Prune-belly syndrome: Therapeutic options including in utero placement of a vesicoamniotic shunt

The prune-belly syndrome (PBS) consists of abdominal wall distention with d eficiency of the abdominal wall musculature, urinary tract abnormalities, a nd cryptorchidism. The impaired drainage of the bladder leads to oligohydra mnios and pulmonary hypoplasia. We present 4 cases of PBS diagnosed by pren atal sonography. In 2 cases, vesicoamniotic shunt therapy was not indicated because of a poor prognosis based on sonographic and laboratory findings; the pregnancies were terminated. In another case, treatment was not perform ed because of a twin pregnancy, and the neonate with PBS died the day of de livery by cesarean section at 31 weeks' menstrual age. In the other case, v esicoamniotic shunt therapy was successfully performed, and a healthy child was delivered. Several conditions must be met for vesicoamniotic shunt the rapy to have a good chance of success: the karyotype must be normal, other malformations must be excluded by careful sonographic examination, and rena l function must be normal, as determined by serial analyses of fetal urine. Generally, the shunt should be inserted as early as possible. (C) 2000 Joh n Wiley & Sons, Inc.