歪嘴哭面容新生儿的临床特征及随访研究

目的：评估歪嘴哭面容新生儿的临床特征及近期转归，为临床医师更深刻地认识该疾病提供信息。方法：回顾性研究2010年1月至2012年2月间收治的11例歪嘴哭面容患儿的临床特点，随访纠正胎龄44周和3月龄时的体格及神经发育情况。结果：11例歪嘴哭面容患儿中，4例合并同侧耳部发育异常，2例先天性心脏病，1例多指和并指畸形。男性8例，女性3例。8例病变为左侧，3例为右侧。父亲年龄超过35岁者8例，母亲年龄超过30岁者7例；多次妊娠者8例，不良孕产史者4例，母亲妊娠期糖尿病者2例。纠正胎龄44周时1例体格发育指标在同胎龄同性别的P10以下，2例NBNA评分小于35分，2例GMs评估扭动阶段为单调（轻度异常）；生后3月时11例患儿体格发育指标均在P10~P90之间，GMs评估不安运动阶段均正常，但歪嘴哭面容均无改善。结论：歪嘴哭面容患儿易合并其他畸形；患儿预后相对较好，但仍需长期随访，适时给予多学科合作干预。

Clinical features and follow-up study of neonatal asymmetric crying facies

Objective To evaluate the clinical characteristics and short-term outcomes of neonatal asymmetric crying facies(ACF),in order to improve recognition of the disease.Methods The clinical data of 11 infants with ACF between January 2010 and February 2012 were retrospectively studied.Physical and neurological development were followed up at correct gestational age 44 weeks and 3 months.Results Of the 11 infants with ACF,4 had ipsilateral ear malformation,2 had congenital heart disease and 1 had syndactyly and polydactyly.Of the 11 infants,8 were male and 3 were female.Eight infants presented with lesions on the left side and 3 presented with lesions on the right.The fathers were aged over 35 in 8 cases and the mothers were over 30 in 7 cases.Eight mothers had a history of at least 3 pregnancies and 2 infants were born to mothers with diabetes mellitus.Physical index was below P10 in 1 case and 2 cases showed a low NBNA score and mild abnormal GMs(poor repertoire PR) during the writhing period at correct gestational age 44 weeks.Physical index was between P10-P90 and GM assessment during the fidgety period showed normal movements in all infants at correct gestational age 3 months,but they still had ACF.Conclusions ACF is associated with a high rate of other congenital malformations.The short-term outcomes of ACF infants are satisfactory,but long-term follow-up and interdisciplinary cooperation are necessary to improve prognosis.