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Pediatrics
Diagnosis
Genitourinary
Congenital Urinary Tract Abnormalities
Ureteropelvic Duplications
Ureteropelvic Duplications
Sara M. O'Hara, MD, FAAP
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Duplicated kidney, duplex collecting system, partial/incomplete/complete duplication, bifid pelvis

          • Definitions

            • Presence of 2 separate pelvicalyceal collecting systems in 1 kidney
              • 2 draining ureters may
                • Join above bladder (partial duplication)
                • Insert into bladder separately (complete duplication)

          IMAGING

          • General Features

            • Ultrasonographic Findings

              • Fluoroscopic Findings

                • CT Findings

                  • MR Findings

                    • Nuclear Medicine Findings

                      • Multimodality Findings

                        • Imaging Recommendations

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          Selected References

                                          1. Houat AP et al: Congenital anomalies of the upper urinary tract: a comprehensive review. Radiographics. 41(2):462-86, 2021
                                          2. Chan KKC et al: Unusual ureteric duplication abnormality in a child with pelvoureteric junction obstruction. BMJ Case Rep. 13(3), 2020
                                          3. Ji H et al: Prenatal diagnosis of renal duplication by magnetic resonance imaging. J Matern Fetal Neonatal Med. 33(14):2342-7, 2020
                                          4. Abdelhalim A et al: Ipsilateral ureteroureterostomy for ureteral duplication anomalies: predictors of adverse outcomes. J Pediatr Urol. 15(5):468.e1-6, 2019
                                          5. Damasio MB et al: Comparative study between functional mr urography and renal scintigraphy to evaluate drainage curves and split renal function in children with congenital anomalies of kidney and urinary tract (CAKUT). Front Pediatr. 7:527, 2019
                                          6. Tsiflikas I et al: Functional magnetic resonance urography in infants: feasibility of a feed-and-sleep technique. Pediatr Radiol. 49(3):351-7, 2019
                                          7. Rodriguez MV et al: Robot-assisted laparoscopic common sheath ureteral reimplantation in duplex ureters: LUAA technique tips for optimal outcomes. J Pediatr Urol. 14(4):353-5, 2018
                                          8. Didier RA et al: The duplicated collecting system of the urinary tract: embryology, imaging appearances and clinical considerations. Pediatr Radiol. 47(11):1526-38, 2017
                                          9. Expert Panel on Pediatric Imaging:. et al: ACR Appropriateness Criteria® urinary tract infection-child. J Am Coll Radiol. 14(5S):S362-71, 2017
                                          10. Giorlando F et al: Duplex collecting system in a pelvic kidney - an unusual combination. J Radiol Case Rep. 11(12):8-15, 2017
                                          11. Mendichovszky I et al: Nuclear medicine in pediatric nephro-urology: an overview. Semin Nucl Med. 47(3):204-28, 2017
                                          12. Michaud JE et al: Upper pole heminephrectomy versus lower pole ureteroureterostomy for ectopic upper pole ureters. Curr Urol Rep. 18(3):21, 2017
                                          13. Flanagan SM et al: Fetal suprarenal masses--assessing the complementary role of magnetic resonance and ultrasound for diagnosis. Pediatr Radiol. 46(2):246-54, 2016
                                          14. Khrichenko D et al: Intra- and inter-observer variability of functional MR urography (fMRU) assessment in children. Pediatr Radiol. 46(5):666-73, 2016
                                          15. Ramanathan S et al: Multi-modality imaging review of congenital abnormalities of kidney and upper urinary tract. World J Radiol. 8(2):132-41, 2016
                                          16. Sirisreetreerux P et al: Duplicated renal collecting system with ectopic ureter in female bladder exstrophy: a case report. Urology. 89:129-31, 2016
                                          17. Cezarino BN et al: Diagnostic work-up and laparoscopic correction of an ectopic ureter. J Pediatr Urol. 11(5):285-6, 2015
                                          18. Lee YS et al: Complications after common sheath reimplantation in pediatric patients with complicated duplex system. Urology. 85(2):457-62, 2015
                                          19. Mansouri R et al: A case of obstructed hemivagina with ectopic ureter leading to severe hydrocolpos and contralateral renal outflow tract obstruction in a neonate. J Pediatr Adolesc Gynecol. 28(5):e131-3, 2015
                                          20. Narchi H et al: Renal tract abnormalities missed in a historical cohort of young children with UTI if the NICE and AAP imaging guidelines were applied. J Pediatr Urol. 11(5):252.e1-7, 2015
                                          21. Rubenwolf P et al: Presentation, management and long-term outcome of ureteropelvic junction obstruction in duplex kidneys. J Urol. 194(2):427-32, 2015
                                          22. Schlomer B et al: Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome should be redefined as ipsilateral renal anomalies: cases of symptomatic atrophic and dysplastic kidney with ectopic ureter to obstructed hemivagina. J Pediatr Urol. 11(2):77.e1-6, 2015
                                          23. Wang MH: Persistent urinary incontinence: a case series of missed ectopic ureters. Urol Case Rep. 3(6):223-5, 2015
                                          24. Zderic SA et al: Voiding dysfunction: what can radiologists tell patients and pediatric urologists? AJR Am J Roentgenol. 205(5):W532-41, 2015
                                          25. Bansal D et al: Pediatric laparo-endoscopic single site partial nephrectomy: feasibility in infants and small children for upper urinary tract duplication anomalies. J Pediatr Urol. 10(5):859-63, 2014
                                          26. Figueroa VH et al: Utility of MR urography in children suspected of having ectopic ureter. Pediatr Radiol. 44(8):956-62, 2014
                                          27. Avlan D et al: Pyeloureterostomy in the management of the lower pole pelvi-ureteric junction obstruction in incomplete duplicated systems. Urology. 76(6):1468-71, 2010
                                          28. Jain P et al: Uncrossed complete ureteral duplication with dysplastic lower moiety: a violation of the Weigert-Meyer law. J Pediatr Urol. 4(5):404-6, 2008
                                          29. Lipson JA et al: Subtle renal duplication as an unrecognized cause of childhood incontinence: diagnosis by magnetic resonance urography. J Pediatr Urol. 4(5):398-400, 2008
                                          30. Castagnetti M et al: Transurethral incision of duplex system ureteroceles in neonates: does it increase the need for secondary surgery in intravesical and ectopic cases? BJU Int. 93(9):1313-7, 2004
                                          31. Davidovits M et al: Unilateral duplicated system: comparative length and function of the kidneys. Clin Nucl Med. 29(2):99-102, 2004
                                          32. Perez-Brayfield M et al: Endoscopic treatment with dextranomer/hyaluronic acid for complex cases of vesicoureteral reflux. J Urol. 172(4 Pt 2):1614-6, 2004
                                          33. Wah TM et al: Lower moiety pelvic-ureteric junction obstruction (PUJO) of the duplex kidney presenting with pyonephrosis in adults. Br J Radiol. 76(912):909-12, 2003
                                          34. Whitten SM et al: Accuracy of antenatal fetal ultrasound in the diagnosis of duplex kidneys. Ultrasound Obstet Gynecol. 21(4):342-6, 2003
                                          35. De Caluwe D et al: Fate of the retained ureteral stump after upper pole heminephrectomy in duplex kidneys. J Urol. 168(2):679-80, 2002
                                          36. Husmann DA et al: Is endoscopic decompression of the neonatal extravesical upper pole ureterocele necessary for prevention of urinary tract infections or bladder neck obstruction? J Urol. 167(3):1440-2, 2002
                                          37. Decter RM: Renal duplication and fusion anomalies. Pediatr Clin North Am. 44(5):1323-41, 1997
                                          38. Avni EF et al: Renal duplications: the impact of perinatal ultrasound on diagnosis and management. Eur Urol. 20(1):43-8, 1991
                                          Related Anatomy
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                                          Related Differential Diagnoses
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                                          References
                                          Tables

                                          Tables

                                          KEY FACTS

                                          • Terminology

                                            • Imaging

                                              • Pathology

                                                • Clinical Issues

                                                  TERMINOLOGY

                                                  • Synonyms

                                                    • Duplicated kidney, duplex collecting system, partial/incomplete/complete duplication, bifid pelvis

                                                  • Definitions

                                                    • Presence of 2 separate pelvicalyceal collecting systems in 1 kidney
                                                      • 2 draining ureters may
                                                        • Join above bladder (partial duplication)
                                                        • Insert into bladder separately (complete duplication)

                                                  IMAGING

                                                  • General Features

                                                    • Ultrasonographic Findings

                                                      • Fluoroscopic Findings

                                                        • CT Findings

                                                          • MR Findings

                                                            • Nuclear Medicine Findings

                                                              • Multimodality Findings

                                                                • Imaging Recommendations

                                                                  DIFFERENTIAL DIAGNOSIS

                                                                    PATHOLOGY

                                                                    • General Features

                                                                      • Gross Pathologic & Surgical Features

                                                                        • Microscopic Features

                                                                          CLINICAL ISSUES

                                                                          • Presentation

                                                                            • Demographics

                                                                              • Natural History & Prognosis

                                                                                • Treatment

                                                                                  Selected References

                                                                                  1. Houat AP et al: Congenital anomalies of the upper urinary tract: a comprehensive review. Radiographics. 41(2):462-86, 2021
                                                                                  2. Chan KKC et al: Unusual ureteric duplication abnormality in a child with pelvoureteric junction obstruction. BMJ Case Rep. 13(3), 2020
                                                                                  3. Ji H et al: Prenatal diagnosis of renal duplication by magnetic resonance imaging. J Matern Fetal Neonatal Med. 33(14):2342-7, 2020
                                                                                  4. Abdelhalim A et al: Ipsilateral ureteroureterostomy for ureteral duplication anomalies: predictors of adverse outcomes. J Pediatr Urol. 15(5):468.e1-6, 2019
                                                                                  5. Damasio MB et al: Comparative study between functional mr urography and renal scintigraphy to evaluate drainage curves and split renal function in children with congenital anomalies of kidney and urinary tract (CAKUT). Front Pediatr. 7:527, 2019
                                                                                  6. Tsiflikas I et al: Functional magnetic resonance urography in infants: feasibility of a feed-and-sleep technique. Pediatr Radiol. 49(3):351-7, 2019
                                                                                  7. Rodriguez MV et al: Robot-assisted laparoscopic common sheath ureteral reimplantation in duplex ureters: LUAA technique tips for optimal outcomes. J Pediatr Urol. 14(4):353-5, 2018
                                                                                  8. Didier RA et al: The duplicated collecting system of the urinary tract: embryology, imaging appearances and clinical considerations. Pediatr Radiol. 47(11):1526-38, 2017
                                                                                  9. Expert Panel on Pediatric Imaging:. et al: ACR Appropriateness Criteria® urinary tract infection-child. J Am Coll Radiol. 14(5S):S362-71, 2017
                                                                                  10. Giorlando F et al: Duplex collecting system in a pelvic kidney - an unusual combination. J Radiol Case Rep. 11(12):8-15, 2017
                                                                                  11. Mendichovszky I et al: Nuclear medicine in pediatric nephro-urology: an overview. Semin Nucl Med. 47(3):204-28, 2017
                                                                                  12. Michaud JE et al: Upper pole heminephrectomy versus lower pole ureteroureterostomy for ectopic upper pole ureters. Curr Urol Rep. 18(3):21, 2017
                                                                                  13. Flanagan SM et al: Fetal suprarenal masses--assessing the complementary role of magnetic resonance and ultrasound for diagnosis. Pediatr Radiol. 46(2):246-54, 2016
                                                                                  14. Khrichenko D et al: Intra- and inter-observer variability of functional MR urography (fMRU) assessment in children. Pediatr Radiol. 46(5):666-73, 2016
                                                                                  15. Ramanathan S et al: Multi-modality imaging review of congenital abnormalities of kidney and upper urinary tract. World J Radiol. 8(2):132-41, 2016
                                                                                  16. Sirisreetreerux P et al: Duplicated renal collecting system with ectopic ureter in female bladder exstrophy: a case report. Urology. 89:129-31, 2016
                                                                                  17. Cezarino BN et al: Diagnostic work-up and laparoscopic correction of an ectopic ureter. J Pediatr Urol. 11(5):285-6, 2015
                                                                                  18. Lee YS et al: Complications after common sheath reimplantation in pediatric patients with complicated duplex system. Urology. 85(2):457-62, 2015
                                                                                  19. Mansouri R et al: A case of obstructed hemivagina with ectopic ureter leading to severe hydrocolpos and contralateral renal outflow tract obstruction in a neonate. J Pediatr Adolesc Gynecol. 28(5):e131-3, 2015
                                                                                  20. Narchi H et al: Renal tract abnormalities missed in a historical cohort of young children with UTI if the NICE and AAP imaging guidelines were applied. J Pediatr Urol. 11(5):252.e1-7, 2015
                                                                                  21. Rubenwolf P et al: Presentation, management and long-term outcome of ureteropelvic junction obstruction in duplex kidneys. J Urol. 194(2):427-32, 2015
                                                                                  22. Schlomer B et al: Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome should be redefined as ipsilateral renal anomalies: cases of symptomatic atrophic and dysplastic kidney with ectopic ureter to obstructed hemivagina. J Pediatr Urol. 11(2):77.e1-6, 2015
                                                                                  23. Wang MH: Persistent urinary incontinence: a case series of missed ectopic ureters. Urol Case Rep. 3(6):223-5, 2015
                                                                                  24. Zderic SA et al: Voiding dysfunction: what can radiologists tell patients and pediatric urologists? AJR Am J Roentgenol. 205(5):W532-41, 2015
                                                                                  25. Bansal D et al: Pediatric laparo-endoscopic single site partial nephrectomy: feasibility in infants and small children for upper urinary tract duplication anomalies. J Pediatr Urol. 10(5):859-63, 2014
                                                                                  26. Figueroa VH et al: Utility of MR urography in children suspected of having ectopic ureter. Pediatr Radiol. 44(8):956-62, 2014
                                                                                  27. Avlan D et al: Pyeloureterostomy in the management of the lower pole pelvi-ureteric junction obstruction in incomplete duplicated systems. Urology. 76(6):1468-71, 2010
                                                                                  28. Jain P et al: Uncrossed complete ureteral duplication with dysplastic lower moiety: a violation of the Weigert-Meyer law. J Pediatr Urol. 4(5):404-6, 2008
                                                                                  29. Lipson JA et al: Subtle renal duplication as an unrecognized cause of childhood incontinence: diagnosis by magnetic resonance urography. J Pediatr Urol. 4(5):398-400, 2008
                                                                                  30. Castagnetti M et al: Transurethral incision of duplex system ureteroceles in neonates: does it increase the need for secondary surgery in intravesical and ectopic cases? BJU Int. 93(9):1313-7, 2004
                                                                                  31. Davidovits M et al: Unilateral duplicated system: comparative length and function of the kidneys. Clin Nucl Med. 29(2):99-102, 2004
                                                                                  32. Perez-Brayfield M et al: Endoscopic treatment with dextranomer/hyaluronic acid for complex cases of vesicoureteral reflux. J Urol. 172(4 Pt 2):1614-6, 2004
                                                                                  33. Wah TM et al: Lower moiety pelvic-ureteric junction obstruction (PUJO) of the duplex kidney presenting with pyonephrosis in adults. Br J Radiol. 76(912):909-12, 2003
                                                                                  34. Whitten SM et al: Accuracy of antenatal fetal ultrasound in the diagnosis of duplex kidneys. Ultrasound Obstet Gynecol. 21(4):342-6, 2003
                                                                                  35. De Caluwe D et al: Fate of the retained ureteral stump after upper pole heminephrectomy in duplex kidneys. J Urol. 168(2):679-80, 2002
                                                                                  36. Husmann DA et al: Is endoscopic decompression of the neonatal extravesical upper pole ureterocele necessary for prevention of urinary tract infections or bladder neck obstruction? J Urol. 167(3):1440-2, 2002
                                                                                  37. Decter RM: Renal duplication and fusion anomalies. Pediatr Clin North Am. 44(5):1323-41, 1997
                                                                                  38. Avni EF et al: Renal duplications: the impact of perinatal ultrasound on diagnosis and management. Eur Urol. 20(1):43-8, 1991