Prune belly syndrome (PBS) is a congenital disorder characterized by a partial or complete lack of abdominal musculature, severe urinary tract abnormalities, and bilateral cryptorchidism. The syndrome is typically recognized at birth or by ultrasound in utero. Severe renal dysplasia resulting in end-stage renal disease (ESRD) occurs in 50% of patients. ESRD and its complications are the most common reason a patient with PBS comes to the ED. Patients with PBS are at higher risk for recurrent urinary tract infection (UTI), chronic constipation, splenic torsion, and midgut and cecal volvulus.
Management and Disposition
Patients with PBS will most commonly frequent the ED because of complications of ESRD and their hemodialysis, continuous ambulatory peritoneal dialysis, or transplant. Because patients with PBS are at higher risk for splenic torsion and midgut or cecal volvulus, CT scan should be performed if sudden onset of severe abdominal pain occurs in these patients. Frequent UTIs and chronic constipation may prompt ED visits.
About one-half of patients progress to ESRD.
Chronic constipation is a common symptom in patients with PBS.
Gastrointestinal abnormalities are seen in 30% of patients with PBS.
Prune Belly Syndrome. A 44-year-old man with PBS, status post renal transplant, comes to the ED for abdominal pain and was ultimately diagnosed with constipation. (Photo contributor: Lawrence B. Stack, MD.)
Prune Belly Syndrome. A 2-year-old with PBS on hemodialysis comes to the ED for fever. Patient was bacteremic due to a dialysis catheter infection. (Photo contributor: Lawrence B. Stack, MD.)