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International Birth Defects Information Systems
Abdominal Muscles - "Prune Belly" Syndrome

International Birth Defects Information Systems



Abdominal Muscles - "Prune Belly" Syndrome


Topics: | Abdominal Muscles - "Prune Belly" Syndrome | Abdominal Muscle Deficiency A. | Abdominal Muscle Deficiency S. | Abdominal Musculature Aplasia S. | Absence of Abdominal Muscle S. | Aplastic Abdominal Muscle S. | Congenital Absence of the Abdominal Muscles | Defective Abdominal Wall S. | Eagle - Barrett S. | Fröhlich S. | Obrinsky S. |

Service Related: | Support Groups | Professional Associations | Key Information Sources |

Languages: | English | Spanish | Ukrainian |

ABDOMINAL MUSCLES, ABSENCE OF, WITH URINARY TRACT ABNORMALITY AND CRYPTORCHIDISM
Victor A. McKusick, OMIM, Johns Hopkins University, September 6, 2007
Visitor Comments [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
Pedsurology.com
Visitor Comments [for Professionals mainly] [Support Groups] [Illustrated]
Management ... has posed a significant amount of trouble to the pediatric urologist ... calls for conservative management of the urinary tract in these boys by some authors while others have advocated an aggressive approach ... operating on them at ten days of life. At the present time, no definitive timing for therapy has been substantiated one way or the other ... The management of the abdominal wall abnormalities has several camps with some going for no surgical reconstruction of the abdominal wall while others advocate surgical reconstruction and this group being divided amongst two separate factions advocating different techniques of abdominal wall reconstruction ... Obstruction and/or upper urinary tract dilatation is not a rare occurrence in these children ...
The management of the undescended testis in the patient with prune belly syndrome is some thing that should be left to the experienced pediatric urologist ...
Abdominal wall reconstruction is performed in most boys to improve respiratory function as well as for cosmetic reasons ...
Obstruction at the uretero - pelvic junction has been observed in patients with prune belly syndrome ... Infravesical obstruction or obstruction at the prostatic urethra was originally thought to be due to a Type I valve. This theory has been replaced by a new thinking in which the obstruction is felt to be caused by severe angulation at the prostatic and membranous urethral junction. This may be possibly due to a lack of striated muscle in the membranous urethra or the urogenital diaphragm, or a ring of obstructive tissue acting as a flap valve due to hypoplasia of the prostate, creating a ballooning of the prostatic urethra. In these patient's, studies have shown also that there is a reduction in the smooth muscle in the prostate with an increase in the connective tissue content which has been thought to lead to a functional obstruction ... A patent urachus can be frequently found in patients with urethral obstruction ... Posterior urethral valves are occasionally seen in patients with prune belly syndrome ...
The prune belly syndrome is characterized by elongated, dilated and tortuous megaureters ... ureteral abnormalities ...
Megacystis in prune belly patients is typically described. The bladder is routinely enlarged, although trabeculations are rarely present and muscular hypertrohyy is inconsistent ...
Pre operative view prior to Abdominal plication!
Post operative results after Abdominal wall plication.

Prune Belly Syndrome
National Organization for Rare Disorders, Inc. (NORD), November 5, 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
Fröhlich 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 Prune Belly
Dr. Manuel Pantoja Ludueña et al., Sociedad Boliviana de Pediatría
Visitor Comments [Spanish]
Caso Clinico ... Recién nacido a termino, sexo masculino y de un día de vida. Fue referido al Hospital del Niño «Dr. Ovidio Aliaga Uría» por presentar un abdomen prominente y el antecedente de oligohidroamnios durante el embarazo. En el examen físico destacó: abdomen flácido con ausencia parcial de músculos abdominales correspondiente a los rectos anteriores, piel redundante y arrugada. A través del ombligo se observó la salida de orina. En la región genital se encontró ausencia de ambos testículos en las bolsas escrotales. El diagnostico que se planteó a su ingreso fue un síndrome de Prune Belly con persistencia del uraco. Se solicitó una ecografía renal donde se observó ambos riñones aumentados de tamaño con una ectasia pieloureteral y vejiga aumentada de tamaño. El laboratorio confirmo el compromiso de la función renal y una infección urinaria. Se realizó interconsulta con urología quienes confirmaron el diagnóstico. El paciente falleció a los tres días de vida por las complicaciones de una urosepsis ...

Síndrome de Prunne-Belly
Patricia Cabanillas L, Rev. chil. pediatr. v.72 n.2 Santiago mar. 2001
Visitor Comments [Spanish]
El síndrome de Prunne-Belly se caracteriza por una tríada constituida por deficiencia congénita de la musculatura de la pared abdominal, anormalidades del tracto urinario y criptorquidia bilateral. A ella se agregan malformaciones musculoesqueléticas y viscerales. Se considera que el defecto de este síndrome reside en la placa intermedio/lateral mesodérmica o prominencia genital. Se comunica un caso de síndrome de Prunne-Belly en un neonato que sobrevivió 6 días. Clínicamente presentó abdomen distendido con piel arrugada, megacisto, fístula vesicocutánea con emisión de orina y pie equinovaro bilateral. El bebé falleció por insuficiencia renal y respiratoria. En la necropsia se demostró la ausencia de musculatura en la pared abdominal, atresia de la unión vesicouretral, megacisto, dilatación quística de los uréteres, riñones con cambios quísticos y displásicos, criptorquidia bilateral, hipoplasia pulmonar, ciego móvil, pie equinovaro bilateral, pliegue simiano en la mano izquierda y fascies dismórfica con implantación baja de las orejas. Llamaron la atención los cambios coilocitóticos en el epitelio del glande. Falleció por insuficiencia renal y respiratoria ... (Palabras clave: síndrome Prunne-Belly, obstrucción uretral.) ...

Los Trastornos Genitourinarios y de Los Riñones
University of Virginia Health System, March 9, 2004
Visitor Comments [Spanish]
¿Qué es el síndrome del abdomen en ciruela pasa? ... El síndrome del abdomen en ciruela pasa también se denomina síndrome de la tríada o síndrome de Eagle-Barrett. Se caracteriza por una tríada de anomalías que incluyen lo siguiente:
  • ausencia de los músculos abdominales

  • testículos no descendidos - trastorno que se observa en los recién nacidos donde uno o ambos testículos no descendieron a la bolsa testicular

  • vejiga dilatada anormal y problemas en el tracto urinario superior, que pueden incluir la vejiga, los uréteres y los riñones

Prune Belly Syndrome
I.B.I.S. Birth Defects, 2002
Visitor Comments [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: 2008/3/10

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American Medical Association


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