Perlman J M, Burns D K, Twickler D M, Weinberg A G
Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas 75235-9063, USA.
Pediatrics. 1995 Sep;96(3 Pt 1):521-3.
We report a set of triplets, two of whom were monochorionic diamnionic and demonstrated cerebellar hypoplasia and progressive arthrogryposis on an antenatal sonogram. At delivery the infants exhibited a Pena-Shokier phenotype. At autopsy, the twins were concordant for severe disruptive lesions of the cerebrum. The mechanism resulting in the devastating symmetric lesions may have been a transient cerebral vascular compromise associated with placenta vascular anastomoses characteristic of monochorionic twinning. This report accentuates the vulnerability of the monochorionic twin for ischemic cerebral injury.
我们报告了一组三胞胎，其中两个为单绒毛膜双羊膜囊双胎，产前超声检查显示小脑发育不全和进行性关节挛缩。分娩时，这些婴儿表现出佩纳 - 绍基尔综合征（Pena-Shokier syndrome）的表型。尸检时，这对双胞胎大脑均有严重破坏性病变。导致这种毁灭性对称病变的机制可能是与单绒毛膜双胎特有的胎盘血管吻合相关的短暂脑血管损伤。本报告强调了单绒毛膜双胎对缺血性脑损伤的易感性。
