Abstract
A 50-year-old man who had been diagnosed as having von Recklinghausen disease was admitted to our emergency room after experiencing a severe pain in his abdomen and back and collapsing. Abdominal CT and US examinations revealed a retroperitoneal hematoma with intraperitoneal hemorrhaging, and an emergency operation was performed. During the operation, active bleeding was observed around the pancreas and the retroperitoneum. The origin of the bleeding could not be found, so gauze packing was used. After this first operation, a celiac angiography and an abdominal CT examination showed leakage from blood vessels, indicating bleeding. Four additional operations for hemostasis were performed over the next three consecutive days. Though the patient's vital signs were stable after the 4th operation, except for liver dysfunction and jaundice, he collapsed suddenly 8 days after admission, and an emergency angiography and operation were immediately performed. The uncontrolled retroperitoneal bleeding was completely different from the previous episode, and gauze packing was performed once again. In spite of receiving more than 30, 000ml of blood transfusions, the patients died of blood loss. A hematoma in the pancreas communicating with the portal vein was found at the time of autopsy, but no signs of arteriosclerosis or aneurysm in the main arteries of the abdomen and retroperitoneum were observed. Microscopically, the fragmentation of the elastic laminae was observed in the superior mesenteric artery. Retroperitoneal hemorrhaging in association with von Recklinghausen disease may require an emergency angiography, TAE and laparotomy, even if the patient's condition appears to be stable.
