单卵双胞胎中的OEIS综合征（脐膨出-泄殖腔外翻-肛门闭锁-脊柱畸形）

OEIS complex (omphalocele-exstrophy-imperforate anus-spinal defects) in monozygotic twins.

作者信息

Lee D H, Cottrell J R, Sanders R C, Meyers C M, Wulfsberg E A, Sun C C

机构信息

Department of Pathology, University of Maryland, Baltimore, USA.

出版信息

Am J Med Genet. 1999 May 7;84(1):29-33.

PMID:10213043

Abstract

The omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex is a consistent and recognizable pattern of midline abdominal and pelvic defects. It is rare, affecting 1 in 200,000 to 400,000 pregnancies and is even rarer in twin gestations. This is an autopsy study of OEIS complex in monozygotic twins after pregnancy termination at 20 weeks of gestation. Unremarkable family history but concordance of monozygotic twins for the defects may support the theory that early malformation complexes, e.g., OEIS, and monozygotic twinning are manifestations of the same disturbance of early blastogenesis.

摘要

脐膨出-膀胱外翻-肛门闭锁-脊柱裂（OEIS）综合征是一种具有连贯性且可识别的中线腹部和盆腔缺陷模式。它很罕见，在20万至40万次妊娠中出现1例，在双胎妊娠中更为罕见。这是一项对妊娠20周终止妊娠后的单卵双胎中OEIS综合征进行的尸检研究。家族史无异常，但单卵双胎的缺陷一致性可能支持这样一种理论，即早期畸形综合征，如OEIS，和单卵双胎是早期胚泡形成同一干扰的表现。