1/14. Pelvic actinomycosis presenting as malignant large bowel obstruction: a case report and a review of the literature.actinomycosis is an infrequent chronic infectious disease. In most cases the diagnosis is made postoperatively because of its unusual clinical presentation. Moreover, abdominal actinomycosis may mimic cancer, inflammatory bowel disease, or diverticulitis. Delay in diagnosis leading to inadequate management and unnecessary procedures has been reported. We report the case of a 49-year-old woman with large bowel obstruction secondary to extensive pelvic actinomycosis involving the rectosigmoid and cecum. She required emergency surgery, which involved both resection and colostomy. A review of the literature on abdominal actinomycosis during the last 50 years is also reported. Rarely has emergency surgery been described in this condition. Although the incidence of actinomycosis has decreased, the abdominal-pelvic form has been increasing over the past 10 years secondary to increased prolonged use of the intrauterine device. As the clinical spectrum of actinomycosis has dramatically changed, so have the therapeutic considerations. Aggressive surgical management in advanced cases with multiorganic involvement seems to have reemerged in recent years. Consideration of actinomycosis in a woman with prolonged use of an intrauterine device and symptoms of bowel obstruction could help to improve the preoperative diagnosis and management of this rare disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/14. The abdominal cocoon: a case report.The abdominal cocoon is a rare disease that is characterized by a total or partial encasement of the small bowel by a thick and fibrotic membrane. Thirty-five cases were reported since it was first described. It occurs primarily in females. Preoperative diagnosis is a matter of challenge and usually made at laparotomy. We report a patient with partial intestinal obstruction and abdominal cocoon which was diagnosed peri-operatively. We review the literature and discuss the etiology of this disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/14. Small bowel obstruction caused by anisakiasis of the small intestine: report of a case.Enteric anisakiasis is a relatively rare disease that is difficult to diagnose preoperatively. We report a case of small bowel obstruction caused by enteric anisakiasis in a 59-year-old Japanese man who presented with abdominal pain a few hours after eating sliced, raw fish. Because of signs of an intestinal obstruction, a laparotomy was performed. Focal thickening and stenosis of the ileocecal region were seen about 100 cm from the end of the ileum and the lesion was excised. We found a moving anisakis thrusting its head into the mucosa of the excised small intestine. Histopathological examination revealed the infiltration of eosinophils in all layers of the intestinal wall and severe edema. Enteric anisakiasis is very rare, and its diagnosis is usually only made after laparotomy. Nevertheless, when signs of acute abdomen develop after the ingestion of raw fish, such as sushi or sashimi, the possibility of enteric anisakiasis should be borne in mind.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/14. Severe large bowel obstruction secondary to infection with actinomyces israelii.Abdomino-pelvic actinomycosis is a rare disease, which usually follows a chronic course. However, bowel obstruction may reveal an acute clinical picture. We report on a 38-y-old female with abdomino-pelvic actinomycosis, characterized by a 10 cm long filiform stenosis of the sigmoid colon and bilateral ovarian abscesses. She developed acute ileus that required emergency surgery.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/14. A fatal pseudo-tumour: disseminated basidiobolomycosis.BACKGROUND: Basidiobolomycosis is a rare disease caused by the fungus Basidiobolus ranarum, member of the class Zygomycetes, order entomophthorales, found worldwide. Usually basidiobolomycosis is a subcutaneous infection but rarely gastrointestinal manifestations have been described; 13 adults and 10 children and a few retroperitoneal or pulmonary cases. In gastrointestinal basidiobolomycosis the colon is most frequently involved, usually presenting with subacute mild abdominal pain. In contrast to children only very few described adult patients had hepatic masses. Definitive diagnosis requires culture, serological testing can be helpful. The fungal morphology and the Splendore-Hoeppli phenomenon are characteristic histological features. There are no prominent risk factors. Usually surgery and prolonged antifungal therapy are required. CASE PRESENTATION: A 61 year old man presented with progressive left abdominal pain and constipation since a few months. colonoscopy showed an obstructing tumour in the descending colon, and a hemicolectomy was performed. histology showed inflammation, possibly caused by a fungal or parasitic infection, without definite identification of an organism. A few weeks postoperatively a CT scan made because of abdominal discomfort, revealed a livermass (6 cm). Treatment with metronidazole, directed against an amoebic liver abscess, was unsuccessful. He developed a marked eosinophilia (27.7%). A liver biopsy was performed and the patient was referred to a university hospital.A repeated CT scan showed a livermass of 9 cm diameter. review of colon and liver biopsy samples showed extensive necrosis and histiocytes, multinucleated giant cells and numerous eosinophils. Grocott stained sections contained unusually large hyphae surrounded by strongly eosinophilic material in haematoxylin and eosin stained sections (Splendore-Hoeppli phenomenon). A presumptive diagnosis of Basidiobolus spp. infection was made and treated with amphotericin b (Itraconazol contra-indicated because of renal insufficiency). A few days later the patient died of a septic shock. After autopsy Basidiobolus ranarum was cultured from liver, gallbladder and colon. CONCLUSION: Our patient died of gastrointestinal basidiobolomycosis with an obstructing colon tumour and a large hepatic mass. This was a rare presentation of basidiobolomycosis and the second fatal case described worldwide.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/14. mesenteric vascular occlusion in infants and children: report of two cases and review of the literature.mesenteric vascular occlusion is a rare disease in the paediatric age group. The pertaining literature is reviewed and two cases are reported. In the first one the vascular obstruction developed after a Wilms tumour on the right side had been removed. Following bowel resection and end-to-end anastomosis the patient died of uraemia. Post mortem examination revealed a diffuse membranous glomerulonephritis and pseudoxanthomatosis in the remaining left kidney, the function of which had probably been affected by the shock associated with mesenteric thrombosis. In the second case a mixed mesenteric vascular occlusion was found without any previous disease; after ileal resection and ileo-coecal anastomosis the baby made a smooth recovery.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/14. Idiopathic sclerosing peritonitis in a man.Idiopathic sclerosing peritonitis is a rare disease described in young adolescent women, characterized by fibrosis and adhesions of the peritoneum to loops of the small bowel. Here we describe a 35-year-old man who underwent exploratory laparotomy for repeated small bowel obstruction. Only partial resection of the terminal ileum was possible because of adhesions; recurrent abdominal infections and leakage from anastomosis required further resection, which ultimately resulted in short bowel syndrome and malabsorption. The clinical and pathological findings were characteristic for idiopathic sclerosing peritonitis. We review the relevant literature, to confirm, to the best of our knowledge, that this is the first report of a male patient who has developed this rare disease.- - - - - - - - - - ranking = 2keywords = rare disease (Clic here for more details about this article) |
8/14. Motility malfunction of the gastrointestinal tract by rare diseases--fibrosis of the intestinal wall.We report on two children who were admitted with chronic ileus without mechanical obstruction. In the 4-month-old female newborn, high-dose radiation was applied after extirpation of a sympathicoblastoma. Within a few years a metaplasia of the muscle coat of the small intestine developed with a resulting malabsorption syndrome. Although the damaged part of the intestine was resected, the process progressed and the child died. In the second case, a chronic ileus developed at the age of 10 years as a result of fibrosis of the intestinal tract. Repeated laparotomies were performed, and no mechanical obstruction could be found. The most probable diagnosis is a form of scleroderma affecting mainly the alimentary tract without any skin involvement. The patient died in a severe cachexia.- - - - - - - - - - ranking = 4keywords = rare disease (Clic here for more details about this article) |
9/14. Mesosigmoidoplasty in the treatment of sigmoid volvulus in children.Sigmoid volvulus is a rare disease of childhood, which requires surgery. The case of a 12-year-old boy is reported. The main aetiopathogenetic causes and the proper surgical approach are also described with particular reference to the so-called mesosigmoidoplasty, which consists of shortening the mesosigmoid by incision along its axis and transverse suture.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
10/14. Eosinophilic gastroenteritis presenting with biliary and duodenal obstruction.Eosinophilic gastroenteritis is a rare disease associated with eosinophilic infiltration of the gastrointestinal tract which can result in gastroduodenal obstruction. Biliary obstruction due to duodenal disease is rare, and the simultaneous occurrence of obstruction of the duodenum and biliary tract has not been previously reported. We now present a patient with abrupt onset of both biliary tract and duodenal obstruction due to eosinophilic infiltration of the muscularis of the duodenum and stomach. The differential diagnosis and unique features of this disease are reviewed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
| | Next -> |