[A rare case of Marfan syndrome with involvement of the thoracoabdominal aorta--its surgical treatment].
A 49-year-old woman with classic manifestations of Marfan's syndrome, having previously been submitted to two surgical procedures for ascending aorta and aortic valve replacement, was admitted with diagnosis of a large dissecting thoracoabdominal aortic aneurysm, with indication for surgical repair. The patient underwent a successful reconstruction of the aorta by means of the "simplified technique" introduced by ourselves in 1984, as an alternative to the conventional S. Crawford's "inclusion technique", for the management of thoracoabdominal aortic aneurysms. Assessed two years after the operation, the patient was found to be in excellent clinical condition and a control angiography showed the vascular prosthesis and its side branches working properly. This unusual aortic complication of Marfan's syndrome and the usefulness of the unique and original technique employed to replace the thoracoabdominal aorta justify the presentation of this clinical case.
da Gama
A D
AD
Clínica Universitária de Cirurgia Vascular, Hospital de Santa Maria, Faculdade de Medicina, Universidade de Lisboa.
Almeida
C H
CH
Sarmento
C
C
do Carmo
G X
GX
D000784:Aortic Dissection / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D001012:Aorta, Abdominal / Q000601:surgery; D001013:Aorta, Thoracic / Q000601:surgery; D017544:Aortic Aneurysm, Abdominal / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D017545:Aortic Aneurysm, Thoracic / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D019917:Blood Vessel Prosthesis Implantation; D004630:Emergencies; D005260:Female; D006801:Humans; D008382:Marfan Syndrome / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D008875:Middle Aged