Multicystic Dysplastic Kidney
Basics
Description
- Multicystic dysplastic kidney (MCDK) disease is the most severe type of cystic renal dysplasia and is characterized by multiple, noncommunicating cysts which are divided by dysplastic tissue.
- In general, there is no functioning renal tissue in a MCDK.
- Involutes over time (usually within the first 5 years)
Epidemiology
- Incidence is 0.3 to 1 in 1,000 live births
- Most cases are detected antenatally.
- Affects boys > girls
- Left kidney > right kidney
- Usually unilateral but can be bilateral
Pathophysiology
- Initial growth of the ureteric bud is normal; then renal development halts at a later stage.
- Histology shows disordered renal tissue with areas of undifferentiated mesenchymal cells, abnormal differentiation (i.e., cartilage), rare nephrons.
- Etiology unknown; recent studies suggest genetic cause.
Commonly Associated Conditions
Associated anomalies of contralateral GU tract include the following:
- Vesicoureteral reflux (VUR), most common, occurs in ~25%.
- Renal hypoplasia
- Ureterocele
- Ureteropelvic junction (UPJ) obstruction
- Genital anomalies
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Citation
Cabana, Michael D., editor. "Multicystic Dysplastic Kidney." 5-Minute Pediatric Consult, 8th ed., Wolters Kluwer, 2019. Pediatrics Central, peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/618136/all/Multicystic_Dysplastic_Kidney.
Multicystic Dysplastic Kidney. In: Cabana MDM, ed. 5-Minute Pediatric Consult. Wolters Kluwer; 2019. https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/618136/all/Multicystic_Dysplastic_Kidney. Accessed November 4, 2024.
Multicystic Dysplastic Kidney. (2019). In Cabana, M. D. (Ed.), 5-Minute Pediatric Consult (8th ed.). Wolters Kluwer. https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/618136/all/Multicystic_Dysplastic_Kidney
Multicystic Dysplastic Kidney [Internet]. In: Cabana MDM, editors. 5-Minute Pediatric Consult. Wolters Kluwer; 2019. [cited 2024 November 04]. Available from: https://peds.unboundmedicine.com/pedscentral/view/5-Minute-Pediatric-Consult/618136/all/Multicystic_Dysplastic_Kidney.
* Article titles in AMA citation format should be in sentence-case
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T1 - Multicystic Dysplastic Kidney
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ED - Cabana,Michael D,
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