Impact of Management of Abdominal Aortic Dissection Following the Successful Operation of Annulo-Aortic Ectasia in Marfan's Syndrome - a Case Report

一位21歲女性馬方氏症之病人因慢性昇主動脈剝離及主動脈瓣閉鎖不全而接受Cabrol式的全主動脈根置換手術。手術後病人情況穩定，恢復良好。不幸在手術後第五天病人突發腹部劇痛，隨即完全無尿。核磁共振檢查顯示腹主動脈剝離同時造成所有腹部器官血流灌注不全，SGOT和SGPT迅速上昇，嚴重的酸中毒及迅速的生命現象惡化皆發生在數小時之內。一切不可逆的反應使我們無法嘗試手術治療，這類病例處理上的迷思將被討論。

關鍵字

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並列摘要

A 21-year-old female patient with Marfan's syndrome suffering from chronic ascending aortic dissection and aortic insufficiency was treated with total aortic root replacement by Cabrol technique (or procedure). The post-operative course was smooth and the patient recovered satisfactorily with very stable hernodynamic condition and good appetite. Unfortunately she complained of sudden severe abdominal pain followed by complete anuria on the fifth post-operative day. The MRI demonstrated abdominal aortic dissection with malperfusion of all the abdominal organs. Rapid increase of aminotransferases ( SGOT and SGPT ), severe acidosis and rapid deterioration of vital signs within 10 hours discouraged us from trying surgical intervention. The puzzle of management in those cases will be discussed.

並列關鍵字

Marfan's syndrome； aortic dissection； fenestration； endovascular stent.

國際替代計量

Impact of Management of Abdominal Aortic Dissection Following the Successful Operation of Annulo-Aortic Ectasia in Marfan's Syndrome - a Case Report

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