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

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. © 2000 John Wiley & Sons, Inc. J Clin Ultrasound 28:500–507, 2000. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Journal of Clinical Ultrasound Wiley

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

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References (27)

Publisher
Wiley
Copyright
Copyright © 2000 John Wiley & Sons, Inc.
ISSN
0091-2751
eISSN
1097-0096
DOI
10.1002/1097-0096(200011/12)28:9<500::AID-JCU10>3.0.CO;2-8
Publisher site
See Article on Publisher Site

Abstract

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. © 2000 John Wiley & Sons, Inc. J Clin Ultrasound 28:500–507, 2000.

Journal

Journal of Clinical Ultrasound – Wiley

Published: Nov 1, 2000

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