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Pyelitis, Ureteritis and Cystitis Cystica1
PYELITIS, URETERITIS AND CYSTITIS CYSTICA1 JULES H. KOPP From the Department of Urology, Washington University Out-patient Department, and Pathology Department of St. Louis Jewish Hospital, St. Louis
The etiology, pathology and history of this disease have been well reviewed recently by Bothe and Cristol in the American Journal of Roentgenology (1945). Most investigators agree that the cysts are formed by a downward proliferation of the lining mucous membrane and overgrowth of the epithelium, or upward proliferation of connective tissue severing the connection of the cell buds and forming cell nests. The cysts are formed by degeneration of the central cells or by definite secretion of mucin-like material by goblet cells found and described by Stoerk and Zuckerkandl. It is also generally agreed that the lesion is caused by some chronic inflammatory process. Stones are present in many of the cases reported. In 1933 Kindall diagnosed a case preoperatively on the bubbly or frothy appearance of the ureter seen on the pyelograms. At operation the ureter had a shot-like feel. On opening the ureter the wall was found to be thickened and to contain many ovoid cysts 1 to 5 mm. in diameter. The cysts were not pedunculated, ruptured easily and contained a yellowish fluid with the consistency of mineral oil. The method of treatment employed by Kindall consisted of passing a No. 10 Blasucci catheter and leaving it in place for 3 days. Ten cubic centimeters of 2 per cent silver nitrate solution was injected before withdrawal of the catheter. Subsequent ureterograms failed to show the presence of the cysts. The purposes of this paper are to present 3 cases of ureteritis cystica, one of which was diagnosed during life, and t::> describe the diagnosis and method of treatment. CASE REPORTS
Case 1. A white male, 60 years of age, said that his chief 'Complaints were frequency, hesitancy, urgency and bloody stools. A vesical calculus was removed and a smaller calculus passed. Two years later the patient was again seen with the same symptoms. Examination revealed an enlarged prostate, which was removed. A diagnosis of diabetes was also made at this time. Ten years later the patient returned. He gave a history of acute attacks of abdominal pain, vomiting and abdominal tenderness. X-ray revealed a stone in the left kidney pelvis. The urine contained many white blood cells and a few red blood cells. At a later date the same year the patient expired during the closure of a perforated duodenal ulcer. Autopsy disclosed a perforated duodenal ulcer and generalized fibrinous peritonitis. The right kidney was normal. The left kidney was scarred in the upper pole and on section this was found to be 1
Read at annual meeting, South Central Section, Kansas City, Mo., November 27, 1945.
28
PYELITIS, URET.ERITIS AND CYSTITIS CYSTICA
29
around a large stone in one of the major calyces. Adjacent to the stone was a, large cyst four centimeters in diameter which contained a cloudy fluid. Many small cysts were seen to line the pelves and ureters of both kidneys. The ureters had the characteristic shott:v feel. Sections revealed many small cysts lining the ureters. The cysts ,Yere thin walled and lined with transitional epithelium similar to the epithelium lining the ureter. There was an increase in the amount of fibrous tissue present (figs. 1 to 3).
Fm. 1. Case 1.
FIG. 2. Case 1.
Three cysts filling lumen of ureter
Large cyst completely filling lumen of ureter
Case 2. A 59 year old white female was admitted to the hospital complaining of pains in both ankles, tiredness for 8 months, constipation, nocturia 2 to 3 times, loss of weight, slight chills and fever. Urinalysis shmred bacilluri£t, pyuria and microscopic hematuria. A diagnosis 11-as made of chronic myocarditis, arteriosclerosis, and chronic nephritis. At cystoscopy the bladder was seen to be inflamed and the trigone was covered with small red nodules. The ureters were not obstructed. The kidney function was impaired, the phthalein output on the right side being 2½ per cent for JO minutes, and on the left 5 per cent. The bladder specimen contained 2½ per
30
JULES H. KOPP
cent phthalein. The urine specimen from the right kidney showed many red cells and a few white blood cells; that from the left kidney, a few red cells, many white blood cells, and colon bacilli. The left renal pelvis appeared normal in size and shape in the pyelogram, but the calyces were club shaped. The left ureter showed an unusual worm-eaten appearance most marked in the middle third. The same picture was seen on the right, but not as pronounced as on the left. Two subsequent x-ray examinations shovrnd the same picture.
Fm. 3. Case 1.
Fm. 4. Case 2.
Two cysts filling lumen of ureter
Sections of ureter showing downward proliferation of epithelium and a cyst already formed
A few days later the patient was seized with severe colicky pains in the left kidney region. A catheter was passed up the left ureter with relief. At this time the urine from the left kidney ,rns clear. Two days later the patient had a recurrence of the same pains and a retention catheter 1Yas placed in the left ureter. Patient progressed satisfactorily and was discharged. The patient was re-admitted 2 months later with a history of fever for 5 days. At the time of this examination the right kidney was palpable and there was
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FIG. 5. Serial sections showing formation of a cyst by union of two folds in epithelium of ureter
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32
JULES H. KOPP
tenderness and rebound pain in the kidney., region. The patient gradually became comatose and expired. The last non-protein nitrogen estimation was 140. At autopsy the right kidney was enlarged, white, and contained many small hemorrhages throughout. The cortex was 3 mm. thick. The left kidney was normal in size and appearance. Both ureters had a shotty feel and thickened walls. They both contained small cysts, more numerous on the left than on the right. Microscopic examination showed infoldings of the epithelium of the ureters and an increase in fibrous tissue. Serial sections of one area showed what I interpreted to be the formation of a cyst. Evidence of chronic inflammation was seen (figs. 4 and 5).
Fm. 6. Case 3.
Left, pyelogram showing negative shadows caused by cysts; (right) pyelogram showing absence of cysts after treatment
Case 3. A ,vhite male, aged 23, complained of nervousness, weakness, sweating in the evenings and occasional burning on urination for 4 or 5 months. The family history was negative and also the past history except for an unexplained fever of 1 weeks' duration 3 years ago. On examination tenderness over the bladder area was the only positive finding. The urine showed many white cells and a few red cells. The patient was told to return for observation and further examination but did not return for 17 months. At this time he gave a history of chills, fever, nausea and vomiting for which he had been treated at home. On examination the patient was tender in the left lumbar and suprapubic areas. The urine showed white and red blood cells and culture was reported positive for Staphylococcus albus and diphtheroids. Cystoscopic examination was made and the bladder found to be inflamed. The trigone was covered with many small
PYELITIS, URETERITIS AND CYSTITIS CYSTICA
33
translucent cysts. Ureteral catheters were passed without obstruction. The phthalein appeared in 4 minutes on the right side with a concentration of 22 per cent and appeared in 6 minutes on t he left side with a concentration of 7 per cent for 10 minutes. Both kidney specimens contained white blood cells, but no organisms were found. Culture and guinea pig inoculations were negative. The right kidney and ureter appeared normal except for the size of the pelvis (on the pyelograms) . The left kidney pelvis appeared large but normal and there were several discrete filling defects in the middle third of the left ureter. X-ray diagnosis was made of normal right kidney and ureter, normal left kidney with two areas of rarefaction in the left ureter opposite the fourth lumbar vertebra, either non-opaque stones or air bubbles (fig. 6, left) . The patient was kept under observation and cystoscopy ·was again performed 2 months later. White blood cells were found in the urine. The findings were the same at this time and a diagnosis of ureteritis cystica was made. The patient was treated by ureteral dilatation and instillation of silver nitrate, as recommended by Kindall. The treatment was repeated weekly in the clinic for 9 weeks using the appearance of the kidney specimens and trigone as proof of cure. The cysts of the trigone cleared up and the urine specimens became clear. The patient was re-admitted 4 months after the first admission to check our results with x-ray. Pyelograms were made and the areas of rarefaction were no longer present. Urine specimens did not show white blood cells (fig. 6, right). SUMMARY
The chief complaints of ureteritis and pyelitis cystica are usually pain, acute or dull, constant or intermittent, of long duration, and located in the lumbar area. The history may be that of a severe pain lasting one or more days followed by a dull ache. Chills and fever are frequently present, often with nausea and vomiting. The condition is usually associated with chronic inflammation of the urinary tract and stones are frequently present. In some cases congenital malformation or urinary obstruction with retention have been found. On physical examination costovertebral tenderness of the involved side is usually present. On cystoscopic examination the trigone is seen to be covered with many small translucent cysts and the ureteral orifice of the involved side or sides is usually edematous and reddened. The ureteral catheter meets with temporary obstruction due to rupturing of the cysts as it is passed. This may cause the passage of some blood around the catheter, which is very significant of ureteritis cystica. Relief of pain following passage of the catheter is also diagnostic. There may be a delay in the appearance of the dye, but the kidney function is usually not impaired. The characteristic appearance of the lesion on x-ray, as described by Kindall, is diagnostic. The filling defects in the ureter, usually in the upper two-thirds, give the ureter a bubbly or frothy appearance. These defects can easily be mistaken for non-opaque calculi or air bubbles. Any co-existent
34
JULES H . KOPP
renal disease will also be seen at this time. The treatment consists of dilating the ureter, thereby rupturing the cysts, and the instillation of silver nitrate to destroy the traumatized cysts by its astringent action. Recently Bothe and Cristol have reported successful treatment of the lesion with sulfathiazole. CONCLUSION
Three cases of cystic lesions of the urinary tract are presented. One case was diagnosed and successfully treated with ureteral dilatations and silver nitrate. The etiology, pathology and diagnosis are briefly presented.
Lister Bldg., St. Louis 8, Mo.
The etiology, pathology and history of this disease have been well reviewed recently by Bothe and Cristol in the American Journal of Roentgenology (1945). Most investigators agree that the cysts are formed by a downward proliferation of the lining mucous membrane and overgrowth of the epithelium, or upward proliferation of connective tissue severing the connection of the cell buds and forming cell nests. The cysts are formed by degeneration of the central cells or by definite secretion of mucin-like material by goblet cells found and described by Stoerk and Zuckerkandl. It is also generally agreed that the lesion is caused by some chronic inflammatory process. Stones are present in many of the cases reported. In 1933 Kindall diagnosed a case preoperatively on the bubbly or frothy appearance of the ureter seen on the pyelograms. At operation the ureter had a shot-like feel. On opening the ureter the wall was found to be thickened and to contain many ovoid cysts 1 to 5 mm. in diameter. The cysts were not pedunculated, ruptured easily and contained a yellowish fluid with the consistency of mineral oil. The method of treatment employed by Kindall consisted of passing a No. 10 Blasucci catheter and leaving it in place for 3 days. Ten cubic centimeters of 2 per cent silver nitrate solution was injected before withdrawal of the catheter. Subsequent ureterograms failed to show the presence of the cysts. The purposes of this paper are to present 3 cases of ureteritis cystica, one of which was diagnosed during life, and t::> describe the diagnosis and method of treatment. CASE REPORTS
Case 1. A white male, 60 years of age, said that his chief 'Complaints were frequency, hesitancy, urgency and bloody stools. A vesical calculus was removed and a smaller calculus passed. Two years later the patient was again seen with the same symptoms. Examination revealed an enlarged prostate, which was removed. A diagnosis of diabetes was also made at this time. Ten years later the patient returned. He gave a history of acute attacks of abdominal pain, vomiting and abdominal tenderness. X-ray revealed a stone in the left kidney pelvis. The urine contained many white blood cells and a few red blood cells. At a later date the same year the patient expired during the closure of a perforated duodenal ulcer. Autopsy disclosed a perforated duodenal ulcer and generalized fibrinous peritonitis. The right kidney was normal. The left kidney was scarred in the upper pole and on section this was found to be 1
Read at annual meeting, South Central Section, Kansas City, Mo., November 27, 1945.
28
PYELITIS, URET.ERITIS AND CYSTITIS CYSTICA
29
around a large stone in one of the major calyces. Adjacent to the stone was a, large cyst four centimeters in diameter which contained a cloudy fluid. Many small cysts were seen to line the pelves and ureters of both kidneys. The ureters had the characteristic shott:v feel. Sections revealed many small cysts lining the ureters. The cysts ,Yere thin walled and lined with transitional epithelium similar to the epithelium lining the ureter. There was an increase in the amount of fibrous tissue present (figs. 1 to 3).
Fm. 1. Case 1.
FIG. 2. Case 1.
Three cysts filling lumen of ureter
Large cyst completely filling lumen of ureter
Case 2. A 59 year old white female was admitted to the hospital complaining of pains in both ankles, tiredness for 8 months, constipation, nocturia 2 to 3 times, loss of weight, slight chills and fever. Urinalysis shmred bacilluri£t, pyuria and microscopic hematuria. A diagnosis 11-as made of chronic myocarditis, arteriosclerosis, and chronic nephritis. At cystoscopy the bladder was seen to be inflamed and the trigone was covered with small red nodules. The ureters were not obstructed. The kidney function was impaired, the phthalein output on the right side being 2½ per cent for JO minutes, and on the left 5 per cent. The bladder specimen contained 2½ per
30
JULES H. KOPP
cent phthalein. The urine specimen from the right kidney showed many red cells and a few white blood cells; that from the left kidney, a few red cells, many white blood cells, and colon bacilli. The left renal pelvis appeared normal in size and shape in the pyelogram, but the calyces were club shaped. The left ureter showed an unusual worm-eaten appearance most marked in the middle third. The same picture was seen on the right, but not as pronounced as on the left. Two subsequent x-ray examinations shovrnd the same picture.
Fm. 3. Case 1.
Fm. 4. Case 2.
Two cysts filling lumen of ureter
Sections of ureter showing downward proliferation of epithelium and a cyst already formed
A few days later the patient was seized with severe colicky pains in the left kidney region. A catheter was passed up the left ureter with relief. At this time the urine from the left kidney ,rns clear. Two days later the patient had a recurrence of the same pains and a retention catheter 1Yas placed in the left ureter. Patient progressed satisfactorily and was discharged. The patient was re-admitted 2 months later with a history of fever for 5 days. At the time of this examination the right kidney was palpable and there was
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c:1
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8 )--< ("J
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FIG. 5. Serial sections showing formation of a cyst by union of two folds in epithelium of ureter
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......
32
JULES H. KOPP
tenderness and rebound pain in the kidney., region. The patient gradually became comatose and expired. The last non-protein nitrogen estimation was 140. At autopsy the right kidney was enlarged, white, and contained many small hemorrhages throughout. The cortex was 3 mm. thick. The left kidney was normal in size and appearance. Both ureters had a shotty feel and thickened walls. They both contained small cysts, more numerous on the left than on the right. Microscopic examination showed infoldings of the epithelium of the ureters and an increase in fibrous tissue. Serial sections of one area showed what I interpreted to be the formation of a cyst. Evidence of chronic inflammation was seen (figs. 4 and 5).
Fm. 6. Case 3.
Left, pyelogram showing negative shadows caused by cysts; (right) pyelogram showing absence of cysts after treatment
Case 3. A ,vhite male, aged 23, complained of nervousness, weakness, sweating in the evenings and occasional burning on urination for 4 or 5 months. The family history was negative and also the past history except for an unexplained fever of 1 weeks' duration 3 years ago. On examination tenderness over the bladder area was the only positive finding. The urine showed many white cells and a few red cells. The patient was told to return for observation and further examination but did not return for 17 months. At this time he gave a history of chills, fever, nausea and vomiting for which he had been treated at home. On examination the patient was tender in the left lumbar and suprapubic areas. The urine showed white and red blood cells and culture was reported positive for Staphylococcus albus and diphtheroids. Cystoscopic examination was made and the bladder found to be inflamed. The trigone was covered with many small
PYELITIS, URETERITIS AND CYSTITIS CYSTICA
33
translucent cysts. Ureteral catheters were passed without obstruction. The phthalein appeared in 4 minutes on the right side with a concentration of 22 per cent and appeared in 6 minutes on t he left side with a concentration of 7 per cent for 10 minutes. Both kidney specimens contained white blood cells, but no organisms were found. Culture and guinea pig inoculations were negative. The right kidney and ureter appeared normal except for the size of the pelvis (on the pyelograms) . The left kidney pelvis appeared large but normal and there were several discrete filling defects in the middle third of the left ureter. X-ray diagnosis was made of normal right kidney and ureter, normal left kidney with two areas of rarefaction in the left ureter opposite the fourth lumbar vertebra, either non-opaque stones or air bubbles (fig. 6, left) . The patient was kept under observation and cystoscopy ·was again performed 2 months later. White blood cells were found in the urine. The findings were the same at this time and a diagnosis of ureteritis cystica was made. The patient was treated by ureteral dilatation and instillation of silver nitrate, as recommended by Kindall. The treatment was repeated weekly in the clinic for 9 weeks using the appearance of the kidney specimens and trigone as proof of cure. The cysts of the trigone cleared up and the urine specimens became clear. The patient was re-admitted 4 months after the first admission to check our results with x-ray. Pyelograms were made and the areas of rarefaction were no longer present. Urine specimens did not show white blood cells (fig. 6, right). SUMMARY
The chief complaints of ureteritis and pyelitis cystica are usually pain, acute or dull, constant or intermittent, of long duration, and located in the lumbar area. The history may be that of a severe pain lasting one or more days followed by a dull ache. Chills and fever are frequently present, often with nausea and vomiting. The condition is usually associated with chronic inflammation of the urinary tract and stones are frequently present. In some cases congenital malformation or urinary obstruction with retention have been found. On physical examination costovertebral tenderness of the involved side is usually present. On cystoscopic examination the trigone is seen to be covered with many small translucent cysts and the ureteral orifice of the involved side or sides is usually edematous and reddened. The ureteral catheter meets with temporary obstruction due to rupturing of the cysts as it is passed. This may cause the passage of some blood around the catheter, which is very significant of ureteritis cystica. Relief of pain following passage of the catheter is also diagnostic. There may be a delay in the appearance of the dye, but the kidney function is usually not impaired. The characteristic appearance of the lesion on x-ray, as described by Kindall, is diagnostic. The filling defects in the ureter, usually in the upper two-thirds, give the ureter a bubbly or frothy appearance. These defects can easily be mistaken for non-opaque calculi or air bubbles. Any co-existent
34
JULES H . KOPP
renal disease will also be seen at this time. The treatment consists of dilating the ureter, thereby rupturing the cysts, and the instillation of silver nitrate to destroy the traumatized cysts by its astringent action. Recently Bothe and Cristol have reported successful treatment of the lesion with sulfathiazole. CONCLUSION
Three cases of cystic lesions of the urinary tract are presented. One case was diagnosed and successfully treated with ureteral dilatations and silver nitrate. The etiology, pathology and diagnosis are briefly presented.
Lister Bldg., St. Louis 8, Mo.