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The Treatment of Retrocaval Ureter in the Solitary Kidney
THE ,JocH~AL Ol' U1wr,onY
.Jttlv
& ,Yilkiil1:3
TIIE TREATJ\JK\JT OF RETROCAVAL UllETER IN THE S0L1TAHY KIDJ\ EY J{ENNETH E. BLUNDON
ll.etrocuval meter is still a rather rnre entity, less than 100 cases having bcet1 reported. Five of t,he8e have been complicated by the opposite left kidney being buclly d,mmged or absent. Restorati,.·e surgical treatnwnt bas varied greatly. In Lowsley's 1 case (1946) the solitary ureter was dctachc:d from the bladder, brought out from behind the vena crwn anrl rcimplanted in the bladder. "\nastomotic: stricture of the ureter cle· vdopcd ancl after two operations permanent cutaneous ureterostomy was established. In Hurrill's 2 case (1940) the left kidney was rudi .. mentary. Successful reunastomosis of the right ureter to the renal pelvis wus effected, and this m hfts seemed to be the most successful procedmc. performed ureterourc:terostomy in his case in 11-hich the left kidney 1rns non-f'unct.ioning, and a, successful result wfts obtftined. Corbus 4 (1948) ligated the vena cava to r:orreet the deformity nnd Goodwin, Durke ftncl ,\Iuller 6 (1957) performed vena caval reanasto. mosis successfully in R case in which left renal d,unagc WftS present. Since the anomaly is rare and the form of treatment subject to considerable variation in the solitary kidney, the following case is reported. CASE HEPORT
J, ~5-year-olcl ,rnm111l with lifelong episodes of uri1mry infedion was fouucl on both excretory nrography ancl retrogrn,cle pyelogrnphy to have the typical S shaped deformity of retrocaval ureter draining a solitary kidney (fig. 1). Lateral (ilms 8howe
UroL, 44: 450-457, 1940. 3 Creevy, C. D.: Recognition and surgical correct.ion of reirocaval ureter. J. Urol., 60: 26:30. HJ48. 4 Corbus, B. C.: TJroloµ;ist's Conespondence Letter Club, .lune 7, ID54. 5 Good,Yin, W. K, Burke, D, E., and Muller, W, H.· Hetrocavnl ureter. Surg., Gynee. & Obst., 104: :3:H
overlying the vertebral body of the fourth lumbar vertebra. No normally placed or ectopic left uretl,ral orifice was ever found. A signet shadow in the region of the left kiclrwy wns thought to represent a cakiffod tH-:phrogenic rnm1mnt, probably resulting from fnilure of the left renftl artery to develop properly from nn anoma lous aorta bifurcating at the L3 kveL Exploration in Febnrnry l 9fi l lT\Takcl a retro ca.val ureter sandwiched between tlie ahernrnt right common iliac ftrtery exteuding npwanl to the third lumbar level. The un-,ter ,rns clivickd in its dilated portion ftnd slipperl from nnder it, unattftehed position bt;hincl tlw vrna ca,'a (fig, 2). The postcavnl ureteral segment appeared normal with no evidence of stricture or iRclwmi:1 Because of the marked redundanc.v of thr: severed ureter a 3 inch segment of ureter· was n·st;cted in an oblique nmnner. The uretcral wall was grrntly thickened. Anftstomosis without splinting 1rn~ accomplished using interrupted sut1Hcs of 0000 plain catgut (fig. 3). NephroRtomy drainage 1rn,, considered desirable since this mis n solibry kidney. The cliversionary nephrostorny ,nis continued for :3 ,rnelrn until ftntcgmde pyclng ra.phy indicated no leakage or stricture of the ftnastomosis. 1Vound drairmge cea~cd immediately, The patient has been s:rntptom-fn:t'. :ind had clear nrine 8 months follo\\'ing surgi·1·y, Excretory urograms (fig. 4) indirnte smne residual clubbing of the calyces bnt witl1 recl11ccrl dilatation of the upper uretr:r nnd renal peh·i:,. The blood urea nitrogen has drnpped from a prP operative 20 mg. per cent to JO mg. per et!nt. Tlm urine culture, which previously produced Escher ichi coli, is now sterile. EMBRYOLOGY
Tlw embryology of this condition lms bcc'n disrnssed ftt length by earli<:r authors. Bridly tltc, defect lies in ftbnormft! development of the venu, cava, with persistence of the postcanliual \Tin 01 failure of the subcarclinal-suprncanlina! :tn,tsto mosis to form the adult vena cam,. Fi,·e possible recognized variations to this anomaly lmn· lweu described in detftil hy Pick and .\.nNmt,n l\lcC!nn, 6 Pick, J. W. and Ansou, B. ,J, 1fotrocavnl ureter; report of a case with a discussinu of ils clinicn.l significance. J. Urol., 43: 672-68.5, ID40.
30
KENNETH E. BLUNDON
FIG. 1. A, typical appearance of S shaped deformity of retrocaval ureter in excretory urogram. Signet ring density in left renal area without renal shadow demonstrable. B, medial deviation middle third right ureter on retrograde studies. Postcaval segment of ureter filled and does not appear strictured. C, lateral film demonstrates ureteral catheter posterior to normal position.
and Butler,7 Randall and Campbell 8 and others. This aberrant vena cava lying anterior to the ureter acts to compress the ureter as it bridges over it in its middle third. DISCUSSION
Medical treatment has been advocated in elderly patients with retrocaval ureter, and in those with minimal obstructive changes. In advocating surgical treatment it is well to remember that the mean age of discovery of this congenital disease is 30 years after its onset and the procei3s has on occasion been benign enough to be discovered as an incidental finding in a 67-year-old man. 9 U reteroureterostomy and pel vioureterostomy have been the procedures of choice. Anastomotic stricture or leakage has necessitated secondary 7 McClure, C. F. and Butler, E.G.: Development of inferior vena cava in man. Am. J. Anat.,
35: 331-383, 1925.
8 Randall, A. and Campbell, E. W.: Anomalous relationship of right ureter to the vena cava. J. Urol., 34: 565-583, 1935. 9 Rowland, H. S., Jr., Bunts, R. C. and Iwano, J. H.: Operative correction of retrocaval ureter. J. Urol., 83: 820-833, 1960.
nephrectomy in a few cases and has been a troublesome complication in others. However, of some 35 reported cases9 treated in this manner, more than 80 per cent have achieved a satisfactory result, with 2 cases so treated having had either a rudimentary or completely absent left kidney. Proper healing of a ureteral anastomosis is entirely independent of the presence or absence of the opposite kidney. The penalty for failure is of course greater but it iR still possible to resort to a partial ileal ureter as well as less desirable diversionary procedures. After the ureter has been removed from behind the vena cava it will be found to be considerably elongated as well as dilated. In several instances the retrocaval segment has been fibrotic and strictured. 10 - 12 Before vena ca val surgery is elected, 10 Abeshouse, B. S. and Tankin, L. H.: Retrocaval ureter; report of a case and review of the literature. Am. J. Surg., 84: 383-393, 1952. 11 Goyanna, R., Cook, E. N. and Counseller, V. S.: Circumcaval ureter. Proc. Staff Meet., Mayo Clinic, 21: 356-360, 1946. 12 McElhinney, P. P. B. and Dorsey, J. W.: Retrocaval ureter; case report J. Urol., 59: 497-
500, 1948.
TREATMENT OF RETROCAVAL URETER IN SOLITARY KIDNEY
31
FIG. 2. A, ureter suspended and tented by rubber tissue drains shown descending behind vena cava and emerging from beneath it. B, ureter has been cut and removed from behind vena cava. Two inch overlap of each segment demonstrates degree of ureteral elongation.
Fw. 3. Completed anastomosis. Forceps point to vena cava.
thorough inspection of this postureteral segment for ureteral stricture is mandatory. If stricture is present, the need for ureteral surgery rather than vena ca val surgery would be obvious. Postoperative kinking of the elongated ureter has in other instances led to perpetuation of stasis. To correct ureteral tortuosity ncphropexy and ureteral resection have been utilized. Nephropexy alone upon a kidney without ptosis would seem to have limited effect in straightening the considerably elongated retrocaval ureter. A kidney in normal position lies in fairly close juxtaposition to the diaphragm and suspension in an abnormally high position is limited. Excision of a segment of dilated ureter will correct the tortuos-
FIG. 4. Comparative preoperative and 8 month postoperative excretory urograms. ity at the risk of urctcral stricture or leakage. However, anastomosis through dilated, obliquely cut ureteral segments should make stricture less likely. Vena cavas have been ligated without sequelae because of retrocaval ureter, 4 • 13 trauma and other considerations. Since the vena cava may bec represented in retrocaval ureter by both anterior and posterior branches and by a contralateral left 13 Cathro, A. J. M.: Section of inferior vena cava for retrocaval ureter. J. Urol., 67: 464-47.5,
1952.
32
KENNETH E. BLUNDON
branch, ligation in such incidences would appear to be innocuous, since a small venous brd is drained. However, collateral circula';ion of the lumbar and azygos vein at times has been found to be aberrant or deficient in this condition, 6 , 10 , 12 and it is believed that vena caval ligation could, in the face of poor collateral circulation, le;1d to serious venous insufficiency. There is evidence to suggest that anomalies of the vena cava are not rare. Edwards,14 in studying 33 consecutive autopsy cases, noted 5 major anomalies of the vena cava. These included: 2 double vena cava, 1 vena cava on the left side, 1 pre-ureteral vena cava, and 1 renal vein emptying into the hypogastric vein. Anson15 has been quoted to the effect that a vena cava on the left side was found 4 times in 33 cadavers in 1 year. Ligation of the vena cava has largely been abandoned in favor of anticoagulants as the primary form of treatment in thromboembolism. According to lVIahorner, 16 "a major vein is never tied or occluded with impunity ... no patient who has had a vena caval ligation is without thrombophlebitic edema even with a modestly active life." De Takats17 has abandoned the procedure except as a lifesaving measure when anticoagulants have failed. Bowers and Leb,18 in a series of 25 cases, describe late edema in all, leg ulcerations in 18, amputations in 2, poor locomotion in 7, and 60 per cent with inability to work or reduced income. Robertson19 in another series has described a similar number and type of complications. Not all investigators have been convinced that vena caval ligation results in chronic venous inEdwards, E. A.: Clinical anatomy of lesser variations of inferior vena cava. Angiology, 2: 14
85, 1951.
15 Anson, B.: Personal communication cited by Dale, W. A.: Surgery, 43: 24-44, 1958. 16 Mahorner, H.: Section of vascular disease. In: Complications in Surgery and Their Management, ed. by C. P. Artz and J. D. Hardy. Philadelphia: W. B. Saunders Co., 1961, p. 218. 17 de Takats, G.: Vascular Surgery. Philadelphia: W. B. Saunders Co., 1959, p. 267. 18 Bowers, R. F. and Leb, S. M.: Late results of inferior vena cava ligation. Surgery, 37: 622, 1955. 19 Shea, P. C., Jr. and Robertson, R.. L.: Late sequelae of inferior vena cava ligation. Surg., Gynec. & Obst., 93: 153-158, 1951.
sufficiency. Nevertheless, when such controversy is voiced it is apparent that a number of investigators have had serious complications following vena caval ligation, and the procedure is not completely innocuous nor should it be undertaken lightly. Vena ca val reanastomosis has been performed by Goodwin, Burke and lVIuller 5 with an excellent result. It is a more formidable procedure than ureteral anastomosis and some difficulty was experienced at operation in suturing the cut vena cava without tension. In Cathro's 13 case a tendency of the divided encl of the vena cava to retract was also noted and this was likewise attributed to the vena cava being under tension. A great advantage to this method is that the damaged urinary tract is not further insulted and cannot be made worse by surgical intervention. Postoperative kinking of the redundant ureter would not always seem to be amenable to nephropexy. Chronic venous insufficiency could become a problem of magnitude if anastomotic stricture resulted. The vena cava, like the solitary ureter, is not a paired structure and in each instance the anastomosis must succeed. However, an excellent result was obtained by this method and as vascular surgery continues to be more widely employed this method of treatment may become the preferred method. However, before this procedure becomes generally adopted it should be well established that uniformly superior results arc obtained. SUMMARY
Retrocaval ureter in a solitary kidney has been successfully treated by ureteral and vena caval section and reanastomosis, and by vena caval ligation. The rarity of the entity in a solitary kidney invalidates conclusion as to the best form of therapy. Each method of treatment may have sequelae of considerable severity. Anatomic variations in the individual case will be influencing factors in assessing the most judicious type of surgical procedure to be employed.
1221 Virginia St., E., Charleston 1, W.Va.
.Jttlv
& ,Yilkiil1:3
TIIE TREATJ\JK\JT OF RETROCAVAL UllETER IN THE S0L1TAHY KIDJ\ EY J{ENNETH E. BLUNDON
ll.etrocuval meter is still a rather rnre entity, less than 100 cases having bcet1 reported. Five of t,he8e have been complicated by the opposite left kidney being buclly d,mmged or absent. Restorati,.·e surgical treatnwnt bas varied greatly. In Lowsley's 1 case (1946) the solitary ureter was dctachc:d from the bladder, brought out from behind the vena crwn anrl rcimplanted in the bladder. "\nastomotic: stricture of the ureter cle· vdopcd ancl after two operations permanent cutaneous ureterostomy was established. In Hurrill's 2 case (1940) the left kidney was rudi .. mentary. Successful reunastomosis of the right ureter to the renal pelvis wus effected, and this m hfts seemed to be the most successful procedmc. performed ureterourc:terostomy in his case in 11-hich the left kidney 1rns non-f'unct.ioning, and a, successful result wfts obtftined. Corbus 4 (1948) ligated the vena cava to r:orreet the deformity nnd Goodwin, Durke ftncl ,\Iuller 6 (1957) performed vena caval reanasto. mosis successfully in R case in which left renal d,unagc WftS present. Since the anomaly is rare and the form of treatment subject to considerable variation in the solitary kidney, the following case is reported. CASE HEPORT
J, ~5-year-olcl ,rnm111l with lifelong episodes of uri1mry infedion was fouucl on both excretory nrography ancl retrogrn,cle pyelogrnphy to have the typical S shaped deformity of retrocaval ureter draining a solitary kidney (fig. 1). Lateral (ilms 8howe
UroL, 44: 450-457, 1940. 3 Creevy, C. D.: Recognition and surgical correct.ion of reirocaval ureter. J. Urol., 60: 26:30. HJ48. 4 Corbus, B. C.: TJroloµ;ist's Conespondence Letter Club, .lune 7, ID54. 5 Good,Yin, W. K, Burke, D, E., and Muller, W, H.· Hetrocavnl ureter. Surg., Gynee. & Obst., 104: :3:H
overlying the vertebral body of the fourth lumbar vertebra. No normally placed or ectopic left uretl,ral orifice was ever found. A signet shadow in the region of the left kiclrwy wns thought to represent a cakiffod tH-:phrogenic rnm1mnt, probably resulting from fnilure of the left renftl artery to develop properly from nn anoma lous aorta bifurcating at the L3 kveL Exploration in Febnrnry l 9fi l lT\Takcl a retro ca.val ureter sandwiched between tlie ahernrnt right common iliac ftrtery exteuding npwanl to the third lumbar level. The un-,ter ,rns clivickd in its dilated portion ftnd slipperl from nnder it, unattftehed position bt;hincl tlw vrna ca,'a (fig, 2). The postcavnl ureteral segment appeared normal with no evidence of stricture or iRclwmi:1 Because of the marked redundanc.v of thr: severed ureter a 3 inch segment of ureter· was n·st;cted in an oblique nmnner. The uretcral wall was grrntly thickened. Anftstomosis without splinting 1rn~ accomplished using interrupted sut1Hcs of 0000 plain catgut (fig. 3). NephroRtomy drainage 1rn,, considered desirable since this mis n solibry kidney. The cliversionary nephrostorny ,nis continued for :3 ,rnelrn until ftntcgmde pyclng ra.phy indicated no leakage or stricture of the ftnastomosis. 1Vound drairmge cea~cd immediately, The patient has been s:rntptom-fn:t'. :ind had clear nrine 8 months follo\\'ing surgi·1·y, Excretory urograms (fig. 4) indirnte smne residual clubbing of the calyces bnt witl1 recl11ccrl dilatation of the upper uretr:r nnd renal peh·i:,. The blood urea nitrogen has drnpped from a prP operative 20 mg. per cent to JO mg. per et!nt. Tlm urine culture, which previously produced Escher ichi coli, is now sterile. EMBRYOLOGY
Tlw embryology of this condition lms bcc'n disrnssed ftt length by earli<:r authors. Bridly tltc, defect lies in ftbnormft! development of the venu, cava, with persistence of the postcanliual \Tin 01 failure of the subcarclinal-suprncanlina! :tn,tsto mosis to form the adult vena cam,. Fi,·e possible recognized variations to this anomaly lmn· lweu described in detftil hy Pick and .\.nNmt,n l\lcC!nn, 6 Pick, J. W. and Ansou, B. ,J, 1fotrocavnl ureter; report of a case with a discussinu of ils clinicn.l significance. J. Urol., 43: 672-68.5, ID40.
30
KENNETH E. BLUNDON
FIG. 1. A, typical appearance of S shaped deformity of retrocaval ureter in excretory urogram. Signet ring density in left renal area without renal shadow demonstrable. B, medial deviation middle third right ureter on retrograde studies. Postcaval segment of ureter filled and does not appear strictured. C, lateral film demonstrates ureteral catheter posterior to normal position.
and Butler,7 Randall and Campbell 8 and others. This aberrant vena cava lying anterior to the ureter acts to compress the ureter as it bridges over it in its middle third. DISCUSSION
Medical treatment has been advocated in elderly patients with retrocaval ureter, and in those with minimal obstructive changes. In advocating surgical treatment it is well to remember that the mean age of discovery of this congenital disease is 30 years after its onset and the procei3s has on occasion been benign enough to be discovered as an incidental finding in a 67-year-old man. 9 U reteroureterostomy and pel vioureterostomy have been the procedures of choice. Anastomotic stricture or leakage has necessitated secondary 7 McClure, C. F. and Butler, E.G.: Development of inferior vena cava in man. Am. J. Anat.,
35: 331-383, 1925.
8 Randall, A. and Campbell, E. W.: Anomalous relationship of right ureter to the vena cava. J. Urol., 34: 565-583, 1935. 9 Rowland, H. S., Jr., Bunts, R. C. and Iwano, J. H.: Operative correction of retrocaval ureter. J. Urol., 83: 820-833, 1960.
nephrectomy in a few cases and has been a troublesome complication in others. However, of some 35 reported cases9 treated in this manner, more than 80 per cent have achieved a satisfactory result, with 2 cases so treated having had either a rudimentary or completely absent left kidney. Proper healing of a ureteral anastomosis is entirely independent of the presence or absence of the opposite kidney. The penalty for failure is of course greater but it iR still possible to resort to a partial ileal ureter as well as less desirable diversionary procedures. After the ureter has been removed from behind the vena cava it will be found to be considerably elongated as well as dilated. In several instances the retrocaval segment has been fibrotic and strictured. 10 - 12 Before vena ca val surgery is elected, 10 Abeshouse, B. S. and Tankin, L. H.: Retrocaval ureter; report of a case and review of the literature. Am. J. Surg., 84: 383-393, 1952. 11 Goyanna, R., Cook, E. N. and Counseller, V. S.: Circumcaval ureter. Proc. Staff Meet., Mayo Clinic, 21: 356-360, 1946. 12 McElhinney, P. P. B. and Dorsey, J. W.: Retrocaval ureter; case report J. Urol., 59: 497-
500, 1948.
TREATMENT OF RETROCAVAL URETER IN SOLITARY KIDNEY
31
FIG. 2. A, ureter suspended and tented by rubber tissue drains shown descending behind vena cava and emerging from beneath it. B, ureter has been cut and removed from behind vena cava. Two inch overlap of each segment demonstrates degree of ureteral elongation.
Fw. 3. Completed anastomosis. Forceps point to vena cava.
thorough inspection of this postureteral segment for ureteral stricture is mandatory. If stricture is present, the need for ureteral surgery rather than vena ca val surgery would be obvious. Postoperative kinking of the elongated ureter has in other instances led to perpetuation of stasis. To correct ureteral tortuosity ncphropexy and ureteral resection have been utilized. Nephropexy alone upon a kidney without ptosis would seem to have limited effect in straightening the considerably elongated retrocaval ureter. A kidney in normal position lies in fairly close juxtaposition to the diaphragm and suspension in an abnormally high position is limited. Excision of a segment of dilated ureter will correct the tortuos-
FIG. 4. Comparative preoperative and 8 month postoperative excretory urograms. ity at the risk of urctcral stricture or leakage. However, anastomosis through dilated, obliquely cut ureteral segments should make stricture less likely. Vena cavas have been ligated without sequelae because of retrocaval ureter, 4 • 13 trauma and other considerations. Since the vena cava may bec represented in retrocaval ureter by both anterior and posterior branches and by a contralateral left 13 Cathro, A. J. M.: Section of inferior vena cava for retrocaval ureter. J. Urol., 67: 464-47.5,
1952.
32
KENNETH E. BLUNDON
branch, ligation in such incidences would appear to be innocuous, since a small venous brd is drained. However, collateral circula';ion of the lumbar and azygos vein at times has been found to be aberrant or deficient in this condition, 6 , 10 , 12 and it is believed that vena caval ligation could, in the face of poor collateral circulation, le;1d to serious venous insufficiency. There is evidence to suggest that anomalies of the vena cava are not rare. Edwards,14 in studying 33 consecutive autopsy cases, noted 5 major anomalies of the vena cava. These included: 2 double vena cava, 1 vena cava on the left side, 1 pre-ureteral vena cava, and 1 renal vein emptying into the hypogastric vein. Anson15 has been quoted to the effect that a vena cava on the left side was found 4 times in 33 cadavers in 1 year. Ligation of the vena cava has largely been abandoned in favor of anticoagulants as the primary form of treatment in thromboembolism. According to lVIahorner, 16 "a major vein is never tied or occluded with impunity ... no patient who has had a vena caval ligation is without thrombophlebitic edema even with a modestly active life." De Takats17 has abandoned the procedure except as a lifesaving measure when anticoagulants have failed. Bowers and Leb,18 in a series of 25 cases, describe late edema in all, leg ulcerations in 18, amputations in 2, poor locomotion in 7, and 60 per cent with inability to work or reduced income. Robertson19 in another series has described a similar number and type of complications. Not all investigators have been convinced that vena caval ligation results in chronic venous inEdwards, E. A.: Clinical anatomy of lesser variations of inferior vena cava. Angiology, 2: 14
85, 1951.
15 Anson, B.: Personal communication cited by Dale, W. A.: Surgery, 43: 24-44, 1958. 16 Mahorner, H.: Section of vascular disease. In: Complications in Surgery and Their Management, ed. by C. P. Artz and J. D. Hardy. Philadelphia: W. B. Saunders Co., 1961, p. 218. 17 de Takats, G.: Vascular Surgery. Philadelphia: W. B. Saunders Co., 1959, p. 267. 18 Bowers, R. F. and Leb, S. M.: Late results of inferior vena cava ligation. Surgery, 37: 622, 1955. 19 Shea, P. C., Jr. and Robertson, R.. L.: Late sequelae of inferior vena cava ligation. Surg., Gynec. & Obst., 93: 153-158, 1951.
sufficiency. Nevertheless, when such controversy is voiced it is apparent that a number of investigators have had serious complications following vena caval ligation, and the procedure is not completely innocuous nor should it be undertaken lightly. Vena ca val reanastomosis has been performed by Goodwin, Burke and lVIuller 5 with an excellent result. It is a more formidable procedure than ureteral anastomosis and some difficulty was experienced at operation in suturing the cut vena cava without tension. In Cathro's 13 case a tendency of the divided encl of the vena cava to retract was also noted and this was likewise attributed to the vena cava being under tension. A great advantage to this method is that the damaged urinary tract is not further insulted and cannot be made worse by surgical intervention. Postoperative kinking of the redundant ureter would not always seem to be amenable to nephropexy. Chronic venous insufficiency could become a problem of magnitude if anastomotic stricture resulted. The vena cava, like the solitary ureter, is not a paired structure and in each instance the anastomosis must succeed. However, an excellent result was obtained by this method and as vascular surgery continues to be more widely employed this method of treatment may become the preferred method. However, before this procedure becomes generally adopted it should be well established that uniformly superior results arc obtained. SUMMARY
Retrocaval ureter in a solitary kidney has been successfully treated by ureteral and vena caval section and reanastomosis, and by vena caval ligation. The rarity of the entity in a solitary kidney invalidates conclusion as to the best form of therapy. Each method of treatment may have sequelae of considerable severity. Anatomic variations in the individual case will be influencing factors in assessing the most judicious type of surgical procedure to be employed.
1221 Virginia St., E., Charleston 1, W.Va.