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眼耳脊椎发育不良-半侧颜面短小畸形(Goldenhar-Gorlin综合征)中的先天性面神经病变

Congenital facial neuropathy in oculoauriculovertebral dysplasia-hemifacial microsomia (Goldenhar-Gorlin syndrome).

作者信息

Aleksic S, Budzilovich G, Reuben R, Sekhar H C, Feigin I, Finegold M, Boal D, Tokita N, Converse J M

出版信息

Bull Los Angeles Neurol Soc. 1976 Apr;41(2):68-77.

PMID:1030652
Abstract

Four patients with clinical features of Goldenhar-Gorlin syndrome who showed facial paralysis on clinical examination are presented. The fourth case died following surgery for cleft lip. Autopsy revealed hypoplasia of the right facial nerve in its intracranial segment, with small right facial nucleus in the brain stem. Nosological aspects of the Goldenhar-Gorlin syndrome are discussed. Peripheral facial paralysis, as a part of this syndrome, is reviewed in the light of clinical and pathological findings and in its relationship to cardiac anomalies. It is suggested that Goldenhar-Gorlin syndrome is a part of a so-called cardiofacial syndrome.

摘要

本文报告了4例具有Goldenhar-Gorlin综合征临床特征且临床检查显示面瘫的患者。第4例患者在唇裂手术后死亡。尸检发现右侧面神经颅内段发育不全,脑干中右侧面神经核较小。本文讨论了Goldenhar-Gorlin综合征的疾病分类学方面。根据临床和病理结果及其与心脏异常的关系,对作为该综合征一部分的周围性面瘫进行了综述。有人提出Goldenhar-Gorlin综合征是所谓的心面综合征的一部分。

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