1/5. Congenital pleuroperitoneal communication in a patient with pseudomyxoma peritonei.BACKGROUND AND OBJECTIVES: pseudomyxoma peritonei syndrome is a rare disease arising from a perforated appendiceal adenoma. The syndrome is characterized by progressive accumulation of mucinous ascites and tumor within the peritoneal cavity. Direct extension of pseudomyxoma peritonei to the pleural cavity is uncommon and has been associated with surgical penetration of the diaphragm at the time of cytoreduction. methods: We review the case of a patient who presented with mucoid peritoneal and pleural fluid consistent with spontaneous pleural spread of pseudomyxoma peritonei. RESULTS: Surgical exploration confirmed direct pleuroperitoneal communication by macroscopic diaphragmatic fenestration. CONCLUSIONS: This is a rare phenomenon. We outline a therapeutic approach to be applied when pleural involvement is suspected in patients with pseudomyxoma peritonei syndrome.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/5. Treatment of sclerosing mesenteritis with corticosteroids and azathioprine.Sclerosing (idiopathic) mesenteritis is a rare disease that may present with abdominal pain or bowel obstruction. A 21-year-old man was diagnosed with sclerosing mesenteritis, and treated with a partial ileal resection and defunctioning ileostomy. He was subsequently started on corticosteroids and azathioprine. Five months later, at the time of ileostomy reversal, he was disease-free. The diagnosis and management of this disease are discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/5. Mesenteric panniculitis of the sigmoid colon. Report of two cases.Mesenteric panniculitis is an extremely rare disease in which the normal fatty architecture of the mesentery is replaced by fibrosis, necrosis, and calcification. Grossly, the mesentery is massively thickened and rubbery, with irregular areas of reddish-brown to pale yellow foci resembling fat necrosis scattered throughout. Microscopically, inflammatory involvement of the fibroadipose tissue shows round cells, foam cells, and giant cells. macrophages that have ingested fat, also called lipophages, are the hallmark of the diagnosis. One hundred twenty-two cases of mesenteric panniculitis have been described in the literature; only three of these involved the large-bowel mesentery. This paper presents a review of the literature and two new cases that involve exclusively the mesentery of the sigmoid colon.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/5. Peliosis of the spleen with intraperitoneal hemorrhage.We encountered a patient with steroid-related peliosis of the spleen, a rare disease characterized by multiple blood-filled cavities in the splenic parenchyma, with spontaneous intraperitoneal hemorrhage. The ultrasonographic, computed tomographic, and angiographic images were compared with pathologic findings of the material obtained surgically.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/5. A case of abdominal cocoon.Abdominal cocoon is a rare disease of the peritoneum and almost invariably presents as an acute or subacute intestinal obstruction with or without a mass. The etiology of this disease is largely unknown and abdominal cocoon of unknown etiology has been limited to the tropical and subtropical zones and primarily affects young adolescent females. In the temperate zone, only one case has been reported from the United Kingdom, but the patient was also born in pakistan. No case of abdominal cocoon purely developed in the temperate zone has been reported. Recently, we experienced a case of abdominal cocoon in a 34-year-old female patient(Korean) who had never been abroad. The diagnosis was made postoperatively by reviewing the literature. We herein report this rare condition developed in an unusual geographical location with a brief review of the literature.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |