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Multicystic Dysplastic Kidney (MCDK)

Definition of multicystic dysplastic kidney

Multicystic dysplastic kidney (MCDK) is a congenital malformation of the kidney characterized by multiple noncommunicating cysts of varying size, dysplastic renal parenchyma, and absent or only minimal function of the affected kidney (Hains et al., 2009).

Epidemiology:

Incidence: 1 per 4,300 live births.

Etiology (causes) of multicystic dysplastic kidney

The cause is unclear; a disturbed interaction between the ureteric bud and the metanephric blastema during embryonic development is suspected. Most cases of multicystic dysplastic kidney undergo spontaneous involution.

Symptoms (clinical presentation) of multicystic dysplastic kidney

Unilateral multicystic dysplastic kidney is usually asymptomatic and is predominantly diagnosed by prenatal or postnatal ultrasonography. Occasionally, MCDK may present as a palpable abdominal mass. During further follow-up, spontaneous involution is typical. Associated malformations of the contralateral kidney such as ureteropelvic junction obstruction (3–12%) or contralateral vesicoureteral reflux (5–20%) are possible. In bilateral multicystic dysplastic kidney, the prognosis is very poor because of absent renal function and frequently associated oligohydramnios, with Potter sequence and neonatal kidney failure.

Diagnosis of multicystic dysplastic kidney

Ultrasonography:

Cystic mass in the renal fossa.

VCUG:

In cases of suspected vesicoureteral reflux of the contralateral solitary functioning kidney.

Renal scintigraphy:

No uptake on the affected side. This examination is indicated only in the presence of ectasia of the contralateral solitary kidney.

Treatment of multicystic dysplastic kidney

Regular imaging (renal ultrasonography), blood pressure measurement, and serum creatinine assessment depending on the function of the contralateral kidney. In the presence of symptoms (flank pain, infections, hypertension) or suspected malignancy, surgical removal of the cystic mass is necessary.

References

Hains, D. S.; Bates, C. M.; Ingraham, S. & Schwaderer, A. L. Management and etiology of the unilateral multicystic dysplastic kidney: a review.
Pediatr Nephrol, 2009, 24, 233-241.

  Deutsche Version: Multizystische Nierendysplasie

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