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
Choi SM et al: Renal growth slope in children with congenital and acquired solitary functioning kidneys. Ultrasonography.40(3):357-65, 2020
Faruque A et al: Multicystic dysplastic kidney - treat each case on its merits. J Pediatr Surg. 55(11):2497-503, 2020
Gilad N et al: Multicystic dysplastic kidney: prenatal compensatory renal growth pattern. J Ultrasound Med. 40(10):2165-71, 2020
Meyers ML et al: Imaging of fetal cystic kidney disease: multicystic dysplastic kidney versus renal cystic dysplasia. Pediatr Radiol. 50(13):1921-33, 2020
Pettit SM et al: Multicystic dysplastic kidney with mass effect in a neonate treated with nephrectomy: case Report. Urology.149:e11-4 2020
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
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
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
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
Gaither TW et al: Natural history of contralateral hypertrophy in patients with multicystic dysplastic kidneys. J Urol. 199(1):280-6, 2018
Kara A et al: Clinical features of children with multicystic dysplastic kidney. Pediatr Int. 60(8):750-4, 2018
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
Curran-Melendez SM et al: Sorting the alphabet soup of renal pathology: a review. Curr Probl Diagn Radiol. 47(6):417-27, 2016
Han JH et al: Conservative management of segmental multicystic dysplastic kidney in children. Urology. 86(5):1013-8, 2015
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
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
Hayes WN et al: Unilateral multicystic dysplastic kidney: does initial size matter? Pediatr Nephrol. 27(8):1335-40, 2012
Abdulhannan P et al: Multicystic dysplastic kidney disease and hypertension: clinical and pathological correlation. J Pediatr Urol. 8(2):220, 2011
Iscaife A et al: Segmental multicystic dysplastic kidney: a rare situation. J Pediatr Urol. 7(4):491-4, 2011
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
Carta M et al: Unilateral multicystic dysplastic kidney in infants exposed to antiepileptic drugs during pregnancy. Pediatr Nephrol. 22(7):1054-7, 2007
Aslam M et al: Unilateral multicystic dysplastic kidney: long term outcomes. Arch Dis Child. 91(10):820-3, 2006
Siqueira Rabelo EA et al: Ultrasound progression of prenatally detected multicystic dysplastic kidney. Urology. 68(5):1098-102, 2006
Narchi H: Risk of Wilms' tumour with multicystic kidney disease: a systematic review. Arch Dis Child. 90(2):147-9, 2005
Kaneyama K et al: Associated urologic anomalies in children with solitary kidney. J Pediatr Surg. 39(1):85-7, 2004
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
Suzuki K et al: Segmental multicystic dysplastic kidney in an adult woman. Urol Int. 66(1):51-4, 2001
Snodgrass WT: Hypertension associated with multicystic dysplastic kidney in children. J Urol. 164(2):472-3;discussion 473-4, 2000
Srivastava T et al: Autosomal dominant inheritance of multicystic dysplastic kidney. Pediatr Nephrol. 13(6):481-3, 1999
John U et al: Kidney growth and renal function in unilateral multicystic dysplastic kidney disease. Pediatr Nephrol. 12(7):567-71, 1998
Kessler OJ et al: Involution rate of multicystic renal dysplasia. Pediatrics. 102(6):E73, 1998
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
Homsy YL et al: Wilms tumor and multicystic dysplastic kidney disease. J Urol. 158(6):2256-9; discussion 2259-60, 1997
Beckwith JB: Wilms tumor in multicystic dysplastic kidneys: What is the risk? Dialogues in Pediatric Urology. 19:3-5, 1996
Strife JL et al: Multicystic dysplastic kidney in children: US follow-up. Radiology. 186(3):785-8, 1993
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
Choi SM et al: Renal growth slope in children with congenital and acquired solitary functioning kidneys. Ultrasonography.40(3):357-65, 2020
Faruque A et al: Multicystic dysplastic kidney - treat each case on its merits. J Pediatr Surg. 55(11):2497-503, 2020
Gilad N et al: Multicystic dysplastic kidney: prenatal compensatory renal growth pattern. J Ultrasound Med. 40(10):2165-71, 2020
Meyers ML et al: Imaging of fetal cystic kidney disease: multicystic dysplastic kidney versus renal cystic dysplasia. Pediatr Radiol. 50(13):1921-33, 2020
Pettit SM et al: Multicystic dysplastic kidney with mass effect in a neonate treated with nephrectomy: case Report. Urology.149:e11-4 2020
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
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
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
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
Gaither TW et al: Natural history of contralateral hypertrophy in patients with multicystic dysplastic kidneys. J Urol. 199(1):280-6, 2018
Kara A et al: Clinical features of children with multicystic dysplastic kidney. Pediatr Int. 60(8):750-4, 2018
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
Curran-Melendez SM et al: Sorting the alphabet soup of renal pathology: a review. Curr Probl Diagn Radiol. 47(6):417-27, 2016
Han JH et al: Conservative management of segmental multicystic dysplastic kidney in children. Urology. 86(5):1013-8, 2015
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
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
Hayes WN et al: Unilateral multicystic dysplastic kidney: does initial size matter? Pediatr Nephrol. 27(8):1335-40, 2012
Abdulhannan P et al: Multicystic dysplastic kidney disease and hypertension: clinical and pathological correlation. J Pediatr Urol. 8(2):220, 2011
Iscaife A et al: Segmental multicystic dysplastic kidney: a rare situation. J Pediatr Urol. 7(4):491-4, 2011
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
Carta M et al: Unilateral multicystic dysplastic kidney in infants exposed to antiepileptic drugs during pregnancy. Pediatr Nephrol. 22(7):1054-7, 2007
Aslam M et al: Unilateral multicystic dysplastic kidney: long term outcomes. Arch Dis Child. 91(10):820-3, 2006
Siqueira Rabelo EA et al: Ultrasound progression of prenatally detected multicystic dysplastic kidney. Urology. 68(5):1098-102, 2006
Narchi H: Risk of Wilms' tumour with multicystic kidney disease: a systematic review. Arch Dis Child. 90(2):147-9, 2005
Kaneyama K et al: Associated urologic anomalies in children with solitary kidney. J Pediatr Surg. 39(1):85-7, 2004
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
Suzuki K et al: Segmental multicystic dysplastic kidney in an adult woman. Urol Int. 66(1):51-4, 2001
Snodgrass WT: Hypertension associated with multicystic dysplastic kidney in children. J Urol. 164(2):472-3;discussion 473-4, 2000
Srivastava T et al: Autosomal dominant inheritance of multicystic dysplastic kidney. Pediatr Nephrol. 13(6):481-3, 1999
John U et al: Kidney growth and renal function in unilateral multicystic dysplastic kidney disease. Pediatr Nephrol. 12(7):567-71, 1998
Kessler OJ et al: Involution rate of multicystic renal dysplasia. Pediatrics. 102(6):E73, 1998
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
Homsy YL et al: Wilms tumor and multicystic dysplastic kidney disease. J Urol. 158(6):2256-9; discussion 2259-60, 1997
Beckwith JB: Wilms tumor in multicystic dysplastic kidneys: What is the risk? Dialogues in Pediatric Urology. 19:3-5, 1996
Strife JL et al: Multicystic dysplastic kidney in children: US follow-up. Radiology. 186(3):785-8, 1993
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