Hines George L, Lefkowitz Laura, Mohtashemi Manucher
Department of Thoracic and Cardiovascular Surgery, Winthrop-University Hospital, 259 First Street, Mineola, NY 11501, USA.
Ann Vasc Surg. 2002 Nov;16(6):784-6. doi: 10.1007/s10016-001-0221-6. Epub 2002 Nov 6.
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.
神经纤维瘤病以其皮肤表现为特征。它还表现为常见于小动脉和小动脉但很少见于大动脉的动脉病变。我们报告一例患有I型神经纤维瘤病的患者,其腹主动脉自发破裂。在急诊剖腹手术时发现,他的主动脉受到腹膜后神经纤维瘤的直接压迫,且主动脉壁结构完整性异常。