Four cases of diffuse xanthogranulomatous pyelonephritis (XPN) in young children are presented. In three patients the clinical picture was one of weight loss, anaemia and neutrophilia with a large renal mass. The fourth presented with haematuria. Ultrasound (US) and CT findings were almost identical in all four patients. US showed the affected kidney was massively enlarged but retained a reniform shape. Dilated fluid spaces containing calculi were present. CT confirmed the US findings and revealed peripheral enhancement without contrast excretion, with dilated calyceal spaces producing the "bear paw sign". Extrarenal extension into abdominal wall and psoas muscle is typical and was well demonstrated by CT. The affected kidneys were non-functioning and nephrectomy was required. Typical US and CT features allow a confident diagnosis of XPN and appropriate early treatment.
Child, Preschool Humans