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         Prune Belly Syndrome:     more detail
  1. Prune-belly syndrome: An entry from Thomson Gale's <i>Gale Encyclopedia of Genetic Disorders, 2nd ed.</i> by David, MD Greenberg, 2005

1. Prune Belly Syndrome Network
Devoted to health education, information and support for the person with the condition as well as Category Health Conditions and Diseases prune belly syndrome......Website for the prune belly syndrome Network. Last update Monday, March 17 2003Copyright © 19962003, prune belly syndrome Network All rights reserved.
http://www.prunebelly.org/
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Send comments or questions to webmaster@prunebelly.org Welcome! Here you will find a collection of information about Prune Belly Syndrome which is also known as Eagle-Barrett Syndrome. The Prune Belly Syndrome Network, Inc. provides support for those who have Prune Belly, their families, friends and the health care professionals who treat them. NEW!

2. Pediatric Database
Offers a definition of prune belly syndrome, the epidemiology, pathogenesis, pathology, clinical features, investigations and management.
http://www.icondata.com/health/pedbase/files/PRUNEBEL.HTM
  • Pediatric Database (PEDBASE)
  • Discipline: GU
  • Last Updated: 6/05/94
    PRUNE BELLY SYNDROME
    DEFINITION:
    A disorder characterized by the triad of abdominal musculature deficiency, anomalous development of the urinary tract, and bilateral cryptorchidism.
    EPIDEMIOLOGY:
    • incidence: 1/40,000 live births
    • age of onset:
      • newborn
    • risk factors:
      • sporadic
      PATHOGENESIS:
      1. Background
      • etiology is unknown but there are 2 hypotheses:
      1. Urinary Tract Obstruction
    • 2. Primary Mesodermal Developmental Defect
    • PATHOLOGY:
      1. Abdominal Musculature
      1. Myopathy
      • from mild hypoplasia to agenesis of the muscular layer
      • patchy and asymmetrical
      • lower and medial aspects most affected
      2. Appearance
      • initially wrinkled
      • later "pot belly" appearance
      2. Urinary Tract Anomalies
      1. Urethra and Prostate
      1. Urethra
      • often widely dilated
      • may be an area of abrupt narrowing distal to dilatation but urethral stenosis or atresia is rare with only 25% showing urethral obstruction at birth
      2. Prostate
    • 2. Bladder
      • markedly enlarged capacity with thickened wall
      • may show an "hourglass configuration" on voiding cystogram
  • 3. Prune Belly Syndrome Network
    Website for the prune belly syndrome Network. Last update Monday, March 17, 2003Copyright © 19962003, prune belly syndrome Network All rights reserved.
    http://www.prunebelly.org/prune2.html
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    About Prune Belly
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    About Prune Belly
    Definition:
    Also known as Eagle-Barrett syndrome, the Prune Belly syndrome consists of a triad of features:
  • Anterior abdominal wall: musculature is deficient or absent.
  • Urinary tract anomalies (mega-ureters, large bladder).
  • Bilateral cryptorchidism (undescended testicles).
    General:
    The incidence of prune belly syndrome is 1 in 40,000 live births; 95% of cases occur in males. In the past, it was suggested that the abdominal wall was deformed by pressure from a distended bladder due to bladder outlet obstruction in utero. Currently it is thought that prune belly syndrome is a multisystem disease complex which derives from a primary defect in mesodermal development. The major prognostic factor is the degree of dilation of the urinary tract; 20% of patients are stillborn, 30% die of renal failure or urosepsis within the first two years of life, and the remaining 50% have varying degrees of urinary pathology.
    Imaging:
    Renal abnormalities vary widely. There may be agenesis or hypoplasia, which is rare, or hydronephrosis. On IVP, 80% of patients have evident tortuous ureters with dilatation distally. Vesicoureteral reflux is common. The bladder is generally dilated, and the trabecula are thinned. A patent urachus may allow the bladder to decompress.
  • 4. Prune Belly Syndrome
    prune belly syndrome. Synonym EagleBarrett Syndrome
    http://www.familyvillage.wisc.edu/lib_prune-belly.html
    Prune Belly Syndrome
    Synonym: Eagle-Barrett Syndrome
    Who to Contact
    Where to Go to Chat with Others

    Learn More About It

    Web Sites
    ...
    Search AltaVista for "Prune Belly Syndrome"
    Who to Contact
    Prune Belly Syndrome Network, Inc.
    P.O. Box 45763
    Baton Rouge, LA 70895-4763
    USA
    Email: prunebelly@healthcon.com
    Website: http://www.prunebelly.org
    Where to Go to Chat with Others
    The Prune Belly Syndrome Support Network mailing list
    To join send an e-mail message to: prunebelly-list-request@cybern.com Leave Subject blank In the body of the message type: Subscribe Your Name
    Learn More About It
    Web Sites
    Back to [ O - P Family Village Home Library Coffee Shop ... Information Last Updated April 30, 2001by familyvillage@waisman.wisc.edu Document Source: http://www.familyvillage.wisc.edu/lib_prune-belly.html

    5. Prune Belly Syndrome
    prune belly syndrome. INTRODUCTION. Management of the child with prune belly syndrome(PBS) has posed a significant amount of trouble to the pediatric urologist.
    http://www.pedsurology.com/prune.htm
    Prune Belly Syndrome INTRODUCTION Management of the child with prune belly syndrome (PBS) has posed a significant amount of trouble to the pediatric urologist. There have been calls for conservative management of the urinary tract in these boys by some authors while others have advocated an aggressive approach to the management of these patients, 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. What we have been able to see is that prune belly syndrome can present with a spectrum of abnormalities ranging from the stillborn infant with severe urogenital and pulmonary problems to the child with little, if any, urological abnormalities requiring no therapy other than orchidopexies to correct the undescended testes. It appears that the decision to intervene in the management of the urinary tract of these children should be based on the clinical presentation and not solely on radiographic appearance. There is little controversy in the literature as to the present management of the undescended testes in these children. If controversy or confusion arises, it is to the optimal timing for the surgery for the orchidopexy as well the type of orchidopexy to be performed. Similarly, 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.

    6. Prune Belly Syndrome
    prune belly syndrome. National prune belly syndrome Association. 30 Salem Blvd.
    http://www.kumc.edu/gec/support/prunbely.html
    Prune Belly syndrome
    National Prune Belly Syndrome Association
    30 Salem Blvd. Naugatuck, CT 06770 Phone: (203) 729-6054 contact: Brian Beirne E-mail: bbeirne@healthcon.com
    Web site URL: http://healthcon.com
    GUMS (Guardians/Grandparents United for Moral Support)
    c/o Joyce Henderson 4512 Wilcox Pl. Jamesville, NY 13078 Phone: (315) 492-0090
    Also See:
    To locate a genetic counselor, or clinical geneticist in your area:

    Genetic Societies
    Clinical Resources ... Search
    Genetics Education Center
    Debra Collins, M.S. CGC
    , Genetic Counselor, dcollins@kumc.edu
    This site subscribes to the principles of the HONcode
    (Health on the Net, Code of Conduct for Medical and Health Web Sites)
    of the Health On the Net Foundation

    7. Prune Belly (Eagle-Barrett) Syndrome
    NORD prune belly syndrome - Offers a list of synonyms, a general discussion and further resources.
    http://rarediseases.about.com/library/weekly/aa032302a.htm
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    Prune Belly (Eagle-Barrett) Syndrome Set of distinctive physical problems Join the Discussion "I just wanted to point out to any parent or relative of a child with this disease that the outcome is often far better than conventional medical wisdom would have it. To ask questions or talk to others who are dealing with this rare and orphan disease, please visit www.prunebelly.org. There is a support forum."
    Related Resources Internet links on Prune Belly syndrome
    Elsewhere on the Web Prune Belly Syndrome Network Mary Kugler, MSN, RN,C Guide to Rare/Orphan Diseases What is it? Prune Belly syndrome, also known as Eagle-Barrett or triad syndrome, is a distinctive set of physical problems that a child is born with. These problems are:

    8. NORD - Prune Belly Syndrome
    Offers a list of synonyms, a general discussion and further resources.
    http://www.stepstn.com/cgi-win/nord.exe?proc=GetDocument&rectype=0&recnu

    9. Virtual Children's Hospital: Paediapaedia: Prune-Belly Syndrome (Eagle-Barrett S
    DEVELOPMENT OF THE URINARY SYSTEM prune belly syndrome Select Entry from OMIM This page is for computers without external internet access. List of OMIM search results The appellation 'prune belly syndrome' is descriptive because the intestinal pattern is evident through the thin, lax,
    http://www.vh.org/Providers/TeachingFiles/PAP/GUDiseases/Prune.html
    Paediapaedia: Genitourinary Diseases
    Prune-Belly Syndrome (Eagle-Barrett Syndrome)
    Michael P. D'Alessandro, M.D.
    Peer Review Status: Internally Peer Reviewed Clinical Presentation:
    Triad of deficient or absent abdominal musculature, undescended testicles, and dysplasia of the urinary tract. Physical exam shows a wrinkled anterior abdominal wall. Etiology/Pathophysiology:
    Etiology is unknown.Twenty percent are still born or die as neonates, and an additional 50% die in the first 2 years of life. Other associated anomalies include hip dislocations / club foot / limb deformities (40% ), cardiac defects(30% ), and malrotation (30% ). Pathology:
    Aplasia of the muscles of the lower medial abdominal wall. The kidneys have a decreased number of functioning nephrons with variable cystic dysplasia. The dilated ureters and bladder have a patchy absence of smooth muscle. Imaging Findings:
    The ureters and bladder are dilated. Urethral abnormalities that can be seen include posterior urethral dilation, diverticula, stenosis / atresia, and megaurethra. They can have umbilical-bladder attachment or a patent urachus. DDX:
    • Posterior urethral valves
    References:
    See References Chapter.

    10. Prune Belly Syndrome - Lucile Packard Children's Hospital
    Genitourinary Kidney Disorders. prune belly syndrome What is prunebelly syndrome? Prune What causes prune belly syndrome? Prune
    http://www.lpch.org/DiseaseHealthInfo/HealthLibrary/urology/pbs.html
    Urology
    Urologists
    Prune Belly Syndrome
    What is prune belly syndrome?
    Prune belly syndrome is also known as triad syndrome or Eagle-Barrett syndrome. It is characterized by a triad of abnormalities that include the following:
    • absence of abdominal muscles undescended testicles - a condition seen in newborns whereby one (or both) of the male testes has not passed down into the scrotal sac an abnormal, expanded bladder and problems in the upper urinary tract, which may include the bladder, ureters, and kidneys
    Because of the substantial involvement of the urinary tract, children with prune belly syndrome are usually unable to completely empty their bladders and have serious bladder, ureter, and kidney impairment. A child with prune belly syndrome may also have other birth defects. Most commonly, these defects involve the skeletal system, intestines, and heart. Girls may have defects in their external genitalia, as well. Some infants who have prune belly syndrome may be stillborn or die within a few months of birth.

    11. EMedicine - Prune Belly Syndrome : Article By Beverly P Wood, MD, MS
    prune belly syndrome The child with prune belly syndrome typically is male witha thin or lax abdominal wall (variable in severity) and a long and dilated
    http://www.emedicine.com/radio/topic575.htm
    (advertisement) Home Specialties CME PDA ... Patient Education Articles Images CME Patient Education Advanced Search Link to this site Back to: eMedicine Specialties Radiology Pediatrics
    Prune Belly Syndrome
    Last Updated: December 7, 2001 Rate this Article Email to a Colleague Synonyms and related keywords: congenital absence of abdominal musculature, deficiency of abdominal musculature, Eagle-Barrett syndrome, triad syndrome AUTHOR INFORMATION Section 1 of 10 Author Information Introduction Differentials X-ray ... Bibliography
    Author: Beverly P Wood, MD, MS , Professor, Departments of Radiology and Pediatrics, Division of Medical Education, Keck School of Medicine, University of Southern California Beverly P Wood, MD, MS, is a member of the following medical societies: American Academy of Pediatrics American Association for Women Radiologists American College of Radiology American Institute of Ultrasound in Medicine ... American Roentgen Ray Society , Association of University Radiologists, Radiological Society of North America , and Society for Pediatric Radiology Editor(s): Robert J Starshak, MD

    12. EMedicine - Prune Belly Syndrome : Article By Israel Franco, MD
    prune belly syndrome In 1839, Frölich first described PBS, and Osler gavethe condition its name. prune belly syndrome. Last Updated August 24, 2001,
    http://www.emedicine.com/med/topic3055.htm
    (advertisement) Home Specialties CME PDA ... Patient Education Articles Images CME Patient Education Advanced Search Link to this site Back to: eMedicine Specialties Medicine, Ob/Gyn, Psychiatry, and Surgery Urology
    Prune Belly Syndrome
    Last Updated: August 24, 2001 Rate this Article Email to a Colleague Synonyms and related keywords: PBS, Eagle-Barrett syndrome, triad syndrome AUTHOR INFORMATION Section 1 of 11 Author Information Introduction Indications Relevant Anatomy And Contraindications ... Bibliography
    Author: Israel Franco, MD , Assistant Professor, Departments of Urology and Pediatrics, Division of Pediatric Urology, New York Medical College Israel Franco, MD, is a member of the following medical societies: American Academy of Pediatrics American College of Surgeons American Urological Association Endourological Society ... Medical Society of the State of New York , and Society of Laparoendoscopic Surgeons Editor(s): Peter Langenstroer, MD , Assistant Professor, Department of Surgery, Division of Urology, Medical College of Wisconsin; Francisco Talavera, PharmD, PhD

    13. Prune Belly Syndrome
    prune belly syndrome (EagleBarrett Syndrome, Fröhlich Syndrome, Obrinsky Syndrome).prune belly syndrome; prune belly syndrome; Short description of cell lines.
    http://www.bdid.com/prune.htm

    HOME
    Prune Belly Syndrome (Eagle-Barrett Syndrome, Fröhlich Syndrome, Obrinsky Syndrome)

    HOME

    14. Birth Disorder Information Directory - P
    List of Sites. Proximal Femoral Focal Deficiency List of Sites. prune belly syndrome(Abdominal Muscle Absence/Aplasia/Deficiency/Defect Anomalad/Syndrome, Eagle
    http://www.bdid.com/defectp.htm

    HOME
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    P-4 Deletion Syndrome p47-PHOX, Deficiency of p67-PHOX, Deficiency of Pachydermopreiostosis (Hypertrophic Osteoarthropathy, Primary or Idiopathic) Pachyonychia Congenita Pagod Syndrome Pagon Bird Detter Syndrome (Anemia Sideroblastic Spinocerebellar Ataxia) Pagon Syndrome Pallister Syndrome Pallister Hall Syndrome (Hypothalamic Hamartoblastoma, Hypopituitarism, Imperperforate Anus, and Postaxial Polydactyly) Pallister Killian Syndrome

    15. UNSW Embryology-OMIM PRUNE BELLY SYNDROME
    Embryology Home Page. prune belly syndrome. Select Entry from OMIM. Alternativetitles; symbols. prune belly syndrome. TABLE OF CONTENTS.
    http://anatomy.med.unsw.edu.au/cbl/embryo/OMIMfind/kidney/OMIM-100100.htm
    UNSW Embryology
    DEVELOPMENT OF THE URINARY SYSTEM Embryology Home Page
    PRUNE BELLY SYNDROME
    Select Entry from OMIM
    Online Mendelian Inheritance in Man (Internet Link) This page is for computers without external internet access. Back to UNSW Embryology-Kidney Notes List of OMIM search results
    100100 ABDOMINAL MUSCLES, ABSENCE OF, WITH URINARY TRACT ABNORMALITY AND CRYPTORCHIDISM
    Alternative titles; symbols
    PRUNE BELLY SYNDROME
    TABLE OF CONTENTS
    • TEXT REFERENCES SEE ALSO CREATION DATE ... "16 MEDLINE Citations" Note: pressing the symbol will find the citations in MEDLINE whose text most closely matches the text of the preceding OMIM paragraph, using the Entrez MEDLINE neighboring function.
      TEXT
      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

    16. UNSW Embryology-OMIM PRUNE BELLY SYNDROME
    prune belly syndrome. Select Entry from OMIM. 264140 prune belly syndrome WITHPULMONIC STENOSIS, MENTAL RETARDATION, AND DEAFNESS. TABLE OF CONTENTS.
    http://anatomy.med.unsw.edu.au/cbl/embryo/OMIMfind/kidney/OMIM-264140.htm
    UNSW Embryology
    DEVELOPMENT OF THE URINARY SYSTEM Embryology Home Page
    PRUNE BELLY SYNDROME
    Select Entry from OMIM
    Online Mendelian Inheritance in Man (Internet Link) This page is for computers without external internet access. Back to UNSW Embryology-Kidney Notes List of OMIM search results
    264140 PRUNE BELLY SYNDROME WITH PULMONIC STENOSIS, MENTAL RETARDATION, AND DEAFNESS
    TABLE OF CONTENTS
    • TEXT REFERENCES CREATION DATE EDIT HISTORY ... "1 MEDLINE Citation"
      TEXT
      Lockhart et al. (1979) described a family in which 2 brothers and a sister had this combination. A fourth sib may have been affected; the mother reported that the male, who died 25 hours after birth, had 'a big bladder, big ureters, bad kidneys.' The hearing loss was sensorineural in nature. Because the involvement in the female was less marked than in the males, the possibility of X-linked inheritance was suggested. No information concerning abnormalities in the mother was provided.
      REFERENCES
      1. Lockhart, J. L.; Reeve, H. R.; Bredael, J. J.; Krueger, R. P. :

    17. OHSU Doernbecher - Genitourinary & Kidney Disorders
    prune belly syndrome, RELATED SERVICES. , Urology. What is prune belly syndrome?prune belly syndrome is also known as triad syndrome or EagleBarrett syndrome.
    http://www.ohsuhealth.com/dch/health/urology/prune.asp

    Anatomy of the Urinary System

    Overview of Genitourinary Disorders

    Male Conditions

    Ambiguous Genitalia
    ...
    Polycystic Kidney Disease (PKD)

    Prune Belly Syndrome
    Ureterocele and Ureteral Duplication

    Urinary Tract Infections (UTI)

    Vesicoureteral Reflux (VUR)

    Renal Failure
    ... Online Resources You have selected the following Health topic: - Adolescent Medicine - Burns - Cardiovascular Disorders - Care of the Terminally Ill Child - Children Having Surgery - Common Childhood Injuries and Poisoning - Craniofacial Anomalies - Dermatology - Diabetes - Eye Care - Genetics - Growth and Development - High-Risk Newborn - High-Risk Pregnancy - Immunizations - Infectious Diseases - Mental Health - Neurological Disorders - Normal Newborn - Oncology - Orthopaedics - The Pediatrician - Pregnancy and Childbirth - Respiratory Disorders - Transplantations - Well-Care Visits Prune Belly Syndrome RELATED SERVICES Urology What is prune belly syndrome? Prune belly syndrome is also known as triad syndrome or Eagle-Barrett syndrome. It is characterized by a triad of abnormalities that include the following:
    • absence of abdominal muscles
    • undescended testicles - a condition seen in newborns whereby one (or both) of the male testes has not passed down into the scrotal sac
    • an abnormal, expanded bladder and problems in the upper urinary tract, which may include the bladder, ureters, and kidneys

    18. MEDLINEplus Medical Encyclopedia: Prune Belly Syndrome
    prune belly syndrome. Causes, incidence, and risk factors Return totop The underlying causes of prune belly syndrome are unknown.
    http://www.nlm.nih.gov/medlineplus/ency/article/001269.htm
    Skip navigation
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    Prune belly syndrome
    Contents of this page:
    Illustrations
    Abdominal muscles Alternative names Return to top Eagle-Barrett syndrome Definition Return to top A disease characterized by three major findings (triad): deficient abdominal muscles (causing the skin of the abdomen to wrinkle like a prune), undescended testicles , and a dilated abnormal urinary tract. Causes, incidence, and risk factors Return to top The underlying causes of prune belly syndrome are unknown. There is blockage to the flow of urine from the kidney to the outside of the body during fetal life. For example, dilatation of the urinary tract may develop as a result of urethral obstruction in the womb. The incidence is approximately 1 in 30,000 to 40,000 births. Boys are almost solely affected.
    Expectant mothers carrying affected infants may develop varying degrees of oligohydramnios (insufficient amniotic fluid ) that predisposes the infant to lung problems (see Potter syndrome ). The

    19. Prune Belly Syndrome (PBS)
    View the Full Record Syndrome, prune belly syndrome (PBS). prune belly syndrome mayoccur with or without pulmonic stenosis, deafness, and mental retardation.
    http://www.nlm.nih.gov/mesh/jablonski/syndromes/syndrome542.html
    Multiple Congenital Anomaly/Mental Retardation (MCA/MR) Syndromes
    View the Full Record
    Syndrome 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 Summary A syndrome of hypoplasia or aplasia of the abdominal muscle, presenting a thin, loose, wrinkled and shriveled (prunelike) abdominal wall, with furrowlike umbilicus, and other anomalies, including persistent urachus, pigeon breast deformity, and a variety of gastrointestinal, cardiovascular, urogenital, and skeletal abnormalities. Prune belly syndrome may occur with or without pulmonic stenosis, deafness, and mental retardation. Distention of the bladder and urethra in utero is considered as a possible cause of abdominal overdistention. Major Features Nose: Recurrent epistaxis. Mouth and oral structures: Occasional cleft lip.

    20. Child Health Library - Urology - Prune Belly Syndrome
    Information Concerning prune belly syndrome from the Child Health Libraryof Children's Hopital of Pittsburgh. prune belly syndrome
    http://www.chp.edu/greystone/urology/pbs.php
    About Parents Providers Library ... Research Select Another Subject Adolescent Medicine Arthritis Burns Cardiovascular Disorders Craniofacial Anomalies Dermatology Eye Care Growth and Development High-Risk Newborn High-Risk Pregnancy Infectious Diseases Medical Genetics Mental Health Neurological Disorders Normal Newborn Oncology Orthopaedics Respiratory Disorders Surgery Terminally Ill, Care of Transplantation Urology About Pittsburgh Directions and Parking Departments and Services Neighborhood Locations ... Online Resources Prune Belly Syndrome What is prune belly syndrome?
    Prune belly syndrome is also known as triad syndrome or Eagle-Barrett syndrome.It ischaracterized by a triad of abnormalities that include the following:
    • absence of abdominal muscles
      undescended testicles - a condition seen in newborns whereby one (or both) of the male testes has not passed down into the scrotal sac

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