Multicystic Dysplastic Kidney
- 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)
- 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
- 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
- Ureteropelvic junction (UPJ) obstruction
- Genital anomalies
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