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[马凡氏综合征、重度漏斗胸和主动脉瓣环扩张患者的一期手术]

[One-stage operation in a patient with Marfan's syndrome, severe pectus excavatum and annulo-aortic ectasia].

作者信息

Kamihira S, Hara Y, Kobayashi T, Ishiguro S, Sasaki S, Mori T

机构信息

Second Department of Surgery, Tottori University, School of Medicine, Japan.

出版信息

Nihon Kyobu Geka Gakkai Zasshi. 1991 Nov;39(11):2057-62.

PMID:1774486
Abstract

A case of Marfan's syndrome complicated by annulo-aortic ectasia with pectus excavatum (Wada's 3rd grade classification) and aortic regurgitation (Seller's 1st grade) is reported in a 36-year-old man. We performed one-stage operation combining Bentall's procedure for the annulo-aortic ectasia and sternal elevation for the pectus excavatum. Few reports of one-stage operation for pectus excavatum and annulo-aortic ectasia in Marfan's syndrome have been published. If both diseases are present and surgical indications permit, one-stage operation can be performed to ensure protection of the heart and a satisfactory postoperative course. The best approach to the heart to obtain the most favorable operating field and the best chestplasty method that also assures closure of the median sternotomy are important considerations in one-stage operation. The most appropriate combination of these two operational variables must be assessed in each case.

摘要

本文报道了一名36岁男性马凡综合征患者,该患者合并主动脉瓣环扩张症、漏斗胸(和田3级分类)和主动脉瓣关闭不全(塞勒1级)。我们实施了一期手术,将针对主动脉瓣环扩张症的Bentall手术与针对漏斗胸的胸骨抬高术相结合。关于马凡综合征合并漏斗胸和主动脉瓣环扩张症的一期手术报道很少。如果两种疾病同时存在且手术指征允许,可以实施一期手术以确保心脏得到保护并获得满意的术后过程。在一期手术中,获得最有利手术视野的最佳心脏入路以及确保正中胸骨切开术闭合的最佳胸廓成形术方法是重要的考虑因素。必须针对每个病例评估这两个手术变量的最合适组合。

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