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Multicystic Dysplastic Kidney
Sara M. O'Hara, MD, FAAP
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Definitions

            • Multicystic dysplastic kidney (MCDK): Congenital, nonfunctional kidney replaced by multiple cysts & dysplastic tissue
            • 2 forms of MCDK are classically recognized
              • Pelvoinfundibular (more common): Theoretically results from atresia of ureter or renal pelvis; cysts are remnants of dilated calyces
              • Hydronephrotic (occurs less frequently): Results from atretic segment of ureter; cysts replace entire pelvocalyceal system
            • Tend to involute with time: Cysts shrink & residual tissue may lose reniform shape

          IMAGING

          • General Features

            • Radiographic Findings

              • Ultrasonographic Findings

                • CT Findings

                  • MR Findings

                    • Nuclear Medicine Findings

                      • Other Modality Findings

                        • Imaging Recommendations

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Gross Pathologic & Surgical Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        Selected References

                                        1. Choi SM et al: Renal growth slope in children with congenital and acquired solitary functioning kidneys. Ultrasonography.40(3):357-65, 2020
                                        2. Faruque A et al: Multicystic dysplastic kidney - treat each case on its merits. J Pediatr Surg. 55(11):2497-503, 2020
                                        3. Gilad N et al: Multicystic dysplastic kidney: prenatal compensatory renal growth pattern. J Ultrasound Med. 40(10):2165-71, 2020
                                        4. Meyers ML et al: Imaging of fetal cystic kidney disease: multicystic dysplastic kidney versus renal cystic dysplasia. Pediatr Radiol. 50(13):1921-33, 2020
                                        5. Pettit SM et al: Multicystic dysplastic kidney with mass effect in a neonate treated with nephrectomy: case Report. Urology.149:e11-4 2020
                                        6. Takemura K et al: Seminal vesicle cysts with upper urinary tract abnormalities: a single-center case series of pediatric Zinner syndrome. Urology. 149:e11-4, 2020
                                        7. Brown CT et al: Trends in surgical management of multicystic dysplastic kidney at USA children's hospitals. J Pediatr Urol. 15(4):368-73, 2019
                                        8. Brown C et al: Knowledge of vesicoureteral reflux obtained by screening voiding cystourethrogram in children with multicystic dysplastic kidney does not change patient management or prevent febrile urinary tract infection. J Pediatr Urol. 15(3):267.e1-5, 2019
                                        9. Gimpel C et al: Imaging of kidney cysts and cystic kidney diseases in children: an International Working Group consensus statement. Radiology. 290(3):769-82, 2019
                                        10. Gaither TW et al: Natural history of contralateral hypertrophy in patients with multicystic dysplastic kidneys. J Urol. 199(1):280-6, 2018
                                        11. Kara A et al: Clinical features of children with multicystic dysplastic kidney. Pediatr Int. 60(8):750-4, 2018
                                        12. Matsumura K et al: Trajectory of estimated glomerular filtration rate predicts renal injury in children with multicystic dysplastic kidney. Nephron. 140(1):18-23, 2018
                                        13. Curran-Melendez SM et al: Sorting the alphabet soup of renal pathology: a review. Curr Probl Diagn Radiol. 47(6):417-27, 2016
                                        14. Han JH et al: Conservative management of segmental multicystic dysplastic kidney in children. Urology. 86(5):1013-8, 2015
                                        15. Eickmeyer AB et al: The natural history of the multicystic dysplastic kidney--is limited follow-up warranted? J Pediatr Urol. 10(4):655-61, 2014
                                        16. Whittam BM et al: Ultrasound diagnosis of multicystic dysplastic kidney: is a confirmatory nuclear medicine scan necessary? J Pediatr Urol. 10(6):1059-62, 2014
                                        17. Hayes WN et al: Unilateral multicystic dysplastic kidney: does initial size matter? Pediatr Nephrol. 27(8):1335-40, 2012
                                        18. Abdulhannan P et al: Multicystic dysplastic kidney disease and hypertension: clinical and pathological correlation. J Pediatr Urol. 8(2):220, 2011
                                        19. Iscaife A et al: Segmental multicystic dysplastic kidney: a rare situation. J Pediatr Urol. 7(4):491-4, 2011
                                        20. Schreuder MF et al: Unilateral multicystic dysplastic kidney: a meta-analysis of observational studies on the incidence, associated urinary tract malformations and the contralateral kidney. Nephrol Dial Transplant. 24(6):1810-8, 2009
                                        21. Carta M et al: Unilateral multicystic dysplastic kidney in infants exposed to antiepileptic drugs during pregnancy. Pediatr Nephrol. 22(7):1054-7, 2007
                                        22. Aslam M et al: Unilateral multicystic dysplastic kidney: long term outcomes. Arch Dis Child. 91(10):820-3, 2006
                                        23. Siqueira Rabelo EA et al: Ultrasound progression of prenatally detected multicystic dysplastic kidney. Urology. 68(5):1098-102, 2006
                                        24. Narchi H: Risk of Wilms' tumour with multicystic kidney disease: a systematic review. Arch Dis Child. 90(2):147-9, 2005
                                        25. Kaneyama K et al: Associated urologic anomalies in children with solitary kidney. J Pediatr Surg. 39(1):85-7, 2004
                                        26. Abidari JM et al: Serial followup of the contralateral renal size in children with multicystic dysplastic kidney. J Urol. 168(4 Pt 2):1821-5; discussion 1825, 2002
                                        27. Suzuki K et al: Segmental multicystic dysplastic kidney in an adult woman. Urol Int. 66(1):51-4, 2001
                                        28. Snodgrass WT: Hypertension associated with multicystic dysplastic kidney in children. J Urol. 164(2):472-3;discussion 473-4, 2000
                                        29. Srivastava T et al: Autosomal dominant inheritance of multicystic dysplastic kidney. Pediatr Nephrol. 13(6):481-3, 1999
                                        30. John U et al: Kidney growth and renal function in unilateral multicystic dysplastic kidney disease. Pediatr Nephrol. 12(7):567-71, 1998
                                        31. Kessler OJ et al: Involution rate of multicystic renal dysplasia. Pediatrics. 102(6):E73, 1998
                                        32. Zerin JM et al: The impact of vesicoureteral reflux on contralateral renal length in infants with multicystic dysplastic kidney. Pediatr Radiol. 28(9):683-6, 1998
                                        33. Homsy YL et al: Wilms tumor and multicystic dysplastic kidney disease. J Urol. 158(6):2256-9; discussion 2259-60, 1997
                                        34. Beckwith JB: Wilms tumor in multicystic dysplastic kidneys: What is the risk? Dialogues in Pediatric Urology. 19:3-5, 1996
                                        35. Strife JL et al: Multicystic dysplastic kidney in children: US follow-up. Radiology. 186(3):785-8, 1993
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Pathology

                                              • Clinical Issues

                                                TERMINOLOGY

                                                • Definitions

                                                  • Multicystic dysplastic kidney (MCDK): Congenital, nonfunctional kidney replaced by multiple cysts & dysplastic tissue
                                                  • 2 forms of MCDK are classically recognized
                                                    • Pelvoinfundibular (more common): Theoretically results from atresia of ureter or renal pelvis; cysts are remnants of dilated calyces
                                                    • Hydronephrotic (occurs less frequently): Results from atretic segment of ureter; cysts replace entire pelvocalyceal system
                                                  • Tend to involute with time: Cysts shrink & residual tissue may lose reniform shape

                                                IMAGING

                                                • General Features

                                                  • Radiographic Findings

                                                    • Ultrasonographic Findings

                                                      • CT Findings

                                                        • MR Findings

                                                          • Nuclear Medicine Findings

                                                            • Other Modality Findings

                                                              • Imaging Recommendations

                                                                DIFFERENTIAL DIAGNOSIS

                                                                  PATHOLOGY

                                                                  • General Features

                                                                    • Gross Pathologic & Surgical Features

                                                                      CLINICAL ISSUES

                                                                      • Presentation

                                                                        • Demographics

                                                                          • Natural History & Prognosis

                                                                            • Treatment

                                                                              Selected References

                                                                              1. Choi SM et al: Renal growth slope in children with congenital and acquired solitary functioning kidneys. Ultrasonography.40(3):357-65, 2020
                                                                              2. Faruque A et al: Multicystic dysplastic kidney - treat each case on its merits. J Pediatr Surg. 55(11):2497-503, 2020
                                                                              3. Gilad N et al: Multicystic dysplastic kidney: prenatal compensatory renal growth pattern. J Ultrasound Med. 40(10):2165-71, 2020
                                                                              4. Meyers ML et al: Imaging of fetal cystic kidney disease: multicystic dysplastic kidney versus renal cystic dysplasia. Pediatr Radiol. 50(13):1921-33, 2020
                                                                              5. Pettit SM et al: Multicystic dysplastic kidney with mass effect in a neonate treated with nephrectomy: case Report. Urology.149:e11-4 2020
                                                                              6. Takemura K et al: Seminal vesicle cysts with upper urinary tract abnormalities: a single-center case series of pediatric Zinner syndrome. Urology. 149:e11-4, 2020
                                                                              7. Brown CT et al: Trends in surgical management of multicystic dysplastic kidney at USA children's hospitals. J Pediatr Urol. 15(4):368-73, 2019
                                                                              8. Brown C et al: Knowledge of vesicoureteral reflux obtained by screening voiding cystourethrogram in children with multicystic dysplastic kidney does not change patient management or prevent febrile urinary tract infection. J Pediatr Urol. 15(3):267.e1-5, 2019
                                                                              9. Gimpel C et al: Imaging of kidney cysts and cystic kidney diseases in children: an International Working Group consensus statement. Radiology. 290(3):769-82, 2019
                                                                              10. Gaither TW et al: Natural history of contralateral hypertrophy in patients with multicystic dysplastic kidneys. J Urol. 199(1):280-6, 2018
                                                                              11. Kara A et al: Clinical features of children with multicystic dysplastic kidney. Pediatr Int. 60(8):750-4, 2018
                                                                              12. Matsumura K et al: Trajectory of estimated glomerular filtration rate predicts renal injury in children with multicystic dysplastic kidney. Nephron. 140(1):18-23, 2018
                                                                              13. Curran-Melendez SM et al: Sorting the alphabet soup of renal pathology: a review. Curr Probl Diagn Radiol. 47(6):417-27, 2016
                                                                              14. Han JH et al: Conservative management of segmental multicystic dysplastic kidney in children. Urology. 86(5):1013-8, 2015
                                                                              15. Eickmeyer AB et al: The natural history of the multicystic dysplastic kidney--is limited follow-up warranted? J Pediatr Urol. 10(4):655-61, 2014
                                                                              16. Whittam BM et al: Ultrasound diagnosis of multicystic dysplastic kidney: is a confirmatory nuclear medicine scan necessary? J Pediatr Urol. 10(6):1059-62, 2014
                                                                              17. Hayes WN et al: Unilateral multicystic dysplastic kidney: does initial size matter? Pediatr Nephrol. 27(8):1335-40, 2012
                                                                              18. Abdulhannan P et al: Multicystic dysplastic kidney disease and hypertension: clinical and pathological correlation. J Pediatr Urol. 8(2):220, 2011
                                                                              19. Iscaife A et al: Segmental multicystic dysplastic kidney: a rare situation. J Pediatr Urol. 7(4):491-4, 2011
                                                                              20. Schreuder MF et al: Unilateral multicystic dysplastic kidney: a meta-analysis of observational studies on the incidence, associated urinary tract malformations and the contralateral kidney. Nephrol Dial Transplant. 24(6):1810-8, 2009
                                                                              21. Carta M et al: Unilateral multicystic dysplastic kidney in infants exposed to antiepileptic drugs during pregnancy. Pediatr Nephrol. 22(7):1054-7, 2007
                                                                              22. Aslam M et al: Unilateral multicystic dysplastic kidney: long term outcomes. Arch Dis Child. 91(10):820-3, 2006
                                                                              23. Siqueira Rabelo EA et al: Ultrasound progression of prenatally detected multicystic dysplastic kidney. Urology. 68(5):1098-102, 2006
                                                                              24. Narchi H: Risk of Wilms' tumour with multicystic kidney disease: a systematic review. Arch Dis Child. 90(2):147-9, 2005
                                                                              25. Kaneyama K et al: Associated urologic anomalies in children with solitary kidney. J Pediatr Surg. 39(1):85-7, 2004
                                                                              26. Abidari JM et al: Serial followup of the contralateral renal size in children with multicystic dysplastic kidney. J Urol. 168(4 Pt 2):1821-5; discussion 1825, 2002
                                                                              27. Suzuki K et al: Segmental multicystic dysplastic kidney in an adult woman. Urol Int. 66(1):51-4, 2001
                                                                              28. Snodgrass WT: Hypertension associated with multicystic dysplastic kidney in children. J Urol. 164(2):472-3;discussion 473-4, 2000
                                                                              29. Srivastava T et al: Autosomal dominant inheritance of multicystic dysplastic kidney. Pediatr Nephrol. 13(6):481-3, 1999
                                                                              30. John U et al: Kidney growth and renal function in unilateral multicystic dysplastic kidney disease. Pediatr Nephrol. 12(7):567-71, 1998
                                                                              31. Kessler OJ et al: Involution rate of multicystic renal dysplasia. Pediatrics. 102(6):E73, 1998
                                                                              32. Zerin JM et al: The impact of vesicoureteral reflux on contralateral renal length in infants with multicystic dysplastic kidney. Pediatr Radiol. 28(9):683-6, 1998
                                                                              33. Homsy YL et al: Wilms tumor and multicystic dysplastic kidney disease. J Urol. 158(6):2256-9; discussion 2259-60, 1997
                                                                              34. Beckwith JB: Wilms tumor in multicystic dysplastic kidneys: What is the risk? Dialogues in Pediatric Urology. 19:3-5, 1996
                                                                              35. Strife JL et al: Multicystic dysplastic kidney in children: US follow-up. Radiology. 186(3):785-8, 1993