Higginbottom M C, Jones K L, Hall B D, Smith D W
J Pediatr. 1979 Oct;95(4):544-9. doi: 10.1016/s0022-3476(79)80759-3.
Seventy-nine patients with the amniotic band disruption complex, including 54 infants with multiple system involvement and 25 with affected limbs alone, were evaluated. No two cases of the disorder were exactly alike. Defects varied from simple digital band constrictions to major craniofacial and visceral defects; fetal death may also occur. Amniotic rupture appeared to cause injury through three basic mechanisms: (1) interruption of normal morphogenesis; (2) crowing of fetal parts; and (3) disruption of previously differentiated structure. Comparison of 35 cases in which the timing of amniotic rupture could be estimated suggests that early amniotic rupture results in multiply affected infants who are frequently aborted or stillborn, whereas later rupture results primarily in limb involvement. Our findings indicate that both the spectrum of the developmental pathology and the nature of fetal outcome are determined by the timing of amniotic rupture. Appreciation of the mechanism which explains the disparate appearances of infants with the amniotic band disruption complex will allow more acurate diagnosis and appropriate counseling with respect to the sporadic nature of the disorder.
对79例羊膜带破裂综合征患者进行了评估，其中包括54例有多系统受累的婴儿和25例仅肢体受累的婴儿。该疾病的任何两例都不完全相同。缺陷从简单的手指束带狭窄到严重的颅面和内脏缺陷不等；也可能发生胎儿死亡。羊膜破裂似乎通过三种基本机制造成损伤：(1) 正常形态发生的中断；(2) 胎儿部分的拥挤；(3) 先前分化结构的破坏。对35例可估计羊膜破裂时间的病例进行比较表明，早期羊膜破裂会导致多系统受累的婴儿，这些婴儿常发生流产或死产，而晚期破裂主要导致肢体受累。我们的研究结果表明，发育病理学的范围和胎儿结局的性质均由羊膜破裂时间决定。了解解释羊膜带破裂综合征婴儿不同表现的机制，将有助于更准确的诊断，并就该疾病的散发性进行适当的咨询。
