A Case Report of Ehlers-Danlos Syndrome Type IV Complicating Ruptured Ileal Artery Aneurysm

Hirofumi Fujita, Kunio Uesaka and Shinichi Murao^*

Department of Surgery, Wadayama Hospital
^*Second Department of Pathology, Kobe University School of Medicine

Ehlers-Danlos syndrome (EDS) type IV, the so-called arterialecchymotic type, has a high incidence of mortality because of arterial and bowel rupture. We report an extremely rare case of EDS type IV complicated with massive intraperitoneal hemorrhage resulting from a ruptured aneurysm of the ileal artery. A 49-year-old man suffering from right lower abdominal pain and diarrhea was admitted to the hospital. Intraperitoneal hemorrhage was detected by abdominal CT and paracentesis, and an emergent laparotomy was performed. During laparotomy, a bleeding tumor 3.0cm in diameter was detected in the mesentery of the ileum. Partial resection of the ileum was performed. A total of 1,144 ml of hematoma and hemorrhage was removed. Histological investigation revealed that the bleeding tumor was a ruptured aneurysm of the ileal artery. The patient was finally diagnosed as EDS type IV with an ileal artery aneurysm rupture causing intraperitoneal hemorrhage because of aortic insufficiency, scoliosis, hyperextensible skin and hypermobile joints. He was alive after the surgery without any further hemorrhagic complications. Thirty one cases of EDS type IV have been reported in the Japanese literature. This is the first report of a curable case having a rupture of the ileal artery and massive intraperitoneal hemorrhage.

Key words
Ehlers-Danlos syndrome type IV, ileal artery aneurysm rupture, intraperitoneal hemorrhage

Reprint requests
Hirofumi Fujita Department of Surgery, Wadayama Hospital 2021 Takeda, Wadayama-cho, Asago-gun, Hyogo, 669-5252 JAPAN

Accepted
February 24, 1999

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