Vol. 105, April
THE JoURNAL OF UROLOGY
Copyright © 1971 by The Williams & Wilkins Co.
Printed in U.S.A.
RETROCAVAL URETER: 4 CASES THOMAS E. SHOWN*
Retrocaval ureter is a rare congenital anomaly that occurs in patients almost exclusively on the right side. The usual cause is persistence of the fetal posterior cardinal vein, although several other anomalous venous arrangements have been described, such as double vena cava (on the right side) and bilateral vena cava. The incidence of this condition is greater in male subjects than in female subjects (2.8: 1) although there is no clear anatomic or embryologic explanation. Ethnic, racial and familial factors have no bearing on incidence. The low reported incidence in children is a reflection of the gradual development of the associated hydronephrosis. Since 1893, when Hochstetter first described this condition/ approximately 150 cases have been reported. Herein we will report on 4 cases and describe the clinical history, diagnostic radiographic picture and the varying possibilities in management. CASE REPORTS
Case 1. H. L. D., a 20-year-old Negro, was hospitalized on March 5, 1967 after being kicked in the back. An excretory urogram (IVP) performed to evaluate the back pain revealed right hydronephrosis. The patient was referred to the Second General Hospital. Past history and physical examination were unremarkable. A midstream urine specimen contained 5 to 8 white and O to 3 red blood cells per high power field. A right retrograde pyelogram confirmed hydronephrosis and medial deviation of the upper ureter aroused suspicion of a retrocaval ureter. Because of this suspicion a percutaneous femoral inferior venacavogram was done with a ureteral catheter in place (fig. 1, A). On March 28 the right ureter was explored through a paramedian transperitoneal approach. The renal pelvis was divided 1.5 cm. above the Accepted for publication June 13, 1970. * Current address: Suite 770, Forsyth Medical Park, 1900 South Hawthorne Road, Winston-Salem, North Carolina 27103. t Current address: Commanding Officer, 65th Medical Group, APO San Francisco 96301. 1 Hochstetter, F.: Beitriige zur Entwicklungsgeschichte des Venensystems der Amnioten: II. Reptilien (La.certa, Tropidonotus). Morphol. Jahrb. Leipz., 19: 428, 1892-93.
C. A. MOOREt
ureteropelvic junction and the ureter was relocated from behind the vena cava. The retrocaval portion was only slightly redundant and did not appear strictured. A 24F Malecot nephrostomy tube was placed through the lower pole calix and primary reanastomosis with 4-zero chromic interrupted sutures was performed. Postoperative drainage across the anastomosis was delayed, necessitating prolonged nephrostomy diversion. Attempts to clamp the nephrostomy tube for extended periods were unsuccessful until 50 days postoperatively when drainage spontaneously ceased. In comparison to preoperative x-rays, an IVP revealed improvement in function. The tube was removed and the tract closed spontaneously. The patient had no further postoperative problems and the urine remained free of infection. An IVP on December 28 revealed almost complete resolution of hydronephrosis (fig. 1, B). Case 2. R. W., a 28-year-old white man, consulted his dispensary physician after 4 days of dull, aching pain in the right flank with radiation into the groin. Microhematuria was noted, a presumptive diagnosis of ureteral calculus was made and the patient was taken to Second General Hospital. History and physical examination were unremarkable. The midstream urine specimen contained 8 to 10 red and O to 3 white blood cells per high power field. An IVP demonstrated dilatation of the pelvis and proximal ureter with non-visualization of the distal ureter (fig. 2, A). Subsequent pyelogram and venacavogram confirmed the suspected diagnosis (fig. 2, B and C). On September 26, 1967 the ureter was explored through an anterior, subcostal, extraperitoneal approach. Diagnosis was confirmed and the retrocaval portion of the ureter was calibrated. Since an 8F bougie a boule would not pass with ease, segmental resection was performed. An oblique anastomosis just proximal to the ureteropelvic junction was performed with 4-zero chromic interrupted sutures after a slash pyelostomy had been made. Convalescence was complicated by a superficial wound infection and persistent urinary drainage from the drain site for 3 weeks. Followup 497
SHOWN AND :VIOORE
Fm. 1. Case 1, H. L. D. A, preoperative combination venacavogram and right retrograde pyelogram. B, IVP performed 9 months postoperatively.
Fm. 2. Case 2, R. W. A, preoperative !VP-delayed film. B, preoperative right retrograde pyelogram. C, preoperative venacavogram in combination with retrograde pyelogram. IVPs through July 1968, when the patient was lost ..to followup, showed good function with moderate improvement in the hydronephrosis (fig. 3). He had remained free of pain during this interval. Case 3. E. B., a 35-year-old Negro, was found to have hypertension on routine periodic physical examination in September 1967. History was non-contributory and physical examination was unremarkable except for slight obesity and persistent diastolic hypertension, ranging from 90 to
110 mm. Hg. A renogram showed delayed clearance from the right kidney and an IVP revealed right hydronephrosis and proximal hydro-ureter with medial deviation of the upper ureteral segment. Infusion IVP in conjunction with an inferior venacavogram verified the presence of a retrocaval ureter (fig. 4, A). On March 7, 1968 at Letterman General Hospital the patient underwent excision of the redundant posterior caval ureter and dismembered Foley pyeloplasty through a flank incision. The
anastomosis was splinted with an SF red rubber catheter and a 28F nephrostomy tube was used to divert the urine. The ureteral splint was removed 28 days postoperatively and the nephrostomy tube was removed 3 days later. Interval followup IVPs within the ensuing 18 months showed gradual but incomplete resolution of hydronephrosis (fig. 4, B). Blood pressure returned to normal early in the postoperative period in this case and has remained so (18 months). To our knowledge this has been reported previously only once. 2 Case 4. L. J. H., a 19-year-old white man, had complaints of intermittent right flank pain for the previous 7 years. IVP revealed right hydronephrosis and the patient was transferred to Letterman General Hospital for evaluation. There was no history of urinary tract infection or hematuria. Examination was unremarkable. A retrograde pyelogram revealed the characteristic picture of retrocaval ureter (fig. 5, A). On September 30, 1967 the patient underwent surgical exploration through a flank incision. The retrocaval ureter was confirmed and a 5 cm. segment of redundant ureter containing the postcaval segment was resected. The ureteral ends were spatulated and approximated with interrupted chromic sutures over an SF rubber catheter. The splinting catheter exited from a lower pole calix and contained holes in the intrapelvic portion to promote drainage. In addition a slash pyelostomy was performed. A Penrose drain was left in the area of the anastomosis and exited through the incision. Convalescence was uncomplicated and the splint was removed without incident 21 days postoperatively. Interval IVPs have shown continued resolution of hydronephrosis over an 18-month JJeriod (fig. 5, B). DISCUSSION
Clinical history. There is no typical story for the patient with a retrocaval ureter. Usually they seek medical attention because of pain in the right flank. Pain is usually episodic, dull and aching but may resemble renal colic. Symptoms may actually be due to stones, the incidence of which is increased secondary to the stasis of hydronephrosis. Hematuria in some form is frequently present. Many cases have been re2 Heslin, J. E. and Mamonas, C.: Retrocaval ureter: report of four cases and review of Ii terature. J. Urol., 65: 212, 1951.
Fm. 3. Case 2, R W. IVP performed 10 months postoperatively.
ported, however, in which right hydronephrosis was incidentally found on an IVP performed to evaluate other complaints (for example case 3). Radiographic picture. Retrocaval ureter should be considered whenever right hydronephrosis due to obstruction of the upper third of the ureter is encountered. The reversed-J appearance of the dilated proximal pyeloureteral segment is characteristic. A ureteropyelogram will demonstrate the typical S-shaped or sickle-shaped curve of the upper ureter with deviation to or beyond the midline. It is often not possible to pass a ureteral catheter beyond the postcaval segment. )t oblique film will demonstrate the close of the right upper ureter to the vertebral column. Inferior venacavography in conjunction with ureteral catheterization confirms the circumcaval route of the ureter. This procedure is fairly to perform but adds relatively little to the information at hand if the IVP and pyelogram are of good quality. Early postoperative reversal of should not be expected. In all of our cases it was gradual and was never complete.
SHOWN AND MOORE
Fm. 4. Case 3, E. B. A, combination venacavogram and infusion pyelogram. Catheter is in aorta. B, IVP performed 18 months postoperatively.
Fm. 5. Case 4, L. J. H. A, preoperative retrograde pyelogram. B, IVP performed 12 months postoperatively.
Treatment. The mere presence of a retrocaval ureter is no indication for surgical correction. The indications for operation should be those considered in evaluating any hydronephrosis and each case must be considered on its own merits. Our 4 cases demonstrate that there is no single operation that can or should be done exclusively. The final decision as to what particular procedure
to perform must be based on the operative findings. The transabdominal surgical approach seems preferable because the surgeon has easy access to the vital structures should any unforeseen problems arise. 1) Observation should be maintained in a patient who is without symptoms and whose evaluation reveals only minimal calicectasis and/or
cortical atrophy and who will be available for interval medical followup. 2) N ephrectomy is indicated in the presence of far-advanced hydronephrosis with cortical atrophy in patients who have adequate contralateral renal function. N ephrectomy is seldom used as the initial operation because of the usual slow development of hydronephrosis with the appearance of symptoms before the kidney is totally destroyed. 3) Division of the pelvis with transposition and reanastomosis was initially described by Harrill and has been the most popular form of treatment. 3 Considine points out that Harrill's procedure is more appropriately called caudal division of the dilated segment since the original ureteropelvic junction is often obliterated by dilatation of the proximal ureter. 4 He also stresses the importance of making the anastomosis as far distal on the dilated segment as feasible, to insure adequate vascular supply, since the pelvis and proximal ureter receive their blood supply from the renal artery and the aorta and it is plentiful compared to that of the middle third of the ureter. Thus, the farther down the dilated segment the anastomosis is made, the smaller the relatively less vascular segment. If the postcaval segment is preserved then this area should be calibrated with the bougie a boule to rule out intrinsic stricture; an SF bougie should pass without resistance. If there is any question, then it should be excised. This operation is essentially a dismembered pyeloplasty and the use of splints and urinary diversion is left to the preference of the surgeon. 4) Ureteroureterostomy is indicated if segmental resection must be done in the absence of a dilated proximal segment. This situation occurs in cases in which the adhesions are so severe that lysis from the vena cava is impossible, when there is demonstrable stricturing of the postcaval segment or when there is excessive redundancy of the ureter. The operation has the obvious disadvantage of a relatively small anastomosis and we believe urinary splinting and diversion should be used. 5) Transection, transposition and reimplantation of the ureter into the bladder should not be 3 Harrill, H. C.: Retrocaval ureter. Report of a case with operative correction of the defect. J. Urol., 44: 450, 1940. 4 Considine, J.: Retrocaval ureter. A review of the literature with a report on two new cases followed for fifteen years and two years respectively. Brit. J. Urol., 38: 412, 1966.
used. Mobilization of the lower two-thirds of the ureter is likely to result in ischemia of the lower ureter with failure of the anastomosis. In 1946 Lowsley reported on a case treated in this manner in an attempt to preserve the ureteral nerve supply. 5 The ureter strictured 3 cm. above the ureterovesical junction and the patient's complicated convalescence terminated with a cutaneous ureterostomy. 6) Ligation and section of the vena cava without reanastomosis were first described by Cathro for treatment of retrocaval ureter. 6 Some early postoperative complications were noted but the status of the patient was described as excellent 6 months postoperatively. Goodwin and associates reported on a patient with some evidence of disease of the contralateral kidney whose vena cava was sectioned and reanastomosed satisfactorily.7 They suggested that this might be the most conservative approach to the problem in a patient with contralateral renal disease if a competent vascular surgeon was present. In view of 1) the few but rather serious complications that can result from this operation; 2) the fact that it can not be used if any intrinsic ureteral abnormality is present, for example stricture and/or stone, and 3) the relatively high rate of success with other forms of treatment; it appears that its indications are somewhat nebulous at the present. We believe the urologist is the physician who is responsible for the care of the patient and he should use the materials and methods of his specialty. In all fairness, however, it must be stated that there have not been enough cases treated in this manner to judge it with finality. SUMMARY
Four patients with retrocaval ureter have been described. One patient (case 3) was hypertensive and became normotensive postoperatively. The characteristic radiographic appearance has been illustrated. The various forms of treatment are outlined and briefly discussed. 5 Lowsley, 0. S.: Postcaval ureter, with description of new operation for its correction. Surg., Gynec. & Obst., 82: 549, 1946. 6 Cathro, A. J. McG.: Section of theinferiorvena cava for retrocaval ureter: a new method of treatment. J. Urol., 67: 464, 1952. 7 Goodwin, W. E., Burke, D. E. and Muller, W. H.: Retrocaval ureter. Surg., Gynec. & Obst.,
104: 337, 1957.