Long esophageal stricture in Crohn's disease: a case report

Crohn's disease of the esophagus is rare, and it is very unusual for it to be located only in the esophagus. We report a case of Crohn's disease confined to the esophagus in a 26-year-old female. The patient was admitted because of progressive dysphagia, odynophagia and weight loss. A barium-swallow examination showed an irregular narrowing of the esophagus below the level of the aortic arch which was 15 cm long, with marginal ulcers and a pseudopolypoid appearance of the mucosa; a computed tomographic scan of the thorax revealed a thickened esophageal wall. Esophagoscopy revealed an esophageal stricture 25 cm distal to the incisor teeth, 2 mm in diameter, with "punched out" ulcers and pseudopolypoid mucosa. Endobiopsy specimens showed chronic lymphocytic infiltration into the corion in the absence of neutrophils, basal-cell hyperplasia and elongation of the stromal papillae. The patient underwent an esophagectomy through a combined cervico-abdominal approach followed by a cervical esogastrostomy. The specimen was 18 cm long, the thickness of the wall was 1.7 cm with fibrosis involving all layers of the esophageal wall and a cobblestone appearance of the mucosa. A heavy lymphoplasmocytic infiltrate extended from the mucosa deep into the muscularis, fibrosis and granulomas were found transmurally. Crohn's disease of the esophagus is a rare and specific entity which can present in various ways; strictures resembling those from reflux esophagitis or a tumor are common. Diagnosis may be suggested by the presence of a chronic lymphocytic infiltrate with or without non-caseating granulomas, and no histologic evidence of chronic reflux esophagitis.     

Bladder cancer associated with von Recklinghausen's disease: a case report

A 53-year-old man was admitted to our hospital with urinary frequency and miction pain. He had von Recklinghausen's disease with multiple café-au-lait spots and neurofibromatosis. Computed tomography scan and magnetic resonance imaging revealed an invasive bladder tumor 10 cm in diameter, and not metastasis. He was diagnosed as having a bladder tumor (T3a N0 M0 with von Recklinghausen's disease. After balloon occluded arterial infusion (BOAI) chemotherapy, total cystectomy was performed. Pathological diagnosis was transitional cell carcinoma, G3, pT3aN0M0. We reviewed and discussed 97 cases of carcinoma associated with von Recklinghausen's disease reported in the Japanese literature. Only 5 cases of bladder cancer have been reported, including the present case.     

Menetrier's disease: report of a transient case associated with chylous ascites

We present a case of transient Menetrier's disease (MD) that was associated with chylous ascites. Using immunohistochemistry, we studied the expression of transforming growth factor alpha in this patient's gastric mucosa biopsy over time; a growth factor that has previously been shown to play an active role in the pathogenesis of MD. Excessive expression and altered localization of transforming growth factor alpha was observed while the patient had active disease with return to the normal pattern after resolution of the disease. This case is the first one reported of transient MD associated with chylous ascites; it lends further credible evidence to the concept that altered transforming growth factor alpha expression may play an important role in the pathogenesis of MD.     

Jejunal perforation caused by blunt abdominal trauma in a patient with Crohn's disease: report of a case

We report herein the case of a 23-year-old man with Crohn's disease who was found to have a perforated small bowel following blunt abdominal trauma sustained in a traffic accident. The general findings of diffuse peritonitis were identified by physical examination, and a plain X-ray film showed free air in the abdominal cavity. An emergency laparotomy was performed which revealed three perforated ulcers in the affected intestine. An abrupt increase in intraluminal pressure due to the striking force of the steering wheel to the abdomen was assumed to have been the cause of these perforations.     

Myoclonus associated with hyperthyroidism and thymoma: a case report

Movement disorders associated with hyperthyroidism or thymoma are extremely rare. A 52-year-old woman had developed myoclonus since June, 1989. Hyperthyroidism and thymoma were diagnosed 1 month and 9 months later. Myoclonus improved after thyroid treatment and thymectomy. If the association was not only a coincident phenomenon, autoimmunity should be considered in this previously unrecognized association.     

Syringomyelia associated with posterior fossa cyst: a case report

We report a case of a 47-year-old woman with a posterior fossa cyst associated with syringomyelia and hydrocephalus. Her birth was traumatic and she had suffered a fractured skull. About 10 years prior to coming to our department she had occipitalgia and a pain had developed over a month from her right shoulder to hand. Shortly before presenting occasional electric-like shocks were felt in her right hand associated with coughing and hiccuping. Magnetic resonance imaging (MRI) revealed a large midline posterior fossa cyst, hydrocephalus and syrinx (C1-Th11). Cisternography could not demonstrate communication between the cyst and the subarachnoid space and the 4th ventricle was present but without communication with the cyst. The cyst was tentatively diagnosed as an arachnoid cyst. One week after placement of a cyst-peritoneal shunt, CT scans showed a decrease in cyst and ventricle size and 2 months later a follow-up MRI revealed resolution of the syringomyelia. We suspected that cyst-peritoneal shunt was effective for this patient who had syringomyelia associated with incommunicated posterior fossa cyst.     

Oral problems associated with CREST syndrome: a case report

A patient with CREST syndrome is described, with such severe limitation of mouth opening that extractions were required before upper gastrointestinal endoscopy could be performed. Features of CREST syndrome of importance to the dental and oral surgeon are described.     

Disseminated histoplasmosis associated with disseminated tuberculosis: a case report

Disseminated histoplasmosis is reported in a 70-year-old White man. There was also evidence of a previous disseminated tuberculosis infection. This is the 9th reported case of disseminated histoplasmosis in the Republic of South Africa.     

Adult-onset Still's disease associated with G6PD deficiency: a case report and literature review

A 37-year-old man presented with symptoms consistent with adult-onset Still's disease. Fever and leukocytosis were prominent, and the patient was started on high-dose aspirin for possible acute rheumatic fever. He developed severe anemia as a result of glucose-6-phosphate dehydrogenase deficiency. His treatment was changed to naproxen, and he recovered with restoration of his hematologic parameters. Although Still's disease is frequently accompanied by mild-to-moderate anemia, the development of severe anemia should raise the possibilities of hemolysis secondary to glucose-6-phosphate dehydrogenase deficiency.     

Crohn's disease of the prepuce in a 12-year-old boy: a case report and review of the literature

We report a case of Crohn's disease with involvement of the foreskin in a 12-year-old boy. One year previously, on the basis of clinical features (diarrhea with blood, perianal fissures) and histologic examination, a diagnosis of Crohn's disease was made. Subsequently, he developed phimosis and balanitis and underwent circumcision. Sections submitted from the foreskin revealed noncaseating granulomatous inflammation consistent with Crohn's disease. Crohn's disease with involvement of the genitalia is unusual. Only 26 cases including our case have been reported in the scientific literature. We have analyzed these cases with emphasis on gender, age, clinical features, duration of Crohn's disease, and probable mode of spread to the genitalia. Careful examination of sections from genital lesions, including those submitted from the foreskin, is essential to detect small isolated granulomas that may then lead to the diagnosis of inflammatory bowel disease.     

Bilateral pheochromocytomas with von Hippel-Lindau's disease: a case report

A case of bilateral pheochromocytomas with von Hippel-Lindau's disease (VHL) is reported. A 46-year-old woman who had hyperglycemia was admitted to our hospital because of abdominal tumors. Her elder sister and niece had been diagnosed as VHL. Ultrasonography, CT, and MRI revealed bilateral adrenal tumors. Noradrenaline levels in serum and urine were elevated and 131I-MIBG scintigraphy showed accumulation in bilateral adrenal glands. Moreover, she had bilateral renal cysts and cerebellar hemangioblastoma. Bilateral adrenalectomies were performed and pathological diagnosis was pheochromocytoma. This is the seventh case of bilateral pheochromocytomas with VHL reported in Japan.     

Giant cell fibroblastoma associated with dermatofibrosarcoma protuberans: a case report

Umbilical cord blood (UCB) was disclosed to possess the proliferative capacity containing hematopoietic progenitors and has recently been applied for allogeneic transplantation as an attractive alternative to bone marrow or peripheral blood stem cells. UCB contains similar and higher proportions of immature hematopoietic progenitors, compared to bone marrow stem cells, although the number of collectable cells is limited. The yield of collectable UCB volume ranges from 70 to 150 ml. The colony formation of CFU-Mix of UCB was higher, but that of CFU-GM and CFU-E was lower, compared to those of bone marrow. The analyses of expression of differentiation antigens and adhesion molecules on CD34+ cells of UCB by flow cytometer, revealed that the coexpression rates of CD38 and CD44 on CD34+ cells were almost the same, but the mean fluorescence intensity of those was low compared to adult bone marrow. These results indicate that UCB contains more primitive hematopoietic progenitors. UCB transplantation has greater advantages of lower incidences of graft versus host disease, and unlimited number of donors compared with other allogeneic transplantation would widen the indication of transplantation by technical and methodological development.     

Relapse of pulmonary Mycobacterium kansasii disease associated with large-cell cancer of the lung: a case report

A 43-year-old caucasian male diabetic presented with purulent cough and a history of weight-loss, elevated temperature, night-sweat and dyspnea. Four years previously, the patient had undergone a 12-month antimycobacterial regimen because of pulmonary mycobacterium kansasii (MK) disease of the left upper lobe (LUL). Treatment had led to complete recovery with the exception of minor fibrous residuals in the involved pulmonary segments. Chest radiograph and computed tomography (CT), performed on recent admission, revealed a dense infiltration of these residual-containing segments. Microbiological evaluation of bronchial brushings, aspirates and histology of the transbronchial biopsies indicated a relapse of pulmonary MK disease. Although antimycobacterial treatment was started immediately, therapeutic effects were only minimal and remained to be limited to the initial phase of the treatment. After four weeks of treatment, the patient's general condition worsened again. Follow-up CT of the lung showed a marked increase of the infiltration in the left apicoposterior lobe and re-bronchoscopy showed a tumorous protrusion of the bronchial wall involving the apicoposterior segment ostium, a finding which was not seen in the previous bronchoscopy. Histology of the transbronchial biopsies revealed a carcinoma mainly from large-cell type.     

Acute autoimmune sensorineural hearing loss associated with Crohn's disease

We report the sudden onset of bilateral hearing loss in a patient with Crohn's disease while the intestinal disease was quiescent. Antibodies directed against collagen type II were detected in the serum. Dramatic improvement of his hearing was observed under corticosteroid therapy. Diagnosis of autoimmune sensorineural hearing loss was established. This condition could be considered as an extraintestinal manifestation of Crohn's disease. Early diagnosis is mandated, as prompt and intensive treatment with corticosteroids and/or immunosuppressive drugs is effective and could prevent irreversible hearing loss.