我国321例先天性膈疝患儿流行病学分析

为了解我国先天性膈疝的基本状况和影响患儿预后的危险因素。１９８６年９月～１９９３年１２月，在全国出生缺陷监测网中监测妊娠２８周至出生后７天的住院分娩围产儿。结果显示：在４７７７２２０例围产儿中，共检出３２１例先天性膈疝，膈疝发病率０．７／万；患儿围产期总病死率为８４．１％，其中死胎、死产占２８．１％，新生儿７天内死亡占７１．９％。围产期死亡与存活病例在出生体重上存在显著性差异。５４．５％的患儿伴有其他畸形；死亡患儿中伴发心、肺和中枢神经系统严重畸形所占的比例最大，是引起患儿死亡的重要因素。单发畸形病例病死率为７４．７％，多发畸形病例为９２．０％，两者有显著性差异。经产前诊断的患儿占１１．３％，以多发畸形所占比例大。说明我国膈疝畸形产前诊断率低，畸形严重，患儿围产期病死率高，预后差。

Epidemiological Studies on 321 Children with Congenital Diaphragmatic Hernia in China

Perinatal babies delivered in hospitals with gestation of 28 weeks to seven days after birth were monitored by National Birth Defects Monitoring Network from September 1986 to December 1993 to study the prevalence of congenital diaphragmatic hernia in China and the risk factors contributing to their prognosis. Results showed that 321 cases of congenital diaphragmatic hernia in 4 777 220 perinatal babies were detected with an incidence of 0.7 per ten thousand, a case fatality ratio of 84.1 percent and , fetal death and stillbirth accounting for 28.1 percent of the total perinatal deaths. There was significant difference in birth weight between perinatal deaths and survival perinatal. And, 54.4 percent of the cases complicated with other congenital deformities and severe deformities in heart, lung and central nervous system accounted for the most proportion of them, which were the most important factors contributing to death. A case fatality ratio of cases with single malformation was 74.7 percent and that of those with multiple malformation 92 percent, with a statistically significant difference. The cases diagnosed antenatally accounted for 11.3 percent of the total, and multiple malformation accounted for a large proportion of them. It indicated that proportion of antenatal diagnosis for congenital diaphragmatic hernia deformity was lower, and their perinatal case fatality ratio was higher and their prognosis worse.