De Galan-Roosen A E, Kuijpers J C, Meershoek A P, van Velzen D
Department of Obstetrics and Gynaecology, Tweesteden Ziekenhuis, Tilburg, The Netherlands.
Eur J Obstet Gynecol Reprod Biol. 1998 Sep;80(1):55-61. doi: 10.1016/s0301-2115(98)00085-2.
: To determine the precise contribution of congenital malformations to perinatal mortality in a region.
Prospective, descriptive.
Region, Delft-Westland-Oostland (DWO) in the Netherlands.
The registration was based on data concerning all deliveries of women domiciled in the health region DWO of the Netherlands. The incidence and contribution of congenital malformations to perinatal death was evaluated by a team consisting of a gynaecologist. a paediatrician and a paediatric pathologist. Malformations were classified as lethal or nonlethal and recorded separately for stillbirth (from 28 weeks gestation) and liveborn infants with 7-day follow-up.
In 10 years (1993-1992) 28983 children were born in the region DWO. The perinatal mortality was calculated as 247 cases (0.85%). The overall incidence of congenital malformations in the perinatal death-group was 33%. Lethal congenital malformations were found in 51% of the cases in the stillbirth-group and 70% of the cases in the neonatal death-group. Congenital malformations of the central nervous system are mostly lethal in the stillbirth-group (45%). Cardiovascular- and pulmonary-defects were more prominent in the neonatal period (27% and 33% respectively of the neonatal deaths). Uro-genital and minor malformations (miscellaneous) are more often seen in perinatal deaths without being a contributor to the cause of death.
As most congenital malformations are multifactorial in origin, it is in the understanding and control of such conditions that efforts and resources should now be turned. Through a detailed postmortem fetal and placental examination and clinical-pathological correlations lethal congenital malformations were found in 51% in stillbirths (mainly central nervous system) and 70% in neonates (mainly cardiovascular and pulmonary defects).
确定某地区先天性畸形对围产期死亡率的确切影响。
前瞻性描述性研究。
荷兰代尔夫特 - 韦斯特兰 - 奥斯特兰（DWO）地区。
登记基于荷兰DWO健康区域内所有产妇分娩的数据。由一名妇科医生、一名儿科医生和一名儿科病理学家组成的团队评估先天性畸形对围产期死亡的发生率和影响。畸形被分为致死性或非致死性，并分别记录死产（孕28周起）和出生后7天随访的活产婴儿情况。
在10年（1993 - 1992年）间，DWO地区共出生28983名儿童。围产期死亡率计算为247例（0.85%）。围产期死亡组中先天性畸形的总体发生率为33%。死产组中51%的病例发现有致死性先天性畸形，新生儿死亡组中这一比例为70%。中枢神经系统先天性畸形在死产组中大多是致死性的（45%）。心血管和肺部缺陷在新生儿期更为突出（分别占新生儿死亡的27%和33%）。泌尿生殖系统和轻微畸形（其他）在围产期死亡中更常见，但并非死亡原因。
由于大多数先天性畸形是多因素起源的，现在应将努力和资源转向对这类情况的理解和控制。通过详细的胎儿和胎盘尸检以及临床病理相关性分析，发现死产中51%（主要是中枢神经系统）和新生儿中70%（主要是心血管和肺部缺陷）存在致死性先天性畸形。
