Pediatric Radiology
Pediatr. Radiol, 8, 10-16 (1979)
9 bySpringer-Verlag1979
Xanthogranulomatous Pyelonephritis in Childhood Report of Three Cases and a Review of the Literature K. Fahr, H. C. Oppermann, K. Schfirer, and I. Greinacher Departments of Pediatric Radiology and Nephrology, University Children's Hospital, Heidelberg and Department of Pediatric Radiology, University Children's Hospital, Mainz, FRG
Abstract.
Xanthogranulomatous pyelonephritis (XPN) is a rare, unilateral manifestation of chronic pyelonephritis. It is characterized by extensive damage to the parenchyma and its replacement by xanthogranulomatous tissue. The main roentgenological feature is a functionless kidney, often with calcification. The clinical, pathological and roentgenological findings of our three patients are analyzed and compared with previously reported findings, most notably those in childhood. A tentative diagnosis of XPN can be made from intravenous urography and echography of the kidney. In general, angiography does not provide additional pathognomonic features. The final diagnosis of X P N can only be made by histological microscopic examination. The prognosis of this disease is considered to be good, when the affected kidney is removed and function on the contralateral side is good.
Key words: Xanthogranulomatous pyelonephritis T u m o r - Nonfunctioning kidney - Foam cells - A n giography - Ultrasonography
Case Reports The salient aspects of the clinical features and roentgenological findings of our three patients are shown in Table 1; recurrent fever, a palpable abdominal tumor and leucocyturiawere characteristic. The urograms showed an unilateral nonfunctioning kidney with calcification. The diagnoses were confirmedby a histological examination. Recovery after nephrectomy was invariably uneventful.
Review of Literature All cases documented in the world literature are summarized in Table 2. The most important traits were as follows: 1. Recurrent fever was found in 24 of 34 patients; 2. An abdominal tumor was palpable in 6 cases; 3. In 14 cases Proteus vulgaris, in 7 Escherichia coli and in 2 cases Pseudomonasaeruginosacould be cultured in urine samples; 4. The main roentgenologicalsign was a nonfunctioningkidney in 24 children and in 17 of these intrarenal calcification was also found. The lesion was equally common on both sides. 5. Girls were more often affected than boys (19 girls, 15 boys),
Discussion Xanthogranulomatous pyelonephritis (XPN), first described by Schlagenhaufer (29) in 1916, is an unusual type of pyelonephritis. It has been observed mainly in female adults. Over the years some cases have been reported in the pediatric literature [1, 3, 4, 5, 6, 8, 13, 14, 15, 16, 19, 21, 24, 28, 30, 31]. Schulman [30] published a comprehensive report about XPN in childhood. He reported one case of his own and summarized 21 other cases from the literature. We report three further cases of X P N and compare our findings with those previously published.
0301-0449/79/0008/0010/$01.40
XPN is a special form of a chronic renal disease. It is characterized by an extensive chronic destruction of the renal parenchyma which is replaced by granulation tissue, abscesses and lipid foam cells. U p to 1971 only 90 cases of this disease, all adults, had been described in the literature. Female patients predominated. Only a few pediatric cases are on record. However, this disease may develop even in early childhood. The youngest patient described in the literature was 9 months old [5]. Most cases have a diag-
K. Fahr et al.: Xanthogranulomatous Pyelonephritis
11
Table L Clinical data and roentgenological findings in three own cases with XPN Patient 1
Patient 2
Patient 3
9
cy
cf
1 45/12 Recurrent fever, growth retardation
1 2~/~_, Recurrent fever, leucocyturia
16k2 la/,2 Loss of appetite, unknown fever, anemia
Hepatomegaly, palp. tumor in left renal bed 100/70
Hepatomegaly, painful, palp. tumor in right renal bed 105/60
Visuable and palp. tumor in the left abdomen 100/70
36/67 17 000 Normal Alb. + leucocyturia
71/82 19 600 Normal Alb. + leucocyturia
116/159 21600 Normal Alb. + leucocyturia
Bact. findings
Proteus vulgaris
Proteus vulgaris
-
Urography
Nonfunctioning left kidney, calcifications (Fig. la/1 b)
Nonfunctioning right kidney, calcifications
Nonfunctioning left kidney, calcifications (Fig. 2 a)
Arreriography
-
-
Atypical course of blood vessels, no neovascularity (Fig. 2 b)
Echography
Internal echoes
-
Internal echoes (Fig. 3)
Histological examination
Xanthogranulomatous pyelonephritis (Fig. 4)
Xanthogranulomatous pyelonephritis
Xanthogranulomatous pyelonephritis
Therapy
Nephrectomy
Nephrectomy
Nephrectomy
Clinical course
No symptoms
No symptoms
No symptoms
Sex Onset of first symptoms (years) Age at diagnosis Clinical symptoms Clinical findings RR (mmHg) Laboratory findings Sedimentation Rate (ram) Leucocytes Creatinin und urea Urine
Table 2. Clinical data and roentgenological findings of children with XPN reported in the literature Author and year
Age (years) and sex
Symptoms
Avnet 1963
9,6 F
Recurrent fever
Friedenberg 1963
13 F
Black 1968
5F
Habib 1968
2,11 M
Habib 1968
2,6 M
Habib 1968
0,11 F
Habib 1968
4,7 M
Hemet 1969
4M
Hemet 1969
4F
Lackner 1969
6F
Urography
Mass lesion in the upper pole of right kidney Recurrent fever, Mass in upper pole of abdominal pain enlarged left kidney Fever, pyuria, ab- Lower pole and middominal pain dle calyceal system stretched by left intrarenal mass Recurrent fever, Non-visualized left pyuria kidney Proteinuria, leuNon-Visualized right cocyturia kidney Fever, urinary No contrast of right tract infections kidney Polydipsy Non-visualized right kidney Dysuria No function of left kidney Fever, palpable Non-visualized right tumor on the right kidney side Fever, leucocyMass in the upper turia albuminuria third of left kidney
Bacteriology
Calcification
Side
E. coli
-
Right
Prot. vulgaris
-
Left
Negative
-
Left
Prot. vulgaris
-
Right
Prot. vulgaris
+
Left
Prot. vulgaris
+
Right
Prot. vulgaris
+
Right
Prot. vulgaris
+
Left
Prot. vulgaris
+
Right
-
-
Left
12
K. Fahr et al.: Xanthogranulomatous Pyelonephritis
Table 2 (continued) Author and year
Age (years) and sex
Symptoms
Urography
Bacteriology
Calcification
Side
Souto 1969
10 M
Enlarged left kidney
E. colt
-
Left
Ceccarelli 1970
1,5 F
Recurrent fever, pyuria Recurrent fever
Staphylococcus aureus
--
Left
Hardy 1971
3F
Prot. vulgaris
+
left
Kessler 1971
7M
Expanding mass in the inf. pole of left kidney Non-visualizedleft kidney No nephrographic effect
E. colt
+
Right
Habib 1972
2,6F
Prot. vulgaris
+
Right
Habib 1972
1,8 M
+
Right
Graivier 1972
3F
Staphylococcus aureus
-
Left
Mering 1973
0,11 F
Klebsiella
-
Left
Bruezi6re 1974
1,5 F
Pyocyaneus
-
Right
Bruezi~re 1974
3,6 M
-
-
Left
Bruezi6re 1974
10 F
Prot. vulgaris
+
Left
Bruezi6re 1974
3,6 M
Prot. vulgaris
+
Right
Bruezi6re 1974
0,4 F
E. coli
-
Left
Bruezi6re 1974
0,9 F
Prot. vulgaris
+
Right
Habib 1974
6,4 F
Prot. vulgaris
+
Right
Habib 1974
1,4 M
Pyocyaneus
-
Left
Habib 1974
0,13 F
E. coil
+
Right
Gammil11975
14 M
E. coli
-
Right
Shanser 1975
4F
-
-
Left
Abbate 1976
7F
E. coli
-
Left
Schulman 1976
2,10 M
Prot. vulgaris
-
Right
Schulman 1976
3M
Prot. vulgaris
+
Right
Klugo 1977
10 F
E. coli
+
Left
Schindera 1977
4M
Prot. mirabilis
-
Right
Fever, abdominal tumor Recurrent fever, palp. abdom, tumor Haematuria
Non-functioning right kidney Pyuria Non-functioning right kidney Fever, palpable Displacement of left mass on the left kidney by a retropcriflank toneal mass Recurrent urinary No nephrographic eltract infections fect of left kidney Fever, urinary Non-visualized right tract infection kidney Recurrent fever Non-functioning right kidney Recurrent fever Enlarged and nonfunctioning left kidney Palpable mass on No nephrographic efthe right flank fect of right kidney Fever, pyuria Non-functioning left kidney, ureterocele Fever No nephrographic effect of the right kidney Recurrent fever Non-functioning right kidney Recurrent fever Non-functioning left kidney Pain in the right Non-functioning right renal bed kidney Fever, pyuria Non-functioning right kidney Infections of the Enlargement of the urinary tract upper pole of the left kidney Recurrent fever Non-visualization of the lower pole of the left kidney Recurrent fever Non-functioning of the right kidney Fever, palpable Non-functioning of tumor in the abthe right kidney domen Recurrent urinary Left upper pole renal tract infections mass Micro-haemaNon-functioning of turia, leucocyturia the right kidney
K. Fahr et al.: Xanthogranulomatous Pyelonephritis
13
Fig. 1. a Intravenous urogram (15 rain. film): Large but nonfunctioning left kidney. Right kidney normal, right ureter shows a streaked pattern, b Nephrotomogram (25 rain. post infection): Streaky calcifications in the left kidney
Fig. 2. a Abdomen supine position (tomogram)." Staghorn calculus in the left ureteropelvic junction, two further intrarenal calculi, b Selective renal arteriogram (left)." Stretched, curved vessels at the upper pole but no neovascularity nostic delay of a few months to several years [5, 11, 14, 21]. Our three patients showed a diagnostic delay of I/2-21/2 years before the diagnosis was made. Clinical s y m p t o m s of X P N are mainly recurrent fever, dysuria, leucocyturia and sometimes an unilateral palpable t u m o r (table 2). As a rule the general condition of children with X P N is poor. A n e m i a and loco-
m o t o r retardation indicate the chronic course of the disease [5, 6, 13, 14, 26, 30, 31]. Causative bacteria are usually Proteus vulgarls and E. coli and in some cases Staphylococcus aureus [5, 9, 10, 30]. Proteus vulgaris was also found in two of our patients. The etiology of this disease is still obscure. A dis-
K. Fahr et al.: Xanthogranulomatous Pyelonephritis
14 ventral
Longitudinal section
Fig. 3. B-mode-ultrasonogram (longitudinal section); Enlarged left kidney with multiple internal echoes; ovoid form of the middle echo. - Splitting of the middle echo:_ Non characteristic echo mass (staghorn calculus), i = kidney - ~ -- middle echo - ~ = splitting
of the middle-echo
I
turbance of the lipid metabolism has been considered as a possible cause. Laboratory examinations of lipid and cholesterin levels in blood, and function tests to exclude a latent diabetes mellitus in patients with the disease have been unable to demonstrate any dysfunction of metabolism [9, 13, 18]. Insufficient antibiotic treatment for urinary tract infections and obstruction in the urinary tract have also been suggested as possible etiological factors [26]. Habib and co-workers [14, 15] believe that the primary lesion could be an ischaemic papillary necrosis with subsequent calcification or development of concretions. If this view is correct the xanthogranulomatous reaction of the tissue and chronic pyelonephritis must be regarded as secondary phenomena. It is doubtful if pre-, peri or postnatal hypoxemia could be responsible for these changes. Finally Klugo [21] considered a transitory immundeficiency as a possible pathogenetic factor. Radiographic findings: The intravenous urogram (IVU) demonstrates no specific features in XPN. A frequent roentgenological finding is an unilateral nonfunctioning and enlarged kidney such as we could demonstrate in our patients [3, 4, 5, 6, 9, 10, 13, 22, 23, 27, 34]. Sometimes only one pole of the kidney may be affected [5, 7, 9, 19, 13, 31, 33]. Hydro- or pyonephrosis, a kidney-tumor and an unilateral renal vein thrombosis may be considered in differential
Fig. 4..Microscopic finding (1 ,'535, HE stain&g/." Multiple lipid-storing foam cells 1 Institute of Pathology, University Heidelberg (Director: Prof. Dr. W. Doerr)
K. Fahr et al.: Xanthogranulomatous Pyelonephritis
diagnosis. Very often intrarenal calcification develops, as in our three patients [7, 10, 13, 31, 33]. Noyes [25] found in four of five patients and Bruezi6re [5] in three of six cases a nonfunctioning kidney with obstruction at the ureteropelvic junction by a stone; our third patient had this finding. If intrarenal calcification is present, renal-carcinoma, teratoma, infected hamartoma, renal tuberculosis and calcified renal cysts have to be considered in the differential diagnosis. A kidney affected by XPN could be mistaken for a Wilms' tumor, but intrarenal calcification in Wilms' tumor is well known to be rare (5-10%). There are no specific angiographic features in XPN [2, 7, 10, 31]. The vessels may be displaced and sometimes distorted vessels may be found in the involved areas. In our third patient only stretching and a curved course of vessels in the involved kidney could be demonstrated (Fig. 2 b). The good vascularisation was in contrast to nonfunction of the same kidney at IVU, even when followed up for 10 hours. We can give no explanation for this phenomenon. Gammill [10] differentiates, angiographically, a parenchymatous and vascular type of XPN. Arterio-venous anastomoses or early filling of the intrarenal veins are not found in XPN. In general the vascularisation of the XPN-kidney is poorer than in Wilms' tumor or hypernephroid carcinoma [10]. This finding correlates well with the cortical atrophy shown by microscopy. In the diagnosis of XPN, echography has no advantage over radiography, but it always provides important additional information. This is of special value in childhood, because it is a nonaggressive examination [12, 20]. Shanser and Klugo found multiple internal echoes in two patients examined with B-mode echography. We also found a similar state in two of our patients. How far this echographic state is specific for XPN can only be shown through further investigations using this method. Contrary to Schindera [28], we believe that in the diagnosis of XPN, the combined use of intravenous urography and renal echography can strongly suggest the diagnosis XPN. Arteriography gives an additional information, but it is invasive. However, the final diagnosis of XPN rests on the pathological examination. Macroscopically the renal pelvis and calyces are dilated and filled with purulent material and concretions. Both renal cortex and medulla are considerably atrophied and contain multiple abscesses. The renal papillae are mainly necrotic. In most cases the interstitial inflammation spreads to the renal capsule and the perirenal tissue. Microscopic examination shows an acute nephritis and chronic destructive pyelonephritis. The most impor-
15
tant sign is the xanthogranulomatous reaction, which is characterized by necrotic granulation tissue and lipid filled macrophages (foam cells). In addition there are many lymphocytes, plasmocytes, polymorphonuclear cells and granulomata with giant cells. The glomeruli show a segmental and general hyalinosis. Endarteritis and endophlebitis are occasionally found in the renal vessels [3, 9, 14, 15]. Generally, XPN can be differentiated from a renal tumor or renal tuberculosis without any difficulty at all. Treatment for XPN consists of nephrectomy and the removal of the adherent lymph-nodes and the ureter. All the cases described so far have been cured by nephrectomy. The prognosis of this disease is considered to be good. Addendum. 1977 Harzmann reported on two further pediatric patients with XPN and Bagley published a diffuse XPN in children for the first time.
References 1. Abbate, A.D., Meyers, J.: Xanthogranulomatous pyelonephritis in childhood. J. Urol. 160, 231 (1976) 2. Anhalt, M. A., Cawood, C.D., Scott, R.: Xanthogranulomatous pyelonephritis: A comprehensive review with report of 4 additional cases. J. Urol. 105, 10 (1971) 3. Avnet, N.L., Roberts, Th. W,, Goldberg, H. R.: Tumefactive xanthogranulomatous pyelonephritis, Am. J. Roentgenol. 90, 89 (1963) 4. Black, W. C., Ragsdale, E. F.: Wilms' tumor. Am. J. Roentgenol. 103, 53 (1968) 5. Brueziere, J., Begue, P., Polani, C., Boccon-Gibod, L.: Les pydlon6phrites xantogranulomateuses diffuses de l'enfant. J. Urol. Nephrol. (Paris) 80, 13 (1974) 6. Ceccarelli, F. E., Wurster, J. C., Chandor, S. B.: Xanthogranulomatous pyelonephritis in an infant. J. Urol. 104, 755 (1970) 7. Delsen, J., Hardmeier, Th.: Die xanthogranulomat6se Pyelonephritis. Helv. Chir. Acta 43, 363 (1976) 8. Egli, F., Jenny, P., Schindera, F., Mihatsch, M. J.: Xanthogranulomat0se Pyelonephritis. Jahresversammlung der Schweizerischen Gesellschaft fiir Pfidiatrie, Locarno 1977 9. Friedenberg, M. J., Spjut, H. J.: Xanthogranulomatous pyelonephritis. Am. J. Roentgenol. 90, 97 (1963) 10. Gammill, S., Rabinowitz, J. G., Peace, R., Sorgen, S., Hurwitz, L., Himmelfarb, E.: New thoughts concerning xanthogranulomatous pyelonephritis (X-P). Am. J. Roentgenol. 125, 154 (1975) 11. Gingell, J. C., Roylance, J., Davies, E. R., Penry, J. B.: Xanthogranulomatous pyelonephritis. Br. J. Radiol. 46, 99 (1973) 12. Goldberg, B. B., Pollack, H. M.: Differentiation of renal masses using A-mode ultrasound. J. Urol. 105, 765 (1971) 13. Graivier, L., Vargas, M.A.: Xanthogranulomatous pyelonephritis in childhood. Am. J. Dis. Child. 123, 156 (1972) 14. Habib, R., Levy, M.: Py61on6phrite xanthogranulomateuse. In: Royer, P., Habib, R., Matthieu, H.: Nephrologie P6diatrique. Paris: Flammarion 1972 15. Habib, R., Levy, M., Royer, P.: La py61on6phrite xanthogranulomateuse chez l'enfant. Arch. Fr. Pediatr. 25, 489 (1968) 16. Hardy, J. C., Ardichvili, D.: Un cas de py61on6phrite xanthogranulomateuse chez l'enfant. Acta Urol. Belg. 39, 509 ( 1971)
16 17. Hemet, J., Morin, C., Fondimare, A., Borde, J., Mitrofanoff, B., Menibus, CI. H.: La py61on6phrite xanthogranulomateuse de l'enfant. Apropos de deux observations. Arch. Anat. Cytol. Pathol. 17, 193 (1969) 18. Hooper, R. G., Kempson, R. L., Schlegel, J. V.: Xanthogranulomatous pyelonephdtis. J. Urol. 88, 585 (1962) 19. Kessler, E., Grunebaum, M., Lazebnik, J.: Xanthogranulomatous pyelonephritis in childhood with ascaris lumbricoides infestation. Path. Microbiol. (Basel) 37, 401 (1971) 20. King, D.L.: Renal ultrasonography. Radiology 105, 633 (1972) 21. Klugo, R.C., Anderson, J.A., Reid, R., Rowell, I., Cemy, J.C.: Xanthogranulomatous pyelonephritis in children. J. Urol. 117, 350 (1977) 22. Lackner, H., Wolfel, D., Banowsky, L., Kornfeld, M.: Xanthogranulomatous pyelonephritis. J. Pediatr. 75, 482 (1969) 23. McCullough, D. L., Tiguor, M. R.: Xanthogranulomatous pyelonephritis. Am. J. Med. 52, 395 (1972) 24. Mering, J. H., Kaplan, G. W., Mc Laughlin, A. P.: Xanthogranulomatous pyelonephritis. Unusual clinical presentations. Urology 1, 338 (1973) 25. Noyes, E.W., Palubinskas, A.J.: Xanthogranulomatous pyelonephritis. J. Urol. 101, 132 (1969) 26. Parker, J.M.: Xanthogranulomatous pyelonephritis. J. Urol. 96, 290 (1966) 27. Saeed, S.M., Fine, G.: Xanthogranulornatous pyelonephritis. Am. J. Clin. Pathol. 39, 616 (1963) 28. Schindera, F., Rohrbach, R., Limberg, J.: Xanthogranulomat6se Pyelonephritis bei einem Kleinkind. Klin. Piidiatr. 189, 277 (1977) 29. Schlagenhaufer, F.: Uber eigentiimliche Staphylomykosen der Nieren und des pararenalen Bindegewebes. Frankf. Z. Pathol. 19, 139 (1916)
K. Fahr et al.: Xanthogranulomatous Pyelonephritis 30. Schulman, C.C., Denis, R.: Py61on6phrite xanthogranulomateuse diffuse chez l'enfant. Ann. Chir. Inf. 17, 251 (1976) 31. Shanser, J.D., Herzog, K. A., Palubinskas, A. J.: Xanthogranulomatous pyelonephritis in childhood. Pediatr. Radiol. 3, 12 (1975) 32. Souto, C. A. V., Monteiro, S. A., Andrade, L. C.: Xanthogranulomatous pyelonephritis: 2 case reports. J. Urol. 102, 393 (1969) 33. Strasberg, J., Jacobson, S.A., Srolovitz, H., Sedlezky, H., Schneiderman, C.: Xanthogranulomatous pyelonephritis- radiologicconsiderations. J. Can. Assoc. Radiol. 21, 173 (1970) 34. Vfizquez, J.J., Herranz, G.: Pie!onefritis xanthogranulomatosa. Rev. IVied.Univ. Navarra 9, 127 (1965) Addendum
Harzmann, R., Bichler, K. H., Schmitz-Moormann, P., Erdmann, D.: Xanthogranulomat6se Pyelonephritis. Akt. Urol. 8, 319 (1977) Bagley, F. H., Stewart, A. M., Jones, P. F.: Diffuse xanthogranulomatous pyelonephritis in children: an unrecognized variant. J. Urol. 118, 434 (1977) Date of final acceptance: March 14, 1978
Dr. K. Fahr R/Sntgenabteilung Universit~its-Kinderklinik Im Neuenheimer Feld 150 D-6900 Heidelberg 1 Federal Republic of Germany
Pediatr. Radiol, 8, 10-16 (1979)
9 bySpringer-Verlag1979
Xanthogranulomatous Pyelonephritis in Childhood Report of Three Cases and a Review of the Literature K. Fahr, H. C. Oppermann, K. Schfirer, and I. Greinacher Departments of Pediatric Radiology and Nephrology, University Children's Hospital, Heidelberg and Department of Pediatric Radiology, University Children's Hospital, Mainz, FRG
Abstract.
Xanthogranulomatous pyelonephritis (XPN) is a rare, unilateral manifestation of chronic pyelonephritis. It is characterized by extensive damage to the parenchyma and its replacement by xanthogranulomatous tissue. The main roentgenological feature is a functionless kidney, often with calcification. The clinical, pathological and roentgenological findings of our three patients are analyzed and compared with previously reported findings, most notably those in childhood. A tentative diagnosis of XPN can be made from intravenous urography and echography of the kidney. In general, angiography does not provide additional pathognomonic features. The final diagnosis of X P N can only be made by histological microscopic examination. The prognosis of this disease is considered to be good, when the affected kidney is removed and function on the contralateral side is good.
Key words: Xanthogranulomatous pyelonephritis T u m o r - Nonfunctioning kidney - Foam cells - A n giography - Ultrasonography
Case Reports The salient aspects of the clinical features and roentgenological findings of our three patients are shown in Table 1; recurrent fever, a palpable abdominal tumor and leucocyturiawere characteristic. The urograms showed an unilateral nonfunctioning kidney with calcification. The diagnoses were confirmedby a histological examination. Recovery after nephrectomy was invariably uneventful.
Review of Literature All cases documented in the world literature are summarized in Table 2. The most important traits were as follows: 1. Recurrent fever was found in 24 of 34 patients; 2. An abdominal tumor was palpable in 6 cases; 3. In 14 cases Proteus vulgaris, in 7 Escherichia coli and in 2 cases Pseudomonasaeruginosacould be cultured in urine samples; 4. The main roentgenologicalsign was a nonfunctioningkidney in 24 children and in 17 of these intrarenal calcification was also found. The lesion was equally common on both sides. 5. Girls were more often affected than boys (19 girls, 15 boys),
Discussion Xanthogranulomatous pyelonephritis (XPN), first described by Schlagenhaufer (29) in 1916, is an unusual type of pyelonephritis. It has been observed mainly in female adults. Over the years some cases have been reported in the pediatric literature [1, 3, 4, 5, 6, 8, 13, 14, 15, 16, 19, 21, 24, 28, 30, 31]. Schulman [30] published a comprehensive report about XPN in childhood. He reported one case of his own and summarized 21 other cases from the literature. We report three further cases of X P N and compare our findings with those previously published.
0301-0449/79/0008/0010/$01.40
XPN is a special form of a chronic renal disease. It is characterized by an extensive chronic destruction of the renal parenchyma which is replaced by granulation tissue, abscesses and lipid foam cells. U p to 1971 only 90 cases of this disease, all adults, had been described in the literature. Female patients predominated. Only a few pediatric cases are on record. However, this disease may develop even in early childhood. The youngest patient described in the literature was 9 months old [5]. Most cases have a diag-
K. Fahr et al.: Xanthogranulomatous Pyelonephritis
11
Table L Clinical data and roentgenological findings in three own cases with XPN Patient 1
Patient 2
Patient 3
9
cy
cf
1 45/12 Recurrent fever, growth retardation
1 2~/~_, Recurrent fever, leucocyturia
16k2 la/,2 Loss of appetite, unknown fever, anemia
Hepatomegaly, palp. tumor in left renal bed 100/70
Hepatomegaly, painful, palp. tumor in right renal bed 105/60
Visuable and palp. tumor in the left abdomen 100/70
36/67 17 000 Normal Alb. + leucocyturia
71/82 19 600 Normal Alb. + leucocyturia
116/159 21600 Normal Alb. + leucocyturia
Bact. findings
Proteus vulgaris
Proteus vulgaris
-
Urography
Nonfunctioning left kidney, calcifications (Fig. la/1 b)
Nonfunctioning right kidney, calcifications
Nonfunctioning left kidney, calcifications (Fig. 2 a)
Arreriography
-
-
Atypical course of blood vessels, no neovascularity (Fig. 2 b)
Echography
Internal echoes
-
Internal echoes (Fig. 3)
Histological examination
Xanthogranulomatous pyelonephritis (Fig. 4)
Xanthogranulomatous pyelonephritis
Xanthogranulomatous pyelonephritis
Therapy
Nephrectomy
Nephrectomy
Nephrectomy
Clinical course
No symptoms
No symptoms
No symptoms
Sex Onset of first symptoms (years) Age at diagnosis Clinical symptoms Clinical findings RR (mmHg) Laboratory findings Sedimentation Rate (ram) Leucocytes Creatinin und urea Urine
Table 2. Clinical data and roentgenological findings of children with XPN reported in the literature Author and year
Age (years) and sex
Symptoms
Avnet 1963
9,6 F
Recurrent fever
Friedenberg 1963
13 F
Black 1968
5F
Habib 1968
2,11 M
Habib 1968
2,6 M
Habib 1968
0,11 F
Habib 1968
4,7 M
Hemet 1969
4M
Hemet 1969
4F
Lackner 1969
6F
Urography
Mass lesion in the upper pole of right kidney Recurrent fever, Mass in upper pole of abdominal pain enlarged left kidney Fever, pyuria, ab- Lower pole and middominal pain dle calyceal system stretched by left intrarenal mass Recurrent fever, Non-visualized left pyuria kidney Proteinuria, leuNon-Visualized right cocyturia kidney Fever, urinary No contrast of right tract infections kidney Polydipsy Non-visualized right kidney Dysuria No function of left kidney Fever, palpable Non-visualized right tumor on the right kidney side Fever, leucocyMass in the upper turia albuminuria third of left kidney
Bacteriology
Calcification
Side
E. coli
-
Right
Prot. vulgaris
-
Left
Negative
-
Left
Prot. vulgaris
-
Right
Prot. vulgaris
+
Left
Prot. vulgaris
+
Right
Prot. vulgaris
+
Right
Prot. vulgaris
+
Left
Prot. vulgaris
+
Right
-
-
Left
12
K. Fahr et al.: Xanthogranulomatous Pyelonephritis
Table 2 (continued) Author and year
Age (years) and sex
Symptoms
Urography
Bacteriology
Calcification
Side
Souto 1969
10 M
Enlarged left kidney
E. colt
-
Left
Ceccarelli 1970
1,5 F
Recurrent fever, pyuria Recurrent fever
Staphylococcus aureus
--
Left
Hardy 1971
3F
Prot. vulgaris
+
left
Kessler 1971
7M
Expanding mass in the inf. pole of left kidney Non-visualizedleft kidney No nephrographic effect
E. colt
+
Right
Habib 1972
2,6F
Prot. vulgaris
+
Right
Habib 1972
1,8 M
+
Right
Graivier 1972
3F
Staphylococcus aureus
-
Left
Mering 1973
0,11 F
Klebsiella
-
Left
Bruezi6re 1974
1,5 F
Pyocyaneus
-
Right
Bruezi~re 1974
3,6 M
-
-
Left
Bruezi6re 1974
10 F
Prot. vulgaris
+
Left
Bruezi6re 1974
3,6 M
Prot. vulgaris
+
Right
Bruezi6re 1974
0,4 F
E. coli
-
Left
Bruezi6re 1974
0,9 F
Prot. vulgaris
+
Right
Habib 1974
6,4 F
Prot. vulgaris
+
Right
Habib 1974
1,4 M
Pyocyaneus
-
Left
Habib 1974
0,13 F
E. coil
+
Right
Gammil11975
14 M
E. coli
-
Right
Shanser 1975
4F
-
-
Left
Abbate 1976
7F
E. coli
-
Left
Schulman 1976
2,10 M
Prot. vulgaris
-
Right
Schulman 1976
3M
Prot. vulgaris
+
Right
Klugo 1977
10 F
E. coli
+
Left
Schindera 1977
4M
Prot. mirabilis
-
Right
Fever, abdominal tumor Recurrent fever, palp. abdom, tumor Haematuria
Non-functioning right kidney Pyuria Non-functioning right kidney Fever, palpable Displacement of left mass on the left kidney by a retropcriflank toneal mass Recurrent urinary No nephrographic eltract infections fect of left kidney Fever, urinary Non-visualized right tract infection kidney Recurrent fever Non-functioning right kidney Recurrent fever Enlarged and nonfunctioning left kidney Palpable mass on No nephrographic efthe right flank fect of right kidney Fever, pyuria Non-functioning left kidney, ureterocele Fever No nephrographic effect of the right kidney Recurrent fever Non-functioning right kidney Recurrent fever Non-functioning left kidney Pain in the right Non-functioning right renal bed kidney Fever, pyuria Non-functioning right kidney Infections of the Enlargement of the urinary tract upper pole of the left kidney Recurrent fever Non-visualization of the lower pole of the left kidney Recurrent fever Non-functioning of the right kidney Fever, palpable Non-functioning of tumor in the abthe right kidney domen Recurrent urinary Left upper pole renal tract infections mass Micro-haemaNon-functioning of turia, leucocyturia the right kidney
K. Fahr et al.: Xanthogranulomatous Pyelonephritis
13
Fig. 1. a Intravenous urogram (15 rain. film): Large but nonfunctioning left kidney. Right kidney normal, right ureter shows a streaked pattern, b Nephrotomogram (25 rain. post infection): Streaky calcifications in the left kidney
Fig. 2. a Abdomen supine position (tomogram)." Staghorn calculus in the left ureteropelvic junction, two further intrarenal calculi, b Selective renal arteriogram (left)." Stretched, curved vessels at the upper pole but no neovascularity nostic delay of a few months to several years [5, 11, 14, 21]. Our three patients showed a diagnostic delay of I/2-21/2 years before the diagnosis was made. Clinical s y m p t o m s of X P N are mainly recurrent fever, dysuria, leucocyturia and sometimes an unilateral palpable t u m o r (table 2). As a rule the general condition of children with X P N is poor. A n e m i a and loco-
m o t o r retardation indicate the chronic course of the disease [5, 6, 13, 14, 26, 30, 31]. Causative bacteria are usually Proteus vulgarls and E. coli and in some cases Staphylococcus aureus [5, 9, 10, 30]. Proteus vulgaris was also found in two of our patients. The etiology of this disease is still obscure. A dis-
K. Fahr et al.: Xanthogranulomatous Pyelonephritis
14 ventral
Longitudinal section
Fig. 3. B-mode-ultrasonogram (longitudinal section); Enlarged left kidney with multiple internal echoes; ovoid form of the middle echo. - Splitting of the middle echo:_ Non characteristic echo mass (staghorn calculus), i = kidney - ~ -- middle echo - ~ = splitting
of the middle-echo
I
turbance of the lipid metabolism has been considered as a possible cause. Laboratory examinations of lipid and cholesterin levels in blood, and function tests to exclude a latent diabetes mellitus in patients with the disease have been unable to demonstrate any dysfunction of metabolism [9, 13, 18]. Insufficient antibiotic treatment for urinary tract infections and obstruction in the urinary tract have also been suggested as possible etiological factors [26]. Habib and co-workers [14, 15] believe that the primary lesion could be an ischaemic papillary necrosis with subsequent calcification or development of concretions. If this view is correct the xanthogranulomatous reaction of the tissue and chronic pyelonephritis must be regarded as secondary phenomena. It is doubtful if pre-, peri or postnatal hypoxemia could be responsible for these changes. Finally Klugo [21] considered a transitory immundeficiency as a possible pathogenetic factor. Radiographic findings: The intravenous urogram (IVU) demonstrates no specific features in XPN. A frequent roentgenological finding is an unilateral nonfunctioning and enlarged kidney such as we could demonstrate in our patients [3, 4, 5, 6, 9, 10, 13, 22, 23, 27, 34]. Sometimes only one pole of the kidney may be affected [5, 7, 9, 19, 13, 31, 33]. Hydro- or pyonephrosis, a kidney-tumor and an unilateral renal vein thrombosis may be considered in differential
Fig. 4..Microscopic finding (1 ,'535, HE stain&g/." Multiple lipid-storing foam cells 1 Institute of Pathology, University Heidelberg (Director: Prof. Dr. W. Doerr)
K. Fahr et al.: Xanthogranulomatous Pyelonephritis
diagnosis. Very often intrarenal calcification develops, as in our three patients [7, 10, 13, 31, 33]. Noyes [25] found in four of five patients and Bruezi6re [5] in three of six cases a nonfunctioning kidney with obstruction at the ureteropelvic junction by a stone; our third patient had this finding. If intrarenal calcification is present, renal-carcinoma, teratoma, infected hamartoma, renal tuberculosis and calcified renal cysts have to be considered in the differential diagnosis. A kidney affected by XPN could be mistaken for a Wilms' tumor, but intrarenal calcification in Wilms' tumor is well known to be rare (5-10%). There are no specific angiographic features in XPN [2, 7, 10, 31]. The vessels may be displaced and sometimes distorted vessels may be found in the involved areas. In our third patient only stretching and a curved course of vessels in the involved kidney could be demonstrated (Fig. 2 b). The good vascularisation was in contrast to nonfunction of the same kidney at IVU, even when followed up for 10 hours. We can give no explanation for this phenomenon. Gammill [10] differentiates, angiographically, a parenchymatous and vascular type of XPN. Arterio-venous anastomoses or early filling of the intrarenal veins are not found in XPN. In general the vascularisation of the XPN-kidney is poorer than in Wilms' tumor or hypernephroid carcinoma [10]. This finding correlates well with the cortical atrophy shown by microscopy. In the diagnosis of XPN, echography has no advantage over radiography, but it always provides important additional information. This is of special value in childhood, because it is a nonaggressive examination [12, 20]. Shanser and Klugo found multiple internal echoes in two patients examined with B-mode echography. We also found a similar state in two of our patients. How far this echographic state is specific for XPN can only be shown through further investigations using this method. Contrary to Schindera [28], we believe that in the diagnosis of XPN, the combined use of intravenous urography and renal echography can strongly suggest the diagnosis XPN. Arteriography gives an additional information, but it is invasive. However, the final diagnosis of XPN rests on the pathological examination. Macroscopically the renal pelvis and calyces are dilated and filled with purulent material and concretions. Both renal cortex and medulla are considerably atrophied and contain multiple abscesses. The renal papillae are mainly necrotic. In most cases the interstitial inflammation spreads to the renal capsule and the perirenal tissue. Microscopic examination shows an acute nephritis and chronic destructive pyelonephritis. The most impor-
15
tant sign is the xanthogranulomatous reaction, which is characterized by necrotic granulation tissue and lipid filled macrophages (foam cells). In addition there are many lymphocytes, plasmocytes, polymorphonuclear cells and granulomata with giant cells. The glomeruli show a segmental and general hyalinosis. Endarteritis and endophlebitis are occasionally found in the renal vessels [3, 9, 14, 15]. Generally, XPN can be differentiated from a renal tumor or renal tuberculosis without any difficulty at all. Treatment for XPN consists of nephrectomy and the removal of the adherent lymph-nodes and the ureter. All the cases described so far have been cured by nephrectomy. The prognosis of this disease is considered to be good. Addendum. 1977 Harzmann reported on two further pediatric patients with XPN and Bagley published a diffuse XPN in children for the first time.
References 1. Abbate, A.D., Meyers, J.: Xanthogranulomatous pyelonephritis in childhood. J. Urol. 160, 231 (1976) 2. Anhalt, M. A., Cawood, C.D., Scott, R.: Xanthogranulomatous pyelonephritis: A comprehensive review with report of 4 additional cases. J. Urol. 105, 10 (1971) 3. Avnet, N.L., Roberts, Th. W,, Goldberg, H. R.: Tumefactive xanthogranulomatous pyelonephritis, Am. J. Roentgenol. 90, 89 (1963) 4. Black, W. C., Ragsdale, E. F.: Wilms' tumor. Am. J. Roentgenol. 103, 53 (1968) 5. Brueziere, J., Begue, P., Polani, C., Boccon-Gibod, L.: Les pydlon6phrites xantogranulomateuses diffuses de l'enfant. J. Urol. Nephrol. (Paris) 80, 13 (1974) 6. Ceccarelli, F. E., Wurster, J. C., Chandor, S. B.: Xanthogranulomatous pyelonephritis in an infant. J. Urol. 104, 755 (1970) 7. Delsen, J., Hardmeier, Th.: Die xanthogranulomat6se Pyelonephritis. Helv. Chir. Acta 43, 363 (1976) 8. Egli, F., Jenny, P., Schindera, F., Mihatsch, M. J.: Xanthogranulomat0se Pyelonephritis. Jahresversammlung der Schweizerischen Gesellschaft fiir Pfidiatrie, Locarno 1977 9. Friedenberg, M. J., Spjut, H. J.: Xanthogranulomatous pyelonephritis. Am. J. Roentgenol. 90, 97 (1963) 10. Gammill, S., Rabinowitz, J. G., Peace, R., Sorgen, S., Hurwitz, L., Himmelfarb, E.: New thoughts concerning xanthogranulomatous pyelonephritis (X-P). Am. J. Roentgenol. 125, 154 (1975) 11. Gingell, J. C., Roylance, J., Davies, E. R., Penry, J. B.: Xanthogranulomatous pyelonephritis. Br. J. Radiol. 46, 99 (1973) 12. Goldberg, B. B., Pollack, H. M.: Differentiation of renal masses using A-mode ultrasound. J. Urol. 105, 765 (1971) 13. Graivier, L., Vargas, M.A.: Xanthogranulomatous pyelonephritis in childhood. Am. J. Dis. Child. 123, 156 (1972) 14. Habib, R., Levy, M.: Py61on6phrite xanthogranulomateuse. In: Royer, P., Habib, R., Matthieu, H.: Nephrologie P6diatrique. Paris: Flammarion 1972 15. Habib, R., Levy, M., Royer, P.: La py61on6phrite xanthogranulomateuse chez l'enfant. Arch. Fr. Pediatr. 25, 489 (1968) 16. Hardy, J. C., Ardichvili, D.: Un cas de py61on6phrite xanthogranulomateuse chez l'enfant. Acta Urol. Belg. 39, 509 ( 1971)
16 17. Hemet, J., Morin, C., Fondimare, A., Borde, J., Mitrofanoff, B., Menibus, CI. H.: La py61on6phrite xanthogranulomateuse de l'enfant. Apropos de deux observations. Arch. Anat. Cytol. Pathol. 17, 193 (1969) 18. Hooper, R. G., Kempson, R. L., Schlegel, J. V.: Xanthogranulomatous pyelonephdtis. J. Urol. 88, 585 (1962) 19. Kessler, E., Grunebaum, M., Lazebnik, J.: Xanthogranulomatous pyelonephritis in childhood with ascaris lumbricoides infestation. Path. Microbiol. (Basel) 37, 401 (1971) 20. King, D.L.: Renal ultrasonography. Radiology 105, 633 (1972) 21. Klugo, R.C., Anderson, J.A., Reid, R., Rowell, I., Cemy, J.C.: Xanthogranulomatous pyelonephritis in children. J. Urol. 117, 350 (1977) 22. Lackner, H., Wolfel, D., Banowsky, L., Kornfeld, M.: Xanthogranulomatous pyelonephritis. J. Pediatr. 75, 482 (1969) 23. McCullough, D. L., Tiguor, M. R.: Xanthogranulomatous pyelonephritis. Am. J. Med. 52, 395 (1972) 24. Mering, J. H., Kaplan, G. W., Mc Laughlin, A. P.: Xanthogranulomatous pyelonephritis. Unusual clinical presentations. Urology 1, 338 (1973) 25. Noyes, E.W., Palubinskas, A.J.: Xanthogranulomatous pyelonephritis. J. Urol. 101, 132 (1969) 26. Parker, J.M.: Xanthogranulomatous pyelonephritis. J. Urol. 96, 290 (1966) 27. Saeed, S.M., Fine, G.: Xanthogranulornatous pyelonephritis. Am. J. Clin. Pathol. 39, 616 (1963) 28. Schindera, F., Rohrbach, R., Limberg, J.: Xanthogranulomat6se Pyelonephritis bei einem Kleinkind. Klin. Piidiatr. 189, 277 (1977) 29. Schlagenhaufer, F.: Uber eigentiimliche Staphylomykosen der Nieren und des pararenalen Bindegewebes. Frankf. Z. Pathol. 19, 139 (1916)
K. Fahr et al.: Xanthogranulomatous Pyelonephritis 30. Schulman, C.C., Denis, R.: Py61on6phrite xanthogranulomateuse diffuse chez l'enfant. Ann. Chir. Inf. 17, 251 (1976) 31. Shanser, J.D., Herzog, K. A., Palubinskas, A. J.: Xanthogranulomatous pyelonephritis in childhood. Pediatr. Radiol. 3, 12 (1975) 32. Souto, C. A. V., Monteiro, S. A., Andrade, L. C.: Xanthogranulomatous pyelonephritis: 2 case reports. J. Urol. 102, 393 (1969) 33. Strasberg, J., Jacobson, S.A., Srolovitz, H., Sedlezky, H., Schneiderman, C.: Xanthogranulomatous pyelonephritis- radiologicconsiderations. J. Can. Assoc. Radiol. 21, 173 (1970) 34. Vfizquez, J.J., Herranz, G.: Pie!onefritis xanthogranulomatosa. Rev. IVied.Univ. Navarra 9, 127 (1965) Addendum
Harzmann, R., Bichler, K. H., Schmitz-Moormann, P., Erdmann, D.: Xanthogranulomat6se Pyelonephritis. Akt. Urol. 8, 319 (1977) Bagley, F. H., Stewart, A. M., Jones, P. F.: Diffuse xanthogranulomatous pyelonephritis in children: an unrecognized variant. J. Urol. 118, 434 (1977) Date of final acceptance: March 14, 1978
Dr. K. Fahr R/Sntgenabteilung Universit~its-Kinderklinik Im Neuenheimer Feld 150 D-6900 Heidelberg 1 Federal Republic of Germany
