title: Successful combined management for ruptured superior rectal artery aneurysm in neurofibromatosis type 1.
abstract: Neurofibromatosis type 1 (NF1) is an autosomally dominant inherited disorder characterized by neurocutaneous and bony lesions with multisystem vasculopathy in the form of stenotic and aneurysmal disease. The multisystemic nature of NF1 aneurysmal disease can have potentially serious clinical sequelae and complicated management. A 55-year-old woman with NF1 presented acutely with severe abdominal and back pain, diarrhea, and hypotension with a delayed fall in her hemoglobin. Imaging demonstrated a retroperitoneal bleed from a superior rectal artery aneurysm. The inferior mesenteric artery was coiled with cessation of bleeding. She became unstable again and underwent a successful laparotomy for aneurysm exclusion and evacuation of hematoma. Vessel position and relatively nonspecific symptoms on presentation make awareness among front-line clinicians critical. Endovascular management can be difficult, and the clinician should be prepared for open surgery if the patient displays signs of recurrent bleeding.
authors.affiliation: Department of Surgery, Whipps Cross Hospital, London, UK. Electronic address: hengyow@yahoo.co.uk.
authors.forename: Kuan Heng
authors.initials: KH
authors.lastname: Yow
authors.affiliation: Department of Surgery, Whipps Cross Hospital, London, UK.
authors.forename: Jonathan
authors.initials: J
authors.lastname: Bennett
authors.affiliation: Department of Surgery, Whipps Cross Hospital, London, UK.
authors.forename: Patrice
authors.initials: P
authors.lastname: Baptiste
authors.affiliation: Department of Surgery, Whipps Cross Hospital, London, UK.
authors.forename: Pasquale
authors.initials: P
authors.lastname: Giordano
mesh_terms: D017542:Aneurysm, Ruptured / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D015901:Angiography, Digital Subtraction; D001158:Arteries / Q000601:surgery*; D004621:Embolization, Therapeutic; D005260:Female; D006801:Humans; D008026:Ligation; D008875:Middle Aged; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000175:diagnosis; D012007:Rectum / Q000098:blood supply*; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome