Abstract
A 38-year-old Japanese patient with hemophilia A with an acute abdomen was admitted to our hospital complaining of severe abdominal pain and vomiting, despite infusion of factor VIII concentrate. Physical examination revealed marked tenderness over the right abdomen and intraperitoneal irritation. A plain X-ray film of the abdomen revealed intestinal dilatation with a large amount of gas, indicating obstruction of the small intestine. Abdominal CT scan revealed circumferentially thickened small bowel, suggesting the possibility of intestinal strangulation. Laparotomy was performed under continuous infusion of recombinant factor VIII concentrate. The dilated small intestine and an area of dark-reddened intestine and mesenteric membrane were found and resected. The postoperative course was uncomplicated. Pathological examination of the specimen revealed an intramural small intestinal and intramesenteric hemorrhage. Spontaneous intramural hematoma is a rare complication of hemophilia. A literature search from 1950 revealed 34 such cases. In 17 of these cases, the hematoma occurred in the small intestine, which is the most frequent site of occurrence of spontaneous intramural hematoma, and in many of these cases bowel obstruction occurred. Basic therapy for intramural hematoma is conservative management with replacement of coagulant factor. However, for patients with an acute abdomen or intestinal strangulation, surgery is often performed. Special attention should be paid to abdominal pain in hemophiliacs since it may indicate spontaneous intramural hematoma.
