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Double Vas Deferens Associated with Ipsilateral Renal Agenesis, Simulating Ectopic Ureter
Vol. 108, October Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright © 1972 by The Williams & Wilkins Co.
Pediatric Urology DOUBLE VAS DEFERENS ASSOCIATED WITH IPSILATERAL RENAL AGENESIS, SIMULATING ECTOPIC URETER TOMOHIKOKOYANAGI, ICHIROTSUJI, TETSUOKUDO, TOKIJI ISHIKAWA AND KENICHI SASAKI From the Department of Urology and Central Pathology, Hokkaido University School of Medicine, Sapporo, Japan
The incidence of renal agenesis varies with different reports, with the most common figure given at 1 to l,000. 1- 4 Among 1,498 cases of renal agenesis, there were 277 patients with associated genital anomalies (18.5 per cent). Of these, 92 subjects were male and 185 subjects were female, indicating female preponderance. 4 Anomalies of the vas deferens are quite rare. 1 • 5 • 6 Herein we present a case in which ectopic ureter was suspected preoperatively and yet pathological diagnosis disclosed a combination of extremely rare anomalies involving the kidney, vas deferens and testis. We have found no similar case other than one reported by Mathe and Dunn. 7 CASE REPORT
A 15-year-old boy was admitted to the Hokkaido University Hospital on February 18, 1971 because of intermittent fever, perineal pain and sanguineous urethral discharge 2 months in duration. He was initially treated with broad-spectrum antibiotics for pericystitis secondary to acute appendicitis. This diagnosis was based on the findings of tenderness in the right lower quadrant of the abdomen and in the right pelvic fossa, aseptic hematopyuria and patchy submucous hemorrhagic change on the right side of the bladder wall demonstrated by cystoscopy. Cystoscopy also showed hemi-atrophy of the right trigone with absence· of the right ureteral orifice. Subjective and objective response during a 1-week period was impressive. An excretory urogram disclosed non-visualization of the right renal system. Aortography, renal scanning, voiding cystourethrography, retrograde urethrography and thorough enAccepted for publication January 7, 1972. 1 Campbell, M. F. and Harrison, J. H.: Urology. Philadelphia: W. B. Saunders Co., vol. 2, 1970. 2 Bell, E. T.: Renal Diseases, 2nd ed. Philadelphia: Lea & Febiger, 1950. 3 Ashley, D. J.B. and Mostofi, F. K.: Renal agenesis and dysgenesis. J. Urol., 83: 211, 1960. 4 Doroshow, L. W. and Abeshouse, B. S.: Congenital unilateral solitary kidney: report of 37 cases and a review of the literature. Urol. Survey, 11: 219, 1961. 6 Seitzman, D. M. and Patton, J. F.: Ureteral ectopia: combined ureteral and vas deferens anomaly. J. Urol., 84: 604, 1960. 6 Michelson, L.: Congenital anomalies of the ductus deferens and epididymis. J. Urol., 61: 384, 1949. 7 Mathe, C. P. and Dunn, G.: Double vas deferens associated with solitary kidney: case report. J. Urol., 69: 461, 1948.
doscopy were carried out. However, pelvic angiography was not done. The absence of the right hemitrigone and non-visualization of the renal tissue on aortogram were suggestive of an ectopic ureter from the pelvic kidney. However, urethroscopy failed to confirm the ureteral orifice in the bladder, bladder neck or posterior urethra. We believed it possible that the pelvic kidney was draining ectopically to the seminal tract. The patient was discharged from the hospital on March 9 for outpatient followup. Additional diagnoses included slight mental retardation, spina bifida occulta of Sl and congenital absence of the second left rib. There were 2 similar episodes of fever associated with perineal and rectal pain during the followup. Physical findings then were compatible with either acute prostatitis or seminal vesiculitis. Treatment with broad-spectrum antibiotics gave satisfactory response each time. Approximately 4 months after discharge from the hospital proteinuria, labile hypertension of recent onset and hyperuricemia were detected. Although he was free of symptoms of the previous illness, the patient was admitted to the pediatric ward on June 25 for evaluation of possible nephritis. Extensive studies merely confirmed the previous findings: hypertension 160 to 140/100 to 90 mm. Hg, increased peripheral renin activity 33 to 7 5 ng. per cc, hyperuricemia 6.0 to 8.0 mg. per cen:t and a 24-hour proteinuria of 2 gm. The patient was transferred to the urology service for renal biopsy. Because of the risk of biopsy in a solitary kidney and the great possibility of a right pelvic kidney and ectopic ureter, a right vasogram was carried out instead of biopsy. This revealed a tubal structure ascending from the area of the right seminal vesicle, which showed some ectatic change (fig. 1). The patient underwent exploration on August 4. The preoperative impression was right ectopic ureter draining into the seminal vesicle. The previous illnesses simulating acute appendicitis and later prostatovesiculitis would easily be explained by recurrent ureteritis of the ectopic ureter. With a modified extended Gibson incision, the right retroperitoneal space and pelvis were entered. A tubular structure similar to the ureter was easily identified over the right iliac vessel and this extended caudally, crossing the seminal vesicle along its posterolateral aspect, and ended in either the ejaculatory duct or the posterior urethra. Cephalically this terminated 631
632
KOYANAGI AND ASSOCIATES
in a structure at about the L3 level that simulated a rudimentary kidney and was connected to the vena cava by a thin venous pedicle. The whole structure from its cephalic end to the caudal end was excised (fig. 2). The preoperative diagnosis of ectopic ureter was entertained until the final pathology report arrived.
Fm. 1. Preoperative right seminal vesiculogram
The specimen was composed of the tubal structure some 25 cm. in length and 0.5 cm. diameter and the rudimentary mass. measuring 3 by 0.8 by 0.3 cm. Histologically the tubal structure was comprised of cubic or columnar epithelia, 3 distinct muscular , layers and an outer fibrous element (fig. 3). The rudimentary structure contained glandular elements that were lined by tall columnar epithelia, with stereocilia and stroma that showed moderate inflammatory cellular infiltration. In no section were renal or testicular elements present (fig. 4). Subsequently, we concluded that the specimen was the vas deferens and epididymis. Convalescence was satisfactory. Because of the pathology report, ipsilateral testicular biopsy and bilateral vasograms were done to evaluate the gonadal anomaly and the result of operation. Biopsy showed germinal cell aplasia. The vasogram demonstrated the persistence of the ectatic change of the seminal vesicle and residuum of seminal vesiculitis. We also noted that the duplicated vas deferens was completely excised and the left vas and seminal vesicle were normal (fig. 5). Interestingly enough disappearance of albuminuria was confirmed on 2 occasions postoperatively. The blood pressure stabilized in the normotensive range and the peripheral renin activity was normalized (3.0 and 3.(i ng. per cc). However, serum uric acid remained elevated.
I.V,C. Aorta
Rudimentary Kidney (Epididymis) Ectopic Ureter ? - - - - - · (pouble Vas Deferens)
Vas Deferetis Proper
Seminal Vesicle
Fm. 2. Schematic illustration of operative finding
DOUBLE VAS DEFERENS ASSOCIATED WITH IPSILATERAL RENAL AGENESIS
Fm. 3. Microscopic appearance of ductal structure which we concluded was vas deferens. DISCUSSIOX
There are various embryological explanations for agenesis of the kidney. Campbell stressed failure of the vascular supply and resulting renal ischemia as its cause. 8 Potter 9 and others10 • 11 generally believed that failure of ureteral bud formation from the lower end of the mesonephric duct leads to the undifferentiation of the renal parenchyma. Potter states, "A kidney is never formed in the absence of ureter". Ashley and Mostofi found 10 cases of renal dysgenesis in which there was complete absence of the ureters. 3 They suggested that potentiality for renal differentiation rests in the structure of the nephrogenic ridge itself. The pronephros and mesonephros form their tubules and ducts in response to organizers as yet unknown. The stimulus for the outgrowth of the ureteral bud from the lower end of the mesonephric duct arises in the metanephric blastema at 8 Campbell, M. F.: Urology. Philadelphia: W. B. Saunders Co., vol. 1, 1954. 9 Potter, E. L.: Pathology of the Fetus and the Newborn. Chicago: Year Book Medical Publishers, 1952. 10 Radasch, H. E.: Congenital unilateral absence of the urogenital system and its relation to the development of the wolffian and milllerian ducts. Amer. J. Med. Sci., 136: 111, 1908. 11 Allen, A. C.: The Kidney: Medical and Surgical Diseases. New York: Grune & Stratton, 1951.
633
the lower end of the nephrogenic ridge, while the metanephric blastema has within itself the potentiality for differentiation into a kidney albeit one without the drainage necessary for function. For the full development of the kidney this entire mechanism must be intact. Ashley and Mostofi classified renal defects into 2 major groups: 1) those in which the nephrogenic ridge fails to show any potentiality of organogenesis and 2) those in which the nephrogenic ridge fails to show potentiality for renal differentiation after the formation of pronephric and mesonephric ducts. The latter group is subdivided into 5 additional types. Ashley and Mostofi believe that all anomalies of the renal tract are most easily explained by this hypothesis. The present case is explained to some extent by the type 2a anomaly proposed by Ashley and Mostofi, in which the metanephric blastema fails to develop and induction of the formation of ureteral bud is absent, while the wolffian duct is present. N evertheless, the duplication of vas deferens and epididymis requires additional explanations. In the English literature only Mathe and Dunn have reported a similar case, although their case did not include double epididymis. They thought that the double vas developed from the double wolffian duct. One duct united normally with the primitive testis and descended into the scrotum. Its caudal end terminated in the ejaculatory duct in the posterior urethra. The second vas remained unattached to the testis and retained its primitive anatomical relationship extending from the renal fossa to the point where it entered the seminal vesicle. Whether the division of the wolffian duct is related to the factor responsible for the failure of the ureteral bud to form from the lower end of mesonephric duct is a matter for speculation. The fifth or sixth week of intrauterine life, at which time the metanephros, ureteral bud and mesonephric duct are developing, is also a critical period for other systems. The effect of any damage to the fetus at this time can easily be appreciated by the frequency of associated anomalies. The present case demonstrates this association of anomalies. There were germinal cell aplasia of the ipsilateral testis, absence of the second left rib, spina bifida occulta and slight mental retardation in addition to the genitourinary anomalies discussed herein. Some agent or agents must have worked on the germinal ridge (nephrogenic and genital ridge), inducing ipsilateral renal agenesis, double vas deferens and germinal cell aplasia of the testis. This case is unique in that the roentgenographic appearance was hardly distinguishable from an ectopic ureter. Among previously reported cases of ectopic ureter in a male subject there must have been cases that were erroneously reported as such without careful histological examination. Our case i~ also unique in that there were the associated clinical features of hypertension, albuminuria and hyperuricemia. The postoperative disappearance of the hypertension and albuminuria is difficult to explain. The amount of proteinaceous material contained in
634
KOYANAGI AND ASSOCIATES
FIG. 4. A, histology of rudimentary structure. Note epididymal architecture and absence of any renal element. B, greater magnification of part A.Note stereocilia, characteristic of epididymal epithelia.
the specimen was too slight to account for the albuminuria. Whether focal nephritis secondary to the source of infection in the double vas and epididymis could explain the transient albuminuria and hypertension is a matter of conjecture. We regret that we did not perform the renal biopsy. The problem of hyperuricemia awaits further study. SUMMARY
FIG. 5. Postoperative bilateral seminal vesiculogram.
A rare anomaly of double vas deferens and epididymis associated with ipsilateral renal agenesis and germinal cell aplasia of the testis, simulating ectopic ureter was presented. Factors of as yet undetermined nature are thought to have caused a defect in the urogenital ridge at a critical time of fetal development. Extra-embryonic factors acting on the embryo at this crucial stage caused concurrent anomalies in other parts of the body. Our case was unique in that there was associated hypertension with increased peripheral renin activity and albuminuria which returned to normal postoperatively.
THE JOURNAL OF UROLOGY
Copyright © 1972 by The Williams & Wilkins Co.
Pediatric Urology DOUBLE VAS DEFERENS ASSOCIATED WITH IPSILATERAL RENAL AGENESIS, SIMULATING ECTOPIC URETER TOMOHIKOKOYANAGI, ICHIROTSUJI, TETSUOKUDO, TOKIJI ISHIKAWA AND KENICHI SASAKI From the Department of Urology and Central Pathology, Hokkaido University School of Medicine, Sapporo, Japan
The incidence of renal agenesis varies with different reports, with the most common figure given at 1 to l,000. 1- 4 Among 1,498 cases of renal agenesis, there were 277 patients with associated genital anomalies (18.5 per cent). Of these, 92 subjects were male and 185 subjects were female, indicating female preponderance. 4 Anomalies of the vas deferens are quite rare. 1 • 5 • 6 Herein we present a case in which ectopic ureter was suspected preoperatively and yet pathological diagnosis disclosed a combination of extremely rare anomalies involving the kidney, vas deferens and testis. We have found no similar case other than one reported by Mathe and Dunn. 7 CASE REPORT
A 15-year-old boy was admitted to the Hokkaido University Hospital on February 18, 1971 because of intermittent fever, perineal pain and sanguineous urethral discharge 2 months in duration. He was initially treated with broad-spectrum antibiotics for pericystitis secondary to acute appendicitis. This diagnosis was based on the findings of tenderness in the right lower quadrant of the abdomen and in the right pelvic fossa, aseptic hematopyuria and patchy submucous hemorrhagic change on the right side of the bladder wall demonstrated by cystoscopy. Cystoscopy also showed hemi-atrophy of the right trigone with absence· of the right ureteral orifice. Subjective and objective response during a 1-week period was impressive. An excretory urogram disclosed non-visualization of the right renal system. Aortography, renal scanning, voiding cystourethrography, retrograde urethrography and thorough enAccepted for publication January 7, 1972. 1 Campbell, M. F. and Harrison, J. H.: Urology. Philadelphia: W. B. Saunders Co., vol. 2, 1970. 2 Bell, E. T.: Renal Diseases, 2nd ed. Philadelphia: Lea & Febiger, 1950. 3 Ashley, D. J.B. and Mostofi, F. K.: Renal agenesis and dysgenesis. J. Urol., 83: 211, 1960. 4 Doroshow, L. W. and Abeshouse, B. S.: Congenital unilateral solitary kidney: report of 37 cases and a review of the literature. Urol. Survey, 11: 219, 1961. 6 Seitzman, D. M. and Patton, J. F.: Ureteral ectopia: combined ureteral and vas deferens anomaly. J. Urol., 84: 604, 1960. 6 Michelson, L.: Congenital anomalies of the ductus deferens and epididymis. J. Urol., 61: 384, 1949. 7 Mathe, C. P. and Dunn, G.: Double vas deferens associated with solitary kidney: case report. J. Urol., 69: 461, 1948.
doscopy were carried out. However, pelvic angiography was not done. The absence of the right hemitrigone and non-visualization of the renal tissue on aortogram were suggestive of an ectopic ureter from the pelvic kidney. However, urethroscopy failed to confirm the ureteral orifice in the bladder, bladder neck or posterior urethra. We believed it possible that the pelvic kidney was draining ectopically to the seminal tract. The patient was discharged from the hospital on March 9 for outpatient followup. Additional diagnoses included slight mental retardation, spina bifida occulta of Sl and congenital absence of the second left rib. There were 2 similar episodes of fever associated with perineal and rectal pain during the followup. Physical findings then were compatible with either acute prostatitis or seminal vesiculitis. Treatment with broad-spectrum antibiotics gave satisfactory response each time. Approximately 4 months after discharge from the hospital proteinuria, labile hypertension of recent onset and hyperuricemia were detected. Although he was free of symptoms of the previous illness, the patient was admitted to the pediatric ward on June 25 for evaluation of possible nephritis. Extensive studies merely confirmed the previous findings: hypertension 160 to 140/100 to 90 mm. Hg, increased peripheral renin activity 33 to 7 5 ng. per cc, hyperuricemia 6.0 to 8.0 mg. per cen:t and a 24-hour proteinuria of 2 gm. The patient was transferred to the urology service for renal biopsy. Because of the risk of biopsy in a solitary kidney and the great possibility of a right pelvic kidney and ectopic ureter, a right vasogram was carried out instead of biopsy. This revealed a tubal structure ascending from the area of the right seminal vesicle, which showed some ectatic change (fig. 1). The patient underwent exploration on August 4. The preoperative impression was right ectopic ureter draining into the seminal vesicle. The previous illnesses simulating acute appendicitis and later prostatovesiculitis would easily be explained by recurrent ureteritis of the ectopic ureter. With a modified extended Gibson incision, the right retroperitoneal space and pelvis were entered. A tubular structure similar to the ureter was easily identified over the right iliac vessel and this extended caudally, crossing the seminal vesicle along its posterolateral aspect, and ended in either the ejaculatory duct or the posterior urethra. Cephalically this terminated 631
632
KOYANAGI AND ASSOCIATES
in a structure at about the L3 level that simulated a rudimentary kidney and was connected to the vena cava by a thin venous pedicle. The whole structure from its cephalic end to the caudal end was excised (fig. 2). The preoperative diagnosis of ectopic ureter was entertained until the final pathology report arrived.
Fm. 1. Preoperative right seminal vesiculogram
The specimen was composed of the tubal structure some 25 cm. in length and 0.5 cm. diameter and the rudimentary mass. measuring 3 by 0.8 by 0.3 cm. Histologically the tubal structure was comprised of cubic or columnar epithelia, 3 distinct muscular , layers and an outer fibrous element (fig. 3). The rudimentary structure contained glandular elements that were lined by tall columnar epithelia, with stereocilia and stroma that showed moderate inflammatory cellular infiltration. In no section were renal or testicular elements present (fig. 4). Subsequently, we concluded that the specimen was the vas deferens and epididymis. Convalescence was satisfactory. Because of the pathology report, ipsilateral testicular biopsy and bilateral vasograms were done to evaluate the gonadal anomaly and the result of operation. Biopsy showed germinal cell aplasia. The vasogram demonstrated the persistence of the ectatic change of the seminal vesicle and residuum of seminal vesiculitis. We also noted that the duplicated vas deferens was completely excised and the left vas and seminal vesicle were normal (fig. 5). Interestingly enough disappearance of albuminuria was confirmed on 2 occasions postoperatively. The blood pressure stabilized in the normotensive range and the peripheral renin activity was normalized (3.0 and 3.(i ng. per cc). However, serum uric acid remained elevated.
I.V,C. Aorta
Rudimentary Kidney (Epididymis) Ectopic Ureter ? - - - - - · (pouble Vas Deferens)
Vas Deferetis Proper
Seminal Vesicle
Fm. 2. Schematic illustration of operative finding
DOUBLE VAS DEFERENS ASSOCIATED WITH IPSILATERAL RENAL AGENESIS
Fm. 3. Microscopic appearance of ductal structure which we concluded was vas deferens. DISCUSSIOX
There are various embryological explanations for agenesis of the kidney. Campbell stressed failure of the vascular supply and resulting renal ischemia as its cause. 8 Potter 9 and others10 • 11 generally believed that failure of ureteral bud formation from the lower end of the mesonephric duct leads to the undifferentiation of the renal parenchyma. Potter states, "A kidney is never formed in the absence of ureter". Ashley and Mostofi found 10 cases of renal dysgenesis in which there was complete absence of the ureters. 3 They suggested that potentiality for renal differentiation rests in the structure of the nephrogenic ridge itself. The pronephros and mesonephros form their tubules and ducts in response to organizers as yet unknown. The stimulus for the outgrowth of the ureteral bud from the lower end of the mesonephric duct arises in the metanephric blastema at 8 Campbell, M. F.: Urology. Philadelphia: W. B. Saunders Co., vol. 1, 1954. 9 Potter, E. L.: Pathology of the Fetus and the Newborn. Chicago: Year Book Medical Publishers, 1952. 10 Radasch, H. E.: Congenital unilateral absence of the urogenital system and its relation to the development of the wolffian and milllerian ducts. Amer. J. Med. Sci., 136: 111, 1908. 11 Allen, A. C.: The Kidney: Medical and Surgical Diseases. New York: Grune & Stratton, 1951.
633
the lower end of the nephrogenic ridge, while the metanephric blastema has within itself the potentiality for differentiation into a kidney albeit one without the drainage necessary for function. For the full development of the kidney this entire mechanism must be intact. Ashley and Mostofi classified renal defects into 2 major groups: 1) those in which the nephrogenic ridge fails to show any potentiality of organogenesis and 2) those in which the nephrogenic ridge fails to show potentiality for renal differentiation after the formation of pronephric and mesonephric ducts. The latter group is subdivided into 5 additional types. Ashley and Mostofi believe that all anomalies of the renal tract are most easily explained by this hypothesis. The present case is explained to some extent by the type 2a anomaly proposed by Ashley and Mostofi, in which the metanephric blastema fails to develop and induction of the formation of ureteral bud is absent, while the wolffian duct is present. N evertheless, the duplication of vas deferens and epididymis requires additional explanations. In the English literature only Mathe and Dunn have reported a similar case, although their case did not include double epididymis. They thought that the double vas developed from the double wolffian duct. One duct united normally with the primitive testis and descended into the scrotum. Its caudal end terminated in the ejaculatory duct in the posterior urethra. The second vas remained unattached to the testis and retained its primitive anatomical relationship extending from the renal fossa to the point where it entered the seminal vesicle. Whether the division of the wolffian duct is related to the factor responsible for the failure of the ureteral bud to form from the lower end of mesonephric duct is a matter for speculation. The fifth or sixth week of intrauterine life, at which time the metanephros, ureteral bud and mesonephric duct are developing, is also a critical period for other systems. The effect of any damage to the fetus at this time can easily be appreciated by the frequency of associated anomalies. The present case demonstrates this association of anomalies. There were germinal cell aplasia of the ipsilateral testis, absence of the second left rib, spina bifida occulta and slight mental retardation in addition to the genitourinary anomalies discussed herein. Some agent or agents must have worked on the germinal ridge (nephrogenic and genital ridge), inducing ipsilateral renal agenesis, double vas deferens and germinal cell aplasia of the testis. This case is unique in that the roentgenographic appearance was hardly distinguishable from an ectopic ureter. Among previously reported cases of ectopic ureter in a male subject there must have been cases that were erroneously reported as such without careful histological examination. Our case i~ also unique in that there were the associated clinical features of hypertension, albuminuria and hyperuricemia. The postoperative disappearance of the hypertension and albuminuria is difficult to explain. The amount of proteinaceous material contained in
634
KOYANAGI AND ASSOCIATES
FIG. 4. A, histology of rudimentary structure. Note epididymal architecture and absence of any renal element. B, greater magnification of part A.Note stereocilia, characteristic of epididymal epithelia.
the specimen was too slight to account for the albuminuria. Whether focal nephritis secondary to the source of infection in the double vas and epididymis could explain the transient albuminuria and hypertension is a matter of conjecture. We regret that we did not perform the renal biopsy. The problem of hyperuricemia awaits further study. SUMMARY
FIG. 5. Postoperative bilateral seminal vesiculogram.
A rare anomaly of double vas deferens and epididymis associated with ipsilateral renal agenesis and germinal cell aplasia of the testis, simulating ectopic ureter was presented. Factors of as yet undetermined nature are thought to have caused a defect in the urogenital ridge at a critical time of fetal development. Extra-embryonic factors acting on the embryo at this crucial stage caused concurrent anomalies in other parts of the body. Our case was unique in that there was associated hypertension with increased peripheral renin activity and albuminuria which returned to normal postoperatively.