Sudden Death in Splenic Artery Segmental Mediolytic Arteriopathy
A Edgecombe, I Teo, M Marinescu, C Milroy. University of Ottawa, Ottawa, ON, Canada; The Ottawa Hospital, Ottawa, ON, Canada
Background: Segmental mediolytic arteriopathy (SMA), a variant of fibromuscular dysplasia, is a rare non-inflammatory vascular disease. Mediolysis of the arterial media may be associated with aneurysm, dissection and stenosis. Visceral SMA primarily involves the branches of the celiac and superior mesenteric arteries and the middle-aged to elderly are typically afflicted. Potential complications include organ infarction and catastrophic intra-abdominal hemorrhage.
Design: A 20-year-old male had a nine-year history of type I diabetes, without diabetic complications. After retiring to bed one night, he suddenly cried out and collapsed. On arrival to hospital, vital signs were absent and resuscitation was unsuccessful. There was no evidence of illicit drug or alcohol abuse. His diabetes was well controlled with a recent haemoglobin A1c of 6.3%. A medico-legal autopsy was ordered.
Results: At autopsy, hemoperitoneum with 3000 ml of blood and clot was evident. The splenic artery was found to be ruptured 13 cm from the celiac artery origin. No other vascular anomalies were identified. Microscopically, there was segmental transmural mediolysis of the splenic artery media with extensive loss of the external elastica. Intima and internal elastica were focally absent. Extravasated erythrocytes were focally prominent within the media. Peri-adventitial inflammation was noted. The adjacent splenic artery had fibromuscular dysplasia. Neither vasculitis nor atherosclerosis was identified. Other vessels were normal.
Conclusions: Visceral SMA is a rare cause of fatal intra-abdominal hemorrhage. Isolated splenic artery SMA is exceedingly uncommon. The aetiology remains unknown. To our knowledge, we describe the youngest patient with visceral artery aneurysm and rupture associated with SMA. SMA of visceral arteries, therefore, must be included in the differential diagnosis of spontaneous intra-abdominal bleeding in all age groups.
Wednesday, March 24, 2010 9:30 AM
Poster Session V # 4, Wednesday Morning