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J Korean Surg Soc. 2012 Apr;82(4):261-5. doi: 10.4174/jkss.2012.82.4.261. Epub 2012 Mar 27.

Spontaneous aortic rupture in a patient with neurofibromatosis type 1.

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  • 1Division of Vascular Surgery, Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

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.

KEYWORDS:

Aortic aneurysm; Aortic rupture; Neurofibromatosis 1

PMID:
22493769
[PubMed]
PMCID:
PMC3319782
Free PMC Article
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