Pathogenesis of the prune-belly syndrome: a functional urethral obstruction caused by prostatic hypoplasia

Abdominal muscle deficiency, urinary tract abnormalities, and cryptorchidism are the three major features of the prune-belly syndrome, also referred to as triad syndrome or Eagle-Barrett syndrome. The etiology is unclear and the pathogenesis a subject of continuing debate. Clinical and pathologic ex...

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Bibliographic Details
Published in:Pediatrics (Evanston) 1984-04, Vol.73 (4), p.470-475
Main Authors: MOERMAN, P, FRYNS, J.-P, GODDEERIS, P, LAUWERYNS, J. M
Format: Article
Language:English
Subjects:
Abdominal Muscles - abnormalities
Abnormalities, Multiple - pathology
Ascites - complications
Biological and medical sciences
Female
Humans
Infant, Newborn
Kidney - abnormalities
Male
Malformations of the urinary system
Medical sciences
Nephrology. Urinary tract diseases
Pregnancy
Prostate - abnormalities
Prune Belly Syndrome - etiology
Ureter - abnormalities
Urethra - abnormalities
Urethral Obstruction - etiology
Urinary Bladder - abnormalities
Urinary tract. Prostate gland
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Description
Summary:Abdominal muscle deficiency, urinary tract abnormalities, and cryptorchidism are the three major features of the prune-belly syndrome, also referred to as triad syndrome or Eagle-Barrett syndrome. The etiology is unclear and the pathogenesis a subject of continuing debate. Clinical and pathologic experience with seven cases of prune-belly syndrome is reviewed. Findings indicate that the urogenital anomalies can be attributed to a functional urethral obstruction which in turn is the result of prostatic hypoplasia. The histology of the abdominal wall is that of atrophy-ie, the degeneration of already formed muscle--and not of primitive muscle. This observation supports the theory that the abdominal muscle hypoplasia is a nonspecific lesion, resulting from fetal abdominal distension of various causes. Transient fetal ascites may be an important feature of the prune-belly syndrome.
ISSN:0031-4005
1098-4275
DOI:10.1542/peds.73.4.470