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
