ABSTRACT

Infants who have mild or incomplete external features are candidates for long-term surveillance and a nonoperative approach.38 Recently, Noh et al. summarized clinical parameters which predict renal failure in children with prune belly syndrome.53 A total of 35 children with prune belly syndrome were analyzed. The authors concluded that bilateral abnormal kidneys on ultrasound or renal scan, a nadir serum creatinine of greater than 0.7 mg/dL, and clinical pyelonephritis are significant prognostic factors for the development of renal failure in these children. When a neonate has recurring bacteriuria, rising serum creatinine levels or increasing dilatation of the upper urinary tract, vesicostomy may be indicated. This expenditious procedure will usually provide effective bladder drainage and allow a major reconstructive procedure to be postponed until it can be performed under optimal circumstances. Cutaneous vesicostomy is an effective procedure for temporary diversion in patients with severe hydronephrosis, hydroureterosis and a poorly emptying bladder.56 Details of the operative technique of vesicostomy are described in Chapter 91. The urinary tracts in patients with prune belly syndrome are characterized by stasis; stasis predisposes to bacteriuria, which leads to deteriorating renal function as well as to troublesome symptoms. Some surgeons advocate pyelostomy in the neonatal period to achieve drainage. Cutaneous ureterostomies are offered only if the ureters are enormously dilated near the kidney and the pelvis are quite small.56 Patients with prune belly syndrome have a variability of degrees of renal and urinary tract involvement, so therapeutic options must be contemplated on an individual case-by-case basis, whether medical or surgical.