降嘴角肌發育不全症候君羣：11病例報告

不對稱哭相（或稱際嘴角肌發育不全症候羣）之特徵為病急在哭泣時，嘴角會歪向一側容易合併其他先天性畸形，當合併先天性心臟病時，特稱之爲心面症候羣。國奉醫院在最近三年間發現不對稱哭相之病例有11例，經過詳細檢查，其中6例診斷係心面症候羣。此6例包括4男2女，診斷之年齡自2天至6歲不等，分別因爲心臟病、臉部不對稱哭相或其他感染症而來院檢查或治療。我們將列舉這些先天性心臓病的種類以及合併的腦部、臉部及泌尿器等畸形。11例不對稱哭相病人中，有10例曾安排肌電圖檢查，都證實係單側降嘴角肌發育不全所效。另外，我們也發現不對稱哭相病側在右者合併畸形的機會似乎比左側高。本文結論爲，不對稱哭相可以當做先天性畸形之指標，若發現小孩有不對稱哭相，應進一步檢查是否合併包括先天性心臓病的畸形。

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並列摘要

Asymmetric crying facies (ASCF), characterized by downward displacement of mouth to one side during crying, is thought to be due to hypoplasia or agenesis of depressor anguli oris muscle (DAOM) on the other side. It is frequently associated with congenital anomalies of cardiovascular, musculoskeletal, central nervous, gastrointestinal and genitourinary systems. Its association with congenital heart diseases was first described by Cayler in 1969. He named this association the ”CARDIOFACIAL SYNDROME”. From February 1982 to August 1985, 11 cases of ASCF were observed in Cathay General Hospital and, after investigation, 6 were diagnosed as cardiofacial syndrome. These included 4 males and 2 females. The age at diagnosis ranged from 2 days to 6 years. The presenting findings included heart problems as well as facial asymmetry during crying. Among these cardiac lesions, 2 were patent ducts arteriosus, 1 pulmonary stenosis, 1 tetralogy of Fallot (TF), 1 TF with pulmonary atresia, and 1 cleft mitral valve. They all had other associated anomalies involving eye, brain and genitourinary system. We conclude that ASCF may be considered as an index of congenital anomalies. Any child with ASCF should be examined thoroughly for the possibility of other associated congenital anomalies including congenital heart diseases.

並列關鍵字

asymmetric crying facies ； depressor anguli oris muscle ； congenital anomaly ； congenital heart disease ； cardiofacial syndrome

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降嘴角肌發育不全症候君羣：11病例報告

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