Prune belly syndrome

Prune belly syndrome
Other namesAbdominal muscle deficiency syndrome, congenital absence of the abdominal muscles, Eagle-Barrett syndrome,[1] Obrinsky syndrome,[2] Fröhlich syndrome,[3] triad syndrome
Prune belly syndrome in an Egyptian child with Down syndrome.
SpecialtyMedical genetics Edit this on Wikidata

Prune belly syndrome is a rare, genetic birth defect affecting about 1 in 40,000 births.[4] About 97% of those affected are male. Prune belly syndrome is a congenital disorder of the urinary system, characterized by a triad of symptoms. The syndrome is named for the mass of wrinkled skin that is often (but not always) present on the abdomen of those with the disorder.

  1. ^ Eagle JF, Barrett GS (1950). "Congenital deficiency of abdominal musculature with associated genitourinary abnormalities: A syndrome. Report of 9 cases". Pediatrics. 6 (5): 721–36. doi:10.1542/peds.6.5.721. PMID 14797335. S2CID 26235702.
  2. ^ Obrinsky W (1949). "Agenesis of abdominal muscles with associated malformation of the genitourinary tract; a clinical syndrome". Am J Dis Child. 77 (3): 362–73. doi:10.1001/archpedi.1949.02030040372008. PMID 18116668.
  3. ^ Frolich, F. Der Mangel der Muskeln, insbesondere der Seitenbauchmuskeln. Dissertation: Wurzburg 1839.
  4. ^ Baird PA, MacDonald EC (1981). "An epidemiologic study of congenital malformations of the anterior abdominal wall in more than half a million consecutive live births". Am. J. Hum. Genet. 33 (3): 470–8. PMC 1685049. PMID 6454342.