Describe the findings of contrast-enhanced CT of the abdomen.
Contrast-enhanced CT (coronal view, Fig. 1a) shows left kidney is enlarged with moderate left hydronephrosis (6-point star) and multiple coarse calcified foci of varying sizes, which are seen scattered within the cortex of the left kidney (thick arrows). Small peripheral enhancing collection is seen at the left paracolic gutter which is seen extending to the lower pole of the left kidney. In the sagittal view (Fig. 1b), perinephric and paranephric fat stranding (6-point star) with thickening of Gerota’s fascia, Zuckerkandl’s fascia, and lateral conal fascia is seen.
2.
What findings do you expect the kidney biopsy and histopathological examination to reveal?
Pseudotuberculous pyelonephritis is characterized morphologically by diffuse or focal granulomatous inflammation, which is confined to the renal pelvis and is closely associated with loss of pelvic urothelium. There will be an area of necrosis, cheesy and gritty material reminiscent of matrix stone substance. The histopathological examination of our patient’s specimen showed a granulomatous lesion with central necrosis in the background of chronic pyelonephritis.
3.
What is the likely diagnosis of this patient and its association?
The final diagnosis was pseudotuberculous pyelonephritis. Recurrent untreated urinary tract infection may be the common contributing factor to the disease for this patient.
4.
What is the treatment and prognosis?
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There is no clear guideline for the management of pseudotuberculous pyelonephritis in children. Multiple strategies of treatment may be employed. In individuals with clinical presentation and radiologic evaluation which are consistent with the diagnosis and when the disease is segmental, the first-line management with antibiotic therapy and percutaneous drainage has been advocated. In those individuals with diffuse renal involvement, nephrectomy is often necessary. In our patient, he showed poor response to multiple antibiotic courses with persistent overt pus cells in the urine with aggressive kidney involvement; hence, subcapsular nephrectomy would be a better treatment option.