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Prune-Belly Syndrome
Sara M. O'Hara, MD, FAAP
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Eagle-Barrett syndrome, abdominal muscle deficiency, triad syndrome
          • Definitions

            • Prune-belly syndrome (PBS): Congenital triad of
              • Urinary tract dilation
              • Cryptorchidism
              • Abdominal wall muscle deficiency/laxity (with thin, wrinkled skin resembling prune)

          IMAGING

          • General Features

            • Radiographic Findings

              • Fluoroscopic Findings

                • Ultrasonographic Findings

                  • MR Findings

                    • Nuclear Medicine Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    Selected References

                                    1. Loganathan AK et al: Unusual variant of pseudo prune belly syndrome. BMJ Case Rep. 13(10):e236611, 2020
                                    2. Meyers ML et al: Imaging of fetal cystic kidney disease: multicystic dysplastic kidney versus renal cystic dysplasia. Pediatr Radiol. 50(13):1921-33, 2020
                                    3. Wiener JS et al: Challenges in pediatric urologic practice: a lifelong view. World J Urol. 39(4):981-91, 2020
                                    4. Arlen AM et al: Prune belly syndrome: current perspectives. Pediatric Health Med Ther. 10:75-81, 2019
                                    5. De Bernardo G et al: Pseudo prune belly syndrome: a case report with unilateral abdominal defect. Radiol Case Rep. 14(8):941-5, 2019
                                    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. Smith EA et al: Abdominoplasty in prune belly syndrome: modifications in Monfort technique to address variable patterns of abdominal wall weakness. J Pediatr Urol. 13(5):502.e1-6, 2017
                                    9. Awad K et al: It's not all doom and gloom: prune belly syndrome associated with VACTERL. J Neonatal Surg. 5(3):33, 2016
                                    10. Garcia-Roig ML et al: Detailed evaluation of the upper urinary tract in patients with prune belly syndrome using magnetic resonance urography. J Pediatr Urol. 12(2):122.e1-7, 2016
                                    11. National Organization of Rare Diseases: Prune belly syndrome. http://rarediseases.org/rare-diseases/prune-belly-syndrome. Reviewed July 14, 2016. Accessed July 14, 2016
                                    12. Ramagopal G et al: Prune belly syndrome with situs inversus abdominus. J Neonatal Surg. 5(3):38, 2016
                                    13. Dénes FT et al: Abdominoplasty in prune belly syndrome. J Pediatr Urol. 11(5):291-2, 2015
                                    14. Grimsby GM et al: Impact and frequency of extra-genitourinary manifestations of prune belly syndrome. J Pediatr Urol. 11(5):280.e1-6, 2015
                                    15. Lopes RI et al: 27 years of experience with the comprehensive surgical treatment of prune belly syndrome. J Pediatr Urol. 11(5):276.e1-7, 2015
                                    16. Seidel NE et al: Clinical manifestations and management of prune-belly syndrome in a large contemporary pediatric population. Urology. 85(1):211-5, 2015
                                    17. Dénes FT et al: Modified abdominoplasty for patients with the Prune Belly syndrome. Urology. 83(2):451-4, 2014
                                    18. 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
                                    19. Hassett S et al: Prune belly syndrome. Pediatr Surg Int. 28(3):219-28, 2012
                                    20. Zugor V et al: The prune belly syndrome: urological aspects and long-term outcomes of a rare disease. Pediatr Rep. 4(2):e20, 2012
                                    21. Chen IL et al: Urachal catheter provides new choice for long-term urinary diversion in prune belly syndrome. Urology. 77(2):466-8, 2011
                                    22. Giuliani S et al: Prune belly syndrome associated with cloacal anomaly, patent urachal remnant, and omphalocele in a female infant. J Pediatr Surg. 45(11):e39-42, 2010
                                    23. Herman TE et al: Prune belly syndrome. J Perinatol. 29(1):69-71, 2009
                                    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

                                            TERMINOLOGY

                                            • Synonyms

                                              • Eagle-Barrett syndrome, abdominal muscle deficiency, triad syndrome
                                            • Definitions

                                              • Prune-belly syndrome (PBS): Congenital triad of
                                                • Urinary tract dilation
                                                • Cryptorchidism
                                                • Abdominal wall muscle deficiency/laxity (with thin, wrinkled skin resembling prune)

                                            IMAGING

                                            • General Features

                                              • Radiographic Findings

                                                • Fluoroscopic Findings

                                                  • Ultrasonographic Findings

                                                    • MR Findings

                                                      • Nuclear Medicine Findings

                                                        • Imaging Recommendations

                                                          DIFFERENTIAL DIAGNOSIS

                                                            PATHOLOGY

                                                            • General Features

                                                              CLINICAL ISSUES

                                                              • Presentation

                                                                • Demographics

                                                                  • Natural History & Prognosis

                                                                    • Treatment

                                                                      Selected References

                                                                      1. Loganathan AK et al: Unusual variant of pseudo prune belly syndrome. BMJ Case Rep. 13(10):e236611, 2020
                                                                      2. Meyers ML et al: Imaging of fetal cystic kidney disease: multicystic dysplastic kidney versus renal cystic dysplasia. Pediatr Radiol. 50(13):1921-33, 2020
                                                                      3. Wiener JS et al: Challenges in pediatric urologic practice: a lifelong view. World J Urol. 39(4):981-91, 2020
                                                                      4. Arlen AM et al: Prune belly syndrome: current perspectives. Pediatric Health Med Ther. 10:75-81, 2019
                                                                      5. De Bernardo G et al: Pseudo prune belly syndrome: a case report with unilateral abdominal defect. Radiol Case Rep. 14(8):941-5, 2019
                                                                      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. Smith EA et al: Abdominoplasty in prune belly syndrome: modifications in Monfort technique to address variable patterns of abdominal wall weakness. J Pediatr Urol. 13(5):502.e1-6, 2017
                                                                      9. Awad K et al: It's not all doom and gloom: prune belly syndrome associated with VACTERL. J Neonatal Surg. 5(3):33, 2016
                                                                      10. Garcia-Roig ML et al: Detailed evaluation of the upper urinary tract in patients with prune belly syndrome using magnetic resonance urography. J Pediatr Urol. 12(2):122.e1-7, 2016
                                                                      11. National Organization of Rare Diseases: Prune belly syndrome. http://rarediseases.org/rare-diseases/prune-belly-syndrome. Reviewed July 14, 2016. Accessed July 14, 2016
                                                                      12. Ramagopal G et al: Prune belly syndrome with situs inversus abdominus. J Neonatal Surg. 5(3):38, 2016
                                                                      13. Dénes FT et al: Abdominoplasty in prune belly syndrome. J Pediatr Urol. 11(5):291-2, 2015
                                                                      14. Grimsby GM et al: Impact and frequency of extra-genitourinary manifestations of prune belly syndrome. J Pediatr Urol. 11(5):280.e1-6, 2015
                                                                      15. Lopes RI et al: 27 years of experience with the comprehensive surgical treatment of prune belly syndrome. J Pediatr Urol. 11(5):276.e1-7, 2015
                                                                      16. Seidel NE et al: Clinical manifestations and management of prune-belly syndrome in a large contemporary pediatric population. Urology. 85(1):211-5, 2015
                                                                      17. Dénes FT et al: Modified abdominoplasty for patients with the Prune Belly syndrome. Urology. 83(2):451-4, 2014
                                                                      18. 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
                                                                      19. Hassett S et al: Prune belly syndrome. Pediatr Surg Int. 28(3):219-28, 2012
                                                                      20. Zugor V et al: The prune belly syndrome: urological aspects and long-term outcomes of a rare disease. Pediatr Rep. 4(2):e20, 2012
                                                                      21. Chen IL et al: Urachal catheter provides new choice for long-term urinary diversion in prune belly syndrome. Urology. 77(2):466-8, 2011
                                                                      22. Giuliani S et al: Prune belly syndrome associated with cloacal anomaly, patent urachal remnant, and omphalocele in a female infant. J Pediatr Surg. 45(11):e39-42, 2010
                                                                      23. Herman TE et al: Prune belly syndrome. J Perinatol. 29(1):69-71, 2009