Follow-up of unilateral multicystic kidney dysplasia after prenatal diagnosis

Abstract

Objectives: Since the introduction of antenatal diagnostic screening, multicystic kidney dysplasia (MCKD) has been diagnosed in 1 of 4300 live births. We analyze our own experience and demonstratea management regime based on these results and existing studies.Methods: Retrospective data analysis was carried out in 110 patients, prenatally diagnosed with MCDK. A total of 93 patients withconfirmed diagnosis of unilateral MCDK were born alive and followed up in our institutions.Results: A total of 110 unilateral cystic kidneys were prenatally diagnosed; 93 were confirmed postnatally.These children were retrospectively allocated to two treatment groups: 51 were operated upon; 42 were treated conservatively. A micturition cystourethrogram was performed 88 times, yielding 20 pathologicalfindings. Vesicoureteral reflux was identified 11 times (12.5%). Eleven children (12%) had associated non-urological abnormalities. Cardiac and musculoskeletal malformations predominated. After 1995, nephrectomywas performed only when clinically indicated. Thirty-seven children were treated conservatively for between 4 months and 6 years (mean 33 months); 28 dysplastic kidneys exhibited size reduction. Completeinvolution was noted in 16 of 28 children between 7 and 29 months (mean 16.2 months).Conclusion: This study provides evidence that neonatal nephrectomy of unilateral MCDK is rarely required.It also shows that the discussed malformation is not an isolated developmental abnormality. In a high proportion, associated urogenital abnormalities were present and therapeutically relevant, and determinedthe overall prognosis.