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Single ectopic ureter: Unusual presentation in adult male
SINGLE ECTOPIC URETER: UNUSUAL PRESENTATION IN ADULT MALE UNYIME 0. NSEYO, M.D. HARRY J. WILBUR,
M.D.
WILLIAM
CROMIE,
M.D.
ALAN H. BENNETT,
M.D.
From the Division of Urological Surgery, Albany VA Medical Center Hospital and Albany Medical Center Hospital, Albany, New York
ABSTRACTWe present a case of an unusual and late presentation of a single ectopic ureter in an adult male. The patient presented with gross painless hematuria. The left ureter draining a dysplastic kidney terminated in the ipsilateral seminal vesicle.
Single ectopic ureter is rare in males. An abnormal opening of the ectopic ureter into the male genital tract is even a rarer congenital abnormality, Since Day’s first case in 1932, 97 cases of ectopic ureter involving the genital tract have been reported in the literature, and only 26 cases have been diagnosed clinically.1~2 The most common sites of incorporation of ectopic ureters into the wolffian duct derivatives in the male are seminal vesicle, vas deferens, and ejaculatory ducts, in decreasing order of frequency. The presenting complaints include: pyuria, recurrent urinary tract infection, painful micturition, and perineal and rectal discomfort. Most of the cases reported in the literature have presented in adult life.3 In the series of 8 cases reviewed by Schnitzer,3 patients ranged in age from sixteen to thirty-eight, with an average age of twenty-three. Increased awareness and available diagnostic modalities have increased the chances for early clinical diagnosis. This congenital anomaly has been noted to involve the left side more frequently than the right: 7/8 (88%) in Schnitzer’s series; 2/3 (67 % ) cases reported by Brannan and Henry2 involved the left ureter and left genital tract. We herein present a case of an unusual manifestation of a left ureter terminating in the left seminal vesicle.
238
Case Report A twenty-five-year-old white man was admitted with a three-day history of gross total hematuria associated with a history of slight left lower abdominal pain and post-ejaculatory perineal discomfort. His past history revealed that as a child he had bilateral repair of inguinal hernias. A year prior to admission the patient underwent left hydrocelectomy. There was no documentation of previous epididymitis. The patient was treated successfully with tetracycline for an episode of nonspecific urethritis. On physical examination, the patient was afebrile, with well-healed bilateral inguinal and left hemiscrotal incisional scars. The genitalia were normal, except for a slightly tender and swollen left epididymis. Rectal examination revealed a normal prostate and no palpable mass. Urinalysis revealed numerous red blood cells and 2-3 white cells. Urine culture was negative, as was urine cytology. Excretory urogram (IVP) revealed nonfunction of the left side and compensatory hypertrophy of the right kidney (Fig. 1A). A voiding cystourethrogram revealed a normal outline of the bladder and no reflux. Cystoscopy revealed an elevation in the left hemitrigone
UROLOGY
/ SEPTEMBER
1986
/ VOLUME
XXVIII,
NUMBER 3
FIGURE 1. (A) Excretory urogram (IVP) showing absent left kidney and compensatory hypertrophy of right kidney. (B) Left retrograde ureteropyelogram showing rudimentary left collecting system, reflux of contrast material into saccular seminal vesicle.
which receded as the bladder was being filled. A left ureterocele was suspected. A dimple in the center of this elevation was thought to represent the orifice which opened into a cystic structure filled with old blood when probed with a ureteral catheter. Retrograde pyelography delineated a dilated distal left ureter, joining a saccular seminal vesicle and reflux into a left vas deferens (Fig. 1B). A rudimentary collecting system could also be seen. Renal ultrasound, as well as renal isotope scan, failed to reveal any renal mass or functional renal substance on the left side. Because of concern for the patient’s fertility, he underwent preoperative semen analysis which showed a volume of 1.5 ml, count of 17 million with 60 per cent motility and normal morphology. Subsequently, the patient underwent a left vasogram which showed reflux into the distal left ureter (Fig. 2).
FIGURE 2. Left vasogram with reflux of contrast material into dilated distal left ureter.
UROLOGY
At the time of surgery, the left vas was ligated. On flank exploration, a rudimentary left kidney with the proximal two thirds of ureter was removed, The patient had just married and desired children, and because of this consideration, the left seminal vesicle which appeared on the radiographic studies to be in the midline was not removed, to prevent possible injury to the genital system on the right side. The pathologic report was a 20 Gm kidney, which was histologically dysplastic without any recognizable renal parenchyma. The patient has noted significant improvement in his symptoms since operation. Comment The symptoms of pain and epididymitis on the involved side are the most common clinical manifestations in the patient with the ectopic ureter involving the genital tract.4,5 Our patient sought medical attention because of gross painless hematuria. Hematuria as a presenting symptom is rare. Brannan and Henry2 reported 2 other cases; one presenting with gross hematuria and another with bloody ejaculate. The patient’s perineal pain was postejaculatory and might have been due to contraction of the seminal vesicle which, although abnormally incorporated into the left ureter, might still have retained functional ability. Both the clinical and gross findings of a slightly swollen, tender and thickened left vas deferens suggest chronic subclinical epididymitis. The absence of reflux into the left ureter on voiding cystourethrography rules out recurrent pyelonephritis as the
/ SEPTEMBER1986 / VOLUMEXXVIILNUMBER3
239
FIGURE3. Diagrammatic representation of congenital anomaly of this patient. Rudimentary left kidney, dilated and saccular left seminal vesicle are noted. source of infection. The left hydrocele represented a reactive process to his left epididymitis. A supraprostatic cystic mass on the involved side presents in 95-100 per cent of the patients with ectopic ureters terminating in seminal vesiclesl-’ The absence of this finding in our patient raises the speculation that the mass which is usually the dilated seminal vesicle decompressed to cause the gross hematuria. Brannan and Henry2 reported the diagnosis of a case of a ureter terminating in a seminal vesicle during infertility workup in a young man. An infertile male with a history of recurrent urinary tract infection and genital pain might benefit from full workup to rule out this developmental anomaly. Increased awareness and availability of better diagnostic modalities should permit early diagnosis of this entity. In addition to thorough history and physical examination, excretory urogram (IVP), voiding cystourethrogram, urethrocystoscopy, seminal vesiculography, and retrograde ureterography are most helpful diagnostic tools. The treatment of choice is surgical once the diagnosis has been made. Surgi-
240
cal treatment depends on the extent of damage of renal tissue affected by the diseased portion,2 as well as the state of the contralateral kidney. Embryologically, the ureteric bud giving rise to the single ectopic ureter originates more cephalad in the mesonephric duct, giving rise to a longer trigone precursor segment.81QThe farther the opening of the ureter is from the normal location in the posterolateral extremity of the trigone, the more dysplastic is the kidney it drains. The ectopic ureter opening into such an extravesical site as the seminal vesicle should be much more dysplastic as is the case in our patient (Fig. 3). Nephrectomy and nephroureterectomy with removal of the involved seminal vesicle has been employed to manage these patients. The involved seminal vesicle in our patient was not removed in consideration for his fertility. There has not been any report of malignant degeneration in either the urinary tract or seminal specimens. A single ectopic ureter terminating in the seminal vesicle is a rare congenital anomaly. Gross hematuria is an uncommon presenting symptom. History of recurrent urinary tract infection, genital pain, and epididymitis warrant a search for the anomaly. A subfertile male showing any of these clinical manifestations should be fully evaluated also. Nephrectomy with or without seminal vesiculectomy remains the standard treatment depending on the extent of renal damage. Excellent results have been reported in all cases. Department of Urologic Oncology Roswell Park Memorial Institute 666 Elm Street Buffalo, New York 14263 (DR. NSEYO) References 1. Riba LW, Schmidlapp CJ, and Bosworth NL: Ectopic ureter draining into the seminal vesicle. 1 Urol 56: 332 0946). 2. Brannan W, and Henry HH:*Ureteral ectopia: report of 39 cases, ibid 109: 192 (1973). 3. Schnitzer B: Ectopic ureteral opening into seminal vesicle: a report of four cases, ibid 93: 576 (1965). 4. Williams DI, and Royle M: Ectopic ureter in the male child, Br J Urol 41: 421 (1969). 5. Mogg RA: The single ectopic ureter, ibid 46: 3 (1974). 6. Johnston JH, and Davenport TJ: The single ectopic ureter, ibid 41: 428 (1961). 7. Stutzman DM, and Patton JF: Ureteral ectopia: combined ureteral and vas deferens anomaly, J Urol84: 604 (1960). 8. Tanagho EA: Embryologic basis for lower ureteral anomalies: a hypothesis, Urology 7: 451 (1976). 9. Malek RS, Kelalis PP, Stickler GB, and Burke EC: Observations on ureteral ectopy in children, J Urol 107: 308 (1972).
UROLOGY
/
SEPTEMBER
1986
I
VOLUME KKVIII, NUMBER 3
M.D.
WILLIAM
CROMIE,
M.D.
ALAN H. BENNETT,
M.D.
From the Division of Urological Surgery, Albany VA Medical Center Hospital and Albany Medical Center Hospital, Albany, New York
ABSTRACTWe present a case of an unusual and late presentation of a single ectopic ureter in an adult male. The patient presented with gross painless hematuria. The left ureter draining a dysplastic kidney terminated in the ipsilateral seminal vesicle.
Single ectopic ureter is rare in males. An abnormal opening of the ectopic ureter into the male genital tract is even a rarer congenital abnormality, Since Day’s first case in 1932, 97 cases of ectopic ureter involving the genital tract have been reported in the literature, and only 26 cases have been diagnosed clinically.1~2 The most common sites of incorporation of ectopic ureters into the wolffian duct derivatives in the male are seminal vesicle, vas deferens, and ejaculatory ducts, in decreasing order of frequency. The presenting complaints include: pyuria, recurrent urinary tract infection, painful micturition, and perineal and rectal discomfort. Most of the cases reported in the literature have presented in adult life.3 In the series of 8 cases reviewed by Schnitzer,3 patients ranged in age from sixteen to thirty-eight, with an average age of twenty-three. Increased awareness and available diagnostic modalities have increased the chances for early clinical diagnosis. This congenital anomaly has been noted to involve the left side more frequently than the right: 7/8 (88%) in Schnitzer’s series; 2/3 (67 % ) cases reported by Brannan and Henry2 involved the left ureter and left genital tract. We herein present a case of an unusual manifestation of a left ureter terminating in the left seminal vesicle.
238
Case Report A twenty-five-year-old white man was admitted with a three-day history of gross total hematuria associated with a history of slight left lower abdominal pain and post-ejaculatory perineal discomfort. His past history revealed that as a child he had bilateral repair of inguinal hernias. A year prior to admission the patient underwent left hydrocelectomy. There was no documentation of previous epididymitis. The patient was treated successfully with tetracycline for an episode of nonspecific urethritis. On physical examination, the patient was afebrile, with well-healed bilateral inguinal and left hemiscrotal incisional scars. The genitalia were normal, except for a slightly tender and swollen left epididymis. Rectal examination revealed a normal prostate and no palpable mass. Urinalysis revealed numerous red blood cells and 2-3 white cells. Urine culture was negative, as was urine cytology. Excretory urogram (IVP) revealed nonfunction of the left side and compensatory hypertrophy of the right kidney (Fig. 1A). A voiding cystourethrogram revealed a normal outline of the bladder and no reflux. Cystoscopy revealed an elevation in the left hemitrigone
UROLOGY
/ SEPTEMBER
1986
/ VOLUME
XXVIII,
NUMBER 3
FIGURE 1. (A) Excretory urogram (IVP) showing absent left kidney and compensatory hypertrophy of right kidney. (B) Left retrograde ureteropyelogram showing rudimentary left collecting system, reflux of contrast material into saccular seminal vesicle.
which receded as the bladder was being filled. A left ureterocele was suspected. A dimple in the center of this elevation was thought to represent the orifice which opened into a cystic structure filled with old blood when probed with a ureteral catheter. Retrograde pyelography delineated a dilated distal left ureter, joining a saccular seminal vesicle and reflux into a left vas deferens (Fig. 1B). A rudimentary collecting system could also be seen. Renal ultrasound, as well as renal isotope scan, failed to reveal any renal mass or functional renal substance on the left side. Because of concern for the patient’s fertility, he underwent preoperative semen analysis which showed a volume of 1.5 ml, count of 17 million with 60 per cent motility and normal morphology. Subsequently, the patient underwent a left vasogram which showed reflux into the distal left ureter (Fig. 2).
FIGURE 2. Left vasogram with reflux of contrast material into dilated distal left ureter.
UROLOGY
At the time of surgery, the left vas was ligated. On flank exploration, a rudimentary left kidney with the proximal two thirds of ureter was removed, The patient had just married and desired children, and because of this consideration, the left seminal vesicle which appeared on the radiographic studies to be in the midline was not removed, to prevent possible injury to the genital system on the right side. The pathologic report was a 20 Gm kidney, which was histologically dysplastic without any recognizable renal parenchyma. The patient has noted significant improvement in his symptoms since operation. Comment The symptoms of pain and epididymitis on the involved side are the most common clinical manifestations in the patient with the ectopic ureter involving the genital tract.4,5 Our patient sought medical attention because of gross painless hematuria. Hematuria as a presenting symptom is rare. Brannan and Henry2 reported 2 other cases; one presenting with gross hematuria and another with bloody ejaculate. The patient’s perineal pain was postejaculatory and might have been due to contraction of the seminal vesicle which, although abnormally incorporated into the left ureter, might still have retained functional ability. Both the clinical and gross findings of a slightly swollen, tender and thickened left vas deferens suggest chronic subclinical epididymitis. The absence of reflux into the left ureter on voiding cystourethrography rules out recurrent pyelonephritis as the
/ SEPTEMBER1986 / VOLUMEXXVIILNUMBER3
239
FIGURE3. Diagrammatic representation of congenital anomaly of this patient. Rudimentary left kidney, dilated and saccular left seminal vesicle are noted. source of infection. The left hydrocele represented a reactive process to his left epididymitis. A supraprostatic cystic mass on the involved side presents in 95-100 per cent of the patients with ectopic ureters terminating in seminal vesiclesl-’ The absence of this finding in our patient raises the speculation that the mass which is usually the dilated seminal vesicle decompressed to cause the gross hematuria. Brannan and Henry2 reported the diagnosis of a case of a ureter terminating in a seminal vesicle during infertility workup in a young man. An infertile male with a history of recurrent urinary tract infection and genital pain might benefit from full workup to rule out this developmental anomaly. Increased awareness and availability of better diagnostic modalities should permit early diagnosis of this entity. In addition to thorough history and physical examination, excretory urogram (IVP), voiding cystourethrogram, urethrocystoscopy, seminal vesiculography, and retrograde ureterography are most helpful diagnostic tools. The treatment of choice is surgical once the diagnosis has been made. Surgi-
240
cal treatment depends on the extent of damage of renal tissue affected by the diseased portion,2 as well as the state of the contralateral kidney. Embryologically, the ureteric bud giving rise to the single ectopic ureter originates more cephalad in the mesonephric duct, giving rise to a longer trigone precursor segment.81QThe farther the opening of the ureter is from the normal location in the posterolateral extremity of the trigone, the more dysplastic is the kidney it drains. The ectopic ureter opening into such an extravesical site as the seminal vesicle should be much more dysplastic as is the case in our patient (Fig. 3). Nephrectomy and nephroureterectomy with removal of the involved seminal vesicle has been employed to manage these patients. The involved seminal vesicle in our patient was not removed in consideration for his fertility. There has not been any report of malignant degeneration in either the urinary tract or seminal specimens. A single ectopic ureter terminating in the seminal vesicle is a rare congenital anomaly. Gross hematuria is an uncommon presenting symptom. History of recurrent urinary tract infection, genital pain, and epididymitis warrant a search for the anomaly. A subfertile male showing any of these clinical manifestations should be fully evaluated also. Nephrectomy with or without seminal vesiculectomy remains the standard treatment depending on the extent of renal damage. Excellent results have been reported in all cases. Department of Urologic Oncology Roswell Park Memorial Institute 666 Elm Street Buffalo, New York 14263 (DR. NSEYO) References 1. Riba LW, Schmidlapp CJ, and Bosworth NL: Ectopic ureter draining into the seminal vesicle. 1 Urol 56: 332 0946). 2. Brannan W, and Henry HH:*Ureteral ectopia: report of 39 cases, ibid 109: 192 (1973). 3. Schnitzer B: Ectopic ureteral opening into seminal vesicle: a report of four cases, ibid 93: 576 (1965). 4. Williams DI, and Royle M: Ectopic ureter in the male child, Br J Urol 41: 421 (1969). 5. Mogg RA: The single ectopic ureter, ibid 46: 3 (1974). 6. Johnston JH, and Davenport TJ: The single ectopic ureter, ibid 41: 428 (1961). 7. Stutzman DM, and Patton JF: Ureteral ectopia: combined ureteral and vas deferens anomaly, J Urol84: 604 (1960). 8. Tanagho EA: Embryologic basis for lower ureteral anomalies: a hypothesis, Urology 7: 451 (1976). 9. Malek RS, Kelalis PP, Stickler GB, and Burke EC: Observations on ureteral ectopy in children, J Urol 107: 308 (1972).
UROLOGY
/
SEPTEMBER
1986
I
VOLUME KKVIII, NUMBER 3