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Prune-Belly Syndrome
Priyanka Jha, MBBS; Paula J. Woodward, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Synonyms

              • Eagle-Barrett syndrome
            • Definitions

              • 1 in group of disorders causing lower urinary tract obstruction (LUTO)
                • General features of LUTO: Hydronephrosis, hydroureter, bladder distention
              • Prune-belly syndrome (PBS) characterized by 3 principle components
                • Dramatic collecting system dilatation
                • Deficiency or laxity 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. Apostel HJCL et al: Respiratory support in the absence of abdominal muscles: a case study of ventilatory management in prune belly syndrome. Paediatr Respir Rev. 37:44-7, 2021
                                2. Marchal S et al: Long-term outcome of renal transplantation in patients with congenital lower urinary tract malformations: a multicenter study. Transplantation. 104(1):165-71, 2020
                                3. Arlen AM et al: Prune belly syndrome: current perspectives. Pediatric Health Med Ther. 10:75-81, 2019
                                4. Wong DG et al: Phenotypic severity scoring system and categorisation for prune belly syndrome: application to a pilot cohort of 50 living patients. BJU Int. 123(1):130-9, 2019
                                5. Moscardi PRM et al: Prenatal renal parenchymal area as a predictor of early end-stage renal disease in children with vesicoamniotic shunting for lower urinary tract obstruction. J Pediatr Urol. 14(4):320.e1-6, 2018
                                6. White JT et al: Vesicoamniotic shunting improves outcomes in a subset of prune belly syndrome patients at a single tertiary center. Front Pediatr. 6:180, 2018
                                7. Yalcinkaya F et al: Outcomes of renal replacement therapy in boys with prune belly syndrome: findings from the ESPN/ERA-EDTA Registry. Pediatr Nephrol. 33(1):117-24, 2018
                                8. Kapoor S: "Pseudo-prune belly syndrome": an under-reported phenotypic variant of prune belly syndrome. J Pediatr Urol. 13(6):656-7, 2017
                                9. Pellegrino M et al: Prenatal detection of megacystis: not always an adverse prognostic factor. Experience in 25 consecutive cases in a tertiary referral center, with complete neonatal outcome and follow-up. J Pediatr Urol. 13(5):486.e1-10, 2017
                                10. Taghavi K et al: Fetal megacystis: a systematic review. J Pediatr Urol. 13(1):7-15, 2017
                                11. Wijesinghe US et al: Further evidence of the etiology of prune belly syndrome provided by a transient massive intraabdominal cyst in a female. J Pediatr Surg. 51(8):1390-3, 2016
                                12. Seidel NE et al: Clinical manifestations and management of prune-belly syndrome in a large contemporary pediatric population. Urology. 85(1):211-5, 2015
                                13. Clayton DB et al: Lower urinary tract obstruction in the fetus and neonate. Clin Perinatol. 41(3):643-59, 2014
                                14. 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
                                15. Rogers S et al: Prune belly with megalourethra. Ultrasound Q. 29(3):235-6, 2013
                                16. 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
                                17. Hassett S et al: Prune belly syndrome. Pediatr Surg Int. 28(3):219-28, 2012
                                18. Nemec SF et al: Male sexual development in utero: testicular descent on prenatal magnetic resonance imaging. Ultrasound Obstet Gynecol. 38(6):688-94, 2011
                                19. Osborne NG et al: Fetal megacystis: differential diagnosis. J Ultrasound Med. 30(6):833-41, 2011
                                20. Chen L et al: Two- and three-dimensional prenatal sonographic diagnosis of prune-belly syndrome. J Clin Ultrasound. 38(5):279-82, 2010
                                21. 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. 23(10):1263-7, 2010
                                22. Routh JC et al: Contemporary epidemiology and characterization of newborn males with prune belly syndrome. Urology. 76(1):44-8, 2010
                                23. Comstock CH et al: Rupture of the fetal abdomen in prune belly syndrome. Ultrasound Obstet Gynecol. 33(4):487-8, 2009
                                24. Herman TE et al: Prune belly syndrome. J Perinatol. 29(1):69-71, 2009
                                25. Munch EM et al: Magnetic resonance imaging for prenatal diagnosis of multisystem disease: megacystis microcolon intestinal hypoperistalsis syndrome. Urology. 74(3):592-4, 2009
                                26. 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. 13(4):322-5, 2009
                                27. Wu S et al: Fetal lower urinary tract obstruction. Clin Perinatol. 36(2):377-90, x, 2009
                                28. Lissauer D et al: Fetal lower urinary tract obstruction. Semin Fetal Neonatal Med. 12(6):464-70, 2007
                                29. Woods AG et al: Prune belly syndrome. A focused physical assessment. Adv Neonatal Care. 7(3):132-43; quiz 144-5, 2007
                                30. 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
                                31. 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
                                32. Perez-Brayfield MR et al: In utero intervention in a patient with prune-belly syndrome and severe urethral hypoplasia. Urology. 57(6):1178, 2001
                                Related Anatomy
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                                Related Differential Diagnoses
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                                References
                                Tables

                                Tables

                                KEY FACTS

                                • Terminology

                                  • Imaging

                                    • Top Differential Diagnoses

                                      • Clinical Issues

                                        • Diagnostic Checklist

                                          TERMINOLOGY

                                          • Synonyms

                                            • Eagle-Barrett syndrome
                                          • Definitions

                                            • 1 in group of disorders causing lower urinary tract obstruction (LUTO)
                                              • General features of LUTO: Hydronephrosis, hydroureter, bladder distention
                                            • Prune-belly syndrome (PBS) characterized by 3 principle components
                                              • Dramatic collecting system dilatation
                                              • Deficiency or laxity 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. Apostel HJCL et al: Respiratory support in the absence of abdominal muscles: a case study of ventilatory management in prune belly syndrome. Paediatr Respir Rev. 37:44-7, 2021
                                                              2. Marchal S et al: Long-term outcome of renal transplantation in patients with congenital lower urinary tract malformations: a multicenter study. Transplantation. 104(1):165-71, 2020
                                                              3. Arlen AM et al: Prune belly syndrome: current perspectives. Pediatric Health Med Ther. 10:75-81, 2019
                                                              4. Wong DG et al: Phenotypic severity scoring system and categorisation for prune belly syndrome: application to a pilot cohort of 50 living patients. BJU Int. 123(1):130-9, 2019
                                                              5. Moscardi PRM et al: Prenatal renal parenchymal area as a predictor of early end-stage renal disease in children with vesicoamniotic shunting for lower urinary tract obstruction. J Pediatr Urol. 14(4):320.e1-6, 2018
                                                              6. White JT et al: Vesicoamniotic shunting improves outcomes in a subset of prune belly syndrome patients at a single tertiary center. Front Pediatr. 6:180, 2018
                                                              7. Yalcinkaya F et al: Outcomes of renal replacement therapy in boys with prune belly syndrome: findings from the ESPN/ERA-EDTA Registry. Pediatr Nephrol. 33(1):117-24, 2018
                                                              8. Kapoor S: "Pseudo-prune belly syndrome": an under-reported phenotypic variant of prune belly syndrome. J Pediatr Urol. 13(6):656-7, 2017
                                                              9. Pellegrino M et al: Prenatal detection of megacystis: not always an adverse prognostic factor. Experience in 25 consecutive cases in a tertiary referral center, with complete neonatal outcome and follow-up. J Pediatr Urol. 13(5):486.e1-10, 2017
                                                              10. Taghavi K et al: Fetal megacystis: a systematic review. J Pediatr Urol. 13(1):7-15, 2017
                                                              11. Wijesinghe US et al: Further evidence of the etiology of prune belly syndrome provided by a transient massive intraabdominal cyst in a female. J Pediatr Surg. 51(8):1390-3, 2016
                                                              12. Seidel NE et al: Clinical manifestations and management of prune-belly syndrome in a large contemporary pediatric population. Urology. 85(1):211-5, 2015
                                                              13. Clayton DB et al: Lower urinary tract obstruction in the fetus and neonate. Clin Perinatol. 41(3):643-59, 2014
                                                              14. 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
                                                              15. Rogers S et al: Prune belly with megalourethra. Ultrasound Q. 29(3):235-6, 2013
                                                              16. 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
                                                              17. Hassett S et al: Prune belly syndrome. Pediatr Surg Int. 28(3):219-28, 2012
                                                              18. Nemec SF et al: Male sexual development in utero: testicular descent on prenatal magnetic resonance imaging. Ultrasound Obstet Gynecol. 38(6):688-94, 2011
                                                              19. Osborne NG et al: Fetal megacystis: differential diagnosis. J Ultrasound Med. 30(6):833-41, 2011
                                                              20. Chen L et al: Two- and three-dimensional prenatal sonographic diagnosis of prune-belly syndrome. J Clin Ultrasound. 38(5):279-82, 2010
                                                              21. 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. 23(10):1263-7, 2010
                                                              22. Routh JC et al: Contemporary epidemiology and characterization of newborn males with prune belly syndrome. Urology. 76(1):44-8, 2010
                                                              23. Comstock CH et al: Rupture of the fetal abdomen in prune belly syndrome. Ultrasound Obstet Gynecol. 33(4):487-8, 2009
                                                              24. Herman TE et al: Prune belly syndrome. J Perinatol. 29(1):69-71, 2009
                                                              25. Munch EM et al: Magnetic resonance imaging for prenatal diagnosis of multisystem disease: megacystis microcolon intestinal hypoperistalsis syndrome. Urology. 74(3):592-4, 2009
                                                              26. 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. 13(4):322-5, 2009
                                                              27. Wu S et al: Fetal lower urinary tract obstruction. Clin Perinatol. 36(2):377-90, x, 2009
                                                              28. Lissauer D et al: Fetal lower urinary tract obstruction. Semin Fetal Neonatal Med. 12(6):464-70, 2007
                                                              29. Woods AG et al: Prune belly syndrome. A focused physical assessment. Adv Neonatal Care. 7(3):132-43; quiz 144-5, 2007
                                                              30. 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
                                                              31. 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
                                                              32. Perez-Brayfield MR et al: In utero intervention in a patient with prune-belly syndrome and severe urethral hypoplasia. Urology. 57(6):1178, 2001