Urethral atresia: long-term outcome in 6 children who survived the neonatal period

Urethral atresia is incompatible with life unless an alternative communication between the bladder and amniotic sac exists. Although antenatal intervention may improve perinatal mortality, clinical consequences remain. We outline the outcome after treatment of 6 patients born with urethral atresia.

We reviewed the charts of 6 patients with urethral atresia treated at the Children's Hospital of Michigan between 1982 and 1999. Patient age ranged from 3 to 17 years (mean 9). All patients were males (46 XY) and presented at birth or in early infancy.

Oligohydramnios was demonstrated in 4 of the 6 patients (67%). A vesico-amniotic shunt had been placed in 2 patients (at more than 30 weeks of gestation and in 1 at 17 weeks of gestation). The remaining 3 patients presented at birth with either a vesicocutaneous fistula or patent urachus. Bilateral or unilateral hydronephrosis was identified in 4 patients while 2 had severe renal dysplasia. Cystography identified moderate to high grade vesicoureteral reflux in all patients, and 5 (83%) had the prune belly syndrome. Mean serum creatinine at age 1 year was 1.3 mg/dl (range 0.5 to 2.1). Renal failure occurred in 5 patients (83%) before age 10 years and 4 of them have received a renal transplant. An average of 7.8 (range 9 to 14) urological procedures were performed on each patient. Progressive urethral dilation was not successful in the majority of our cases and ultimately 67% required some form of supravesical diversion.

Our study demonstrates that urethral atresia is not necessarily fatal. Prenatal decompression allows survival and in some cases may even lead to normal bladder and renal function. A complicated clinical course requiring extensive reconstruction is to be expected.