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Bilharzial Pseudo-Calculus of the Ureter
Vol. 99, Jan. Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright © 1968 by The Williams & Wilkins Co.
BILHARZIAL PSEUDO-CALCULUS OF THE URETER ALY MAGED
AND
L.A.M. SOLIMAN
From the Departments of Urology and Pathology, Faculty of Medicine, Ein Shams University, Cairo, Egypt, U.A.R.
A rare form of bilharzial calcification of the ureter is herein described. It has been referred to as bilharzial pseudo-calculus of the ureter because a well-defined calcareous ureteral shadow, similar in appearance to a stone, was noted by x-ray and the true condition was not identified until the operation.
U reteral bilharziasis is a common disease producing different types of lesions. Mild lesions cause ureteral irritation and spasms, while severe lesions cause serious ureteral damage and, ultimately, lead to the destruction of the renal parenchyma. 1 The oviposition and accompanying cellular reaction may be diffuse involving the major part of the ureter but is more commonly localized. 2 Heavy bilharzial infiltrations occur selectively in certain segments of the organ because of the communication between the ureteral veins and portal system. These infiltrations usually lead to stenotic lesions; therefore, strictures are frequently seen in the intramural and juxtavesical portions of the ureter and, to a lesser extent, at the level of the third lumbar vertebra. Calcification in the ureteral wall is common and pathognomonic. It is usually caused by the large number of dead and calcified Bilharzia ova which is deposited in the submucosa. On a plain x-ray, the calcification resembles a faint outline of the ureter and sometimes appears linear. These areas are usually found in the lower end of the ureter but sometimes the complete ureter may be calcified. Calcification of a different nature is seen in ureteritis calcinosa. In this disease the area has punctate calcific spots due to calcification occurring in the contents of ureteritis cystica with further deposition of urinary salts on top resulting in the formation of small primary ureteral calculi. 3 Occasionally, calcification may occur in a bilharzial polyp arising in the dilated segment proximal to a stricture and appear in x-rays as a dense calcified shadow. 4
MATERIAL
Only 5 cases have been encountered in more than 2,000 cases of bilharzial ureters. All 5 cases involved male patients. The lesions were on the right side opposite the L5 vertebra in 4 cases and on the left side in 1 case. In the latter case, the lesion was quite extensive, infiltrating part of the renal pelvis; it was diagnosed as a giant calculus of the ureter. In 2 cases, associated renal calculi were present. In all cases, a marked degree of ureteral obstruction at the level of the lesion was noted. All patients had symptoms of ureteral or renal calculi and suffered frequent, recurrent attacks of renal colic, hematuria and, sometimes, passage of gravel. Radiological studies revealed a welldefined dense radiopaque shadow in the ureter which resembled a stone. On excretory urography, gross obstruction around the calcified area and marked hydrometer and hydronephrosis were noted. A diagnosis of ureteral calculus was made in all cases and operative removal was the choice of treatment. Exposure of the ureter at the site of the alleged stone revealed a well-defined, fusiform, hard swelling which varied from 1.5 and 2.5 cm. in 4 cases and was 10 cm. in 1 case. This swelling was surrounded by dense fibrofatty tissue, on the surface of which were tortuous and dilated thinwalled veins. This particular part of the ureter was very adherent to the surrounding tissues and overlying peritoneum. Dissection was always difficult and resulted in tearing of the dilated veins with troublesome ooze. On incision a very tough and gritty tissue was noted along the thickness of the ureter until the lumen was reached. The lumen was void of calculi and neither stenosed or narrowed. The cut surface of the ureter was pale yellow with a rough and ho-
Accepted for publication September 27, 1966. Ibrahim, B.: Bilharziasis of the ureter. Lancet,
1
2: 1184, 1923. 2 Zaher, M. F., Safwat, lVI. M., Fawzi, R. M. and Badr, lVI. M.: Bilharzial non-obstructive spindle shaped ureter. J. Egypt Med. Ass., 39: 213-219, 1956. 3 El Ghorab, M. A.: Ureteritis calcinosa: A complication of bilharzial ureteritis and its relation to primary ureteric stone formation. Brit. J. Urol., 34: 33, 1962. 4 Makar, N.: The bilharzial ureter: Some observations on the surgical pathology and surgical treatment. Brit. J. Surg., 36: 148, 1948.
30
:BILHARZIAL PSEUDO-CALCULUS OF URETER
FIG. 1. Case 1. A, plain x-ray shows giant calculus in left ureter. B, excretory urogram shows nephrosis. C, removed segment of ureter. D, cut sections, upper and lower portions.
mogeneous surface and usually revealed a nonuniform thickness of the ureteral wall, varying from 3 to 8 mm. Because of the thickness of the wall, the lumen acquired an eccentric position in relation to it. The ureter was markedly dilated above the hard, thick portion, while the lower part of the lesion faded gradually into the distal portion of the ureter with no sharp limit. lVIicroscopic examination of the resected ureteral segment revealed massive infiltration by Bilharzia ova; wide areas of dead and calcified ova ,-vith hardly any tissue in between were seen. Such a picture is obtained in the well-known sandy patches which is essentially a submucosal lesion. However, the lesions herein reported on replaced not only the submucosa but practically the complete muscle coat of the ureter. The latter being completely, or very near completely,
absent and, instead, there were Bilharzia ova, a high proportion of which being calcified and of the hematobium variety. The mucosa! lining; was either ulcerated or revealed ureteritis cysticti, or squamous metaplasia with marked keratiniza. tion. Bilharzia] pseudo-calculus is always discovered during an operation originally planned for re-· moval of a stone. Although no actual narrowing of the lumen is present there is evidence of gross obstruction at the level of the lesion. Therefore, resection of the affected segment must be performed. The continuity of the ureter is restored by an oblique end-to-end anastomosis without splinting of the ureter or urine deviation nephrostomy or ureterostomy. 5 This procedure 5 Bovee, J. W.: A critical survey of ureternl implantation. Ann. Surg., 32: 165, 1900.
32
MAGED AKD SOLIMAN
has been found to be quite satisfactory and results in great improvement in renal function. Urine will leak for sometime from the wound but will gradually cease.
Fm. 2. Case 1. Microphotograph of section shows squamous metaplasia and keratinization of mucosa.
CASE REPORT
Case 1. A. S., a 43-year-old man, complained of bilateral renal colic. X-ray studies revealed bilharzial calcification of the ureters and bladder and a 12 cm. radiopaque shadow occupying the upper third of the left ureter and part of the renal pelvis (fig. 1, A.). Severe left hydronephrosis and a dilated kinked ureter below the opaque shadow were seen on excretory urography (fig. 1, B). The diagnosis was a giant calculus of the left ureter. The left ureter was exposed through a lumbar incision. No stone was found but the previously described thickening of the ureter was resected with part of the renal pelvis (fig. 1, C and D). A ureteropyeloplasty was performed. Convalescence was uneventful. The removed ureteral segment measured 10 cm. in length. Microscopically sandy patches replaced the complete thickness of the ureter and the mucosa revealed
Fm. 3. Case 2. A., plain x-ray shows 2 stones in right kidney and stone in ureter at L5. B, excretory urogram shows severe hydroureter and hydronephrosis. C, cut sections of calcareous segment, upper and lower portions. Note homogeneous surface and fading of lesion in lower section. D, microscopic examination reveals massive invasion of muscle coat by Bilharzia ova.
BILHARZIAL PSEUDO-CALCULUS OF URETER
FIG. 01. Case 3. A, cut section of removed segment. B, microscopic study reveals ureterit,i.s cy,c;Liea rn m.ucosa and Bilharzia ova in submucosa. ·
Frn. 5. Case 4. A, plain hydroureter. C, cnt section
shows calcified shadow at level of Li3. B, excretory urogram show::: removed segment.
squamous mctaplasia with marked keratinization (fig. 2). Case 2. S. S., a 32-year-old man, suffered from recurreut attacks of right renal colic with passage of gravel. X-ray studies showed 2 stones in the right kidney and a racliopaque shadow in the ureter opposite L5 (fig. 3, . An excretory urogram revealed no function on the left side and severe right hydrometer and hydronephrosis (fig. 3, B). The right kidney and ureter were exposed through a lumbar incision. The segment of ureter with bilharzia! pseudo-calculus was resected and the 2 renal calculi were removed through the proximal cut encl of the ureter (fig. 3, C). An oblique end-to-encl anastomosis was performed. Postoµerativdy urine leaked from the wound for 9 clays but, thereafter, cmwalescence was uneventful. ~\Iic:ro,c;copieal examination revealed sandy patches of bilharziasis and an ulcerated mucosa (fig. 3, D). Case 3. A. H., a 26-year-old man, complained of right renal colic. X-ray studies showed a racliopaque shadow in the ureter opposite L5. A poorly functioning h_1'dronephrotic right kidney and a normal left kidney were seen on excre-
tory urography. On operation, the described lesion was found and resected The ureter was re-anastomosed. the removed segment showed bilharziasis involving the muscle showed ureteritis cy,c;tica (fig. 4, B). Case 4. G. B., a 47-year-olcl man, had recurTrnt attacks of bilateral renal colic, more pronorniced on the right side, and hematuria. .:;tudies revealed a radiopaque shadow in the ureter at the level of the fifth lumbar vertebra 5,, L). An excretory urogram revealed a marked of hydrometer but the right kidney almost normal (fig. 5, B). The right ureter was exposed a low lumbar inci,ion and the pl'e· viou:;ly described le:;ion was ~een: thi:; ~cgmcnt of ureter was resectecl (fig. 5, C) and an end-to-encl anastomosis was cl.one. cally the remcrrncl segment of the ureter revt'aled sandy patches of bilharziasi:; and the mnco~a wa.'i ulcerated. Case 5. F. :VI., a 20-year-old man, had renal colic. An excretory urogram revealed a stoue in the lmver calyx of the right and a 2.5 cm. den~e radiopaquc shadow in the right ureter
34
MAGED AND SOLIMAN
FIG. 6. Case 5. A, excretory urogram shows stone in lower calyx of poorly functioning right kidnev and 2.5 cm. calcareous shadow in ureter opposhe L5. B, cut section of removed specimen. C, microscopic examination reveals replacement of muscle coat by Bilharzia ova.
opposite L5 (fig. 6, A). At operation a pseudocalculus was seen. The affected segment of the ureter was resected (fig. 6, B) and the ureter was re-anastomosed; the kidney stone was removed. M.icroscopically the same picture previously described was found and the mucosa was ulcerated (fig. 6, C).
ureteritis. Microscopically sandy patches with clusters of dead and calcified Bilharzia ova replace the thickness of the ureteral wall. There is minimal tissue reaction and hardly any muscle tissue in between the calcified areas. Such a picture is unusual as sandy patches are not known to extend beyond the submucosa. Since the majority of the ova are calcified, the density of the shadow is great in the x-rays. The extent of infiltration in the wall of the ureter is not the same along the whole circumference since part of the wall is 7 or 8 mm. in thickness, while the remainder is not more than 3 mm. As a result, the lumen of the ureter is not central in most cases. The ureteral lumen is not stenosed at the site of the lesion and the mucosal lining may be absent or show changes, such as ureteritis oystica or squamous metaplasia. Even though the lumen is not narrowed, there is definite obstruction at the site of the lesion. This can be explained by the marked rigidity of the ureter and its being fixed in this portion, therefore, obstructing any peristaltic wave and preventing the normal worm-like mobility of the ureter. The character of this lesion denotes a very severe degree of bilharzial infiltration and it was therefore expected to be seen in those portions of the ureter which correspond anatomically to communications between the ureteral and portal veins, i.e. the lower ends and opposite L3. The occurrence of this lesion far away from these areas (as previously reported) is rather difficult to explain, but it would suggest the presence of abnormal communication in these parts, either congenital or recently acquired due to obstruction of the portal veins by the Bilharzia worms.
DISCUSSION
Bilharzial pseudo-calculus of the ureter is a massive infiltration of Bilharzia ova in a localized portion of the ureter. The shadow seen by x-rays resembles a stone. The affected portion of the ureter becomes transformed into hard calcareous and tough tissue surrounded by marked peri-
SUMMARY
A new type of bilharzial calcification of the ureter resembling ureteral calculus is described. The clinical picture, radiological appearances, pathology and treatment are disrussed and 5 cases are reported.
THE JOURNAL OF UROLOGY
Copyright © 1968 by The Williams & Wilkins Co.
BILHARZIAL PSEUDO-CALCULUS OF THE URETER ALY MAGED
AND
L.A.M. SOLIMAN
From the Departments of Urology and Pathology, Faculty of Medicine, Ein Shams University, Cairo, Egypt, U.A.R.
A rare form of bilharzial calcification of the ureter is herein described. It has been referred to as bilharzial pseudo-calculus of the ureter because a well-defined calcareous ureteral shadow, similar in appearance to a stone, was noted by x-ray and the true condition was not identified until the operation.
U reteral bilharziasis is a common disease producing different types of lesions. Mild lesions cause ureteral irritation and spasms, while severe lesions cause serious ureteral damage and, ultimately, lead to the destruction of the renal parenchyma. 1 The oviposition and accompanying cellular reaction may be diffuse involving the major part of the ureter but is more commonly localized. 2 Heavy bilharzial infiltrations occur selectively in certain segments of the organ because of the communication between the ureteral veins and portal system. These infiltrations usually lead to stenotic lesions; therefore, strictures are frequently seen in the intramural and juxtavesical portions of the ureter and, to a lesser extent, at the level of the third lumbar vertebra. Calcification in the ureteral wall is common and pathognomonic. It is usually caused by the large number of dead and calcified Bilharzia ova which is deposited in the submucosa. On a plain x-ray, the calcification resembles a faint outline of the ureter and sometimes appears linear. These areas are usually found in the lower end of the ureter but sometimes the complete ureter may be calcified. Calcification of a different nature is seen in ureteritis calcinosa. In this disease the area has punctate calcific spots due to calcification occurring in the contents of ureteritis cystica with further deposition of urinary salts on top resulting in the formation of small primary ureteral calculi. 3 Occasionally, calcification may occur in a bilharzial polyp arising in the dilated segment proximal to a stricture and appear in x-rays as a dense calcified shadow. 4
MATERIAL
Only 5 cases have been encountered in more than 2,000 cases of bilharzial ureters. All 5 cases involved male patients. The lesions were on the right side opposite the L5 vertebra in 4 cases and on the left side in 1 case. In the latter case, the lesion was quite extensive, infiltrating part of the renal pelvis; it was diagnosed as a giant calculus of the ureter. In 2 cases, associated renal calculi were present. In all cases, a marked degree of ureteral obstruction at the level of the lesion was noted. All patients had symptoms of ureteral or renal calculi and suffered frequent, recurrent attacks of renal colic, hematuria and, sometimes, passage of gravel. Radiological studies revealed a welldefined dense radiopaque shadow in the ureter which resembled a stone. On excretory urography, gross obstruction around the calcified area and marked hydrometer and hydronephrosis were noted. A diagnosis of ureteral calculus was made in all cases and operative removal was the choice of treatment. Exposure of the ureter at the site of the alleged stone revealed a well-defined, fusiform, hard swelling which varied from 1.5 and 2.5 cm. in 4 cases and was 10 cm. in 1 case. This swelling was surrounded by dense fibrofatty tissue, on the surface of which were tortuous and dilated thinwalled veins. This particular part of the ureter was very adherent to the surrounding tissues and overlying peritoneum. Dissection was always difficult and resulted in tearing of the dilated veins with troublesome ooze. On incision a very tough and gritty tissue was noted along the thickness of the ureter until the lumen was reached. The lumen was void of calculi and neither stenosed or narrowed. The cut surface of the ureter was pale yellow with a rough and ho-
Accepted for publication September 27, 1966. Ibrahim, B.: Bilharziasis of the ureter. Lancet,
1
2: 1184, 1923. 2 Zaher, M. F., Safwat, lVI. M., Fawzi, R. M. and Badr, lVI. M.: Bilharzial non-obstructive spindle shaped ureter. J. Egypt Med. Ass., 39: 213-219, 1956. 3 El Ghorab, M. A.: Ureteritis calcinosa: A complication of bilharzial ureteritis and its relation to primary ureteric stone formation. Brit. J. Urol., 34: 33, 1962. 4 Makar, N.: The bilharzial ureter: Some observations on the surgical pathology and surgical treatment. Brit. J. Surg., 36: 148, 1948.
30
:BILHARZIAL PSEUDO-CALCULUS OF URETER
FIG. 1. Case 1. A, plain x-ray shows giant calculus in left ureter. B, excretory urogram shows nephrosis. C, removed segment of ureter. D, cut sections, upper and lower portions.
mogeneous surface and usually revealed a nonuniform thickness of the ureteral wall, varying from 3 to 8 mm. Because of the thickness of the wall, the lumen acquired an eccentric position in relation to it. The ureter was markedly dilated above the hard, thick portion, while the lower part of the lesion faded gradually into the distal portion of the ureter with no sharp limit. lVIicroscopic examination of the resected ureteral segment revealed massive infiltration by Bilharzia ova; wide areas of dead and calcified ova ,-vith hardly any tissue in between were seen. Such a picture is obtained in the well-known sandy patches which is essentially a submucosal lesion. However, the lesions herein reported on replaced not only the submucosa but practically the complete muscle coat of the ureter. The latter being completely, or very near completely,
absent and, instead, there were Bilharzia ova, a high proportion of which being calcified and of the hematobium variety. The mucosa! lining; was either ulcerated or revealed ureteritis cysticti, or squamous metaplasia with marked keratiniza. tion. Bilharzia] pseudo-calculus is always discovered during an operation originally planned for re-· moval of a stone. Although no actual narrowing of the lumen is present there is evidence of gross obstruction at the level of the lesion. Therefore, resection of the affected segment must be performed. The continuity of the ureter is restored by an oblique end-to-end anastomosis without splinting of the ureter or urine deviation nephrostomy or ureterostomy. 5 This procedure 5 Bovee, J. W.: A critical survey of ureternl implantation. Ann. Surg., 32: 165, 1900.
32
MAGED AKD SOLIMAN
has been found to be quite satisfactory and results in great improvement in renal function. Urine will leak for sometime from the wound but will gradually cease.
Fm. 2. Case 1. Microphotograph of section shows squamous metaplasia and keratinization of mucosa.
CASE REPORT
Case 1. A. S., a 43-year-old man, complained of bilateral renal colic. X-ray studies revealed bilharzial calcification of the ureters and bladder and a 12 cm. radiopaque shadow occupying the upper third of the left ureter and part of the renal pelvis (fig. 1, A.). Severe left hydronephrosis and a dilated kinked ureter below the opaque shadow were seen on excretory urography (fig. 1, B). The diagnosis was a giant calculus of the left ureter. The left ureter was exposed through a lumbar incision. No stone was found but the previously described thickening of the ureter was resected with part of the renal pelvis (fig. 1, C and D). A ureteropyeloplasty was performed. Convalescence was uneventful. The removed ureteral segment measured 10 cm. in length. Microscopically sandy patches replaced the complete thickness of the ureter and the mucosa revealed
Fm. 3. Case 2. A., plain x-ray shows 2 stones in right kidney and stone in ureter at L5. B, excretory urogram shows severe hydroureter and hydronephrosis. C, cut sections of calcareous segment, upper and lower portions. Note homogeneous surface and fading of lesion in lower section. D, microscopic examination reveals massive invasion of muscle coat by Bilharzia ova.
BILHARZIAL PSEUDO-CALCULUS OF URETER
FIG. 01. Case 3. A, cut section of removed segment. B, microscopic study reveals ureterit,i.s cy,c;Liea rn m.ucosa and Bilharzia ova in submucosa. ·
Frn. 5. Case 4. A, plain hydroureter. C, cnt section
shows calcified shadow at level of Li3. B, excretory urogram show::: removed segment.
squamous mctaplasia with marked keratinization (fig. 2). Case 2. S. S., a 32-year-old man, suffered from recurreut attacks of right renal colic with passage of gravel. X-ray studies showed 2 stones in the right kidney and a racliopaque shadow in the ureter opposite L5 (fig. 3, . An excretory urogram revealed no function on the left side and severe right hydrometer and hydronephrosis (fig. 3, B). The right kidney and ureter were exposed through a lumbar incision. The segment of ureter with bilharzia! pseudo-calculus was resected and the 2 renal calculi were removed through the proximal cut encl of the ureter (fig. 3, C). An oblique end-to-encl anastomosis was performed. Postoµerativdy urine leaked from the wound for 9 clays but, thereafter, cmwalescence was uneventful. ~\Iic:ro,c;copieal examination revealed sandy patches of bilharziasis and an ulcerated mucosa (fig. 3, D). Case 3. A. H., a 26-year-old man, complained of right renal colic. X-ray studies showed a racliopaque shadow in the ureter opposite L5. A poorly functioning h_1'dronephrotic right kidney and a normal left kidney were seen on excre-
tory urography. On operation, the described lesion was found and resected The ureter was re-anastomosed. the removed segment showed bilharziasis involving the muscle showed ureteritis cy,c;tica (fig. 4, B). Case 4. G. B., a 47-year-olcl man, had recurTrnt attacks of bilateral renal colic, more pronorniced on the right side, and hematuria. .:;tudies revealed a radiopaque shadow in the ureter at the level of the fifth lumbar vertebra 5,, L). An excretory urogram revealed a marked of hydrometer but the right kidney almost normal (fig. 5, B). The right ureter was exposed a low lumbar inci,ion and the pl'e· viou:;ly described le:;ion was ~een: thi:; ~cgmcnt of ureter was resectecl (fig. 5, C) and an end-to-encl anastomosis was cl.one. cally the remcrrncl segment of the ureter revt'aled sandy patches of bilharziasi:; and the mnco~a wa.'i ulcerated. Case 5. F. :VI., a 20-year-old man, had renal colic. An excretory urogram revealed a stoue in the lmver calyx of the right and a 2.5 cm. den~e radiopaquc shadow in the right ureter
34
MAGED AND SOLIMAN
FIG. 6. Case 5. A, excretory urogram shows stone in lower calyx of poorly functioning right kidnev and 2.5 cm. calcareous shadow in ureter opposhe L5. B, cut section of removed specimen. C, microscopic examination reveals replacement of muscle coat by Bilharzia ova.
opposite L5 (fig. 6, A). At operation a pseudocalculus was seen. The affected segment of the ureter was resected (fig. 6, B) and the ureter was re-anastomosed; the kidney stone was removed. M.icroscopically the same picture previously described was found and the mucosa was ulcerated (fig. 6, C).
ureteritis. Microscopically sandy patches with clusters of dead and calcified Bilharzia ova replace the thickness of the ureteral wall. There is minimal tissue reaction and hardly any muscle tissue in between the calcified areas. Such a picture is unusual as sandy patches are not known to extend beyond the submucosa. Since the majority of the ova are calcified, the density of the shadow is great in the x-rays. The extent of infiltration in the wall of the ureter is not the same along the whole circumference since part of the wall is 7 or 8 mm. in thickness, while the remainder is not more than 3 mm. As a result, the lumen of the ureter is not central in most cases. The ureteral lumen is not stenosed at the site of the lesion and the mucosal lining may be absent or show changes, such as ureteritis oystica or squamous metaplasia. Even though the lumen is not narrowed, there is definite obstruction at the site of the lesion. This can be explained by the marked rigidity of the ureter and its being fixed in this portion, therefore, obstructing any peristaltic wave and preventing the normal worm-like mobility of the ureter. The character of this lesion denotes a very severe degree of bilharzial infiltration and it was therefore expected to be seen in those portions of the ureter which correspond anatomically to communications between the ureteral and portal veins, i.e. the lower ends and opposite L3. The occurrence of this lesion far away from these areas (as previously reported) is rather difficult to explain, but it would suggest the presence of abnormal communication in these parts, either congenital or recently acquired due to obstruction of the portal veins by the Bilharzia worms.
DISCUSSION
Bilharzial pseudo-calculus of the ureter is a massive infiltration of Bilharzia ova in a localized portion of the ureter. The shadow seen by x-rays resembles a stone. The affected portion of the ureter becomes transformed into hard calcareous and tough tissue surrounded by marked peri-
SUMMARY
A new type of bilharzial calcification of the ureter resembling ureteral calculus is described. The clinical picture, radiological appearances, pathology and treatment are disrussed and 5 cases are reported.