To the Editor.—The retrospective review of 19 patients with prune-belly syndrome reported by Burke et al1 provides additional insight in-to the natural history of this interesting disease. However, certain aspects of the disorder are worthy of additional comment.
The urinary abnormality in the prune-belly syndrome represents a spectrum of severity characterized by varying degrees of altered development of the kidneys, ureters, and bladder. In extreme cases, renal dysplasia is so severe that the number of glomerulo-tubular units may be markedly reduced or even absent, and early death from uremia, or profound and progressive azotemia, is inevitable. Conversely, the renal lesion may be minimal. The ureters are variably dilated, and ineffectual ureteral peristalsis may lead to severe degrees of stasis. The bladder is variably enlarged, and poor detrusor contraction, especially of the dome, is usually a factor in incomplete vesical emptying and measurable postvoiding residuals.2
We agree that
PERLMUTTER AD, RETIK AB. PRUNE-BELLY SYNDROME. Am J Dis Child. 1970;119(2):191. doi:10.1001/archpedi.1970.02100050193025