- Email: [email protected]
Triplication of the Ureter and Renal Pelvis
THE JOURNAL OF UROLOGY
Vol. 68, No. 1, July 1952 Printed in U.S.A.
TRIPLICATION OF THE URETER AND RENAL PELVIS RICHARD D. GILL
Unusual anomalies of the genito-urinary tract occur fairly frequently compared with the other systems of human anatomy. The average urologic diagnostician manifests much more interest in the unusual anomaly and is much more apt to record his observations. Such was the case when we encountered a patient with three kidney pelves and three ureters on one side and two kidney pelves and two ureters on the opposite side. EMBRYOLOGY
To understand the occurrence of ureteral anomalies one must be somewhat conversant with the fundamentals of the development of the genito-urinary tract. The metanephros frequently referred to as the permanent kidney makes its appearance in the human embryo about the fourth week. The metanephros may be divided into two portions, the secretory and the efferent apparatus. The efferent apparatus actually develops from the primary excretory duct, which by an outgrowth forms a ureter on either side. The ureteric anlage appears, according to Keibel and Mall, between the stages of 4.5 mm. and 5.3 mm. total length. The short ureteral bud grows at first almost altogether dorsally toward the vertebral column and the blood vessels lying in front of it, but later in embryos of 8.5 to 9.5 mm. it forms a curve, which becomes flatter with advancing age and the ureter grows cranially. The end of the bud becomes dilated, forming a cap, the beginning of the renal pelvis. The cap divides into a cephalic and caudal branch, each giving rise to smaller evaginations which are destined to become the calyces and the collecting tubules which eventually unite with the uniniferous tubules, these having been derived from the nephrogenic cord (renal blastema) or the true secretory anlage, the metanephros. The rectum, bladder, urethra, and the urogenital sinus are formed from a triple division of the cloaca. The lower portion of the excretory duct enlarges and becomes continuous with the adjoining portion of the cloaca. The ureteral bud is incorporated into that portion of the cloaca which is to become the bladder. About the fifth or sixth week the cloaca separates into a dorsal rectal and a ventral urogenital segment. Thus the ureter and wolffian duct come to empty into the vesico-urethral anlage, but acquire separate openings, the former emptying above and laterally into the future trigonum vesicae. From the wolffian duct is formed the posterior urethra, vas deferens, epididymis and the seminal vesicle. Particularly pertinent to the understanding of the anomaly under discussion here, is the fact that there may arise two or three ureteral buds from the primary excretory duct or there may be a precocious splitting of a single ureteral bud, ureter fissus. This clefting may extend all the way down to its entrance into the bladder, thus giving rise to the cleft ureter with but a single orifice in the bladder. In instances of two or three or even multiple ureteral buds developing from the Read at annual meeting of Mid-Atlantic Section, American Urological Association, April 12, 1951.
140
141
TRIPLICATION OF URETER
primary excretory ducts the most caudal one usually shows the strongest development and reaches a more cranial level. This accounts for the fact that the lowest ureteral orifice on the trigone in case of multiple orifices connects with the upper most pelvis. This is referred to as the inversion relation of the ureteral orifices and their corresponding pelves. A mishap at this stage of development readily explains the congenital misplacement of some ureteral orifices, that is extravesical orifices or ureteral ectopia. There are those who disagree with this theory and think that multiple ureteral buds do not develop, that clefting occurs in a single ureteral bud and the result obtained depends only on the degree of clefting. Probably one embryologic theory will not explain all cases. From this extremely brief review or l n t e s t i n . : , l f f i a ~ -w.d. •duct
trifid bud '
teral ud (a)
(b)
w.d. ~licate ~bud )
Diagl'ammatic attempt to show development of"\OJ Normal u1·eter \blTl'i fid urete1· \cJT,·iplicate urete1·
Fm. 1 Multi branched Urete:·s - 3 Cases
R
R
R
Niemeyer 1814
Furstner 1897
L
Campbell 1937
Fm. 2
summary of the embryology involved it is not difficult to understand the manner in which triplication of the ureter might possibly occur. See figure 1. CLASSIFICATION AND INCIDENCE
Ureteral anomalies may be classified as I) anomalies of number; 2) anomalies of origin and termination; and 3) anomalies of form, caliber, and structure. As to the first classification, there may be A, agenesis of the ureter, B, duplication of the ureter, C, triplication of the ureter, D, multi-branched ureters (fig. 2). In this discussion we are dealing only with triplication of the ureter. Smith and Orkin in a statistical study of 471 congenital anomalies of the kidneys and ureters occurring in 18,460 consecutive urological admissions found no case of triplicate ureter. Campbell studying the uropathology in 26,480 autopsies
142
RICHARD D. GILL
found no instance of this anomaly. Abeshouse, in a most excellent discussion of ureteral ectopia and review of nearly 300 cases, found many concomitant anomalies of the genito-urinary tract but not one of ureteral triplication. While duplication of the ureter and kidney pelvis is relatively quite frequent, occurring in approximately 0.5 per cent of autopsy material and hundreds of cases being reported clinically, triplication of the ureter is undoubtedly one of the rarest anomalies of the upper urinary tract. Lau and Henline in 1931 gave us one of our earliest discussions on this subject and reported a case of triplication of the ureter and also reviewed the literature. They found 7 cases reported in the world literature from postmortem examinaTypes of trip Iicate urete1·
)
(
a b C d (a)Triple Cb) Double with one bifid Cc)Trifid
Cd) Double with inversion Y bifurcatio11
Tri pie Ureter 4 cases
R
Case I Case 2 LauWoodruffe Henline 1941
1931
Case 3 Chwalla 1935
Case 4 Burt-LaneHamilton 1941
Fm. 3
tions, but there were insufficient data to authentically classify these cases. Miller, in 1938, reported a proven case of triplication of the ureter. Burt, Lane, and Hamilton reported a case in 1941, and incidentally in their case one of the three ureters was ectopic. Woodruff, also, in 1941 reported a case. MacLean and Harding reported a case in 1945. Irvine Smith, in 1946, reviewed the world literature and gave a very excellent report on this subject and added a case of his own. He classified the triplicate ureter into the following types (fig. 3): 1) triple ureter, in which there are three separate and distinct ureters opening into the bladder or elsewhere; 2) double ureter with one bifid, in which two ureters may unite, leaving only two orifices (fig. 4); 3) trifid ureter, in which all three ureters may unite and drain through a single orifice (fig. 5) and 4) double, with inversion Y bifurcation and resulting three orifices.
143
TRIPLICATION OF URETER
Smith, in his exhaustive review of the literature in 1946, was able to findfour cases of triple ureter, two cases of double ureter with one bifid and four cases of trifid ureter and added one of his own making five or a total of eleven cases of ureteral triplication. We have been able to find one additional case, that reported by Withycombe in 1950 which is classified as a trifid ureter. The case herein reported is classified as a trifid ureter also and therefore brings this group to seven reported cases, and all cases of triplication of the ureter to thirteen. There is no doubt that there have · been a fair number of unreported cases. The average text in urology does not Double Ureter with I .Bifid · 2 Cases
L
R
Cose I
Cose 2
"'fr:~y
MIiier 11158
Fm. 4 Tl"ifid Urete1· • 7 Cases-
L
Coa&I
Cose2
Sehmutte FrGnk 19211
11133
Tl'ifid Ureter - Continued
Col& 3
Case4
Cose 5
Case 6
Sehroter
Perrin
Smith
Withycombe
11137
1927
1946
1950
Case 7 Authors 1951
Fm. 5
mention the possibility of this anomaly. However, in Campbell's Pediatric Urology there is a picture of a trifid pelvis in a horseshoe kidney. In Wesson's Urologic Roentgenology there is a photograph, courtesy of A. K. Merchant, of triple renal pelves and ureters. In an article by Braasch and Scholl in 1922, on duplication of the renal pelvis and ureter, there is a photograph of three separate pelves leading from a common ureter. These have apparently been overlooked in the various surveys of the literature. In spite of the undoubted possibility of a number of unofficially reported cases, the fact still remains that triplication of the ureter is extraordinarily rare. SIGNS AND SYMPTOMS
There are no pathognomonic signs or symptoms of this condition; the diagnosis is made by urography, at operation or at autopsy.
144
RICHARD D. GILL CASE REPORT
Mrs. M. Y., a white married woman, 31 years of age, was referred to one of our general surgical colleagues with a working diagnosis of subacute appendicitis. The patient revealed that she had had some moderate attacks of pain in the right lower quadrant for several months. The present attack during the previous 48 hours had been much more severe and had required two hypodermics for relief. There had been no pain in the right kidney region posteriorly and no radiation of the pain to the genitalia or bladder region. There had been no dysuria or hematuria, and no chills or high fever. The patient's past genito-urinary history was entirely negative. There had been no attacks of any febrile condition in childhood that might have been interpreted as pyelitis. She was the mother of three children and had had no genito-urinary complications during pregnancy. On admission to the hospital patient's temperature was 99.6F. Routine blood work and blood chemistry showed no abnormalities except a leukocytosis of 13,000. Physical examination was negative except for the right abdomen, where there was well marked tenderness over McBurney's point, but no muscle spams, and there appeared to be a rather large indefinite mass, moderately tender, extending upward into the right upper quadrant and into the right flank. The mass was soft and extremely hard to outline and appeared to move somewhat with respiration. The catheterized bladder urine was persistently alkaline and loaded with pus and the patient was referred to us for complete urological study. At cystoscopy the bladder showed a chronic inflammatory reaction throughout with much purulent debris over the bladder floor. On the left side of the trigone there were two ureteral orifices. On the right side there was one orifice which was slightly larger than normal, and it was noted that the efflux of urine from the right orifice was more frequent than usual and the orifice seemed to gape open in a rather peculiar manner. Some obstruction was met about three inches above the bladder to the passage of a ureteral catheter on the right side. No obstruction was met to the passage of catheter in either of the two orifices on the left side. Retrograde pyelographic pictures demonstrated that we were dealing with an unusual anomaly, the exact nature of which would require further study, plus an impacted, rather large, ureteral calculus on the right side (fig. 6, A). There were two large atypical renal pelves with two hydro-ureters which apparently united above the impacted stone. There obviously was marked destruction of renal parenchyma and marked decrease in function on the right. Culture of the urine showed B. coli and bacillus alcaligenes. Function on the left side was normal. Shortly after the original urological study, the patient had severe chills and fever with an acute exacerbation of pain in the right flank and right kidney region posteriorly and an increase in the size of the mass in the right abdomen. In fact, she became sicker than she had ever been in her life. Attempt was made to introduce a ureteral catheter up the ureter past the stone for drainage of the acute process. This, however, met with failure. It was believed that the stone should be removed from the right ureter immediately and this was done without further delay. The ureter at the site of the stone was found very much enlarged and thickened. Upon opening the ureter and removing the calculus, there was a great gush of dammed-up purulent urine. A small catheter was introduced up the ureter for
TRIPLICATION OF URETER
145
drainage. The patient made a very good and prompt recovery and further urological studies were then carried out. The ureterostomy tube apparently had passed up the more medial of the two ureters and gradually there was some reduction in the size of the hydro-ureters and hydronephroses. The patient was discharged from the hospital with the ureterostomy tube in place and studies were
Fm. 6. A, first x-ray demonstrating calculus lying low in right ureter at tip of catheter. B, demonstrating two large, atypical pelves on right side with ureters joining in lower third. C, demonstrating two renal pelves on left side with two separate ureters. D, excretory urogram demonstrnting, for first time, a third pelvis on right side above the other two.
repeated intermittently. However, the tube accidentally came out, the wound healed with rapidity and with practically no escape of urine. The patient's condition continued to improve. She was entirely symptom free and refused further operative interference for approximately a year. She did, however, return frequently for observation and further urological study. During the course of
146
RICHARD D. GILL
these studies or approximately three or four months after removal of the ureteral calculus, there appeared in various x-ray films a shadow in the region of the upper pole of the right kidney above the two pelves which had not previously been present. This led us to suspect further additional renal tissue or pelvis which was
Fm. 7. A, flat film with catheter in right ureter approximately a year after original examination. Recurrence of calculi. B, right retrograde pyelogram demonstrating two pelves with joining ureters. Calculi apparently in third pelvis and ureter, but impossible to demonstrate by retrograde pyelography. ~
Fm. 8. X-ray demonstration of operative specimen
proven by excretory urography. A repeated search for an ectopic ureter gave negative results. There was not sufficient concentration of the dye to completely outline the ureter leading from the third pelvis. Repeated attempts to outline a third ureter, by retrograde pyelography, met with no success. After a considerable time, however, three fairly large stones, all apparently from this upper pelvis, developed and they moved up and down the ureter freely and without pain.
TRIPLICATION OF URETER
147
Finally, a little over a year after her original operation, the patient consented to enter the hospital for further observation and operation. She had started having further attacks of rather severe pain in the right flank accompanied by chills and fever, loss of weight and general malaise. There had been no attacks of pain or discomfort on the left side. Repeated urological study revealed the two left pelves to be essentially normal as to function. The function on the right side was remarkably reduced. See Figure 7. From our studies, we believed that we were dealing with three pelves on the right side and with two ureters from the lower two pelves fusing and a third ureter from the upper pelvis which we could not visualize except faintly in its upper portion by excretory urography. Preoperatively we were of the opinion that we were probably dealing with supernumerary kidneys. At operation, however, it was found that the three pelves were contained in one large capsule and that there were three ureters leading from the pelves, the two lateral of which united about half way down, running from this point to the bladder in one sheath. The third ureter ran separately down to the bladder wall where it apparently united with the other intramurally. There was very little renal parenchyma left in the entire kidney. The whole right renal mass was removed together with the ureters with some difficulty. As might well be imagined, the blood supply was quite anomalous and very copious. The pateient made a very excellent and quick recovery and in the matter of a few months she had gained approximately 20 pounds in weight, the urine became free of infection, acid in reaction, and urinary cultures became negative. Study of the pathological specimen revealed that there were three separate and distinct pelves with no communication between them. There were three separate ureters with fusion of the lateral two above the bladder. The third ureter apparently fused in the bladder wall, thereby presenting only one ureteral orifice on the right side. See figure 8. This case then can be classified as triplication of the renal pelvis and ureter with fusion of the three ureters or the so called trifid ureter. CONCLUSION
The incidence and embryology of triplication of the ureter and kidney pelvis has been reviewed and an additional case of this interesting anomaly has been reported. Wheeling Clinic, Wheeling, W. Va. REFERENCES ABESHOUSE, B. S.: Urol. & Cutan. Rev. 47: 447, 1943. BRAASCH, W. F. AND SCHOLL, A. J. JR.: J. Urol. 8: 507-546, 1922. BURT, J.C., LANE, C. M. AND HAMILTON, J. L.: J. Urol. 46: 235, 1941. CAMPBELL, M. F.: Pediatric Urology. New York: Macmillan, vol. 1, 1937. KEIBEL, F. AND MALL, F. P.: Human Embryology. Philadelphia: J.B. Lippincott Co., 1912. LAU, F. T. AND HENLINE, R. B.: J.A.M.A., 96: 587, 1931. MACLEAN, J. T. AND HARDING, E.W.: J. Urol., 54: 381, 1945. MILLER, J.: Brit. J. Urol., 10: 249, 1938. SMITH, E. C. AND ORKIN, L.A.: J. Urol., 53: 11, 1945. SMITH, I.: Brit. J. Surg., 34: 182, 1946. WEssoN, M. B.: Urologic Roentgenology. Lea & Febiger, 1950, 3rd ed. WITHYCOMBE, J. F. R.: Brit. J. Surg., 38: 113, 1950. WooDRUFF, S. R.: J. Urol., 46: 376, 1941.
Vol. 68, No. 1, July 1952 Printed in U.S.A.
TRIPLICATION OF THE URETER AND RENAL PELVIS RICHARD D. GILL
Unusual anomalies of the genito-urinary tract occur fairly frequently compared with the other systems of human anatomy. The average urologic diagnostician manifests much more interest in the unusual anomaly and is much more apt to record his observations. Such was the case when we encountered a patient with three kidney pelves and three ureters on one side and two kidney pelves and two ureters on the opposite side. EMBRYOLOGY
To understand the occurrence of ureteral anomalies one must be somewhat conversant with the fundamentals of the development of the genito-urinary tract. The metanephros frequently referred to as the permanent kidney makes its appearance in the human embryo about the fourth week. The metanephros may be divided into two portions, the secretory and the efferent apparatus. The efferent apparatus actually develops from the primary excretory duct, which by an outgrowth forms a ureter on either side. The ureteric anlage appears, according to Keibel and Mall, between the stages of 4.5 mm. and 5.3 mm. total length. The short ureteral bud grows at first almost altogether dorsally toward the vertebral column and the blood vessels lying in front of it, but later in embryos of 8.5 to 9.5 mm. it forms a curve, which becomes flatter with advancing age and the ureter grows cranially. The end of the bud becomes dilated, forming a cap, the beginning of the renal pelvis. The cap divides into a cephalic and caudal branch, each giving rise to smaller evaginations which are destined to become the calyces and the collecting tubules which eventually unite with the uniniferous tubules, these having been derived from the nephrogenic cord (renal blastema) or the true secretory anlage, the metanephros. The rectum, bladder, urethra, and the urogenital sinus are formed from a triple division of the cloaca. The lower portion of the excretory duct enlarges and becomes continuous with the adjoining portion of the cloaca. The ureteral bud is incorporated into that portion of the cloaca which is to become the bladder. About the fifth or sixth week the cloaca separates into a dorsal rectal and a ventral urogenital segment. Thus the ureter and wolffian duct come to empty into the vesico-urethral anlage, but acquire separate openings, the former emptying above and laterally into the future trigonum vesicae. From the wolffian duct is formed the posterior urethra, vas deferens, epididymis and the seminal vesicle. Particularly pertinent to the understanding of the anomaly under discussion here, is the fact that there may arise two or three ureteral buds from the primary excretory duct or there may be a precocious splitting of a single ureteral bud, ureter fissus. This clefting may extend all the way down to its entrance into the bladder, thus giving rise to the cleft ureter with but a single orifice in the bladder. In instances of two or three or even multiple ureteral buds developing from the Read at annual meeting of Mid-Atlantic Section, American Urological Association, April 12, 1951.
140
141
TRIPLICATION OF URETER
primary excretory ducts the most caudal one usually shows the strongest development and reaches a more cranial level. This accounts for the fact that the lowest ureteral orifice on the trigone in case of multiple orifices connects with the upper most pelvis. This is referred to as the inversion relation of the ureteral orifices and their corresponding pelves. A mishap at this stage of development readily explains the congenital misplacement of some ureteral orifices, that is extravesical orifices or ureteral ectopia. There are those who disagree with this theory and think that multiple ureteral buds do not develop, that clefting occurs in a single ureteral bud and the result obtained depends only on the degree of clefting. Probably one embryologic theory will not explain all cases. From this extremely brief review or l n t e s t i n . : , l f f i a ~ -w.d. •duct
trifid bud '
teral ud (a)
(b)
w.d. ~licate ~bud )
Diagl'ammatic attempt to show development of"\OJ Normal u1·eter \blTl'i fid urete1· \cJT,·iplicate urete1·
Fm. 1 Multi branched Urete:·s - 3 Cases
R
R
R
Niemeyer 1814
Furstner 1897
L
Campbell 1937
Fm. 2
summary of the embryology involved it is not difficult to understand the manner in which triplication of the ureter might possibly occur. See figure 1. CLASSIFICATION AND INCIDENCE
Ureteral anomalies may be classified as I) anomalies of number; 2) anomalies of origin and termination; and 3) anomalies of form, caliber, and structure. As to the first classification, there may be A, agenesis of the ureter, B, duplication of the ureter, C, triplication of the ureter, D, multi-branched ureters (fig. 2). In this discussion we are dealing only with triplication of the ureter. Smith and Orkin in a statistical study of 471 congenital anomalies of the kidneys and ureters occurring in 18,460 consecutive urological admissions found no case of triplicate ureter. Campbell studying the uropathology in 26,480 autopsies
142
RICHARD D. GILL
found no instance of this anomaly. Abeshouse, in a most excellent discussion of ureteral ectopia and review of nearly 300 cases, found many concomitant anomalies of the genito-urinary tract but not one of ureteral triplication. While duplication of the ureter and kidney pelvis is relatively quite frequent, occurring in approximately 0.5 per cent of autopsy material and hundreds of cases being reported clinically, triplication of the ureter is undoubtedly one of the rarest anomalies of the upper urinary tract. Lau and Henline in 1931 gave us one of our earliest discussions on this subject and reported a case of triplication of the ureter and also reviewed the literature. They found 7 cases reported in the world literature from postmortem examinaTypes of trip Iicate urete1·
)
(
a b C d (a)Triple Cb) Double with one bifid Cc)Trifid
Cd) Double with inversion Y bifurcatio11
Tri pie Ureter 4 cases
R
Case I Case 2 LauWoodruffe Henline 1941
1931
Case 3 Chwalla 1935
Case 4 Burt-LaneHamilton 1941
Fm. 3
tions, but there were insufficient data to authentically classify these cases. Miller, in 1938, reported a proven case of triplication of the ureter. Burt, Lane, and Hamilton reported a case in 1941, and incidentally in their case one of the three ureters was ectopic. Woodruff, also, in 1941 reported a case. MacLean and Harding reported a case in 1945. Irvine Smith, in 1946, reviewed the world literature and gave a very excellent report on this subject and added a case of his own. He classified the triplicate ureter into the following types (fig. 3): 1) triple ureter, in which there are three separate and distinct ureters opening into the bladder or elsewhere; 2) double ureter with one bifid, in which two ureters may unite, leaving only two orifices (fig. 4); 3) trifid ureter, in which all three ureters may unite and drain through a single orifice (fig. 5) and 4) double, with inversion Y bifurcation and resulting three orifices.
143
TRIPLICATION OF URETER
Smith, in his exhaustive review of the literature in 1946, was able to findfour cases of triple ureter, two cases of double ureter with one bifid and four cases of trifid ureter and added one of his own making five or a total of eleven cases of ureteral triplication. We have been able to find one additional case, that reported by Withycombe in 1950 which is classified as a trifid ureter. The case herein reported is classified as a trifid ureter also and therefore brings this group to seven reported cases, and all cases of triplication of the ureter to thirteen. There is no doubt that there have · been a fair number of unreported cases. The average text in urology does not Double Ureter with I .Bifid · 2 Cases
L
R
Cose I
Cose 2
"'fr:~y
MIiier 11158
Fm. 4 Tl"ifid Urete1· • 7 Cases-
L
Coa&I
Cose2
Sehmutte FrGnk 19211
11133
Tl'ifid Ureter - Continued
Col& 3
Case4
Cose 5
Case 6
Sehroter
Perrin
Smith
Withycombe
11137
1927
1946
1950
Case 7 Authors 1951
Fm. 5
mention the possibility of this anomaly. However, in Campbell's Pediatric Urology there is a picture of a trifid pelvis in a horseshoe kidney. In Wesson's Urologic Roentgenology there is a photograph, courtesy of A. K. Merchant, of triple renal pelves and ureters. In an article by Braasch and Scholl in 1922, on duplication of the renal pelvis and ureter, there is a photograph of three separate pelves leading from a common ureter. These have apparently been overlooked in the various surveys of the literature. In spite of the undoubted possibility of a number of unofficially reported cases, the fact still remains that triplication of the ureter is extraordinarily rare. SIGNS AND SYMPTOMS
There are no pathognomonic signs or symptoms of this condition; the diagnosis is made by urography, at operation or at autopsy.
144
RICHARD D. GILL CASE REPORT
Mrs. M. Y., a white married woman, 31 years of age, was referred to one of our general surgical colleagues with a working diagnosis of subacute appendicitis. The patient revealed that she had had some moderate attacks of pain in the right lower quadrant for several months. The present attack during the previous 48 hours had been much more severe and had required two hypodermics for relief. There had been no pain in the right kidney region posteriorly and no radiation of the pain to the genitalia or bladder region. There had been no dysuria or hematuria, and no chills or high fever. The patient's past genito-urinary history was entirely negative. There had been no attacks of any febrile condition in childhood that might have been interpreted as pyelitis. She was the mother of three children and had had no genito-urinary complications during pregnancy. On admission to the hospital patient's temperature was 99.6F. Routine blood work and blood chemistry showed no abnormalities except a leukocytosis of 13,000. Physical examination was negative except for the right abdomen, where there was well marked tenderness over McBurney's point, but no muscle spams, and there appeared to be a rather large indefinite mass, moderately tender, extending upward into the right upper quadrant and into the right flank. The mass was soft and extremely hard to outline and appeared to move somewhat with respiration. The catheterized bladder urine was persistently alkaline and loaded with pus and the patient was referred to us for complete urological study. At cystoscopy the bladder showed a chronic inflammatory reaction throughout with much purulent debris over the bladder floor. On the left side of the trigone there were two ureteral orifices. On the right side there was one orifice which was slightly larger than normal, and it was noted that the efflux of urine from the right orifice was more frequent than usual and the orifice seemed to gape open in a rather peculiar manner. Some obstruction was met about three inches above the bladder to the passage of a ureteral catheter on the right side. No obstruction was met to the passage of catheter in either of the two orifices on the left side. Retrograde pyelographic pictures demonstrated that we were dealing with an unusual anomaly, the exact nature of which would require further study, plus an impacted, rather large, ureteral calculus on the right side (fig. 6, A). There were two large atypical renal pelves with two hydro-ureters which apparently united above the impacted stone. There obviously was marked destruction of renal parenchyma and marked decrease in function on the right. Culture of the urine showed B. coli and bacillus alcaligenes. Function on the left side was normal. Shortly after the original urological study, the patient had severe chills and fever with an acute exacerbation of pain in the right flank and right kidney region posteriorly and an increase in the size of the mass in the right abdomen. In fact, she became sicker than she had ever been in her life. Attempt was made to introduce a ureteral catheter up the ureter past the stone for drainage of the acute process. This, however, met with failure. It was believed that the stone should be removed from the right ureter immediately and this was done without further delay. The ureter at the site of the stone was found very much enlarged and thickened. Upon opening the ureter and removing the calculus, there was a great gush of dammed-up purulent urine. A small catheter was introduced up the ureter for
TRIPLICATION OF URETER
145
drainage. The patient made a very good and prompt recovery and further urological studies were then carried out. The ureterostomy tube apparently had passed up the more medial of the two ureters and gradually there was some reduction in the size of the hydro-ureters and hydronephroses. The patient was discharged from the hospital with the ureterostomy tube in place and studies were
Fm. 6. A, first x-ray demonstrating calculus lying low in right ureter at tip of catheter. B, demonstrating two large, atypical pelves on right side with ureters joining in lower third. C, demonstrating two renal pelves on left side with two separate ureters. D, excretory urogram demonstrnting, for first time, a third pelvis on right side above the other two.
repeated intermittently. However, the tube accidentally came out, the wound healed with rapidity and with practically no escape of urine. The patient's condition continued to improve. She was entirely symptom free and refused further operative interference for approximately a year. She did, however, return frequently for observation and further urological study. During the course of
146
RICHARD D. GILL
these studies or approximately three or four months after removal of the ureteral calculus, there appeared in various x-ray films a shadow in the region of the upper pole of the right kidney above the two pelves which had not previously been present. This led us to suspect further additional renal tissue or pelvis which was
Fm. 7. A, flat film with catheter in right ureter approximately a year after original examination. Recurrence of calculi. B, right retrograde pyelogram demonstrating two pelves with joining ureters. Calculi apparently in third pelvis and ureter, but impossible to demonstrate by retrograde pyelography. ~
Fm. 8. X-ray demonstration of operative specimen
proven by excretory urography. A repeated search for an ectopic ureter gave negative results. There was not sufficient concentration of the dye to completely outline the ureter leading from the third pelvis. Repeated attempts to outline a third ureter, by retrograde pyelography, met with no success. After a considerable time, however, three fairly large stones, all apparently from this upper pelvis, developed and they moved up and down the ureter freely and without pain.
TRIPLICATION OF URETER
147
Finally, a little over a year after her original operation, the patient consented to enter the hospital for further observation and operation. She had started having further attacks of rather severe pain in the right flank accompanied by chills and fever, loss of weight and general malaise. There had been no attacks of pain or discomfort on the left side. Repeated urological study revealed the two left pelves to be essentially normal as to function. The function on the right side was remarkably reduced. See Figure 7. From our studies, we believed that we were dealing with three pelves on the right side and with two ureters from the lower two pelves fusing and a third ureter from the upper pelvis which we could not visualize except faintly in its upper portion by excretory urography. Preoperatively we were of the opinion that we were probably dealing with supernumerary kidneys. At operation, however, it was found that the three pelves were contained in one large capsule and that there were three ureters leading from the pelves, the two lateral of which united about half way down, running from this point to the bladder in one sheath. The third ureter ran separately down to the bladder wall where it apparently united with the other intramurally. There was very little renal parenchyma left in the entire kidney. The whole right renal mass was removed together with the ureters with some difficulty. As might well be imagined, the blood supply was quite anomalous and very copious. The pateient made a very excellent and quick recovery and in the matter of a few months she had gained approximately 20 pounds in weight, the urine became free of infection, acid in reaction, and urinary cultures became negative. Study of the pathological specimen revealed that there were three separate and distinct pelves with no communication between them. There were three separate ureters with fusion of the lateral two above the bladder. The third ureter apparently fused in the bladder wall, thereby presenting only one ureteral orifice on the right side. See figure 8. This case then can be classified as triplication of the renal pelvis and ureter with fusion of the three ureters or the so called trifid ureter. CONCLUSION
The incidence and embryology of triplication of the ureter and kidney pelvis has been reviewed and an additional case of this interesting anomaly has been reported. Wheeling Clinic, Wheeling, W. Va. REFERENCES ABESHOUSE, B. S.: Urol. & Cutan. Rev. 47: 447, 1943. BRAASCH, W. F. AND SCHOLL, A. J. JR.: J. Urol. 8: 507-546, 1922. BURT, J.C., LANE, C. M. AND HAMILTON, J. L.: J. Urol. 46: 235, 1941. CAMPBELL, M. F.: Pediatric Urology. New York: Macmillan, vol. 1, 1937. KEIBEL, F. AND MALL, F. P.: Human Embryology. Philadelphia: J.B. Lippincott Co., 1912. LAU, F. T. AND HENLINE, R. B.: J.A.M.A., 96: 587, 1931. MACLEAN, J. T. AND HARDING, E.W.: J. Urol., 54: 381, 1945. MILLER, J.: Brit. J. Urol., 10: 249, 1938. SMITH, E. C. AND ORKIN, L.A.: J. Urol., 53: 11, 1945. SMITH, I.: Brit. J. Surg., 34: 182, 1946. WEssoN, M. B.: Urologic Roentgenology. Lea & Febiger, 1950, 3rd ed. WITHYCOMBE, J. F. R.: Brit. J. Surg., 38: 113, 1950. WooDRUFF, S. R.: J. Urol., 46: 376, 1941.