THE JOURNAL OF UROLOGY
Vol. 71, No. 2, February 1954 Printed in U.S.A.
RETROCAVAL (CIRCUMCAVAL) URETER ASSOCIATED WITH SOLITARY KIDNEY VICTOR C. LAUGHLIN From the Department of Urology, Huron Road Hospital, Cleveland 12, Ohio
The peculiar position which the ureter assumes in this condition gives origin to a variety of terms to describe it, viz., retrocaval ureter, postcaval ureter, circumcaval ureter, deflected ureter and pre-ureteric vena cava. More properly, the deformity is classified as an abnormality of the vascular system, a point which is substantiated by embryological study. The ureter, instead of passing lateral to the vena cava, passes behind it, partially encircles it for a short distance, and then pursues its normal course ventral to the vena cava, downward into the bony pelvis and thence to the bladder. In 1893, Hochstetter first described retrocaval ureter. Mullen and Engel state that 55 cases have been reported. Twenty-one were discovered in dissecting room cadavers or at autopsies, thirty-four at surgery, and one was diagnosed by x-ray but not operated upon. These authors add one of their own. The vast majority of cases have occurred in males; a comparative few have been reported in females. In every instance except one, the acardiac monster of Gladstone, the lesion has occurred on the right side. In Gladstone's case, the lesion was bilateral. In no case reported to date has the lesion been found associated with a solitary kidney. The case we are reporting suggests this type of deformity and brings the total number of reported cases to fifty-seven. There are a number of unreported cases. The apparent rarity of this condition must be based upon unfamiliarity with its existence. The pyelographic course of the ureter is so typical that once seen, it is not easily forgotten. EMBRYOLOGY
Simply stated, the embryological explanation of this condition lies in the development of the venous system, principally the vena cava, rather than the abnormal development of the ureter. In the normal embryo, the permanent kidneys, having formed in the pelvis, ascend cranialward. After passing the bifurcation of the aorta, they encounter on either side the trunk of the postcardinal veins. The kidneys pass medial to these veins and then shift laterally behind them so that the ureter virtually becomes wound around the posterior cardinal veins. Because of the rich anastomosis of veins in the area, the kidney and ureter pass through three complex venous rings during their ascent. Normally, certain of these veins function only temporarily, then disappear. Others persist to adult life. Thus, in man, if the ureter lies in front or lateral to the vena cava, it is the supracardinal vein which has persisted; whereas, if the ureter lies behind the inferior vena cava, the posterior cardinal vein has persisted. Should both the Accepted for publication February 3, 1953. 195
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supracardinal vein and posterior cardinal vein persist, the cava will be double and the ureter will pass between the two divisions. Therefore, failure of atrophy of the posterior cardinal system of veins is the accepted embryological explanation of this anomaly. CLINICAL FEATURES
Unfortunately, there are no symptoms pathognomonic of postcaval ureter. Usually the symptoms are those of obstruction, sometimes complicated by the symptoms of infection or stone. All of the surgical cases have had hydronephrosis. This was the reason for operation. Life is not incompatible with this condition. It is probable that, if sufficient time elapses, hydronephrosis from ureteral compression would occur in every instance. The mechanism may be compression alone or by kinking or stricture, due to the anomalous course which the ureter
assumes. Symptomatology has varied from no symptoms at all to severe renal colic with sharp pain radiating along the ureter. Frequency following each attack may occur. Nocturia is often seen. Hematuria, gross or microscopic, has been a fairly constant feature. Pyuria has been frequent with secondary infection or stone. Age is a factor. It has not been reported clinically below the age of twelve, principally because the time element for the development of hydronephrosis is important. Hydronephrosis is the rule, therefore, in older patients. Frequency may intensify the symptoms and hasten the obstructive pathological process. This happened in our case. Hypertension reported has apparently been a coincidental finding, associated with other pathological change, such as nephrolithiasis, etc. Hematuria and loin pain in the cases reported have existed for variable periods of a few weeks to seven years.
Postcaval ureter should be considered a possibility in all cases of right hydronephrosis. Its x-ray features are so distinctive as to be almost pathognomonic; therefore, much diagnostic reliance must be placed upon x-ray studies for the proper preoperative diagnosis and subsequent corrective surgery. Surgeons employing the lumbar renal incision may not observe the entire course of the ureter and may miss the obstructive factor at the vena cava. The distinctive roentgenographic features are as follows: Anterior ureteropyelography reveals hydronephrosis with a very long proximal segment of the ureter involved (fig. 1, A). The ureter is dislocated medially to or beyond the midline, beginning with the third, fourth, or fifth lumbar vertebrae. The resultant redundancy from obstruction and hydronephrosis produces a characteristic hook and a distinct sickle-shape or S-curve is imparted to the ureter. After the ureter has passed around the cava, it comes downward into the pelvis with a normal course and caliber. Its medial portion is not dilated. In oblique and lateral views (fig. 1, B) the retrocaval ureter hugs the spinal column instead of falling away from it. It becomes anterior to the vertebral bodies at about the third lumbar vertebra. This third dimension view is an added measure in the diagnosis and almost pathognomonic in a high percentage of cases. SURGERY
Surgical therapy has consisted of nephrectomy in far advanced cases and attempts at renal conservation in the cases with a partially damaged kidney. In order to avoid postoperative stenosis of a re-anastomosed ureter, better surgical results have followed the division of the hydronephrotic segment above the ureteropelvic outlet, carefully withdrawing the detached segment from behind the vena cava, and re-anastomosing the pelvis. Thus, the ureter is brought anterior to the vena cava. Division of the pelvis at a point above the insertion of the ureter provides a tube of greater diameter, thus lessening the possibility of postoperative stricture. Obviously, in some instances, the surgeon may be forced to resect a constricted portion of the ureter. Ten instances of successful surgical repair have been reported. Certain dangers and difficulties and to be considered in the repair. Removal of adhesions at the uretero-vena caval attachment may strip the local ureteral blood supply and interfere with proper healing of the anastomosis. The vena cava may be torn where adhesions to it and the ureter are dense. This was formerly considered a serious complication. Now, elective vena caval interruption is not an unusual procedure. CASE REPORT
Mrs. L.B., aged 36, white, case No. 51-8517, was admitted to the obstetrical department of Huron Road Hospital in East Cleveland, Ohio, by Dr. Samuel Vinci and Dr. Jess J. Woodworth, on October 11, 1951. Chief complaint: "Pain in right flank, 18 hours duration, and no urine in bladder since onset of the
VICTOR C. LAUGHLIN
pain." The patient was 7 months into her fifth pregnancy, which had been uneventful except for ankle edema. Approximately 18 hours before admission, the patient had a sudden onset of severe pain in the right flank and loin associated with nausea. The pain tended to remain steady and occasionally radiated to the genitalia. No urine had passed since the onset of the pain. Blood pressure, 120/80. Temperature, 36.2. Pulse, 96. The patient was apprehensive and abnormal findings were limited to the abdomen which was enlarged, consistent with a seven-month pregnancy. There was marked tenderness to punch percussion over the right costovertebral angle with questionable muscle guarding below the right costal margin. Laboratory findings: The blood chemistry was as follows: Nonprotein nitrogen 47.5 mg/100 cc. Uric acid 5.6 mg/100 cc. Blood urea nitrogen 26.0 mg/100 cc. Hemoglobin 68.4 per cent; red blood cells 3,710,000; white blood cells 15,950; neutrophils 88; eosinophils 1; lymphocytes 6; monocytes 5. Slight hypochromia; slight anisocytosis; and slight left shift. On the obstetrical service, after finding no urine at two catheterizations, the patient was given sedation, antispasmotics and intravenous fluids. The urological service was called the night of admission. The patient was cystoscoped, retrograde pyelograms made, massive pyelectasis discovered, the course of the ureter delineated, and a diagnosis of retrocaval ureter made. A very careful search failed to reveal a left ureteral orifice. A No. 6 olive-tip catheter was left in the right renal pelvis for decompression. The bladder was left empty. One hour after the procedure, the patient voided profusely from drainage around the catheter. The following morning the ureteral catheter was removed. The patient continued to void. By the fourth hospital day, the blood chemistry was normal. The urine was still low in specific gravity with red and white blood cells and the sedimentation rate was 74 mm., dropping later to 58 mm. Excretory urograms failed to show any evidence of the presence of a left kidney. They continued to show the persistent pyelectasis on the right side. The patient was discharged on the eighth hospital day in good condition, voiding very well. She had received antibiotic supportive therapy. The fetal heart rate remained normal throughout the hospital stay. Two months later, on December 6, 1951, the patient was re-admitted and delivered of a normal infant. The delivery was uneventful. On the eighth day postpartum, the cystoscopy was repeated under local anesthesia. The mucosa of the bladder was normal except in the region of the right ureteral orifice where it appeared moderately inflamed and slightly edematous. The orifice was patent. Using a Woodruff engaging tip catheter and 40 per cent skiodan, by gravity only, a pyelo-ureterogram was made which showed some slight reduction in the degree of pyelectasis, (fig. 1, A). A No. 4 olive-tip catheter was passed to the renal pelvis and a lateral pyelogram made (fig. 1, B). Again, a careful search for the left ureter failed to disclose it. Oxygen 1000 cc was introduced retroperitoneally and a series of x-rays made. A third set of excretory urograms were taken. No evidence of a left kidney was present on any
of those films. The patient had no symptoms. Five children and a husband depended on her. She declined surgical correction of the deformity. vV e are waiting patiently. Further developments will undoubtedly change her mind. SUMMARY
A case of circumcaval, postcaval, or retrocaval ureter has been reported, probably the fifty-seventh. Possibly it is the first reported associated with a solitary right kidney. No kidney could be demonstrated on the left side by non-operative means. Retrocaval ureter should always be considered in cases of right hydronephrosis, especially where the proximal ureteral segment is long and dilated and the course of the ureter extends to the midline or beyond. The S-shape deformity in the proximal ureter is characteristic. The symptomatology is that of the obstructive lesion which develops, complicated or not by infection or stone. Pregnancy hastens the development of the obstructive changes and, therefore, the symptoms likewise. Surgical procedure of choice, when surgery is performed, is division of the hydronephrotic segment above the ureteropelvic junction, removal of the ureter from behind the vena cava and re-anastomosis . .l831 Forest Hills Blvd., East Cleveland 12, Ohio REFEREN"CES GLADSTONE, R. J.: A case in which the right ureter passed behind the inferior vena cava with a short note upon a case in which the left renal vein passed behind the abdominal aorta, and the bearing of these abnormalities on the development of the abdominal veins. Anat. and Physiol., 45: 225-231, 1911. JVIuLLEN, W. H. A"1D E"!GEL, W. J.: J. Radial., 59: 528-537, 1952. A complete bibliography covering the published literature to date is included in this paper. This material has been separately reviewed by us. There appears to be no reason to duplicate its listing here. Grateful appreciation is hereby expressed to these authors for the opportunity to review their paper previous to its publication.