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Renal Ectopia and Fusion
Sara M. O'Hara, MD, FAAP
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Includes horseshoe or pancake kidney; crossed fused ectopia; pelvic, iliac/pelvic (ptotic/mobile), & thoracic kidneys
          • Definitions

            • Normal renal tissue in abnormal location
            • Results from abnormal ascent & rotation of fetal kidney
            • Kidneys form at sacral level & ascend to L1 by term; renal pelvis is initially directed anteriorly but rotates 90° medially as it ascends
            • Malpositioned kidneys are more susceptible to trauma, iatrogenic injury, obstruction, infection, & stones
            • High incidence of multiple renal arteries & veins

          IMAGING

          • General Features

            • Radiographic Findings

              • Fluoroscopic Findings

                • Ultrasonographic Findings

                  • CT Findings

                    • MR Findings

                      • Angiographic Findings

                        • Nuclear Medicine Findings

                          • Imaging Recommendations

                            DIFFERENTIAL DIAGNOSIS

                              PATHOLOGY

                              • General Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        Selected References

                                        1. Asanad K et al: Grade IV renal laceration in a 13-year-old boy with cross-fused renal ectopia. Urology. 145:243-6, 2020
                                        2. Kini S et al: Renal anomalies and microtia: determining the clinical utility of screening affected children. Int J Pediatr Otorhinolaryngol. 133:109957, 2020
                                        3. Sarhan O et al: Crossed fused renal ectopia: diagnosis and prognosis as a single-center experience. J Pediatr Surg. 56(9):1632-7, 2020
                                        4. Loganathan AK et al: Crossed fused renal ectopia in children: a review of clinical profile, surgical challenges, and outcome. J Pediatr Urol. 15(4):315-21, 2019
                                        5. Lomoro P et al: Pancake kidney, a rare and often misdiagnosed malformation: a case report and radiological differential diagnosis. J Ultrasound. 22(2):207-13, 2019
                                        6. Taghavi K et al: The horseshoe kidney: surgical anatomy and embryology. J Pediatr Urol. 12(5):275-80, 2016
                                        7. Ratola A et al: Crossed renal ectopia without fusion: an uncommon cause of abdominal mass. Case Rep Nephrol. 2015:679342, 2015
                                        8. Szmigielska A et al: Rare renal ectopia in children - intrathoracic ectopic kidney. Dev Period Med. 19(2):186-8, 2015
                                        9. Kesan K et al: Solitary crossed renal ectopia with vesicoureteric reflux presenting with impaired renal function in a neonate. J Clin Neonatol. 2(3):140-2, 2013
                                        10. Schreuder MF: Unilateral anomalies of kidney development: why is left not right? Kidney Int. 80(7):740-5, 2011
                                        11. Oh KY et al: Prenatal diagnosis of renal developmental anomalies associated with an empty renal fossa. Ultrasound Q. 26(4):233-40, 2010
                                        12. Schwartz MJ et al: Pelvic cake kidney with a solitary ureter and bilateral congenital absence of the vas deferens. Urology. 75(1):170-2, 2010
                                        13. van den Bosch CM et al: Urological and nephrological findings of renal ectopia. J Urol. 183(4):1574-8, 2010
                                        14. Glodny B et al: Kidney fusion anomalies revisited: clinical and radiological analysis of 209 cases of crossed fused ectopia and horseshoe kidney. BJU Int. 103(2):224-35, 2009
                                        15. Arena F et al: Is a complete urological evaluation necessary in all newborns with asymptomatic renal ectopia? Int J Urol. 14(6):491-5, 2007
                                        16. Sözübir S et al: Ectopic thoracic kidney in a child with congenital diaphragmatic hernia. Eur J Pediatr Surg. 15(3):206-9, 2005
                                        17. Guarino N et al: The incidence of associated urological abnormalities in children with renal ectopia. J Urol. 172(4 Pt 2):1757-9; discussion 1759, 2004
                                        18. Yuksel A et al: Sonographic findings of fetuses with an empty renal fossa and normal amniotic fluid volume. Fetal Diagn Ther. 19(6):525-32, 2004
                                        19. Watanabe T: Reflux nephropathy in a patient with crossed renal ectopia with fusion. Pediatr Nephrol. 17(8):617-9, 2002
                                        20. Buyukdereli G et al: Tc-99m DMSA and Tc-99m DTPA imaging in the diagnosis of crossed renal ectopia. Clin Nucl Med. 26(3):257-8, 2001
                                        21. Jefferson KP et al: Thoracic kidney: a rare form of renal ectopia. J Urol. 165(2):504, 2001
                                        22. Kumar A et al: Live donation of ectopic kidneys: a feasible option under compelling circumstances. J Urol. 165(2):505-6, 2001
                                        23. Rinat C et al: Familial inheritance of crossed fused renal ectopia. Pediatr Nephrol. 16(3):269-70, 2001
                                        24. Wang RY et al: Syndromic ear anomalies and renal ultrasounds. Pediatrics. 108(2):E32, 2001
                                        25. Pérez LM et al: A rare form of crossed-fused renal ectopia: fusion anterior to the left orthotopic kidney. Urology. 54(4):742-3, 1999
                                        26. Lezcano L et al: Crossed renal ectopia associated with maternal alkaloid cocaine abuse: a case report. J Perinatol. 14(3):230-3, 1994
                                        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

                                                  • Includes horseshoe or pancake kidney; crossed fused ectopia; pelvic, iliac/pelvic (ptotic/mobile), & thoracic kidneys
                                                • Definitions

                                                  • Normal renal tissue in abnormal location
                                                  • Results from abnormal ascent & rotation of fetal kidney
                                                  • Kidneys form at sacral level & ascend to L1 by term; renal pelvis is initially directed anteriorly but rotates 90° medially as it ascends
                                                  • Malpositioned kidneys are more susceptible to trauma, iatrogenic injury, obstruction, infection, & stones
                                                  • High incidence of multiple renal arteries & veins

                                                IMAGING

                                                • General Features

                                                  • Radiographic Findings

                                                    • Fluoroscopic Findings

                                                      • Ultrasonographic Findings

                                                        • CT Findings

                                                          • MR Findings

                                                            • Angiographic Findings

                                                              • Nuclear Medicine Findings

                                                                • Imaging Recommendations

                                                                  DIFFERENTIAL DIAGNOSIS

                                                                    PATHOLOGY

                                                                    • General Features

                                                                      CLINICAL ISSUES

                                                                      • Presentation

                                                                        • Demographics

                                                                          • Natural History & Prognosis

                                                                            • Treatment

                                                                              Selected References

                                                                              1. Asanad K et al: Grade IV renal laceration in a 13-year-old boy with cross-fused renal ectopia. Urology. 145:243-6, 2020
                                                                              2. Kini S et al: Renal anomalies and microtia: determining the clinical utility of screening affected children. Int J Pediatr Otorhinolaryngol. 133:109957, 2020
                                                                              3. Sarhan O et al: Crossed fused renal ectopia: diagnosis and prognosis as a single-center experience. J Pediatr Surg. 56(9):1632-7, 2020
                                                                              4. Loganathan AK et al: Crossed fused renal ectopia in children: a review of clinical profile, surgical challenges, and outcome. J Pediatr Urol. 15(4):315-21, 2019
                                                                              5. Lomoro P et al: Pancake kidney, a rare and often misdiagnosed malformation: a case report and radiological differential diagnosis. J Ultrasound. 22(2):207-13, 2019
                                                                              6. Taghavi K et al: The horseshoe kidney: surgical anatomy and embryology. J Pediatr Urol. 12(5):275-80, 2016
                                                                              7. Ratola A et al: Crossed renal ectopia without fusion: an uncommon cause of abdominal mass. Case Rep Nephrol. 2015:679342, 2015
                                                                              8. Szmigielska A et al: Rare renal ectopia in children - intrathoracic ectopic kidney. Dev Period Med. 19(2):186-8, 2015
                                                                              9. Kesan K et al: Solitary crossed renal ectopia with vesicoureteric reflux presenting with impaired renal function in a neonate. J Clin Neonatol. 2(3):140-2, 2013
                                                                              10. Schreuder MF: Unilateral anomalies of kidney development: why is left not right? Kidney Int. 80(7):740-5, 2011
                                                                              11. Oh KY et al: Prenatal diagnosis of renal developmental anomalies associated with an empty renal fossa. Ultrasound Q. 26(4):233-40, 2010
                                                                              12. Schwartz MJ et al: Pelvic cake kidney with a solitary ureter and bilateral congenital absence of the vas deferens. Urology. 75(1):170-2, 2010
                                                                              13. van den Bosch CM et al: Urological and nephrological findings of renal ectopia. J Urol. 183(4):1574-8, 2010
                                                                              14. Glodny B et al: Kidney fusion anomalies revisited: clinical and radiological analysis of 209 cases of crossed fused ectopia and horseshoe kidney. BJU Int. 103(2):224-35, 2009
                                                                              15. Arena F et al: Is a complete urological evaluation necessary in all newborns with asymptomatic renal ectopia? Int J Urol. 14(6):491-5, 2007
                                                                              16. Sözübir S et al: Ectopic thoracic kidney in a child with congenital diaphragmatic hernia. Eur J Pediatr Surg. 15(3):206-9, 2005
                                                                              17. Guarino N et al: The incidence of associated urological abnormalities in children with renal ectopia. J Urol. 172(4 Pt 2):1757-9; discussion 1759, 2004
                                                                              18. Yuksel A et al: Sonographic findings of fetuses with an empty renal fossa and normal amniotic fluid volume. Fetal Diagn Ther. 19(6):525-32, 2004
                                                                              19. Watanabe T: Reflux nephropathy in a patient with crossed renal ectopia with fusion. Pediatr Nephrol. 17(8):617-9, 2002
                                                                              20. Buyukdereli G et al: Tc-99m DMSA and Tc-99m DTPA imaging in the diagnosis of crossed renal ectopia. Clin Nucl Med. 26(3):257-8, 2001
                                                                              21. Jefferson KP et al: Thoracic kidney: a rare form of renal ectopia. J Urol. 165(2):504, 2001
                                                                              22. Kumar A et al: Live donation of ectopic kidneys: a feasible option under compelling circumstances. J Urol. 165(2):505-6, 2001
                                                                              23. Rinat C et al: Familial inheritance of crossed fused renal ectopia. Pediatr Nephrol. 16(3):269-70, 2001
                                                                              24. Wang RY et al: Syndromic ear anomalies and renal ultrasounds. Pediatrics. 108(2):E32, 2001
                                                                              25. Pérez LM et al: A rare form of crossed-fused renal ectopia: fusion anterior to the left orthotopic kidney. Urology. 54(4):742-3, 1999
                                                                              26. Lezcano L et al: Crossed renal ectopia associated with maternal alkaloid cocaine abuse: a case report. J Perinatol. 14(3):230-3, 1994