Saunders Sarah, Harmse Dean, Sheppard Mary
Derriford Hospital, Histopathology, Derriford Road, Crownhill, Plymouth, Devon, PL6 8DH, UK.
BMJ Case Rep. 2009;2009. doi: 10.1136/bcr.04.2009.1740. Epub 2009 Aug 17.
A 35-year-old man presented to the Emergency Department with a history of severe acute central chest pain and simultaneous bilateral paralysis the legs with double incontinence. There was no significant past medical or family history. A CT scan showed a thoracic dissection of the aorta extending from the aortic root to the aortic bifurcation and in to the common iliac arteries. The patient was consented for an axillo-femoral bypass and was taken to theatre and operated on for 7 hours. The patient unfortunately died under anaesthesia. A hospital post-mortem was requested to identify the cause of the dissection. The patient's heart was sent to a cardiac pathologist who identified an undiagnosed coarctation of the aorta. Genetic testing was negative for Marfan syndrome.As a result of the post-mortem, it was recommended that first degree relatives of the deceased undergo ultrasound examination of the cardiovascular system as appropriate to exclude coarctation of the aorta.
一名35岁男性因严重急性中央胸痛病史,同时伴有双腿双侧瘫痪及大小便失禁,就诊于急诊科。既往无重大病史及家族病史。CT扫描显示主动脉夹层,从主动脉根部延伸至主动脉分叉,并累及双侧髂总动脉。患者同意接受腋-股旁路手术,被送往手术室并接受了7小时的手术。不幸的是,患者在麻醉下死亡。医院要求进行尸检以确定夹层的病因。患者的心脏被送去给心脏病理学家,后者发现了未被诊断出的主动脉缩窄。马凡综合征的基因检测结果为阴性。尸检结果建议,死者的一级亲属应酌情接受心血管系统超声检查,以排除主动脉缩窄。