Congenital Cystic Adenomatoid Malformation: Is There a Need for Pregnancy Termination?

C Iavazzo,D Hassiakos,M Eleftheriades,D Botsis,A M Bacanu

doi:10.1155/2012/397508

Abstract

Aim. Congenital cystic adenomatoid lung malformation is a rare unilateral dysplasia of the lung. Three pathologic types are described in the literature: type I with cysts >2 cm, type II with cysts <1 cm, and type III with microcysts. The aim of this paper is to present a case of a fetus with congenital cystic adenomatoid lung malformation and discuss the necessity for pregnancy termination according to its prognosis and future mortality. Case. A 36-year-old pregnant woman (para: 1, gravida: 1) presented in our department for anatomy ultrasound screening at 20 + 1 weeks of gestation. The ultrasound detected a cystic adenomatoid right lung malformation measuring 1.45 × 1.67 cm which caused mediastinal shift of the heart and the lung to the left side. Other findings were cysts of the choroid plexus and echogenic intracardiac foci. The parents after genetic counseling decided pregnancy termination. The pregnant received cabergoline for ablactation. Conclusion. Congenital cystic adenomatoid lung malformation has different prognosis according to the type (69% in type I, 0% in types II and III). Fetal hydrops, cardiac and skeletal anomalies, Potter's syndrome, and gastrointestinal atresia are common cofindings. Genetic counseling is necessary, and pregnancy termination is proposed to the cases with poor prognosis.

Highlights

Congenital cystic adenomatoid lung malformation (CCAM) is a rare, usually, unilateral dysplasia of the lung
The aim of this paper is to present a case of a fetus with congenital cystic adenomatoid lung malformation and discuss the need for pregnancy termination in the cases with poor prognosis
CCAM is a rather rare entity which is usually identified in the anatomy ultrasound scanning of the second trimester

Summary

Introduction

Congenital cystic adenomatoid lung malformation (CCAM) is a rare, usually, unilateral dysplasia of the lung. The entity was first reported in 1787 with the description of the absence of the lungs [1]. It results from an abnormal maturation of the bronchopulmonary tree, especially an overgrowth of the terminal bronchioles [2]. The etiology of the entity remains unknown. Hamartomatous change in the terminal bronchioles or an arrest in their embryological development between 7 and 15 weeks of gestation is suspected [3]. Decreased apoptosis plays a role [3]. The related genes in the pathogenesis are HOXB5, Fgf, and PDGFB [4, 5]

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Conclusion