Wang J K, Changchien C C
Department of Obstetrics and Gynecology, Chang Gung Memorial Hospital, Kaohsiung, Taiwan, R.O.C.
Changgeng Yi Xue Za Zhi. 1990 Dec;13(4):343-9.
The amniotic band syndrome (ABS) includes intrauterine amputations, syndactyly, fetal disruption, fetal compression deformities and constriction rings associated with disruption of the amniotic membrane. The defects caused by ABS varied from simple digital band constrictions, limb defects to major visceral and craniofacial defects; fetal death may also occur. Because the ABS is a sporadic disorder that may mimic recurrent disorders, such as achondroplasia, anencephaly, Apert's syndrome, etc, it is very important to do appropriate genetic counseling and accurate diagnosis for every infant born with multiple birth defects. We report two severely damaged cases with typical features. This article also reviews the clinical features of ABS, its etiology, pathogenesis, and the status of prenatal and neonatal diagnosis are also discussed.
羊膜带综合征(ABS)包括宫内截肢、并指(趾)畸形、胎儿破坏、胎儿压迫畸形以及与羊膜破裂相关的缩窄环。ABS所致缺陷范围从简单的指(趾)带缩窄、肢体缺陷到严重的内脏和颅面缺陷;也可能发生胎儿死亡。由于ABS是一种散发性疾病,可能类似复发性疾病,如软骨发育不全、无脑儿、阿佩尔综合征等,因此对每例出生时伴有多种出生缺陷的婴儿进行适当的遗传咨询和准确诊断非常重要。我们报告两例具有典型特征的严重受损病例。本文还综述了ABS的临床特征、病因、发病机制,并讨论了产前和新生儿诊断情况。