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Case Reports
. 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

Yang Jin Park  1 , Keun Myoung Park, Jiyoung Oh, Hong Suk Park, Jung-Sun Kim, Young-Wook Kim
Affiliations
Case Reports

Spontaneous aortic rupture in a patient with neurofibromatosis type 1

Yang Jin Park et al. J Korean Surg Soc. 2012 Apr.

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.

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Conflict of interest statement

No potential conflict of interest relevant to this article was reported.

Figures

Fig. 1
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
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
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.
See this image and copyright information in PMC

References

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