What is the pathophysiology of prune-belly syndrome?

Updated: Nov 05, 2019
  • Author: James G Glasser, MD, MA, FACS; Chief Editor: Dharmendra J Nimavat, MD, FAAP  more...
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Answer

Prune-belly syndrome

Prune-belly syndrome includes dilatation of the ureter, collecting system, and bladder; undescended testes; and other abnormalities. This syndrome is caused by increased "apoptotic" cell death in the body-wall placode or inadequate migration of mesodermal cells, with retention of yolk sac elements. [13]

Characteristics of prune-belly syndrome include the following:

  • There is attenuation of the abdominal musculature.

  • Muscle fibers are absent and are replaced by thick collagenous aponeuroses.

  • Paradoxically, hypoplasia of the abdominal wall contrasts with hypertrophy of the bladder wall, which may cause bladder neck obstruction and the characteristic megaureters and dilated renal collecting system.

  • Faulty intercellular electrical conduction causes disordered muscular contraction and ineffective ureteric peristalsis.

  • Approximately 95% of infants with prune-belly syndrome are male; the absence of prostatic and seminal fluid precludes normal sperm development and causes infertility, as may the associated cryptorchidism.


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