Brachial Artery Aneurysm Rupture in a Patient with Neurofibromatosis: A Case Report

Abstract

Peripheral vascular manifestations of neurofibromatosis are rare but may result in fatal haemorrhaging when they rupture. Surgeons should be aware of this life-threatening condition. We report a case of 35-year-old woman with neurofibromatosis who presented with a swollen and tender mass around her right arm. Angiography revealed 2 aneurysms in the brachial artery. Surgical occlusion revealed a large amount of clotted blood within the subfascial space, and the bleeding point was identified as a pinpoint opening in the aneurysm. The brachial artery abutting the aneurysm and the surrounding soft tissues was extremely brittle and fragile, with massive oozing during dissection. The brachial artery was irreparable and was resected after ligation of the artery and surrounding soft tissues and the aneurysm. Despite an uneventful recovery, the patient died on day 4.

Ruptured brachial artery aneurysm in a patient with type 1 neurofibromatosis

“…12,13 Although the true incidence of arterial aneurysms associated with NF1 is uncertain, arterial aneurysms in the upper extremity (axillary, brachial, radial artery aneurysm) may be the least common. 5 Three patients with NF1 suffering from ruptured brachial artery were reported (Table); 6-8 two required resection of the aneurysms and the brachial artery and vein after ligation of the vessels, 6,8 while the third required a transhumeral amputation after an unsuccessful attempt to reconstruct a ruptured aneurysm with a vein graft. 7 Thus, there have been no reports on the successful reconstruction of the ruptured brachial artery in patients with NF1.…”

“…Three patients with NF1 suffering from ruptured brachial artery have been reported; the outcome was death in two cases and amputation in one, with no successful repairs. [6][7][8] We report here a patient with NF1, who underwent resection of a ruptured brachial artery aneurysm and successful reconstruction with an autologous great saphenous vein graft.…”

A safe method for excision of a giant neurofibroma on both buttocks using a loop-shaped suture

“…In larger vessels (aorta, carotid, and proximal renal artery), direct invasion by Schwann cells, intimal thickening, and destruction of the media and elastic tissue lead to either stenosis or aneurysm. In smaller vessels, mesodermal dysplasia causes the proliferation of smooth muscle in the intima leading to stenotic lesions and, occasionally, poststenotic aneurysms [8].…”

Successful Repair of a Vasculopathic Aneurysmal Brachial Artery in a Patient with Type 1 Neurofibromatosis

“…Meanwhile, occlusive diseases most frequently affect the renal arteries, abdominal aorta (coarctation) and mesenteric arteries. 3,4,[5][6][7][8] In the context of NF1 other rarer vasculopathies have been reported such as peripheral arterial aneurysms, venous malformations 2 , and extracranial aneurysms of the vertebral arteries 9 .…”

“…Despite its rarity, NF1 associated peripheral vasculopathy may have significant clinical sequelae including fatal hemorrhage resulting from aneurysmal rupture. 8,[10][11][12][13][14] Surgical treatment of these aneurysms may prove challenging due to the friable nature of the soft tissues of patients having NF1. 8,10,14,15 Consequently, the few cases reported in the literature are associated with relatively high mortality and morbidity, including major amputations.…”