eMedicine Specialties > Radiology > Pediatrics

Prune Belly Syndrome: Follow-up

Author: Beverly P Wood, MD, PhD, Professor Emerita, Departments of Radiology and Pediatrics, Division of Medical Education, Keck School of Medicine, University of Southern California; Professor of Clinical Radiology, Loma Linda University School of Medicine
Contributor Information and Disclosures

Updated: Oct 2, 2009

Intervention

Nonoperative management: Because there is no clear evidence indicating that reconstructive surgery improves function for patients with prune belly syndrome, some patients do well without reconstruction, provided freedom from infection is maintained.

Surgery is indicated to repair cryptorchidism.

For urinary drainage, vesicostomy is used in patients with severe reflux who have an inability to empty the bladder. Reconstruction of the urinary tract with tapering and reimplantation of the ureters is undertaken in the more severely affected infants or as a staged procedure in children; it is beneficial in decreasing the urinary stasis that occurs in severely affected patients.

Despite extensive attempts to drain urine adequately, children with prune belly syndrome may develop renal failure.6,14,15,16

 


More on Prune Belly Syndrome

Overview: Prune Belly Syndrome
Imaging: Prune Belly Syndrome
Follow-up: Prune Belly Syndrome
Multimedia: Prune Belly Syndrome
References
Further Reading
[ CLOSE WINDOW ]

References

  1. Eagle JF, Barrett GS. Congenital deficiency of abdominal musculature with associated genitourinary abnormalities: A syndrome. Report of 9 cases. Pediatrics. Nov 1950;6(5):721-36. [Medline].

  2. Wakhlu AK, Wakhlu A, Tandon RK, Kureel SN. Congenital megalourethra. J Pediatr Surg. Mar 1996;31(3):441-3. [Medline].

  3. Woods AG, Brandon DH. Prune belly syndrome. A focused physical assessment. Adv Neonatal Care. Jun 2007;7(3):132-43; quiz 144-5. [Medline].

  4. Siebert JR, Walker MP. Familial recurrence of urethral stenosis/atresia. Birth Defects Res A Clin Mol Teratol. Aug 2009;85(8):715-9. [Medline].

  5. Laborie LB, Mackay DJ, Temple IK, Molven A, Søvik O, Njølstad PR. DNA hypomethylation, transient neonatal diabetes, and prune belly sequence in one of two identical twins. Eur J Pediatr. Jun 13 2009;[Medline].

  6. Leeners B, Sauer I, Schefels J, et al. Prune-belly syndrome: therapeutic options including in utero placement of a vesicoamniotic shunt. J Clin Ultrasound. Nov-Dec 2000;28(9):500-7. [Medline].

  7. Wheatley JM, Stephens FD, Hutson JM. Prune-belly syndrome: ongoing controversies regarding pathogenesis and management. Semin Pediatr Surg. May 1996;5(2):95-106. [Medline].

  8. Woolf AS, Thiruchelvam N. Congenital obstructive uropathy: its origin and contribution to end-stage renal disease in children. Adv Ren Replace Ther. Jul 2001;8(3):157-63. [Medline].

  9. Kupferman JC, Druschel CM, Kupchik GS. Increased prevalence of renal and urinary tract anomalies in children with Down Syndrome. Pediatrics. Sep 14 2009;[Medline].

  10. Vemulakonda VM, Kopp RP, Sorensen MD, Grady RW. Recurrent nephrogenic adenoma in a 10-year-old boy with prune belly syndrome : a case presentation. Pediatr Surg Int. May 2008;24(5):605-7. [Medline].

  11. Cromie WJ. Implications of antenatal ultrasound screening in the incidence of major genitourinary malformations. Semin Pediatr Surg. Nov 2001;10(4):204-11. [Medline].

  12. Kaefer M, Peters CA, Retik AB, Benacerraf BB. Increased renal echogenicity: a sonographic sign for differentiating between obstructive and nonobstructive etiologies of in utero bladder distension. J Urol. Sep 1997;158(3 Pt 2):1026-9. [Medline].

  13. Weiner Z, Goldstein I, Bombard A, Applewhite L, Itzkovits-Eldor J. Screening for structural fetal anomalies during the nuchal translucency ultrasound examination. Am J Obstet Gynecol. Aug 2007;197(2):181.e1-5. [Medline].

  14. Noh PH, Cooper CS, Winkler AC, et al. Prognostic factors for long-term renal function in boys with the prune-belly syndrome. J Urol. Oct 1999;162(4):1399-401. [Medline].

  15. Saxena AK, Brinkmann OA. Unique features of prune belly syndrome in laparoscopic surgery. J Am Coll Surg. Aug 2007;205(2):217-21. [Medline].

  16. Levine E, Taub PJ, Franco I. Laparoscopic-assisted abdominal wall reconstruction in prune-belly syndrome. Ann Plast Surg. Feb 2007;58(2):162-5. [Medline].

[ CLOSE WINDOW ]

Further Reading

Prune Belly Syndrome (Urology)

[ CLOSE WINDOW ]

Keywords

prune belly syndrome, congenital absence of abdominal musculature, deficiency of abdominal musculature, Eagle-Barrett syndrome, triad syndrome

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Beverly P Wood, MD, PhD, Professor Emerita, Departments of Radiology and Pediatrics, Division of Medical Education, Keck School of Medicine, University of Southern California; Professor of Clinical Radiology, Loma Linda University School of Medicine
Beverly P Wood, MD, PhD 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 Medical Association, American Roentgen Ray Society, Association of University Radiologists, Radiological Society of North America, and Society for Pediatric Radiology
Disclosure: Nothing to disclose.

Medical Editor

Robert J Starshak, MD, Medical Director, Assistant Clinical Professor, Department of Radiology, Medical College of Wisconsin, Falls Medical Group
Disclosure: Nothing to disclose.

Pharmacy Editor

Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.

CME Editor

Robert M Krasny, MD, Resolution Imaging Medical Corporation
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.

Chief Editor

John Karani, MBBS, FRCR, Clinical Director of Radiology and Consultant Radiologist, Department of Radiology, King's College Hospital, London
John Karani, MBBS, FRCR is a member of the following medical societies: British Institute of Radiology, British Society of Interventional Radiology, Cardiovascular and Interventional Radiological Society of Europe, European Society of Gastrointestinal and Abdominal Radiology, European Society of Radiology, Radiological Society of North America, and Royal College of Radiologists
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.