Renal transplantation in children with oculorenal syndrome

The association of tapetoretinal degeneration with familial juvenile nephronophthisis is a rare oculorenal syndrome. Without treatment all children die of renal failure prior to adulthood. Information regarding renal transplantation is lacking in these children. This article reflects our experience in two children affected with this syndrome who achieved excellent functional results following living-related kidney transplants. Surgery can be done safely in these children with little or no morbidity. We believe that children with this syndrome benefit from transplantation and that this offers a significant improvement in their quality of life.