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目的通过病理解剖学诊断分析中国出生缺陷高发区死胎死产病因及孕周的分布特点,为提高出生缺陷监测诊断准确性提供重要依据。方法由山西省吕梁地区医院收集2004年2月至2006年3月间160例死胎死产病例,由儿科病理学专家进行尸体解剖及病理学检查建立数据库,将病理检查发现的出生缺陷按照国际疾病ICD-10编码分类,同时将不同部位出生缺陷按照不同孕周分组后进行统计分析。结果①160例死亡病例中确诊为出生缺陷的病例占84.4%(135/160),无出生缺陷的病例占15.6%(25/160);②孕周<20、20周～和28周～中有出生缺陷的病例占全部尸检病例的比例分别为6.3%(10/160)、26.9%(43/160)和51.3%(82/160);③单发体表和单发内脏畸形共占28.2%(38/135),多发体表、多发内脏及二者合并畸形占71.9%(97/135);孕28周前死亡的病例中有内脏畸形的占11.9%(19/160),孕28周～死亡的病例中有内脏畸的占45.0%(72/160);④神经管畸形仍然是死胎死产的主要死因,占36.3%(58/160),其他非致死性出生缺陷的死因包括弥漫性肺出血、胎粪吸入综合征、呼吸窘迫综合征、颅内出血等,其中28.1%(45/160)死于孕28周后。结论对死胎死产病例进行病理解剖学检查,可以大大提高出生缺陷诊断准确性,降低部分内脏畸形漏报率,为完善出生缺陷监测、提高出生缺陷监测质量提供可靠依据。
Objective To analyze the distribution of the causes of stillbirth and gestational age in China with high incidence of birth defects by pathological anatomy and provide an important basis for improving the diagnostic accuracy of birth defects monitoring. Methods A total of 160 cases of stillbirths were collected from Luliang Regional Hospital in Shanxi Province from February 2004 to March 2006. The autopsy and pathological examination were performed by pediatric pathologists to establish a database of birth defects detected by pathology according to international diseases ICD-10 coding classification, at the same time different parts of birth defects grouped according to different gestational age for statistical analysis. Results ① Among the 160 deaths, 84.4% (135/160) were diagnosed as having birth defects and 15.6% (25/160) without birth defects. The gestational age was less than 20 weeks and 28 weeks The incidences of birth defects accounted for 6.3% (10/160), 26.9% (43/160) and 51.3% (82/160) of the total autopsy cases, respectively. ③ The total number of single and single visceral malformations accounted for 28.2% (38/135), multiple surface disease, multiple internal organs and the combined malformations accounted for 71.9% (97/135); those who died 28 weeks before pregnancy had visceral malformations accounted for 11.9% (19/160), 28 weeks pregnant 45.0% (72/160) of visceral malformations occurred in the cases of death; ④ The incidence of neural tube defects was still the leading cause of stillbirths, accounting for 36.3% (58/160); the causes of other non-fatal birth defects included diffuse Pulmonary hemorrhage, meconium aspiration syndrome, respiratory distress syndrome, intracranial hemorrhage and so on, of which 28.1% (45/160) died after 28 weeks pregnant. Conclusion The pathological anatomy of stillbirth patients can greatly improve the diagnostic accuracy of birth defects and reduce the false negative rate of some visceral malformations, and provide a reliable basis for improving the monitoring of birth defects and improving the quality of birth defects monitoring.