Multicystic renal dysplasia diagnosed in the antenatal period: a note of caution

Abstract

To document the urological problems associated with multicystic renal dysplasia (MCRD), to assess the efficacy of radiological methods for following the natural history of MCRD and to review current experience in the light of historical data regarding this condition. From January 1982 to December 1992 data were obtained prospectively on 62 patients (41 boys and 21 girls, age range 1-11 years) who had a diagnosis of MCRD suspected on antenatal ultrasonography. All patients had the diagnosis confirmed with radioisotope scans and micturating cystograms. Serum creatinine was measured in each patient and repeated clinically as indicated. Follow-up was from 1 to 11 years, with a clinical review and an annual ultrasonogram. The natural history of these patients was compared with those in other published studies. Of the 62 patients, 12 had associated vesicoureteric reflux. Four patients developed impaired renal function during the course of observation and 10 patients required urological surgery for other pathologies. In the patients managed conservatively, 38% underwent no resolution on sequential ultrasonograms during the 2-year follow-up, and in four children, where complete resolution of the MCRD had occurred on ultrasonography, significant cellular renal masses were excised at subsequent surgery. Patients with MCRD have significant associated urological malformations and the natural history in such patients is unpredictable. We do not regard MCRD as an entirely benign condition and feel that all patients require appropriate investigation of their urinary tract and long-term follow-up where conservative treatment is instituted. From the natural history of these patients, reviewed in conjunction with the literature, we recommend that decisions for management can only be taken with the full informed consent of the parents and that surgical excision is offered as part of that process.