Xanthogranulomatous pyelonephritis in childhood: A rare but important clinical entity

Purpose: Xanthogranulomatous pyelonephritis (XGPN) is extremely rare in chi ldren. The authors review their experience of this condition. Methods: Medical records were investigated to conduct a retrospective study of 4 patients with XGPN (3 boys, 1 girl; age range, 2 months to 7 years) a t the authors' institute over the past 14 years. Results: Three of the 4 patients presented with fever of unknown origin and 1 with general fatigue. An abdominal mass was palpable in two cases at ini tial presentation. Although all patients had pyuria or hematuria, preoperat ive urine culture was positive in only 2 cases. Preoperative radiologic stu dies showed that 1 kidney was affected completely in 2 cases and affected p artially in 2 cases. Preoperatively, the provisional diagnosis was XGPN in 3 cases, and Wilms' tu mor in 1 case. Total nephrectomy was performed in 3 cases and enucleation in 1 case. XGPN was confirmed in all cases by histopa thologic studies, but the underlying disease could be identified only in 1 case (cystinuria), All patients did well postoperatively and have had no fu rther health problems over a mean follow-up period of 4.8 years, Conclusions: XGPN should be considered when there is a history of recurrent or therapy-resistant pyelonephritis. Preoperative radiologic investigation is paramount for diagnosis, and nephrectomy is the treatment of choice, al though partial resection or enucleation are adequate for partially affected kidneys. Copyright (C) 2000 by W.B. Saunders Company.