Abstract
A patient with Marfan's syndrome who had been suffering from multiple abdominal aneurysms and was presented with acute abdomen is described.
A 21-year-old male, a monozygotic twin with Marfan's habitus visited the hospital because of an epigastralgia. There were surgical histories of pes varus and luxation of the hip joint. Laboratory tests of blood revealed leukocytosis and an increase in creatinine phosphokinase (CPK) value. A plain film of the abdomen revealed an intestinal obstruction. CT scan showed an accumulation of peripancreatic fluid in the retroperitoneal space. Abdominal angiography revealed aneurysms of the celiac artery, superior mesenteric artery, and bilateral renal arteries, but there were no findings of dissecting aneurysm of the aorta. Epigastralgia became worse and an emergency surgery was performed. There were unquestionable retroperitoneal hematoma and sanguineous ascitic fluid. The aneurysm of the superior mesenteric artery, which was suspected of rupture, was replaced with a greater saphenous vein graft.
The postoperative course was uneventful, but the patient died of sudden cardiac arrest on the fifth postoperative day. An autopsy revealed a dissecting aneurysm (DeBakey type I) extending to carotid arteries and renal arteries; massive intrathoracic hemorrhage; subarachnoidal hemorrhage with ruptured cerebral ventricle; and distinct cystic medial necrosis of the wall of arteries. No intra-abdominal hemorrhage was found.
