影响先天性膈疝患儿生存率的危险因素分析

The analysis for risk factors of congenital diaphragmatic hernia

目的探讨影响先天性膈疝(CDH)患儿生存率的危险因素。方法青岛市第八人民医院产科1995年10月至2010年09月15年间分娩临床资料完整的CDH患儿11例,回顾性分析出生胎龄、母体分娩方式、诊断时机、患儿染色体核型、膈疝发生的部位、合并的其他结构畸形、是否有肝突出、纵膈移位等因素。结果 11例CDH中单纯CDH 6例,染色体核型异常CDH 3例,伴相关结构畸形者4例(其中2例同时伴染色体核型异常)。左侧膈疝8例,右侧膈疝3例。产前诊断1例,其余均产后诊断或死亡后尸检诊断。患儿的总体病死率为81.8%。其中围产儿死亡率72.7%(8/11),新生儿晚期死亡率9.1%(1/11),幸存率18.2%(2/11)。结论 CDH患儿病死率很高。右侧膈疝、肝突出、纵膈移位等可能是单纯性CDH预后不良的危险因素。CDH合并相关结构畸形和染色体核型异常者预后不良。加强产前诊断中高危因素的评估有望提高CDH幸存率。

Objective：To evaluate the influencing factors which have bad-prognosis predictive value for CDH.Methods： We reviewed data from 11 cases of congenital diaphragmatic hernia from Oct 1995 to Sept 2010 in Obstetrical Department of The eighth people′s Hospital of Qingdao.The main data analyzed were gestational age at birth,fetal karyotyping,side of diaphragmatic defect,presence of associated structural malformations,hepatic herniation,and severe mediastinal shift.Results： The overall CDH mortality rate was 81.8%.Rates of perinatal deaths,late neonatal deaths,and survival were 72.7%,9.1%,and 18.2%,respectively.Eight（72.7%） cases had a left diaphragmatic defect and 3（27%） had a right lesion.Associated structural malformations were observed in 4（36%） cases,in which 1 fetuses had a normal karyotype and 3 had chromosomal abnormalities.Isolated congenital diaphragmatic hernia was confirmed in 6（55%） cases.Conclusion： CDH mortality was very high.In cases of isolated congenital diaphragmatic hernia,mortality is probably related to the presence of herniated liver,right-sided lesion,and major mediastinal shift.Bad prognosis are associated with the presence of other structural defects or chromosomal abnormalities.Enhancing the evaluation of above factors in antenatal diagnosis might improve CDH prognosis.