Publication:

Symphony of challenges-prune belly syndrome with congenital heart disease

Rare congenital anomaly prune belly syndrome (PBS) affects male infants. Usually characterized by anterior abdominal wall muscle aplasia, cryptorchidism, and urinary tract defects. This is a similar case of a term male baby admitted to our NICU. Cryptorchidism, anterior abdominal wall weakness, and massive bladder dilation were found. The diagnosis was made clinically and then we confirmed the diagnosis by ultrasonography which revealed massive urinary bladder dilation with bilateral hydronephrosis, and thinning of the renal cortex. On clinical evaluation murmur could be heard for which 2D echo was planned and a large arterial septal defect (Left to right shunt) was present. Treatment mainly in volves orchidopexy, anterior abdominoplasty as well as urinary tract reconstruction.