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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.

Prune belly syndrome (Eagle-Barrett syndrome)

The absence of the abdominal muscles in a newborn with Eagle-Barrett syndrome results in the visibility of the contours of the bowel loops, explaining the synonym prune belly syndrome.

Prune belly syndrome (Eagle-Barrett syndrome)

Eagle-Barrett syndrome in a 9-year-old boy.

Eagle-Barrett Syndrome; Abdominal Muscular Deficiency Syndrome; Triad Syndrome; Fröhlich Syndrome.

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.

1:40,000 live births; male:female ratio of 20:1 (95% in males).

Autosomal recessive inheritance with sex-limited expression that partially mimics autosomal dominant X-linkage has been suggested, but not proven.

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.

Based on the triad of (a) deficient abdominal wall musculature, (b) urinary tract dilatation, and (c) cryptorchidism.

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, ...

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