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Congenital absence of abdominal musculature leading to
urinary and respiratory problems. The major prognostic factor is the degree
of dilation of the urinary tract; 20% of patients are stillborn, 30%
die of renal failure or urosepsis within the first 2 years of life, and the
remaining 50% have varying degrees of urinary pathology requiring
numerous surgical procedures.
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Eagle-Barrett Syndrome; Abdominal Muscular Deficiency
Syndrome; Triad Syndrome; Fröhlich Syndrome.
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First described in 1839 by F. Fröhlich and the name “prune-belly syndrome” was given by W. Osler in 1901.
It was fully described by W. Obrinsky in 1949. G.S. Barrett and J.F. Eagle, Jr., American
urologists, popularized this medical condition. The pathogenesis is not clearly
understood. The triad (see Diagnosis) may arise simply from the effects of early
urethral obstruction and distended bladder, or, alternatively, from a basic defect of
the mesoderm. The full syndrome probably occurs only in males.
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1:40,000 live births; male:female ratio of 20:1 (95%
in males).
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Autosomal recessive inheritance with
sex-limited expression that partially mimics autosomal dominant X-linkage
has been suggested, but not proven.
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In 1903, Strumme proposed that the syndrome may be caused
by in utero obstruction of the bladder, stating that dilation of the urinary tract in
utero leads to secondary pressure atrophy of the abdominal wall and the subsequent
clinical findings. The prevailing theory is the theory of mesodermal arrest, which would
explain the involvement of the genitourinary tract, the testes, and the abdominal wall. A
noxious insult would have to occur between the sixth and tenth weeks of gestation. It has
been suggested that the embryogenesis of this disorder resides in the 3 weeks of
development which could also explain the prostatic hypoplasia and poor glandular
development. The mesodermal arrest theory is supported by the histologic findings in the
abdominal wall, the urinary tract, and the male genital system. Today, the etiology of
prune belly syndrome remains a mystery. The thinned abdominal wall has been attributed to
hydronephrosis and the distended urinary system to interfering with normal descent of the
testes. However, other patients with severe hydronephrosis may not show the same
abdominal wall disorder.
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Based on the triad of (a) deficient abdominal wall
musculature, (b) urinary tract dilatation, and (c) cryptorchidism.
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There is an absence of the muscles in the lower
central and medial part of the abdominal wall. The abdominal wall is lax and
thin, ...