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Congenital Ureteral Valve
THE JOURNAL OF UROLOGY
Vol. 81, No. 2, February 1959 Printed in U.S.A.
CONGENITAL URETERAL VALVE EZATOLLAH FOROUGHI
AND
J. A. TURNER
From the Department of Pathology of Veterans Administration Hospital, Kansas City 12, Mo.
Congenital ureteral valve is a very rare cause of intrinsic upper urinary tract obstruction. W olffer1 drew attention to the presence of the ureteral valve for the first time. The presence of mucosal folds has been shown in 5 to 20 per cent of newborn autopsies. 2- 4 These mucosal folds disappear before or soon after birth. 5 The mucosa] valve and kink-formation arc found commonly in dilated and tortuous hydroureters due to any obstructive cause. Study of the world litcrature 6 in 1952 revealed five authentic cases of congenital ureteral valve; since then three more cases, apart from our case, have been added. The criteria 6 which are suggested in the evaluation of the authenticity of the congenital ureteral valve are: 1) Anatomically demonstrable transverse folds, containing bundles of smooth muscle fibers. 2) Secondary obstructive changes above and absence of these changes below the site of the valve. 3) Absence of any other ureteral obstructive cause, either mechanical or functional. The etiology of the ureteral valve is unknown. The persistence of the formed Chwalla's membrane, in 30 mm. embryo, in closing the lumen of the uretcral bud can explain the etiology of the low-located ureteral valves,7 as in our case. This has also been shown in mice, 19-25 days old. 8 This assumption is not applicable to the valves of high location. The oldest previously recorded Accepted for publication July 29, 1958. 1 Wolffer, J.: Arch. ldin. Chir., 21: 694, 1887. 2 Eisendrath, D. N. and Rolnick, H. C.: Textbook of Urology. Philadelphia: J. B. Lippincott Co., 1943, p. 537. 3 Caulk, J. R.: Megaloureter: The importance of uretero-vesical valve. J. Urol., 9: 315, 1923. 4 Eisendrath, D. N.: Congenital strictures of the ureter. Surg. Clin. N. Amer., 4: 565, 1924. 5 Vermooten, V.: A new etiology for certain types of dilated ureters in children. J. Urol., 41: 455, 1939. 6 Wall, B. and Wachter, H.: Congenital ureteral valve; its role as a primary obstructive lesion; classification of the literature and report of an authentic case: J. Urol., 68: 684, 1952. 7 Chwalla, R.: Urol. and Cutan. Rev., 31: 499,
1927. 8
Brown, A.: Am. J. Anat., 47: 117, 1931. 272
case is a 43-year-old man. 9 The lesion appears in equal proportion in both sexes. The valve is usually in the form of an annular iridic diaphragm with a pinpoint opening. The valve in one case10 and also in our case are cuspshaped (fig. 1). All layers of the wall of the ureter except adventitia are present in the structure of the valve. The lesion is found in four reported cases in the right ureter, three cases in the left ureter, and in one bilaterally. There is a record of complete right ureteral valve obstruction associated with urethral valve in a newborn infant.11 The symptoms arc commonly those related to the upper urinary tract obstruction or possible superimposed infection. Hypertension was present in two previously reported c:ases 9 •12 and in our c:ase. CA8B REPORT
A 68-year-old man was admitted to Veterans Administration Hospital, Kansas City, Missouri, on October 10, 19.57, in a state of shock, while complaining of diffuse abdominal pain, and died six hours later. He had experienced retrosternal pain radiating to left shoulder ancl arm and a few spells of vomiting for three weeks prior to admission. He had been a miner in lead, tin, zinc, copper mines for 40 years. He was a moderate drinker and smoked a pack of cigarettes a day. He was treated in this hospital for duodenal ulcer, in 1953 and 1955; he had a gradual increase in his blood pressure to about 190/95 mm. of mercury. The urine revealed an increase in 2 albumin up to plus, with concomitant occasional white and red blood cells and a few hyalin and granule casts. Urine specific gravity in all admissions was about 1.005. Intravenous pyelography did not visualize a right renal shadow. The excretion of dye in 9 Cabot case records of Massachusetts General Hospital, case 13072, two diagnoses are better than one. Boston Med. and Surg. J., 196: 276-279, 1927. 10 Runner, H. and Wharton, L.: The pathological findings in cases clinically diagnosed as ureteral stricture. J. Urol., 15: 57, 1926. 11 Robert, R.: J. Urol., 76: 62-65, 1956. 12 Simon, H. B., Culp, 0. S. and Parkhill, E.: Congenital ureteral valve, report of two cases. J. Urol., 74: 336, 1955.
CONGENITAL URETERAL VALVE
FIG. 1. Atrophic a11cl h:nlroncphrnlic kidney wi1h cn,sp-slrnped valve at. ureterove:sical junction.
plwnolsulfonphthalcin test was :3.'i pm cent at 2 hours. The: patient was a well dcvrlopecl and nourished man, with cyanotic extrc:mitics while perspiring profusefr The systolic arterial blood pressure was 7G mm. of mercury, pubc J20 ancl respiration ;30 pc-:r minute. The liver extended 8 cm. below the right costal margin. There were coarse, crackling, mies posteriorly over the right base of the clirst. A graclc; 3 systolic murmur m1s heard in all areas of the heart, but loudest at the apex. Laboratory and x-ray studies: Hemoglobin was 1::Ul gm., and hematocrit 43 volurnecs per cent. Serum transaminase m1s 288 units per ml. Portable x-ray of the chest revrnled some accentuation of the bronchcrvm,cnlar markings in the base~. .\n electrocarcliogran1 revealed changc:s typical of posterior septa] myocardial infarct. An topsy: ~lild gencra.lizc,cl atlwrosclerosis, except for marked change in corom1r:,· arteries, ,vas found. The right corom1r)· artery reveak'd recent hemorrhage into an atheromatous plaque and thrombus formation. Tlwre was a recent posterior septa] myocarcli,t! infarct. Marked passive congestion of the viscem mLs present. Frequent fibrocaseous nodules of O.ii to l cm. were scattc,rc,cl in both lungs aud carinal lymph nodes. Cryptoeoecus neoformans was demonstrated in these, fibrocaseous lesions.
The left kidney weighed 2 lO gm. and had the microscopical findings of arteriolar sis. The right kidney weighed 50 gm. and mea,. ured 8 by 6 by 4 cm. in the greatest diameters. The perircnal fat was adhered to the kidney cap sule. The exkrior surface was bossalatcd with intervening thin, atrophic fibrous kidnc:y tisRuc (fig. 1). The fibrous capsule Rtrippc:d with di/iiculty. The thickness of the whole parenchyma varied from 11 to 5 mm. a.nc! that of th(, cortex from 7 to 3 mm. The calyces and pelvis 11cre markedly dilated and thiekened. The single remd artery which entered into the kidney through the hilus 11·as hypoplastic and measured 2 mm. in the e:xterior diameter. The, microscopic examination of the right kidney revealed pyelonephritic changes. The right ureter had a normal course to the urinary bladder, but was dilated throughout to the ureterovesical junction. The circumforcnce uf tbe ureter was 9 mm. The uretcral orifice was easily identified, probed ancl opened from th,; urinary bladder. Cpon opening the right ureter, there appeared a cusp-sh,1pecl valve 1 cm. in length, which had the basal attachment near the ureterovesic:al :junction while the frne c:dgc attached anteriorly 1 cm. proxinrnl (fig. Thi:, valve was fibrotic, semitransparent near the: free edge, thicker and whitish near the site of attach· ment. The section of the lm1cc,r part of tlw 1miter revealed the cusp as being a projection along the
FrG. 2. Perpendicular section of valve of ureter shows bundle:s of smooth mmcle fibers in valves.
274
EZATOLLAH FOROt;GHI .-\Kll J. A. 'l't;R~ER
wall of the ureter. This projection consisted of connective tissue, containing bundles of smooth muscle fibers. The muscle fibers were more abundant at the base near the site of attachment. The interstitial tissue of the valve contained slight diffuse infiltrate of lymphocytes and plasma cells (fig. 2). SUMMARY
The case reported here is the ninth authentic reported case of the congenital ureteral valve.
The patient, aged 68, is the oldest such case reported in the literature. The cusp-like shape of the valve is the second such case reported in the literature. The association of the cong:enital ureteral valve with hypertension is interesting:, this being the third case with this relationship reported. The "pathogenic Chwalla" theory is applicable to our case because of the anatomic location of the valve.
Vol. 81, No. 2, February 1959 Printed in U.S.A.
CONGENITAL URETERAL VALVE EZATOLLAH FOROUGHI
AND
J. A. TURNER
From the Department of Pathology of Veterans Administration Hospital, Kansas City 12, Mo.
Congenital ureteral valve is a very rare cause of intrinsic upper urinary tract obstruction. W olffer1 drew attention to the presence of the ureteral valve for the first time. The presence of mucosal folds has been shown in 5 to 20 per cent of newborn autopsies. 2- 4 These mucosal folds disappear before or soon after birth. 5 The mucosa] valve and kink-formation arc found commonly in dilated and tortuous hydroureters due to any obstructive cause. Study of the world litcrature 6 in 1952 revealed five authentic cases of congenital ureteral valve; since then three more cases, apart from our case, have been added. The criteria 6 which are suggested in the evaluation of the authenticity of the congenital ureteral valve are: 1) Anatomically demonstrable transverse folds, containing bundles of smooth muscle fibers. 2) Secondary obstructive changes above and absence of these changes below the site of the valve. 3) Absence of any other ureteral obstructive cause, either mechanical or functional. The etiology of the ureteral valve is unknown. The persistence of the formed Chwalla's membrane, in 30 mm. embryo, in closing the lumen of the uretcral bud can explain the etiology of the low-located ureteral valves,7 as in our case. This has also been shown in mice, 19-25 days old. 8 This assumption is not applicable to the valves of high location. The oldest previously recorded Accepted for publication July 29, 1958. 1 Wolffer, J.: Arch. ldin. Chir., 21: 694, 1887. 2 Eisendrath, D. N. and Rolnick, H. C.: Textbook of Urology. Philadelphia: J. B. Lippincott Co., 1943, p. 537. 3 Caulk, J. R.: Megaloureter: The importance of uretero-vesical valve. J. Urol., 9: 315, 1923. 4 Eisendrath, D. N.: Congenital strictures of the ureter. Surg. Clin. N. Amer., 4: 565, 1924. 5 Vermooten, V.: A new etiology for certain types of dilated ureters in children. J. Urol., 41: 455, 1939. 6 Wall, B. and Wachter, H.: Congenital ureteral valve; its role as a primary obstructive lesion; classification of the literature and report of an authentic case: J. Urol., 68: 684, 1952. 7 Chwalla, R.: Urol. and Cutan. Rev., 31: 499,
1927. 8
Brown, A.: Am. J. Anat., 47: 117, 1931. 272
case is a 43-year-old man. 9 The lesion appears in equal proportion in both sexes. The valve is usually in the form of an annular iridic diaphragm with a pinpoint opening. The valve in one case10 and also in our case are cuspshaped (fig. 1). All layers of the wall of the ureter except adventitia are present in the structure of the valve. The lesion is found in four reported cases in the right ureter, three cases in the left ureter, and in one bilaterally. There is a record of complete right ureteral valve obstruction associated with urethral valve in a newborn infant.11 The symptoms arc commonly those related to the upper urinary tract obstruction or possible superimposed infection. Hypertension was present in two previously reported c:ases 9 •12 and in our c:ase. CA8B REPORT
A 68-year-old man was admitted to Veterans Administration Hospital, Kansas City, Missouri, on October 10, 19.57, in a state of shock, while complaining of diffuse abdominal pain, and died six hours later. He had experienced retrosternal pain radiating to left shoulder ancl arm and a few spells of vomiting for three weeks prior to admission. He had been a miner in lead, tin, zinc, copper mines for 40 years. He was a moderate drinker and smoked a pack of cigarettes a day. He was treated in this hospital for duodenal ulcer, in 1953 and 1955; he had a gradual increase in his blood pressure to about 190/95 mm. of mercury. The urine revealed an increase in 2 albumin up to plus, with concomitant occasional white and red blood cells and a few hyalin and granule casts. Urine specific gravity in all admissions was about 1.005. Intravenous pyelography did not visualize a right renal shadow. The excretion of dye in 9 Cabot case records of Massachusetts General Hospital, case 13072, two diagnoses are better than one. Boston Med. and Surg. J., 196: 276-279, 1927. 10 Runner, H. and Wharton, L.: The pathological findings in cases clinically diagnosed as ureteral stricture. J. Urol., 15: 57, 1926. 11 Robert, R.: J. Urol., 76: 62-65, 1956. 12 Simon, H. B., Culp, 0. S. and Parkhill, E.: Congenital ureteral valve, report of two cases. J. Urol., 74: 336, 1955.
CONGENITAL URETERAL VALVE
FIG. 1. Atrophic a11cl h:nlroncphrnlic kidney wi1h cn,sp-slrnped valve at. ureterove:sical junction.
plwnolsulfonphthalcin test was :3.'i pm cent at 2 hours. The: patient was a well dcvrlopecl and nourished man, with cyanotic extrc:mitics while perspiring profusefr The systolic arterial blood pressure was 7G mm. of mercury, pubc J20 ancl respiration ;30 pc-:r minute. The liver extended 8 cm. below the right costal margin. There were coarse, crackling, mies posteriorly over the right base of the clirst. A graclc; 3 systolic murmur m1s heard in all areas of the heart, but loudest at the apex. Laboratory and x-ray studies: Hemoglobin was 1::Ul gm., and hematocrit 43 volurnecs per cent. Serum transaminase m1s 288 units per ml. Portable x-ray of the chest revrnled some accentuation of the bronchcrvm,cnlar markings in the base~. .\n electrocarcliogran1 revealed changc:s typical of posterior septa] myocardial infarct. An topsy: ~lild gencra.lizc,cl atlwrosclerosis, except for marked change in corom1r:,· arteries, ,vas found. The right corom1r)· artery reveak'd recent hemorrhage into an atheromatous plaque and thrombus formation. Tlwre was a recent posterior septa] myocarcli,t! infarct. Marked passive congestion of the viscem mLs present. Frequent fibrocaseous nodules of O.ii to l cm. were scattc,rc,cl in both lungs aud carinal lymph nodes. Cryptoeoecus neoformans was demonstrated in these, fibrocaseous lesions.
The left kidney weighed 2 lO gm. and had the microscopical findings of arteriolar sis. The right kidney weighed 50 gm. and mea,. ured 8 by 6 by 4 cm. in the greatest diameters. The perircnal fat was adhered to the kidney cap sule. The exkrior surface was bossalatcd with intervening thin, atrophic fibrous kidnc:y tisRuc (fig. 1). The fibrous capsule Rtrippc:d with di/iiculty. The thickness of the whole parenchyma varied from 11 to 5 mm. a.nc! that of th(, cortex from 7 to 3 mm. The calyces and pelvis 11cre markedly dilated and thiekened. The single remd artery which entered into the kidney through the hilus 11·as hypoplastic and measured 2 mm. in the e:xterior diameter. The, microscopic examination of the right kidney revealed pyelonephritic changes. The right ureter had a normal course to the urinary bladder, but was dilated throughout to the ureterovesical junction. The circumforcnce uf tbe ureter was 9 mm. The uretcral orifice was easily identified, probed ancl opened from th,; urinary bladder. Cpon opening the right ureter, there appeared a cusp-sh,1pecl valve 1 cm. in length, which had the basal attachment near the ureterovesic:al :junction while the frne c:dgc attached anteriorly 1 cm. proxinrnl (fig. Thi:, valve was fibrotic, semitransparent near the: free edge, thicker and whitish near the site of attach· ment. The section of the lm1cc,r part of tlw 1miter revealed the cusp as being a projection along the
FrG. 2. Perpendicular section of valve of ureter shows bundle:s of smooth mmcle fibers in valves.
274
EZATOLLAH FOROt;GHI .-\Kll J. A. 'l't;R~ER
wall of the ureter. This projection consisted of connective tissue, containing bundles of smooth muscle fibers. The muscle fibers were more abundant at the base near the site of attachment. The interstitial tissue of the valve contained slight diffuse infiltrate of lymphocytes and plasma cells (fig. 2). SUMMARY
The case reported here is the ninth authentic reported case of the congenital ureteral valve.
The patient, aged 68, is the oldest such case reported in the literature. The cusp-like shape of the valve is the second such case reported in the literature. The association of the cong:enital ureteral valve with hypertension is interesting:, this being the third case with this relationship reported. The "pathogenic Chwalla" theory is applicable to our case because of the anatomic location of the valve.