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

Purpose: Urethral atresia is incompatible with life unless an alternative c ommunication between the bladder and amniotic sac exists. Although antenata l intervention may improve perinatal mortality, clinical consequences remai n. We outline the outcome after treatment of 6 patients born with urethral atresia. Materials and Methods: We reviewed the charts of 6 patients with urethral a tresia 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. Results: Oligohydramnios was demonstrated in 4 of the 6 patients (67%). A v esico-amniotic shunt had been placed in 2 patients (at more than 30 weeks o f gestation and in 1 at 17 weeks of gestation). The remaining 3 patients pr esented at birth with either a vesicocutaneous fistula or patent urachus. B ilateral or unilateral hydronephrosis was identified in 4 patients while 2 had severe renal dysplasia. Cystography identified moderate to high grade v esicoureteral reflux in all patients, and 5 (83%) had the prune belly syndr ome. 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 the m have received a renal transplant. An average of 7.8 (range 9 to 14) urolo gical procedures were performed on each patient. Progressive urethral dilat ion was not successful in the majority of our cases and ultimately 67% requ ired some form of supravesical diversion. Conclusions: Our study demonstrates that urethral atresia is not necessaril y fatal. Prenatal decompression allows survival and in some cases may even lead to normal bladder and renal function. A complicated clinical course re quiring extensive reconstruction is to be expected.