Further evidence of the etiology of prune belly syndrome provided by a transient massive intraabdominal cyst in a female

J Pediatr Surg. 2016 Aug;51(8):1390-3. doi: 10.1016/j.jpedsurg.2016.05.017. Epub 2016 Jun 1.

Abstract

We present a female neonate born with prune belly syndrome (PBS) in whom a large intraabdominal cyst was diagnosed at 12weeks of gestation. Rapid and exponential growth of the cyst caused pressure effects on the intraabdominal organs and stretching of the anterior abdominal wall by 19weeks of gestation. This led to drainage of the massive cyst at 20weeks of gestation to prevent fetal demise. This case provides further clues to the likely etiology of PBS: transient stretching and attenuation of the fetal abdominal wall secondary to gross fetal abdominal distension - from any cause.

Keywords: Etiology; Fetal cyst; Pathogenesis; Prune belly syndrome (PBS).

Publication types

  • Case Reports

MeSH terms

  • Abdominal Wall / abnormalities*
  • Abdominal Wall / embryology*
  • Adult
  • Cysts / diagnostic imaging
  • Cysts / embryology*
  • Drainage
  • Female
  • Fetal Death / prevention & control
  • Fetal Therapies
  • Humans
  • Infant, Newborn
  • Male
  • Pregnancy
  • Prune Belly Syndrome / diagnostic imaging
  • Prune Belly Syndrome / embryology*
  • Ultrasonography, Prenatal