D. Innes Williams

The Prune Belly Syndrome

Agenesis of the abdominal musculature, urinary tract abnormalities and undescended testicles form a well known clinical complex often known as the prune belly syndrome (Fig. 141) of which many features have become better defined in recent years. The disease is undoubtedly rare, and most reported series are small; the present author’s total experience is of 34 cases. In its classical form the disorder is confined to the male, and although female examples of absent abdominal muscles have been reported they have not, in general, been associated with the classical anomaly of the urinary tract. A familial incidence is distinctly rare, but Harley et al. (1972) have reported two siblings with a mosaic chromosomal pattern. Burkholder and Williams (1967) found no chromosomal abnormalities in their cases.