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 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.
D000009:Abdominal Muscles / Q000002:abnormalities; D000015:Abnormalities, Multiple / Q000473:pathology; D001201:Ascites / Q000150:complications; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D007668:Kidney / Q000002:abnormalities; D008297:Male; D011247:Pregnancy; D011467:Prostate / Q000002:abnormalities*; D011535:Prune Belly Syndrome / Q000209:etiology*; D014513:Ureter / Q000002:abnormalities; D014521:Urethra / Q000002:abnormalities; D014524:Urethral Obstruction / Q000209:etiology*; D001743:Urinary Bladder / Q000002:abnormalities