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贝克威思-维德曼综合征巨舌症的治疗

Treatment of macroglossia in Beckwith-Wiedemann syndrome.

作者信息

Clauser Luigi, Tieghi Riccardo, Polito Jessica

机构信息

Department of Cranio-Maxillo-Facial Surgery, Centre for Craniofacial Deformities, Corso Giovecca, 203, 44100 Ferrara, Italy.

出版信息

J Craniofac Surg. 2006 Mar;17(2):369-72. doi: 10.1097/00001665-200603000-00030.

Abstract

A case of macroglossia caused by Beckwith Wiedemann syndrome is reported. Beckwith-Wiedemann Syndrome is an overgrowth disorder characterized by a constellation of congenital anomalies. The most common manifestations are omphalocele, macroglossia, gigantism, and visceromegaly. When the tongue reaches a huge dimension, clinical symptoms are represented by dysphagia, alterations in speech, difficulty in chewing, obstruction of the upper airways, and psychologic consequences derived from the patient's physical appearance. The authors describe the surgical strategy performed in the reported case.

摘要

报告了一例由贝克威思-维德曼综合征引起的巨舌症病例。贝克威思-维德曼综合征是一种过度生长障碍,其特征为一系列先天性异常。最常见的表现是脐膨出、巨舌症、巨人症和内脏肿大。当舌头变得巨大时,临床症状表现为吞咽困难、言语改变、咀嚼困难、上呼吸道梗阻以及患者外貌带来的心理影响。作者描述了该病例所采用的手术策略。

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