Abstract
We report an extremely rare case of vascular type Ehlers-Danlos syndrome complicated with retroperitoneal hemorrhage. A 14-year-old boy suffering from lower abdominal pain was transferred to our hospital and a lower abdominal mass was detected by abdominal CT. After admission the patient went into shock. Emergent laparotomy revealed massive preperitoneal and retroperitoneal hemorrhage. Arteriography performed just after the surgery showed interruption of the right external iliac artery and collateral flow from internal iliac feeding to the femoral artery without extravasation. We diagnosed a retroperitoneal hemorrhage due to bleeding from the right external iliac artery with unknown origin. Skin biopsy and chromosomal analysis revealed vascular type Ehlers-Danlos syndrome. Regular follow-up was performed, however, he died 18 months after surgery due to sudden bleeding.
