ABDOMINAL MUSCLES, ABSENCE OF, WITH URINARY TRACT ABNORMALITY AND CRYPTORCHIDISM
[for Professionals mainly]
Alternative titles; symbols
PRUNE BELLY SYNDROME
This condition was first described by Frolich (1839). The appellation "prune belly syndrome" is descriptive because the intestinal pattern is evident through the thin, lax, protruding abdominal wall in the infant ( Osler, 1901 ). ( Osler did not use the term "prune belly" ... "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".) The full syndrome probably occurs only in males ... Multiple cases (of the full syndrome) in families have rarely been reported, ... In British Columbia, Baird and MacDonald (1981) found a frequency of 1 in 29,231 live births ... similar to Poland syndrome (173800) in being rather consistently reproduced in many cases but having no clearly demonstrable mendelian basis ...

Prune Belly Syndrome
National Organization for Rare Disorders, Inc. (NORD), July 23, 2007
Abdominal Muscle Deficiency Syndrome
Congenital Absence of the Abdominal Muscles
Eagle - Barrett Syndrome
Obrinsky Syndrome

Prune Belly Syndrome
United States National Library of Medicine, December 12, 2006
prune belly syndrome ( PBS )
Synonyms
Eagle - Barrett syndrome
Frohlich syndrome
Obrinsky syndrome
abdominal muscle deficiency anomalad
abdominal muscle deficiency syndrome
abdominal musculature aplasia syndrome
absence of abdominal muscle syndrome
aplastic abdominal muscle syndrome
defective abdominal wall syndrome

Sindrome de Prunne-Belly
Patricia Cabanillas L, Rev. chil. pediatr. v.72 n.2 Santiago mar. 2001
[Spanish]
El sindrome de Prunne-Belly se caracteriza por una triada constituida por deficiencia congenita de la musculatura de la pared abdominal, anormalidades del tracto urinario y criptorquidia bilateral. A ella se agregan malformaciones musculoesqueleticas y viscerales. Se considera que el defecto de este sindrome reside en la placa intermedio/lateral mesodermica o prominencia genital. Se comunica un caso de sindrome de Prunne-Belly en un neonato que sobrevivio 6 dias. Clinicamente presento abdomen distendido con piel arrugada, megacisto, fistula vesicocutanea con emision de orina y pie equinovaro bilateral. El bebe fallecio por insuficiencia renal y respiratoria. En la necropsia se demostro la ausencia de musculatura en la pared abdominal, atresia de la union vesicouretral, megacisto, dilatacion quistica de los ureteres, rinones con cambios quisticos y displasicos, criptorquidia bilateral, hipoplasia pulmonar, ciego movil, pie equinovaro bilateral, pliegue simiano en la mano izquierda y fascies dismorfica con implantacion baja de las orejas. Llamaron la atencion los cambios coilocitoticos en el epitelio del glande. Fallecio por insuficiencia renal y respiratoria ... (Palabras clave: sindrome Prunne-Belly, obstruccion uretral.) ...

Prune Belly Syndrome
I.B.I.S. Birth Defects, 2002
[Ukrainian]
Two fact sheets for specialists in Ukrainian.
Synonyms ... Main diagnostic criteria ... Clinical features ... Association of symptoms ... Etiology ... Pathogenesis ... Treatment ...

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Last Updated: 2023/07/19

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