ABSTRACT
Prune belly syndrome is characterized by a triad of abnorm-
alities, including an absence or deficiency of abdominal wall
musculature, cryptorchism, and anomalies of the urinary
tract. The characteristic deficiency of the abdominal wall
musculature was first described by Frohlich in 1839.1 Parker
first reported the association of the genitourinary anomalies
with the deficient abdominal musculature.2 The term ‘prune
belly syndrome’ was coined for this complex by Osler in
1901.3 In 1950, Eagle and Barrett further defined the triad of
absent abdominal wall musculature, undescended testes, and
urinary tract abnormalities.4 The incidence of prune belly
syndrome is estimated to be one in 29000 to one in 50000
live births.513 This syndrome occurs almost exclusively in
boys.13 It is very rare in females;13 only 5% of cases described
in the world literature have been reported to occur in
The pathogenesis of prune belly syndrome remains contro-
versial and many theories have been proposed to explain it.3,4
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.5,6,1517 An embryological theory
proposes that failure of primary mesodermal differentiation
leads to defective muscularization of both the abdominal wall
and the urinary tract.6,1517 Although both theories explain
some elements of the syndrome, they fail to explain others.
Reinberg et al.18 recently suggested that the two theories
should be regarded as complementary mechanisms, both
operating in any given case. They theorized that teratogenic
agents produce abnormal development of derivatives of the
lateral plate mesoderm and abnormal epithelialmesenchymal interactions, resulting in abnormal organ development
and mechanical or functional obstruction of the urinary tract.
Recently, Stephens and Gupta proposed a theory of abnormal
