Diffuse xanthogranulomatous pyelonephritis in a child with severe complications

Abstract

Xanthogranulomatous pyelonephritis is a rare disease in childhood. Because the symptoms and signs are chronic and non-specific, preoperative diagnosis is usually difficult. We report an 8-year-old boy who had an abdominal mass and anemia for more than 6 months. Fever and dyspnea occurred 4 days prior to admission. Ultrasonography revealed an enlarged right kidney with multiple parenchymal hypoechogenic areas, absence of normal parenchymal structures, and perinephric thickening with multiple calcifications. An abdominal computed tomogram demonstrated an irregular, enlarged right kidney with multiple low-density round areas consistent with hydronephrosis and calculi. Diminished excretion of contrast media and a severe perinephric inflammatory reaction were present. Poor right kidney function was demonstrated by Tc99m-diethylenetriamine penta-acetic acid split renal function examination. We diagnosed xanthogranulomatous pyelonephritis preoperatively based on the clinical and radiological features. The child first had drainage of an extrarenal abscess and antibiotic therapy, followed by definitive nephrectomy. The hospital course was complicated with pleural effusion, peritonitis, pelvic abscess, and sepsis. A two-stage nephrectomy requiring less radical resection and decreasing the surgical complications would have been preferable.

Acute retroperitoneal hemorrhage complicating a xanthogranulomatous pyelonephritis

“…XGP may [3]. XGP often closely mimics neoplastic and other renal parenchymal inflammatory diseases, which may result in misdiagnosis [1,3]. Several complications have been reported, such as psoas abscess, renocolic fistula, nephrobronchial fistula and necrotizing fasciitis [2].…”

“…The typical patient with XGP is a middleaged woman who presents with recurrent fever and flank pain. XGP may [3]. XGP often closely mimics neoplastic and other renal parenchymal inflammatory diseases, which may result in misdiagnosis [1,3].…”

Successfully treated bilateral xanthogranulomatous pyelonephritis in a child

“…This disease is characterized by progressing renal parenchymal damage, ill-defined margins with inflammatory infiltration of the perinephric fat, and focal inflammatory tissue destruction [1][2][3]. The hallmark of pathological alterations are foamy cells that are macrophages, and focal sclerosing soft-tissue calcification and microthrombosis may also be present.…”

“…The hallmark of pathological alterations are foamy cells that are macrophages, and focal sclerosing soft-tissue calcification and microthrombosis may also be present. Bilateral XPN is rather a rare exception [1,2]. The most common bacteria initiating renal destructive inflammation are gram-negative bacteria.…”

Duodenal Diverticulum Causing Xanthogranulomatous Pyelonephritis with Multiorgan Involvement: First Case Report

“…fat necrosis, or malnutrition; however, the exact mechanism remains unclear [2] . A review of the literature finds no other report of duodenal diverticulum involved in the process.…”