ANEURYSM OF THE MAJOR VESSELS IN NEUROFIBROMATOSIS

The case of a patient who presented with a ruptured aneurysm of the br achial artery and type I neurofibromatosis is presented. Angiography r evealed a ruptured aneurysm of the brachial artery in the middle of th e upper arm. Repair of the artery with autogenous vein grafting was im possible due to the extremely brittle brachial artery and accompanying veins. The blood supply distal to the aneurysm was secund by collater als, and the aneurysm, including a relatively long portion of the brac hial artery and veins adjacent to the aneurysm, was resected. The pati ent died of massive hemorrhage from the subclavian artery of the invol ved side 9 days postoperatively. Histological and immunohistological e xaminations of the tissues involved in the ruptured aneurysm were cond ucted, The resected brachial artery and veins were surrounded by hyper trophied tissue which tested positive for S-100 protein and negative f or desmin and action. These findings suggest that the origin of the pr oliferating tissue was not mesodermal dysplasia, but neurofibroma occu rring near or in the vessels. A ruptured aneurysm in a patient with ne urofibromatosis should not be treated with reconstruction of the vesse ls. The treatment of choice is surgical or endovascular occlusion of t he vessels involved.