had normalized (for our case, 10 days after the parathyroidectomy), closed reduction and internal fixation of the left proximal femoral shaft was performed. The orthopedic surgeon used a cephalomedullary nail (Zimmer Natural Nail; Zimmer Inc., Warsaw, Ind., USA). Other fractures were treated conservatively. The patient recovered well and was discharged 10 days after surgical fixation, with no postoperative complications.\nSix months following the parathyroidectomy, the patient demonstrated a significant improvement in pain relief and level of ambulation. She switched from ambulation in a wheelchair to full weight bearing on her left leg. At the 1.5-year follow-up, plain radiographs of her extremities showed union of all fracture sites and complete healing of the brown tumors (Fig. ). Although she was free of bone pain and able to walk without an assistive device, there was still some limping due to the shortened right femur.
This is a 68-year-old gentleman with past medical history of hypertension, hypothyroidism, depression, and panic attacks, presented with epigastric pain. No nausea, vomiting, hematemesis, or weight loss reported. He underwent endoscopic biopsy that showed a poorly differentiated adenocarcinoma. He was referred to a tertiary center for further evaluation. His physical examination was unremarkable. Laboratories were normal. Upper endoscopy revealed an ulcerated polypoid tumor involving the greater curvature. The endoscopic ultrasound (EUS) showed thickening of the gastric wall with destruction of the layered structure without infiltration or penetration of the serosa.\nThe repeated biopsy revealed a carcinoma with solid proliferation and associated moderate chronic gastritis without evidence of Helicobacter pylori. The histological type of the tumor could not be identified (Figure ). The dense stromal lymphocyte infiltrate thought to be superimposed probably secondary to the ulceration. The computed tomography (CT) was negative for ascites, lymph nodes enlargement, peritoneal carcinomatosis, or liver metastases. On the basis of these findings, and considering the patient’s age and general condition a gastrectomy was performed with lymph nodes dissection and a Roux-en-Y reconstruction.\nThe gross examination of the gastrectomy specimen revealed a patelliform tumor measuring 3.2 cm in diameter with depression and central ulcer. The cut section showed that the tumor invades the muscular layer of the gastric wall (the muscularis propria). Five centimeter apart from the tumor a 0.7 cm polypoid lesion was observed.\nMicroscopically, the tumor was composed of sheets and nests of round to polygonal cells with poorly defined cell borders within a dense lymphocytic background. Cytologically, the tumor cells were large and pleomorphic with abundant eosinophilic cytoplasm, large vesicular nuclei and prominent nucleoli (Figure a and b). No vascular or lymphatic invasion was observed. No epithelioid cell granulomas or Mott cells identified. None of the 27 dissected lymph nodes showed metastasis. The accompanying small polypoid lesion at the greatest curvature was a hyperplastic polyp without adenomatous changes.\nImmunohistochemical staining for pan-cytokeratin (CAM5.2 and AE1/AE3) was positive. The tumor was negative for CK7, CK20, synaptophysin, CDX2 and CD45.\nThe in situ hybridization for EBV-encoded small RNAs (EBER-1 and 2) showed an intensive nuclear hybridization signal corresponding to the carcinoma cells (Figure c), whereas the tumor was negative for both LMP1 and EBNA2. No EBV hybridization signal evident in the adjacent non-neoplastic gastric mucosa, in the lymphocytes around the tumor cell or in the dissected lymph nodes.\nP53 showed strong and diffuse positivity in the tumor cells (Figure d). Regarding of the mismatch repair proteins there was retention of expression of all four of the proteins (MLH-1, PMS-2, MSH-2 and MSH-6) (Figure e and f).\nFinally, EBV-associated LLC of the stomach was diagnosed and staged as (T2, N0, M0) according to the WHO classification of tumors 2010.\nThe clinical course was complicated on the 5th postoperative day with insufficiency of the duodenal stump which necessitated revision, and at nine months with perforation of the left colonic flexure most probably of ischemic genesis which was managed by laparotomy and subtotal colectomy with side/side ileodescendostomy. Fifteen months after the operation the patient remains well with no evidence of recurrent disease.
A 49-year-old Caucasian woman was referred to our center. She was known for years to have fibrous dysplasia in the left femur and tibia.\nWhen she was 49 years old, an attempt was made to excise the fibrous dysplasia from the proximal femur. Extreme hemorrhaging complicated the operation. The attending orthopedic surgeon decided to refer the patient to our specialized center. At the time of her visit to our center, she complained about progressive pain in her left thigh and lower leg. Her clinical physical examination revealed soft swelling in the left quadriceps region.\nConventional X-rays of the left leg showed typical features of fibrous dysplasia in the proximal femur (Figure ) and the proximal tibia (Figure ) with ground glass appearance and a shepherd's crook deformity.\nMagnetic resonance imaging (MRI) of her left leg showed extensive fibrous dysplasia in the entire femur with expansive growth in the greater trochanter. The left proximal tibia also showed signs of fibrous dysplasia extending 20 cm distally from the tibial plateau. Four well-delineated, cyst-like, intra-muscular soft-tissue lesions were seen in the quadriceps region. Surgery was planned in two sessions. Prior to both operations, angiography and embolization of the pathologic vascularization in both the femur and the tibia were performed.\nDuring the first operation, intra-lesional excision of the tumor of the left proximal femur was performed, followed by cryosurgery. A correctional osteotomy of the left hip was performed, followed by homologous bone transplantation and fixation with a proximal femoral nail. Because of a weakened femoral head, the femoral neck screw had to be stabilized with bone cement. Allograft bone chips were impacted to induce bone matrix. During the procedure, an incisional biopsy of the soft-tissue lesions in the quadriceps muscle was performed.\nThree weeks later an intra-lesional excision of the fibrous dysplasia of the left tibia was performed, followed by cryosurgery and implantation of an massive allograft inlay in the tibia. The allograft was fixed with AO screws. During this procedure, the soft-tissue lesions in the quadriceps muscle were excised.\nMicroscopic examination of the bone lesions of both femur and tibia showed hypocellular fibrous tissue with irregular bone formations and without cytologic atypia compatible with fibrous dysplasia. In one lesion, the remnants of fracture callus were present (Figure ).\nThe soft-tissue lesions showed a paucicellular tumor with spindle-shaped or stellate not atypical cells embedded in a loose myxoid alcian blue-positive inter-cellular matrix with sparse capillary blood vessels. Confocal microscopy revealed a thin fibrous capsule at the tumor margin; however, at the interface with skeletal muscle, infiltration between the individual muscle fibers was evident, which is typical of intra-muscular myxomas (Figure ), proving the diagnosis of Mazabraud's syndrome in our patient.\nPost-operatively, the patient's treatment consisted of non-weight-bearing mobilization for three months in plaster of Paris. During follow-up, plain films were obtained at six-week intervals. Eventually, these images showed consolidation of the femur osteotomy and incorporation of the allograft.\nNine months post-operatively the patient developed progressive pain in the left thigh. Conventional X-rays showed protrusion of the femoral neck screw. Repositioning of the screw was performed. Her post-operative follow-up with X-rays obtained regularly (Figures and ) was satisfactory.\nAlmost two years later the patient complained about a painless mass localized on the lateral side of the left knee. A solid mass was felt anterolaterally of the proximal tibia. Plain film radiographs did not show abnormalities; however, an ultrasound examination showed a homogeneous, hypoechogenic, soft-tissue mass with a cyst-like aspect. MRI showed a well-defined soft-tissue lesion in the lateral retinaculum and vastus lateralis muscle with a homogeneous high signal intensity on T2-weighted images and a homogeneous low signal intensity on T1-weighted images, consistent with fluid. A second intra-osseous lesion with an approximate diameter of 3 cm and the same signal characteristics and enhancement pattern was detected in the lateral tibial plateau (Figures and ).\nThe lesions were considered to be a new soft-tissue myxoma and a relapse of fibrous dysplasia in the proximal tibia, respectively. Surgical excision of both lesions was performed without pre-operative biopsies, followed by cryosurgery and homologous bone implantation. A histopathological examination of the intra-muscular lesion showed a myxoma. As a unique finding, histopathological examination of the intra-osseous lesion in the lateral tibial plateau was consistent with myxoma as well (Figure ).\nDuring follow-up, MRI of the patient's left leg was performed at regular intervals. These scans showed no signs of recurrence in the first post-operative years.\nThree years after her last operation multiple tumors in her left upper leg were felt, which raised clinical suspicions of myxomas. MRI scans showed five new soft-tissue lesions consistent with characteristics of myxoma in the upper and lower leg regions. All lesions were marked ultrasonographically and excised, followed by cryosurgery. Histopathological examination of all intra-muscular lesions confirmed the diagnoses. Post-operatively the patient recovered gradually. Every three months ultrasonography of her left leg was performed, which showed no signs of myxoma until 2009. By then, she had developed several new intra-muscular myxomas, which were not treated. At her last follow-up examination in early 2010, our patient was in good condition. Several myxomas had developed but were found to be stable on the basis of MRI.
A 65-year-old male presented to the Tokyo Metropolitan Cancer and Infectious disease Center Komagome Hospital (Tokyo, Japan) with a dry cough and mild hemoptysis. The patient had been treated with chemotherapy since 2002 for advanced thymic squamous cell carcinoma, and showed solitary cerebral and pulmonary metastases in the right lower lobe (RLL) on presentation. At the time of the initial diagnosis, a specimen was acquired from the primary site using computed tomography-guided biopsy. First-line chemotherapy was comprised of cisplatin (80 mg/m2 on day one, every 28 days) and irinotecan (60 mg/m2 on days one, eight and 15, every 28 days). The patient subsequently underwent irradiation of the primary site and pulmonary metastasis in the RLL with a total dose of 60 Gy over 6 months, followed by whole-brain irradiation (WBI) for the solitary brain metastasis with a dose of 40 Gy two years into therapy (). In terms of the clinical course, cognitive function gradually declined due to WBI, and cerebral atrophy was shown on head imaging (). Pulmonary metastasis in the RUL was histologically confirmed as thymic squamous cell carcinoma following transbronchial biopsy (). Histologically, the tumor was composed of highly atypical epithelioid cells, with nuclear atypia and eosinophilic cytoplasms in the fibrotic stroma (). Immunohistochemically, the tumor cells were positive for CD5 and c-Kit (). The patient was treated with nine lines of chemotherapy and palliative radiotherapy for local control of the brain metastasis and two pulmonary metastases. The patient then underwent second-line chemotherapy comprised of two cycles of carboplatin (area under the curve 6 on day one, every 21 days)/paclitaxel (80 mg/m2 on days one, eight and 15, every 21 days). In the fourth year, pulmonary metastasis in the RUL was targeted and irradiated once at a dose of 60 Gy, but recurrence subsequently developed. Following completion of radiation therapy, the patient received six lines of chemotherapy in 4 years, comprised of four cycles of gemcitabine (800 mg/m2 on days one and eight, every 21 days) and vinorelbine (25 mg/m2 on days one and eight, every 21 days), two cycles of docetaxel (60 mg/m2 on day one, every 21 days), 12 cycles of S-1 (80 mg/m2 on days one to 14, every 21 days), four cycles of amrubicin (35 mg/m2 on days one to three, every 21–28 days), eight cycles of irinotecan (100 mg/m2 on days one, eight and 15, every 28 days), three cycles of pemetrexed (500 mg/m2 on day one, every 21 days), and lastly, the combination of four cycles of doxorubicin 40 mg/m2, cisplatin (50 mg/m2 on day one, every 21 days), vincristine (0.6 mg/m2 on day three, every 21 days) and cyclophosphamide (700 mg/m2 on day four, every 21 days) (ADOC). The patient succumbed to carcinomatous lymphangiosis in the 10th year of treatment following the initiation of first-line chemotherapy.
A 50-year-old male came to the emergency room of our hospital and stated that a hand-made silicon rod had accidentally passed into his rectum 12 h prior to presentation. He could not remove it on his own. His vital signs were stable, and a physical examination showed that his abdomen was soft and without tenderness. There was a palpable elastic, hard mass in the lower abdomen and no signs of peritonitis. A digital rectal examination by palpation located the end of the foreign body in the deep rectum. An attempt to manually remove it in a transanal manner failed due to its shape, large size, and deep position. Laboratory data were essentially normal, with the exception of slightly elevated C-reactive protein (1.76 mg/dL). Plain abdominal X-ray imaging showed a foreign body in the pelvic area and absence of free air (Fig ), while computed tomography (CT) revealed an 18 × 4 cm foreign body with a cylindrical shape in the rectum (Fig ). No severe complications, such as perforation or obstruction, were seen. We decided to attempt removal in a transanal manner under endoscopic and X-ray fluoroscopic observation.\nFollowing intravenous administration of 15 mg of pentazocine, a scope (PCF-Q260AZI; Olympus, Japan) was inserted transanally with the patient in a left lateral decubitus position to directly visualize the foreign body. The lateral pelvic view obtained by X-ray fluoroscopy showed the foreign body in the rectosigmoid position. We attempted removal with endoscopic instruments, including a snare, biopsy forceps, grasping forceps, and net, though that failed because of the round shape and large diameter. When manually compressed from the abdominal wall under endoscopic and X-ray fluoroscopic observation, we noticed that the axis of the foreign body changed (Fig ). Thus, we were able to guide it using manual abdominal compression with endoscopic and X-ray fluoroscopic assistance. Once the object moved to the lower rectum, it could be removed transanally by manual extraction. After removal, we found that the foreign body was constructed of a silicon material and made by the patient himself, with a size of 18 × 4 cm (Fig ). Sigmoidoscopy following extraction showed mild erosion in the rectal mucosa with no findings of bleeding or perforation. The patient was released without hospitalization or surgery.
A 4-year-old boy was presented to our unit on referral with a three-week history of high-grade fever and a five-day history of generalized abdominal pain and abdominal distension. There was an associated history of headaches and body weakness. He had no history of jaundice. He had several episodes of vomiting which was initially none bilious, but later became bilious. There was an associated history of passage of diarrhea stools two days before presentation, although the patient had not passed stools on the day of presentation. He had no history of passage of melena or of hematochezia. His parents complained that he had been passing scanty urine for about five days before presentation. He had been receiving medications from the referring hospital for about two weeks before presentation.\nAt presentation, he was chronically ill looking with a toxic facie, febrile (temperature 38.5°C), pale, dehydrated, anicteric, and had no pedal edema. His respiratory rate was 28/minute; he had reduced air entry on both lung bases posteriorly. His pulse rate was 128/minute, which was regular but of small volume. Only first and second heart sounds were heard. Abdominal examination showed a distended abdomen which did not move with respiration. He had generalized tenderness with guarding. The bowel sounds were absent. A digital rectal examination showed an empty rectum with a full and tender rectovesical pouch. An initial assessment of a generalized peritonitis was made. The patient was placed on NPO with nasogastric tube for gastric decompression. He was commenced on IV fluid resuscitation and broad spectrum IV antibiotics (ceftriaxone and metronidazole) and was worked up for surgery. Initial laboratory investigations showed a hematocrit of 24% and hypokalemia (2.5 mmol/l) and a serum urea of 10 mmol/l. After an initial fluid resuscitation and correction of serum potassium, he had 300 ml of whole blood transfused. He had an exploratory laparotomy on the second day of admission. At surgery, about 600 ml of a feculent peritoneal fluid was drained, and a single perforation on the antimesenteric border of the terminal ileum was closed in two layers. An intraoperative diagnosis of perforated typhoid enteritis was made. Blood and tissue cultures were not done. He was continued on IV fluids and the same empiric antibiotics after the surgery.\nOn the third day of postoperation, the abdominal wound had a purulent discharge from the distal end. The wound was opened and stitches were removed from the site of drainage, with the institution of daily wound dressing. By the fifth day of postsurgery, a dark patch was noticed to have developed in the skin over the right iliac fossa and measured about 5 cm in its widest diameter. At about the 7th day of postoperation, the dark dry patch had extended to the right hypochondrium, and a similar patch had appeared along the edges of the abdominal incision and the left iliac fossa. A diagnosis of anterior abdominal wall gangrene was made. On the 8th day of postsurgery, the patient was noticed to have developed a complete wound dehiscence () and a fecal fistula. He had fluid resuscitation and was taken back to the theatre on the 10th day after the first surgery, for wound exploration and debridement of the anterior abdominal wall gangrene. A new intestinal perforation was seen at about 2 cm from the initial perforation. The intestinal perforation was exteriorized as an ileostomy, and the abdominal wound closed with tension sutures. Postoperatively, the patient's condition remained unstable, with intractable shock. He died six days after the reexploration of septic shock.
A 43-year-old healthy male was admitted with abdominal pain and vomiting for 3 days. He had a previous history of a hemorrhagic gastric ulcer 10 years before and no trauma. X-ray and computed tomography (CT) revealed a left diaphragmatic hernia (Fig. a, b). CT showed that the entire stomach, spleen, pancreatic tail, and splenic flexure occupied two thirds of the left thoracic cavity. Upside-down stomach and gastric volvulus causing vomiting were detected. The patient was diagnosed with Bochdalek hernia based on the presence of a hernia orifice in the posterolateral aspect of the left diaphragm. When CT imaging performed 10 years before was retrospectively reviewed, a more modest degree of Bochdalek hernia was detected, which may have caused the gastric ulcer at that time (Fig. ).\nA nasogastric tube was inserted to decompress the stomach; however, the patient had persistent abdominal pain and incarcerated obstruction. He was referred to our hospital for a surgical intervention. The results of laboratory examinations were within normal ranges, and the respiratory function test showed a percentage of vital capacity of 93.9% and forced expiratory volume in 1 s of 106.2%. We preoperatively discussed with an anesthesiologist about perioperative administration of neutrophil elastase inhibitor or steroids for re-expansion pulmonary edema which we have expected to occur after restoring herniated viscera.\nThe patient underwent L-shaped laparotomy to surgically repair Bochdalek hernia. A 7-cm hernia orifice was observed in the left posterior diaphragm (Fig. ). Herniated viscera were carefully returned back into the abdominal cavity, and a hernia sac was not detected. There were no adhesions between abdominal viscera and the thoracic cavity or left lung. The left diaphragmatic defect was repaired by primary closure with 0 absorbable thread without diaphragmatic tension. Chest X-ray immediately after surgery showed a fully expanded left lung and that 5 h after surgery showed no pleural effusion or re-expansion pulmonary edema (Fig. ).\nThe postoperative course was uneventful, and diaphragmatic hernia has not recurred for 3 months.
A 22-year-old multiparous woman with a history of primary sclerosing cholangitis complicated by portal hypertension, esophageal varices, and thrombocytopenia was admitted at 29 0/7 weeks for evaluation and management of shortness of breath and lower extremity edema. Her obstetric history was significant for prior spontaneous preterm birth with placement of an ultrasound-indicated McDonald cerclage at 20 1/7 weeks of gestation in the index pregnancy. At the time of cerclage placement, the 5 mm Mersilene (polyester) tape was noted to be suboptimally placed along the posterior rim, distal to the internal os, so a second McDonald cerclage of 0-polyester suture was placed cephalad to the tape. She was admitted at 28 0/7 weeks for preterm labor and the cerclages were removed with no remarkable anatomic irregularities. She received antenatal corticosteroids and magnesium sulfate during her admission for preterm labor and was discharged on hospital day #3 with a cervical exam of 1cm dilation and 70% effacement. During her readmission at 29 0/7 weeks for shortness of breath, she began to have contractions and moderate vaginal bleeding on hospital day #3. Her cervical exam was 4 cm and 90% effaced. She was transferred to labor and delivery, where she continued to have a rapid cervical change to 6 cm. A brief fetal heart rate bradycardia occurred with spontaneous recovery to a normal baseline. At this time, findings were concerning for placental abruption or possible coagulopathy from decompensated liver failure with a total estimated blood loss of 500 mL. Amniotomy was performed to expedite vaginal delivery. However, cervical dilation did not continue and complete cessation of vaginal bleeding was noted. Careful visualization of the cervix revealed that the posterior rim of cervix was detached and traversing the presenting fetal head creating a tension band that prevented further dilation or descent. This band of cervical tissue was clamped and suture-ligated followed by rapid delivery. She delivered a male infant with a weight of 1325 g and Apgar scores of 5 and 7 at 1 and 5 minutes, respectively; arterial cord pH was 7.33. Following delivery of the placenta, vaginal exam revealed a partial posterior cervical detachment from 2 to 9 o'clock. The avulsed portion of the cervix did not appear necrotic and was bleeding briskly. Hemostasis was achieved with a single running locked suture. Her postpartum course was otherwise unremarkable, and she was discharged on postpartum day #2. At her six-week postpartum visit her cervical exam was remarkable for a normal appearing anterior lip; however the posterior lip was not visible and not palpable on digital exam (). Pathology was not available for the fragments of cervix that were excised and the placenta was remarkable for acute chorioamnionitis.
A 70-year-old male with history of amyotrophic lateral sclerosis (ALS), dysphagia status post gastrostomy tube placement, chronic respiratory failure status post tracheostomy, and gastroesophageal reflux disease presents with six-day duration of melanotic stools. The patient was mechanically ventilated with gastrostomy tube and tracheostomy in place. His physical exam otherwise was benign. He was found to have acute onset anemia with hemoglobin of 7.1 g/dl. He was suspected of having peptic ulcer disease secondary to NSAIDs given the history of chronic musculoskeletal pain with NSAID use. Due to continual decrease in hemoglobin requiring daily blood transfusions despite conservative management, upper endoscopy was performed. It demonstrated a bleeding ulcer distal to pylorus in the duodenal bulb. Endoscopic technique was used to remove the clot, but the procedure resulted in bleeding that could not be stopped leading to poor visualization despite epinephrine injections. Due to his multiple comorbidities, he was deemed to be a poor surgical candidate. The patient was referred to interventional radiology for embolization of gastroduodenal artery (GDA).\nDuring the embolization of GDA, the celiac angiogram demonstrated no evidence of active extravasation, but contour irregularity at the level of the mid-GDA was noted. Prophylactic coil embolization of GDA was performed achieving GDA stasis. Post-embolization angiography of the SMA demonstrated no evidence of bleeding from IPDA branches. Incidentally noted was a replaced right hepatic artery arising from the SMA.\nDespite embolization of GDA, patient’s hemoglobin continued to decrease to 6.8 g/dl and the patient continued to have melena and hematemesis resulting in hypotension. The patient required an additional nine units of packed red blood cells (pRBCs), six units of platelets, and five units of fresh frozen plasma. Computed tomographic angiography (CTA) showed a large bleed from IPDA off the SMA immediately posterior to the origin of the replaced RHA (Figure ).\nThe patient was referred for urgent embolization by interventional radiology.\nDuring angiography, no active extravasation was identified and given that the GDA was already embolized, the most reasonable source vessel in the suspected territory would be the IPDA. The replaced RHA was selected and IPDA was identified as its first branch (Figures , ).\nWhile the branch arising directly from the replaced RHA represented the posterior IPDA, the anterior IPDA was seen as collateral vessel going back to the middle colic artery (Figure ).\nHowever, no focus of active bleeding could be identified from digital subtraction angiography from the posterior IPDA or the anterior IPDA. Cone beam computed tomography (CBCT) catheter angiography of the posterior IPDA was performed to identify the region of the duodenal bleed analogous to the region of active extravasation seen on abdominal CTA. The same location on the posterior wall of the duodenum seen on abdominal CTA was matched on CBCT angiography (Figure ).\nSubsequently, the posterior IPDA was embolized using n-butyl cyanoacrylate (NBCA) (Trufill n-BCA Liquid Embolic, Codman Neurovascular, Raynham, Massachusetts, USA) mixed with ethiodol 1:4 dilution ratio administered via the microcatheter (Figure ).\nPost-embolization angiography of the SMA demonstrated successful embolization of the posterior IPDA with sparing of the anterior IPDA. The patient became hemodynamically stable post procedure and no longer required blood products transfusion. His hemoglobin stabilized at 8.6 g/dl. The patient was successfully discharged to a skilled nursing facility to continue his recovery. At eight weeks follow-up the patient remained asymptomatic and he agreed to participate in this case report.
We present a case of a giant mature teratoma of the anterior mediastinum in a 35-year-old Caucasian woman. Following the onset of an episode of influenza associated with productive cough, the patient, an ex-smoker, underwent a chest X-ray which revealed the presence of a severe increase of the frontal cardiac area, with particular involvement of the atrial sectors and evident divarication of the sternal angle. Subsequent transthoracic echocardiography showed a rounded parenchyematous extra-cardial formation measuring approximately 9 cm in diameter and pressing against the lower posterior wall of the right atrium, with the chambers and valves of the heart being within the norm (Ejection Fraction = 71%), and an anomalous movement of the inter-ventricular septum. A thoracic computed tomographic scan with contrast medium confirmed the presence of a voluminous expansive formation with a maximum diameter of 125 mm, liquid contents, clear contours, multiple compartments, and parietal calcifications. This tumor was causing compression of the adjacent pulmonary parenchyma, the vascular and bronchial structures of the right lung (subsequently confirmed with fibrobronchoscopy), and the superior vena cava, without signs of infiltration (Figure ). The tumor markers tested while the patient was hospitalized (α-fetoprotein, β-HCG, and CA 19-9) were within normal ranges. Her respiratory function tests showed a prevalent, obstructive type, slight ventilatory incapacity: forced expiratory volume in one second was 2.63 (73% of the former value), forced vital capacity was 3.63 (87% of the former value), and blood gases at baseline and after stress were within the normal range. The day after the patient was admitted to our hospital, she underwent surgery to excise the mediastinal mass with a median sternotomy approach.\nOnce the mediastinal cavity was opened, it was possible to see the presence of the voluminous tumor, which occupied most of the right hemithorax with compression of the adjacent pulmonary parenchyma. There were also widespread adhesions to the superior vena cava, the ascending aorta, the pericardium, and part of the lower pulmonary lobe, where an atypical resection of pulmonary parenchyma was performed.\nThe lesion measured 15 cm × 14 cm × 8 cm and showed smooth, regular external contours. The cut surface of the mass was cystic, filled with a yellowish white proteinaceous material admixed with hair. A thickened area of the cyst wall measuring 6.5 cm across was composed of a more solid white fibrous tissue, which also contained multiple small cysts filled with yellowish material. Fluid from the cysts in the mass was examined after removal, showing an amylase activity of 599 U/l despite serum levels being normal.\nMicroscopically, the lesion resembled a mature cystic teratoma, which is more commonly seen in the ovary. The cystic wall was predominantly lined by a squamous epithelium associated with sebaceous glands, hair follicles, and a chronic inflammatory reaction (Figure ). Other areas of the cyst showed a monostratified ciliated epithelium, cartilage, and mucinous glands recapitulating respiratory tract structures. More solid areas of the mass revealed pancreatic tissue with mature ductal and acinar structures intermixed with islets of Langerhans. In addition, in adipose tissue at the periphery of the mass, a thymic residue with prominent Hassall's corpuscles was identified. Careful examination of the mass did not reveal the presence of immature tissue. The post-operative period was uneventful, and the patient was discharged on post-operative day 10.
A 45-year-old right-handed male with a history of hypertension, diabetes mellitus, and depression—but no other known cognitive issues—presented in November 2013 with altered mental status. He had been found stuck in a basement alcove for an unknown period of time prior to being brought to the emergency room by emergency medical services, and the patient was unable to provide an accurate timeline of events leading up to his presentation. On neurologic examination in the emergency room, he was slow to respond to questions and did not know the correct date. Right-sided weakness but no Parkinsonian features or obvious changes in tone were noted. Magnetic resonance imaging (MRI) of the brain demonstrated a left acute to subacute GP infarct and an old right GP lacunar infarct (). Magnetic resonance angiogram of the head and neck was normal. Basic laboratories on admission were generally unremarkable except for glucose 438 mg/dL, creatinine 1.45 mg/dL, and a white blood cell count of 13 K/uL; all of these values normalized during his hospital stay. A serum toxicology screen on admission was unremarkable. Low-density lipoprotein was 160 mg/dL and hemoglobin A1c was 12.0%. Transthoracic echocardiogram showed a normal ejection fraction and no interatrial shunt. Telemetry showed no signs of arrhythmia.\nThe patient was started on aspirin and atorvastatin as well as medications to control his blood pressure and diabetes. On discharge two days later, he exhibited mild right-sided weakness and slight problems in orientation; he knew that he was in the hospital but did not know its name and could not state the exact date in November 2013.\nHowever, five days later the patient again presented with altered mental status. He was oriented to self only, produced only one-word answers with considerable prompting, and appeared abulic. Overall, his family felt he was a completely different person than prior to his stroke. Repeat MRI of the brain showed interval evolution of the left BG infarct but no other new findings. No other causes for his exam findings (including seizure, infection, or toxic-metabolic etiologies) were discovered. He was discharged in early December 2013 on similar medications as on his prior admission.\nThe patient did not follow up for formal neuropsychological tests, but he had undergone bedside testing during the latter half of his second hospital stay. Deficits in the Montreal Cognitive Assessment were present in all domains but were most prominent in short-term and working memory, visuospatial ability, executive function, attention, and concentration (). He perseverated in the alternate triangles and squares sequencing task. Expressive aphasia was evident while describing the Boston Diagnostic Aphasia Examination's Cookie Theft picture. Micrographia, primarily with his right hand, was also present ().\nIn a follow-up visit with his primary care physician 3 days after discharge from the second hospital admission, he was noted to be oriented to person and date, but not to location. The patient displayed language and comprehension deficits: he was “unable to follow commands to fully assess strength and rarely speaks in full sentences but can answer simple sentences with great effort.” However, more rigorous testing of other aspects of his cognition was not performed.
A 29-year-old male university lecturer presented to the respiratory outpatient clinic with shortness of breath, cough, haemoptysis and recurrent chest infections. The clinical examination was unremarkable. The patient had a history of asthma, with unlimited exercise tolerance and no history of smoking or asbestos exposure. An incidental finding of a right hilar mass was detected on plain chest x-ray. Following the initial assessment, the patient underwent a CT and PET CT, which showed a well circumscribed intraparenchymal mass with an endobronchial component, which had low fluorodeoxyglucose (FDG) uptake (Fig. ).\nThe patient subsequently underwent rigid and flexible bronchoscopies, showing an endobronchial tumour with satisfactory margins for a sleeve lobectomy. Biopsies from the endobronchial mass suggested a diagnosis of benign nerve sheath tumour. The patient underwent a video-assisted thoracoscopic surgery exploration, followed by open sleeve right upper lobectomy, with the tumour positively identified in the resected lobe.\nHistology showed a spindle cell lesion comprised of cells arranged in a haphazard fashion with small amounts of eosinophilic cytoplasm and bland monomorphic nuclei. The tumour was strongly positive on immunohistochemistry for S100 protein and also showed positivity for CD56 and glial fibrillary acidic protein (GFAP), with a subpopulation of CD34 positive cells. The morphological and immunohistochemical findings were unusual and reminiscent of so-called gastrointestinal schwannoma, but lacking the characteristic lymphoid aggregates and inflammatory component. The features were, nevertheless, best characterized as a benign schwannoma and the strongly positive immuno-staining for S100 protein supported this diagnosis (Fig. ).\nPost-operatively the patient complained of fatigue and developed subcutaneous erythematous nodules on the anterior right chest, which raised the suspicion of neurofibromatosis type I. The nodules resolved spontaneously within 2 weeks, with no new changes on the follow-up chest CT scan. The diagnosis of neurofibromatosis type I was ruled out following genetic testing. No recurrence was identified at one year and the patient was discharged from follow-up.
A 70-year-old male with history of amyotrophic lateral sclerosis (ALS), dysphagia status post gastrostomy tube placement, chronic respiratory failure status post tracheostomy, and gastroesophageal reflux disease presents with six-day duration of melanotic stools. The patient was mechanically ventilated with gastrostomy tube and tracheostomy in place. His physical exam otherwise was benign. He was found to have acute onset anemia with hemoglobin of 7.1 g/dl. He was suspected of having peptic ulcer disease secondary to NSAIDs given the history of chronic musculoskeletal pain with NSAID use. Due to continual decrease in hemoglobin requiring daily blood transfusions despite conservative management, upper endoscopy was performed. It demonstrated a bleeding ulcer distal to pylorus in the duodenal bulb. Endoscopic technique was used to remove the clot, but the procedure resulted in bleeding that could not be stopped leading to poor visualization despite epinephrine injections. Due to his multiple comorbidities, he was deemed to be a poor surgical candidate. The patient was referred to interventional radiology for embolization of gastroduodenal artery (GDA).\nDuring the embolization of GDA, the celiac angiogram demonstrated no evidence of active extravasation, but contour irregularity at the level of the mid-GDA was noted. Prophylactic coil embolization of GDA was performed achieving GDA stasis. Post-embolization angiography of the SMA demonstrated no evidence of bleeding from IPDA branches. Incidentally noted was a replaced right hepatic artery arising from the SMA.\nDespite embolization of GDA, patient’s hemoglobin continued to decrease to 6.8 g/dl and the patient continued to have melena and hematemesis resulting in hypotension. The patient required an additional nine units of packed red blood cells (pRBCs), six units of platelets, and five units of fresh frozen plasma. Computed tomographic angiography (CTA) showed a large bleed from IPDA off the SMA immediately posterior to the origin of the replaced RHA (Figure ).\nThe patient was referred for urgent embolization by interventional radiology.\nDuring angiography, no active extravasation was identified and given that the GDA was already embolized, the most reasonable source vessel in the suspected territory would be the IPDA. The replaced RHA was selected and IPDA was identified as its first branch (Figures , ).\nWhile the branch arising directly from the replaced RHA represented the posterior IPDA, the anterior IPDA was seen as collateral vessel going back to the middle colic artery (Figure ).\nHowever, no focus of active bleeding could be identified from digital subtraction angiography from the posterior IPDA or the anterior IPDA. Cone beam computed tomography (CBCT) catheter angiography of the posterior IPDA was performed to identify the region of the duodenal bleed analogous to the region of active extravasation seen on abdominal CTA. The same location on the posterior wall of the duodenum seen on abdominal CTA was matched on CBCT angiography (Figure ).\nSubsequently, the posterior IPDA was embolized using n-butyl cyanoacrylate (NBCA) (Trufill n-BCA Liquid Embolic, Codman Neurovascular, Raynham, Massachusetts, USA) mixed with ethiodol 1:4 dilution ratio administered via the microcatheter (Figure ).\nPost-embolization angiography of the SMA demonstrated successful embolization of the posterior IPDA with sparing of the anterior IPDA. The patient became hemodynamically stable post procedure and no longer required blood products transfusion. His hemoglobin stabilized at 8.6 g/dl. The patient was successfully discharged to a skilled nursing facility to continue his recovery. At eight weeks follow-up the patient remained asymptomatic and he agreed to participate in this case report.
A 74-year-old male patient was diagnosed 3 years ago with a superficial spreading melanoma on his back. The tumor had a Breslow thickness of 4.0 mm and there were two positive sentinel nodes in both axilla. After surgical resection, there was progression of disease 1 year later with increased size of lymph nodes in both axilla and retroperitoneal. There was suspicion of a solitary lung metastasis in the left upper lobe. The patient was referred to our tertiary center for escalation of treatment. Molecular testing showed a NRAS tumor mutation but no BRAF tumor mutation. Immunotherapy with a humanized monoclonal antibody against programmed death receptor-1 (PD-1), that is, pembrolizumab, was started.\nA few months later, good clinical response was observed as the lung metastasis and the lymph nodes regressed. After 9 months of treatment with pembrolizumab, the patient developed urticaria and erythema, which were indicated as an adverse effect and successfully treated with hydroxyzine and a topical corticosteroid. The patient was operated on a presumed appendicitis in another hospital. One and a half years later, the patient developed adrenal insufficiency and oral hydrocortisone supplementation was started. A computed tomography (CT) showed stable disease with disappearance of the lung metastasis, therefore pembrolizumab treatment was continued. After a total of 27 treatments, the immune therapy with pembrolizumab was terminated. Shortly thereafter, the patient developed complaints of dyspnea and coughing. Electrocardiography and echocardiography were normal; however, a CT-scan showed bilateral pleural effusion and mesenteric panniculitis. A diagnostic pleural puncture was performed showing exudate with reactive cells; however, no malignant cells were observed. With the work diagnosis of pleural serositis due to immunotherapy, treatment with prednisolone was initiated (60 mg per day). Because of persistent dyspnea, a pleural drainage (1800 mL) was performed 1 week later with good symptom relieving effects. Again, no malignant cells were observed in the pleural fluid.\nTwo weeks later, the patient presented at the emergency room with dyspnea, fever (38.5°C) and cold shivers. He also had complaints of red and tearing eyes. He was hypoxic and suppletion of oxygen was started. Blood laboratory values are presented in . Electrocardiography, chest X-ray, and microbiological diagnostics showed no abnormalities. A CT-thorax was performed, showing pulmonary emboli in the right lung and progression of the bilateral pleural fluid. There were no signs of pneumonitis. After another pleural drainage (1600 mL), the patient was admitted to the general ward with therapeutic anticoagulation by means of low-molecular-weight heparin. During his stay in the ward, the fever persisted and the C-reactive protein was rising (102 mg/L), . Bacterial pneumonia was suspected, for which piperacillin/tazobactam was started and the prednisolone dose was halved (30 mg per day). On the fourth day of hospital admission, clinical deterioration occurred characterized by means of vomiting, severe diarrhea, oliguria, and blue discoloration of the fingers. Despite fluid resuscitation, no improvement was observed and with suspicion on intestinal ischemia or perforation, a CT-abdomen was performed, showing intra-abdominal free fluid, no free air, a slightly distended transverse colon, and no occluded mesenteric arteries.\nOn the fifth day, the patient was in acute respiratory distress and therefore admitted to the ICU, where respiratory support with nasal positive airway pressure was initiated. Striking findings at physical examination at admittance were a convex, tense, and silent abdomen with diarrhea and poor peripheral circulation with extensive mottling of the legs up to the abdomen; a prolonged capillary refill time of more than 4 s; and severe discoloration (blue-purple) of the fingers on both hands. Laboratory findings are shown in . Blood and sputum cultures were taken, antibiotics were continued, and a multi-disciplinary consultation was arranged with the intensivist, oncologist, the rheumatology and clinical immunology specialist, and gastroenterologist. This team had a high suspicion of an irAE.\nAdditional laboratory markers were investigated (including antinuclear, anti-DNA, and antiphospholipid antibodies and complement C3 and C4). Because of abdominal pain, nausea, and vomiting with suspicion on clostridium, colitis or intestinal ischemia additional fecal diagnostics were performed. The fecal cultures were negative together with a negative clostridium difficile nucleic acid amplification test. Fecal calprotectin levels were increased (305 mg/kg), whereas fecal elastase levels were decreased (115 ug/g).\nThe patient underwent a colonoscopy, showing characteristic ischemic colitis in most parts of the colon, whereas the rectum was partly saved. Biopsies were taken and shortly thereafter an explorative laparotomy was performed. Due to extensive intestinal ischemia, 150 cm of ischemic small intestine combined with the sigmoid was resected. Bowel continuity was not restored and a second look laparotomy was scheduled. In the ICU, the patient was sedated, mechanical ventilated, and hemodynamically supported with noradrenaline. Anti-cardiolipin (aCL) antibodies were detected, and together with the clinical picture of progressive multi-organ thrombosis raised the suspicion of CAPS as an irAE.\nOn the third day of ICU stay, the patient was clinically deteriorating with progressive respiratory failure, oliguria, and impairment of the peripheral circulation. Based on frozen section analysis of the small intestine and sigmoid surgical specimens with macroscopic signs of extensive ischemia ( and ()), no clear evidence for a vasculitis could be found. The laboratory diagnostics showed aCL antibodies; aCL IgM: 26 MPL/mL and aCL IgA: 60 APL/mL, raising the suspicion on a CAPS. Lupus anticoagulant (LAC), aCL IgG, and autoantibodies to B2-glycoprotein 1 were negative together with negative previous test results of antinuclear antibodies, antineutrophil cytoplasmic autoantibodies, and antibodies to double-stranded DNA. Complement C3 and C4 levels were normal. All blood and sputum cultures taken during the hospital—and ICU—stay were negative. Due to the rapid disease progression over the past 24 h, plasmapheresis was started and intravenous corticosteroids were continued. Overnight progression of disease was observed with multiple organ failure of the heart, the lungs, the kidney, and the liver and further purple discoloration of the fingers and toes.\nFurther workup of the resection specimens showed ischemic changes in the mucosa were in the submucosa and more extensive in the subserosa; fibrinoid necrosis was seen in the branches of the small and medium-sized arteries ( and ()).\nA second look laparotomy was performed where extensive, deep ischemia of the small and large intestine was found. In the light of severe mesenteric ischemia and multiple organ failure, further care for recovery was deemed futile. The patient died shortly after cessation of supportive therapy and maximum palliative care.\nDuring autopsy, there were no signs of malignancy nor enlarged lymph nodes. In the large vessels, there were no thrombi nor emboli. The small and large intestine showed signs of ischemia macroscopically. Microscopic examination of the sectioned intestines and one adrenal gland showed however small blood vessels with thrombi in different phases varying from only fibrinoid deposits on the endothelium (existence in days) to re-endothelization (existence in weeks) (–()).\nIn the appendectomy specimen from approximately 1 year earlier, there was chronic active, ulcerative inflammation within the peri-appendiceal fat tissue blood vessels with signs of vasculitis with infiltration of the vessel wall; at that time, this was probably associated with the degree of inflammation. By revision, we consider this finding to be related with the more recent findings.\nBlood samples that were taken before this hospital admission were post mortem analyzed for aCL antibodies, showing a raised aCL IgM: 40 MPL/mL and negative aCL IgA antibodies, indicating that the patient was in the process of developing multiple autoantibodies against cardiolipins.
This 11-year-old girl presented with headache for 3 weeks, weakness of all the four limbs for 2 weeks, and rapidly progressive vision loss for 10 days. At 16 months of age (10 years back), she underwent fenestration of posterior fossa arachnoid cyst with ventriculoperitoneal (VP) shunt insertion when she had become lethargic and lost motor milestones. She improved and returned to baseline and was doing fine.\nThree weeks before presentation to our center, she developed persistent frontal headache and vomiting which progressively increasing. On the 7th day of illness, she was evaluated elsewhere by an ophthalmologist. Visual acuity was reportedly normal. There was no papilledema. Magnetic resonance imaging (MRI) was done, which showed a posterior fossa arachnoid cyst with VP shunt in situ and normal-sized ventricles []. Cerebrospinal fluid (CSF) examination revealed acellular CSF with normal biochemistry, but opening pressure was not measured. She also had aching over the limbs. Headache decreased after the CSF drainage (25 mL).\nDuring hospitalization, she developed symmetrical proximal as well as distal weakness of all the four limbs without sensory loss. She also developed difficulty in chewing and jaw drop without facial sensory loss. There was left-sided facial, palatal, tongue, and neck weakness. Eleven days after the onset of headache, she developed horizontal binocular diplopia. Then, she started having diminution of vision in both the eyes, which progressed over 5 days to complete blindness with worsening of headache. There were no seizures, ataxia, fever, and loss of weight or appetite. There were no other systemic complaints. She received pulse methylprednisolone with no improvement. At presentation at our center (3 weeks after onset of illness), she was conscious, drowsy, and had a normal general physical examination and hemodynamically stable without any features of Cushing's triad but had neck stiffness.\nExamination revealed absent perception of light bilaterally without papilledema. Pupils were dilated and not reacting to light, with ptosis of the right eyelid with bilateral impaired abduction of the eyeballs. Facial sensation was normal with diminished corneal reflex bilaterally, with jaw weakness. There was no facial weakness, palatal, or tongue weakness. Neck flexors as well as extensors were weak.\nThere was symmetrical flaccid quadriparesis. All deep tendon reflexes were absent and plantars were extensor. Sensory examination was normal. Finger–nose test was normal and tandem walking could not be evaluated.\nRoutine investigations were normal. MRI of the brain was done []. Lumbar puncture showed elevated CSF pressure >40 cm CSF. CSF was acellular with sugar of 63 mg/dL (corresponding Random blood sugar (RBS) of 87 mg/dL), protein was 26 mg/dL. Other investigations such as CSF culture, acid–fast bacillus staining, malignant cytology, cryptococcal antigen, and GeneXpert were negative. After CSF tapping of 50 mL, her weakness and cranial nerve deficits except vision improved and she also had relief in headache. Visually evoked potentials were not recordable. Nerve conduction study was suggestive of radicular involvement.\nClinically, she had a meningeal process with contiguous involvement of the cranial nerves on the left side, bilateral 6th nerve involvement as well as polyradiculopathy. Possibility of chronic meningitis, shunt malfunction, or other causes of raised intracranial pressure (ICP) such as chronic sinovenous thrombosis was considered. ICP rise due to idiopathic ICP or drug-induced causes were ruled out because modified Dandy's criteria for IIH were not fulfilled. Moreover, since the shunt was already in situ, if it was functional, the patient was unlikely to be having features of raised ICP. Thus, shunt malfunction was diagnosed.\nSince the patient had loss of vision as well as multiple deficits, lumbar thecoperitoneal shunt (LP shunt) insertion was performed to relieve the raised ICP immediately. LP shunt was preferred in this case over VP shunt revision, as the shunt revision was technically difficult in normal-sized ventricles, and our center has a large experience in LP shunting in patients with malignant idiopathic intracranial hypertension.\nAfter surgery, patient's limb power improved. She also started walking on her own, and all other cranial nerve deficits got relieved, except for vision loss which was persistent. Subsequently, lumbar puncture was repeated, which showed CSF opening pressure of 5 cm CSF and was clear and normal biochemically. Even after 1 year of follow-up, there was no improvement in vision. Vision did not improve probably due to the long duration of symptoms before intervention was done.
A 37-year-old female with a known history of neuroendocrine carcinoma of the cervix (NECC) for the past 11 months who had undergone a partial hysterectomy presented to the neurosurgical service with a severe headache unresponsive to medical treatment after recently undergoing a follow-up completion hysterectomy with pelvic lymph node dissection. Figure presents imaging and pathology findings. A CT head without contrast demonstrated florid obstructive hydrocephalus and several intra-axial lesions occupying the pineal region, left frontal lobe, and posterior fossa (Figure ). A right frontal ventriculostomy was placed to temporize the hydrocephalus. Due to the degree of brainstem compression and the need to obtain a pathologic diagnosis, it was decided to proceed with a posterior fossa craniotomy for the removal of a single lesion. The frozen pathology yielded a small blue cell tumor with positive neuroendocrine markers cluster of differentiation 56 (CD56), chromogranin, and hematoxylin and eosin (H&E) stain consistent with a prior diagnosis of neuroendocrine carcinoma of the cervix (Figure ). The remaining lesions were left alone given the biologic responsiveness of neuroendocrine carcinoma to radiation therapy. An endoscopic third ventriculostomy (ETV) was then performed to remove the ventricular drain. The patient was then discharged from the hospital. She then went on to receive whole-brain radiotherapy (WBRT) for a total of 30 Grays (3000 cGy) over 10 fractions on an outpatient basis. A follow-up CT head without contrast was obtained (Figure ) that demonstrated the resolution of the pineal and left frontal lesions. The patient returned for follow-up ambulatory without any major concern. Subsequently, she presented to the oncologist with quadriparesis three months later. Magnetic resonance imaging (MRI) was obtained, which demonstrated drop metastasis in the cervical, thoracic, and lumbar area (Figure ) most likely related to biopsy, explaining the quadriparesis presentation. She had further spinal radiation of 30 Grays (3000 cGy) over 10 fractions. Her neurologic function improved to antigravity in the upper extremities, however, she remained paraplegic. Palliative care and hospice followed.
A 66-year-old woman with a significant past medical history of well-controlled hypertension was admitted with complaints of microscopic hematuria and mild proteinuria for the past 3 years. Serum creatinine level was within normal range at that time and therefore the anti-GBM antibody was not tested. The first renal biopsy revealed mesangial proliferative glomerulonephritis with fibro-cellular crescents in one out of 18 glomeruli, excluding one global sclerotic glomerulus (Fig. ), and deposition of IgA and C3 in mesangial areas by immunofluorescence microscopy (Fig. ). Weak but significant IgG deposition was also observed in glomeruli in the distribution somewhat different from IgA or C3 (Fig. ). The electron-dense deposits were observed in mesangial areas by electron microscopy. Therefore, the diagnosis was IgA nephropathy. Antihypertensive therapy was initiated, mainly with an RAS inhibitor. Eight months later, the patient’s serum creatinine suddenly rose to 4.53 mg/dL (it was 1.04 mg/dL from the routine blood test 1 month before). Urinalysis showed 100 red blood cells per high power field and urinary protein excretion of 12.3 g/gCr (Fig. ). The serological tests that were performed to differentiate the cause of rapidly progressive glomerulonephritis revealed the presence of anti-GBM antibody at the titer of 116 IU/mL and the absence of anti-nuclear antibody and anti-neutrophil cytoplasmic antibody. Laboratory findings on admission are summarized in the Table .\nAfter admission, treatments with hemodialysis, plasma exchange, and intravenous methylprednisolone pulse therapy followed by oral prednisolone at the dose of 50 mg/day were initiated. The second renal biopsy was performed at 4 weeks after admission in order to assess the probability of renal recovery and to make the final diagnosis. It revealed cellular to fibrocellular crescents in 18 of 25 glomeruli, excluding six global sclerotic glomeruli by light microscopy. By immunofluorescence study, linear IgG deposition along the glomerular capillary walls and mesangial staining for IgA were observed. On the other hand, C3 deposition was observed in the mesangium as well as in the glomerular capillary walls (Fig. ). Electron-dense deposits were observed in mesangial areas, similarly as in the first biopsy, by electron microscopy (Fig. ). Based on the aforementioned findings, the diagnosis of anti-GBM glomerulonephritis and IgA nephropathy was confirmed. Plasmapheresis was performed eight times, anti-GBM antibody gradually decreased, and alveolar hemorrhage was prevented. However, her renal function could not be restored and she underwent maintenance hemodialysis (Fig. ).\nAdditional immunosuppressant was not given because the patient did not show any sign of pulmonary involvement and because the renal recovery was quite unlikely from clinical (continuous oliguria and hemodialysis dependence) as well as histological (crescent formation in most of non-sclerotic glomeruli) point of view.\nClinical and histological presentations from IgA nephropathy (at the time of first renal biopsy) and from anti-GBM disease (at the time of second renal biopsy) were summarized in the Table .
A 47-year-old man, who had presented with acute abdominal pain to a different healthcare facility, was transferred to our emergency room for further evaluation and treatment of dissection of the splanchnic arteries. The patient had a history of uncomplicated dissection of the superior mesenteric artery, and had been taking anti-hypertension medication for 1 year. However, he had stopped taking his prescription medication 10 months prior to admission to the emergency room. On arrival to the emergency room of a local hospital, his blood pressure was 95/60 mm Hg and the pulse rate was 110 beats per min. Laboratory test revealed a hemoglobin level of 10.5 g/dL. Other blood chemistry parameters were within the normal range. After blood transfusion and fluid therapy in local hospital, the patient's blood pressure had increased to 150/100 mm Hg, and there were no obvious abnormalities on the physical examination or in the laboratory findings on admission to our hospital. A contrast-enhanced CT scan showed a newly developed saccular dissecting aneurysm in the celiac artery with surrounding retroperitoneal hematoma, measuring 2.8 × 7.9 × 12.0 cm (). The dissection of the superior mesenteric artery had not changed, compared to the previous CT scan. One day after admission, the patient's hemoglobin level had decreased to 9.6 g/dL again, after cessation of blood transfusion. The patient was offered a less invasive endovascular procedure to avoid surgical repair.\nAfter puncturing of his right common femoral artery, an 8F guiding catheter (Vista brite tip, Cordis, Miami, FL, USA) was inserted into the orifice of the celiac artery, which allowed for celiac angiography that revealed a dissecting aneurysm at the entry point (). The proximal splenic artery was occluded, and the true lumen of the left gastric artery and common hepatic artery were narrowed by intramural hematoma. We placed the 4-9 × 38 mm peripheral stent graft (Jostent, Abbott Vascular, Rangendingen, Germany), which was mounted over a 6 × 40 mm savvy balloon (Cordis, Miami, FL, USA) from the origin of the celiac artery to the common hepatic artery. Post-procedure angiography showed complete coverage of the dissecting aneurysm with the use of a stent graft (). The left gastric artery was visualized by collateral flows from the right gastric artery. The postoperative course was uneventful with normalized hemoglobin level without blood transfusion. Follow-up CT scan, obtained 1 week after the procedure, showed a complete obliteration of the dissecting aneurysm in the celiac artery with preservation of the hepatic arterial flow and marked reduction of the retroperitoneal hematoma. Although occlusion of the proximal splenic artery and proximal left gastric artery after stent graft placement persisted, no abnormalities (i.e., splenic infarction, gastric ischemia, or signal of irritated peritoneum) were noted due to an abundance of collateral blood flow.
A 22-year-old man presented with a 3-day history of fever of 39°C and cough producing yellow sputum. He also appeared general malaise with nausea and vomiting. On examination, the man had rough breath sounds. An abdominal examination revealed mild epigastric tenderness without other definite positive signs, and the patient denied have had previous episodes with abdominal pain. A chest roentgenogram suggested bilateral pneumonia. His leukocyte count was elevated at 16×109/L. He had a history of diabetes for 5 years, which was treated with insulin. He was diagnosed with pneumonia. Unexpectedly, during the 3 days of treatment with azithromycin in the Department of Pneumology, his fever never decreased. He gradually developed right lower quadrant pain with guarding and rebound tenderness. Reexamination revealed an 8×8×6 cm mass. Ultrasound showed decreased echo owing to inflammation. Abdominal computed tomography (CT) revealed gas-liquid levels and dilatation of the intestinal tract (). The appendiceal area was obscured. According to the above examination, we judged that he already had a periappendiceal abscess at that time. The leukocyte count increased to 23×109/L at this time. Then, the patient was transferred to the Department of General Surgery with conservative treatment according to his long medical history and the formation of the appendicular abscess. We used cefoperazone sodium and sulbactam sodium combined with ornidazole. After 3 days of conservative treatment failed, surgery was performed via a right rectus abdominis incision. There was 500 mL of yellow-white festering around the paracolic sulcus. The tenia coli were traced down to their confluence, and the base was identified with a perforation. We decided to determine whether it was terminal, but we separated more than 10 cm without locating the terminus. Due to the severe adhesions, we used silk to bundle the appendix and cut it into two parts (). Then, we separated it to a size of approximately 5 cm and we were surprised to find that the other base grew at the first appendix’s frontal disposition. The diameter of the longer appendix was 1.0–2.0 cm, and that of the shorter one was 0.5–0.8 cm. Both appendix bases were perforated, and we bundled them (). The central mesenteric vessel was fan shaped ( and ). We distinguished the central mesenteric vessel from the ureter and bundled it. Both appendices were resected. The appendix was sent for pathological examination. It reported that there was marked neutrophilic infiltrated within and around the appendix. Two appendix bases were perforated and had their lumens both communicate with cecum. The postoperative recovery was uneventful except for slight hoarseness that recovered after 5 days, and the patient was discharged on postoperative day 9.
A 22-year-old female was admitted our hospital with a gradually enlarging right axillary pulsatile mass. She complained of right upper extremity weakness, and coldness and pain after exercise. She had a history of right axillo-thoracic blunt trauma three months ago. She had noticed the mass 2 months before, and it was gradually enlarging since that time.\nIn our physical examination we found an about 3 × 4 cm solid, palpable pulsating mass in the right axillary region. Brachial blood pressures were 90/50 mmHg on right side and 110/75 mmHg on left side. Upper extremity arterial pulses in right side were poor in comparison with the left side. On auscultation a systolic murmur which was spreading to right axillary region had been heard on the mass. Neurological examination showed a mild loss of sensation in the left arm. There were no signs of vasculitis or connective tissue diseases associated with arterial involvement such as hyperelastic skin, hypermobile joints, or marfanoid habitus. Laboratory examinations, including erythrocyte sedimentation rate, C reactive protein, complete bloodcount, serological test for syphilis, rheumatoid factor, antinuclear antibody, antithrombin III, protein C, and protein S, were normal except for hypercholesterolaemia. A color Doppler ultrasound suggested the presence of an axillary artery aneurysm. Digital subtraction angiography confirmed a 4 × 6 cm fusiform aneurysm at the distal part of right axillary artery (). Thoracic outlet syndrome was excluded by means of careful clinical, radiological, and electrophysiological examinations.\nUnder general anesthesia, we exposed the aneurysm by using the subpectoral-axillary approach. We encountered a large amount of organized thrombus when entering the aneurysm sac. An embolectomy was done using a Fogarty catheter but no embolism was detected. The aneurysm was excised. An adequate calibrate and length saphenous vein graft was interposed between the two transected segment of the axillary artery (Figures and ). After the operation all of the right upper extremity peripheral pulses were pulsatile and both upper extremities blood pressures were approximately equal (115/75 mmHg and 110/75 mmHg resp.) at brachial level. The postoperative course was uneventful and the patient was discharged on the fifth postoperative day. She was symptom-free at follow-up three years later.\nHistopathological study of the specimen obtained from the aneurysmal sac revealed that all three layers of the arterial wall were intact. Degeneration and fibrosis were seen in the media layer ().
The second case involves a 30-year-old Deaf, Hispanic male who presented to the Emergency Department after his mother reported that the he was behaving oddly and not taking his risperidone. Per reports, the patient was talking to his mother about going places in a UFO and exhibiting disorganized and illogical behaviors. He was subsequently placed under a Baker Act by the emergency room physician who documented that the patient was exhibiting auditory hallucinations. Initially an interpreter was brought to the hospital prior to his admission. Per the ASL-interpreter, the patient stated that he felt “fine and not crazy” and that all of these events are happening because his mother does not “understanding Deaf culture.” He also conveyed that he did not like to take his meds because they interfered with him being able to drink alcohol and caused drowsiness.\nUpon initial psychiatric interview an interpreter was not present as the hospital only agreed to set periods of time for the interpreter. As an effort to communicate, questions were prepared for the patient to answer via written responses. highlights a portion of the questions and answers that were constructed. From the responses he maintained bizarre delusions but denied current SI, HI, or AVH. When the ASL-interpreter arrived, the patient appeared jovial and yearned to express himself. The interpreter stated she had difficulties reading his rapid signing at first and had to have him slow down several times. However she did note that this was a common occurrence when addressing Deaf individuals.\nWith the interpreter's assistance, the patient was answering questions logically with a linear thought process. He reported that he had been diagnosed with schizophrenia as a teenager after having several interpersonal issues with his mother. She is Spanish speaking only and he stated that she has never fully understood how to communicate effectively with him. He had been taking risperidone for several years but was tired of continuing with the medication due to the side effects of drowsiness and weight gain, which he was never able to fully discuss with his psychiatrist. Patient reported he was in an ASL school and learning a career in massage therapy. After meeting a girlfriend there he began to develop a sense of independence that he reported his mother disapproved of. This caused an altercation that he reports his mother misinterpreted which precipitated his admission.\nThe patient continued to express that he was abducted by aliens as a child and could understand their language, but besides this he expressed no other psychotic processes. He was observed for two days without medications and remained calm/cooperative but was unable to participate in most activities due to limitations of the interpreter availability. After a family session was completed the patient was discharged home with plans to follow up with his community psychiatrist. The patients' diagnosis was changed to delusional disorder upon his discharge.
This 22-year-old male patient intermittently suffered from hay fever in summer with symptoms of cough and dyspnea, however as his symptoms were minor, he did not undergo any special check-up, evaluation or treatment. When he was studying in college, he had symptoms of gradual backache and radiating pain in both lower limbs, but as symptoms were minor, he did not seek any examination. In November 2011, he started his military service as a conscripted policeman and experienced several rounds of difficulty in breathing and received conservative treatment. In January 2012, he showed severe dyspnea, coughing, chest pain, and muscular weakness in the lower limbs. After the pulmonary function test in a private hospital, he was diagnosed with asthma, so he was hospitalized and treated with a β-agonist and steroid injection. After 2 days, the symptoms continued so he was transferred to the emergency room of our hospital. After treatment in our hospital, his symptoms improved compared to the results of the private hospital's pulmonary function test, as shown by the forced expiratory volume of subnormal 1 second (FEV1) from 1.13 L (32% of predicted value) to 3.50 L (78% of predicted value), the FEV1/forced vital capacity (FVC) from 46% to 77% and forced expiratory flow (FEF; range, 25%-75%) from 11% to 67%. His symptoms of dyspnea and chest pain gradually improved, but he continued to show muscular weakness in both lower limbs. The muscle tone of both upper limbs was within the normal level, but the muscle tone of both lower limbs was accelerated, showing increased deep tendon reflex in the knee and ankle joints, while exhibiting ankle clonus for both ankles. A neurogenic lesion in the upper part was suspected. According to manual muscle test, muscular strength of both upper limbs was normal, and there was no difference in muscular strength between both sides of lower limbs. However, the flexor/extensor muscles of the hip and knee joints were 1/5, and the flexor muscle of the instep/sole had been decreased to 0/5. Moreover, spasticity of both lower limbs was level 1 for ankle dorsiflexion on the modified Ashworth Scale.\nBlood test during his hospitalization showed a potassium level of 3.8 mEq/L; thyroid function test did not show any unusual findings with T3 of 57.7 ng/dL, thyroid-stimulating hormone of 1.15 µIU/mL, and free T4 of 1.38 ng/dL. He did not have a high fever, headache or meningism symptoms including Kernig's sign and Brudzinski's sign, so lumbar puncture test was not performed. From lumbar magnetic resonance imaging (MRI) which was done on the third day of hospitalization in our hospital, a severe stricture and compression were observed from L2 to S1 due to synechia and a cystic mass of posterior intraspinal space (). After 1 day of test, neurosurgical department of our hospital diagnosed him with lumbar cord damage, and then conducted emergency decompression surgery. Since a space between L2 and S1 was filled with a ruptured epidural cystoma, dead skin cells, and cholesterol type substances, they compressed severely the dura mater and nerves. Adhesion in the dura mater and the back spinal cord was also observed, however as it was located outside the dura mater, compressing substances were comparably easily and perfectly removed. Histological examination of the removed substances confirmed a ruptured epidermoid cyst together with simple squamous cells, dead skin cell components, and dystrophic calcification (). As the patient did not undergo back surgery or rachicentesis, he was diagnosed with a congenital epidermoid cyst.\nSeven days since the operation, the patient was transferred to our rehabilitation department; when he was transferred, manual muscle test showed no big difference in the muscular force of both lower limbs, while the flexor/extensor muscles of the hip and knee joints was 2/5 and the flexor muscle of instep/sole was 1/5. He was recovering, but, except for changing positions, sitting by himself and moving around in the wheel chair, his physical strength became weaker such that he needed help from other people to perform everyday activities.\nWhen he was transferred, the Functional Independence Measure (FIM) was 94, the Korean version of Medified Barthel Index (K-MBI) was 56 and the Spinal Cord Independence Measure (SCIM) was 58. According to results of his neurologic examination, he showed hypesthesia and muscle weakness from the dermatomere of L2, while anal contraction could be done by himself. Based on the motor nerve and sensory nerve, a neurologic damage part was L1 in paresis and could be classified as C in the American Spinal Injury Association (ASIA) Impairment Scale. From the initial period of hospitalization, a urethral catheter was inserted due to exacerbation of his entire body, so it was difficult to recognize urination symptoms, yet bulbocavernous reflex existed and was under drug treatment due to symptoms of slight constipation. Before the transfer, he was able to urinate by himself after removing the catheter, but exhibited residual urine and intermittent urinary frequency symptoms. After drug treatment, his symptoms improved. Electrodiagnosis was taken 1 week after the transfer (after 3 weeks since the operation); conduction test of the motor and sensory nerves showed normal latency and amplitude in both lower limbs, while the latency of both sides' H-reflexes was delayed. By conducting somatosensory evoked potential of both tibial nerves and motor evoked potential at both abductor hallucis muscles by stimulating the head, we could observe a delay in the transcranial area (). In the needle electromyographic test, we observed a high level of abnormal spontaneous activities in the paravertebral muscles and lower limb muscles of that particular segment, demonstrating complications of conus medullaris syndrome and radiculopathy of the lumbosacral area (). Due to improved symptoms, the patient declined further examination, so tests for pudendal somatosensory evoked potentials and bulbocavernosus reflex latency were not done.\nDuring the hospitalization period in our hospital, the patient continued to exercise to strengthen the muscular power of the lower limbs through stimulating the functional nerve roots, standing up and gait training. Three months after the operation, the results of the manual muscle test showed the hip joint flexor, knee joint extensor and instep flexor muscles as 4/5, while the hip joint extensor, and knee joint flexor and sole flexor muscles as 3/5. The patient was able to walk a short distance on the flatland using a walker (). Before discharge, he scored 113 for FIM, 87 for K-MBI and 86 for SCIM. Currently, he is hospitalized for the second time for continual rehabilitation treatment and is under continual ambulatory observation by our department. He can now walk on the flatland using a quadripod cane.
A 62 years old male presented with recurrent SCC of the left nasal bulbar conjunctiva. The patient's previous medical history detailed multiple interventions at this site. Initially, the diagnosis of a left nasal pterygium (a wing-shaped growth that starts on the conjunctiva and can spread across the limbus to the cornea affecting visual function) was made 3 years prior to the reported presentation, and was surgically removed with local excision. The lesion recurred 1-year post-surgery, and was subsequently managed surgically with a wide local excision. At this time, pathology revealed positive margins for SCC. A further recurrence 6 months later was treated with Plaque Therapy to a dose of 50 Gy in 5 fractions. Subsequently, the lesion was re-excised with adjuvant cryotherapy 7 months later.\nOn presentation 3 months post-cryotherapy, a recurrent lesion (10 × 5 × 2 mm) was detected on the nasal bulbar conjunctiva (Fig. ). The patient declined enucleation following this SCC recurrence. Subsequently, multidisciplinary consultation offered SXRT as an alternative treatment option (Fig. ).\nThe patient also presented with a history of Crohns Colitis (treated with immunotherapy) and a previous excision of a non-melanoma skin cancer from the right temple, and a subsequent skin graft of the area. The patient was on no other medications and had no allergies.\nThe patient was prescribed a dose of 48.4 Gy in 22 fractions, to a depth of 3 mm. This resulted in a skin surface dose of 54.4 Gy. A 2.0 cm diameter direct applicator was used with a 3 mm margin on the treatment region (Fig. ). To allow for an acceptable treatment margin that encompases the target volume and accounts for set up variability, the left inner canthus and tear duct were included in the treatment field. A single en-face beam of 2.0 mm Aluminium energy (2 mm Al) was used to meet the previously articulated dose prescription. The risk of ulceration, scleral perforation, ischemia and nasolacrimal duct obstruction was explained to the patient. This risk was further exacerbated in this case due to re-irradiation of the previously treated area, and a particularly sensitive region post-cryotherapy, surgery and plaque therapy. Topical anaesthesia (Tetracaine eyedrops) was administered to the left eye prior to retractor positioning, to ensure eyelids remained open throughout SXRT delivery. After positioning of the retractors the patient was instructed to maintain gaze on a target positioned to the left side of the room, and the eye was monitored throughout treatment delivery by video surveillance. Lubricating eye drops were prescribed for use prior to treatment administration to prevent drying of the ocular surface, and for the patients self-use between fractions to relieve dry eye symptoms. Total set up and treatment time for this patient was approximately 20 min, with the retractors in place on average less than 10 min. The anaesthetic drops and ocular surface lubricants induced limited sensation and the patient found it mildly uncomfortable. As a precaution, the patient was informed to avoid contact with the anaesthetised eye and wear a protective patch for 60 min post-treatment delivery daily.
A 69-year-old female presented with chest pain, loss of appetite, and abdominal pain. Upper gastrointestinal endoscopy showed a patch of indurated mucosa of size 10 mm x 10 mm over the proximal body of the stomach along the lesser curvature. An excavated ulcer of size 30 mm x 30 mm was noted in the distal part of the body in the anterior wall along the lesser curvature with edematous adjacent mucosa. The margins of the ulcer appeared rolled out. The first part of the duodenum showed ulceroinfiltrated mucosa with a sloughy base. The second part of the duodenum appeared unremarkable. Contrast-enhanced computed tomography (CECT) of the abdomen showed diffuse circumferential thickening of the body and antropyloric region of the stomach (Figure ). Multiple conglomerated necrotic lymph nodes, the largest measuring 4.3x3.2 cm, were seen in the lesser sac region (Figure ). Numerous irregular hypoechoic deposits over the hepatic and splenic surfaces were observed (Figure ). Based on these findings, gastric carcinoma with nodal spread was suspected. CT chest showed bilateral mild pleural effusion with the collapse of the basal segments. Pleural fluid was aspirated and sent for cytological study. The available hematological and serum biochemical parameters were within normal limits (Table ). She did not have a history of intake of any steroids or other drugs. Her retroviral serology was negative. There were no known comorbidities either.\nThe pleural fluid cytological smears (both wet-fixed and air-dried) showed atypical lymphoid cells of varied morphology, including plasmacytoid cells with reactive mesothelial cells and lymphocytes in the background. There were cells with a moderate amount of cytoplasm and indented and multilobulated nuclei (Figure ). A good number of apoptotic bodies and mitotic figures were noted. The cytological examination was interpreted as lymphomatous effusion with advice for flow cytometric analysis of the fluid. However, the test could not be performed due to financial constraints.\nThe histopathological section from the stomach biopsy showed gastric mucosa infiltrated by a poorly differentiated tumor (Figure ). These cells had a moderate amount of cytoplasm with varied morphology and a prominent nucleolus in some of them. Many apoptotic bodies were noted. The morphological findings on biopsy are well-correlated with those in the fluid smears. Hence, a high-grade non-Hodgkin lymphoma was suggested and immunohistochemical stains were performed for further categorization. Immunohistochemistry for LCA, Cytokeratin, CD3, and CD20 was negative. The antibodies used in the second panel, which showed a positive reaction were CD38 (Figure ), CD138 (Figure ), MUM 1 (Figure ), and Kappa (Figure ). CD79a, CD56, CD30, CD5, CD7, ALK 1, and EMA were proved to be negative. The Ki-67 proliferation index was 95% (Figure ). Finally, a diagnosis of gastric plasmablastic lymphoma was given. Subsequently, bone marrow involvement was seen with 60% plasmacytoid cells (CD38, CD138, and Kappa positive). The patient died within one month of diagnosis.
A 6-year-old, female neutered Rhodesian Ridgeback was presented with a 1.5 year history of right forelimb lameness. No previous examinations had been performed. The dog showed a markedly shortened stance phase in its gait at a walk and trot. Significant muscle atrophy of the right shoulder muscles was present. Orthopaedic examination of the right front leg elicited repeatedly a strong pain response on flexion of the shoulder joint with simultaneously digital pressure medial to the greater tubercle in the region of the biceps brachii tendon, while flexing the shoulder the elbow was held in a ‘neutral standing angle’. No joint distention of the right elbow was present and no pain response was found during manipulation of the joint and palpation of radius and ulna. Neurological examination revealed no abnormalities.\nRadiographs of the shoulders revealed no abnormalities. The extended lateral view of the right elbow showed an abnormally shaped MCP: instead of the normal concave form, a more steep to convex delineation was present. The MCP was blurred and there was a radiolucent area at the level of the radial head surrounded by a heterogeneous zone. The ulnar notch showed sclerosis (Fig. ).\nThe dog was referred for diagnostic imaging to the Ghent University Veterinary Faculty, where additional radiographic projections of both elbow joints were made. The previous findings were confirmed and, based on the two radiographic examinations, the presumptive diagnosis of FCP was made.\nIn addition, CT examination of both elbows was performed. This revealed abnormally steep delineation of the MCP. The sub-trochlear notch showed sclerosis, and a small fissure was visible at the level of the MCP (Fig. a). On the more distal transverse CT images (Fig. b), demineralization of the MCP associated with several opacities was distinct. These findings were also visible on the sagittal and dorsal reformatted images (Fig. c, d).\nClinical and imaging findings suggested the presence of MCP disease, although some atypical findings were present: extensive demineralization associated with several areas with marked new bone formation. Due to these atypical findings, it was decided to conduct a histopathological examination in addition to surgical treatment of the coronoid lesions.\nFor surgery, a standard medial approach to the elbow joint was used []. Macroscopically, only a bone defect at the height of the MCP was noted. The cranial delineation of the MCP was no longer visible. The size of the defect was not measured, but a subtotal coronoidectomy was performed, extending distally 15 mm and caudally 6 mm. The osteotomy surface showed no visible abnormalities. The recovery was uneventful. Meloxicam (MetacamR, Boehringer Ingelheim) [0.1 mg/kg once a day per os (po)] was given post-operatively. At 2 weeks post-operatively the lameness substantially decreased. After explanation of the results of pathology at 6 weeks post-operatively the owner declined any follow up examinations and chose to continue the meloxicam as long as this medication could alleviate the pain sufficiently.\nFour specimens of the excised bone (15 × 6 mm) were sent for histopathological evaluation. The samples were fixed in 10 % neutral buffered formalin and transferred to a formic acid solution for decalcification. After 3 days of decalcification the tissue samples were routinely processed, paraffin embedded, sectioned at 5 μm and stained with hematoxylin and eosin (HE) for histologic evaluation by a board certified veterinary pathologist. Histopathological evaluation of the tissue samples of the proximal ulna at height of the MCP revealed a non-encapsulated, moderately demarcated, invasive mesenchymal neoplasm, extending through the cortex and through the bony trabeculae into the medulla. The neoplastic cells showed moderate anisocytosis and anisokaryosis, they were spindle-shaped with a small amount of eosinophilic cytoplasm, with distinct cell borders and the cells were arranged in streams. There was one mitotic figure in ten high-power fields (400× magnification). The nuclei were ovoid with a stippled chromatin pattern and contained a single, large, centrally located nucleolus. Multinucleated giant cells were scarce. The neoplastic intercellular matrix was composed of small irregular deposits of hyaline eosinophilic material consistent with osteoid (Fig. ) that multifocally revealed a moderate amount of mineralization. Also larger areas with cartilaginous matrix were present within the neoplasm.\nBased on previously published criteria [], the neoplasm was diagnosed as a chondroblastic OS. Based on grading criteria defined by Kirpensteijn et al. [], the dog was classified with low-grade II OS. The edges of the histopathological samples were not free of tumour cells.\nSeveral options—including amputation of the right forelimb, chemotherapy or radiation—were suggested to the owner, but were refused. The dog remained slightly lame in the right front leg during 9 months postoperatively despite daily administration of meloxicam. Obviously right forelimb lameness returned after 10 months. As the OS had not been completely excised based on histopathology margins, local recurrence of the tumour was suspected. On the medio-lateral and cranio-caudal radiographs of the right elbow, fulminant new bone formation around the area of the MCP was visible (Fig. ). This radiographic appearance differed from the expected post-operative images seen after subtotal coronoidectomy, namely a stump coronoid process with little or no bone reaction. The radiographic changes in this case were suggestive of a recurrence of the OS. Biopsy and further therapeutic options were refused by the owner. The dog was treated with daily meloxicam (MetacamR, 0.1 mg/kg once a day po) to relieve the clinical signs.\nTwo and a half years after tumour resection, the dog was presented with severe dyspnea and the right elbow was swollen and painful. Thoracic radiographs showed several large lesions in the lungs suggestive of metastases. The owner requested immediate euthanasia and declined any post-mortem examination.

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