Background: Xanthogranulomatous pyelonephritis (XGP) is an uncommon infectious disease of the kidney known to mimic other renal maladies. A rare presentation of this uncommon disease is spontaneous renal hemorrhage (SRH). Case presentation: We report a case of XGP in a 58 year old woman who presented with abdominal pain, hematuria, and radiating left flank pain. CT scan was felt to be consistent with perirenal hemorrhage abutting a fat-containing renal mass. The patient was eventually taken to surgery for left partial nephrectomy. Pathology report returned as XGP, and the patient has no complications from this disease process at 8 month follow up. Conclusion: Our search of the literature shows XGP presenting as SRH to be a rare clinical entity. Furthermore, this is the first such case managed with a nephron-sparing approach. The “great imitator” XGP should be added to the differential for patients presenting with spontaneous renal hemorrhage. Keywords: Xanthogranulomatous pyelonephritis, Spontaneous renal hemorrhage, Wunderlich syndrome, Subcapsular renal hematoma, Case report Background Herein, we reported a female presenting with abdom- XGP is an uncommon infectious disease of the kidney inal pain and hematuria with radiographic findings first described in 1918 by Schlagenhaufer and character- consistent with SRH who was found to have XGP as the ized by chronic obstruction and inflammation . The underlying cause of her illness. We also review the chronic inflammatory renal mass invades the renal par- epidemiology, diagnosis, and management of these enchyma, replacing it with lipid laden macrophages. two conditions. Pathologically, the lipid laden macrophages give the renal parenchyma a “tan-yellow” appearance . SRH is Case presentation defined as a non-traumatic, spontaneous renal bleed into A 58 year old female presented with hematuria and left the subcapsular and/or perirenal space. It was first flank pain that radiated to the abdomen. Computed observed by Bonet in 1679 and further described by tomography (CT) scan demonstrated a heterogeneous Wunderlich in 1856 [3, 4]. This is only the 5th case 8.3 X 6.5 X 4.9 cm fat-containing mass arising from the report of XGP presenting as SRH. lower pole of the left kidney (Fig. 1c). CT also demonstrated an asymmetrically enlarged left psoas * Correspondence: firstname.lastname@example.org muscle (Fig. 1b), soft tissue stranding (Figs. 1a & 2), mild Department of Urology, Wake Forest Baptist Health, Medical Center Blvd, hydronephrosis, and splenomegaly (Fig. 2). Further evalu- Winston-Salem, NC 27157, USA Full list of author information is available at the end of the article ation of the CT scan goes on to reveal extensive fascial © The Author(s). 2018 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. Ballentine et al. BMC Urology (2018) 18:57 Page 2 of 5 Fig. 1 Axial CT scan demonstrating left renal hydronephrosis a and a non enhancing lesion of the left kidney c (arrowhead). Asymmetric psosas muscle b is identified and noticeable fat stranding along the posterior peritoneal wall (arrowhead). Ureteral calyces identified and demostrate distention possibly hemorrhaging thickening involving the anterior and posterior left renal dissection with a Penfield nerve retractor. All bleeding fascia. Based on these findings, hemorrhagic angiomyoli- vessels were ligated. There was 36 min of cold ischemia poma was felt to be the most likely condition. Past time and 200 ml of estimated blood loss. After appropri- medical history was significant for bipolar disorder and ate closure of the surgical sites, the mass was sent to chronic right foot wounds associated with contiguous pathology and because of considerable need for pyeloca- spread of chronic osteomyelitis of the 5th metatarsal. liceal closure a double J ureteral stent and foley were Physical exam was remarkable for enlarged body hab- placed. Postoperatively, the patient experienced urinary itus (BMI 34.7), limited ROM in right shoulder, arm, retention after foley catheter removal. Once stable, she and neck. Laboratory abnormalities included an elevated was discharged back to skilled nursing facility with a glucose (186 mg/dl) and calcium (6.9 mg/dl). White cell catheter in place that was later removed. count, BUN, and creatinine levels were within nor- At 10 day postoperative outpatient follow up, the pa- mal limits. Urine culture demonstrated no growth in tient was doing well and asymptomatic. BUN and cre- 18–24 h and showed < 10,000 col./ml. atinine levels were within normal limits but calcium The decision was made to treat the patient with open remained low. Ureteral stent was easily removed during left partial nephrectomy. Intraoperatively, the mass was office cystoscopy. Eight months later the patient re- easily found along the inferior portion of the kidney. ported no signs of flank pain or hematuria and has The mass had a large desmoplastic reaction surround it remained at her pre-operative baseline. from a prior bleeding episode. Adhesions surrounding the vascular pedicle were released. The pedicle was then Discussion clamped and cold ischemia was induced after mannitol XGP is well known for its capacity to mimic many other injection. Resection of the mass was performed by scor- disorders, most notably renal neoplasms [5, 6]. Previ- ing the mass with Bovie electrocautery and carefully dis- ously published case series have shown a higher inci- secting the mass from the normal parenchyma by blunt dence of disease in older women, patients obstructed Fig. 2 Coronal and sagittal CT scan demonstrating abnormal mass of left kidney, fat stranding, and hepatospenomegaly. There is a 3.5 cm round low attenuated area with peripheral calcifications in the spleen. Likely relates to prior trauma or infection Ballentine et al. BMC Urology (2018) 18:57 Page 3 of 5 from nephrolithiasis, and infection by Escherichia coli or case of retroperitoneal hemorrhage with hemorrhagic Proteus mirabilis.[7–9]. XGP is classified into either the shock . Briefly, a 36 year old woman presented with more prominent diffuse form or a focal entity . It is right lower abdominal pain with palpable mass over further subdivided into 3 stages (nephric, perinephric, right pelvic area. Laboratory findings included micro- and paranephric) on the basis of the extent of inflamma- cytic anemia and elevated white blood cell count (12,000 tory response. In part due to the rarity of XGP, pre- per mm ). Urinalysis (UA) was positive for leukocytes operative diagnosis is difficult with most cases diagnosed and pyuria. Urine culture was negative. post-operatively on pathological examination. Clinical CT scan demonstrated bilateral central staghorn diagnosis is hampered by non-specific symptoms, and calculi with replacement of renal parenchyma with low pre-operative radiologic imaging has been found to be of attenuated collections in a hydronephrotic pattern. CT only moderate assistance in diagnosis . While the also showed a lower pole mass in the right kidney con- classic “bear’s paw” sign is reasonably diagnostic of XGP, sistent with perinephric inflammatory changes. T1 and further radiologic specificity is limited by the hete- T2 weighted MRI images of the mass showed multiple rogenous nature of the disease. One study of 11 patients enhancing septa. with XGP found that 91% of patients demonstrated Two days following the initial presentation the patient extrarenal extension of inflammatory changes and 82% was seen again for acute abdominal pain with a red cell had multiple dilated calyces and abnormal parenchyma, count of 6 g/dl, blood pressure of 80/60, and was trans- but 27% had focal fat deposits and a separate 27% had ferred to the intensive care unit for hemorrhagic shock. extensive retroperitoneal inflammation up to and includ- CT scan now showed an interval increase in the size of ing inflammation of the abdominal wall . the renal mass and hemoperitoneum. After right neph- SRH, also known as Wunderlich Syndrome, is another rectomy pathological examination of the mass revealed uncommon urologic condition. The etiology of SRH is abundant foamy macrophage aggregates, neutrophils, fi- most frequently due to renal neoplasm but has also been brosis, and inflammatory cell infiltrations, confirming known to arise due to vascular disease or infection . the diagnosis of XGP . While the infectious etiologies of SRH are known to in- Altinoluk et al., describe a case of XGP with spontan- clude emphysematous pyelonephritis, to the best of our eous kidney rupture in a young female . A 25 year knowledge this is only the 5th case in the literature of old woman presented with sudden onset right flank pain XGP presenting with Wunderlich syndrome, although associated with fever and nausea. She was previously this is difficult to assess due to the many different terms treated for UTI the week prior. Her blood pressure was applied to the condition [14–17]. SRH may classically 90/55 mmHg, hemoglobin 6.3 g/dl, white blood cell present with “Lenk’s triad” (acute flank pain, abdominal count was 16,700 per mm . UA revealed hematuria only. tenderness, and symptoms of internal bleeding), but the Abdominal ultrasound and computed tomography disease is also known to present similarly to other ab- displayed a large hypoechoic mass (12 X 7 cm) around the dominal conditions such as appendicitis or dissecting right kidney which extended into the pelvis and abdominal aortic aneurysm [13, 18]. Unlike XGP, CT paravertebral space. Exploration of the mass revealed a imaging of SRH patients is able to identify 100% of pa- large perirenal hematoma, abscess, and renal rupture. tients suffering from the disease, though it lacks sensitiv- Culture of the abscess grew Proteus mirabilis. ity for the etiology of SRH. Ultrasound has also been Histopathological examination following right nephrectomy used for the detection of SRH but was not shown to be revealed foamy, lipid laden macrophages, giant cells, as reliable. polymorphonuclear cells, granulomatous reaction and Three of 4 XGP cases presenting with Wunderlich fibrosis. A diagnosis of XGP was made . syndrome are summarised and can be reviewed in Lastly, Sharma et al., reported a case of a 60 year old Table 1. Canale et al., in a letter to the editor, presents a male who presented with left sided flank pain, Table 1 Summary of XGP cases presenting as Wunderlich syndrome Case Presentation CBC UA Culture (urine or site) Surgery Pathology Canle et al. Right lower abdominal Hgb 8 9/dl +leukocyte Streptococcus Right total Foamy macrophages aggregates, , 2007 pain and asthenia WBC 12000/mm +Pyuria anginosus nephrectomy neutrophils, fibrosis. Inflammatory cell infiltrate Altinoluk et al. Right flank pain, fever, Hgb 6.3 g/dl Hematuria only Proteus mirabilis Right total Foamy, lipid laden macrophages, , 2012 nausea WBC 16.7/ uL nephrectomy giant cells, granulomatous reaction and fibrosis Sharma et al. Left flank pain, Hgb 9 g/dl – Klebsiella Left total Chronic granulomatous , 2013 intermittent fever, WBC 13500/mm3 nephrectomy pyelonephritis. With dilated weakness x15days vascular channels Ballentine et al. BMC Urology (2018) 18:57 Page 4 of 5 intermittent fever, and weakness for 15 days . A All four previous reports of XGP presenting with SRH known diabetic, the patient was anemic (Hgb 9.0) with were managed by radical nephrectomy. We report the an elevated white blood cell count (13,500 per mm ). first case of nephron-sparing surgery for XGP presenting Urine culture was positive for Klebsiella and the patient with SRH. Our management decision was facilitated by a was subsequently started on IV antibiotics. MRI pre-operative differential heavily weighted towards AML abdomen demonstrated a hydronephrotic left kidney as the etiology of SRH. Prior to this procedure our ap- with an ill-defined mass of the lower pole suggestive of proach to cases involving a large XGP lesion would have perinephric hematoma. Further, the patient was sus- been unlikely to include a nephron-sparing approach, pected to have a hemorrhagic angiomyolipoma and was though we are happy to report that such an approach is thus surgically explored. During exploration, the kidney certainly technically feasible. was noted to be hydronephrotic with thinned out parenchyma and palpable thickening of the lower pole Conclusion which warranted nephrectomy. Unlike the previous cases, This report describes a rare presentation of an uncom- lipid-laden macrophage aggregates were not seen though mon infectious disease. Our review of the literature further histopathological examination of the mass revealed confirms the rarity of XGP presenting with SRH. The chronic granulomatous pyelonephritis with dilated importance of a broad differential diagnosis for this con- vascular channels and no evidence of neoplasia . dition cannot be overlooked. Nephron-sparing surgery should always be considered as a possibility even in Management these difficult cases. The traditional approach to XGP has been radical neph- Abbreviations rectomy, though a nephron-sparing approach has been re- CT: Computed tomography; Hgb: Hemoglobin; SRH: Spontaneous renal ported in the management of focal cases of XGP [19–21]. hemorrhage; UA: Urinalysis; WBC: White blood cell count; Conservative management of XGP has been achieved with XGP: Xanthogranulomatous Pyelonephritis parenteral antibiotic therapy or a combination of oral and Acknowledgments parenteral therapy which may be supplemented with We would like to thank Dr. Ray Dyer for his assistance in interpreting drainage of the urinary tract and/or abscesses [22–24]. radiological images used in the study and for his guidance in drafting and Conservative management has even proven successful in editing this manuscript. renal allograft patients with XGP . However, conserva- Availability of data and materials tive management is inappropriate for patients with stage All data generated or analyzed during this study are included in this III or diffuse XGP, as may be seen in many patients at the published article [and its supplementary information files]. time of diagnosis. Nephron-sparing management has been Authors’ contributions successfully attempted in cases of multifocal XGP, a dis- MM was the responsible urologist for the patient and performed the surgery. tinct entity from diffuse XGP. As XGP is often diagnosed WB, FV, & MM contributed to the concept and design of the study. WB & FV first at pathological specimen, the decision for treatment both contributed to the acquisition of data, analysis and interpretation of data, as well as the drafting of the manuscript. MM & RD participated in the is usually based on a presumptive diagnosis in which rad- critical revision of the manuscript for important intellectual content, material ical nephrectomy may be more appropriate. support, and supervision. RD & MM also contributed to the acquisition of Management of SRH is first pursued through conserva- valuable data, analysis and interpretation of data, and final approval of the manuscript. All authors read and approved the final manuscript. tive or minimally invasive approaches. As the underlying cause of SRH may be focally identified by renal arteriog- Ethics approval and consent to participate raphy, embolization is an important therapeutic treatment Not applicable. that may preclude the need for surgery. In cases of iatro- Consent for publication genic renal hemorrhage, the combined use of urokinase Written informed consent was obtained from the patient for publication of injections and external drainage has been reported as an this case report and accompanying images. effective strategy in patients with large hematomas who nonetheless had stable vital signs . This approach Competing interests The authors declare that they have no competing interests. could also prove useful in SRH though we can find no ex- plicit report of this in the literature. In the case of the un- stable patient, surgical exploration is necessary if Publisher’sNote Springer Nature remains neutral with regard to jurisdictional claims in angioembolization is not available or is unsuccessful. Sur- published maps and institutional affiliations. gical exploration can be especially important if the source of the bleeding is found to be a renal neoplasm. Even in Author details Department of Urology, Wake Forest Baptist Health, Medical Center Blvd, cases where bleeding can be managed conservatively, sur- Winston-Salem, NC 27157, USA. Wake Forest School of Medicine, gical evacuation of the clot may be required in instances 3 Winston-Salem, NC 27157, USA. Department of Radiology, Wake Forest of hypertension secondary to Page kidney. Baptist Health, Winston-Salem, NC 27157, USA. Ballentine et al. BMC Urology (2018) 18:57 Page 5 of 5 Received: 27 September 2016 Accepted: 2 May 2018 References 1. Schlagenhaufer F. Uber eigentumlich staphylomykosender neiven und des pararenalen bindegewebes. Frankfurt Z Pathol. 1916(19):139–48. 2. Yoshino T, Moriyama H. Case of the diffuse form of Xanthogranulomatous pyelonephritis. Case Rep Urol. 2013; ID 936035: 3 pages. 3. Bonet T. Sepulchretum, sive anatomiapractica ex cadaverbius morbobdenatis. Geneva: L Chouet; 1679. 4. Wunderlich CR. Handbuck der Pathologie und Therapie. In: ed 2nd, editor. 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