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

Congenital pulmonary airway malformationis 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 megacystisdue to posterior urethral valvesin 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 megacystison iliac veinsand 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.