Pediat. Radio1. 3, 156--157 (1975) 9 by Springer-Verlag 1975
Subepithelial Hemorrhage in the Renal Pelvis and Ureter Simulating Pyeloureteritis Cystica J a y S. T h o m p s o n and William H . McAlister Department of Radiology, Cardinal Glennon Memorial Hospital, St. Louis, Missouri (JST) and The Edward Mallinckrodt Institute of Radiology, Washington University Medical Center, St. Louis, Missouri (WHM), USA Date of final acceptance: February 20, 1975
Abstract. Two children are presented with similar appearing small urographic defects in the ureter and renal pelvis similar to pyeloureteritis cystica. In one patient with aplastic anemia who died the abnormalities represented areas of subepithelial hemorrhage. Another child had clini-
cally diagnosed Henoch-Schonlein purpura and the defects were probably due to subepithelial hemorrhage. Key words: Subepithelial hemorrhage, pyeloureteritis cystica, aplastic anemia, Henoch-Schonlein purpura, ureter, renal pelvis.
Pyeloureteritis cystica is a p a t h o l o g i c entity characterized b y subepithelial cyst f o r m a t i o n in the renal pelvis a n d ureter. Several h u n d r e d cases of pyeloureteritis cystica h a v e a p p e a r e d in the literature. A l m o s t all of these h a v e b e e n adults [3, 5]. T h e p u r p o s e of this p a p e r is to describe t w o children in w h o m the r a d i o g r a p h i c a p p e a r a n c e was one of pyeloureteritis cystica. I n one patient w i t h aplastic a n e m i a the a b n o r m a l i t i e s w e r e p r o v e n to be due to subepithelial h e m o r r h a g e . I n a patient w i t h H e n o c h Schonlein p u r p u r a the defects w e r e p r o b a b l y also due to subepithelial bleeding.
Case II, K V R : A six and one-half year old girl was admitted with a history of 3 days of pain and swelling of the ankles and left elbow and 2 days of red macules over the lower extremities. Ten days prior to admission she had been treated for otitis media with penicillin. The clinical impression was Henoch-Schonlein purpura. Hematuria was present at the time of admission with 10--30 RBC/high power field and persisted over a period of five days. At no time did pynria develop. Five urine cultures had no significant bacterial growth. Intravenous urography showed multiple filling defects in the pelvocalyceal collecting systems and ureters (Fig. 2). Hematuria cleared in five days and the patient was discharged without antibiotic therapy. Follow-up urography two month later was normal.
Case Reports
Comment: Because of the clinical history of HenochSchonlein purpura with systemic evidence of hemorrhage the radiographic interpretation of the findings was subepithelial hemorrhage into the renal pelvis and ureters. The resolution of the findings on subsequent urography supported the hypothesis.
Case I, C W : A five and one-half year old boy was well until twelve days prior to admission at which time he developed fever, headache, and progressive nuchal rigidity. Multiple ecchymoses developed over a two day period. His temperature was 104 ~ ttgb. 7.8, ttcr. 26 and the platelet count was 4,000. The patient was found to have aplastic anemia. There was grossly bloody urine. He was given blood transfusions and the bleeding became microscopic at which time intravenous urography showed an appearance of the left pelvocalyceal collecting system consistent with pyeloureteritis cystica (Fig. 1). At no time was there pyuria. Multiple urine cultures had no growth. Despite intensive therapy he died on the seventeenth hospital day from gastrointestinal and pulmonary bleeding. Post mortem examination showed bilateral pulmonary hemorrhage and hypocellular bone marrow. The roentgen abnormalities shown on intravenous urography were due to subepithelial hemorrhage in the renal pelvis and ureter. Comment: This patient with aplastic anemia had radiographic abnormalities compatible with pyeloureteritis cystica. At autopsy, however, the lesions were produced by subepithelia! hemorrhage,
Discussion
Pyeloureteritis cystica a n d cystitis cystica are occasionally f o u n d at a u t o p s y . T h e y are m o r e c o m m o n in older patients a n d in females. I n general the patients are a s y m p t o m a t i c b u t can h a v e h e m a t u r i a or signs a n d s y m p t o m s of u r i n a r y tract infection. O v e r the years n u m e r o u s theories h a v e b e e n p r o p o s e d to explain the e t i o l o g y a n d p a t h o g e n e s i s of the disease [1]. Hill [1] p r o d u c e d pyeloureteritis cystica in rabbits d u r i n g the course of Escherichia coli i n d u c e d e x p e r i m e n t a l pyelonephritis. T h e r e was metaplasia of the u r o e p i t h e l i u m f r o m transitional to m u c i n filled cysts, lined b y c o l u m n a r cells, If c o m m u n i c a t i o n was established b e t w e e n the
j. s. Thompson and W. H. McAlister: Subepithelial Hemorrhage in the Regal Pelvis
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Fig. 1 Fig. 2 Fig. 1. Case I. Intravenous urography. There is distortion of the peivocalyceal collecting system of the left kidney with stretching of the infundibula, with circumferential compression of the calyces. There are intraluminal and intramural filling defects in the renal pelvis and ureter. At autopsy the intramural defects were shown to be hemorrhage in this patient with aplastie anemia Fig. 2. Case II. Intravenous urogram shows multiple filling defects involving the pelvocalyceal collecting system of the right kidney as well as the left renal pelvis. Subepithelial defects involve the proximal half of both of the ureters compatible with hemorrhage in this patient with Henoch-Schonlein purpura cysts and the lumen, pyeloureteritis glandularis developed. W i t h o u t communication the cysts enlarged and became the visible lesions of pyeloureteris cystica. In addition to bacteria, any n u m b e r of irritating chemicals and foreign substances can produce metaplastic changes in uroepithelium. Marchwald, however, in 1898 f o u n d cysts in n e w b o r n infants in the absence of inflammatory changes [4]. Subepithelial h e m o r r h a g e in the renal pelvis and ureter is rare but obviously must be considered in the differential diagnosis of intramural defects in the renal pelvis and ureter. We were unable to find radiographic appearances similar to our patients in children in the articles reviewed on aplastic anemia or Henoch-Schoniein purpura [2, 6, 7]. Radiographically in pyeloureteritis cystica there are multiple r o u n d and oval projections of varying size usually f r o m a few ram. to 1 cm. that can be located anywhere in the ureter especially near the pelvi-ureteral junction and project into the roentgenographically opacified lumen. Urographically the kidney is usually normal but the cysts may obstruct a ureter or there may be changes of pyelonephritis. T h e cysts may become as large as 2 - - 3 cm. T h e process may be unilateral or bilateral.
In our patients, subepithelial h e m o r r h a g e into the renal pelvis and ureter presented a similar radiographic appearance. Bleeding elsewhere in the b o d y would make this latter diagnosis probable.
1. Hill, G. S.: Experimental production of pyeloureteritis cystica and glandularis. Invest. Urol. 9, 1 (1971) 2. Hughes, L. A., Wenzl, J. E.: Anaphylactoid purpura nephritis in children. Clin, Pediat. 8, 594 (1969) 3. Kohler, R.: Pyelo-ureteritis cystica. Acta. Radiol. Stockh, 4, 123 (1966) 4. Marchwald, H.: Die multiple Cystenbildungen in der Ureteren und der Harnblase, sog. Ureteritis Cystica. Miinchn. med. Wschr. 45, 1049 (1898) 5. Mayall, G. F., Chit, B.: Pyelo-ureteritis cystica. Brit. J. Radiol. 37, 557 (!964) 6. Sahn, D. J., Schwartz, A. D. : Schonlein-Henochsyndrome: Observations on some atypical clinical presentations. Pediatrics 49, 614 (1972) 7. Shackelford, G. D., Bloomberg, G., McAlister, W. H.: The value of roentgenography in differentiating aplastic anemia from leukemia masquerading as aplastic anemia. Amer. J. Roentgenol. 116, 651 (1972) William H. 2VfcAlister, M. D. The Mallinckrodt Inst. of Radiology Washington University Medical Center 510 South Kingshighway Boulevard St. Louis, MO 63110, USA
Subepithelial Hemorrhage in the Renal Pelvis and Ureter Simulating Pyeloureteritis Cystica J a y S. T h o m p s o n and William H . McAlister Department of Radiology, Cardinal Glennon Memorial Hospital, St. Louis, Missouri (JST) and The Edward Mallinckrodt Institute of Radiology, Washington University Medical Center, St. Louis, Missouri (WHM), USA Date of final acceptance: February 20, 1975
Abstract. Two children are presented with similar appearing small urographic defects in the ureter and renal pelvis similar to pyeloureteritis cystica. In one patient with aplastic anemia who died the abnormalities represented areas of subepithelial hemorrhage. Another child had clini-
cally diagnosed Henoch-Schonlein purpura and the defects were probably due to subepithelial hemorrhage. Key words: Subepithelial hemorrhage, pyeloureteritis cystica, aplastic anemia, Henoch-Schonlein purpura, ureter, renal pelvis.
Pyeloureteritis cystica is a p a t h o l o g i c entity characterized b y subepithelial cyst f o r m a t i o n in the renal pelvis a n d ureter. Several h u n d r e d cases of pyeloureteritis cystica h a v e a p p e a r e d in the literature. A l m o s t all of these h a v e b e e n adults [3, 5]. T h e p u r p o s e of this p a p e r is to describe t w o children in w h o m the r a d i o g r a p h i c a p p e a r a n c e was one of pyeloureteritis cystica. I n one patient w i t h aplastic a n e m i a the a b n o r m a l i t i e s w e r e p r o v e n to be due to subepithelial h e m o r r h a g e . I n a patient w i t h H e n o c h Schonlein p u r p u r a the defects w e r e p r o b a b l y also due to subepithelial bleeding.
Case II, K V R : A six and one-half year old girl was admitted with a history of 3 days of pain and swelling of the ankles and left elbow and 2 days of red macules over the lower extremities. Ten days prior to admission she had been treated for otitis media with penicillin. The clinical impression was Henoch-Schonlein purpura. Hematuria was present at the time of admission with 10--30 RBC/high power field and persisted over a period of five days. At no time did pynria develop. Five urine cultures had no significant bacterial growth. Intravenous urography showed multiple filling defects in the pelvocalyceal collecting systems and ureters (Fig. 2). Hematuria cleared in five days and the patient was discharged without antibiotic therapy. Follow-up urography two month later was normal.
Case Reports
Comment: Because of the clinical history of HenochSchonlein purpura with systemic evidence of hemorrhage the radiographic interpretation of the findings was subepithelial hemorrhage into the renal pelvis and ureters. The resolution of the findings on subsequent urography supported the hypothesis.
Case I, C W : A five and one-half year old boy was well until twelve days prior to admission at which time he developed fever, headache, and progressive nuchal rigidity. Multiple ecchymoses developed over a two day period. His temperature was 104 ~ ttgb. 7.8, ttcr. 26 and the platelet count was 4,000. The patient was found to have aplastic anemia. There was grossly bloody urine. He was given blood transfusions and the bleeding became microscopic at which time intravenous urography showed an appearance of the left pelvocalyceal collecting system consistent with pyeloureteritis cystica (Fig. 1). At no time was there pyuria. Multiple urine cultures had no growth. Despite intensive therapy he died on the seventeenth hospital day from gastrointestinal and pulmonary bleeding. Post mortem examination showed bilateral pulmonary hemorrhage and hypocellular bone marrow. The roentgen abnormalities shown on intravenous urography were due to subepithelial hemorrhage in the renal pelvis and ureter. Comment: This patient with aplastic anemia had radiographic abnormalities compatible with pyeloureteritis cystica. At autopsy, however, the lesions were produced by subepithelia! hemorrhage,
Discussion
Pyeloureteritis cystica a n d cystitis cystica are occasionally f o u n d at a u t o p s y . T h e y are m o r e c o m m o n in older patients a n d in females. I n general the patients are a s y m p t o m a t i c b u t can h a v e h e m a t u r i a or signs a n d s y m p t o m s of u r i n a r y tract infection. O v e r the years n u m e r o u s theories h a v e b e e n p r o p o s e d to explain the e t i o l o g y a n d p a t h o g e n e s i s of the disease [1]. Hill [1] p r o d u c e d pyeloureteritis cystica in rabbits d u r i n g the course of Escherichia coli i n d u c e d e x p e r i m e n t a l pyelonephritis. T h e r e was metaplasia of the u r o e p i t h e l i u m f r o m transitional to m u c i n filled cysts, lined b y c o l u m n a r cells, If c o m m u n i c a t i o n was established b e t w e e n the
j. s. Thompson and W. H. McAlister: Subepithelial Hemorrhage in the Regal Pelvis
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Fig. 1 Fig. 2 Fig. 1. Case I. Intravenous urography. There is distortion of the peivocalyceal collecting system of the left kidney with stretching of the infundibula, with circumferential compression of the calyces. There are intraluminal and intramural filling defects in the renal pelvis and ureter. At autopsy the intramural defects were shown to be hemorrhage in this patient with aplastie anemia Fig. 2. Case II. Intravenous urogram shows multiple filling defects involving the pelvocalyceal collecting system of the right kidney as well as the left renal pelvis. Subepithelial defects involve the proximal half of both of the ureters compatible with hemorrhage in this patient with Henoch-Schonlein purpura cysts and the lumen, pyeloureteritis glandularis developed. W i t h o u t communication the cysts enlarged and became the visible lesions of pyeloureteris cystica. In addition to bacteria, any n u m b e r of irritating chemicals and foreign substances can produce metaplastic changes in uroepithelium. Marchwald, however, in 1898 f o u n d cysts in n e w b o r n infants in the absence of inflammatory changes [4]. Subepithelial h e m o r r h a g e in the renal pelvis and ureter is rare but obviously must be considered in the differential diagnosis of intramural defects in the renal pelvis and ureter. We were unable to find radiographic appearances similar to our patients in children in the articles reviewed on aplastic anemia or Henoch-Schoniein purpura [2, 6, 7]. Radiographically in pyeloureteritis cystica there are multiple r o u n d and oval projections of varying size usually f r o m a few ram. to 1 cm. that can be located anywhere in the ureter especially near the pelvi-ureteral junction and project into the roentgenographically opacified lumen. Urographically the kidney is usually normal but the cysts may obstruct a ureter or there may be changes of pyelonephritis. T h e cysts may become as large as 2 - - 3 cm. T h e process may be unilateral or bilateral.
In our patients, subepithelial h e m o r r h a g e into the renal pelvis and ureter presented a similar radiographic appearance. Bleeding elsewhere in the b o d y would make this latter diagnosis probable.
1. Hill, G. S.: Experimental production of pyeloureteritis cystica and glandularis. Invest. Urol. 9, 1 (1971) 2. Hughes, L. A., Wenzl, J. E.: Anaphylactoid purpura nephritis in children. Clin, Pediat. 8, 594 (1969) 3. Kohler, R.: Pyelo-ureteritis cystica. Acta. Radiol. Stockh, 4, 123 (1966) 4. Marchwald, H.: Die multiple Cystenbildungen in der Ureteren und der Harnblase, sog. Ureteritis Cystica. Miinchn. med. Wschr. 45, 1049 (1898) 5. Mayall, G. F., Chit, B.: Pyelo-ureteritis cystica. Brit. J. Radiol. 37, 557 (!964) 6. Sahn, D. J., Schwartz, A. D. : Schonlein-Henochsyndrome: Observations on some atypical clinical presentations. Pediatrics 49, 614 (1972) 7. Shackelford, G. D., Bloomberg, G., McAlister, W. H.: The value of roentgenography in differentiating aplastic anemia from leukemia masquerading as aplastic anemia. Amer. J. Roentgenol. 116, 651 (1972) William H. 2VfcAlister, M. D. The Mallinckrodt Inst. of Radiology Washington University Medical Center 510 South Kingshighway Boulevard St. Louis, MO 63110, USA
