139-Prune-Belly Syndrome

Chapter

Abstract

  • © 2018 Elsevier Inc. All rights reserved. Prune-belly syndrome is characterized by urologic abnormalities, flaccidity, or dysplasia in abdominal wall musculature, and cryptorchidism (in males). Because of the poorly developed abdominal wall muscles, the infant's abdomen has a wrinkled, prune-like appearance, thus giving origins of the syndrome's name. It occurs in 1?:?29,000 to 1?:?50,000 births and is more common in males than females and blacks compared with whites. The most common observation on prenatal ultrasound is bladder distention, which may be accompanied by hydronephrosis, dysplastic kidneys, or unilateral renal agenesis. Oligohydramnios is frequently seen. Prenatal interventions exist and may improve postnatal survival and function. At this time, no specific genetic etiology has been linked definitively to prune-belly syndrome. Recurrence risk is thought to be low, though this may change as genetic etiologies are identified and genetic testing improves.
  • Authors

    Digital Object Identifier (doi)

    International Standard Book Number (isbn) 13

  • 9780323445481
  • Start Page

  • 574
  • End Page

  • 576.e1