多嚢腎に対する治療方針

抄録

The multicystic kidney represents a severe form of dysplasia without any regular lobar development or normal caliceal drainage system. Surgeons generally have opted for nephrectomy, although the precise indications for removing these kidneys have been unclear, we assessed the effect of prenatal and postnatal diagnosis on the presentation of multicystic kidney, and documented the natural history of multicystic kidney that have been left in situ and reappraise the indications for nephrectomy. A total of 21 cases with multicystic kidney have been presented during 17 years in our institution. Of these cases 11 were referred for postnatal evaluation. Two bilateral cases had fatal outcome. Of remaining 19 infants with unilateral lesion, twelve infants have been managed conservatively and 7 underwent nephrectomy. Of the latter cases 5 infants who had no clinical symptoms underwent prophylactic nephrectomy in early part of thid study. The remaining 2, neonates underwent nephrectomy during the last 5 years, since it seemed that abdominal mass might be contributing to the failure to thrive. Anomalies of the contralateral. upper urinary tract included vesicoureteral reflux in 3, pelviureteric junction stenosis in 3, ureteral stenosis in 2, double ureter in 1 and horseshoe kidney in 1. Amang 12 children managed nonoperatively, there was no symptom except for cases with associated anomalies. None had malignant transformation or hypertension. Four patients were lost to follow up. Decrease in absolute dimensions of multicystic kidney was apparent in 6 patients. No change in absolute dimensions but relative decrease in relation to the growing child was noticed in 2 patients. We believe that a more conservative approach should be taken toware mujticystic kidney, unless other distinct reasons indicate the necessity of surgical removal. Careful lifelong surveillance of the patient is needed to detect the late complications.