先天性畸形围产期干预的系统化管理

The systematized management of serial intervention for congenital malformation in perinatal period

目的报告对产前诊断先天性畸形的围产期干预进行系统化管理的实践经验，并讨论相关问题。方法本管理模式由“产前诊断和分娩中心”与“新生儿治疗及随诊中心”组成，两个中心随时保持业务交流与合作。管理模式的运行：超声科及产科医生通过超声等影像学检查发现畸形，家长到新生儿外科门诊咨询，必要时学科问进行业务交流与讨论，按计划分娩，并根据病情选择：配备转运暖箱的救护车接诊、普通救护车常规转诊、或留产院观察及出院后到新生儿外科门诊。转诊患儿急诊手术、择期手术或观察。结果2003年3月至2008年12月本院共接待经产前超声诊断各种类型先天性畸形的产前咨询与诊疗共322例。其中34例（10．6％）引产或生后放弃治疗；89例通过各种方式转诊至本院；76例（23．6％）于新生儿期手术，治愈率为89．5％；212例（65.8％）继续妊娠或出生后观察。结论本管理模式为适应我国现实医疗环境和北京市医疗资源配置的具体情况而建立，可以使患儿（及准父母）得到产前诊断、咨询、监测，生后转运、治疗及随访的系统化管理，对进一步提高先天性畸形的总体治疗水平起到了积极作用。

Objective To report our systematized management experience of the prenatal diagnosis and perinatal intervention for the congenital malformation. Methods The perinatal intervention manage system was composed of two centers, prenatal diagnosis and delivery center and neonate treatment and follow up center, in which the related information and work experience were exchanged routinely. The work mode of this system included the detection of congenital malformations by staff of ultrasound and obstetrician, consultation in clinic of neonate surgery, scheduled delivery, transportation of newborns by suitable vehicle, and surgical interventions. Results From Mar 2003 to Dec 2008, there were 322 cases of different kinds of congenital malformations detected in perinatal period by ultrasound, 89 cases were transported to our hospital by different manner. Among them, 34 cases （10.6%） were aborted or abandoned, and 76 neonates （23.6%） underwent surgical operations with cure rate of 89.5%. The rest 212 case were advised to continue the pregnancy and keep regular follow up. Conclusions Based on the current medical environment and medical resource allocation in Beijing, this perinatal intervention manage system can provide effective detection, consultation and optimal treatment for congenital malformations, and consequently, improve the outcome of these congenital defects.