[肺囊性腺瘤样畸形、双侧肾缺如及左心发育不全。波特综合征中的一种罕见关联]

[Cystic adenomatoid malformation of the lung, bilateral renal agenesis and left heart hypoplasia. An unusual association in Potter's syndrome].

作者信息

Carles D, Dallay D, Serville F, Maugey-Laulom B, Alberti E M, Tissot H, Weichhold W, Wursten-Guitton F

机构信息

Unité de Foetopathologie, Groupe Hospitalier Pellegrin, Bordeaux.

出版信息

Ann Pathol. 1992;12(6):367-70.

PMID:1294159

Abstract

We describe an autopsy case of congenital cystic adenomatoid malformation of the lung (CCAM) associated with bilateral renal agenesis. Prenatal ultrasound examination showed additional left heart hypoplastic syndrome. A therapeutic abortion was induced at 23 weeks of gestation. The association CCAM-bilateral renal agenesis is a rare condition (5 cases previously described) which has to be known because of the mitigation effect of the CCAM on the oligohydramnios determined by bilateral renal agenesis. However, this instance is usually associated with oligohydramnios. The pathogenesis of polyhydramnios in isolated CCAM is discussed in regard with these data.

摘要

我们描述了一例与双侧肾缺如相关的先天性肺囊性腺瘤样畸形（CCAM）的尸检病例。产前超声检查显示合并左心发育不全综合征。妊娠23周时引产。CCAM与双侧肾缺如的关联是一种罕见情况（此前报道过5例），鉴于CCAM对双侧肾缺如所致羊水过少的缓解作用，必须对此有所了解。然而，这种情况通常与羊水过少相关。结合这些数据讨论了孤立性CCAM中羊水过多的发病机制。