Unilateral multicystic dysplastic kidney: does initial size matter?

BACKGROUND

We report the long-term follow-up of children with antenatally detected unilateral multicystic dysplastic kidney (MCDK) who were followed between 1985 and 2009.

METHODS

Involution rates were documented over time based on the initial size of the MCDK, as documented on postnatal ultrasound (USS), as well as long-term complications and renal function.

RESULTS

In 323 patients (182 male), 10 % of MCDK had involuted, as evidenced on the first postnatal USS, with survival function analysis showing the probability of complete involution to be 35 % in 249 patients by 2 years of age, 47 % in 180 patients by 5 years of age and 62 % in 94 patients by 10 years of age. There was a significant difference in the involution rates of MCKD at the 10-year follow-up between MCDK with an initial size of >5 cm versus MCDK with an initial size of ≤5 cm (p < 0.0001). No patients in the whole cohort developed sustained hypertension or malignancy during a median follow-up of 10.1 years (range 0.3-15.4 years). Median estimated glomerular filtration rate (eGFR) in 76 patients (7 at 5 years, 69 at 10 years) was 93 ml/min/1.73 m(2) (range 46-175 ml/min/1.73 m(2)), with 40 (53%) having an eGFR of between 90 and 140 ml/min/1.73 m(2). Twenty-three (30 %) of the 76 patients at 10 years had normal eGFR (90-140 ml/min/1.73 m(2)) as well as complete involution of the MCDK, compensatory hypertrophy of the contralateral kidney, no proteinuria and no hypertension.

CONCLUSIONS

Larger MCDK at birth are less likely to involute during the first decade of life. However, conservative management remains justified due to the lack of complications. A minority of patients fulfil current criteria for discharge from specialty follow-up at 10 years.