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Left Retrocaval Ureter Associated with Situs Inversus
THE JOURNAL OF UROLOGY
Vol. 88, No. 4 October 1962 Copyright @'1962 by The Williams & Wilkins Co. Printed in U.S.A.
LEFT RETROCA VAL URETER ASSOCIATED WITH SITUS INVERSUS RALPH E. BROOKS, JR.* From the Department of Urology, the Bowman Gray School of Medicine, Winston-Salem, N. C.
Hochstetter1 in 1893 described the first case of retrocaval ureter. Although this anomaly will undoubtedly remain a rare phenomenon, recognition of it and its complications is becon1ing more common. The number of annually reported cases has increased from 27 in 19392 to over 90 in 1960. 3 Rowland, Bunts and Juano 3 noted 37 cases in the literature that had surgical correction of retrocaval ureter and added 4 cases. All previously reported operative cases have dealt with right retrocaval ureters. This case concerns a left retrocaval ureter in a patient with situs inversus totalis. CASE REPORT
E. H., NCBH, 33 34 49, a 43-year-old, white, right handed father of 3 children, was first treated for a urinary tract infection with signs and symptoms of gross total hematuria, pyuria, dysuria, chills, fever, and urinary frequency in December 1960. In June 1961, he noticed a dull left flank pain with radiation to the left lower quadrant and presented for further evaluation. Past history indicated a "heart condition" since birth and he reportedly was blue on one occasion as an infant (possibly during the time of a respiratory infection). Growth had been normal although exercise tolerance had been decreased. Palpitations had been a complaint and the patient had been hospitalized elsewhere for this in February 1961. Immediately following this he had suffered a heart attack and had been digitalized. Additional history indicated that the patient had undergone no previous surgery. Physical examination revealed a well-developed, well-nourished, alert white man in no * Present address: High Point Medical Center, High Point, N. C. Accepted for publication March 20, 1962. 1 Hochstetter, F.: Beitrage zur Entwickelungsgeschichte des Venensystems der Amnioten: III. Sauger. Morphol. Jahrb., 20: 543-648, 1893. 2 Pick, J. W. and Anson, B. J.: Retrocaval ureter: report of case with discussion of clinical significance. J. Urol., 43: 672-685, 1940. 3 Rowland, H. S., Jr., Bunts, R. C. and Iwano, J. H.: Operative correction of retrocaval ureter: a report of four cases and review of the literature. J. Urol., 83: 820-833, 1960.
484
apparent distress. The blood pressure was 122/74, pulse 92, and respiration 20. The skin was slightly dusky, but there was no cyanosis. There was anterior perforation of the left eardrum. There were no nasal polyps or evidence of sinusitis. Examination of the chest revealed right anterior chest prominence with a slight precordial lift. The heart was percussed 3 cm. to the left of the left sternal border and 2 cm. to the right of the right midclavicular line. A grade 4 systolic murmur with thrill in the fifth intercostal space at the right sternal border radiated to the neck and sternum. There was a short, harsh grade 2 systolic murmur in the right nipple area. The second sound was diminished in the right second intercostal space. The lungs were clear to percussion and auscultation. Femoral pulses were adequate. There was slight left costovertebral angle tenderness to punch and slight tenderness to deep palpation in the left flank. Examination of the genitalia revealed an uncircumcised man with the left testicle slightly lower in the scrotum than the right. The left lateral lobe of the prostate was somewhat larger than the right and slightly tender to palpation. Urine was am.her and clear with specific gravity 1.018, pH 5.0, protein negative, sugar negative; the spun sediment revealed 3 to 4 white blood cells per high power field. The hemoglobin. was 14.8 gm. and hematocrit was 45 per cent. vVhite blood count was 7,000 with a normal differential. Blood urea nitrogen was 15 mg. per cent and electrolytes were normal. A urine culture grew out beta-hemolytic enterococci, sensitive to chloramphenicol, penicillin. and erythromycin. An electrocardiogram showed normal sinus rhythm and atrioventricular heart block. An excretory urogram revealed a normal collecting system on the right. On the left, however, there was some dilatation of the pelvis and infundibula though the calyces remained cupped, and there was delay of emptying of the left side at 3 hours. Left retrograde pyelogram showed a smooth indentation on the proximal left ureter with kinking and displacement medially (fig. 1).
L~}F'T RETROCA VAL URET.~1R ,~SSOCIATED WITH SITUS INVERaUM
Gastrointestinal studies showed the rolon to :1scend high on the right and low on the left. There was an apparent splcnic shadow on the right and a large soft tissue shadmY on the left. Barium swallow showed the barium to enter the stonmch on the right side of the abdomen. Chest films showed bibteral cel'\'ical ribs, cle:-.:trocarclia, and cardiomegaly. Tlw aorta ,rns on thr right. Cardiac catheteriz:1tion (Dr. Henry S. :.\Iiller) ren-,akd dextrocanlia (mirror image situs in\'ersus), increased oxygenation in the right atrium (atrial septaJ defect), increased oxygenation in the right vrntricle (ventricular septa! dcfrct), patent ductus arteriosus (without shunting), pulmonary hyprrknsion, balanced ventricular and arterial pressures, left to right shunting with adequate oxygen :c;aturation, and a left n:na cava. Surgical intervention 1,·as not inclicatccl. Diagno:;ws ,i-rre made of situs inn'rsus totalis, multiple congenital cardiac anomalies and ldt rctrocaval ureter 1i-ith obstruction and l1?JroucphrosiF. July 2.5, the ldt ureter was explored through a flank incision ancl was found to cross under the vena ca1'a (fig. 2, appro:-.:imately 3 cm. distal to the meteroprh·ic junrtion. The proximal por-
tion of the ureter was dilated. The dilated urekt was divided obliquely, spatulated on both th,· proximal and distal ends and brought out frmn under tlH, vena cava. Reanmcrtomosis was cornplishcd with interrupted mucosa-to-mucosa sutures of 0000 chromic catgut (fig. 2, The poskaval segment of ureter appeared normal with no evidence of stricture formation. A ureternl splint of 0.38 inch polyethylene tubing wa~ in sertecl through a pyelostom.r incision, and drni;,. age ,1,as effectrcl with a 18F .\Ialecot tomy catheter. ::\finimal postopcrati\'e fen'!' and ilrus oc,:urnd and the cardiac status remained unchangrd Ull digitalis and quinidi1w. The urPtern1 splint wn,, remo,,ed 5 weeks postoperatin{r. Six weeks postoperatively antegrade pyelogrnm~ (fig. .l showed good filling of the (·ntirr ureter and tJH; nephrostomy tube was removed. ThrC'e moilth, after operation the urine wa~ clear and the patient had no urologic complaints. c\.n excn-,tory urogram showed prompt functiou \\'itho11t ob strudion or delay in drninagi· (fig. ;{, was confirmed h)- rrtrograde DI,:,CUSSTON
The devclopmrnt of retroran,1 meter in rna.r has ]wen the subject of query b:;., many author:' Most of these discussions revert to the ment of the inferior \'em, cava cts l\IcClure and Butler. 4 During frtal life the 1wrmanent (metanephros), ha1'ing de,,eloped in ascends to the upper lumbar level a ring of venous elrnnnel8 composed of pust,cardinal, subcardinal and supracardinal The postcardinal veins disappear at a earl:i, stage. The lumbar subrnrdinal drain tbe gonads, form the sex wins in the adult. The supracarclinal ,-cin forms tJw mai,1 portion of the inferior nna cant in the middie and lower lumbar areas. Retrocaval ureter oecnrn ffith persistence of the posteardinal and cardinal Yeins. Case reports of thrse variatim,R in man ha,,e been summarized.' The only known reported incident of left retrocaval ureter was that of an acardiac fct,UH having ,louble inforior venae cava with
:FrG. 1. Preoperative retrograde pyelogram shows medial deviation of ureter and
-1 JI/IcClnre, C. !:<'. W. and Butler, KG . ment of vena cava inferior in man. Am. 35: 331-384, 1925. 5 Nielsen, P. B.: Retrocaval itreter: rnpon- of
hydronephrosis.
a case. Acta radio!., 51: 170-188, J\),59
486
RALPH E. BROOKS, JR.
FIG. 2. A, surgical exposure of left retrocaval ureter. Penrose drain is around distal portion of ureter. B, surgical exposure after repair of retrocaval ureter with ureter overlying vena cava.
Fw. 3. A, antegrade pyelogram 6 weeks after repair of retrocaval ureter showing adequate drainage and caliber of ureter throughout its course. B, 15 minute excretory urogram 3 months after operation shows prompt function and drainage bilaterally.
LEFT RETROCAVAL URETER ASSOCIATED WITH SITUS INVERSUS
ureters retroca val. 6 This was attributed to the persistence of the right and left postcardinal veins. Marcellus Leccius in 1643 is credited with the first case of situs inversus in man although Aristotle described it earlier in animals. 7 The incidence of human situs inversus totalis is in the general range of 1 in 10,000 births, though partial inversion of one or more organs is somewhat more common. Only a few facts are available as to the specific etiology of situs inversus; however, the following suppositions have been presented in the recent work of Varano and Merklin: 8 "1) A single autosomal recessive gene with complete penetration is responsible for situs inversus in many cases. 2) This gene is partially lethal, producing visceral, vascular, and other defects. 3) Twinning, which in itself is a complex problem, is also responsible in some areas of situs inversus. For example, situs inversus may result from developmental arrest when fission takes place after bilateral symmetry has been established, with the production of monozygotic twins, one of which is the mirror image of the other. Either of these twins may die at an early stage and be resorbed." Another generally accepted theory is van Baer's theory of rotation,9 according to which situs inversus is thought to be due to rotation of the embryo to the right side instead of the left side so that the yolk sac is located on the right side of the embryo. Retrocaval ureter is universally considered to be a "right sided anomaly" according to Campbell.10 The present case of left sided retrocaval 6 Gladstone, R. J.: Acardiac fetus. J. Anat. Physiol., 40: 71-80, 1905. 7 Schmutzer, K. J. and Linde, L. M.: Situs inversus totals associated with complex cardiovascular anomalies. Am. Heart J., 56: 761-768,
1958.
Varano, N. R. and Merklin, R. J.: Situs inversus: review of the literature, report of four cases and analysis of clinical implications. J. Int. Col. Surg., 33: 131-148, 1960. 9 Baer, E. von: Uber Entwickelungsgeschichte der Tiere, Petersburg, 1828. 1° Campbell, M. F.: Embryology and anomalies 8
487
ureter is probably explained on the basis of the coexistent situs inversus totalis. The diagnosis of retrocaval ureter is made radiographically. The characteristic deformity is hydroureteronephrosis in the upper ureter, medial deviation of the ureter, and a S-shaped deformity as the ureter courses under, around, and back across the vena cava. In dubious cases, the diagnosis may be confirmed by a simultaneous retrograde pyelogram and vena cavagram (injecting a femoral vein) .11 The symptoms of retrocaval ureter are those of obstruction and infection; treatment is directed toward relief of these conditions. If advanced hydronephrosis, pyelonephritis, or calculus disease exists, nephrectomy may be the treatment of choice. More frequently, however, corrective surgery is possible or mandatory. 12 Under these circumstances the affected ureter is divided in its dilated portion or at the renal pelvis, removed from beneath the vena cava and reanastomosed utilizing one of the spatulating techniques to insure the maximum ostium and reduce the possibility of stricture and further obstruction. Some authors have recommended division and reanastomosis of the vena cava rather than the ureter, but this suggests unwarranted surgery. Finally, division of the ureter at the bladder hence is to be condemned because of the risk of devascularizing the ureter. SUMMARY
The first known case of unilateral left retrocaval ureter is reported, all previous such cases having been on the right. The patient had situs inversus totalis whioh explains the situation embryologically. Successful surgical correction is documented. The author wishes to express his sincere thanks to Dr. James F. Glenn, associate professor of urology, for guidance in preparing this paper. of the urogenital tract. In: Urology. Philadelphia: W. B. Saunders Co., section 4: 338-339, 1954. 11 Glenn, J. F.: Unpublished data. 12 Blundon, K. E.: Personal communication.
Vol. 88, No. 4 October 1962 Copyright @'1962 by The Williams & Wilkins Co. Printed in U.S.A.
LEFT RETROCA VAL URETER ASSOCIATED WITH SITUS INVERSUS RALPH E. BROOKS, JR.* From the Department of Urology, the Bowman Gray School of Medicine, Winston-Salem, N. C.
Hochstetter1 in 1893 described the first case of retrocaval ureter. Although this anomaly will undoubtedly remain a rare phenomenon, recognition of it and its complications is becon1ing more common. The number of annually reported cases has increased from 27 in 19392 to over 90 in 1960. 3 Rowland, Bunts and Juano 3 noted 37 cases in the literature that had surgical correction of retrocaval ureter and added 4 cases. All previously reported operative cases have dealt with right retrocaval ureters. This case concerns a left retrocaval ureter in a patient with situs inversus totalis. CASE REPORT
E. H., NCBH, 33 34 49, a 43-year-old, white, right handed father of 3 children, was first treated for a urinary tract infection with signs and symptoms of gross total hematuria, pyuria, dysuria, chills, fever, and urinary frequency in December 1960. In June 1961, he noticed a dull left flank pain with radiation to the left lower quadrant and presented for further evaluation. Past history indicated a "heart condition" since birth and he reportedly was blue on one occasion as an infant (possibly during the time of a respiratory infection). Growth had been normal although exercise tolerance had been decreased. Palpitations had been a complaint and the patient had been hospitalized elsewhere for this in February 1961. Immediately following this he had suffered a heart attack and had been digitalized. Additional history indicated that the patient had undergone no previous surgery. Physical examination revealed a well-developed, well-nourished, alert white man in no * Present address: High Point Medical Center, High Point, N. C. Accepted for publication March 20, 1962. 1 Hochstetter, F.: Beitrage zur Entwickelungsgeschichte des Venensystems der Amnioten: III. Sauger. Morphol. Jahrb., 20: 543-648, 1893. 2 Pick, J. W. and Anson, B. J.: Retrocaval ureter: report of case with discussion of clinical significance. J. Urol., 43: 672-685, 1940. 3 Rowland, H. S., Jr., Bunts, R. C. and Iwano, J. H.: Operative correction of retrocaval ureter: a report of four cases and review of the literature. J. Urol., 83: 820-833, 1960.
484
apparent distress. The blood pressure was 122/74, pulse 92, and respiration 20. The skin was slightly dusky, but there was no cyanosis. There was anterior perforation of the left eardrum. There were no nasal polyps or evidence of sinusitis. Examination of the chest revealed right anterior chest prominence with a slight precordial lift. The heart was percussed 3 cm. to the left of the left sternal border and 2 cm. to the right of the right midclavicular line. A grade 4 systolic murmur with thrill in the fifth intercostal space at the right sternal border radiated to the neck and sternum. There was a short, harsh grade 2 systolic murmur in the right nipple area. The second sound was diminished in the right second intercostal space. The lungs were clear to percussion and auscultation. Femoral pulses were adequate. There was slight left costovertebral angle tenderness to punch and slight tenderness to deep palpation in the left flank. Examination of the genitalia revealed an uncircumcised man with the left testicle slightly lower in the scrotum than the right. The left lateral lobe of the prostate was somewhat larger than the right and slightly tender to palpation. Urine was am.her and clear with specific gravity 1.018, pH 5.0, protein negative, sugar negative; the spun sediment revealed 3 to 4 white blood cells per high power field. The hemoglobin. was 14.8 gm. and hematocrit was 45 per cent. vVhite blood count was 7,000 with a normal differential. Blood urea nitrogen was 15 mg. per cent and electrolytes were normal. A urine culture grew out beta-hemolytic enterococci, sensitive to chloramphenicol, penicillin. and erythromycin. An electrocardiogram showed normal sinus rhythm and atrioventricular heart block. An excretory urogram revealed a normal collecting system on the right. On the left, however, there was some dilatation of the pelvis and infundibula though the calyces remained cupped, and there was delay of emptying of the left side at 3 hours. Left retrograde pyelogram showed a smooth indentation on the proximal left ureter with kinking and displacement medially (fig. 1).
L~}F'T RETROCA VAL URET.~1R ,~SSOCIATED WITH SITUS INVERaUM
Gastrointestinal studies showed the rolon to :1scend high on the right and low on the left. There was an apparent splcnic shadow on the right and a large soft tissue shadmY on the left. Barium swallow showed the barium to enter the stonmch on the right side of the abdomen. Chest films showed bibteral cel'\'ical ribs, cle:-.:trocarclia, and cardiomegaly. Tlw aorta ,rns on thr right. Cardiac catheteriz:1tion (Dr. Henry S. :.\Iiller) ren-,akd dextrocanlia (mirror image situs in\'ersus), increased oxygenation in the right atrium (atrial septaJ defect), increased oxygenation in the right vrntricle (ventricular septa! dcfrct), patent ductus arteriosus (without shunting), pulmonary hyprrknsion, balanced ventricular and arterial pressures, left to right shunting with adequate oxygen :c;aturation, and a left n:na cava. Surgical intervention 1,·as not inclicatccl. Diagno:;ws ,i-rre made of situs inn'rsus totalis, multiple congenital cardiac anomalies and ldt rctrocaval ureter 1i-ith obstruction and l1?JroucphrosiF. July 2.5, the ldt ureter was explored through a flank incision ancl was found to cross under the vena ca1'a (fig. 2, appro:-.:imately 3 cm. distal to the meteroprh·ic junrtion. The proximal por-
tion of the ureter was dilated. The dilated urekt was divided obliquely, spatulated on both th,· proximal and distal ends and brought out frmn under tlH, vena cava. Reanmcrtomosis was cornplishcd with interrupted mucosa-to-mucosa sutures of 0000 chromic catgut (fig. 2, The poskaval segment of ureter appeared normal with no evidence of stricture formation. A ureternl splint of 0.38 inch polyethylene tubing wa~ in sertecl through a pyelostom.r incision, and drni;,. age ,1,as effectrcl with a 18F .\Ialecot tomy catheter. ::\finimal postopcrati\'e fen'!' and ilrus oc,:urnd and the cardiac status remained unchangrd Ull digitalis and quinidi1w. The urPtern1 splint wn,, remo,,ed 5 weeks postoperatin{r. Six weeks postoperatively antegrade pyelogrnm~ (fig. .l showed good filling of the (·ntirr ureter and tJH; nephrostomy tube was removed. ThrC'e moilth, after operation the urine wa~ clear and the patient had no urologic complaints. c\.n excn-,tory urogram showed prompt functiou \\'itho11t ob strudion or delay in drninagi· (fig. ;{, was confirmed h)- rrtrograde DI,:,CUSSTON
The devclopmrnt of retroran,1 meter in rna.r has ]wen the subject of query b:;., many author:' Most of these discussions revert to the ment of the inferior \'em, cava cts l\IcClure and Butler. 4 During frtal life the 1wrmanent (metanephros), ha1'ing de,,eloped in ascends to the upper lumbar level a ring of venous elrnnnel8 composed of pust,cardinal, subcardinal and supracardinal The postcardinal veins disappear at a earl:i, stage. The lumbar subrnrdinal drain tbe gonads, form the sex wins in the adult. The supracarclinal ,-cin forms tJw mai,1 portion of the inferior nna cant in the middie and lower lumbar areas. Retrocaval ureter oecnrn ffith persistence of the posteardinal and cardinal Yeins. Case reports of thrse variatim,R in man ha,,e been summarized.' The only known reported incident of left retrocaval ureter was that of an acardiac fct,UH having ,louble inforior venae cava with
:FrG. 1. Preoperative retrograde pyelogram shows medial deviation of ureter and
-1 JI/IcClnre, C. !:<'. W. and Butler, KG . ment of vena cava inferior in man. Am. 35: 331-384, 1925. 5 Nielsen, P. B.: Retrocaval itreter: rnpon- of
hydronephrosis.
a case. Acta radio!., 51: 170-188, J\),59
486
RALPH E. BROOKS, JR.
FIG. 2. A, surgical exposure of left retrocaval ureter. Penrose drain is around distal portion of ureter. B, surgical exposure after repair of retrocaval ureter with ureter overlying vena cava.
Fw. 3. A, antegrade pyelogram 6 weeks after repair of retrocaval ureter showing adequate drainage and caliber of ureter throughout its course. B, 15 minute excretory urogram 3 months after operation shows prompt function and drainage bilaterally.
LEFT RETROCAVAL URETER ASSOCIATED WITH SITUS INVERSUS
ureters retroca val. 6 This was attributed to the persistence of the right and left postcardinal veins. Marcellus Leccius in 1643 is credited with the first case of situs inversus in man although Aristotle described it earlier in animals. 7 The incidence of human situs inversus totalis is in the general range of 1 in 10,000 births, though partial inversion of one or more organs is somewhat more common. Only a few facts are available as to the specific etiology of situs inversus; however, the following suppositions have been presented in the recent work of Varano and Merklin: 8 "1) A single autosomal recessive gene with complete penetration is responsible for situs inversus in many cases. 2) This gene is partially lethal, producing visceral, vascular, and other defects. 3) Twinning, which in itself is a complex problem, is also responsible in some areas of situs inversus. For example, situs inversus may result from developmental arrest when fission takes place after bilateral symmetry has been established, with the production of monozygotic twins, one of which is the mirror image of the other. Either of these twins may die at an early stage and be resorbed." Another generally accepted theory is van Baer's theory of rotation,9 according to which situs inversus is thought to be due to rotation of the embryo to the right side instead of the left side so that the yolk sac is located on the right side of the embryo. Retrocaval ureter is universally considered to be a "right sided anomaly" according to Campbell.10 The present case of left sided retrocaval 6 Gladstone, R. J.: Acardiac fetus. J. Anat. Physiol., 40: 71-80, 1905. 7 Schmutzer, K. J. and Linde, L. M.: Situs inversus totals associated with complex cardiovascular anomalies. Am. Heart J., 56: 761-768,
1958.
Varano, N. R. and Merklin, R. J.: Situs inversus: review of the literature, report of four cases and analysis of clinical implications. J. Int. Col. Surg., 33: 131-148, 1960. 9 Baer, E. von: Uber Entwickelungsgeschichte der Tiere, Petersburg, 1828. 1° Campbell, M. F.: Embryology and anomalies 8
487
ureter is probably explained on the basis of the coexistent situs inversus totalis. The diagnosis of retrocaval ureter is made radiographically. The characteristic deformity is hydroureteronephrosis in the upper ureter, medial deviation of the ureter, and a S-shaped deformity as the ureter courses under, around, and back across the vena cava. In dubious cases, the diagnosis may be confirmed by a simultaneous retrograde pyelogram and vena cavagram (injecting a femoral vein) .11 The symptoms of retrocaval ureter are those of obstruction and infection; treatment is directed toward relief of these conditions. If advanced hydronephrosis, pyelonephritis, or calculus disease exists, nephrectomy may be the treatment of choice. More frequently, however, corrective surgery is possible or mandatory. 12 Under these circumstances the affected ureter is divided in its dilated portion or at the renal pelvis, removed from beneath the vena cava and reanastomosed utilizing one of the spatulating techniques to insure the maximum ostium and reduce the possibility of stricture and further obstruction. Some authors have recommended division and reanastomosis of the vena cava rather than the ureter, but this suggests unwarranted surgery. Finally, division of the ureter at the bladder hence is to be condemned because of the risk of devascularizing the ureter. SUMMARY
The first known case of unilateral left retrocaval ureter is reported, all previous such cases having been on the right. The patient had situs inversus totalis whioh explains the situation embryologically. Successful surgical correction is documented. The author wishes to express his sincere thanks to Dr. James F. Glenn, associate professor of urology, for guidance in preparing this paper. of the urogenital tract. In: Urology. Philadelphia: W. B. Saunders Co., section 4: 338-339, 1954. 11 Glenn, J. F.: Unpublished data. 12 Blundon, K. E.: Personal communication.