link
Bookmarks
Prune-Belly Syndrome
Karen Y. Oh, MD
To access 4,300 diagnoses written by the world's leading experts in radiology, please log in or subscribe.Log inSubscribe
0
5
0
4

KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Synonyms

                • Eagle-Barrett syndrome
              • Definitions

                • Rare congenital disorder characterized by
                  • Dramatic collecting system dilatation
                  • Deficiency of abdominal musculature
                  • Cryptorchidism

              IMAGING

              • Ultrasonographic Findings

                • Imaging Recommendations

                  DIFFERENTIAL DIAGNOSIS

                    PATHOLOGY

                    • General Features

                      CLINICAL ISSUES

                      • Presentation

                        • Demographics

                          • Natural History & Prognosis

                            • Treatment

                              DIAGNOSTIC CHECKLIST

                              • Consider

                                • Image Interpretation Pearls

                                  Selected References

                                  1. Seidel NE et al: Clinical manifestations and management of prune-belly syndrome in a large contemporary pediatric population. Urology. 85(1):211-5, 2015
                                  2. Clayton DB et al: Lower urinary tract obstruction in the fetus and neonate. Clin Perinatol. 41(3):643-59, 2014
                                  3. Müller Brochut AC et al: Fetal megacystis: experience of a single tertiary center in Switzerland over 20 years. Fetal Diagn Ther. 36(3):215-22, 2014
                                  4. Rogers S et al: Prune belly with megalourethra. Ultrasound Q. 29(3):235-6, 2013
                                  5. Tonni G et al: Prune-belly syndrome: case series and review of the literature regarding early prenatal diagnosis, epidemiology, genetic factors, treatment, and prognosis. Fetal Pediatr Pathol. 31(1):13-24, 2013
                                  6. Hassett S et al: Prune belly syndrome. Pediatr Surg Int. 28(3):219-28, 2012
                                  7. Nemec SF et al: Male sexual development in utero: testicular descent on prenatal magnetic resonance imaging. Ultrasound Obstet Gynecol. 38(6):688-94, 2011
                                  8. Osborne NG et al: Fetal megacystis: differential diagnosis. J Ultrasound Med. 30(6):833-41, 2011
                                  9. Chen L et al: Two- and three-dimensional prenatal sonographic diagnosis of prune-belly syndrome. J Clin Ultrasound. 38(5):279-82, 2010
                                  10. Khatib N et al: Fetal bowel calcifications: a sign of anal atresia with rectourethral fistula. J Clin Ultrasound. 38(6):332-4, 2010
                                  11. Mann S et al: Fetal thoracic and bladder shunts. Semin Fetal Neonatal Med. 15(1):28-33, 2010
                                  12. Papantoniou N et al: Prenatal diagnosis of prune-belly syndrome at 13 weeks of gestation: case report and review of literature. J Matern Fetal Neonatal Med. Epub ahead of print, 2010
                                  13. Routh JC et al: Contemporary epidemiology and characterization of newborn males with prune belly syndrome. Urology. 76(1):44-8, 2010
                                  14. Comstock CH et al: Rupture of the fetal abdomen in prune belly syndrome. Ultrasound Obstet Gynecol. 33(4):487-8, 2009
                                  15. Herman TE et al: Prune belly syndrome. J Perinatol. 29(1):69-71, 2009
                                  16. Melek M et al: Megacystis-microcolon-intestinal hypoperistalsis syndrome: a case report. Gastroenterol Res Pract. 2009:282753, 2009
                                  17. Munch EM et al: Magnetic resonance imaging for prenatal diagnosis of multisystem disease: megacystis microcolon intestinal hypoperistalsis syndrome. Urology. 74(3):592-4, 2009
                                  18. Siebert JR et al: Familial recurrence of urethral stenosis/atresia. Birth Defects Res A Clin Mol Teratol. 85(8):715-9, 2009
                                  19. Szigeti R et al: Absent smooth muscle actin immunoreactivity of the small bowel muscularis propria circular layer in association with chromosome 15q11 deletion in Megacystis-Microcolon-Intestinal Hypoperistalsis Syndrome. Pediatr Dev Pathol. Epub ahead of print, 2009
                                  20. Wu S et al: Fetal lower urinary tract obstruction. Clin Perinatol. 36(2):377-90, x, 2009
                                  21. Rolle U et al: Bladder outlet obstruction causes fetal enterolithiasis in anorectal malformation with rectourinary fistula. J Pediatr Surg. 43(4):e11-3, 2008
                                  22. Lissauer D et al: Fetal lower urinary tract obstruction. Semin Fetal Neonatal Med. 12(6):464-70, 2007
                                  23. Woods AG et al: Prune belly syndrome. A focused physical assessment. Adv Neonatal Care. 7(3):132-43; quiz 144-5, 2007
                                  24. Garel C et al: Contribution of fetal magnetic resonance imaging and amniotic fluid digestive enzyme assays to the evaluation of gastrointestinal tract abnormalities. Ultrasound Obstet Gynecol. 28(3):282-91, 2006
                                  25. Pohl-Schickinger A et al: Echogenic foci in the dilated fetal colon may be associated with the presence of a rectourinary fistula. Ultrasound Obstet Gynecol. 28(3):341-4, 2006
                                  26. Robyr R et al: Correlation between ultrasound and anatomical findings in fetuses with lower urinary tract obstruction in the first half of pregnancy. Ultrasound Obstet Gynecol. 25(5):478-82, 2005
                                  27. Salihu HM et al: Prune belly syndrome and associated malformations. A 13-year experience from a developing country. West Indian Med J. 52(4):281-4, 2003
                                  28. Perez-Brayfield MR et al: In utero intervention in a patient with prune-belly syndrome and severe urethral hypoplasia. Urology. 57(6):1178, 2001
                                  29. Leeners B et al: Prune-belly syndrome: therapeutic options including in utero placement of a vesicoamniotic shunt. J Clin Ultrasound. 28(9):500-7, 2000
                                  30. Shimada K et al: Histology of the fetal prune belly syndrome with reference to the efficacy of prenatal decompression. Int J Urol. 7(5):161-6, 2000
                                  Related Anatomy
                                  Loading...
                                  Related Differential Diagnoses
                                  Loading...
                                  References
                                  Tables

                                  Tables

                                  KEY FACTS

                                  • Terminology

                                    • Imaging

                                      • Top Differential Diagnoses

                                        • Pathology

                                          • Clinical Issues

                                            • Diagnostic Checklist

                                              TERMINOLOGY

                                              • Synonyms

                                                • Eagle-Barrett syndrome
                                              • Definitions

                                                • Rare congenital disorder characterized by
                                                  • Dramatic collecting system dilatation
                                                  • Deficiency of abdominal musculature
                                                  • Cryptorchidism

                                              IMAGING

                                              • Ultrasonographic Findings

                                                • Imaging Recommendations

                                                  DIFFERENTIAL DIAGNOSIS

                                                    PATHOLOGY

                                                    • General Features

                                                      CLINICAL ISSUES

                                                      • Presentation

                                                        • Demographics

                                                          • Natural History & Prognosis

                                                            • Treatment

                                                              DIAGNOSTIC CHECKLIST

                                                              • Consider

                                                                • Image Interpretation Pearls

                                                                  Selected References

                                                                  1. Seidel NE et al: Clinical manifestations and management of prune-belly syndrome in a large contemporary pediatric population. Urology. 85(1):211-5, 2015
                                                                  2. Clayton DB et al: Lower urinary tract obstruction in the fetus and neonate. Clin Perinatol. 41(3):643-59, 2014
                                                                  3. Müller Brochut AC et al: Fetal megacystis: experience of a single tertiary center in Switzerland over 20 years. Fetal Diagn Ther. 36(3):215-22, 2014
                                                                  4. Rogers S et al: Prune belly with megalourethra. Ultrasound Q. 29(3):235-6, 2013
                                                                  5. Tonni G et al: Prune-belly syndrome: case series and review of the literature regarding early prenatal diagnosis, epidemiology, genetic factors, treatment, and prognosis. Fetal Pediatr Pathol. 31(1):13-24, 2013
                                                                  6. Hassett S et al: Prune belly syndrome. Pediatr Surg Int. 28(3):219-28, 2012
                                                                  7. Nemec SF et al: Male sexual development in utero: testicular descent on prenatal magnetic resonance imaging. Ultrasound Obstet Gynecol. 38(6):688-94, 2011
                                                                  8. Osborne NG et al: Fetal megacystis: differential diagnosis. J Ultrasound Med. 30(6):833-41, 2011
                                                                  9. Chen L et al: Two- and three-dimensional prenatal sonographic diagnosis of prune-belly syndrome. J Clin Ultrasound. 38(5):279-82, 2010
                                                                  10. Khatib N et al: Fetal bowel calcifications: a sign of anal atresia with rectourethral fistula. J Clin Ultrasound. 38(6):332-4, 2010
                                                                  11. Mann S et al: Fetal thoracic and bladder shunts. Semin Fetal Neonatal Med. 15(1):28-33, 2010
                                                                  12. Papantoniou N et al: Prenatal diagnosis of prune-belly syndrome at 13 weeks of gestation: case report and review of literature. J Matern Fetal Neonatal Med. Epub ahead of print, 2010
                                                                  13. Routh JC et al: Contemporary epidemiology and characterization of newborn males with prune belly syndrome. Urology. 76(1):44-8, 2010
                                                                  14. Comstock CH et al: Rupture of the fetal abdomen in prune belly syndrome. Ultrasound Obstet Gynecol. 33(4):487-8, 2009
                                                                  15. Herman TE et al: Prune belly syndrome. J Perinatol. 29(1):69-71, 2009
                                                                  16. Melek M et al: Megacystis-microcolon-intestinal hypoperistalsis syndrome: a case report. Gastroenterol Res Pract. 2009:282753, 2009
                                                                  17. Munch EM et al: Magnetic resonance imaging for prenatal diagnosis of multisystem disease: megacystis microcolon intestinal hypoperistalsis syndrome. Urology. 74(3):592-4, 2009
                                                                  18. Siebert JR et al: Familial recurrence of urethral stenosis/atresia. Birth Defects Res A Clin Mol Teratol. 85(8):715-9, 2009
                                                                  19. Szigeti R et al: Absent smooth muscle actin immunoreactivity of the small bowel muscularis propria circular layer in association with chromosome 15q11 deletion in Megacystis-Microcolon-Intestinal Hypoperistalsis Syndrome. Pediatr Dev Pathol. Epub ahead of print, 2009
                                                                  20. Wu S et al: Fetal lower urinary tract obstruction. Clin Perinatol. 36(2):377-90, x, 2009
                                                                  21. Rolle U et al: Bladder outlet obstruction causes fetal enterolithiasis in anorectal malformation with rectourinary fistula. J Pediatr Surg. 43(4):e11-3, 2008
                                                                  22. Lissauer D et al: Fetal lower urinary tract obstruction. Semin Fetal Neonatal Med. 12(6):464-70, 2007
                                                                  23. Woods AG et al: Prune belly syndrome. A focused physical assessment. Adv Neonatal Care. 7(3):132-43; quiz 144-5, 2007
                                                                  24. Garel C et al: Contribution of fetal magnetic resonance imaging and amniotic fluid digestive enzyme assays to the evaluation of gastrointestinal tract abnormalities. Ultrasound Obstet Gynecol. 28(3):282-91, 2006
                                                                  25. Pohl-Schickinger A et al: Echogenic foci in the dilated fetal colon may be associated with the presence of a rectourinary fistula. Ultrasound Obstet Gynecol. 28(3):341-4, 2006
                                                                  26. Robyr R et al: Correlation between ultrasound and anatomical findings in fetuses with lower urinary tract obstruction in the first half of pregnancy. Ultrasound Obstet Gynecol. 25(5):478-82, 2005
                                                                  27. Salihu HM et al: Prune belly syndrome and associated malformations. A 13-year experience from a developing country. West Indian Med J. 52(4):281-4, 2003
                                                                  28. Perez-Brayfield MR et al: In utero intervention in a patient with prune-belly syndrome and severe urethral hypoplasia. Urology. 57(6):1178, 2001
                                                                  29. Leeners B et al: Prune-belly syndrome: therapeutic options including in utero placement of a vesicoamniotic shunt. J Clin Ultrasound. 28(9):500-7, 2000
                                                                  30. Shimada K et al: Histology of the fetal prune belly syndrome with reference to the efficacy of prenatal decompression. Int J Urol. 7(5):161-6, 2000