Prune-belly syndrome: ongoing controversies regarding pathogenesis and management.
Classical prune-belly syndrome (also known as Triad syndrome, Eagle-Barrett syndrome, abdominal muscular deficiency syndrome) consists of a triad of anomalies: deficient abdominal wall musculature, urinary tract dilatation, and cryptorchidism. Although most investigators consider prune-belly syndrome a distinct entity, there is no consensus as to its pathogenesis despite extensive study of clinical cases and pathological material. Prognosis may vary from death in utero to a near-normal life expectancy. The lack of understanding of pathogenesis and wide range of severity result in dilemmas in treatment planning, and surgeons vary widely in their approach. This article discusses prune-belly syndrome, presents the currently favored hypotheses regarding its pathogenesis, and gives an overview of accepted management strategies.
Wheatley
J M
JM
F. Douglas Stephens Surgical Research Laboratory, Royal Children's Hospital, Melbourne, Australia.
Stephens
F D
FD
Hutson
J M
JM
D000009:Abdominal Muscles / Q000002:abnormalities; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D010641:Phenotype; D011379:Prognosis; D011535:Prune Belly Syndrome / Q000196:embryology* / Q000601:surgery*