Marfan症候群に合併した頸部内頸動脈瘤の1例

Extracranial Internal Carotid Artery Aneurysm Associated with Marfan Syndrome: Case Report

Ⅰ．はじめに

　Marfan症候群は結合組織の障害を主体とする常染色体優性遺伝性疾患で，主として骨格系・眼組織・心血管系に障害が生じるが，脳血管系に合併症が生じることは必ずしも高頻度ではない．今回，われわれはMarfan症候群に合併した頸部内頸動脈瘤の1例を経験したので，その手術適応・手法を中心に報告する．

　Marfan syndrome is a heritable disorder of connective tissue characterized by autosomal dominant inheritance. Cerebrovascular disorders associated with Marfan syndrome are known to be rare. In this report, we described a rare case of a 64-year-old woman with an extracranial internal carotid artery (ICA) aneurysm associated with Marfan syndrome. The extracranial ICA was very tortuous and a 3-cm-diameter aneurysm was observed at the distal portion of the carotid bifurcation. Through anterior neck incision, the aneurysm was dissected from the surrounding tissue. The carotid artery was clamped, and external shunting was performed using a 3-way shunt tube. The aneurysm was excised, and an end-to-end anastomosis of the ICA was made. Postoperative course was uneventful except for mild swallowing disturbance lasting for 2 weeks. The surgical indication and technique are discussed.