Objectives: To review our experience with pediatric xanthogranulomatous pyelonephritis (XGPN) with special focus on surgical management.
Methods: From 1983 to 2007, 6 pediatric patients were found to harbor XGPN. The demographic data, mode of presentation, clinical and radiologic findings, laboratory profile, preoperative diagnosis, operative findings, and pathologic diagnosis of the removed specimen were recorded.
Results: The patients included 5 boys and 1 girl, with a mean age at presentation of 7.8 +/- 2 years. The symptoms included loin pain with or without anorexia or weight loss in all; however, a palpable mass was elicited in only 1 patient. All had pyuria on urinalysis, with positive cultures in 3. The 6 patients were anemic with normal kidney function. Abdominal ultrasonography revealed an enlarged kidney with multiple stones in 4 and a space-occupying lesion in 2 patients. Computed tomography was requested for 3 patients, and the findings raised the suspicion of Wilms tumor in 2. All 6 patients underwent nephrectomy. The lumbar approach was used for 3 patients with high complication rates. Laparoscopic nephrectomy was tried in 1 patient and was converted to open nephrectomy with a lumbar approach. The remaining 2 patients underwent exploration through a transperitoneal paramedian approach, with safer and easier surgical access. Pathologically, focal XGPN was diagnosed in only 1 patient, with the diffuse form diagnosed in 5 patients.
Conclusions: Magnifying the clinical awareness of XGPN in pediatric practice is fundamental to achieving the correct preoperative diagnosis. Nephrectomy through the paramedian transperitoneal approach is probably acceptable as the access route of choice, with a minimal complication rate.