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Ureteral Ectopia: Report of 39 Cases
Vol. 109, February Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright © 1973 by The Williams & Wilkins Co.
URETERAL ECTOPIA: REPORT OF 39 CASES WILLIAM BRANNAN
AND
HECTOR H. HENRY, II
From the Department of Urology, Ochsner Clinic and Ochsner Foundation Hospital, New Orleans, Louisiana
Ureteral ectopia has been considered a rare anomaly, although increased awareness and improved diagnostic methods have made it less rare than in the past. In the last 27 years 39 patients with 44 ectopic ureters have been encountered at Ochsner Clinic and Tulane University. Of these, 13 cases or 18 ectopic ureters have been diagnosed during the past 5 years. Cox and Hutch 1 recently stated that more than 500 cases have been reported, 170 of them between 1934 and 1948. 2 Approximately 75 cases have been reported since April 1967. The first observation of ureteral ectopia was by Schroder in 167 4 and the first case diagnosed clinically was by Albarran in 1874. Of our 39 patients (44 ectopic ureters) 33 cases have been confirmed by operation; 6 patients refused an operation (table 1). One patient had 2 heminephrectomies. All patients operated upon have been cured. AGE AND SEX
All but 8 of our patients were women-a 5: 1 ratio, which is similar to other reported incidences. 3 Since ureteral ectopia is a congenital abnormality, the incidence in children is high. The average age of the 31 female subjects in this series was 12 years; 60 per cent were less than 10 years old. The average age of the 8 male subjects was 18 years and the youngest was 6 years old. In 494 cases compiled by Ellerker in 1958, 60 per cent of the patients were less than 20 years old and 35 per cent were less than 10 years old. 2 LOCATION
In 1958 Eller ker compiled the data regarding the incidence of extravesical ureteral orifice according to possible sites. 2 He failed to include the frequent location of the orifice within the bladder but between its normal position and the vesical neck or the infrequent locations in the rectum 4 or the fallopian tubes. 5 • 6 The general consensus is that Accepted for publication June 2, 1972. Presented at Section on Urology, Southern Medical Association, Dallas, Texas, November 17, 1970. 1 Cox, C. E. and Hutch, J. A.: Bilateral single ectopic ureter: a report of 2 cases and review of the literature. J. Ural., 95: 493, 1966. 2 Ellerker, A.G.: The .extrnvesical ectopic ureter. Brit. J. Surg., 45: 344, 1957-58. 'Moore, T. D.: The diagnosis and management of ureteral ectopia. J. Ural., 60: 50, 1948. 4 Leef, G. S. and Leader, S. A.: Ectopic ureter opening into the rectum: a case report. J. Ural., 87: 338, 1962. 5 Gunther, R. E., Harer, W. B., Jr. and Welebir, R.: Ectopic ureter presenting as a "tuba-ovarian abscess"; report of a case. Obst. Gynec., 25: 259, 1965. 'Srivastava, H. C.: Bilateral termination of ureter in the uterine tube. Indian J. Med. Sci., 22: 37, 1968.
an orifice not opening on the posterolateral aspect of the trigone is ectopic, which can account for the increase in cases during the past 10 years. There have been 23 reported cases of bilateral ectopia of non-duplicated ureters. 7 • 8 The ureter from the upper segment is almost always ectopic because of the embryological development of the wolffian duct, the so-called Weigart-Mayer rule. Only 6 exceptions to this rule have been reported. 7 Table 2 shows the sites of the ectopic ureter in our patients-20 on the left side and 14 on the right, an incidence similar to that of other series. In 4 female patients the ureteral ectopia was bilateral. We have observed 3 cases of ectopic ureter emptying into the seminal vesicle and found only 23 such cases reported since 1960. 8 Herein are summaries of our 3 cases. CASE REPORTS
Case 1. A 15-year-old white boy had had no urogenital complaints until June 1963 when he was kicked in the lower ·part of the abdomen while swimming. Two days later burning on urination, urgency and gross, total hematuria developed. A diagnosis of hemorrhagic cystitis was made and the patient was hospitalized elsewhere. Antibiotic therapy was begun but 2 weeks later hematuria was still present. There was no evidence of a left kidney on an excretory urogram (IVP). Inflammation in the area of the left orifice was noticed on cystoscopy but no orifice was identified. The right orifice was reported to be normal. The patient was admitted to the Ochsner Clinic because of persistent hematuria. Physical examination revealed that the left lower quadrant of the abdomen was slightly tender. The genitalia were normal. On rectal examination a 3 by 4 cm. cystic lesion was palpated above the prostate in the region of the left seminal vesicle. The prostate was normal. Urinalysis revealed numerous red and 4 to 6 white blood cells. Urine culture was negative. IVP revealed non-function on the left side and compensatory hypertrophy of the right kidney. There was some elevation of the floor of the left side of the bladder. Cystography revealed normal contour of the bladder and no reflux. However, no left ureteral orifice was found and an area corresponding to the left aspect of the trigone was elevated. A left ureterocele was suspected but no orifice was visible. An enlarged, tortuous ampulla 7 Burford, C. E., Glenn, J. E. and Burford, E. H.: Ureteral ectopia: a review of the literature and 2 case reports. J. Ural., 62: 211, 1949. 8 Schnitzer, B.: Ectopic ureteral opening into seminal vesicle: a report of four cases. J. Urol., 93: 576, 1965. 192
193
URETERAL ECTOPIA TABLE
1. Surgical procedures in 33 of 39 patients No. Pts.
N ephrectomy N ephroureterectomy Heminephrectomy Heminephroureterectomy Wedge resection of atrophic segment Transurethral meatotomy with conversion of extravesical orifice to intravesical orifice Ureterectomy
1 5 1 23 1
1 2 34*
Total
* One patient had 2 heminephrectomies.
TABLE
2. Location of ectopic ureters in 39 patients No. Ureters(%)
Female patients Urethra Vestibule Vagina Apex oftrigone Bladder neck Total
12 (33.3) 5 (13.9) 6 (16.1) 3 (8.3) 10 (27.8) 36
Male patients Prostatic urethra Seminal vesicles Total
5 (62.3)
3 (37.5) 8
of the left vas and cystic dilatation of the seminal vesicle with extension across the midline were seen in the vasogram of the left side. There was reflux of the opaque medium into a dilated left ureter. No pelvis was visualized. Percutaneous retrograde aortography revealed a large right renal artery but none was seen on the left side. At exploration the left seminal vesicle, ten ureter and a thread-like attachment near the left kidney were removed. The pathologist reported a small, normally functioning rudimentary kidney. The patient has remained asymptomatic since operation. Case 2. * A 33-year-old white physician sought advice because of infertility. As a child he had had a bloody urethral discharge during defecation. When he became sexually mature, he noticed that nocturnal emissions were bloody. When he was in the armed services, examinations because of low back pain revealed an enlarged prostate gland. An IVP in 1946 revealed no function on the right side and compensatory hypertrophy of the left kidney. On cystoscopy no right ureteral orifice was found. Because of recurrent symptoms the patient saw 2 other urologists 10 years later who concluded that he had an absent right kidney and compensatory hypertrophy on the left side. When first examined he was found to have oligospermia and small testicles. Two years later he returned with prostatitis. On rectal examination nothing abnormal was found. The patient * Courtesy of Dr. Jack Daniel, Hattiesburg, Mississippi.
returned 1 week later complaining of a bloody ejaculate. A roentgenogram of the kidney, ureter and bladder revealed what appeared to be an atrophic kidney on the right side and a hypertrophied one on the left. A normal left kidney and ureter were demonstrated by IVP. A right seminal vesiculogram disclosed a dilated right seminal vesicle and reflux of the opaque medium into a dilated right ureter. On cystoscopy the bladder, left ureteral orifice, vesical neck and prostatic urethra were normal. The right half of the trigone and the right ureteral orifice were absent. At exploration an atrophic right kidney and dilated tortuous ureter were removed. The ureter was transected at its entrance into the seminal vesicle. The patient has been asymptomatic since the operation. Case 3. An 18-year-old white boy from Central America was examined because of recurrent episodes of epididymitis. He had been treated by another urologist for this condition for the previous 3 or 4 years. He had no symptoms of lower tract obstruction. An IVP showed a hypertrophied right kidney with no function on the left side. An excretory cystogram showed a filling defect in the bladder. On rectal examination a mass was found which was fluctuant but distinct from the prostate and thought to be separate from the seminal vesicle. Urinalysis and prostatic secretions were negative. At cystoscopy there was absence of the left hemitrigone and orifice, with no evidence of intravesical mass, stone or other abnormality. The patient had a patulous appearing ejaculatory duct. A ureteral catheter was introduced and 300 cc turbid, brownish material was withdrawn. Injection of contrast medium into the catheter showed a large cystic mass, probably the left seminal vesicle. A left seminal vesicogram demonstrated reflux of contrast medium up the left ureter. A percutaneous retrograde arteriogram did not show a left renal artery. Left renal exploration was performed through a classical flank incision with anterior extension. The ureter was traced caudally to the left seminal vesicular attachment and the left ureter, left seminal vesicle and ampulla were removed. Convalescence was uneventful and the patient has remained asymptomatic without medication. SYMPTOMS
Female patients. The classical symptom of ureteral ectopia in female subjects is urinary incontinence, noctural and diurnal, although the voiding pattern is normal. Fifteen of our 31 female patients had urinary incontinence (48.3 per cent) but this symptom could not be evaluated in 6 infants. Patients are incontinent because the ectopic orifice is located distal to the urethral sphincter mechanism. If the orifice is proximal to the sphincter, the patient will be continent. Cases of vestibular ectopia without incontinence owing to a
194
BRANNAN AND HENRY
stenotic ectopic orifice have been reported. 9 • 10 The orifice may enter a urethral diverticulum. 3 It seems reasonable that the urethral sphincter may be adequate at night during recumbency but not during the day, accounting for diurnal incontinence. 11 Because an ectopic ureter often opens into a contaminated field, urinary infection is common. Infection may be manifested by chills, fever, flank pain and irritative urinary symptoms. Eighteen of our 31 female patients had urinary infections. Flank pain or pyuria may be the only complaint if the ureteral ectopia is within the internal vesical orifice, as was the case in one of our patients. Persistent watery or purulent vaginal discharge may be the only presenting complaint. Three of 4 patients with vaginal ureteral ectopia and 1 with urethral ureteral ectopia complained of vaginal discharge. Male patients. Because wolffian duct derivatives are all proximal to the external urinary sphincter, urinary incontinence is never a manifestation of this anomaly in male patients. Instead, the complaints are those of recurrent pyuria, with or without symptoms of obstruction and chronic infection, on the abnormal side. 12 Since the most common sites of ureteral ectopia in male patients are the prostatic urethra and the seminal vesicle, symptoms of lower urinary infection (prostatitis, urethritis and seminal vesiculitis) are common. Six of our 8 male patients had such symptoms. Gross hematuria was present in 1 patient and a bloody ejaculate in another. Two patients were asymptomatic, the anomaly having been discovered on routine examination. DIAGNOSIS
Excretory urography. IVP is indicated in all patients with persistent urinary incontinence, persistent urinary infections or fever of unknown origin. Complete duplication of the collecting system was seen in 20 of our 29 female patients on IVP, an incidence similar to that of other series. 7 Another had a duplicated ureter opening into the urethra and ending blindly at the renal hilus. Duplication was present in 4 of the 8 male patients. Goldstein and Heller also reported that duplication is not as common in male as in female patients. 12 The incidence in both sexes is 55 per cent. On careful examination of the IVP a hypoplastic, poorly functioning upper segment may be seen, as in 8 of our patients, or the upper segment may be dilated, as in 12 of our patients. An 'DeWeerd, J. H. and Litin, R. B.: Ectopia ofureteral orifice (vestibular) with incontinence: report of a case. Proc. Staff Meet. Mayo Clin., 33: 81, 1958. 10 Stuckey, E. S.: Ectopic ureter. Med. J. Australia, 2: 688, 1957. 11 Crenshaw, J. L.: Ureter with extravesical orifice; supernumerary ureter ending blindly; crossed ureteral ectopia; stones in extrapelvic cystocele: report of eleven cases. J. Urol., 43: 82, 1940. 12 Goldstein, A. E. and Heller, E.: Ectopic ureter opening into a seminal vesicle. J. Urol., 75: 57, 1956.
abnormal renal mass, as compared with the collecting system, was seen 9 times and displacement of the renal mass was present 7 times. Four of our 8 male patients had no renal function on the affected side. Delayed films are of utmost importance in showing a faint trace of dye in a poorly functioning segment. Voiding cystourethrography. Voiding cystourethrography, although not used in all of our early cases, was done on 18 female patients. Reflux into the ectopic ureter occurred in 9 patients. In 3 patients reflux was demonstrated in the normally placed ureter. In 2 other female subjects the catheter inserted to perform cystography inadvertently entered the ectopic orifice in the urethra. Voiding cystourethrography was performed in 5 of our male patients and 2 had reflux in the ectopic orifice. Careful examination. Meticulous search for the ectopic orifice is equally important in the diagnosis. Careful examination of the vestibule, appearance of indigo carmine in the vagina and bimanual compression over each kidney to express pus from a dilated ureter are important. Pus may be expressed from a cystic mass in the vagina. The simple maneuver of having an older female patient bear down while in the lithotomy position may cause an otherwise obscure ectopic orifice to spurt urine. Cystoscopy and urethroscopy. Cystoscopy and urethroscopy are mandatory to make the diagnosis of ureteral ectopia. Bimanual examination should again be done while the urethra is being observed. In male patients the examiner should compress the prostate and seminal vesicles while observing the posterior urethra. In male and female patients a globular extravesical mass may be seen in the region of the trigone, representing the greatly dilated lower ureter. This mass is often mistaken for a primary ureterocele. In 2 of our patients the mass was large enough to cause partial obstruction of the vesical neck. One patient had a large rectal mass. Absence of a ureteral orifice, especially with evidence of a renal shadow on the ipsilateral side, should make one suspect ectopia. Seminal vesiculography. Seminal vesiculography should be performed in male patients if the orifice cannot be found otherwise. In all of our cases of ectopia in the seminal vesicle, reflux into the ectopic orifice was demonstrated in this way. Renal arteriography demonstrated a poorly functioning segment with an ectopia in 1 case. Surgical exploration. Finally, in the presence of urinary incontinence or persistent vaginal discharge and a combination of 2 or more of the foregoing radiologic or physical signs, one should not hesitate to explore the suspected kidney to establish the diagnosis definitely and to correct the condition at that time. In 3 of our patients the exact location of the ectopic orifice was not found until the ureter was traced to its termination at exploration and in 1 patient the exact location was never found.
U:=tETERAL ECTOP1A TREATMENT
The method of treatment depends on the extent of damage of renal tissue affected by the diseased portion. Heminephrectomy is usually the procedure of choice if the upper segment is ectopic on one or both sides. Complete ureterectomy in female patients is usually unnecessary because the orifice is rarely completely obstructed and will drain well unless reflux is present. Also, injury to the external urinary sphincter is possible if the dissection is carried down too far. In male patients complete ureterectomy is more easily and safely accomplished. Nephrectomy is usually the procedure of choice for ectopia of an unduplicated ureter, unless a paucity of functioning total renal tissue makes conservation of all renal tissue necessary. Nephrectomy should also be done if the blood supply to the remaining segment might be compromised by heminephrectomy unless ureteroureterostomy can be accomplished. Ligation of the involved ureter, ureteropyelostomy 13 or ureteroneocystostomy 14 may be of value in selected cases. The types of operations that were performed on our patients are shown in table 1. Two patients had only ureterectomy of the blind-ending ureter. One patient was treated converting an ectopic orifice just distal to the vesical neck to an intravesical one by meatotomy. It is noteworthy that of the 16 patients seen from 1959 to 1968, 9 had hemine-
1' de la Pena, A. and Oliveros, M.: Ureterovaginal ectopia: ureteropyelostomy. J. Urol., 83: 30, 1960. 14 Sandegard, E.: The treatment of ureteral ectopia. Acta Chir. Scand., H5: 149, 1958.
and 2 were found to have ureters without an identifiable renal mass. All of the 33 patients operated upon have been followed. They are all asymptomatic and satisfied with the result. Only 2 of these patients have experienced episodes of infected urine and, in both instances, it has been easily controlled with antibiotics. Of the 6 patients who did not undergo operation 2 were advised to have heminephrectomy but refused, 1 was treated for hypertension only, 1 was treated for infection and underwent urethral dilations, 1 refused any treatment or further diagnostic study and 1 had a transurethral resection for prostatism but refused any other treatment for the refluxing, ectopic ureter. SUMMARY
Ureteral ectopia is an anomaly that can be corrected if recognized. Of the 39 cases (44 ureters) that have been described 8 were male patients, 3 of whom had ureteral ectopia into the seminal vesicle. These 3 cases have been reported in detail. The symptoms in female patients are usually urinary incontinence, infection, vaginal discharge or flank pain; in male patients, recurrent pyuria, prostatitis, urethritis or seminal vesiculitis. The most useful diagnostic tools are IVP, voiding cystourethrography, careful examination, cystoscopy, urethroscopy and seminal vesiculography. Heminephrectomy is the treatment of choice in most cases occurring in a duplicated kidney. Excellent results were obtained in all 33 patients treated surgically.
THE JOURNAL OF UROLOGY
Copyright © 1973 by The Williams & Wilkins Co.
URETERAL ECTOPIA: REPORT OF 39 CASES WILLIAM BRANNAN
AND
HECTOR H. HENRY, II
From the Department of Urology, Ochsner Clinic and Ochsner Foundation Hospital, New Orleans, Louisiana
Ureteral ectopia has been considered a rare anomaly, although increased awareness and improved diagnostic methods have made it less rare than in the past. In the last 27 years 39 patients with 44 ectopic ureters have been encountered at Ochsner Clinic and Tulane University. Of these, 13 cases or 18 ectopic ureters have been diagnosed during the past 5 years. Cox and Hutch 1 recently stated that more than 500 cases have been reported, 170 of them between 1934 and 1948. 2 Approximately 75 cases have been reported since April 1967. The first observation of ureteral ectopia was by Schroder in 167 4 and the first case diagnosed clinically was by Albarran in 1874. Of our 39 patients (44 ectopic ureters) 33 cases have been confirmed by operation; 6 patients refused an operation (table 1). One patient had 2 heminephrectomies. All patients operated upon have been cured. AGE AND SEX
All but 8 of our patients were women-a 5: 1 ratio, which is similar to other reported incidences. 3 Since ureteral ectopia is a congenital abnormality, the incidence in children is high. The average age of the 31 female subjects in this series was 12 years; 60 per cent were less than 10 years old. The average age of the 8 male subjects was 18 years and the youngest was 6 years old. In 494 cases compiled by Ellerker in 1958, 60 per cent of the patients were less than 20 years old and 35 per cent were less than 10 years old. 2 LOCATION
In 1958 Eller ker compiled the data regarding the incidence of extravesical ureteral orifice according to possible sites. 2 He failed to include the frequent location of the orifice within the bladder but between its normal position and the vesical neck or the infrequent locations in the rectum 4 or the fallopian tubes. 5 • 6 The general consensus is that Accepted for publication June 2, 1972. Presented at Section on Urology, Southern Medical Association, Dallas, Texas, November 17, 1970. 1 Cox, C. E. and Hutch, J. A.: Bilateral single ectopic ureter: a report of 2 cases and review of the literature. J. Ural., 95: 493, 1966. 2 Ellerker, A.G.: The .extrnvesical ectopic ureter. Brit. J. Surg., 45: 344, 1957-58. 'Moore, T. D.: The diagnosis and management of ureteral ectopia. J. Ural., 60: 50, 1948. 4 Leef, G. S. and Leader, S. A.: Ectopic ureter opening into the rectum: a case report. J. Ural., 87: 338, 1962. 5 Gunther, R. E., Harer, W. B., Jr. and Welebir, R.: Ectopic ureter presenting as a "tuba-ovarian abscess"; report of a case. Obst. Gynec., 25: 259, 1965. 'Srivastava, H. C.: Bilateral termination of ureter in the uterine tube. Indian J. Med. Sci., 22: 37, 1968.
an orifice not opening on the posterolateral aspect of the trigone is ectopic, which can account for the increase in cases during the past 10 years. There have been 23 reported cases of bilateral ectopia of non-duplicated ureters. 7 • 8 The ureter from the upper segment is almost always ectopic because of the embryological development of the wolffian duct, the so-called Weigart-Mayer rule. Only 6 exceptions to this rule have been reported. 7 Table 2 shows the sites of the ectopic ureter in our patients-20 on the left side and 14 on the right, an incidence similar to that of other series. In 4 female patients the ureteral ectopia was bilateral. We have observed 3 cases of ectopic ureter emptying into the seminal vesicle and found only 23 such cases reported since 1960. 8 Herein are summaries of our 3 cases. CASE REPORTS
Case 1. A 15-year-old white boy had had no urogenital complaints until June 1963 when he was kicked in the lower ·part of the abdomen while swimming. Two days later burning on urination, urgency and gross, total hematuria developed. A diagnosis of hemorrhagic cystitis was made and the patient was hospitalized elsewhere. Antibiotic therapy was begun but 2 weeks later hematuria was still present. There was no evidence of a left kidney on an excretory urogram (IVP). Inflammation in the area of the left orifice was noticed on cystoscopy but no orifice was identified. The right orifice was reported to be normal. The patient was admitted to the Ochsner Clinic because of persistent hematuria. Physical examination revealed that the left lower quadrant of the abdomen was slightly tender. The genitalia were normal. On rectal examination a 3 by 4 cm. cystic lesion was palpated above the prostate in the region of the left seminal vesicle. The prostate was normal. Urinalysis revealed numerous red and 4 to 6 white blood cells. Urine culture was negative. IVP revealed non-function on the left side and compensatory hypertrophy of the right kidney. There was some elevation of the floor of the left side of the bladder. Cystography revealed normal contour of the bladder and no reflux. However, no left ureteral orifice was found and an area corresponding to the left aspect of the trigone was elevated. A left ureterocele was suspected but no orifice was visible. An enlarged, tortuous ampulla 7 Burford, C. E., Glenn, J. E. and Burford, E. H.: Ureteral ectopia: a review of the literature and 2 case reports. J. Ural., 62: 211, 1949. 8 Schnitzer, B.: Ectopic ureteral opening into seminal vesicle: a report of four cases. J. Urol., 93: 576, 1965. 192
193
URETERAL ECTOPIA TABLE
1. Surgical procedures in 33 of 39 patients No. Pts.
N ephrectomy N ephroureterectomy Heminephrectomy Heminephroureterectomy Wedge resection of atrophic segment Transurethral meatotomy with conversion of extravesical orifice to intravesical orifice Ureterectomy
1 5 1 23 1
1 2 34*
Total
* One patient had 2 heminephrectomies.
TABLE
2. Location of ectopic ureters in 39 patients No. Ureters(%)
Female patients Urethra Vestibule Vagina Apex oftrigone Bladder neck Total
12 (33.3) 5 (13.9) 6 (16.1) 3 (8.3) 10 (27.8) 36
Male patients Prostatic urethra Seminal vesicles Total
5 (62.3)
3 (37.5) 8
of the left vas and cystic dilatation of the seminal vesicle with extension across the midline were seen in the vasogram of the left side. There was reflux of the opaque medium into a dilated left ureter. No pelvis was visualized. Percutaneous retrograde aortography revealed a large right renal artery but none was seen on the left side. At exploration the left seminal vesicle, ten ureter and a thread-like attachment near the left kidney were removed. The pathologist reported a small, normally functioning rudimentary kidney. The patient has remained asymptomatic since operation. Case 2. * A 33-year-old white physician sought advice because of infertility. As a child he had had a bloody urethral discharge during defecation. When he became sexually mature, he noticed that nocturnal emissions were bloody. When he was in the armed services, examinations because of low back pain revealed an enlarged prostate gland. An IVP in 1946 revealed no function on the right side and compensatory hypertrophy of the left kidney. On cystoscopy no right ureteral orifice was found. Because of recurrent symptoms the patient saw 2 other urologists 10 years later who concluded that he had an absent right kidney and compensatory hypertrophy on the left side. When first examined he was found to have oligospermia and small testicles. Two years later he returned with prostatitis. On rectal examination nothing abnormal was found. The patient * Courtesy of Dr. Jack Daniel, Hattiesburg, Mississippi.
returned 1 week later complaining of a bloody ejaculate. A roentgenogram of the kidney, ureter and bladder revealed what appeared to be an atrophic kidney on the right side and a hypertrophied one on the left. A normal left kidney and ureter were demonstrated by IVP. A right seminal vesiculogram disclosed a dilated right seminal vesicle and reflux of the opaque medium into a dilated right ureter. On cystoscopy the bladder, left ureteral orifice, vesical neck and prostatic urethra were normal. The right half of the trigone and the right ureteral orifice were absent. At exploration an atrophic right kidney and dilated tortuous ureter were removed. The ureter was transected at its entrance into the seminal vesicle. The patient has been asymptomatic since the operation. Case 3. An 18-year-old white boy from Central America was examined because of recurrent episodes of epididymitis. He had been treated by another urologist for this condition for the previous 3 or 4 years. He had no symptoms of lower tract obstruction. An IVP showed a hypertrophied right kidney with no function on the left side. An excretory cystogram showed a filling defect in the bladder. On rectal examination a mass was found which was fluctuant but distinct from the prostate and thought to be separate from the seminal vesicle. Urinalysis and prostatic secretions were negative. At cystoscopy there was absence of the left hemitrigone and orifice, with no evidence of intravesical mass, stone or other abnormality. The patient had a patulous appearing ejaculatory duct. A ureteral catheter was introduced and 300 cc turbid, brownish material was withdrawn. Injection of contrast medium into the catheter showed a large cystic mass, probably the left seminal vesicle. A left seminal vesicogram demonstrated reflux of contrast medium up the left ureter. A percutaneous retrograde arteriogram did not show a left renal artery. Left renal exploration was performed through a classical flank incision with anterior extension. The ureter was traced caudally to the left seminal vesicular attachment and the left ureter, left seminal vesicle and ampulla were removed. Convalescence was uneventful and the patient has remained asymptomatic without medication. SYMPTOMS
Female patients. The classical symptom of ureteral ectopia in female subjects is urinary incontinence, noctural and diurnal, although the voiding pattern is normal. Fifteen of our 31 female patients had urinary incontinence (48.3 per cent) but this symptom could not be evaluated in 6 infants. Patients are incontinent because the ectopic orifice is located distal to the urethral sphincter mechanism. If the orifice is proximal to the sphincter, the patient will be continent. Cases of vestibular ectopia without incontinence owing to a
194
BRANNAN AND HENRY
stenotic ectopic orifice have been reported. 9 • 10 The orifice may enter a urethral diverticulum. 3 It seems reasonable that the urethral sphincter may be adequate at night during recumbency but not during the day, accounting for diurnal incontinence. 11 Because an ectopic ureter often opens into a contaminated field, urinary infection is common. Infection may be manifested by chills, fever, flank pain and irritative urinary symptoms. Eighteen of our 31 female patients had urinary infections. Flank pain or pyuria may be the only complaint if the ureteral ectopia is within the internal vesical orifice, as was the case in one of our patients. Persistent watery or purulent vaginal discharge may be the only presenting complaint. Three of 4 patients with vaginal ureteral ectopia and 1 with urethral ureteral ectopia complained of vaginal discharge. Male patients. Because wolffian duct derivatives are all proximal to the external urinary sphincter, urinary incontinence is never a manifestation of this anomaly in male patients. Instead, the complaints are those of recurrent pyuria, with or without symptoms of obstruction and chronic infection, on the abnormal side. 12 Since the most common sites of ureteral ectopia in male patients are the prostatic urethra and the seminal vesicle, symptoms of lower urinary infection (prostatitis, urethritis and seminal vesiculitis) are common. Six of our 8 male patients had such symptoms. Gross hematuria was present in 1 patient and a bloody ejaculate in another. Two patients were asymptomatic, the anomaly having been discovered on routine examination. DIAGNOSIS
Excretory urography. IVP is indicated in all patients with persistent urinary incontinence, persistent urinary infections or fever of unknown origin. Complete duplication of the collecting system was seen in 20 of our 29 female patients on IVP, an incidence similar to that of other series. 7 Another had a duplicated ureter opening into the urethra and ending blindly at the renal hilus. Duplication was present in 4 of the 8 male patients. Goldstein and Heller also reported that duplication is not as common in male as in female patients. 12 The incidence in both sexes is 55 per cent. On careful examination of the IVP a hypoplastic, poorly functioning upper segment may be seen, as in 8 of our patients, or the upper segment may be dilated, as in 12 of our patients. An 'DeWeerd, J. H. and Litin, R. B.: Ectopia ofureteral orifice (vestibular) with incontinence: report of a case. Proc. Staff Meet. Mayo Clin., 33: 81, 1958. 10 Stuckey, E. S.: Ectopic ureter. Med. J. Australia, 2: 688, 1957. 11 Crenshaw, J. L.: Ureter with extravesical orifice; supernumerary ureter ending blindly; crossed ureteral ectopia; stones in extrapelvic cystocele: report of eleven cases. J. Urol., 43: 82, 1940. 12 Goldstein, A. E. and Heller, E.: Ectopic ureter opening into a seminal vesicle. J. Urol., 75: 57, 1956.
abnormal renal mass, as compared with the collecting system, was seen 9 times and displacement of the renal mass was present 7 times. Four of our 8 male patients had no renal function on the affected side. Delayed films are of utmost importance in showing a faint trace of dye in a poorly functioning segment. Voiding cystourethrography. Voiding cystourethrography, although not used in all of our early cases, was done on 18 female patients. Reflux into the ectopic ureter occurred in 9 patients. In 3 patients reflux was demonstrated in the normally placed ureter. In 2 other female subjects the catheter inserted to perform cystography inadvertently entered the ectopic orifice in the urethra. Voiding cystourethrography was performed in 5 of our male patients and 2 had reflux in the ectopic orifice. Careful examination. Meticulous search for the ectopic orifice is equally important in the diagnosis. Careful examination of the vestibule, appearance of indigo carmine in the vagina and bimanual compression over each kidney to express pus from a dilated ureter are important. Pus may be expressed from a cystic mass in the vagina. The simple maneuver of having an older female patient bear down while in the lithotomy position may cause an otherwise obscure ectopic orifice to spurt urine. Cystoscopy and urethroscopy. Cystoscopy and urethroscopy are mandatory to make the diagnosis of ureteral ectopia. Bimanual examination should again be done while the urethra is being observed. In male patients the examiner should compress the prostate and seminal vesicles while observing the posterior urethra. In male and female patients a globular extravesical mass may be seen in the region of the trigone, representing the greatly dilated lower ureter. This mass is often mistaken for a primary ureterocele. In 2 of our patients the mass was large enough to cause partial obstruction of the vesical neck. One patient had a large rectal mass. Absence of a ureteral orifice, especially with evidence of a renal shadow on the ipsilateral side, should make one suspect ectopia. Seminal vesiculography. Seminal vesiculography should be performed in male patients if the orifice cannot be found otherwise. In all of our cases of ectopia in the seminal vesicle, reflux into the ectopic orifice was demonstrated in this way. Renal arteriography demonstrated a poorly functioning segment with an ectopia in 1 case. Surgical exploration. Finally, in the presence of urinary incontinence or persistent vaginal discharge and a combination of 2 or more of the foregoing radiologic or physical signs, one should not hesitate to explore the suspected kidney to establish the diagnosis definitely and to correct the condition at that time. In 3 of our patients the exact location of the ectopic orifice was not found until the ureter was traced to its termination at exploration and in 1 patient the exact location was never found.
U:=tETERAL ECTOP1A TREATMENT
The method of treatment depends on the extent of damage of renal tissue affected by the diseased portion. Heminephrectomy is usually the procedure of choice if the upper segment is ectopic on one or both sides. Complete ureterectomy in female patients is usually unnecessary because the orifice is rarely completely obstructed and will drain well unless reflux is present. Also, injury to the external urinary sphincter is possible if the dissection is carried down too far. In male patients complete ureterectomy is more easily and safely accomplished. Nephrectomy is usually the procedure of choice for ectopia of an unduplicated ureter, unless a paucity of functioning total renal tissue makes conservation of all renal tissue necessary. Nephrectomy should also be done if the blood supply to the remaining segment might be compromised by heminephrectomy unless ureteroureterostomy can be accomplished. Ligation of the involved ureter, ureteropyelostomy 13 or ureteroneocystostomy 14 may be of value in selected cases. The types of operations that were performed on our patients are shown in table 1. Two patients had only ureterectomy of the blind-ending ureter. One patient was treated converting an ectopic orifice just distal to the vesical neck to an intravesical one by meatotomy. It is noteworthy that of the 16 patients seen from 1959 to 1968, 9 had hemine-
1' de la Pena, A. and Oliveros, M.: Ureterovaginal ectopia: ureteropyelostomy. J. Urol., 83: 30, 1960. 14 Sandegard, E.: The treatment of ureteral ectopia. Acta Chir. Scand., H5: 149, 1958.
and 2 were found to have ureters without an identifiable renal mass. All of the 33 patients operated upon have been followed. They are all asymptomatic and satisfied with the result. Only 2 of these patients have experienced episodes of infected urine and, in both instances, it has been easily controlled with antibiotics. Of the 6 patients who did not undergo operation 2 were advised to have heminephrectomy but refused, 1 was treated for hypertension only, 1 was treated for infection and underwent urethral dilations, 1 refused any treatment or further diagnostic study and 1 had a transurethral resection for prostatism but refused any other treatment for the refluxing, ectopic ureter. SUMMARY
Ureteral ectopia is an anomaly that can be corrected if recognized. Of the 39 cases (44 ureters) that have been described 8 were male patients, 3 of whom had ureteral ectopia into the seminal vesicle. These 3 cases have been reported in detail. The symptoms in female patients are usually urinary incontinence, infection, vaginal discharge or flank pain; in male patients, recurrent pyuria, prostatitis, urethritis or seminal vesiculitis. The most useful diagnostic tools are IVP, voiding cystourethrography, careful examination, cystoscopy, urethroscopy and seminal vesiculography. Heminephrectomy is the treatment of choice in most cases occurring in a duplicated kidney. Excellent results were obtained in all 33 patients treated surgically.