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CC BY-NC-ND 4.0 · Journal of Fetal Medicine 2019; 06(04): 191-194
DOI: 10.1007/s40556-019-00220-y
Brief Communication

Co-existence of CPAM with Megacystis Due to Posterior Urethral Valve and Cardiac Dysfunction in a Monozygotic Twin Pregnancy

Veronica Arora
1   Institute of Medical Genetics and Genomics, Sir Ganga Ram Hospital, New Delhi, India
,
Sunita Bijarnia-Mahay 
1   Institute of Medical Genetics and Genomics, Sir Ganga Ram Hospital, New Delhi, India
,
Meena Lall
1   Institute of Medical Genetics and Genomics, Sir Ganga Ram Hospital, New Delhi, India
,
Ishwar Chander Verma
1   Institute of Medical Genetics and Genomics, Sir Ganga Ram Hospital, New Delhi, India
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Abstract

Congenital pulmonary airway malformation is a rare developmental anomaly of the lower respiratory tract. It is classified into three major types depending on the size and number of cysts. Though the type II CPAM is known to be associated with congenital malformations, co-existence with a lower urinary tract obstruction (LUTO) has not been documented. We describe a novel combination of CPAM with megacystis due to posterior urethral valves in one of the twins of a monozygotic twin pregnancy. Further, we discuss the cardiovascular effects of megacystis. This was previously considered to be due to pressure effect of megacystis on iliac veins and inferior vena cava. Recently activation of RAS system has been shown to predict the severity of cardiac disease. Further studies will clarify the precise etiology of cardiac dysfunction in LUTO and megacystis.

Keywords

CPAM - Megacystis - Cardiomegaly - LUTO - Posterior urethral valve (PUV) - CCAM - Congenital malformations in twins


Publication History

Received: 13 August 2019

Accepted: 13 September 2019

Article published online:
08 May 2023

© 2019. Society of Fetal Medicine. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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