The Prune Belly Syndrome (PBS) may show a spectrum of phenotypic expression.


(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



(1) pulmonary hypoplasia

(2) constipation

(3) scoliosis


Syndromes associated with PBS include:

(1) Duchenne Muscular Dystrophy

(2) Pierre Robin Sequence


(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

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