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Recognition and Surgical Correction of Retrocaval Ureter: A Case Report
RECOGNITION AND SURGICAL CORRECTION OF RETROCAVAL URETER: A CASE REPORT C.D. CREEVY From the Urological Division of the Department of Surgery in the Medical School of the University of Minnesota, Minneapolis
While several examples of retrocaval ureter have been reported recently, the small number of cases in the literature, together with the fact that very few seem to have been recognized clinically or to have had successful corrective operations, appears to justify another report of a single case treated successfully. The literature will not be reviewed except cursorily because this has been well done by recent writers. The etiology has been discussed thoroughly by Randall and Campbell, by Pick and Anson, and by others, and it suffices here to say that the condition is an anomaly of the venous system in which the vena cava is formed from veins which lie anterior to the ureter in the embryo, and which normally disappear early in the course of development. The ureter thus passes behind the vena cava a short distance below the renal pelvis, and the resultant aberration in its course produces a characteristic and readily identifiable pyeloureterogram. This fact was brought out by Randall and Campbell in 1935, but seems to have escaped the attention of a number of writers who have since reported cases, at least until after operation. In many instances the anomaly has apparently caused no symptoms, since over half the reported cases were found at the dissecting table or at autopsy. Often, however, the ureter is squeezed between the vena cava and the underlying tissues, so that hydronephrosis develops. Thus when symptoms are present, they are those of obstruction and infection, and one must rely upon urography for the diagnosis. Lowsley found 33 cases in the literature and added 1 of his own; since then Goyanna, Cook and Counseller, Greene and Kearns, and Nourse and Moody have each reported one, making a total of 37. Of these, 22 were discovered after death, 12 ·were encountered at operation, and 3 were recognized by pyelography (Harrill, Greene and Kearns, Goyanna, et al.). Most of the recorded cases were in males. Failure to identify retrocaval ureter by pyelography must be due to unfamiliarity with the existence of the condition, since the course of the ureter (fig. 1, A) is so typical that, once seen, it is not easily forgotten. While Harrill has advocated stereoscopic, and Nourse and Moody oblique pyeloureterograms, these are not necessary for accurate diagnosis. Harrill found that only 3 corrective operations had been done up to 1940. They consisted of severing the ureter, withdrawing it from behind the vena cava, and uniting it in a normal location. Greene and Kearns, Nourse and Moody, and Goyanna et al. have since reported similar operations. Harrill has lessened the danger of postoperative stricture at the site of anastomosis by dividing the pelvis transversely a short distance above the ureteropelvic junction instead of 26
RETROCAVAL URETER: A CASE REPORT
27
cutting the ureter. The anastomosis is thus made between segments of the dilated pelvis, the much greater circumference of which should practically eliminate postoperative stenosis.
Fm. 1
Lowsley devis~d an operation of his own, so that 8 corrective operations have been reported, but the patient of Nourse and Moody later required a nephrectomy for stone, and Lowsley's operation led to an impermeable stricture of the
28
C. D. CREEVY
terminal ureter with a fistula above it. This is not surprising, since he divided the ureter just above the bladder, dissected it free clear to the renal pelvis, pulled it out from behind the vena cava, and implanted it into the bladder. The stricture and fistula undoubtedly resulted from depriving the lower ureter of its chief blood supply, which comes from the inferior vesical artery, and at the same time dividing the entire extrinsic blood supply of the rest of the ureter. Thus there are in the literature 6 instances of successful correction of retrocaval ureter (Kimbrough, May, Uebelhoer, Harrill, Greene and Kearns, and Goyanna et al. The longest follow-up (1 year) was reported by May. CASE REPORT
J.B., a girl of 12, University Hospital No. 743496, was well until December 1943, when fever, nausea, vomiting, dysuria, and a dull bilateral sacral backache developed. She was kept in bed for 2 weeks, at which time a diagnosis of rheumatic fever was made by her family physician who then referred her to the Children's Cardiac Clinic at Lymanhurst Hospital. Here a urogram, made because of pyuria, showed right hydronephrosis without visualization of the ureter. She was then sent by Dr. M. J. Shapiro to the University Hospital on May 1, 1944. Physical examination gave essentially normal findings. The catheterized urir:e contained many pus cells and E. coli. Cystoscopy disclosed a normal lower urinary tract. Indigo carmine appeared in normal time and in normal concentration from the left ureteral orifice, but was somewhat delayed and reduced in concentration on the right. A catheterized specimen from the right contained many pus cells and E.coli; that from the left was clear and sterile. An x-ray of the urinary tract with a shadow casting catheter (fig. 1, A) showed the deviation of the catheter on the right described by Randall and Campbell as typical of retrocaval ureter. The catheter on the left followed a normal course. The left pyeloureterogram was normal; the right presented the typical deformity of retrocaval ureter (fig. 1, B). The dilated lower pelvis and upper ureter formed a hook with its convexity downward and its pointed tip overlying the lateral margin of the spine. The ureter then arched medially and downward in front of the vertebrae to the level of the sacrum, after which its contour and location were normal. The dilatation terminated abruptly at the lateral margin of the spine where the ureter passed under the vena cava. A diagnosis of right retrocaval ureter with hydronephrosis and secondary infection having been made, the ureter was exposed on May 22, 1944, through a right midrectus transperitoneal incision made under spinal anesthesia. It was followed upward to its point of disappearance beneath the medial margin of the vena cava, where it was doubly ligated and divided. The distal portion was pushed laterally between the vena cava and posterior parietal peritoneum, which was then sutured. The abdominal incision was closed, the patient turned on her left side, and the right renal pelvis was exposed through a lumbar incision. The pelvis was freed together with the upper, ligated ureter which was pulled out from behind the vena cava. The lower end was easily located, and was approximated
RETRO CAVAL URETER: A CASE REPORT
29
loosely to the upper end with fine chromic catgut over a No. 14 Robinson catheter which was brought out through a stab in the renal pelvis. A pelviostomy was made with another Robinson catheter, and both were brought out through the wound. Recovery was uneventful. The catheter was removed from the ureter in 2 weeks. Two days later a pyelogram made through the pyelostomy tube showed the anastomosis swollen but free from leakage and patent, so the tube was removed next day. She was discharged from the hospital on June 12, 1944. Urinary antiseptics were given, and the site of the anastomosis was dilated frequently during the next 3 months. On July 24, 1944, the urine was clear and sterile, and has remained so. She has had no complaints since leaving the hospital. A repeat x-ray and pyelogram were made on April 17, 1947 (figs. 1, C and D). The calyces and pelvis were slightly smaller than before operation, and the course of the ureter was more nearly normal. There was a barely perceptible constriction at the site of suture of the ureter, although an air bubble unfortunately lodged just above this area. COMMENT
The operation which was done was probably needlessly elaborate in that two separate abdominal incisions were used, but its outcome thus far has been entirely successful. This tends t'o nullify Lowsley's argument that division of the ureter is harmful because of injury to its innervation, especially since it has been demonstrated experimentally that the ureter can function successfully as long as its ganglia are intact. Support to this view is given by the success reported by Quinby ,vith Kiister's operation for stricture at the ureteropelvic junction (division of the ureter and implantation into the pelvis). In the unlikely event that another patient with retrocaval ureter comes under the writer's care, Harrill's operation will be done, thus eliminating the need to dilate the ureter postoperatively. In the case just described, success was favored by the age of the patient, the relatively normal amount of renal substance remaining, by the preoperative recognition of the condition so that the operation could be planned in advance, and by the eradication of the renal infection postoperatively. The danger of the occurrence of stricture at the site cf union of the divided ureter is not yet past, and an occasional dilatation is still desirable. SUMMARY AND CONCLUSIONS
A case of right retrocaval ureter in a 12 year old girl is reported. This is apparently the youngest patient subjected to a successful corrective operation. It was recognized preoperatively by the characteristic course of the ureter. It was treated successfully by dividing the ureter medial to the vena cava, pulling the stump out from behind it, and reuniting the ureter in a normal location. The patient is well, the preoperative pyelonephritis having disappeared, 3 years after operation.
30
C. D. CREEVY
This brings the total of reported cases to come to the writer's attention thus far to 38; it is apparently the fourth case to be recognized by pyelography and the ninth to be treated by a conservative operation. It is probably the seventh in which such an operation was successful.
University Hospitals, Minneapolis, Minn. REFERENCES GREENE, L. F. AND KEARNS, W. M.: Circumcaval ureter. J. Urol., 55: 52-59, 1946. GoYANNA, R., Coox, E. N. AND CouNSELLER, V. S.: Circumcaval ureter. Proc. Staff Mayo Clinic, 21: 356-360, 1946. HARRILL, H. C.: Retrocaval ureter. J. Urol., 44: 450-457, 1940. KIMBROUGH, J.C.: Surgical treatment of hydronephrosis. J. Urol., 33: 97-100, 1935. LowsLEY, 0. S.: Postcaval ureter, with description of a new operation for its correction. Surg. Gynec. & Obst., 82: 549-556, 1946. MAY, F.: Versti.ipfungsniere bei dorsalen Ureterverlauf. Ztschr. f. Urol., 32: 316-320, 1938. NouRSE, M. H. AND MooDY, H. C.: Postcaval ureter. J. Urol., 56: 525-529, 1946. PICK, J. W. AND ANSON, B. J.: Retrocaval ureter: Report of a case. J. Urol., 43: 672-695, 1940. QUINBY, W. C. : Factors influencing the operative procedure in hydronephrosis. J. A. M.A., 93:1709-1710,1929. RANDALL, A. AND CAMPBELL, E.W.: Anomalous relationship of right ureter to vena cava. J. Urol., 34: 565-583, 1935. UEBELHOER, R.: Hydronephrose bei Abnormalitat der unteren Hohlvene. Ztschr. f. Urol., 30: 769-772, 1936.
While several examples of retrocaval ureter have been reported recently, the small number of cases in the literature, together with the fact that very few seem to have been recognized clinically or to have had successful corrective operations, appears to justify another report of a single case treated successfully. The literature will not be reviewed except cursorily because this has been well done by recent writers. The etiology has been discussed thoroughly by Randall and Campbell, by Pick and Anson, and by others, and it suffices here to say that the condition is an anomaly of the venous system in which the vena cava is formed from veins which lie anterior to the ureter in the embryo, and which normally disappear early in the course of development. The ureter thus passes behind the vena cava a short distance below the renal pelvis, and the resultant aberration in its course produces a characteristic and readily identifiable pyeloureterogram. This fact was brought out by Randall and Campbell in 1935, but seems to have escaped the attention of a number of writers who have since reported cases, at least until after operation. In many instances the anomaly has apparently caused no symptoms, since over half the reported cases were found at the dissecting table or at autopsy. Often, however, the ureter is squeezed between the vena cava and the underlying tissues, so that hydronephrosis develops. Thus when symptoms are present, they are those of obstruction and infection, and one must rely upon urography for the diagnosis. Lowsley found 33 cases in the literature and added 1 of his own; since then Goyanna, Cook and Counseller, Greene and Kearns, and Nourse and Moody have each reported one, making a total of 37. Of these, 22 were discovered after death, 12 ·were encountered at operation, and 3 were recognized by pyelography (Harrill, Greene and Kearns, Goyanna, et al.). Most of the recorded cases were in males. Failure to identify retrocaval ureter by pyelography must be due to unfamiliarity with the existence of the condition, since the course of the ureter (fig. 1, A) is so typical that, once seen, it is not easily forgotten. While Harrill has advocated stereoscopic, and Nourse and Moody oblique pyeloureterograms, these are not necessary for accurate diagnosis. Harrill found that only 3 corrective operations had been done up to 1940. They consisted of severing the ureter, withdrawing it from behind the vena cava, and uniting it in a normal location. Greene and Kearns, Nourse and Moody, and Goyanna et al. have since reported similar operations. Harrill has lessened the danger of postoperative stricture at the site of anastomosis by dividing the pelvis transversely a short distance above the ureteropelvic junction instead of 26
RETROCAVAL URETER: A CASE REPORT
27
cutting the ureter. The anastomosis is thus made between segments of the dilated pelvis, the much greater circumference of which should practically eliminate postoperative stenosis.
Fm. 1
Lowsley devis~d an operation of his own, so that 8 corrective operations have been reported, but the patient of Nourse and Moody later required a nephrectomy for stone, and Lowsley's operation led to an impermeable stricture of the
28
C. D. CREEVY
terminal ureter with a fistula above it. This is not surprising, since he divided the ureter just above the bladder, dissected it free clear to the renal pelvis, pulled it out from behind the vena cava, and implanted it into the bladder. The stricture and fistula undoubtedly resulted from depriving the lower ureter of its chief blood supply, which comes from the inferior vesical artery, and at the same time dividing the entire extrinsic blood supply of the rest of the ureter. Thus there are in the literature 6 instances of successful correction of retrocaval ureter (Kimbrough, May, Uebelhoer, Harrill, Greene and Kearns, and Goyanna et al. The longest follow-up (1 year) was reported by May. CASE REPORT
J.B., a girl of 12, University Hospital No. 743496, was well until December 1943, when fever, nausea, vomiting, dysuria, and a dull bilateral sacral backache developed. She was kept in bed for 2 weeks, at which time a diagnosis of rheumatic fever was made by her family physician who then referred her to the Children's Cardiac Clinic at Lymanhurst Hospital. Here a urogram, made because of pyuria, showed right hydronephrosis without visualization of the ureter. She was then sent by Dr. M. J. Shapiro to the University Hospital on May 1, 1944. Physical examination gave essentially normal findings. The catheterized urir:e contained many pus cells and E. coli. Cystoscopy disclosed a normal lower urinary tract. Indigo carmine appeared in normal time and in normal concentration from the left ureteral orifice, but was somewhat delayed and reduced in concentration on the right. A catheterized specimen from the right contained many pus cells and E.coli; that from the left was clear and sterile. An x-ray of the urinary tract with a shadow casting catheter (fig. 1, A) showed the deviation of the catheter on the right described by Randall and Campbell as typical of retrocaval ureter. The catheter on the left followed a normal course. The left pyeloureterogram was normal; the right presented the typical deformity of retrocaval ureter (fig. 1, B). The dilated lower pelvis and upper ureter formed a hook with its convexity downward and its pointed tip overlying the lateral margin of the spine. The ureter then arched medially and downward in front of the vertebrae to the level of the sacrum, after which its contour and location were normal. The dilatation terminated abruptly at the lateral margin of the spine where the ureter passed under the vena cava. A diagnosis of right retrocaval ureter with hydronephrosis and secondary infection having been made, the ureter was exposed on May 22, 1944, through a right midrectus transperitoneal incision made under spinal anesthesia. It was followed upward to its point of disappearance beneath the medial margin of the vena cava, where it was doubly ligated and divided. The distal portion was pushed laterally between the vena cava and posterior parietal peritoneum, which was then sutured. The abdominal incision was closed, the patient turned on her left side, and the right renal pelvis was exposed through a lumbar incision. The pelvis was freed together with the upper, ligated ureter which was pulled out from behind the vena cava. The lower end was easily located, and was approximated
RETRO CAVAL URETER: A CASE REPORT
29
loosely to the upper end with fine chromic catgut over a No. 14 Robinson catheter which was brought out through a stab in the renal pelvis. A pelviostomy was made with another Robinson catheter, and both were brought out through the wound. Recovery was uneventful. The catheter was removed from the ureter in 2 weeks. Two days later a pyelogram made through the pyelostomy tube showed the anastomosis swollen but free from leakage and patent, so the tube was removed next day. She was discharged from the hospital on June 12, 1944. Urinary antiseptics were given, and the site of the anastomosis was dilated frequently during the next 3 months. On July 24, 1944, the urine was clear and sterile, and has remained so. She has had no complaints since leaving the hospital. A repeat x-ray and pyelogram were made on April 17, 1947 (figs. 1, C and D). The calyces and pelvis were slightly smaller than before operation, and the course of the ureter was more nearly normal. There was a barely perceptible constriction at the site of suture of the ureter, although an air bubble unfortunately lodged just above this area. COMMENT
The operation which was done was probably needlessly elaborate in that two separate abdominal incisions were used, but its outcome thus far has been entirely successful. This tends t'o nullify Lowsley's argument that division of the ureter is harmful because of injury to its innervation, especially since it has been demonstrated experimentally that the ureter can function successfully as long as its ganglia are intact. Support to this view is given by the success reported by Quinby ,vith Kiister's operation for stricture at the ureteropelvic junction (division of the ureter and implantation into the pelvis). In the unlikely event that another patient with retrocaval ureter comes under the writer's care, Harrill's operation will be done, thus eliminating the need to dilate the ureter postoperatively. In the case just described, success was favored by the age of the patient, the relatively normal amount of renal substance remaining, by the preoperative recognition of the condition so that the operation could be planned in advance, and by the eradication of the renal infection postoperatively. The danger of the occurrence of stricture at the site cf union of the divided ureter is not yet past, and an occasional dilatation is still desirable. SUMMARY AND CONCLUSIONS
A case of right retrocaval ureter in a 12 year old girl is reported. This is apparently the youngest patient subjected to a successful corrective operation. It was recognized preoperatively by the characteristic course of the ureter. It was treated successfully by dividing the ureter medial to the vena cava, pulling the stump out from behind it, and reuniting the ureter in a normal location. The patient is well, the preoperative pyelonephritis having disappeared, 3 years after operation.
30
C. D. CREEVY
This brings the total of reported cases to come to the writer's attention thus far to 38; it is apparently the fourth case to be recognized by pyelography and the ninth to be treated by a conservative operation. It is probably the seventh in which such an operation was successful.
University Hospitals, Minneapolis, Minn. REFERENCES GREENE, L. F. AND KEARNS, W. M.: Circumcaval ureter. J. Urol., 55: 52-59, 1946. GoYANNA, R., Coox, E. N. AND CouNSELLER, V. S.: Circumcaval ureter. Proc. Staff Mayo Clinic, 21: 356-360, 1946. HARRILL, H. C.: Retrocaval ureter. J. Urol., 44: 450-457, 1940. KIMBROUGH, J.C.: Surgical treatment of hydronephrosis. J. Urol., 33: 97-100, 1935. LowsLEY, 0. S.: Postcaval ureter, with description of a new operation for its correction. Surg. Gynec. & Obst., 82: 549-556, 1946. MAY, F.: Versti.ipfungsniere bei dorsalen Ureterverlauf. Ztschr. f. Urol., 32: 316-320, 1938. NouRSE, M. H. AND MooDY, H. C.: Postcaval ureter. J. Urol., 56: 525-529, 1946. PICK, J. W. AND ANSON, B. J.: Retrocaval ureter: Report of a case. J. Urol., 43: 672-695, 1940. QUINBY, W. C. : Factors influencing the operative procedure in hydronephrosis. J. A. M.A., 93:1709-1710,1929. RANDALL, A. AND CAMPBELL, E.W.: Anomalous relationship of right ureter to vena cava. J. Urol., 34: 565-583, 1935. UEBELHOER, R.: Hydronephrose bei Abnormalitat der unteren Hohlvene. Ztschr. f. Urol., 30: 769-772, 1936.