A 5-day-old boy was brought with the complaint of prominent, loose abdomen. The abdominal wall was prominent, wrinkled with evidence of visible peristalsis (Fig. 1). Loops of intestines, liver, spleen and left kidney were all palpable. The left testes was not in the scrotal sac. All other systems were clinically normal. Ultrasonogram of abdomen showed only left kidney with normal parenchyma while other abdominal organs were normal. X-ray of chest was normal. A diagnosis of Prune Belly Syndrome was made. The child came at 6 months age with completely normal milestones. Abdominal wrinkles were less (Fig. 2).

The condition occurs once in 50,000 births, and classically comprises a triad: (i) Abdominal wall deficiency with wrinkled abdominal wall; (ii) Abnormal renal system including hydronephrosis, megaloureters and bladder and urethral abnormalities; and (iii) Cryptorchidism. These children usually die of renal anomalies and survive if the renal system is normal. The prognosis varies from death in utero to near normal life expectancy depending on the degree of renal dysplasia. Torsion of spleen is a rare complication. Respiratory function abnormalities and exercise intolerance are expected at later life due to abdominal muscle deficiency. Treatment is directed towards the type of renal anomaly. Orchidopexy and abdominal wall reconstruction are suggested.
 

A. Ramachandraiah,
Consultant Pediatrician, 4/419 Nagarjpet,
Cuddapah 516 001, AP., India.