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Prune Belly Syndrome

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A syndrome characterized by abdominal wall musculature deficiency, cryptorchism, and urinary tract abnormalities. The syndrome derives its name from its characteristic distended abdomen with wrinkled skin.

Also Known As:

Abdominal Muscle Deficiency Syndrome; Congenital Absence of the Abdominal Muscles; Eagle-Barrett Syndrome; Obrinsky Syndrome; Prune-Belly Syndrome; Belly Syndrome, Prune; Belly Syndromes, Prune; Eagle Barrett Syndrome; Prune Belly Syndromes; Prune-Belly Syndromes; Syndrome, Eagle-Barrett; Syndrome, Obrinsky; Syndrome, Prune Belly; Syndrome, Prune-Belly; Syndromes, Prune Belly; Syndromes, Prune-Belly

Networked: 107 relevant articles (3 outcomes, 4 trials/studies)

Relationship Network

Disease Context: Research Results

Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 668
- Congenital Abnormalities: 15467
  - Multiple Abnormalities: 163
    - Prune Belly Syndrome: 107
      - Urethral obstruction sequence: 5

Related Diseases

1.	Oligohydramnios
2.	Congenital Heart Defects (Congenital Heart Defect)
3.	Down Syndrome (Down's Syndrome)
4.	Neurogenic Urinary Bladder (Bladder, Neurogenic)
5.	Vesico-Ureteral Reflux (Vesicoureteral Reflux)

Experts

1.	Baker, Linda A: 2 articles (01/2012 - 12/2011)
2.	Giuliani, Stefano: 2 articles (11/2010 - 10/2004)
3.	Carr, Michael C: 2 articles (10/2005 - 09/2005)
4.	Legros, L: 1 article (11/2015)
5.	Wese, F-X: 1 article (11/2015)
6.	Feyaerts, A: 1 article (11/2015)
7.	Revencu, N: 1 article (11/2015)
8.	Nassogne, M-C: 1 article (11/2015)
9.	Sampaio, Francisco J B: 1 article (05/2015)
10.	Costa, Waldemar S: 1 article (05/2015)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Prune Belly Syndrome:

1.	MegaduodenumIBA 09/01/1997 - "We reviewed the medical records and prenatal imaging studies of 18 cases of marked in utero bladder distension in which a diagnosis of posterior urethral valves, the megacystis-megaureter association or the prune-belly syndrome was confirmed postnatally. " 06/01/2011 - "All male fetuses with megacystis originally had a diagnosis of prune belly syndrome. " 09/01/2001 - "The findings further suggest that abnormal prostatic development and the prune belly syndrome may arise from either anatomic obstruction of various types or functional obstruction from megacystis." 07/01/1993 - "The case further demonstrates that relief of the megacystis may not prevent prune belly syndrome, bladder dysfunction or renal damage. " 04/01/1985 - "Prune belly syndrome with ultrasound demonstration of reduction of megacystis in utero."
2.	Pentetic Acid (DTPA)FDA LinkGeneric 02/01/1986 - "An 18-year-old man with prune belly syndrome had negative renal outlines during a Tc-99m DTPA study. "
3.	Gracile bone dysplasiaIBA 01/01/2004 - "In this study, we report four unrelated congenital anomaly cases presenting trisomy 21, osteocraniostenosis syndrome, isolated congenital heart defect, and oligohydramnios sequence due to prune belly syndrome, in which fusion was observed. "
4.	Megacystis microcolon intestinal hypoperistalsis syndromeIBA 04/01/2012 - "We present a case of a rare association of a male neonate with Megacystis Microcolon Intestinal Hypoperistalsis Syndrome who in addition had the classical triad of Prune Belly Syndrome and thus suggest a possibility of different spectrums with a common pathogenesis. " 04/01/2012 - "Megacystis-microcolon-intestinal hypoperistalsis syndrome associated with prune belly syndrome: a case report." 06/01/2011 - "In conclusion, in fetuses with megacystic bladders, it is possible to distinguish between cases with prune belly syndrome, posterior urethral valves, urethral atresia, and megacystis-microcolon-intestinal hypoperistalsis syndrome by a detailed anatomic survey using 2- and 3-/4-dimensioinal sonographic techniques." 09/01/2001 - "Though less frequent in incidence, more complex sequences of anomalies such as Prune Belly Syndrome or Megacystis-Microcolon-Intestinal-Hypoperistalsis-Syndrome (MMIHS) can also be detected in early gestational age. " 01/01/1983 - "Megacystis-microcolon-intestinal hypoperistalsis syndrome in a newborn girl whose brother had prune belly syndrome: common pathogenesis?"
5.	VACTERL associationIBA 12/01/2011 - "Megalourethra is a rare spectrum of urologic malformations of penile corporal structures frequently associated with multiple congenital anomalies, such as prune belly syndrome or vertebral, anorectal, cardiac, trachea-esophageal, renal, and limb (VACTERL association) defects. " 08/01/2004 - "VACTERL association with Prune-Belly syndrome." 09/01/1994 - "As far as we know, the combination of the Prune-Belly-syndrome and the VACTERL-association has not been reported in literature until now." 09/01/1994 - "[Concordance of prune belly syndrome and VACTERL association]."
6.	Dihydrotachysterol (AT 10)IBA 11/01/1998 - "We present a case of prune belly syndrome in a 12-week fetus whose previous anomaly scan at 10 weeks had been normal. " 09/01/1997 - "Graft survival in the prune-belly syndrome and control groups was 50 and 72% at 5 years, and 50 and 47% at 10 years, respectively (not statistically significant). " 09/01/1997 - "No statistically significant increase in serum creatinine was noted at 10 years in children with the prune-belly syndrome. "
7.	CreatinineIBA 01/01/2000 - "One of the patients with prune belly syndrome was 18 months old at last follow-up, with hydrocephalus and a creatinine level of 0.2 mg/dL. " 09/01/1997 - "No statistically significant increase in serum creatinine was noted at 10 years in children with the prune-belly syndrome. "
8.	CosmeticsIBA 01/01/2012 - "Total abdominal wall reconstruction using the double-breasted technique in patients with prune belly syndrome is a safe and durable procedure that achieves excellent cosmetic results. " 09/01/1998 - "Various techniques of reconstruction have been developed to improve the cosmetic and functional status of the abdominal wall in the prune-belly syndrome. "
9.	Anorectal atresiaIBA 10/01/2004 - "Prune-belly syndrome with anorectal malformation." 04/01/2005 - "The diagnoses were: neurogenic bladder (n=22), bladder or cloacal exstrophy (n=20), anorectal malformation (n=6), persistent cloaca (n=3), Hirschsprung's disease (n=2), posterior urethral valves (n=1) and prune belly syndrome (n=1). "
10.	Sacral agenesisIBA 08/01/1986 - "Since 1976, 23 children with bladder or cloacal exstrophy, meningomyelocele, sacral agenesis, the prune belly syndrome and noncompliant bladders associated with urethral valves or prior diversion underwent augmentation cystoplasty. " 10/01/2005 - "Etiologies of bladder dysfunction included posterior urethral valves in 1 case, The Hinman-Allen syndrome in 4, myelomeningocele in 2, neuroblastoma in 1, prune-belly syndrome in 1, transverse myelitis in 1 and sacral agenesis in 1. The amount of post-obstructive diuresis was calculated during urodynamics as the volume drained minus volume instilled divided by weight per hour. "

Therapies and Procedures

1.	Orchiopexy 04/01/1995 - "This case demonstrates that therapeutic laparoscopic procedures are possible in the prune belly syndrome and that laparoscopic orchidopexy may have promise in older affected children or in those requiring no other concomitant surgery." 07/01/2011 - "Laparoscopic orchidopexy in boys with prune belly syndrome--outcome and technical considerations." 04/01/2004 - "The majority of boys with prune-belly syndrome had a satisfactory outcome after orchiopexy with division of the gonadal vessels with testicular function sufficient to induce puberty and maintain satisfactory sexual function in adult life." 04/01/2004 - "We reviewed a series of adults with prune-belly syndrome to assess the morphological and functional outcome of orchiopexy in childhood with specific reference to the spontaneous onset of puberty, hormonal profiles and sexual function. " 04/01/2004 - "Intra-abdominal testes in boys with prune-belly syndrome have been conventionally managed by 1 or 2-stage orchiopexy with division of the gonadal vessels. "
2.	Kidney Transplantation 09/01/1997 - "We assessed the long-term efficacy of renal transplantation in children with the prune-belly syndrome. " 04/01/2007 - "To present the long-term outcome of deceased-donor kidney transplantation in prune belly syndrome (PBS). " 04/01/2007 - "Deceased-donor kidney transplantation in prune belly syndrome." 10/01/2004 - "We assess the effect of the prune-belly syndrome (PBS) on renal transplantation outcome. " 10/01/2004 - "Renal transplantation in prune-belly syndrome."
3.	Decompression 05/01/2000 - "Histology of the fetal prune belly syndrome with reference to the efficacy of prenatal decompression." 06/01/2003 - "Even though most children with Prune Belly Syndrome respond to lower urinary tract drainage, a cutaneous pyelostomy may be necessary when the ureters are tortuous and do not drain adequately following bladder decompression." 07/01/1993 - "Development of the Eagle-Barrett (prune belly) syndrome and a thickened, poorly functional bladder wall after early second-trimester decompression of fetal megacystis."
4.	Intermittent Urethral Catheterization 08/01/1992 - "Analysis of multiple urodynamic studies during long-term followup periods ranging from 6 months to 19 years, with particular regard to any reconstructive procedures performed, revealed that voiding in the prune belly syndrome is variable, with 44% of the patients achieving spontaneous voiding and 56% requiring clean intermittent catheterization. "
5.	Transplants (Transplant) 09/01/2010 - "A malconfigured urinary bladder on post renal transplant scintigraphy in a patient with Prune Belly syndrome." 09/01/2005 - "Posterior urethral valves were confirmed in 7 males, urethral atresia in 4, and prune belly syndrome in 7. Eight children had acceptable renal function, 4 had mild insufficiency, and 6 required dialysis and eventual renal transplant. " 12/01/1989 - "There was no statistically significant difference in patient deaths (1 versus 3), graft survival (75 versus 69%) or graft function between patients with the prune belly syndrome and controls. " 09/01/2003 - "Torsion of a renal transplant around its pedicle is a rare complication, which has been described in renal transplant children with Prune Belly syndrome, but also exceptionally in adults undergoing kidney-pancreas transplantation. " 02/01/1995 - "We report a 26-month-old child diagnosed with prune-belly syndrome and end-stage renal disease who received intraperitoneal implantation of an adult cadaveric renal graft which functioned very well for approximately 6 weeks. "