In utero intervention in a patient with prune-belly syndrome and severe urethral hypoplasia.
Prenatal ultrasound scanning of a 20-year-old woman at 17 weeks of gestation revealed findings suggestive of bladder outlet obstruction, including bladder distension, dilated bilateral ureters, urinary ascites, and oligohydramnios. Vesicoamniotic shunts were placed with decompression of the bladder and correction of the amniotic fluid levels. Labor was induced at 36 weeks' gestation. At birth, the infant was noted to have prune-belly syndrome with severe urethral hypoplasia, a variant usually associated with a poor prognosis, necessitating vesicostomy for bladder drainage. We present a case of a patient with prune-belly syndrome and bladder outlet obstruction in whom early intervention resulted in an excellent outcome with preservation of renal and pulmonary function.
Perez-Brayfield
M R
MR
Department of Urology, Emory University School of Medicine, Children's Healthcare of Atlanta, Atlanta, Georgia, USA.
Gatti
J
J
Berkman
S
S
Eller
D
D
Broecker
B
B
Massad
C
C
Kirsch
A
A
Smith
E
E
D000328:Adult; D001201:Ascites / Q000151:congenital / Q000000981:diagnostic imaging / Q000628:therapy; D002404:Catheterization / Q000379:methods*; D005260:Female; D005315:Fetal Diseases / Q000000981:diagnostic imaging / Q000628:therapy*; D006801:Humans; D008297:Male; D016104:Oligohydramnios / Q000000981:diagnostic imaging / Q000628:therapy; D011247:Pregnancy; D011535:Prune Belly Syndrome / Q000000981:diagnostic imaging / Q000628:therapy*; D016216:Ultrasonography, Prenatal; D001748:Urinary Bladder Neck Obstruction / Q000151:congenital / Q000000981:diagnostic imaging / Q000628:therapy*; D014556:Urine