Neonatal multicystic dysplastic kidney with mass effect: A systematic review

Published:September 11, 2021DOI:



      Multicystic Dysplastic Kidney (MCDK) is a common cause of palpable abdominal mass in a neonate, yet reports are few of resultant life-threatening extrinsic compression. This systematic review is the first to review all known neonatal MCDK cases complicated by external compression due to mass effect. Our aim is to foster communication about these unusual cases to inform management of future comparable cases.


      This systematic review followed Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Medical Subject Headings (MeSH) were used to search PubMed through June 8, 2021 as follows: ((((Polycystic Kidney Diseases/complications [MeSH Terms]) OR (Multicystic Dysplastic Kidney/complications [MeSH Terms])) AND (Humans [MeSH Terms]) AND (Infant, Newborn [MeSH Terms]) AND (Case Reports [Publication Type]) AND (("multicystic dysplastic kidney") OR ("multicystic kidney")))) OR ((unusual respiratory distress in newborn [Title]) AND (kidney)) OR (large MCDK). Resulting papers were screened and included if they reported neonatal MCDK complicated by external compression by mass effect. Excluded papers lacked an MCDK diagnosis, a neonatal patient, or clear indication of mass effect. Salient data was extracted from each case for comparison.


      Of 51 papers screened, seven met inclusion criteria, presenting eight neonatal MCDK patients exhibiting varied combinations of symptoms secondary to external compression, including respiratory distress, gastrointestinal obstruction, and contralateral ureteral obstruction. All eight cases, ultimately managed with neonatal nephrectomy, had positive outcomes. Unreported and undiagnosed cases are not included in this review, making it susceptible to publication bias.


      Large neonatal MCDK is typically managed conservatively, however it can be complicated by external compression due to mass effect. In this scenario, nephrectomy is a reasonable and definitive treatment. Less invasive management options may exist, e.g., aspiration with sclerotherapy. Communication about exceptional cases like these should be encouraged even when outcomes are poor.



      MCDK (Multicystic Dysplastic Kidney), PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses), MeSH (Medical Subject Headings)
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic and Personal
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Journal of Pediatric Urology
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Kumar M.
        • Gathwala G.
        • Ratttan K.
        • Lather S.
        • Dalal P.
        Congenital giant dysplastic kidney presenting as respiratory distress: a case report.
        J Neonatal Surg. 2017; 6
        • K E.
        • H J.
        • P V.
        • B J.
        Segmental multicystic dysplastic kidney in children.
        Urology. 2006; 68: e9-e11
        • Siqueira Rabelo E.A.
        • Oliveira E.A.
        • Silva J.M.P.
        • Oliveira D.S.
        • Colosimo E.A.
        Ultrasound progression of prenatally detected multicystic dysplastic kidney.
        Urology. 2006; 68: 1098-1102
        • Chang A.
        • Sivananthan D.
        • Nataraja R.M.
        • Johnstone L.
        • Webb N.
        • Lopez P.-J.
        Evidence-based treatment of multicystic dysplastic kidney: a systematic review.
        J Pediatr Urol. 2018; 14: 510-519
        • Hains D.S.
        • Bates C.M.
        • Ingraham S.
        • Schwaderer A.L.
        Management and etiology of the unilateral multicystic dysplastic kidney: a review.
        Pediatr Nephrol Berl Ger. 2009; 24: 233-241
        • Eickmeyer A.B.
        • Casanova N.F.
        • He C.
        • Smith E.A.
        • Wan J.
        • Bloom D.A.
        • et al.
        The natural history of the multicystic dysplastic kidney--is limited follow-up warranted?.
        J Pediatr Urol. 2014; 10: 655-661
        • Erlich T.
        • Lipsky A.M.
        • Braga L.H.
        A meta-analysis of the incidence and fate of contralateral vesicoureteral reflux in unilateral multicystic dysplastic kidney.
        J Pediatr Urol. 2019; 15: 77.e1-77.e7
        • Lim F.-F.
        • Tsao T.-F.
        • Chang H.-M.
        • Sheu J.-N.
        Multicystic dysplastic kidney disease presenting with a single large cyst in a fetus-anatomical basis and radiological aspects.
        Pediatr Neonatol. 2011; 52: 227-231
        • Wise H.A.
        • Schirmer H.K.
        An unusual cause of respiratory distress in a newborn.
        J Urol. 1972; 108: 792-793
        • Pettit S.M.
        • Devan W.J.
        • Chalmers D.J.
        • Zanno A.
        Multicystic dysplastic kidney with mass effect in a neonate treated with nephrectomy: case report.
        Urology. 2020;
        • Triest Jeffrey A.
        • Bukowski Timothy P.
        Multicystic dysplastic kidney as cause of gastric outlet obstruction and respiratory compromise.
        J Urol. 1999; 161: 1918-1919
        • Middleton A.W.
        • Melzer R.B.
        Neonatal multicystic kidney with associated respiratory distress, obstruction of contralateral ureter, and gastric compromise.
        Urology. 1989; 34: 36-38
        • Porterfield G.N.
        • Campbell D.P.
        Duodenal obstruction in a newborn secondary to a multicystic kidney.
        J Pediatr Surg. 1977; 12: 767-769
        • Komura M.
        • Kanamori Y.
        • Sugiyama M.
        • Nakahara S.
        • Kawashima H.
        • Hatanaka A.
        • et al.
        Antenatally diagnosed giant multicystic dysplastic kidney resected during the neonatal period.
        J Pediatr Surg. 2008; 43: 2118-2120
        • Hayes W.N.
        • Watson A.R.
        • Trent & Anglia MCDK Study Group
        Unilateral multicystic dysplastic kidney: does initial size matter?.
        Pediatr Nephrol. 2012; 27: 1335-1340
        • Yadav P.
        • Sureka S.K.
        • Ansari M.S.
        • Soni R.
        • Lal H.
        Large multicystic dysplastic kidney mimicking a large cystic renal neoplasm.
        J Clin Diagn Res JCDR. 2017; 11: PD03-PD04
        • Maffi M.
        • Lima M.
        Multicystic dysplastic kidney.
        in: Lima M. Reinberg O. Neonatal surg. contemp. strateg. fetal life first year age. Springer International Publishing, Cham2019: 527-531
        • Paananen I.
        • Hellström P.
        • Leinonen S.
        • Merikanto J.
        • Perälä J.
        • Päivänsalo M.
        • et al.
        Treatment of renal cysts with single-session percutaneous drainage and ethanol sclerotherapy: long-term outcome.
        Urology. 2001; 57: 30-33
        • Ali T.A.
        • Abdelaal M.A.
        • Enite A.
        • Badran Y.A.
        Ultrasound-guided percutaneous sclerotherapy of simple renal cysts with n-butyl cyanoacrylate and iodized oil mixture as an outpatient procedure.
        Urol Ann. 2016; 8: 51-55
        • Hanna R.M.
        • Dahniya M.H.
        Aspiration and sclerotherapy of symptomatic simple renal cysts: value of two injections of a sclerosing agent.
        AJR Am J Roentgenol. 1996; 167: 781-783
        • Brown D.
        • Nalagatla S.
        • Stonier T.
        • Tsampoukas G.
        • Al-Ansari A.
        • Amer T.
        • et al.
        Radiologically guided percutaneous aspiration and sclerotherapy of symptomatic simple renal cysts: a systematic review of outcomes.
        Abdom Radiol. 2021;