Xanthogranulamatous pyelonephritis associated with Left PUJ matrix staghorn calculus

A rare form of kidney infection, xanthogranulomatous pyelonephritis (XPGN), causes between 0.6 and 1% of all cases^{. 1,2} Obstructive uropathy is considered to be the major cause. The renal parenchyma is invaded by lipid-laden macrophages known as xanthoma cells, which are a characteristic of this condition. ^2. The preferred modality for the diagnosis and staging of XGP is contrast-enhanced CT scans, which define the extent of the parenchymal damage and the involvement of the perinephric space. ³ Advanced disease is characterised by the presence of hydronephrosis, thinning, and extension of the inflammation beyond the renal cortex and the renal capsule. The cause of the disease is the chronic obstruction of the urinary drainage. The presence of the disease is characterised by the presence of the stone, which is staghorn or large. The stones cause the obstruction of the drainage of the urine. ^4,5 The chronic disease is responsible for the stasis of the urine, which in turn becomes infected, leading to inflammation. This inflammation distorts the normal architecture of the kidney.⁶ The management of the established disease has always been radical nephrectomy, given the irreversible damage to the renal architecture ^{7, 8}. However, the possibility of kidney-sparing surgery is present if the inflammation is in the early stages of the disease and the renal function is adequate.

MATERIALS & METHODS

Study Design: Single-patient case report

Diagnostic Tool Used:  ultrasonography

Data Sources: Clinical presentation, physical examination, imaging findings

Ethical Considerations: Patient identity not disclosed

CASE PRESENTATION

A 32-year-old female patient, had a history of Ureteroscopic Lithotripsy (URSL) Double-J Stenting ( DJ). She also underwent left Percutaneous Nephrolithotomy (PCNL) with DJ stent and Percutaneous Nephrostomy (PCN) tube placement along with ureterscopy , presented with left flank pain and intermittent fever that was sudden in onset, rapidly progressive since one month.

On physical examination:

• Blood pressure: 130/90 mmHg
• Pulse rate: 82 beats per minute
• Afebrile at presentation
• Left flank tenderness on examination

Laboratory investigations showed haemoglobin levels of 11.g/dL and reduced at MCV 69.4fL, MCH 22.5pg and RDW-CV elevated at 25%, indicating microcytic anemia. Total leukocyte count was elevated at 15270cells/cumm suggesting significant infection.

USG of the kidneys, ureters, and bladder (KUB) revealed an bulky left kidney (~15.5 X 8.0 cm) with mildly thinned out cortex and prominent renal calyces and pelvis. The pelvicalyceal system showed echogenic contents within the calyces.Few non-obstructive calculi were noted in the inter polar region and lower pole, the largest measuring 16 X 7 mm. DJ stent was noted in-situ. The right kidney was normal in size (10.5 X 3.9 cm) with preserved shape and echotexture. No evidence of hydroureteronephrosis was seen. The urinary bladder was well distended with free-floating internal echoes and showed no evidence of wall thickening or irregularity. The patient received treatment of antibiotics (meropenem1g, metronidazole, and amikacin 500 mg), NSAIDs (diclofenac sodium), and antipyretics (DOLO 650 mg). After pre-operavtive evaluation and informed constent .She had a percutaneous nephrolithotomy (PCNL). Percutaneous access was established under fluoroscopic guidance and stone fragmentation and extraction were completed successfully. A double-J stent was placed on order to ensure appropriate postoperative drainage. The procedure was uneventful. She developed sepsis with thrombocytopenia due to urosepsis and Injection meropenem. On postoperative day one platelet count was decreased (35000 lakh/cumm) and on day2 (25000 lakh/cummm). On day 3 (38000 lakh/cumm) elevated Four RDPs were transfused, and she was managed conservatively.

DISCUSSION

Xanthogranulomatous pyelonephritis has been defined as a chronic inflammatory condition resulting from urinary obstruction and infection. ² studies have shown that there exists a significant correlation between XGP and renal calculi, with a large number of cases showing the presence of renal calculi. ⁵ Obstructive urinary calculi are the main cause of chronic urinary obstruction, which plays a major role in the development of XGP. ⁶. Large staghorn stones are traditionally known for causing XGP, but smaller renal pelvic stones can also be responsible for the development of the condition if the urinary drainage mechanism remains compromised. Radiological examination plays a vital role in assessing the extent of the disease. Contrast CT scans can be used for the differentiation of renal inflammation and the extent of the disease, which might have spread to the perinephric tissues. ³ In the later stages, the surgical procedures for the removal of the affected organ are technically challenging because of the adhesions that are formed.7 In the case of diffuse XGP, the organ has to be removed, as the damage would be irreversible. 8. Histopathological examination shows the presence of granulomatous tissue with lipid-laden macrophages and chronic inflammation. 9 In our case, early percutaneous nephrolithotomy was successful in relieving the urinary obstruction and ensuring the free flow of urine, as is advisable in the presence of significant urinary symptoms due to renal calculi, as per urological guidelines.¹? The early correction of the urinary pathology likely arrested the progression of irreversible changes to the renal parenchyma, as seen in the xanthogranulomatous spectrum. The observed thrombocytopenia, though present, was likely due to the underlying condition of urosepsis and the use of intravenous meropenem, and it resolved as expected^..

RESULT

In conclusion, this case report highlights a rare instance of xanthogranulomatous pyelonephritis (XGP) in a patient with left pelvic-ureteric junction matrix staghorn calculus. She had a history of ureteroscopic lithotripsy (URSL) and DJ stent. She underwent left-sided PCNL, DJ stenting, and PCN tube, resulting in restoration of urinary drainage and significant improvement in renal function. Postoperative sepsis and persistent thrombocytopenia managed with supportive treatment and RDP transfusion. Early diagnosis with USG-KUB imaging and rapid medical care with antibiotics resulted in dramatically improved kidney function. This instance emphasises the significance of early management in avoid serious renal consequences in XGP.

REFERENCES

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