Abstract
A 58-year-old male with neurofibromatosis type 1 (NF 1) was transferred to our hospital in pre-shock condition with severe abdominal pain of sudden onset. A CT scan demonstrated a large retroperitoneal hematoma and active bleeding from the infrarenal abdominal aorta. Emergency open surgical repair was performed under diagnosis of aortic rupture. Intricate thick fibrous tissue around the aorta made aortic exposure confusing and time-consuming. Suture ligation to control bleeding from lumbar arteries was also found troublesome due to tissue fragility. Although the abdomen needed two staged closure because of large retroperitoneal hematoma and bowel edema, his subsequent postoperative course was uneventful. Pathological exam revealed that observed thick fibrous tissue was plexiform neurofibroma which is highly characteristic to NF 1. Rupture of large vessels in NF 1 patients is a very rare and challenging complication. According to published reports, the possibility of unusual fragility of vascular wall and/or easy bleeding of neurogenic tumors around the vessel should be taken into consideration when you make strategies for treatment.
