Vol. 115, June Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright© 1976 by The Williams & Wilkins Co.
CROSSED ECTOPIA OF SEMINAL VESICLES, RENAL APLASIA AND ECTOPIC URETER HANS-EBERHARD MELLIN, TORBEN B. KJAER
AND
PAUL 0. MADSEN*
From the Urology Section, Veterans Administration Hospital and the Department of Surgery, University of Wisconsin School of Medicine, Madison, Wisconsin
ABSTRACT
A unique case of left ureteral opening into a seminal vesicle, ipsilateral renal hyperplasia and crossed ectopia of the seminal vesicles is reported. This 24-year-old white man underwent a nephroureterectomy for relief of symptoms of lower urinary tract infection. The embryological development of this abnormality is discussed briefly. About 50 cases of renal aplasia in patients with an ectopic ureter emptying into the ipsilateral seminal vesicle have been reported in the literature. Herein we present an additional case report that is unique since the patient also had crossed ectopia of the seminal vesicles. CASE REPORT
J.C., a 24-year-old white man, was admitted to the hospital with a 1-year history of a mild suprapubic and left lower flank pain and dysuria. He had been treated for urinary tract infection, left epididymitis and seminal vesiculitis at another
Cystoscopy showed that the right ureteral orifice was normal in size and location. The left orifice was seen at the 6 o'clock position at the bladder neck. An attempt to visualize the left ureter by retrograde catheterization failed. The verumontanum was seen with a well defined ejaculatory duct orifice on the left side but none on the right side. A voiding cystourethrogram showed a normal bladder and urethra without ureteral reflux. Bilateral vasovesiculography revealed a normal vas deferens on the left side entering a deformed cystic seminal vesicle (part A of figure). The left ureter was filled via the cyst and showed distal dilatation and tapering off in its proximal part. The right vasovesiculography showed the right vas
A, Left vasovesiculography shows deformed cystic left seminal vesicle and reflux of contrast medium into left dilated ureter. B, right vasovesiculography shows right vas emptying into ectopic seminal vesicle on left side (crossed ectopia).
hospital, although urine cultures on 2 occasions were negative. Physical examination was completely negative. The scrotal contents were normal and non-tender. Repeated cultures were negative. Rectal examination was unremarkable. Excretory urography (IVP) demonstrated a slightly enlarged, normal functioning right kidney but no evidence of a left kidney and this was confirmed by renal arteriography, revealing no left renal artery. Accepted for publication November 7, 1975. * Requests for reprints: Veterans Administration Hospital, 2500 Overlook Terrace, Madison, Wisconsin 53705.
deferens crossing over to the left sidevand emptying into a radiologically normal appearing seminal vesicle located next to the other seminal vesicle. There was no reflux into the left ureter via this route (part B of figure). The ejaculate had a volume of only 0.5 ml. with a low sperm count of 114,000 sperm per cm. Eighty per cent of the sperm showed good motility and 98 per cent had a normal shape. The patient was explored and a left nephroureterectomy was performed without operative or postoperative complications. The left aplastic kidney measured 2 by 1.5 by 1 cm. and had no true renal parenchyma and only a small renal pelvis. Micro-
766
MELLIN, KJAER AND MADSEN
scopically, the kidney tissue consisted of dilated ducts and cysts lined by columnar epithelium and containing eosinophilic protein fluid with calcification. No glomeruli were seen. DISCUSSION
During the fifth and sixth weeks of fetal life the cloaca, into which the allantois and the wolffian ducts open, is divided by the urorectal septum into a dorsal rectal and a ventral urogenital segment. From this urogenital segment the ureteral buds, which finally form the urinary collecting system, originate. Thus, the ureter and wolffian duct become the vesicoureteral anlage. Later the ureteral orifice on each. side shifts upward to open into the lateral angle of the trigone. Failure to do so results in ureteral ectopy. The wolffian duct finally forms the posterior urethra, vas deferens, epididymis and seminal vesicles. 1 This early intimate neighborhood of ureteral buds and wolffian ducts explains the ureteral and seminal vesicle anomalies. In a review of the literature on ectopic ureters opening into a seminal vesicle, ipsilateral renal aplasia was found in all cases. 2• 3 However, a congenital uretero-seminal vesicle fistula• or a vaso-ureteral fistula 5 is not necessarily associated with renal aplasia. Thus, we believe this combined anomaly has to be regarded as an entity and not as a coincidence of renal aplasia and ectopic ureter. The clinical picture of this anomaly with drainage of the ureter into a seminal vesicle usually first presents as epididymitis, prostatitis or urinary tract infection with fever, dysuria, frequency, hematuria and suprapubic or flank pain. As in our patient, the urine cul·!;ure is not infrequently negative despite the symptoms. 6 In most cases rectal examination reveals a cystic mass above the prostate. IVP, cystoscopy and specifically vasography confirm the diagnosis. The therapy of choice appears to be nephroureterectomy and excision of the seminal vesicle cyst. In patients with large infected cysts, total seminal vesiculectomy may be necessary. In patients such as ours with small non-infected cysts draining well into the urinary tract, a nephroureterectomy appears to be sufficient. Our patient had crossed ectopia of the seminal vesicles on the left side. The right vas deferens emptied on the left side into 1 of the seminal vesicles. The 2 seminal vesicles located beside each other were of different shapes, with the deformed cystic one filling from the left side. Only 1 orifice was found in the prostatic urethra, presumably draining seminal fluid from both parts of this double seminal vesicle. We can find no embryological explanation in the literature for the ectopia of this seminal vesicle. Our patient, who had concomitant crossed ectopia of the seminal vesicles, appears to represent the first case of such a congenital abnormality to be reported in the literature.
REFERENCES 1. Campbell, M. F.: Embryology of the ureter. In: Urology, 3rd ed.
2. 3. 4. 5. 6.
Edited by M. F. Campbell and J. H. Harrison. Philadelphia: W. B. Saunders Co., vol. II, 1970. Riha, L. W., Schmidlapp, C. J. and Bosworth, N. L.: Ectopic ureter draining into the seminal vesicle. J. Urol., 56: 332, 1946. Allansmith, R.: Ectopic ureter terminating in seminal vesicle; unilateral polycystic kidney: report of a case and review of the literature. J. Urol., 80: 425, 1958. Mulholland, S. G., Edson, M. and O'Connell, K. J.: Congenital uretero-seminal vesicle fistula. J. Urol., 106: 649, 1971. Alfert, H.J. and Gillenwater, J. Y.: Ectopic vas deferens communicating with lower ureter: embryological considerations. J. Urol., 108: 172, 1972. Reddy, Y. N. and Winter, C. C.: Cyst of the seminal vesicle: a case report and review of the literature. J. Urol., 108: 134, 1972. COMMENT
The ureter develops as a bud from the mesonephric duct as it bends to join the urogenital sinus. The segment of the mesonephric duct caudad to the ureteral bud is known as the common nephric duct and it becomes gradually absorbed into the urogenital sinus, carrying the ureter with it. When the common nephric duct is completely absorbed, the ureter and the mesonephric duct achieve a separate opening into the urogenital sinus. The 2 orifices rotate around each other and the ureteral orifice ascends upward and laterally. The mesonephric duct will become the vas deferens and an outpouching from its caudal end will differentiate, after multiple convolutions, into a seminal vesicle. The most caudal segment of the mesonephric duct beyond the origin of the seminal vesicle will become the ejaculatory duct. It is postulated that if the ureteral bud arises high on the mesonephric duct the common nephric duct will be too long and might not be completely absorbed into the urogenital sinus. As a· result, the ureteral bud will remain attached to the most caudal end of the mesonephric duct, which later will be included in the formation of the seminal vesicle or become the ejaculatory duct. Most commonly, such an ectopic ureter will open into the ejaculatory duct and less commonly into the seminal vesicle. Ureteral buds having started high on the mesonephric duct will, as they grow, miss the center of the nephrogenic ridge and meet the latter at its peripheral cranial end. This peripheral nephrogenic ridge will usually fail to differentiate into proper kidney tissue and result in either renal agenesis or marked dysplasia-the rule with such ectopic ureters. I know of no embryologic explanation for crossed seminal vesicles, unless one would postulate fusion of lower ends of both mesonephric ducts (as normally happens with the miillerian ducts) but this is an unlikely hypothesis. It would have been interesting if the authors had catheterized the single ejaculatory duct seen and filled the genital duct system with contrast medium; thus, their exact anatomic interrelations could have been visualized roentgenographically. One should not rule out the possibility that the apparent crossed ectopic seminal vesicles represent a displacement by a mass rather than a genuine ectopy. Emil A. Tanagho University of California San Francisco, California
THE JOURNAL OF UROLOGY
Copyright© 1976 by The Williams & Wilkins Co.
CROSSED ECTOPIA OF SEMINAL VESICLES, RENAL APLASIA AND ECTOPIC URETER HANS-EBERHARD MELLIN, TORBEN B. KJAER
AND
PAUL 0. MADSEN*
From the Urology Section, Veterans Administration Hospital and the Department of Surgery, University of Wisconsin School of Medicine, Madison, Wisconsin
ABSTRACT
A unique case of left ureteral opening into a seminal vesicle, ipsilateral renal hyperplasia and crossed ectopia of the seminal vesicles is reported. This 24-year-old white man underwent a nephroureterectomy for relief of symptoms of lower urinary tract infection. The embryological development of this abnormality is discussed briefly. About 50 cases of renal aplasia in patients with an ectopic ureter emptying into the ipsilateral seminal vesicle have been reported in the literature. Herein we present an additional case report that is unique since the patient also had crossed ectopia of the seminal vesicles. CASE REPORT
J.C., a 24-year-old white man, was admitted to the hospital with a 1-year history of a mild suprapubic and left lower flank pain and dysuria. He had been treated for urinary tract infection, left epididymitis and seminal vesiculitis at another
Cystoscopy showed that the right ureteral orifice was normal in size and location. The left orifice was seen at the 6 o'clock position at the bladder neck. An attempt to visualize the left ureter by retrograde catheterization failed. The verumontanum was seen with a well defined ejaculatory duct orifice on the left side but none on the right side. A voiding cystourethrogram showed a normal bladder and urethra without ureteral reflux. Bilateral vasovesiculography revealed a normal vas deferens on the left side entering a deformed cystic seminal vesicle (part A of figure). The left ureter was filled via the cyst and showed distal dilatation and tapering off in its proximal part. The right vasovesiculography showed the right vas
A, Left vasovesiculography shows deformed cystic left seminal vesicle and reflux of contrast medium into left dilated ureter. B, right vasovesiculography shows right vas emptying into ectopic seminal vesicle on left side (crossed ectopia).
hospital, although urine cultures on 2 occasions were negative. Physical examination was completely negative. The scrotal contents were normal and non-tender. Repeated cultures were negative. Rectal examination was unremarkable. Excretory urography (IVP) demonstrated a slightly enlarged, normal functioning right kidney but no evidence of a left kidney and this was confirmed by renal arteriography, revealing no left renal artery. Accepted for publication November 7, 1975. * Requests for reprints: Veterans Administration Hospital, 2500 Overlook Terrace, Madison, Wisconsin 53705.
deferens crossing over to the left sidevand emptying into a radiologically normal appearing seminal vesicle located next to the other seminal vesicle. There was no reflux into the left ureter via this route (part B of figure). The ejaculate had a volume of only 0.5 ml. with a low sperm count of 114,000 sperm per cm. Eighty per cent of the sperm showed good motility and 98 per cent had a normal shape. The patient was explored and a left nephroureterectomy was performed without operative or postoperative complications. The left aplastic kidney measured 2 by 1.5 by 1 cm. and had no true renal parenchyma and only a small renal pelvis. Micro-
766
MELLIN, KJAER AND MADSEN
scopically, the kidney tissue consisted of dilated ducts and cysts lined by columnar epithelium and containing eosinophilic protein fluid with calcification. No glomeruli were seen. DISCUSSION
During the fifth and sixth weeks of fetal life the cloaca, into which the allantois and the wolffian ducts open, is divided by the urorectal septum into a dorsal rectal and a ventral urogenital segment. From this urogenital segment the ureteral buds, which finally form the urinary collecting system, originate. Thus, the ureter and wolffian duct become the vesicoureteral anlage. Later the ureteral orifice on each. side shifts upward to open into the lateral angle of the trigone. Failure to do so results in ureteral ectopy. The wolffian duct finally forms the posterior urethra, vas deferens, epididymis and seminal vesicles. 1 This early intimate neighborhood of ureteral buds and wolffian ducts explains the ureteral and seminal vesicle anomalies. In a review of the literature on ectopic ureters opening into a seminal vesicle, ipsilateral renal aplasia was found in all cases. 2• 3 However, a congenital uretero-seminal vesicle fistula• or a vaso-ureteral fistula 5 is not necessarily associated with renal aplasia. Thus, we believe this combined anomaly has to be regarded as an entity and not as a coincidence of renal aplasia and ectopic ureter. The clinical picture of this anomaly with drainage of the ureter into a seminal vesicle usually first presents as epididymitis, prostatitis or urinary tract infection with fever, dysuria, frequency, hematuria and suprapubic or flank pain. As in our patient, the urine cul·!;ure is not infrequently negative despite the symptoms. 6 In most cases rectal examination reveals a cystic mass above the prostate. IVP, cystoscopy and specifically vasography confirm the diagnosis. The therapy of choice appears to be nephroureterectomy and excision of the seminal vesicle cyst. In patients with large infected cysts, total seminal vesiculectomy may be necessary. In patients such as ours with small non-infected cysts draining well into the urinary tract, a nephroureterectomy appears to be sufficient. Our patient had crossed ectopia of the seminal vesicles on the left side. The right vas deferens emptied on the left side into 1 of the seminal vesicles. The 2 seminal vesicles located beside each other were of different shapes, with the deformed cystic one filling from the left side. Only 1 orifice was found in the prostatic urethra, presumably draining seminal fluid from both parts of this double seminal vesicle. We can find no embryological explanation in the literature for the ectopia of this seminal vesicle. Our patient, who had concomitant crossed ectopia of the seminal vesicles, appears to represent the first case of such a congenital abnormality to be reported in the literature.
REFERENCES 1. Campbell, M. F.: Embryology of the ureter. In: Urology, 3rd ed.
2. 3. 4. 5. 6.
Edited by M. F. Campbell and J. H. Harrison. Philadelphia: W. B. Saunders Co., vol. II, 1970. Riha, L. W., Schmidlapp, C. J. and Bosworth, N. L.: Ectopic ureter draining into the seminal vesicle. J. Urol., 56: 332, 1946. Allansmith, R.: Ectopic ureter terminating in seminal vesicle; unilateral polycystic kidney: report of a case and review of the literature. J. Urol., 80: 425, 1958. Mulholland, S. G., Edson, M. and O'Connell, K. J.: Congenital uretero-seminal vesicle fistula. J. Urol., 106: 649, 1971. Alfert, H.J. and Gillenwater, J. Y.: Ectopic vas deferens communicating with lower ureter: embryological considerations. J. Urol., 108: 172, 1972. Reddy, Y. N. and Winter, C. C.: Cyst of the seminal vesicle: a case report and review of the literature. J. Urol., 108: 134, 1972. COMMENT
The ureter develops as a bud from the mesonephric duct as it bends to join the urogenital sinus. The segment of the mesonephric duct caudad to the ureteral bud is known as the common nephric duct and it becomes gradually absorbed into the urogenital sinus, carrying the ureter with it. When the common nephric duct is completely absorbed, the ureter and the mesonephric duct achieve a separate opening into the urogenital sinus. The 2 orifices rotate around each other and the ureteral orifice ascends upward and laterally. The mesonephric duct will become the vas deferens and an outpouching from its caudal end will differentiate, after multiple convolutions, into a seminal vesicle. The most caudal segment of the mesonephric duct beyond the origin of the seminal vesicle will become the ejaculatory duct. It is postulated that if the ureteral bud arises high on the mesonephric duct the common nephric duct will be too long and might not be completely absorbed into the urogenital sinus. As a· result, the ureteral bud will remain attached to the most caudal end of the mesonephric duct, which later will be included in the formation of the seminal vesicle or become the ejaculatory duct. Most commonly, such an ectopic ureter will open into the ejaculatory duct and less commonly into the seminal vesicle. Ureteral buds having started high on the mesonephric duct will, as they grow, miss the center of the nephrogenic ridge and meet the latter at its peripheral cranial end. This peripheral nephrogenic ridge will usually fail to differentiate into proper kidney tissue and result in either renal agenesis or marked dysplasia-the rule with such ectopic ureters. I know of no embryologic explanation for crossed seminal vesicles, unless one would postulate fusion of lower ends of both mesonephric ducts (as normally happens with the miillerian ducts) but this is an unlikely hypothesis. It would have been interesting if the authors had catheterized the single ejaculatory duct seen and filled the genital duct system with contrast medium; thus, their exact anatomic interrelations could have been visualized roentgenographically. One should not rule out the possibility that the apparent crossed ectopic seminal vesicles represent a displacement by a mass rather than a genuine ectopy. Emil A. Tanagho University of California San Francisco, California
