Spontaneous aortic rupture in a patient with neurofibromatosis type 1.

Abstract

Neurofibromatosis type I (NF-1) is a rare autosomal dominant genetic disorder occurring in 1 in 3,000 individuals. Vasculopathy is a rarely reported finding in patients with NF-1. Here, we report a case of recurrent aortic pseudoaneurysm after endovascular aneurysm repair in a 49-year-old male patient with NF-1. On the sixth postoperative day following a successful open surgical repair of an aortic pseudoaneurysm, he developed hemoperitoneum due to a delayed rupture of the mesenteric artery branch. This was treated with endovascular coil embolization. We report the clinical features and histologic findings of this rare vascular disorder with a review of the relevant literature.

• Authors: Yang Jin Park, Keun Myoung Park, Jiyoung Oh, Hong Suk Park, Jung-Sun Kim, Young-Wook Kim
• Journal: Journal of the Korean Surgical Society (J Korean Surg Soc) Vol. 82 Issue 4 Pg. 261-5 (Apr 2012) ISSN: 2093-0488 [Electronic] Korea (South)
• PMID: 22493769 (Publication Type: Case Reports)