January 15, 2009

Prune belly syndrome

Prune belly syndrome: Partial or complete absence of the abdominal muscles so that the outlines of the intestines are visible through the thin, lax, protruding abdominal wall. The abdominal wall looks shrunken, shriveled and wrinkled like a prehistoric prune.

The prune belly syndrome probably occurs only in males. In addition to the abdominal muscle deficiency, they tend to have genital and urinary abnormalities including dilation (widening) of the urinary tract and cryptorchidism (failure for the testes to descend into the scrotum).

The condition was recognized during the 19th century. The renowned physician William Osler wrote: "In the summer of 1897 a case of remarkable distension of the abdomen was admitted to the wards, with greatly distended bladder, and on my return in September, Dr. Futcher, knowing that I would be interested in it, sent for the child."

There is also a form of abdominal muscle deficiency that is associated with narrowing (stenosis) of the pulmonary artery, mental retardation and deafness. It occurs in both boys and girls

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