ABSTRACT

Prune belly syndrome is characterized by a triad of abnormalities, including an absence or deficiency of abdominal wall musculature, cryptorchism, and anomalies of the urinary tract. The pathogenesis of prune belly syndrome remains controversial, and many theories have been proposed to explain it. One theory proposes that prenatal obstruction or dysfunction of the urinary tract causes urinary tract dilatation, fetal abdominal distension, and subsequent muscle wall hypoplasia and cryptorchism in males. Prune belly syndrome represents a spectrum of disease severity, ranging from those that die within the first few days of life to those that survive with relatively stable renal function in childhood. Abnormalities of the urinary tract are the major factors affecting the prognosis of patients with prune belly syndrome. The kidney in prune belly syndrome has many ranges of disorders, from total agenesis or dysplasia to no significant aberration.