Prognostic factors for long-term renal function in boys with the prune-belly syndrome

Renal failure develops in 25 to 30% of patients with the prune-belly syndrome. The clinical parameters associated with renal failure in this condition are not well understood. We determined which factors predict renal failure in children with the prune-belly syndrome.

We reviewed the records of 35 patients with the prune-belly syndrome who were treated at our hospital during a 37-year period. Nadir serum creatinine, time to nadir creatinine, presence or absence of a patent urachus, presence and severity of urinary tract infection, and renal failure were analyzed.

Mean followup was 14 years 2 months. In 2 of the 17 patients with a nadir serum creatinine of less than 0.7 mg./dl. renal failure developed. Each of these 2 children also had clinical pyelonephritis and urosepsis. In 12 of the 13 patients nadir serum creatinine greater than 0.7 mg./dl. strongly predicted renal failure. Nonfebrile urinary tract infection within the first year of life was not predictive of outcome (p = 0.8) but a history of clinical pyelonephritis correlated with eventual renal failure (p<0.001). Urosepsis also correlated with renal failure (p = 0.008). Children with at least 1 normal kidney on renal ultrasound or renal scan had a significantly lower chance of renal failure (p<0.001).

Bilaterally abnormal kidneys on ultrasound or renal scan, a nadir serum creatinine of greater than 0.7 mg./dl. and clinical pyelonephritis are prognostic for renal failure.