474例先天性畸形儿尸检分析

目的:分析474例先天性畸形儿系统畸次及疾病畸次;探索较为科学的先天性脑积水的病理诊断指标及脐带畸形对发育的影响.方法:主要根据WHO及张汇泉等对先天畸形的分类法,分析本院474例先天性畸形的尸检材料,对其中37例脑积水采用测量脑积液的量、脑皮质厚度及脑/体比例作为诊断指标,对脐带畸形等采用常规方法.结果:消化、心血管、神经系统畸形三者合计占先天性畸形儿的57.40%;脑积水占神经系统畸形的44.58%,脑/体比值＜0.125,积水超过10 ml;30例脐带畸形合并其他畸形占66%.结论:提出先天性脑积水诊断标准的初步意见,胎盘脐带畸形引发其他先天畸形有重要意义.

Analysis to the autopsy of congenital malformation in 474 cases

Objective:To analyze the number of System Malformation and Disease Malformation of congenital malformation in 474 cases . To explore the more scientific pathological diagnosis standard of congenital hydrocephalus,and to explore the relationship between umbilical cord and the fetus's growth and development.Methods:Of 474 cases autcpsy in our hospital were analyzed based on the classification of congenital malformation by WHO and Zhang Hui-quan. Of whom 37 cases of congenital hydrocephalus measure the quantity of the stagnant water and the thickness of the pallium were measured,and the brain/body value was calculated of the umbilical cord.Their length and diameter was measured,and the umbilical cords were observed under the microscope.Results:57.40% were digestive,cardioascular and nervous system malformation. 39 cases were hydrocephalus,which account for 44.58% of nervous system malformation. The brain/body value was less than 0.125,volume of the stagnant water exceeds 10ml. 79.49% cases of umbilical cord malformation accompanied other kind of malformation.Conclusion:A primary opinion on the diagnosis standard for congenital hydrocephalus is suggested.That umbilical and placental cord malformation trissers by other congenital malformations has important significance.