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Duplicated urethra: an anomaly best repaired
DUPLICATED URETHRA: AN ANOMALY BEST REPAIRED ANTHONY W. MIDDLETON, JR., M.D. RICHARD B. MELZER, M.D.
From the Divisions of Urology, Primary Children’s Medical Center and University of Utah College of Medicine, Salt Lake City, Utah ABSTRACTThree cases ofurethral duplication are described-two complete and one incomplete. The surgical approaches used in 2 of the 3 cases are described, the third not having come to definitive surgery as yet. In the patient with an incomplete urethral duplication the accessory urethra and associated ventral preputial hood were excised. A repair of the complete duplication was devised in which an end-to-side urethrourethrostomy and penile plastic revision were accomplished. The literature is reviewed and a new classification system for urethral duplication is proposed.
Urethral duplication is an unusual anomaly recognized since ancient times. The duplication may be complete, extending all the way to the bladder, or more commonly incomplete.’ We recently have cared for two children with complete duplications and another with an incomplete duplication. A surgical procedure which has not been previously described was devised for one of the children with complete duplication. Our operative management is described and a brief literature review is given. Case Reports Case 1 The patient was born with incomplete duplication, with a ventral preputial hood and an epispadic groove astride the dorsal corona of the glans (Fig. 1). A normal primary urethra was noted on cystoscopy and voiding cystourethrogram (VCUG). A plastic surgical repair was accomplished with excision of the epispadic urethra and ventral foreskin.
In complete urethral ending epispadiac groove.
FIGURE 1. blindly
538
duplic :ation
with
UROLOGY
i JUNE1992 / VOLUMEXXXIX,NUMBERf?
FIGURE:3. Completely duplicated urethra with indwelline catheters. FIGUHE:2. Completely duplicated urethra with sac&a r dilation of midportion of acce.ssory urethra which exits through perineum. Case 2 At age five weeks the patient was noted to have a. complete urethral duplication, with an accessory duplicated urethra joining the left base of the prostatic urethra and exiting through the midline perineum. The accessory urethra was noted with voiding from both the penile urethra and the perineal meatus. The accessor;; urethra had a saccular dilation along its mid portion on VCUG, with narrowing at its proximal and distal end (Fig. 2). A future excision o.f the accessory perineal urethra up to the level of the external sphincter is planned. At birth this patient was noted to have a normal-appearing urethral meatus at the tip of the glans penis with a second meatus lying on the ventrum of the penis three fourths of the way down the shaft distally (Fig. 3). He voided approximately equal streams from each meatus. There was no chordee. An intravenous pyelogram (IVP) revealed a normal right upper tract and nonfunction on the left; the VCUG demon-
strated complete urethral duplication (Fig. 4A). A left multicystic kidney was removed surgically in the neonatal period. Both urethras were too small to permit cystoscopy initially, so correction was deferred until age four years. Gradually over the first four years the urine stream via the normally placed meatus lessened, all urine passing from the ventral meatus eventually. A VCUG at age four years revealed the bulbous dorsal u.rethra had become obliterated (Fig. 4B). C>stoscopy revealed the dorsal urethra to be patent for about 3 cm, calibrating to 8 F. An impassable obstruction was encountered 3 cm from the meatus. The ventral meatus calibrated to 10 F, and cystoscopy along the ventral urethra demonstrated a normal verumontanum, a patulous bladder neck, and the dorsal urethra opening just distal to the bladder neck in the midline anteriorly. The bladder was normal. We devised an operation to reconstruct the urethra whereby we anastomosed the distal end of the ventral urethra to the patent dorsal distal urethra at the mid penile shaft level. After dissecting the distal ventral urethra free from the penile shaft, an infant feeding tube was passed into the distal dorsal urethra and the dorsal
FIGURE 4. Voiding cystourethrograms. (A) Complete patency of both urethras, performed in neonatal period. (B) Repeat VCUG at age four years, demonstrating obliteration of dorsal urethral duplication in midbulbous urethra.
FIGURE 5. Schematic diagrams of surgical correction. (A) Ventral urethra has been dissected free from shaft of penis. (B) Anastomosis of ventral urethra to dorsal urethra.
urethra was incised along its ventral surface longitudinally down onto the feeding tube. An oblique end-to-side urethrourethrostomy was accomplished using interrupted 6-O polyglycolic acid sutures (Fig. 5), placing a second row of interrupted 6-O polyglycolic acid sutures over the first. The skin on the penile shaft was appropriately tailored, and then anastomosed in two layers back onto the shaft of the penis with interrupted 5-O polyglycolic acid suture, the first layer being submucosal tissue and the final layer a skin-to-skin closure. A 5-F infant feeding tube was left along the urethra to drain the bladder, anchoring it to the penis with a silk stay suture placed through the glans. Five days postoperatively the dressings and catheter were removed. Six months following
540
surgery the patient is voiding with a single strong stream from the penile tip meatus (Fig. 6). Comment Several reports of urethral duplication have appeared in the literature1e7 including its first mention by Aristotle and later by Vesalius.’ Incomplete duplication, in which the second urethra does not extend to the prostatic urethra or bladder is much more common than complete duplication. Only 19 of 83 cases reviewed by Gross and Moore were complete duplication.’ In the literature a common definition of complete urethral duplication has been one in which both urethras connect the bladder to a
UROLOGY
i JUNE1992 / VOLUMEXXXIX,NUMBER6
Two theories have been proposed for the genesis of urethral duplication: bifurcation of the urethral anlage by continuation of the splitting process of the urorectal septums: and infolding of lateral mesodermal elements into the urethral anlage. g The former theory is suspect because of the failure to find any rectal fistulas in the series. The latter theory is a possibility, but remains uncorroborated by any experimental or observational data. The distal meatus of the completely duplicated accessory urethra has been located anywhere from the glans to the perineum. Of the 19 complete duplication cases catalogued by Gross and Moore,’ 4 were at the glans, 8 at the balanopreputial groove, 1 at midshaft, and 6 opened at the base of the penis. In those cases in which the dorsal urethra opens proximally; an epispadiac groove is present. 4 Often patients with accessory urethras or urethral duplication are asymptomatic.3.5 Symptoms generally relate to infection of a poorly draining dorsal tract. Prior to the advent of antibiotics the dorsal urethra would often harbor gonorrhea while the ventral tract was clear of infection. Occasionally the dorsal tract has been reported to collect debris and impinge on the primary urethra resulting in obstructive symptoms. Several classification systems have been proposed for urethral duplication.‘,“,‘.‘“-” We believe that the various schema are unnecessarily complex, trying to establish a separate category for every possible urethral configuration. We propose simplifying the classification to: (1) complete duplication, with the accessory urethra extending from any point proximal to the external sphincter and opening anywhere other than the normal meatus location; (2) incomplete duplication, with the accessory urethral meatus located anywhere other than the normal meatus location and with accessory urethra either ending blindly or communicating with the primary urethra at some point distal to the external sphincter; and (3) internal duplication, without an accessory urethral meatus, with the accessory urethra arising from the urethra and re-entering the urethra at another location.
FIGURE 6. Voiding with single stream from normally located dorsal urethral meatus eight months a_fter surgical correction.
point outside the body. l-3 However, we propose modifying the definition such that a complete duplication is one in which both urethras extend to a point above the external sphincter. Seven of the 19 patients with complete urethral duplication reported on by Gross and Moore had incontinence,’ but only a mild stress incontinence, the other patients being fully continent. Woodhouse and Williams7 noted 22 of 26 patients in their series with full urinary continence. We believe that this common finding of full or partial continence in patients despite having complete duplication is usually explaineld by a competent bladder neck with the accessory urethra arising distal to that point. Incomplete duplication occurs in a variety of configurations, with the second urethral meatus lying dorsal or ventral to the normal meatus. Only 1 of 7 cases of incomplete accessory u.rethras communicated with the normal urethra in Gross and Moore’s study, the rest ending blindly.’ Those that do communicate with the normal urethra make the communication in any possible location distal to the external sphincter.
UROLO(:\r
JUNE 1892
:
VOLUME
XXXIX,
NUMRER
Surgical Approaches Most early authors limited surgical intervention to those cases with related chronic or recurrent infection, severe penile deformity, or incontinence. In general we disagree, feeling that
6
541
cosmetic reasons are enough. Just as newer techniques have made the repair of all but the very mildest forms of hypospadias not only feasible but advisable, techniques such as we describe herein should make the reconstruction of most cases of urethral duplication advisable. Each case must be individually judged, however, and we would not now repair a very mild incomplete form of duplication seen in which the meatus of both urethras lie side by side at the tip of the otherwise cosmetically normal penis. Early attempts at complete excision of continent completely duplicated systems5 now seem unwise and put the sphincter at risk of injury. O’Herron and Webster6 describe a case with an accessory urethra with a primary hypospadias in which the septum between the two orifices was incised longitudinally. A poor result was obtained with a “nozzled” stream. In dealing with complete urethral duplication we believe the principle of joining the duplicated urethras combined with a plastic repair of the penis, as was done in Case 3, will be applicable to many cases of complete urethral duplication with an epispadiac or hypospadiac accessory urethral meatus, and should be performed even if the patient has been asymptomatic. The exception is where the duplication exists proximal to the penoscrotal junction and the primary urethra is normal, as in Case 2. In that case an excision of the accessory urethra to a level near, but still distal to, the external
542
sphincter is the best approach. In cases in which a urethrourethrostomy is done it is essential to adequately mobilize the urethra and accomplish a long oblique anastomosis to preclude an anastomotic stenosis after healing has occurred. Also, it is essential that the caliber of the distal segment be large enough to handle a normal urinary stream before conjoining the two urethras. 1060 East First South #lOO Salt Lake City, Utah 84102 (DR. MIDDLETON) References 1. Gross RE, and Moore TC: Duplication of the urethra, Arch Surg 60: 749 (1950). 2. Taruffi C: Sui canali anomali de1 pene, Bull SC. Med., Bologna (7) 2: 275 (1891). 3. Lowsley OS: Accessory urethra: report of two cases with a review of the literature. NY State Med 39: 1022 (1939). 4. Thevathasan C: Accessory urethra in the male ihild, Aust NZ J Surg 31: 134 (1961). 5. Tripathi VNP and Dick VS: Complete duplication of the male urethra, J Urol 101: 866 (1969). 6. O’Herron MK, and Webster FA: Report of two cases of double urethra in the male, J Urol 55: 391 (1946). 7. Woodhouse CR, and Williams DI: Duplications of the urinary tract in children, Br J Urol 51: 481 (1979). 8. Slotkin EA, and Mercer A: Case of epispadias with a double urethra, J Urol 70: 743 (1953). 9. Meyer R: Ueber embryonale Gewebsanomalien und ihre pathologische Bedentung im allegemeinen und solche bes mannlithen Genitalaparates im besonderen, Ergebn. d. allg. Path. u. path. Anat. 15: 430 (1911). 10. Das S, and Brosman SA: Duplication of the male urethra, J Urol 117: 452 (1977). 11. Sohrabi A, Belis JA, Durig JC, and McCuskey BM: Duplication of male urethra, Urology 12: 704 (1978).
UROLOGY
i
JUNE
1992
/
VOLUME
XXXIX, NUMBER
6
From the Divisions of Urology, Primary Children’s Medical Center and University of Utah College of Medicine, Salt Lake City, Utah ABSTRACTThree cases ofurethral duplication are described-two complete and one incomplete. The surgical approaches used in 2 of the 3 cases are described, the third not having come to definitive surgery as yet. In the patient with an incomplete urethral duplication the accessory urethra and associated ventral preputial hood were excised. A repair of the complete duplication was devised in which an end-to-side urethrourethrostomy and penile plastic revision were accomplished. The literature is reviewed and a new classification system for urethral duplication is proposed.
Urethral duplication is an unusual anomaly recognized since ancient times. The duplication may be complete, extending all the way to the bladder, or more commonly incomplete.’ We recently have cared for two children with complete duplications and another with an incomplete duplication. A surgical procedure which has not been previously described was devised for one of the children with complete duplication. Our operative management is described and a brief literature review is given. Case Reports Case 1 The patient was born with incomplete duplication, with a ventral preputial hood and an epispadic groove astride the dorsal corona of the glans (Fig. 1). A normal primary urethra was noted on cystoscopy and voiding cystourethrogram (VCUG). A plastic surgical repair was accomplished with excision of the epispadic urethra and ventral foreskin.
In complete urethral ending epispadiac groove.
FIGURE 1. blindly
538
duplic :ation
with
UROLOGY
i JUNE1992 / VOLUMEXXXIX,NUMBERf?
FIGURE:3. Completely duplicated urethra with indwelline catheters. FIGUHE:2. Completely duplicated urethra with sac&a r dilation of midportion of acce.ssory urethra which exits through perineum. Case 2 At age five weeks the patient was noted to have a. complete urethral duplication, with an accessory duplicated urethra joining the left base of the prostatic urethra and exiting through the midline perineum. The accessory urethra was noted with voiding from both the penile urethra and the perineal meatus. The accessor;; urethra had a saccular dilation along its mid portion on VCUG, with narrowing at its proximal and distal end (Fig. 2). A future excision o.f the accessory perineal urethra up to the level of the external sphincter is planned. At birth this patient was noted to have a normal-appearing urethral meatus at the tip of the glans penis with a second meatus lying on the ventrum of the penis three fourths of the way down the shaft distally (Fig. 3). He voided approximately equal streams from each meatus. There was no chordee. An intravenous pyelogram (IVP) revealed a normal right upper tract and nonfunction on the left; the VCUG demon-
strated complete urethral duplication (Fig. 4A). A left multicystic kidney was removed surgically in the neonatal period. Both urethras were too small to permit cystoscopy initially, so correction was deferred until age four years. Gradually over the first four years the urine stream via the normally placed meatus lessened, all urine passing from the ventral meatus eventually. A VCUG at age four years revealed the bulbous dorsal u.rethra had become obliterated (Fig. 4B). C>stoscopy revealed the dorsal urethra to be patent for about 3 cm, calibrating to 8 F. An impassable obstruction was encountered 3 cm from the meatus. The ventral meatus calibrated to 10 F, and cystoscopy along the ventral urethra demonstrated a normal verumontanum, a patulous bladder neck, and the dorsal urethra opening just distal to the bladder neck in the midline anteriorly. The bladder was normal. We devised an operation to reconstruct the urethra whereby we anastomosed the distal end of the ventral urethra to the patent dorsal distal urethra at the mid penile shaft level. After dissecting the distal ventral urethra free from the penile shaft, an infant feeding tube was passed into the distal dorsal urethra and the dorsal
FIGURE 4. Voiding cystourethrograms. (A) Complete patency of both urethras, performed in neonatal period. (B) Repeat VCUG at age four years, demonstrating obliteration of dorsal urethral duplication in midbulbous urethra.
FIGURE 5. Schematic diagrams of surgical correction. (A) Ventral urethra has been dissected free from shaft of penis. (B) Anastomosis of ventral urethra to dorsal urethra.
urethra was incised along its ventral surface longitudinally down onto the feeding tube. An oblique end-to-side urethrourethrostomy was accomplished using interrupted 6-O polyglycolic acid sutures (Fig. 5), placing a second row of interrupted 6-O polyglycolic acid sutures over the first. The skin on the penile shaft was appropriately tailored, and then anastomosed in two layers back onto the shaft of the penis with interrupted 5-O polyglycolic acid suture, the first layer being submucosal tissue and the final layer a skin-to-skin closure. A 5-F infant feeding tube was left along the urethra to drain the bladder, anchoring it to the penis with a silk stay suture placed through the glans. Five days postoperatively the dressings and catheter were removed. Six months following
540
surgery the patient is voiding with a single strong stream from the penile tip meatus (Fig. 6). Comment Several reports of urethral duplication have appeared in the literature1e7 including its first mention by Aristotle and later by Vesalius.’ Incomplete duplication, in which the second urethra does not extend to the prostatic urethra or bladder is much more common than complete duplication. Only 19 of 83 cases reviewed by Gross and Moore were complete duplication.’ In the literature a common definition of complete urethral duplication has been one in which both urethras connect the bladder to a
UROLOGY
i JUNE1992 / VOLUMEXXXIX,NUMBER6
Two theories have been proposed for the genesis of urethral duplication: bifurcation of the urethral anlage by continuation of the splitting process of the urorectal septums: and infolding of lateral mesodermal elements into the urethral anlage. g The former theory is suspect because of the failure to find any rectal fistulas in the series. The latter theory is a possibility, but remains uncorroborated by any experimental or observational data. The distal meatus of the completely duplicated accessory urethra has been located anywhere from the glans to the perineum. Of the 19 complete duplication cases catalogued by Gross and Moore,’ 4 were at the glans, 8 at the balanopreputial groove, 1 at midshaft, and 6 opened at the base of the penis. In those cases in which the dorsal urethra opens proximally; an epispadiac groove is present. 4 Often patients with accessory urethras or urethral duplication are asymptomatic.3.5 Symptoms generally relate to infection of a poorly draining dorsal tract. Prior to the advent of antibiotics the dorsal urethra would often harbor gonorrhea while the ventral tract was clear of infection. Occasionally the dorsal tract has been reported to collect debris and impinge on the primary urethra resulting in obstructive symptoms. Several classification systems have been proposed for urethral duplication.‘,“,‘.‘“-” We believe that the various schema are unnecessarily complex, trying to establish a separate category for every possible urethral configuration. We propose simplifying the classification to: (1) complete duplication, with the accessory urethra extending from any point proximal to the external sphincter and opening anywhere other than the normal meatus location; (2) incomplete duplication, with the accessory urethral meatus located anywhere other than the normal meatus location and with accessory urethra either ending blindly or communicating with the primary urethra at some point distal to the external sphincter; and (3) internal duplication, without an accessory urethral meatus, with the accessory urethra arising from the urethra and re-entering the urethra at another location.
FIGURE 6. Voiding with single stream from normally located dorsal urethral meatus eight months a_fter surgical correction.
point outside the body. l-3 However, we propose modifying the definition such that a complete duplication is one in which both urethras extend to a point above the external sphincter. Seven of the 19 patients with complete urethral duplication reported on by Gross and Moore had incontinence,’ but only a mild stress incontinence, the other patients being fully continent. Woodhouse and Williams7 noted 22 of 26 patients in their series with full urinary continence. We believe that this common finding of full or partial continence in patients despite having complete duplication is usually explaineld by a competent bladder neck with the accessory urethra arising distal to that point. Incomplete duplication occurs in a variety of configurations, with the second urethral meatus lying dorsal or ventral to the normal meatus. Only 1 of 7 cases of incomplete accessory u.rethras communicated with the normal urethra in Gross and Moore’s study, the rest ending blindly.’ Those that do communicate with the normal urethra make the communication in any possible location distal to the external sphincter.
UROLO(:\r
JUNE 1892
:
VOLUME
XXXIX,
NUMRER
Surgical Approaches Most early authors limited surgical intervention to those cases with related chronic or recurrent infection, severe penile deformity, or incontinence. In general we disagree, feeling that
6
541
cosmetic reasons are enough. Just as newer techniques have made the repair of all but the very mildest forms of hypospadias not only feasible but advisable, techniques such as we describe herein should make the reconstruction of most cases of urethral duplication advisable. Each case must be individually judged, however, and we would not now repair a very mild incomplete form of duplication seen in which the meatus of both urethras lie side by side at the tip of the otherwise cosmetically normal penis. Early attempts at complete excision of continent completely duplicated systems5 now seem unwise and put the sphincter at risk of injury. O’Herron and Webster6 describe a case with an accessory urethra with a primary hypospadias in which the septum between the two orifices was incised longitudinally. A poor result was obtained with a “nozzled” stream. In dealing with complete urethral duplication we believe the principle of joining the duplicated urethras combined with a plastic repair of the penis, as was done in Case 3, will be applicable to many cases of complete urethral duplication with an epispadiac or hypospadiac accessory urethral meatus, and should be performed even if the patient has been asymptomatic. The exception is where the duplication exists proximal to the penoscrotal junction and the primary urethra is normal, as in Case 2. In that case an excision of the accessory urethra to a level near, but still distal to, the external
542
sphincter is the best approach. In cases in which a urethrourethrostomy is done it is essential to adequately mobilize the urethra and accomplish a long oblique anastomosis to preclude an anastomotic stenosis after healing has occurred. Also, it is essential that the caliber of the distal segment be large enough to handle a normal urinary stream before conjoining the two urethras. 1060 East First South #lOO Salt Lake City, Utah 84102 (DR. MIDDLETON) References 1. Gross RE, and Moore TC: Duplication of the urethra, Arch Surg 60: 749 (1950). 2. Taruffi C: Sui canali anomali de1 pene, Bull SC. Med., Bologna (7) 2: 275 (1891). 3. Lowsley OS: Accessory urethra: report of two cases with a review of the literature. NY State Med 39: 1022 (1939). 4. Thevathasan C: Accessory urethra in the male ihild, Aust NZ J Surg 31: 134 (1961). 5. Tripathi VNP and Dick VS: Complete duplication of the male urethra, J Urol 101: 866 (1969). 6. O’Herron MK, and Webster FA: Report of two cases of double urethra in the male, J Urol 55: 391 (1946). 7. Woodhouse CR, and Williams DI: Duplications of the urinary tract in children, Br J Urol 51: 481 (1979). 8. Slotkin EA, and Mercer A: Case of epispadias with a double urethra, J Urol 70: 743 (1953). 9. Meyer R: Ueber embryonale Gewebsanomalien und ihre pathologische Bedentung im allegemeinen und solche bes mannlithen Genitalaparates im besonderen, Ergebn. d. allg. Path. u. path. Anat. 15: 430 (1911). 10. Das S, and Brosman SA: Duplication of the male urethra, J Urol 117: 452 (1977). 11. Sohrabi A, Belis JA, Durig JC, and McCuskey BM: Duplication of male urethra, Urology 12: 704 (1978).
UROLOGY
i
JUNE
1992
/
VOLUME
XXXIX, NUMBER
6