J Korean Surg Soc.  2012 Apr;82(4):261-265. 10.4174/jkss.2012.82.4.261.

Spontaneous aortic rupture in a patient with neurofibromatosis type 1

• Park YJ
• Park KM
• Oh J
• Park HS
• Kim JS
• Kim YW

• KMID: 1820076
• DOI: http://doi.org/10.4174/jkss.2012.82.4.261

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.

Keyword

Aortic aneurysm; Aortic rupture; Neurofibromatosis 1

MeSH Terms

Aneurysm
Aneurysm, False
Aortic Aneurysm
Aortic Rupture
Hemoperitoneum
Humans
Male
Mesenteric Arteries
Middle Aged
Neurofibromatoses
Neurofibromatosis 1
Rupture

Figure

• 

  Fig. 1 (A, B) Preoperative computed tomography (CT) scan shows retroperitoneal hematoma and saccular-shaped pseudoaneurysm at left lateral wall of distal abdominal aorta at 1 cm proximal to aortic bifurcation. (C) On follow-up CT scan, small pseudoaneurysm was detected distal to aortic stent graft.

• 

  Fig. 2 (A) Microscopic section of aorta shows medial destruction with elastic fiber degeneration (upper left), thrombus in lumen, and adventitial fibrosis (Movat pentachrome stain, ×100). (B) S-100 protein-positive cells (arrow) are detected in adventitia of aorta (×200).

• 

  Fig. 3 (A) Mesenteric angiography on 6th day after open surgical repair of aortic aneurysm shows pseudoaneurysm formation at small branch of middle colic artery. (B) With coil embolization of proximal and distal segments of artery to pseudoaneurysm, bleeding from middle colic artery was successfully treated.

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