Pathogenesis of the prune-belly syndrome: a functional urethral obstruction caused by prostatic hypoplasia

Pediatrics. 1984 Apr;73(4):470-5.

Abstract

Abdominal muscle deficiency, urinary tract abnormalities, and cryptorchidism are the three major features of the prune-belly syndrome, also referred to as triad syndrome or Eagle-Barrett syndrome. The etiology is unclear and the pathogenesis a subject of continuing debate. Clinical and pathologic experience with seven cases of prune-belly syndrome is reviewed. Findings indicate that the urogenital anomalies can be attributed to a functional urethral obstruction which in turn is the result of prostatic hypoplasia. The histology of the abdominal wall is that of atrophy-ie, the degeneration of already formed muscle--and not of primitive muscle. This observation supports the theory that the abdominal muscle hypoplasia is a nonspecific lesion, resulting from fetal abdominal distension of various causes. Transient fetal ascites may be an important feature of the prune-belly syndrome.

MeSH terms

  • Abdominal Muscles / abnormalities
  • Abnormalities, Multiple / pathology
  • Ascites / complications
  • Female
  • Humans
  • Infant, Newborn
  • Kidney / abnormalities
  • Male
  • Pregnancy
  • Prostate / abnormalities*
  • Prune Belly Syndrome / etiology*
  • Ureter / abnormalities
  • Urethra / abnormalities
  • Urethral Obstruction / etiology*
  • Urinary Bladder / abnormalities