Prune Belly Syndrome Clinical Presentation

Updated: Jan 17, 2020
  • Author: Israel Franco, MD, FAAP, FACS; Chief Editor: Bradley Fields Schwartz, DO, FACS  more...
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Presentation

History and Physical Examination

Children with prune belly syndrome can present with myriad renal, ureteral, and urethral abnormalities. Obstruction and/or upper urinary tract dilatation is not unusual in these children. The site of obstruction can vary from as high as the pelviureteral junction to as low as the prostatic membranous urethra.

A lack of abdominal muscles leads to a poor cough mechanism, which, in turn, leads to increased pulmonary secretions. Weak abdominal muscles lead to constipation because of an inability to perform the Valsalva maneuver, which helps push the stool out of the rectum during defecation.

In a review of 46 patients (44 boys and 2 girls) with prune belly syndrome seen at a pediatric urology clinic, the following were the most common clinical manifestations [2] :

  • Hydroureteronephrosis – 97.8%
  • Vesicoureteral reflux – 78.3%
  • Significant pulmonary insufficiency – 10.9%
  • Congenital malformations – 39.1%
  • Chronic kidney disease – 39.1%; 17.4% underwent kidney transplantation