Diagnostic Radiology
with ipsilateral renal agenesis. The diagnostic technique and salient aspects of the embryological development of the genitourinary tract are considered.
Ureteral Ectopia Associated with Seminal Vesicle Cyst and Ipsilateral Renal Agenesis 1
CASE REPORT Maj. Gerald L. Levisay, MC USA,2 John Holder, M.D., and Col . John W. Weigel, MC USA A case of an ectopic ureter entering a seminal vesicle cyst and associated with ipsilateral renal agenesis is presented. The differential diagnosis and the embryological explanation of the anomaly are considered. INDEX TERMS:
Kidneys, abnormalities • Seminal Vesicles, cysts • Ureters, ab-
normalities Radiology 114:575-576, March 1975
ApPROXIMATEl Y 30-40 % of all congenital anomalies ' " involve the genitourinary system (2). Most are of no consequence and are discovered as incidental findings. Ureteral anomalies such as duplication are seen most commonly and often produce no symptoms. We wish to report a case of an ectopic ureter entering a seminal vesicle cyst and associated
Fig. 1. Fig. 2. Fig. 3.
A 21-year-old white man was referred to the Urology Clinic with the chief complaint of discomfort during urination and painful swelling in the left hemiscrotum . His medical history showed no significant abnormalities. On examination, the left epididymis was tender, enlarged, and indurated, indicating acute inflammation. The penis, right testis, and right epididymis were normal. Rectal examination disclosed a 5-cm tender cystic mass above the prostate. Urinalysis revealed red blood cells too numerous to count. Urine culture showed no growth . The white blood cell count was 10,900/mm3 and the hemoglobin level was 17 g/ 100 ml. Excretory urography revealed no evidence .of a functioning left renal unit and no apparent renal contour. There was compensatory hypertrophy of the right kidney with a normal collecting system. A smooth-walled 5-cm extravesical filling defect distorting the base of the bladder was seen on the urogram (Fig. 1). The patient was treated for acute left epididymitis with cephalexin, bed rest, and ice packs to the scrotum, and the infection resolved completely over the next 5 days. Cystoscopy revealed a large, smooth, translucent, thin walled cystic lesion on the left side of the bladder. The left hem itrigone and left ureteral or ifice were absent. About 5 m l
There is no renal function on the left. The right kidney is normal. Note the lucent filling defect at the base of the bladder. Seminal vesiculogram (anteroposteriorview) demonstratesa dilated, cyst-containing seminal vesicle and a dilated, tortuous ureter . Seminal vesiculDgram (lateral view) shows the extravesical portion of the cyst and the vas deferens entering its superior aspect .
1 From the Departments of Urology (G. L. L., J. W. W.) and Radiology (J. H.), Fitzsimons Army Medical Center, Denver, Colo. Accepted for publicationin October 1974. The opinions or assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the Department 01 the Army or the Departmentof Defense. sjh 2 Presentaddress: Fort Riley, Kansas.
575
576
GERALD L. LEVISA Y AND OTHERS
of opalescent fluid was aspirated from the cyst by means of a #5 ureteral catheter with a 21-gauge needle inserted into the tip. Nonmotile sperm were seen on microscopic examination. The patient was placed in the lithotomy position, the scrotum was prepared and draped, and the left vas was palpated. Following injection of 1% Xylocaine into the subcutaneous tissue of the scrotum, the left vas was isolated and grasped with a towel clip. A small incision was made in the skin of the anterior scrotum and carried down to the vas deferens. Approximately 2 cm of the vas was dissected free, a small vertical vasotomy was made, and a 22-gauge blunt-tipped needle was introduced into the lumen. Approximately 10 ml of contrast material (Conray-400) was injected slowly, while about 200 ml of air was injected transurethrally into the bladder to provide a double-contrast study. Radiographs obtained in the anteroposterior, oblique, and lateral projections (Figs. 2 and 3) revealed a communication between the dilated seminal vesicle and the tortuous, dilated left ureter, terminating in several saccular projections at the level of L-1 and L-2. The seminal vesicle cyst conformed well to the bladder-filling defect noted on the excretory urogram. In light of the patient's history of acute epididymitis, it was felt that the seminal vesicle cyst and ectopic ureter should be surgically excised because of the distinct possibility of recurring infection. This was done without difficulty via a Gibsontype extra peritoneal incision. The postoperative course was uncomplicated, and the patient has remained asymptomatic. Histological examination of the surgical specimen revealed chronic ureteritis, dilatation of the ectopic left ureter, and chronic inflammation and dilatation of a left seminal vesicle cyst. No renal tissue could be identified from the area of termination of the proximal ureter. DISCUSSION A knowledge of embryology is essential to an understanding of this anomaly. According to Arey, the mesonephros appears in the four-week-old embryo and establishes a connection with the elongated mesonephric or wolffian duct ( 1). The latter grows caudally and enters the primitive cloaca of the hindgut. The orifice then migrates, terminating in the urogenital sinus. A portion of the mesonephros forms the globus major of the epididymis, while other portions develop into vestigial structures. The duct forms the body and globus minor of the epididymis. Seminal vesicles appear as evaginations in the distal end of the mesonephric ducts. The final orifice of the mesonephric duct in the male is the ejaculatory duct, located at the site of the veru montanum. Before the cloaca divides to form the urogenital sinus and rectum secondary to the development of the urorectal fold, the ureteral buds appear on the dorsal surface of the wolffian ducts a short distance from the cloacal wall. These grow in a cephalad fashion into each metanephros to form the adult kidneys. The ureteral bud appears at the sharp bend in the
March 1975
mesonephric duct. The seminal vesicles do not appear until the thirteenth fetal week. Failure of the ureteral bud to develop or migrate into a normal trigonal position in the bladder could cause agenesis or ectopia. Developmental cysts of wolffian-duct origin or those arising from the seminal vesicle may secondarily affect ureteral and hence renal development. Cysts in this region must be differentiated from those of mullerlan-duct origin, which occur in the midline. Ureteral ectopia is rare in males; it is seen most commonly in females, accounting for 90% of cases (4). Ureteral duplication is associated with an ectopic ureteral orifice in 70 % of cases (3). In the female, the ectopic ureteral orifice may occur in the distal urethra or in the vagina. The diagnosis is often suspected before special studies are undertaken, due to continuous urinary leakage and recurrent urinary tract infection. Urinary incontinence secondary to an ectopic ureteral orifice is rarely if ever encountered in the male, since the ectopic orifice is always proximal to the external urethral sphincter. Ureteral ectopia in the male usually involves drainage into the neck of the bladder, posterior urethra, or seminal vesicle, all of which originate from the wolffian duct. Ectopia in these areas may be associated with reflux or obstruction, since renal function is impaired or absent. The correct diagnosis requires special diagnostic techniques. The differential diagnosis of a smooth-walled filling defect in the bladder is limited. Tumors and benign prostatic hypertrophy were considered unlikely in our patient in view of the cystic appearance of the lesions and the man's age. An ectopic ureterocele may present as a large, smooth-walled bladder-filling defect; however, it is usually associated with duplication of the upper urinary tract, the lower portion of which functions normally. Deep pelvic cysts may present as smooth-walled extravesical filling defects. Cysts of the mullerian duct are midline structures posterior to the bladder and never contain spermatozoa. Other cystic structures in the area would include cysts of the utricle and prostate. Seminal vesicle cysts are extravesical and are lateral to the midline unless they are massive in size; moreover they are always associated with renal agenesis or dysgenesis on the ipsilateral side. I
Col. John W. Weigel, MC USA Department of Urology Fitzsimons Army Medical Center Denver, Colo. 80240
REFERENCES 1. Arey LB: Developmental Anatomy. Philadelphia, Saunders, 6th Ed. 1954, pp 325-336 2. Campbell MF, ed: Urology. Philadelphia, Saunders, 2d Ed, 1963, Vol 2, P 1379 3. Emmett JL, Witten DM: Clinical Urography. Philadelphia, Saunders, 3d Ed, 1971, Vol 2, P 1319 4. Grossman H, Winchester PH, Muecke EC: Solitary ectopic ureter. Radiology 89:1069-1072, Dec 1967
(See also page 693)
with ipsilateral renal agenesis. The diagnostic technique and salient aspects of the embryological development of the genitourinary tract are considered.
Ureteral Ectopia Associated with Seminal Vesicle Cyst and Ipsilateral Renal Agenesis 1
CASE REPORT Maj. Gerald L. Levisay, MC USA,2 John Holder, M.D., and Col . John W. Weigel, MC USA A case of an ectopic ureter entering a seminal vesicle cyst and associated with ipsilateral renal agenesis is presented. The differential diagnosis and the embryological explanation of the anomaly are considered. INDEX TERMS:
Kidneys, abnormalities • Seminal Vesicles, cysts • Ureters, ab-
normalities Radiology 114:575-576, March 1975
ApPROXIMATEl Y 30-40 % of all congenital anomalies ' " involve the genitourinary system (2). Most are of no consequence and are discovered as incidental findings. Ureteral anomalies such as duplication are seen most commonly and often produce no symptoms. We wish to report a case of an ectopic ureter entering a seminal vesicle cyst and associated
Fig. 1. Fig. 2. Fig. 3.
A 21-year-old white man was referred to the Urology Clinic with the chief complaint of discomfort during urination and painful swelling in the left hemiscrotum . His medical history showed no significant abnormalities. On examination, the left epididymis was tender, enlarged, and indurated, indicating acute inflammation. The penis, right testis, and right epididymis were normal. Rectal examination disclosed a 5-cm tender cystic mass above the prostate. Urinalysis revealed red blood cells too numerous to count. Urine culture showed no growth . The white blood cell count was 10,900/mm3 and the hemoglobin level was 17 g/ 100 ml. Excretory urography revealed no evidence .of a functioning left renal unit and no apparent renal contour. There was compensatory hypertrophy of the right kidney with a normal collecting system. A smooth-walled 5-cm extravesical filling defect distorting the base of the bladder was seen on the urogram (Fig. 1). The patient was treated for acute left epididymitis with cephalexin, bed rest, and ice packs to the scrotum, and the infection resolved completely over the next 5 days. Cystoscopy revealed a large, smooth, translucent, thin walled cystic lesion on the left side of the bladder. The left hem itrigone and left ureteral or ifice were absent. About 5 m l
There is no renal function on the left. The right kidney is normal. Note the lucent filling defect at the base of the bladder. Seminal vesiculogram (anteroposteriorview) demonstratesa dilated, cyst-containing seminal vesicle and a dilated, tortuous ureter . Seminal vesiculDgram (lateral view) shows the extravesical portion of the cyst and the vas deferens entering its superior aspect .
1 From the Departments of Urology (G. L. L., J. W. W.) and Radiology (J. H.), Fitzsimons Army Medical Center, Denver, Colo. Accepted for publicationin October 1974. The opinions or assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the Department 01 the Army or the Departmentof Defense. sjh 2 Presentaddress: Fort Riley, Kansas.
575
576
GERALD L. LEVISA Y AND OTHERS
of opalescent fluid was aspirated from the cyst by means of a #5 ureteral catheter with a 21-gauge needle inserted into the tip. Nonmotile sperm were seen on microscopic examination. The patient was placed in the lithotomy position, the scrotum was prepared and draped, and the left vas was palpated. Following injection of 1% Xylocaine into the subcutaneous tissue of the scrotum, the left vas was isolated and grasped with a towel clip. A small incision was made in the skin of the anterior scrotum and carried down to the vas deferens. Approximately 2 cm of the vas was dissected free, a small vertical vasotomy was made, and a 22-gauge blunt-tipped needle was introduced into the lumen. Approximately 10 ml of contrast material (Conray-400) was injected slowly, while about 200 ml of air was injected transurethrally into the bladder to provide a double-contrast study. Radiographs obtained in the anteroposterior, oblique, and lateral projections (Figs. 2 and 3) revealed a communication between the dilated seminal vesicle and the tortuous, dilated left ureter, terminating in several saccular projections at the level of L-1 and L-2. The seminal vesicle cyst conformed well to the bladder-filling defect noted on the excretory urogram. In light of the patient's history of acute epididymitis, it was felt that the seminal vesicle cyst and ectopic ureter should be surgically excised because of the distinct possibility of recurring infection. This was done without difficulty via a Gibsontype extra peritoneal incision. The postoperative course was uncomplicated, and the patient has remained asymptomatic. Histological examination of the surgical specimen revealed chronic ureteritis, dilatation of the ectopic left ureter, and chronic inflammation and dilatation of a left seminal vesicle cyst. No renal tissue could be identified from the area of termination of the proximal ureter. DISCUSSION A knowledge of embryology is essential to an understanding of this anomaly. According to Arey, the mesonephros appears in the four-week-old embryo and establishes a connection with the elongated mesonephric or wolffian duct ( 1). The latter grows caudally and enters the primitive cloaca of the hindgut. The orifice then migrates, terminating in the urogenital sinus. A portion of the mesonephros forms the globus major of the epididymis, while other portions develop into vestigial structures. The duct forms the body and globus minor of the epididymis. Seminal vesicles appear as evaginations in the distal end of the mesonephric ducts. The final orifice of the mesonephric duct in the male is the ejaculatory duct, located at the site of the veru montanum. Before the cloaca divides to form the urogenital sinus and rectum secondary to the development of the urorectal fold, the ureteral buds appear on the dorsal surface of the wolffian ducts a short distance from the cloacal wall. These grow in a cephalad fashion into each metanephros to form the adult kidneys. The ureteral bud appears at the sharp bend in the
March 1975
mesonephric duct. The seminal vesicles do not appear until the thirteenth fetal week. Failure of the ureteral bud to develop or migrate into a normal trigonal position in the bladder could cause agenesis or ectopia. Developmental cysts of wolffian-duct origin or those arising from the seminal vesicle may secondarily affect ureteral and hence renal development. Cysts in this region must be differentiated from those of mullerlan-duct origin, which occur in the midline. Ureteral ectopia is rare in males; it is seen most commonly in females, accounting for 90% of cases (4). Ureteral duplication is associated with an ectopic ureteral orifice in 70 % of cases (3). In the female, the ectopic ureteral orifice may occur in the distal urethra or in the vagina. The diagnosis is often suspected before special studies are undertaken, due to continuous urinary leakage and recurrent urinary tract infection. Urinary incontinence secondary to an ectopic ureteral orifice is rarely if ever encountered in the male, since the ectopic orifice is always proximal to the external urethral sphincter. Ureteral ectopia in the male usually involves drainage into the neck of the bladder, posterior urethra, or seminal vesicle, all of which originate from the wolffian duct. Ectopia in these areas may be associated with reflux or obstruction, since renal function is impaired or absent. The correct diagnosis requires special diagnostic techniques. The differential diagnosis of a smooth-walled filling defect in the bladder is limited. Tumors and benign prostatic hypertrophy were considered unlikely in our patient in view of the cystic appearance of the lesions and the man's age. An ectopic ureterocele may present as a large, smooth-walled bladder-filling defect; however, it is usually associated with duplication of the upper urinary tract, the lower portion of which functions normally. Deep pelvic cysts may present as smooth-walled extravesical filling defects. Cysts of the mullerian duct are midline structures posterior to the bladder and never contain spermatozoa. Other cystic structures in the area would include cysts of the utricle and prostate. Seminal vesicle cysts are extravesical and are lateral to the midline unless they are massive in size; moreover they are always associated with renal agenesis or dysgenesis on the ipsilateral side. I
Col. John W. Weigel, MC USA Department of Urology Fitzsimons Army Medical Center Denver, Colo. 80240
REFERENCES 1. Arey LB: Developmental Anatomy. Philadelphia, Saunders, 6th Ed. 1954, pp 325-336 2. Campbell MF, ed: Urology. Philadelphia, Saunders, 2d Ed, 1963, Vol 2, P 1379 3. Emmett JL, Witten DM: Clinical Urography. Philadelphia, Saunders, 3d Ed, 1971, Vol 2, P 1319 4. Grossman H, Winchester PH, Muecke EC: Solitary ectopic ureter. Radiology 89:1069-1072, Dec 1967
(See also page 693)
