Multicystic Dysplastic Kidney: What Changed in Three Decades?

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DOI:

https://doi.org/10.71749/pkj.44

Keywords:

Child, Multicystic Dysplastic Kidney/complications, Multicystic Dysplastic Kidney/diagnosis

Abstract

Introduction: Multicystic dysplastic kidney (MCDK) is a severe form of cystic renal dysplasia that typically exhibits a favorable natural history. Though complications are low, systematic follow‐up is needed.
Methods: Retrospective analysis covering children diagnosed with MCDK, undergoing follow‐up in a tertiary pediatric nephrology unit, from January/2011‐December/2023. Variables assessed included demographics, diagnosis details, evolution and treatment. A comparison with prior studies (1989‐2000 and 2000‐2010) was also conducted.
Results: Were included 53 children, most with prenatal diagnosis. Left kidney was most often involved, and additional kidney abnormalities were present in 39.6%. Ultrasound was conducted in all patients, and at least one nuclear med‐ icine test was conducted in 94.3%. Involution of the affected kidney occurred in 96.2%, with complete involution in 49.1%. Complications, including urinary tract infections, were reported in 34.0%. Compared with the previous study periods, the latest period was notable for higher percentage of prenatal diagnosis, fewer voiding cystourethrographies and nephrectomies performed, and higher percentage of contralateral anomalies and complications.
Discussion: MCKD was predominantly diagnosed prenatally. The incidence of associated anomalies aligns with previous findings. Antenatal ultrasonography is the diagnostic method of choice. Our study demonstrated the typical natural his‐ tory of the disease, with high partial and complete involution rates. Management trends shifted from surgery to conser‐ vative approaches, reflecting the improved understanding of MCDK’s favorable prognosis. Long‐term follow‐up, typically less invasive in recent years, remains crucial especially for patients with contralateral abnormalities, emphasizing serial ultrasonographic evaluation, and frequent monitoring of blood pressure, proteinuria and kidney function markers.

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References

Niaudet P. Renal cystic diseases in children. Mattoo TK, Hoppin AG, editors. [Internet]. 2024 [cited 2024 July 10]. Available from: https://www.uptodate.com/contents/renal-cystic-diseases-in-children

Rosenblum ND. Overview of congenital anomalies of the kidney and urinary tract (CAKUT)Mattoo TK, Baskin LS, Hoppin AG, editors. [Internet]. 2024 [cited 2024 July 10]. Available from: https://www.uptodate.com/contents/overview-of-congenital-anomalies-of-the-kidney-and-urinary-tract-cakut

Cardona-Grau D, Kogan BA. Update on multicystic dysplastic kidney. Curr Urol Rep. 2015 Aug 9;16(10). doi:10.1007/s11934-015-0541-7

Chetty S. Multicystic dysplastic kidney. Am J Obstet Gynecol. 2021 Nov; 225(5):B21–2. doi:10.1016/j.ajog.2021.06.046

Kara A, Gurgoze MK, Aydin M, Koc ZP. Clinical features of children with multicystic dysplastic kidney. Pediatr Int. 2018 Jul 31;60(8):750–4. doi:10.1111/ped.13612

Xi Q, Zhu X, Wang Y, Ru T, Dai C, Wang Z, et al. Copy number variations in multicystic dysplastic kidney: Update for prenatal diagnosis and genetic counseling. Prenat Diagn. 2016 Apr 2;36(5):463–8. doi:10.1002/pd.4807

Cambio AJ, Evans CP, Kurzrock EA. Non‐surgical management of multicystic dysplastic kidney. BJU Int. 2008 Jan 8;101(7):804–8. doi:10.1111/j.1464-410x.2007.07328.x

Narchi H. Risk of hypertension with multicystic kidney disease: A systematic review. Arch Dis Child. 2005 May 4;90(9):921–4. doi:10.1136/adc.2005.075333

Mansoor O, Chandar J, Rodriguez MM, Abitbol CL, Seeherunvong W, Freundlich M, et al. Long-term risk of chronic kidney disease in unilateral multicystic dysplastic kidney. Pediatr Nephrol. 2011 Jan 15;26(4):597–603. doi:10.1007/s00467-010-1746-0

Gaither TW, Patel A, Patel C, Chuang K, Cohen RA, Baskin LS. Natural history of contralateral hypertrophy in patients with multicystic dysplastic kidneys. J Urol. 2018 Jan 1;199(1):280–6. doi:10.1016/j.juro.2017.06.075

Rios H, Santos R, Gomes C, Correia AJ. Multicystic dysplastic kidney: a review of eleven years (2000 – 2010). Portuguese Journal of Nephrology and Hypertension. 2012 Feb 14;26(1):41–6.

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Published

29-10-2024

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Multicystic Dysplastic Kidney: What Changed in Three Decades?. (2024). Portuguese Kidney Journal. https://doi.org/10.71749/pkj.44

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