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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT Table of Contents   
Year : 2010  |  Volume : 21  |  Issue : 3  |  Page : 515-517
Xanthogranulomatous pyelonephritis in a Horse-Shoe kidney

1 Department of Urology, Institute of Post-Graduate Medical Education and Research (IPGMER) & SSKM Hospital, Kolkata, India
2 Department of Pathology, Institute of Post-Graduate Medical Education and Research (IPGMER) & SSKM Hospital, Kolkata, India

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Date of Web Publication26-Apr-2010


Xanthogranulomatous pyelonephritis (XGPN) represents an unusual suppurative gra要ulomatous reaction to chronic infection, often in the presence of chronic obstruction from a calculus. We present a case of XGPN in a horse shoe kidney in an adult. Hemi-nephrectomy of the involved side was followed by clinical improvement. The case highlights the importance of early hemi-nephrectomy in XPGN with horse shoe kidney.

How to cite this article:
Mongha R, Dutta A, Vijay M, Chatterjee U, Chakraborty SC. Xanthogranulomatous pyelonephritis in a Horse-Shoe kidney. Saudi J Kidney Dis Transpl 2010;21:515-7

How to cite this URL:
Mongha R, Dutta A, Vijay M, Chatterjee U, Chakraborty SC. Xanthogranulomatous pyelonephritis in a Horse-Shoe kidney. Saudi J Kidney Dis Transpl [serial online] 2010 [cited 2022 Aug 10];21:515-7. Available from: https://www.sjkdt.org/text.asp?2010/21/3/515/62696

   Introduction Top

Xanthogranulomatous pyelonephritis (XGPN) is a chronic renal infection typically resulting in diffuse renal destruction. The disease is asso苞iated with obstructive uropathy secondary to nephrolithiasis resulting in a nonfunctioning, enlarged kidney in most cases. The pathologic process is usually unilateral, though cases of bilateral disease have been reported. [1] Literature review revealed only one case of XGPN in a horse shoe kidney, reported in a child. No such case has been earlier reported in an adult. [2] We present a case of Xanthogranulomatous pyelo要ephritis in a horse shoe kidney in an adult. Radiological and histopathological features of the disease are also discussed.

   Case Report Top

A 62-year-old male was admitted with dull aching and non-radiating pain in the left lumbar region and low grade fever of ten days duration. The patient had no past history of renal disease orhypertension. Physical examination showed mild pallor, tachycardia, and temperature of 101΀F. Abdominal examination showed a soft and lax abdomen with no lump or organome茆aly. Laboratory tests revealed hemoglobin of 9.9 gm/dL, leukocyte count of 9000/mm 3 with polymorphs 74%, urea 84 mg%, serum creati要ine 2.0 mg%, Na + 128 Meq /L and serum K + 4.6 Meq/L. Urinalysis revealed alkaline pH and pus cells 22-24 per high power field. Urine culture grew E. Coli sensitive to ceftriaxone and oflo軍acin. Patient's abdominal ultrasound showed left sided nephrolithiasis and hydronephrosis with complex cystic SOL in the upper pole of left kidney with ectopic right kidney. Computed to衫ographic (CT) scan revealed horseshoe kidney, hydronephrotic left kidney with multiple cal苞uli; renal parenchyma on left side was replaced by low attenuation masses representing dilated calyces and abscess filled cavities suggestive of Xanthogranulomatous pyelonephritis [Figure 1]. DMSA scan showed enlarged left Kidney ex負ending downward towards ectopically placed right kidney. Outline of the left side was smooth and showed only 9.45% of total relative uptake. GFR of left kidney was 4 mL/min and of the right side 14 mL/min on DTPA scan.

Surgical exploration revealed horse-shoe kid要ey with left kidney side having dense peri要ephric inflammatory extension into the retro計eritoneum upto the midline. Left Hemi-neph訃ectomy was done with removal of all inflamed and infected perinephric tissues. Specimen was full of purulent material and contained multiple calculi within the pelvi-calyceal system. Post operative course was uneventful and serum crea負inine came down to 1.2 mg% on 7 th post-ope訃ative day and the patient was discharged on oral antibiotics for 2 weeks.

Biopsy of the resected specimen showed sheets of inflammatory cells, lymphoid follicles, atro計hic glomeruli and tubules on low power (H & E Χ 40) [Figure 2]. High power view (H & E Χ 400), revealed sheets of foamy macrophages (Xanthoma cells) confirming XGPN [Figure 3]. At 16 weeks follow up patient was asympto衫atic and the serum creatinine level further de苞lined to1.0 mg%. DTPA scan showed im計rovement in GFR of the right kidney to 29.03 mL/min with all phases of renogram within normal limits.

   Discussion Top

XGPN represents an unusual suppurative gra要ulomatous reaction to chronic infection, often in the presence of chronic obstruction from a calculus. XGPN occurs in approximately 1% of all renal infections. XGPN is four times more common in women than men and is usually no負ed in the fifth and sixth decades of life. XGPN displays neoplasm like properties capable of local tissue invasion and destruction and has been referred to as a pseudo tumor. [3] XGPN has been described in three stages, as follows:

  1. kidney alone,
  2. kidney and perinephric fat, and
  3. kidney, perinephric fat and retroperitoneum extension. [1]
The gross appearance of XGPN is a mass of yellow tissue with regional necrosis and hemo訃rhage, superficially resembling renal cell carci要oma. The microscopic features of XGPN are the presence of foamy lipid-containing macro計hages (xanthoma cells), diffuse infiltration with plasma cells, and histiocytes. [4] Focal abscesses may be observed. Complete Blood Count may reveal leukocytosis and anemia. Serum chemis負ries determine the presence of any baseline electrolyte abnormalities, though none are pa負hognomonic of XGPN. Contrast enhanced CT is a reliable method to diagnose XGPN and to establish the presence and extent of extrarenal involvement. The CT findings of XGPN are calculi (75-86%), increase in renal volume (5560%), hydronephrosis (80%), hypodense areas with density measurements from 15 to 25 HU representing focal areas of parenchymal des負ruction filled with pus and/or debris, rim en虐ancement of these low density areas and ex負rarenal involvement, which is seen in 15-20% of cases. [5],[6] This disease process shares many characteristics with a true neoplasm in terms of its imaging appearance and ability to involve adjacent structures or organs. The present in苞reasingly sensitive radiological investigations, especially the CT-scan, in combination with clinical suspicion, have made the preoperative diagnosis of XGPN possible. [7] XGPN is noto訃ious for fistulization; pyelo-or uretero-cutaneous fistulae are well described, and occasionally may involve the bowel resulting in pyeloenteric fis負ula. The overall prognosis for XGPN is good. Death from this entity is exceedingly rare, though morbidity is substantial. [8]

In patients of XGPN in a fused kidney, we believe that local extension of disease and in苯lammatory mediators secreted from the Xan負hogranulomatous kidney may be the possible causes of deterioration of function on the other side too. Nephrectomy is the standard treatment for XGPN and in patients with fused kidney should be done early to limit the extension of disease to the better kidney and for its reco赳erability. The function in the other half of horse-shoe kidney improved after hemi-neph訃ectomy in our case. Radionuclide studies are extremely useful in assessing differential renal function in patients of XGPN as well as in follow up of these patients. [9] Patients should be on antibiotics for about two weeks postope訃atively for control of infection and a close long term follow up is recommended.

   References Top

1.Malek RS, Elder JS. Xanthogranulomatous pyelone計hritis: A critical analysis of 26 cases and of the literature. J Urol 1978;119(5):589-93.  Back to cited text no. 1      
2.Hammadeh MY, Calder CJ, Corkery JJ. Paedia-tric xanthogranulomatous pyelonephritis in a horseshoe kidney: Department of Paediatric Surgery, Children's Hospital, Birmingham, UK. Br J Urol 1994;73(6): 721-2.  Back to cited text no. 2      
3.Lorentzen M, Nielsen HO. Xanthogranulomatous pye衍onephritis. Scand J Urol Nephrol 1980;14(2): 193-200.  Back to cited text no. 3      
4.Moller JC, Kristensen IB. Xanthogranulomatous pye衍onephritis. A clinico-pathological study with special reference to pathogenesis. Acta Pathol Microbiol Scand 1980;88(2):89-96.  Back to cited text no. 4      
5.Kaplan DM, Rosenfield AT, Smith RC. Ad-vances in the imaging of renal infection-helical CT and modern coordinated imaging. Infect Dis Clin North Am 1997;11(3):681-705.  Back to cited text no. 5      
6.Kim JC. US and CT findings of Xanthogranulomatous pyelonephritis. Clin Imaging. 2001;25 (2):118-21.  Back to cited text no. 6      
7.Zugor V, Schott GE, Labanaris AP. Xanthogranulo衫atous pyelonephritis in childhood: A critical ana衍ysis of 10 cases and of the literature. Urology 2007;70(1):157-60.  Back to cited text no. 7      
8.Chuang CK, Lai MK, Chang PL, et al. Xanthogranu衍omatous pyelonephritis: experience in 36 cases. J Urol 1992;147(2):333-6.  Back to cited text no. 8      
9.Gregg CR, Rogers TE, Munford RS. Xanthogranulo衫atous pyelonephritis. Curr Clin Top Infect Dis 1999;19:287-304.  Back to cited text no. 9  [PUBMED]    

Correspondence Address:
Sudip C Chakraborty
Department of Urology, IPGMER & SSKM Hospital, Kolkata
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Source of Support: None, Conflict of Interest: None

PMID: 20427880

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  [Figure 1], [Figure 2], [Figure 3]

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