Prune belly syndrome is a rare congenital disorder, belonging to the group of fetal lower urinary tract obstructions (LUTO), involving variable dilation of the lower. RELATO DE CASO. Síndrome de Prune Belly. Prune Belly syndrome. Roni Leonardo Teixeira, ACBC- SP; Alexandre Petreca; Patrícia Andréa Torres Garcia. Abstract. TEIXEIRA, Roni Leonardo; PETRECA, Alexandre and GARCIA, Patrícia Andréa Torres. Prune Belly syndrome. Rev. Col. Bras. Cir. [online].
|Published (Last):||25 July 2007|
|PDF File Size:||3.22 Mb|
|ePub File Size:||10.76 Mb|
|Price:||Free* [*Free Regsitration Required]|
Prune belly syndrome is a rare, genetic birth defect affecting about 1 in 40, births. Progressive urethral dilation, as described by Passerini-Glazel et al, is the preferred method of treatment. Prune belly syndrome often presents antenatally on routine ultrasound with oligohydramnios and a very large, distended bladder, mild to severe bilateral hydroureteronephrosis, fetal ascites and occasionally renal dysplasia see this term and a patent urachus.
Snyder et al describe a typical shift to the right, and some patients with prune belly syndrome can void normally. All may be due to a persistence of the embryonic wide mesentery, with absent fixation to the posterior abdominal wall.
Clinical description Prune belly syndrome often presents antenatally on routine ultrasound with oligohydramnios and a very large, distended bladder, mild to severe bilateral hydroureteronephrosis, fetal ascites and occasionally renal dysplasia see this term and a patent urachus.
Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. This abnormality in the prostatic urethra can be similar to a valve created by anterior urethral diverticulum. Pediatric urologists have observed that boys with prune belly syndrome can present with a spectrum of abnormalities.
Prune Belly Syndrome
Complications of peritoneal dialysis in children with Eagle-Barrett syndrome. The site of obstruction can vary from as high as the pelviureteral junction to as low as the prostatic membranous urethra.
Pseudo—prune belly syndrome, which is associated with prune belly syndrome uropathy, is characterized by normal abdominal wall examination findings and incomplete or absent cryptorchidism.
Congenital disorders of urinary system Congenital disorders of musculoskeletal system Rare syndromes Medical triads Syndromes affecting the kidneys.
Prune belly syndrome
Congenital Malformations of the Urinary Tract. We are determined to keep this website freely accessible. This theory has been replaced by a new notion that the obstruction may be caused by severe angulation at the prostatic and membranous urethral junction. A number sign is used with this entry because of evidence that prune belly syndrome PBS is caused by homozygous mutation in the CHRM3 gene on chromosome 1q In its rare complete form, ‘prune belly’ syndrome comprises megacystis massively enlarged bladder with disorganized detrusor muscle, cryptorchidism, and thin abdominal musculature with overlying lax skin summary by Weber et ed.
Share cases and questions with Physicians on Medscape consult. There was no earlier family history of prune belly syndrome. Autosomal dominant and X linked inheritances have been observed. A third possibility is that the muscle deficiency and the urinary abnormalities have a common cause that has not yet been discovered.
Likewise, Pagon et al. Incomplete emptying of the bladder leading to urinary retention and infection can occur as a result. Imperforate anus, anorectal agenesis, omphalocele, and gastroschisis are uncommon but have been reported.
In cases of siblings with Prune Prine Syndrome and mosaicism, a genetic etiology has been suggested but the majority of cases are of a sporadic nature. Chronic constipation has been attributed to a decrease in abdominal wall pressure, which is necessary to aid in evacuation. Those with less severe renal disease may survive infancy, but may have recurrent urinary tract infection or progressive renal insufficiency.
Abnormalities associated with oligohydramnios also are observed.
Greskovich and Nyberg gave a review in which they stated incorrectly that the term prune belly syndrome was coined by Prume. Congenital diaphragm and abdominal ds defects, abdominopelvic cavity Q A more drastic procedure is a surgical “remodeling” of the abdominal wall and urinary tract.
Urodynamics in the prune belly syndrome. Some prefer no surgical reconstruction of the abdominal wall, while others advocate surgical reconstruction. This condition was first described by Frolich The group that advocates surgical reconstruction is divided into 2 separate factions that support different techniques of abdominal wall reconstruction. The mesodermal arrest theory is supported by the histologic findings in the abdominal wall, the urinary tract, and the male genital system. The histopathology of the abdominal wall muscles demonstrates ds pattern of developmental arrest rather than one of atrophy.
Prune belly syndrome – Wikipedia
In addition, a decrease in nerve plexuses is reported, with irregularity in degeneration of nonmyelinated Schwann fibers. The literature contains little controversy regarding management of undescended testes in these children.
Prune belly syndrome affects 1 per 30, live births. Many individuals with prune belly syndrome have good physical and mental health, despite all the concerns.