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戈尔登哈综合征:一家参考中心的外科治疗方案

Goldenhar syndrome: surgical management protocol in a reference center.

作者信息

Guevara-Valmaña Osvaldo I, Nahas-Combina Luciano, Andrade-Delgado Laura, Apellaniz-Campo Armando Guillermo, Leyva-Sotelo Leslie M, Gaspar-Daniel Ángeles

机构信息

Departamento de Cirugía Plástica y Reconstructiva, Clínica de Cirugía Craneofacial, Hospital "Dr. Manuel Gea González", Ciudad de México, México.

出版信息

Cir Cir. 2019;87(5):516-527. doi: 10.24875/CIRU.19000654.

Abstract

OBJECTIVE

To present our experience treating 42 patients with Goldenhar syndrome.

METHOD

A descriptive, observational, retrospective study was carried out using the medical and photographic record of all patients diagnosed with Goldenhar syndrome treated by the craniofacial surgery unit of the plastic and reconstructive surgery department of the Dr. Manuel Gea González hospital between 2010 and 2018.

RESULTS

A total of 42 patients were obtained,54% male of which all underwent at least one procedure. The majority of patients were of the first decade of age (57%). Surgical procedures could be divided mainly into 14 auricular (20%), 17 mandibular (24%), 2 Lefort (4%), 10 volume (14%), 9 macrostoma (13%) and 16 other (21%). A total of 71 procedures were performed.

CONCLUSION

Goldenhar syndrome is a rare entity that affects various structures, which is why an early diagnosis and multidisciplinary management headed by a team of plastic surgeons is necessary.

摘要

目的

介绍我们治疗42例Goldenhar综合征患者的经验。

方法

采用描述性、观察性、回顾性研究方法,使用2010年至2018年间曼努埃尔·吉亚·冈萨雷斯博士医院整形与重建外科颅面外科治疗的所有诊断为Goldenhar综合征患者的病历和照片记录。

结果

共纳入42例患者,其中54%为男性,所有患者均至少接受过一次手术。大多数患者处于第一个十年年龄段(57%)。手术程序主要可分为14例耳部手术(20%)、17例下颌骨手术(24%)、2例勒福手术(4%)、10例容积手术(14%)、9例大口畸形手术(13%)和16例其他手术(21%)。共进行了71例手术。

结论

Goldenhar综合征是一种影响多种结构的罕见疾病,因此早期诊断以及由整形外科医生团队主导的多学科管理是必要的。

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