Prune-belly syndrome: therapeutic options including in utero placement of a vesicoamniotic shunt.
The prune-belly syndrome (PBS) consists of abdominal wall distention with deficiency of the abdominal wall musculature, urinary tract abnormalities, and cryptorchidism. The impaired drainage of the bladder leads to oligohydramnios and pulmonary hypoplasia. We present 4 cases of PBS diagnosed by prenatal 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 performed because of a twin pregnancy, and the neonate with PBS died the day of delivery by cesarean section at 31 weeks' menstrual age. In the other case, vesicoamniotic shunt therapy was successfully performed, and a healthy child was delivered. Several conditions must be met for vesicoamniotic shunt therapy to have a good chance of success: the karyotype must be normal, other malformations must be excluded by careful sonographic examination, and renal function must be normal, as determined by serial analyses of fetal urine. Generally, the shunt should be inserted as early as possible.
Leeners
B
B
Department of Obstetrics and Gynaecology, Rhenish-Westphalian Technical University Hospital Aachen, Pauwelsstrasse 30, D-52057 Aachen, Germany.
Sauer
I
I
Schefels
J
J
Cotarelo
C L
CL
Funk
A
A
D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging; D000328:Adult; D004200:Diseases in Twins; D017809:Fatal Outcome; D005260:Female; D005315:Fetal Diseases / Q000000981:diagnostic imaging / Q000235:genetics / Q000601:surgery*; D005333:Fetus / Q000601:surgery*; D006801:Humans; D007231:Infant, Newborn; D007621:Karyotyping; D007668:Kidney / Q000196:embryology / Q000502:physiology; D008297:Male; D011247:Pregnancy; D011379:Prognosis; D011535:Prune Belly Syndrome / Q000000981:diagnostic imaging / Q000235:genetics / Q000601:surgery*; D016896:Treatment Outcome; D018084:Ultrasonography, Interventional; D016216:Ultrasonography, Prenatal; D001743:Urinary Bladder; D014546:Urinary Catheterization / Q000295:instrumentation