[Prune belly syndrome. Prenatal diagnosis and obstetric procedure]

With an incidence of between 1 in 30,000 and 1 in 50,000 births, prune-belly syndrome (PBS) is a rare malformation syndrome. The phenotypical and pathoanatomic changes range from discrete expression to very severe malformation complexes with extremely poor prognosis. Malformations of the kidneys and the efferent urinary tract are common. In such cases, oligohydramnios often develops early in such fetuses, followed by Potter's sequence. This syndrome can generally be diagnosed by timely ultrasound investigations, i.e., before the end of the first half of pregnancy. Striking features in the fetus are often oligohydramnios and a greatly dilated urinary bladder, more rarely multicystic-dysplastic kidneys and other malformations of the urogenital tract. Diagnostic possibilities and obstetric procedure are described on the basis of ten cases seen between January 1984 and July 1987 at Heidelberg University Gynecological Clinic.