Xanthogranulomatous pyelonephritis in a child: A rare entity

Abstract

Xanthogranulomatous pyelonephritis (XGP) is chronic pyelonephritis uncommonly described in adults and is rare in children. It is characterized by the replacement of renal parenchymal tissue by lipomatous cells making it nonfunctional and may be mistaken for pyonephrosis, cystic or solid tumor, and the most commonly, Wilms tumor. It often presents as abdominal mass in children and more commonly involves left kidney. Imaging by computed tomography (CT) scan is characteristic, and histology is diagnostic. We are reporting a case of a child who presented with prolonged febrile illness and documented urinary tract infection with nephrolithiasis without any abdominal lump and received antibiotics for multiple times without any improvement. Ultrasonography was suggestive of pyonephrosis and multiple abscesses. However, on CT scan, was diagnosed as XGP of the right kidney which was confirmed on histology. After documenting, no function in affected kidney with other being normal, unilateral nephrectomy was done resulting in rapid symptomatic improvement.