Chandran S, Lim M K, Yu V Y
Special Care Baby unit, Department of Paediatrics, RIPAS Hospital, Bandar Seri Begawan, Brunei Darussalam.
Arch Dis Child Fetal Neonatal Ed. 2000 Jan;82(1):F11-3. doi: 10.1136/fn.82.1.f11.
A case of amniotic band syndrome (ABS) presenting with acalvaria is reported. ABS includes a spectrum of non-genetic anomalies, varying from simple digital band constriction to major craniofacial and visceral defects, and even fetal death. Acalvaria is a rare congenital malformation characterised by the absence of the dome-like superior portion of the cranium comprising the frontal, parietal, and occipital bones and dura mater, in the presence of a normal skull base and facial bones with complete cranial contents. No two cases are the same. Acrania or absence of the flat skull bones with disorganised cerebral hemispheres have been reported in the presence of amniotic bands. ABS is an aetiological factor in acalvaria. Appropriate counselling for affected families needs to be given after prenatal diagnosis.
本文报道了一例以无颅骨畸形表现的羊膜带综合征（ABS）病例。羊膜带综合征包括一系列非遗传性异常，从简单的手指带状缩窄到严重的颅面和内脏缺陷，甚至导致胎儿死亡。无颅骨畸形是一种罕见的先天性畸形，其特征是颅顶穹窿状上部（包括额骨、顶骨和枕骨以及硬脑膜）缺失，而颅底和面部骨骼正常且颅内内容物完整。没有两例病例是相同的。有报道称，在存在羊膜带的情况下会出现无脑盖畸形或扁平颅骨缺失且脑半球紊乱的情况。羊膜带综合征是无颅骨畸形的一个病因。产前诊断后需要为受影响的家庭提供适当的咨询。
