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改良Ravitch手术治疗合并漏斗胸的左侧波兰综合征

Modified Ravitch Procedure for Left Poland Syndrome Combined With Pectus Excavatum.

作者信息

Cortés-Julián Gildardo, Bushra-Nuritu Hussein B, Buenfil-Fuentes Renata, Merino Rajme José A

机构信息

Pneumology and Thoracic Surgery Department, Centro Médico Nacional 20 de Noviembre del Instituto de Seguridad y Servicios Sociales de los Trabajadores del Estado, Mexico City, Mexico.

Pneumology and Thoracic Surgery Department, Centro Médico Nacional 20 de Noviembre del Instituto de Seguridad y Servicios Sociales de los Trabajadores del Estado, Mexico City, Mexico.

出版信息

Ann Thorac Surg. 2017 Oct;104(4):e337-e339. doi: 10.1016/j.athoracsur.2017.05.078.

Abstract

Poland Syndrome is a rare disease with an incidence of 1 in 30,000. This disease is characterized by agenesis of the pectoralis major, hypoplasia of mammarian tissue and the nipple complex, and limb abnormalities. The severe form of this disease can be associated with rib and sternal malformations like pectus excavatum. A 19-year-old man with severe Poland syndrome with cardiac extrasystoles and restrictive ventilation as shown by a spirometry test is presented. A total sternal reconstruction with partial osteotomies and bilateral resection of cartilage was performed. The sternum was stabilized with underlying titanium bars and clips. The cosmetic result was satisfactory.

摘要

波兰综合征是一种罕见疾病,发病率为三万分之一。该疾病的特征为胸大肌发育不全、乳腺组织和乳头复合体发育不全以及肢体异常。这种疾病的严重形式可能与肋骨和胸骨畸形有关,如漏斗胸。本文介绍了一名19岁患有严重波兰综合征的男性,伴有心脏早搏以及肺功能测试显示的限制性通气障碍。实施了全胸骨重建术,包括部分截骨术和双侧软骨切除术。用下方的钛棒和夹子固定胸骨。美容效果令人满意。

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