Fistula reno-apendicular como presentación inusual de pielonefritis xantogranulomatosa: reporte de caso / Reno-appendicular fistulus as an unusual presentation of xanthogranulomatous pyelonephritis: a case report

Resumen La fístula uro-entérica es una comunicación patológica entre la vía urinaria y digestiva. El compromiso del apéndice es infrecuente y son pocos los casos de fístulas reno-apendiculares en la literatura. Se presenta el caso de un paciente con clínica de fiebre, dolor lumbar e hidronefrosis derecha severa secundaria a cálculo coraliforme en la tomografía de vías urinarias. Manejado inicialmente con antibióticos y nefrostomía bajo fluoroscopia, posteriormente suspendida por paso de contraste al intestino. Se realizó una tomografía contrastada que reportó fístula del riñón al intestino. Se llevó a nefrectomía y se encontró fistula hacía el apéndice, por lo cual se realizó apendicectomía concomitante con mejoría clínica evidente. La patología reportó pielonefritis xantogranulomatosa y apendicitis secundaria. La fístula reno-apendicular posee una clínica inespecífica, la tomografía contrastada es una herramienta diagnóstica y la mayoría se detectan como un hallazgo intraoperatorio. El tratamiento usualmente es quirúrgico, con nefrectomía y reparación del segmento intestinal. MÉD. UIS.2021;34(3): 79-84.

Abstract Uro-enteric fistula is a pathological communication between the urinary and digestive tract. Compromise of the appendix is infrequent and few cases of reno-appendicular fistulas have been described in the literature. The case of patient with symptoms of fever, low back pain and severe right hydronephrosis secondary to staghorn calculus on urinary tract tomography is presented. Initially managed with antibiotics and nephrostomy under fluoroscopy, subsequently suspended by passing contrast to the intestine. A contrasted tomography was performed which reported a fistula from the kidney to the intestine. A nephrectomy was carried out and a fistula was found to the appendix, for which a concomitant appendectomy was performed with evident clinical improvement. The pathology reported xanthogranulomatous pyelonephritis and secondary appendicitis. Reno-appendicular fistula has nonspecific symptoms, contrasted tomography is a diagnostic tool and most are detected as an intraoperative finding. Treatment is usually surgical, with nephrectomy and intestinal segment repair. MÉD.UIS.2021;34(3): 79-84.

Xanthogranulomatous pyelonephritis with calculus migration into the psoas abscess: an unusual complication

Xanthogranulomatous pyelonephritis (XGP) is a rare variant of chronic pyelonephritis. It is characterized by progressive parenchymal destruction caused by chronic renal obstruction due to calculus, stricture, or rarely tumor, resulting in kidney function loss. Herein, we describe the case of a 36-year-old female who presented with left loin pain, left lower limb pain, and dysuria. On contrast-enhanced computed tomography (CECT), multiple abscesses and an obstructive staghorn calculus were depicted in the left kidney with the classical appearance of "Bear Paw Sign." An abscess with calculi was also present within the left psoas muscle. Though psoas muscle abscess in association with XGP was described, a ureteric fistula and calculi within the psoas muscle have not yet been reported in the literature. Left nephrostomy was performed, which came out to be positive for E. coli on culture. The patient underwent left nephrectomy, and the histopathological report of the surgical specimen confirmed XGP.

Pielonefritis xantogranulomatosa secundaria a litiasis obstructiva en un paciente pediátrico / Pyelonephritis Xanthogranulomatous Secundary to Obstructive Lithiasis in Pediatric Patient

Introducción La pielonefritis xantogranulomatosa (XGPN), es una enfermedad inflamatoria crónica del parénquima renal, de rara presentación en pediatría. Su etiología es multifactorial comenzando con un proceso obstructivo del tracto urinario por litiasis e infección hasta el daño renal crónico. Su diagnóstico se logra por sospecha clínica, estudio imagenológico e histopatológico, lo que require tratamiento definitivo en la mayoría con nefrectomía simple. Materiales y métodos Paciente masculino de 4 años de edad con infección de la vía urinaria y dilatación de pelvis y uréter causada por cálculos que genera un proceso inflamatorio crónico y destrucción del parénquima renal. Sin respuesta clínica a tratamiento antibiótico quien requirió manejo en unidad de cuidados intensivos y nefrectomía. Discusión Se presenta entre la cuarta a sexta década de vida, con reportes de caso en edad pediátrica. Cursa con fiebre, dolor abdominal y masa palpable. Puede presentar en cambio de cursar con leucocitosis, anemia y piuria. Estudio radiológico de XGPN se puede apreciar de forma focal o difusa. A la histopatología se evidencia necrosis tisular crónica con macrófagos espumosos en su interior entremezclados con células inflamatorias. El tratamiento definitivo requiere nefrectomía de unidad renal disfuncional. Conclusiones Se presenta el caso de paciente pediátrico con XGPN por obstrucción urinaria litiásica; entidad de baja frecuencia en ese grupo poblacional, requiere una adecuada correlación clínico-imagenológica e histológica para lograr el diagnóstico final y puede ser tratado con manejo médico y quirúrgico.

Introduction Xanthogranulomatous pyelonephritis (XGPN) is a chronic inflammatory disease of the renal parenchyma, rare in pediatrics. Etiology is multifactorial ranging from an obstructive urinary tract process through lithiasis and infection generating chronic kidney amage. Its diagnosis is achieved by clinical suspicion, imaging and histopathology, which requires definitive treatment in the majority with simple nephrectomy Materials and Methods A 4-year-old male patient with urinary tract infection and dilation of the pelvis and ureter caused by calculus that generate a chronic inflammatory process and destruction of the renal parenchyma. No clinical response to antibiotic treatment that required management and intensive care unit and nephrectomy. Discussion It occurs between the fourth and sixth decade of life, with case reports in pediatric age. Patients have fever, abdominal pain and palpable mass. They can occur with leukocytosis, anemia and pyuria. Radiological study of XGPN can be seen in focal or diffuse form. Histopathology looks a chronic tissue necrosis with foamy macrophages and inflammatory cells. Definitive treatment requires nephrectomy of damaged renal unit. Conclusions We report the case of pediatric patients with XGPN due to urinary obstruction of the lithiasis; Low frequency entity in this population group, requires an adequate clinical-imaging and histological correlation to achieve final diagnosis and can be treated with medical and surgical management.

Extensive renal sinus lipomatosis in xanthogranulomatous pyelonephritis simulating liposarcoma

ABSTRACT Renal replacement lipomatosis is a condition characterized by varying degrees of renal parenchymal atrophy and perirenal fibrofatty proliferation secondary to chronic inflammation such as xanthogranulomatous pyelonephritis. In severe cases, imaging findings can be misdiagnosed as retroperitoneal liposarcoma.

Laparoscopic nephrectomy outside gerota fascia and en bloc ligation of the renal hilum for management of inflammatory renal diseases

ABSTRACT Objectives This study aims to improve laparoscopic nephrectomy techniques for inflammatory renal diseases (IRD) and to reduce complications. Materials and Methods Thirty-three patients underwent laparoscopic nephrectomy for IRD, with a method of outside Gerota fascia dissection and en-bloc ligation and division of the renal pedicle. Operative time, blood loss, complications, analgesia requirement, post-operative recovery of intestinal function and hospital stay were recorded. The degrees of perinephric adhesion were classified based on the observation during operation and post-operative dissection of the specimen, and the association of different types of adhesion with the difficulty of the procedures was examined. Results Among 33 cases, three were converted to hand-assisted laparoscopy, and one was converted to open surgery. Mean operative time was 99.6±29.2min, and blood loss was 75.2±83.5 mL. Postoperative recovery time of intestinal function was 1.6±0.7 days and average hospital stay was 4.8±1.4 days. By classification and comparison of the perinephric adhesions, whether inflammation extending beyond Gerota fascia or involving renal hilum was found to be not only an important factor influencing the operative time and blood loss, but also the main reason for conversion to hand-assisted laparoscopy or open surgery. Conclusions In laparoscopic nephrectomy, outside Gerota fascia dissection of the kidney and en-bloc ligation of the renal pedicle using EndoGIA could reduce the difficulty of procedure and operative time, with satisfactory safety and reliability. Inflammation and adhesion extending beyond Gerota fascia or involving renal hilum is an important predictor of the difficulty related to laparoscopic nephrectomy for IRD.

Xanthogranulonatous pyelonephritis: report of 5 cases / 北京大学学报(医学版)

Xanthogranulomatous pyelonephritis (XGP) is an unusual form of chronic pyelonephritis in which the renal parenchyma is destroyed and replaced by lipid-laden foamy macrophages. It usually affects middle-aged women with a history of recurrent urinary tract infection, diabetes, or kidney stones. The inflammatory process is usually diffuse and can extend beyond the kidney. The rare focal forms may simulate primary renal tumours. The preoperative imaging diagnosis may be difficult. We reported five cases of XGP, The findings of ours were recorded including kidney size, shape, contour, the echogenecity of the renal parenchyma, the internal echoes of the dilate collecting system, the presence of perinephric fluid accumulation and obstruction. One of the 5 cases was a male patient, and the other four were female, with a mean age of 53 years. He affected kidneys of the 5 cases swelled in different degrees, and one of them was found with line-like anechoic fluid. Among the 5 cases, one kidney appeared as diffusely reducing of the parenchyma echogenicity, multiple hypoechoic areas, disappearance of corticomedullary differentiation and multiple hyperecho with shadow. A round cystic anechoic lesion was found in one kidney, with internal punctate echo and peripheral fluid. Ultrasonographic finding of 1 case was extremely hypoechoic lesion on the left kidney, protruding from the outline of the kidney, with the partial renal capsule discontinuous, the less clear boundary, and a little blood flow in it. Ultrasonographic demonstration of 2 cases was mild dilatation of the collecting system with irregular wall thickening and internal hypoechogenicity, and 1 case was solid lesion with less clear boundary to the pelvic wall and a small amount of blood flow signal, the another 1 case was showed floccule without internal blood flow. Three cases were caused by chronic obstruction verified by operation, of which one was staghorn calculi, one was poorly differentiated squamous cell carcinoma in the middle part of the ureter, and one was inflammatory stricture of upper ureteral. Through analysis of the above five cases and review of related literature, we explored diagnoses and management of the patients with XGP. Xanthogranulomatous pyelonephritis (XGP) is a rare chronic variant of pyelonephritis characterized by destruction of the renal parenchyma. Combining ultrasonographic features of XGP with clinical recurrent urinary infection and chronic obstruction, XGP can be included in the differentiation. The diagnosis of XGP suspected by ultrasound can be clarified by CT, MRI, contrast-enhanced ultrasound.

Pielonefritis Xantogranulomatosa / Xanthogranulomatous Pyelonephritis

Introducción: la pielonefritis xantogranulomatosa es una enfermedad infecciosa, inflamatoria, crónica e inusual que afecta el parénquima renal. El tratamiento definitivo es la nefrectomía. Objetivos: presentar un nuevo caso de pielonefritis xantogranulomatosa, así como la secuencia diagnóstica y terapéutica adoptada. Métodos: paciente masculino de 61 años de edad, con antecedentes de hipertensión arterial, diabetes mellitus tipo II e infecciones urinarias a repetición, que asistió al cuerpo de guardia del Hospital Militar Central Dr. Luis Días Soto, por presentar dolor lumbar y en flanco derechos, de 2meses de evolución, intenso y punzante, asociado a fiebre de 40̊ C, escalofríos y marcada toma del estado general. En el examen del hemiabdomen derecho presentaba una masa tumoral de unos 10cm de diámetro, dolorosa, ligeramente movible y de superficie lisa. El urocultivo reportó Escherichiacoli. El ultrasonido abdominal evidenció una imagen compleja en el riñón derecho, y la tomografía axial computarizada un tumor heterogéneo, que medía 10,7x7, 6 cm de contornos regulares, que distorsionaba la grasa perirrenal con densidades de hasta 73 UH. Resultados: el paciente fue sometido a nefrectomía total derecha transperitoneal, por sospecha de tumor renal, proceder que transcurrió sin complicaciones. Los hallazgos microscópicos de la pieza quirúrgica revelaron infiltración de linfocitos, histiocitos y macrófagos llenos de lípidos, compatible con pielonefritis xantogranulomatosa. La evolución fue satisfactoria. Conclusiones: aunque el diagnóstico definitivo de la pielonefritis xantogranulomatosa es histopatológico, la forma tumoral de presentación de la misma, es uno de los diagnósticos diferenciales a tener en cuenta en pacientes diabéticos con infecciones recurrentes del tracto urinario(AU)

Introduction: Xanthogranulomatous pyelonephritis is an infectious, inflammatory, chronic, and unusual disease that affects the renal parenchyma. The definitive treatment is nephrectomy. Objectives: Present a new case of xanthogranulomatous pyelonephritis, as well as the diagnostic and therapeutic sequence adopted. Methods: A 61-year-old male patient with a history of hypertension, type II diabetes mellitus and recurrent urinary tract infections attended Dr. Luis Díaz Soto Central Military Hospital, for presenting intense and stabbing lumbar and right flank pain, of 2 months of evolution, associated with fever of 40 ° C, chills and marked the general condition. In the examination of the right abdomen a tumor mass was found of about 10 cm in diameter, painful, slightly movable and of smooth surface. The urine culture reported escherichiacoli. Abdominal ultrasound showed a complex image in the right kidney, and computed tomography scan showed a heterogeneous tumor, measuring 10.7x7.6 cm of regular contours, distorting the perirenal fat with densities of up to 73 UH. Results: The patient underwent transperitoneal right total nephrectomy, due to suspicion of renal tumor. This procedure had no complications. Microscopic findings of the surgical specimen revealed infiltration of lymphocytes, histiocytes, and lipid-filled macrophages, consistent with xanthogranulomatous pyelonephritis. The evolution was satisfactory. Conclusions: Although the definitive diagnosis of xanthogranulomatous pyelonephritis is histopathological, the tumoral presentation form is one of the differential diagnoses to be considered in diabetic patients with recurrent urinary tract infections(AU)

Xanthogranulomatous Pyelonephritis Associated with Actinomycosis Misdiagnosed as a Metastatic Kidney Cancer / 대한내과학회지

Xanthogranulomatous pyelonephritis (XP) is an uncommon, chronic, destructive, granulomatous disease of the renal parenchyma. The condition is generally associated with long-term urinary tract obstruction and infection, although the precise etiology of disease remains unknown. The condition often mimics neoplastic and inflammatory diseases, and XP associated with actinomycosis is extremely uncommon. A 60 year-old male was admitted complaining of abdominal pain and weight loss. His radiological findings were suggestive of metastatic kidney cancer, and we performed radical laparoscopic nephrectomy and mesenterectomy for diagnostic purposes. Pathologically, actinomycosis combined with XP was evident. We report here a rare case of XP associated with actinomycosis.

Late complication of a renal calculus: fistulisation to the psoas muscle, skin and bronchi

ABSTRACTKidney disease presenting with cutaneous fistula is a rare condition. We present a case of a 90-year-old woman with dementia who had no prior urological problems and had a cutaneous fistula in the left lumbar region. A fistulogram and computer tomography examination revealed a large staghorn calculus with signs of xanthogranulomatous pyelonephritis in the left kidney and renal fistulisation to the psoas muscle, skin and bronchi. To our knowledge this is the first report in the literature of coexisting renal fistulisation to the psoas major muscle, skin and bronchi. This report illustrates how computed tomography in combination with fistulography can resolve the diagnostic dilemma that pertains to the complex spread of the disease in cases involving nephrocutaneous fistula. Furthermore, the report shows how a renal calculus, even asymptomatic, can cause a serious medical condition, and highlights the importance of early medical intervention.

Xanthogranulomatous Pyelonephritis (XGPN) in a Child with Multiple Ureteric Calculi.

Xanthogranulomatous pyelonephritis (XGPN) is an unusual suppurative granulomatous reaction to chronic renal infection, often in the presence of chronic obstruction, characterised histologically by granulomatous reaction with giant cells and foamy histiocytes1. XGPN is rare in children2. We report a case of XGPN, in a child with multiple lower ureteric calculi. An 11 year old male child presented to us, with features suggestive of acute pyelonephritis of two months duration.complete evaluation including contrast CT of the abdomen a diagnosis of XGPN was made. As the renal unit in question was non functional, he was treated successfully by subcapsular nephrectomy. This case is presented to emphasise the fact that, it is very important to diagnose XGPN early and differentiate it from chronic pyelonephritis. Prolonged antibiotic therapy and surgery is invariably required to relieve the obstruction and to eradicate the infection in patients with XGPN.

Pielonefritis xantogranulomatosa en adultos: presentación de 15 casos clínicos / Xanthogranulomatous pyelonephritis in adults: presentation of 15 clinical cases

Reportar nuestra experiencia en pielonefritis xantogranulomatosa (PXG), para contribuir al conocimiento de la misma. Material y métodos: análisis retrospectivo de las nefrectomías de los pacientes con diagnóstico histopatológico de PXG, entre los años 2002 y 2010. Resultados: se estudiaron 15 casos, 12 mujeres y 3 varones, con una edad promedio de 46,7 años. Todos los pacientes presentaron evolución crónica, malestar general, dolor abdominal y/o lumbar. La fiebre, palidez conjuntival, presencia de litiasis renal y pérdida de peso, fueron síntomas frecuentes. El laboratorio de sangre mostró eritrosedimentación muy incrementada y leucocitosis. La orina se presentó con sedimento alterado mostrando: hematuria, piuria y bacteriuria. En menos de la mitad de los casos el urocultivo presentó desarrollo y en algunas oportunidades con flora polimicrobiana y discrepancia en las recuperaciones por nefrostomías y cultivos convencionales. Los estudios por imágenes mostraron un riñón aumentado de tamaño, con presencia de litiasis en su mayoría y diversas alteraciones estructurales. Conclusiones: la PXG es una enfermedad infrecuente, crónica, benigna infecciosa e inflamatoria que afecta al parénquima renal. Poses predilección por el sexo femenino de mediana edad. El diagnóstico se efectúa ante la sospecha clínica, los estudios de laboratorio, de imágenes y se confirma con la histopatología. El tratamiento definitivo es la nefrectomía...

To report our experience, to contribute to the knowledge about, Xanthogranulomatous Pyelonephritis (XGP). Material and Methods: Retrospective analysis of the nephrectomy of patients with histopathological diagnosis of XGP, between 2002 and 2010. Results: We studied 15 cases, 12 women and 3 men, with an avergage age of 46.7 years. All patientes had chronic evolution, malaise, abdominal pain and/or lumbar spine. Fever, pallor, presence of kidney stones and weight loss were common symptoms. Blood laboratory presented increased erythrocyte sedimentation rate and leukocytosis. The urine was presented showing altered sediment, hematuria, pyuria and bacteriuria. In less than half of the cases the urine culture showed developed, sometimes with polymicrobial flora and discrepancy in recoveries by nephrostomy and conventional cultures. Imaging studies showed an increased kidney size, resence of stones in the most cases and various structural abnormalities. Conclusions: XGP is rare, chronic, infectious and inflammatory beningn disease that affects renal parenchyma and usually presents in middle-aged female. Diagnosis is suspected by clinic, laboratory, imaging studies, and confirmed by histopathology. Nephrectomy is the definitive treatment...

Pielonefritis xantogranulomatosa: estudio retrospectivo de 8 casos / Xanthogranulomatous pyelonephritis: a retrospective study of 8 cases

Hemos revisado las características clínicas, radiológicas y patológicas de la pielonefritis xan-togranulomatosa junto con la evolución de la enfermedad y una revisión bibliográfica de la misma. Se realiza un estudio retrospectivo en nuestro centro, obteniendo una muestra de 8 casos confirmados por anatomía patológica a través de los que describimos las características clínicas y diagnósticas. De los 8 casos (1 varón y 7 mujeres), 6 de ellos debutaron como cólico renal complicado, 1 se presentó con astenia y pérdida de peso y el otro caso fue un hallazgo incidental. El diagnóstico inicial se realizó mediante ecografía en 2 casos y TC en todos los casos, siendo confirmados todos mediante estudio histopatológico.

We have revised the clinical, radiological and pathological characteristics of Xanthogranuloma-tous pyelonephritis (XGP), together with the evolution of the disease and a literature review of the same. A retrospective study was performed at our center, obtaining a sample of 8 pathologically confirmed cases which we used to describe the clinical and diagnostic features. Of the 8 cases (1 male and 7 female), 6 of them presented complicated renal colic, one presented asthenia and weight loss and the other case was an incidental finding. The initial diagnosis was achieved with ultrasonography in 2 of the cases and with CT in all of the cases, all being confirmed by a histopathological study.

Isolated Renal Mucormycosis in a Healthy Immunocompetent Patient: Atypical Presentation and Course

Isolated renal mucormycosis is rarely identified and has been described in only a handful of cases. We hereby report a case of isolated renal mucormycosis with an atypical presentation in an immunocompetent patient with no identifiable risk factors. A 30-year-old nondiabetic male presented with a poorly functioning right kidney with minimal constitutional symptoms. The patient underwent a right simple nephrectomy. Histopathology revealed necrotizing xanthogranulomatous pyelonephritis with mucormycosis. The postoperative period was uneventful and the patient was managed without any antifungal administration. We hereby emphasize that renal mucormycosis can affect immunocompetent healthy adults without any previously known risk factors and that asymptomatic patients with no evidence of fungemia or disseminated disease can be managed without administration of intravenous amphotericin.

Laparoscopic nephrectomy for xanthogranulomatous pyelonephritis: are there predictive factors for success?

OBJECTIVES: Laparoscopic nephrectomy for xanthogranulomatous pyelonephritis is currently associated with great operative difficulty and surgical complications. Herein, we report on our single-center experience and describe predictive factors for successfully accomplishing this procedure. METHOD: Between March 1998 and April 2010, 66 patients (27 men and 39 women) underwent laparoscopic nephrectomy for the treatment of a unilateral nonfunctioning kidney. These patients had previous diagnoses of renal chronic inflammation associated with calculi and previous pyonephrosis. All of the nephrectomies were performed using the transperitoneal approach, and a similar technique was used for radical nephrectomy. RESULTS: Laparoscopic nephrectomy for the treatment of renal chronic inflammation was successful in 58/66 cases (87.9%). Eight cases were converted to the open technique because of difficulty in progression, which was related to the discovery of dense adhesions in the hilar or perirenal region. One major (colonic lesion) and two minor (wound infection) complications occurred in the conversion group. A diagnosis of xanthogranulomatous pyelonephritis was confirmed pathologically for all of the specimens. Of the factors examined, a longitudinal renal length greater than 12 cm (laparoscopy group - 7.2±1.8 cm, versus open group - 13.6±1.5 cm; p<0.05) and time to access the renal vessels (laparoscopy group - 32±18 min, versus open group - 91±11 min; p<0.05) were associated with a higher conversion rate. Although the number of patients in the conversion group was small, the majority of these patients received right-sided nephrectomy. CONCLUSIONS: Laparoscopic nephrectomy for the treatment of xanthogranulomatous pyelonephritis is feasible and associated with low levels of morbidity. Factors including the time required to control the renal vessels, renal length and right-sided nephrectomy were associated with higher chances of conversion into an open procedure.

Masa abdominal: pielonefritisxantogranulomatosa, un caso inusual en pediatría / Abdominal mass: an unusual case of xanthogranulomatous pyelonephritis in pediatrics

La pielonefritis xantogranulomatosa es una forma grave y atípica de infección crónica del parénquima renal, frecuentemente subdiagnosticada, de presentación rara en la edad pediátrica.Los factores que comúnmente están asociados en el desarrollo de la enfermedad son laobstrucción y las infecciones del tracto urinario. La presentación clínica es inespecífica y variable,ocasionalmente puede semejar una masa de características tumorales. de otro lado requiere descartarabscesos y tuberculosis renal por lo que se constituye en un reto diagnóstico y terapéutico. Lapresentación unilateral es la más frecuente, mientras que la bilateral, extremadamente rara. Por logeneral la nefrectomía es el manejo de elección, con un excelente pronóstico si se realiza prontamente.Sin embargo, el manejo de la entidad es difícil, debido al riesgo de disfunción renal permanente.En este trabajo se hace el reporte de un niño de tres años de edad con diagnóstico de pielonefritis xantogranulomatosa, infección urinaria y urolitiasis. El diagnóstico fue hecho por patología en el período posoperatorio.Se hace referencia a las consideraciones clínicas,imaginológicas y quirúrgicas del caso, enfatizando en lapoblación pediátrica, en la cual se encuentran muy pocoscasos reportados en la literatura.

Xanthogranulomatous pyelonephritis (XGPN) is a severe form of chronic and atypical renal parenchymainfection, often underdiagnosed, andrare presentation in childhood. Factors that are often associated in the development of XGPNare genitourinary tract obstruction and urinary tract infections. Clinical presentation is nonspecificand variable, we must rule out a tumordisease, and be aware por renal abscess or renal TBC. The form is predominantly unilateral andbilateral is extremely rare. Usually nephrectomyis the choice management in unilateral XGPN,whit an excellent prognosis if done promptly. However, XGPN management is difficult becausethe risk of permanent renal dysfunction. We presenta case of a 3 year old boy with a diagnosis of xanthogranulomatous pyelonephritis, urinarytract infection and urolithiasis. Diagnosis wasmade by pathology in the postoperative period.We will refer to clinical, surgical and imagingconsiderations, of this case, of particular interestin the pediatric population, in which there are very few cases reported in the literature.

Diagnóstico y tratamiento de la pielonefritis xantogranulomatosa / Diagnosis and treatment of xanthogranulomatous pyelonephritis

La pielonefritis xantogranulomatosa es una enfermedad inflamatoria-infecciosa crónica que involucra el riñón. Generalmente se presenta en adultos, más comúnmente en las mujeres jóvenes, y se asocia con litiasis. En la mayoría de los casos es unilateral y se acompaña de destrucción parcial o total del parénquima renal. Los hallazgos más frecuentes del examen físico son: fiebre, dolor lumbar o abdominal o ambos, masa palpable, pérdida de peso y antecedentes de infecciones urinarias recurrentes. La tomografía computarizada es el método más comúnmente empleado para su diagnóstico. Inicialmente se la puede confundir con un tumor. En los análisis de laboratorio se observa anemia, eritrosedimentación muy acelerada, leucocitosis y piuria en el sedimento urinario. El diagnóstico de certeza es el histopatológico. El tratamiento antibiótico evita las complicaciones sépticas, pero la terapia de elección es la nefrectomía total o parcial. Nuestro objetivo es actualizar esta enfermedad infrecuente, tanto en sus formas de presentación, fisiopatogenia, manifestaciones clínicas, como su diagnóstico y tratamiento.

Clinical Features of 11 Patients with Xanthogranulomatous Pyelonephritis / 대한내과학회지

BACKGROUND/AIMS: Because preoperative diagnosis of xanthogranulomatous pyelonephritis (XGP) is difficult, due to its similarities to other renal diseases, the diagnosis is made postoperatively in most cases. The purpose of this study was to describe the clinical findings in 11 patients with histologically documented XGP. METHODS: We retrospectively reviewed the characteristics, laboratory and radiological findings, preoperative diagnoses and operative methods of 11 patients with XGP, who underwent a surgical procedure or percutaneous renal biopsy. RESULTS: Among eleven patients, nine had flank pain and six had anemia. Preoperatively, three patients were diagnosed as XGP, two with renal cell carcinoma, two with renal tuberculosis, one with renal abscess, one with perirenal abscess, one with renal staghorn calculi with non-functioning kidney, and one with pyelonephrosis. On the basis of the computed tomography (CT) features, the diffuse or global forms (70.0%) were more common than the localized or focal forms (30.0%). One patient diagnosed with renal cell carcinoma preoperatively was diagnosed as XGP through an intraoperative frozen section renal tissue biopsy and underwent partial nephrectomy. One patient diagnosed as focal XGP underwent percutaneous biopsy of the renal mass, which confirmed the diagnosis. This patient received treatment with only antibiotic therapy. CONCLUSIONS: CT can be considered the preferred diagnostic tool for the evaluation of XGP; however, percutaneous renal biopsy seems to be valuable in selected cases for differential diagnosis of renal malignancy.

Xanthogranulomatous Pyelonephritis in Korean Children

PURPOSE: Xanthogranulomatous pyelonephritis (XGP) is rare among children. In most cases, XGP is diffusely or focally enlarged, mimicking the neoplastic process. The aim of this study was to examine clinical characteristics and outcomes of Korean children with XGP. MATERIALS AND METHODS: Fourteen children (9 boys, 5 girls) with XGP were reviewed retrospectively. The cohort included 2 children managed at our institution and 12 children reported in the Korean literature. The patients' records were reviewed with respect to age at diagnosis, clinical presentation, management method, and other characteristic features. RESULTS: The mean age was 79.4+/-66.5 months (range 1-168 months). Common clinical presentations included fever (85.7%), abdominal pain (57.1%), and palpable mass (28.6%). Laboratory abnormalities included leukocytosis (57.1%), anemia (57.1%), and pyuria (57.1%). The types of XGP that were diagnosed based on preoperative radiologic studies included the focal form in 9 children and the diffuse form in 5. Thirteen children underwent nephrectomy, and 1 child received conservative medical therapy. CONCLUSION: The possibility of XGP should be considered if a child is diagnosed with a renal mass, especially if it is a small renal mass associated with fever, leukocytosis, or stone. Nephrectomy is the treatment of choice for the diffuse form, whereas partial nephrectomy or conservative medical therapy may be indicated to manage focal XGP.