Xanthogranulomatous pyelonephritis in childhood: A critical analysis of 10 cases and of the literature

OBJECTIVES To report our experience with 10 cases of pediatric xanthogranulomatous pyelonephritis and review the literature regarding this relatively uncommon disease. METHODS The records of 10 patients presenting to our institution with xanthogranulomatous pyelonephritis between 1996 and 2006 were examined. The mode of presentation, clinical and radiologic findings, blood count, blood chemistry, Urine Culture results, operative findings, and histologic findings of the affected kidney were recorded. RESULTS Staging was performed according to the classification of Malek and Elder. Through radiologic and operative investigations our patients were classified as Stage I (n = 1), Stage 2 (n = 6), and Stage 3 (n = 3). Simple nephrectomy was performed in all 10 patients. CONCLUSIONS Previously the diagnosis of xanthogranulomatous pyelonephritis was normally based on pathologic assessment, most commonly after nephrectomy. During the last decade, however, increasingly sensitive radiologic investigations (sonography, four-phase computed tomography, and magnetic resonance imaging) in combination with clinical suspicion have made its preoperative diagnosis possible. Surgical intervention still remains the cornerstone of treatment for this relatively uncommon disease.