Infrarenal aortic rupture secondary to neurofibromatosis

Neurofibromatosis is characterized by its cutaneous manifestations. It also is manifested by arterial lesions commonly found in arterioles and small arteries but rarely in large arteries. We present a patient with type I neurofibromatosis with spontaneous rupture of his abdominal aorta. He was found at the time of emergency laparotomy to have direct compression of his aorta by retroperitoneal neurofibromas and abnormal aortic structural wall integrity.