Unilateral nephrectomy as palliative therapy in an infant with autosomal recessive polycystic kidney disease.

Abstract

Prenatal diagnosis of autosomal recessive polycystic kidney disease (ARPKD) implies a dire prognosis. Neonates affected by the more severe variants of ARPKD suffer respiratory failure caused by massive kidneys that restrict diaphragmatic expansion and result in pulmonary hypoplasia. Afflicted infants who survive the neonatal period and gain adequate respiratory function may subsequently suffer from an inability to tolerate enteral nutrition due to abdominal compression from the massive kidney and the systemic effects of renal compromise. Palliative unilateral or bilateral nephrectomy may be considered in rare instances to facilitate pulmonary expansion and gastrointestinal function. We report on an infant with severe ARPKD who was able to tolerate enteral nutrition only after left nephrectomy.