Abstract
In children, unilateral multicystic dysplastic kidney (MCDK) is one of the most frequently identified urinary tract abnormalities. A variety of proposed etiologies has been associated with the underlying pathogenesis of MCDK. These include genetic disturbances, teratogens, in utero infections, and urinary outflow tract obstruction. From 5–43% of the time, MCDK has associated genito-urinary anomalies, both structural and functional in nature. A review of the literature reveals that involution rates are reported to be 19–73%, compensatory hypertrophy of the contralateral kidney occurs from 24–81% of the time, and estimated glomerular filtration rates (GFRs) (by the Schwartz formula) range from 86–122 ml/min per 1.73 m2 body surface area. Most authors suggest serial ultrasonography to monitor contralateral growth, routine blood pressure monitoring, and a serum creatinine monitoring algorithm. The risk of hypertension in those with MCDKs does not appear to be greater than that of the general population, and the rates of malignant transformation of MCDK are small, if at all increased, in comparison with those in the general population. If the patient develops a urinary tract infection or has abnormalities of the contralateral kidney, shown on ultrasound, a voiding cystourethrogram is recommended. Finally, the body of literature does not support the routine surgical removal of MCDKs.
Similar content being viewed by others
References
Feldenberg LR, Siegel NJ (2000) Clinical course and outcome for children with multicystic dysplastic kidneys. Pediatr Nephrol 14:1098–1101
James CA, Watson AR, Twining P, Rance CH (1998) Antenatally detected urinary tract abnormalities: changing incidence and management. Eur J Pediatr 157:508–511
Kohaut EC, Tejani A (1996) The 1994 annual report of the North American Pediatric Renal Transplant Cooperative Study. Pediatr Nephrol 10:422–434
Rudnik-Schoneborn S, John U, Deget F, Ehrich JH, Misselwitz J, Zerres K (1998) Clinical features of unilateral multicystic renal dysplasia in children. Eur J Pediatr 157:666–672
Mackie GG, Stephens FD (1975) Duplex kidneys: a correlation of renal dysplasia with position of the ureteral orifice. J Urol 114:274–280
Batourina E, Tsai S, Lambert S, Sprenkle P, Viana R, Dutta S, Hensle T, Wang F, Niederreither K, McMahon AP, Carroll TJ, Mendelsohn CL (2005) Apoptosis induced by vitamin A signaling is crucial for connecting the ureters to the bladder. Nat Genet 37:1082–1089
Viana R, Batourina E, Huang H, Dressler GR, Kobayashi A, Behringer RR, Shapiro E, Hensle T, Lambert S, Mendelsohn C (2007) The development of the bladder trigone, the center of the anti-reflux mechanism. Development 134:3763–3769
Belk RA, Thomas DF, Mueller RF, Godbole P, Markham AF, Weston MJ (2002) A family study and the natural history of prenatally detected unilateral multicystic dysplastic kidney. J Urol 167:666–669
Konig R, Fuchs S, Dukiet C (1994) Branchio-oto-renal (BOR) syndrome: variable expressivity in a five-generation pedigree. Eur J Pediatr 153:446–450
Murer L, Benetti E, Artifoni L (2007) Embryology and genetics of primary vesico-ureteric reflux and associated renal dysplasia. Pediatr Nephrol 22:788–797
Murawski IJ, Gupta IR (2006) Vesicoureteric reflux and renal malformations: a developmental problem. Clin Genet 69:105–117
Murawski IJ, Myburgh DB, Favor J, Gupta IR (2007) Vesico-ureteric reflux and urinary tract development in the Pax2 1Neu+/- mouse. Am J Physiol Renal Physiol 293:F1736–F1745
Fletcher J, Hu M, Berman Y, Collins F, Grigg J, McIver M, Juppner H, Alexander SI (2005) Multicystic dysplastic kidney and variable phenotype in a family with a novel deletion mutation of PAX2. J Am Soc Nephrol 16:2754–2761
Petrikovsky BM, Lipson SM, Kaplan MH (2003) Viral studies on amniotic fluid from fetuses with and without abnormalities detected by prenatal sonography. J Reprod Med 48:230–232
Chan M, Hecht JL, Boyd T, Rosen S (2007) Congenital cytomegalovirus infection: a cause of renal dysplasia? Pediatr Dev Pathol 10:300–304
Carta M, Cimador M, Giuffre M, Sergio M, Di Pace MR, De Grazia E, Corsello G (2007) Unilateral multicystic dysplastic kidney in infants exposed to antiepileptic drugs during pregnancy. Pediatr Nephrol 22:1054–1057
Woolf AS (1997) Multiple causes of human kidney malformations. Arch Dis Child 77:471–473
Peters CA, Carr MC, Lais A, Retik AB, Mandell J (1992) The response of the fetal kidney to obstruction. J Urol 148:503–509
Shibata S, Nagata M (2003) Pathogenesis of human renal dysplasia: an alternative scenario to the major theories. Pediatr Int 45:605–609
Miyazaki Y, Ichikawa I (2003) Ontogeny of congenital anomalies of the kidney and urinary tract, CAKUT. Pediatr Int 45:598–604
Aslam M, Watson AR (2006) Unilateral multicystic dysplastic kidney: long term outcomes. Arch Dis Child 91:820–823
Wacksman J, Phipps L (1993) Report of the Multicystic Kidney Registry: preliminary findings. J Urol 150:1870–1872
Kaneyama K, Yamataka A, Satake S, Yanai T, Lane GJ, Kaneko K, Yamashiro Y, Miyano T (2004) Associated urologic anomalies in children with solitary kidney. J Pediatr Surg 39:85–87
Kessler OJ, Ziv N, Livne PM, Merlob P (1998) Involution rate of multicystic renal dysplasia. Pediatrics 102:E73
Kuwertz-Broeking E, Brinkmann OA, Von Lengerke HJ, Sciuk J, Fruend S, Bulla M, Harms E, Hertle L (2004) Unilateral multicystic dysplastic kidney: experience in children. BJU Int 93:388–392
Menster M, Mahan J, Koff S (1994) Multicystic dysplastic kidney. Pediatr Nephrol 8:113–115
Miller DC, Rumohr JA, Dunn RL, Bloom DA, Park JM (2004) What is the fate of the refluxing contralateral kidney in children with multicystic dysplastic kidney? J Urol 172:1630–1634
Onal B, Kogan BA (2006) Natural history of patients with multicystic dysplastic kidney—what followup is needed? J Urol 176:1607–1611
Rahman RC, Amoreo O (2005) Multicystic dysplastic kidney: diagnosis and evolution. Pediatr Nephrol 20:1023
John U, Rudnik-Schoneborn S, Zerres K, Misselwitz J (1998) Kidney growth and renal function in unilateral multicystic dysplastic kidney disease. Pediatr Nephrol 12:567–571
Merrot T, Lumenta DB, Tercier S, Morisson-Lacombes G, Guys JM, Alessandrini P (2006) Multicystic dysplastic kidney with ipsilateral abnormalities of genitourinary tract: experience in children. Urology 67:603–607
Rabelo EA, Oliveira EA, Diniz JS, Silva JM, Filgueiras MT, Pezzuti IL, Tatsuo ES (2004) Natural history of multicystic kidney conservatively managed: a prospective study. Pediatr Nephrol 19:1102–1107
Rabelo EA, Oliveira EA, Silva GS, Pezzuti IL, Tatsuo ES (2005) Predictive factors of ultrasonographic involution of prenatally detected multicystic dysplastic kidney. BJU Int 95:868–871
Siqueira Rabelo EA, Oliveira EA, Silva JM, Oliveira DS, Colosimo EA (2006) Ultrasound progression of prenatally detected multicystic dysplastic kidney. Urology 68:1098–1102
Ylinen E, Ahonen S, Ala-Houhala M, Wikstrom S (2004) Nephrectomy for multicystic dysplastic kidney: if and when? Urology 63:768–771; discussion 771–762
Abidari JM, Park KH, Kennedy WA, Shortliffe LD (2002) Serial followup of the contralateral renal size in children with multicystic dysplastic kidney. J Urol 168:1821–1825 discussion 1825
Vu KH, Van Dyck M, Daniels H, Proesmans W (2007) Renal outcome of children with one functioning kidney from birth. A study of 99 patients and a review of the literature. Eur J Pediatr DOI https://doi.org/10.1007/s00431-007-0612-y
Mei-Zahav M, Korzets Z, Cohen I, Kessler O, Rathaus V, Wolach B, Pomeranz A (2001) Ambulatory blood pressure monitoring in children with a solitary kidney—a comparison between unilateral renal agenesis and uninephrectomy. Blood Press Monit 6:263–267
Wikstad I, Celsi G, Larsson L, Herin P, Aperia A (1988) Kidney function in adults born with unilateral renal agenesis or nephrectomized in childhood. Pediatr Nephrol 2:177–182
Konda R, Sato H, Ito S, Sakai K, Kimura N, Nagura H (2001) Renin containing cells are present predominantly in scarred areas but not in dysplastic regions in multicystic dysplastic kidney. J Urol 166:1910–1914
Oliveira EA, Silva AC, Rabelo EA, Filgueiras FF, Pereira AK, Mesquita FM (2002) Spontaneous improvement of hypertension in multicystic dysplastic kidney: a case report. Pediatr Nephrol 17:954–958
Javadpour N, Chelouhy E, Moncada L, Rosenthal IM, Bush IM (1970) Hypertension in a child caused by a multicystic kidney. J Urol 104:918–921
Angermeier KW, Kay R, Levin H (1992) Hypertension as a complication of multicystic dysplastic kidney. Urology 39:55–58
Chen YH, Stapleton FB, Roy S 3rd, Noe HN (1985) Neonatal hypertension from a unilateral multicystic dysplastic kidney. J Urol 133:664–665
Husmann DA (1998) Renal dysplasia: the risks and consequences of leaving dysplastic tissue in situ. Urology 52:533–536
Snodgrass WT (2000) Hypertension associated with multicystic dysplastic kidney in children. J Urol 164:472–473; discussion 473–474
Pardo de la Vega R, Herrero Morin D, Ordonez Alvarez FA, Martinez Suarez V, Santos Rodriguez F, Malaga Guerrero S (2003) Ambulatory blood pressure monitoring in multicystic dysplastic kidney disease. An Pediatr (Barc) 58:545–549
Narchi H (2005) Risk of hypertension with multicystic kidney disease: a systematic review. Arch Dis Child 90:921–924
Selzman AA, Elder JS (1995) Contralateral vesicoureteral reflux in children with a multicystic kidney. J Urol 153:1252–1254
Birken G, King D, Vane D, Lloyd T (1985) Renal cell carcinoma arising in a multicystic dysplastic kidney. J Pediatr Surg 20:619–621
Hartman GE, Smolik LM, Shochat SJ (1986) The dilemma of the multicystic dysplastic kidney. Am J Dis Child 140:925–928
Webb NJ, Lewis MA, Bruce J, Gough DC, Ladusans EJ, Thomson AP, Postlethwaite RJ (1997) Unilateral multicystic dysplastic kidney: the case for nephrectomy. Arch Dis Child 76:31–34
Homsy YL, Anderson JH, Oudjhane K, Russo P (1997) Wilms tumor and multicystic dysplastic kidney disease. J Urol 158:2256–2259; discussion 2259–2260
Narchi H (2005) Risk of Wilms’ tumour with multicystic kidney disease: a systematic review. Arch Dis Child 90:147–149
Cambio AJ, Evans CP, Kurzrock EA (2008) Non-surgical management of multicystic dysplastic kidney. BJU Int 101:804–808
Mingin GC, Gilhooly P, Sadeghi-Nejad H (2000) Transitional cell carcinoma in a multicystic dysplastic kidney. J Urol 163:544
Rackley RR, Angermeier KW, Levin H, Pontes JE, Kay R (1994) Renal cell carcinoma arising in a regressed multicystic dysplastic kidney. J Urol 152:1543–1545
Oliveira EA, Diniz JS, Vilasboas AS, Rabelo EA, Silva JM, Filgueiras MT (2001) Multicystic dysplastic kidney detected by fetal sonography: conservative management and follow-up. Pediatr Surg Int 17:54–57
van Eijk L, Cohen-Overbeek TE, den Hollander NS, Nijman JM, Wladimiroff JW (2002) Unilateral multicystic dysplastic kidney: a combined pre- and postnatal assessment. Ultrasound Obstet Gynecol 19:180–183
Avni FE, Garel L, Cassart M, Massez A, Eurin D, Didier F, Hall M, Teele RL (2006) Perinatal assessment of hereditary cystic renal diseases: the contribution of sonography. Pediatr Radiol 36:405–414
Singh I, Sharma D, Singh N, Jain BK, Minocha VR (2002) Hydronephrotic obstructed kidney mimicking a congenital multicystic kidney: case report with review of literature. Int Urol Nephrol 34:179–182
Woodward M, Frank D (2002) Postnatal management of antenatal hydronephrosis. BJU Int 89:149–156
Choyke PL, Siegel MJ, Craft AW, Green DM, DeBaun MR (1999) Screening for Wilms tumor in children with Beckwith-Wiedemann syndrome or idiopathic hemihypertrophy. Med Pediatr Oncol 32:196–200
Ismaili K, Avni FE, Alexander M, Schulman C, Collier F, Hall M (2005) Routine voiding cystourethrography is of no value in neonates with unilateral multicystic dysplastic kidney. J Pediatr 146:759–763
Flack CE, Bellinger MF (1993) The multicystic dysplastic kidney and contralateral vesicoureteral reflux: protection of the solitary kidney. J Urol 150:1873–1874
McMann LP, Kirsch AJ, Scherz HC, Smith EA, Jones RA, Shehata BM, Kozielski R, Grattan-Smith JD (2006) Magnetic resonance urography in the evaluation of prenatally diagnosed hydronephrosis and renal dysgenesis. J Urol 176:1786–1792
Rodriguez LV, Spielman D, Herfkens RJ, Shortliffe LD (2001) Magnetic resonance imaging for the evaluation of hydronephrosis, reflux and renal scarring in children. J Urol 166:1023–1027
National High Blood Pressure Education Program Working Group on High Blood Pressure in Children and Adolescents (2004) The fourth report on the diagnosis, evaluation, and treatment of high blood pressure in children and adolescents. Pediatrics 114:555–576
Kaneko K, Yamashiro Y, Yamataka A, Miyano T (2005) Nephrectomy for multicystic dysplastic kidneys: a new therapeutic option. Pediatr Nephrol 20:690–691
Steven LC, Li AG, Driver CP, Mahomed AA (2005) Laparoscopic nephrectomy for unilateral multicystic dysplastic kidney in children. Surg Endosc 19:1135–1138
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Hains, D.S., Bates, C.M., Ingraham, S. et al. Management and etiology of the unilateral multicystic dysplastic kidney: a review. Pediatr Nephrol 24, 233–241 (2009). https://doi.org/10.1007/s00467-008-0828-8
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00467-008-0828-8