B. Leeners et al., Prune-belly syndrome: Therapeutic options including in utero placement of a vesicoamniotic shunt, J CLIN ULTR, 28(9), 2000, pp. 500-507
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.