Life-threatening brachial artery hemorrhage and a lethal outcome in patients with neurofibromatosis type 1: two case reports and a review of the literature

Abstract

Neurofibromatosis type 1 (NF1) is an autosomal dominant disease characterized by neuorocutaneous lesions and multisystem involvement. Other notable features of NF1 include vasculopathy in the form of stenosis, occlusion, aneurysm, pseudoaneurysm, arteriovenous deformity, and rupture, which are difficult to manage and can have fatal outcomes. We describe two cases of extensive and progressive brachial artery hemorrhage following blunt trauma in patients with NF1. Management of these patients included combined endovascular and surgical treatment based on the patients’ condition. The patients had a poor prognosis because of uncontrolled bleeding. While one patient died, the other survived, but the involved arm was amputated. Endovascular treatment is a widely used, popular, minimally invasive, and safe method to control the bleeding associated with NF1. However, this treatment can be challenging at times. Close collaboration between an interventional radiologist and surgeon is necessary for optimal treatment and careful follow-up for this condition.