0022-534 7/78/1192-0284$02. 00/0
The Journal of Urology
Vol. 119, February
Printed in U.SA.
Copyright © 1978 by The Williams & Wilkins Co.
A CASE REPORT: DUPLICATED VENA CA VA WITH RIGHT RETROCA VAL URETER AND URETERAL TUMOR JOSEPH C. CLEMENTS,* DAVID G. MCLEOD, WILLIAM R. GREENE
AND
RAY E. STUTZMAN
From the Urology Service, Walter Reed Army Medical Center, Washington, D. C.
ABSTRACT
A case of concomitant ureteral tumor, retrocaval ureter and duplicated vena cava is presented with a discussion of these clinical entities. A 15-year-old white boy was hospitalized for a 3-day history of gradually increasing right flank pain associated with a low grade fever. A single episode of gross, terminal painless hematuria had preceded the pain by 12 hours. Physical examination elicited severe right flank and costovertebral angle tenderness, and the patient had a temperature of 100.lF. Urinalysis revealed numerous white blood cells and bacteria. Although the left collecting system was normal on an excretory urogram there was marked delay in function of the right kidney with only minimal visualization of the dilated calices after 8 hours. Diagnosis was right pyelonephri-
possibility of a ureteral filling defect just proximal to the area where the ureter appeared to be compressed by the vena cava (fig. 1, A). An inferior venacavogram, performed through the left femoral vein, demonstrated what appeared to be a left inferior vena cava (fig. 1, B). A repeat study with the right femoral vein showed a normal inferior vena cava on the right side overlying the previously noted course of the right ureter (fig. 1, C). Exploration revealed a markedly hydronephrotic right kidney with a dilated retrocaval ureter. The kidney was deemed
Fm. 1. A, retrograde ureterogram demonstrates retrocaval ureter and ureteral filling defect. B, injection of contrast medium in left femoral vein reveals left inferior vena cava. Note residual contrast medium in right kidney and ureter, and catheter in right ureter. C, injection of contrast medium in right femoral vein shows right inferior vena cava. tis and the patient was started on parental cephalosporin and aminoglycoside. Urine cultures yielded more than 100,000 colonies per ml. Escherichia coli. Blood cultures were positive for the same organism. After a 10-day course of antibiotics and an uneventful recovery cystourethroscopy and a right retrograde ureterogram were performed. Cystourethroscopy was normal but the retrograde ureterogram suggested the characteristic course of a retrocaval ureter. The study also raised the
unsalvageable because of minimal remaining cortical tissue and a right nephroureterectomy was done. Upon opening the specimen a ureteral tumor was discovered just proximal to where the ureter passed behind the vena cava. Subsequent pathological examination revealed a fibroepithelial polyp. Convalescence was uneventful and the patient currently is asymptomatic.
Accepted for publication October 21, 1977. The opinions or assertions contained herein are the private views of the authors and are not to be construed as official reflecting the views of the Department of the Army or the Department of Defense. * Requests for reprints: Urology Service, Walter Reed Army Medical Center, Washington, D. C. 20012.
The frequency of occurrence of inferior vena cava anomalies cannot be known exactly but it is believed that they may occur in 2 to 3 per cent of the population. A retrocaval ureter is significantly rare with an incidence of less than 0.1 per cent of the population. 1 A left inferior vena cava is encoun-
DISCUSSION
284
DUPLICATED VENA CAVA WITH RIGHT RETROCAVAL URETER AND URETERAL TUMOR
285
Fm. 2. a, embryological anatomical relationship of cardinal vein system. b, normal development of inferior vena cava. c, proposed etiology of anomalies in patient with retrocaval ureter and duplicated inferior vena cava. Reprinted with permission. 5
Our patient also had a ureteral tumor. In this age group the most commonly reported tumor of the ureter is a fibroepitheliomatous polyp. 2 This tumor may present with hematuria, either gross or microscopic, and lumbar pain. Rarely, an abdominal mass from hydronephrosis of the affected kidney is the presenting physical finding. The etiology of benign ureteral tumors is not well established but obstruction, infection and chronic irritation have been implicated. The standard treatment for retrocaval ureter is resection of that portion of the ureter that is retrocaval and an end-to-end anastomosis anterior to the vena cava. 3 Occasionally, excision of redundant ureter may be necessary to prevent kinking and continued obstruction. Since ureteral tumors rarely are malignant in this age group a conservative approach should be adopted. 4 Exploration and frozen section may be ol:ltained and a simple resection of the involved portion of ureter is indicated if the pathology report is benign. The kidney is preserved unless it has been damaged severely by obstruction and/or infection as in our case. The occurrence of these 3 lesions, retrocaval ureter, duplicated inferior vena cava and ureteral tumor, is extremely rare and would be expected in a mathematical sense to occur at the rate of approximately less than 1/200,000. This case underlines the insidious course of renal destruction and the presentation of an obstructing lesion as acute pyelonephritis. It also gives impetus to a review of the development of the inferior vena cava and its anomalies.
tered in 0.2 to 0.5 per cent of the population. Since a retrocaval ureter itself implies an abnormality of the development of the inferior vena cava it is inferred that our patient had a bilateral anomalous development. The commonly accepted development of the inferior vena cava is depicted in figure 2, a and b and the mechanism for the development of the anomalies seen in our case is seen in figure 2, c. A persistence of the left supracardinal vein results in a vena cava on the left side. Persistence of the right subcardinal vein results in a retrocaval ureter. In normal development this segment of the inferior vena cava is formed from the right supracardinal vein lying dorsally, while the left supracardinal vein decreases in size and eventually disappears.
1. Chuang, V. P., Mena, C. E. and Hoskins, P. A.: Congenital anomalies of the inferior vena cava. Review of embryogenesis and presentation of a simplified classification. Brit. J. Radiol., 47: 206, 1974. 2. Crum, P. M., Sayegh, E. S., Sacher, E. C. and Wescott, J. W.: Benign ureteral polyps. J. Urol., 102: 678, 1969. 3. Kelalis, P. P. and King, L. R.: Clinical Pediatric Urology. Philadelphia: W. B. Saunders Co., vol. 1, p. 258, 1976. 4. Colgan, J. R., III, Skaist, L. and Morrow, J. W.: Benign ureteral tumors in childhood: a case report and a plea for conservative management. J. Urol., 109: 308, 1973. 5. Hollinshead, W. H.: Anatomy for Surgeons. Volume II-The Thorax, Abdomen and Pelvis, 2nd ed. New York: Harper and Row, Publishers, Inc., p. 559, 1971.
a
C
EI!
Posterior ··· ·· cordinol v.
~
Supracardinal v.
[]l Subcardinal v.
REFERENCES
The Journal of Urology
Vol. 119, February
Printed in U.SA.
Copyright © 1978 by The Williams & Wilkins Co.
A CASE REPORT: DUPLICATED VENA CA VA WITH RIGHT RETROCA VAL URETER AND URETERAL TUMOR JOSEPH C. CLEMENTS,* DAVID G. MCLEOD, WILLIAM R. GREENE
AND
RAY E. STUTZMAN
From the Urology Service, Walter Reed Army Medical Center, Washington, D. C.
ABSTRACT
A case of concomitant ureteral tumor, retrocaval ureter and duplicated vena cava is presented with a discussion of these clinical entities. A 15-year-old white boy was hospitalized for a 3-day history of gradually increasing right flank pain associated with a low grade fever. A single episode of gross, terminal painless hematuria had preceded the pain by 12 hours. Physical examination elicited severe right flank and costovertebral angle tenderness, and the patient had a temperature of 100.lF. Urinalysis revealed numerous white blood cells and bacteria. Although the left collecting system was normal on an excretory urogram there was marked delay in function of the right kidney with only minimal visualization of the dilated calices after 8 hours. Diagnosis was right pyelonephri-
possibility of a ureteral filling defect just proximal to the area where the ureter appeared to be compressed by the vena cava (fig. 1, A). An inferior venacavogram, performed through the left femoral vein, demonstrated what appeared to be a left inferior vena cava (fig. 1, B). A repeat study with the right femoral vein showed a normal inferior vena cava on the right side overlying the previously noted course of the right ureter (fig. 1, C). Exploration revealed a markedly hydronephrotic right kidney with a dilated retrocaval ureter. The kidney was deemed
Fm. 1. A, retrograde ureterogram demonstrates retrocaval ureter and ureteral filling defect. B, injection of contrast medium in left femoral vein reveals left inferior vena cava. Note residual contrast medium in right kidney and ureter, and catheter in right ureter. C, injection of contrast medium in right femoral vein shows right inferior vena cava. tis and the patient was started on parental cephalosporin and aminoglycoside. Urine cultures yielded more than 100,000 colonies per ml. Escherichia coli. Blood cultures were positive for the same organism. After a 10-day course of antibiotics and an uneventful recovery cystourethroscopy and a right retrograde ureterogram were performed. Cystourethroscopy was normal but the retrograde ureterogram suggested the characteristic course of a retrocaval ureter. The study also raised the
unsalvageable because of minimal remaining cortical tissue and a right nephroureterectomy was done. Upon opening the specimen a ureteral tumor was discovered just proximal to where the ureter passed behind the vena cava. Subsequent pathological examination revealed a fibroepithelial polyp. Convalescence was uneventful and the patient currently is asymptomatic.
Accepted for publication October 21, 1977. The opinions or assertions contained herein are the private views of the authors and are not to be construed as official reflecting the views of the Department of the Army or the Department of Defense. * Requests for reprints: Urology Service, Walter Reed Army Medical Center, Washington, D. C. 20012.
The frequency of occurrence of inferior vena cava anomalies cannot be known exactly but it is believed that they may occur in 2 to 3 per cent of the population. A retrocaval ureter is significantly rare with an incidence of less than 0.1 per cent of the population. 1 A left inferior vena cava is encoun-
DISCUSSION
284
DUPLICATED VENA CAVA WITH RIGHT RETROCAVAL URETER AND URETERAL TUMOR
285
Fm. 2. a, embryological anatomical relationship of cardinal vein system. b, normal development of inferior vena cava. c, proposed etiology of anomalies in patient with retrocaval ureter and duplicated inferior vena cava. Reprinted with permission. 5
Our patient also had a ureteral tumor. In this age group the most commonly reported tumor of the ureter is a fibroepitheliomatous polyp. 2 This tumor may present with hematuria, either gross or microscopic, and lumbar pain. Rarely, an abdominal mass from hydronephrosis of the affected kidney is the presenting physical finding. The etiology of benign ureteral tumors is not well established but obstruction, infection and chronic irritation have been implicated. The standard treatment for retrocaval ureter is resection of that portion of the ureter that is retrocaval and an end-to-end anastomosis anterior to the vena cava. 3 Occasionally, excision of redundant ureter may be necessary to prevent kinking and continued obstruction. Since ureteral tumors rarely are malignant in this age group a conservative approach should be adopted. 4 Exploration and frozen section may be ol:ltained and a simple resection of the involved portion of ureter is indicated if the pathology report is benign. The kidney is preserved unless it has been damaged severely by obstruction and/or infection as in our case. The occurrence of these 3 lesions, retrocaval ureter, duplicated inferior vena cava and ureteral tumor, is extremely rare and would be expected in a mathematical sense to occur at the rate of approximately less than 1/200,000. This case underlines the insidious course of renal destruction and the presentation of an obstructing lesion as acute pyelonephritis. It also gives impetus to a review of the development of the inferior vena cava and its anomalies.
tered in 0.2 to 0.5 per cent of the population. Since a retrocaval ureter itself implies an abnormality of the development of the inferior vena cava it is inferred that our patient had a bilateral anomalous development. The commonly accepted development of the inferior vena cava is depicted in figure 2, a and b and the mechanism for the development of the anomalies seen in our case is seen in figure 2, c. A persistence of the left supracardinal vein results in a vena cava on the left side. Persistence of the right subcardinal vein results in a retrocaval ureter. In normal development this segment of the inferior vena cava is formed from the right supracardinal vein lying dorsally, while the left supracardinal vein decreases in size and eventually disappears.
1. Chuang, V. P., Mena, C. E. and Hoskins, P. A.: Congenital anomalies of the inferior vena cava. Review of embryogenesis and presentation of a simplified classification. Brit. J. Radiol., 47: 206, 1974. 2. Crum, P. M., Sayegh, E. S., Sacher, E. C. and Wescott, J. W.: Benign ureteral polyps. J. Urol., 102: 678, 1969. 3. Kelalis, P. P. and King, L. R.: Clinical Pediatric Urology. Philadelphia: W. B. Saunders Co., vol. 1, p. 258, 1976. 4. Colgan, J. R., III, Skaist, L. and Morrow, J. W.: Benign ureteral tumors in childhood: a case report and a plea for conservative management. J. Urol., 109: 308, 1973. 5. Hollinshead, W. H.: Anatomy for Surgeons. Volume II-The Thorax, Abdomen and Pelvis, 2nd ed. New York: Harper and Row, Publishers, Inc., p. 559, 1971.
a
C
EI!
Posterior ··· ·· cordinol v.
~
Supracardinal v.
[]l Subcardinal v.
REFERENCES
