The Prune Belly Syndrome (PBS) may show a spectrum of phenotypic expression.
Classification:
(1) isolated (classic triad plus sequelae)
(2) syndromic (classic triad as part of a genetic syndrome)
(3) BPS-plus (additional manifestations not part of a genetic syndrome)
Classic triad:
(1) lax abdominal musculature
(2) distended urinary tract
(3) bilateral cryptorchidism
Sequeale:
(1) pulmonary hypoplasia
(2) constipation
(3) scoliosis
Syndromes associated with PBS include:
(1) Duchenne Muscular Dystrophy
(2) Pierre Robin Sequence
(3) VACTERL
(4) megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS)
Conditions associated with BPS-Plus:
(1) hepatoblastoma
(2) hearing loss
(3) infantile stroke and optic nerve atrophy
(4) pancreatic atrophy with type 2 diabetes mellitus
(5) mydriasis with pupils nonreactive to light
