- Email: [email protected]
Urethrosphincteric Reconstruction for Congenitally Absent Urethra
THE JOURNAL OF UROLOGY
Vol. 116, August
Copyright © 1976 by The Williams & Wilkins Co.
Printed in U.S.A.
URETHROSPHINCTERIC RECONSTRUCTION FOR CONGENITALLY ABSENT URETHRA EMIL A. TANAGHO* From the Division of Urology, University of California School of Medicine, San Francisco, California
ABSTRACT
Three female patients with complete urinary incontinence owing to congenital absence of the urethra associated with complex congenital anomalies involving the caudal end of the urogenital sinus are reported. A sphincteric tube constructed from a flap of the anterior bladder wall was positioned in place of the missing urethra. Abundance of circularly oriented fibers in this neourethra provided sphincteric function sufficient to maintain continence and eliminate the need for urinary diversion. Details of the congenital anomalies and reconstructive techniques are discussed. Congenital anomalies involving development of the caudal segment of the urogenital sinus sometimes result in the female subject who has an absent or markedly deficient urethra with complete lack of sphincteric function. The most common anomaly is persistence of the urogenital sinus when the urethral development usually is adequate and the sphincteric function is normal. Variation or deviation from this anomaly has been encountered in 3 female patients with total absence of the urethra or its partial or distorted development devoid of functional significance. Complete urinary incontinence was an associated factor. These cases are described and discussed herein. Surgical reconstruction of a sphincteric urethral tube as a substitute for the anatomically absent urethral canal, with restoration of sphincteric function, is presented. CASE REPORTS
Case 1. A 17-year-old girl was seen by a gynecologist because of severe pain during an unsuccessful attempt at sexual intercourse the preceding day. The external genitalia appeared normal but when the labia were spread only 1 large opening could be seen. This opening was believed to be the vaginal canal. Pelvic examination allowed insertion of 2 fingers with difficulty but 1 finger could be introduced easily into a large smooth cavity that did not feel like a vaginal canal. Endoscopic examination revealed that the opening at the depth of the vaginal vestibule was actually the bladder neck. A normal left ureteral orifice and the left hemitrigone could be seen. This left orifice was easily catheterized, filled with dye and a normal kidney was visualized radiologically. Another larger opening was seen slightly distal to the usual site of the right ureteral orifice. This orifice was also easily catheterized, filled with dye and a vaginal canal was visualized (fig. 1, A). Clearly, what initially was thought to be a golfhole right ureteral orifice was actually an ectopic vaginal opening into the base of the bladder. The gross features of the anomaly began to take shape (fig. 1, B). The urethral canal was absent completely. A normal vaginal vestibule existed, with normally developed labia majora and minora. This vaginal vestibule led directly to the bladder cavity, which in turn received an ectopically ending vaginal canal. The right kidney and ureter were absent. A hemitrigone with a solitary left kidney existed. The vaginal canal revealed a rudimentary unicornous uterus with 1 right Accepted for publication December 12, 1975. Read at annual meeting of Western Section, American Urological Association, Portland, Oregon, April 13-17, 1975. * Requests for reprints: Division of Urology, A-644, University of California, San Francisco, California 94143. 237
fallopian tube and ovary. It seems that besides the lower urogenital sinus anomaly there was complete development of the left mesonephric duct system with its ureteral bud and nephric blastema, and complete absence of such structures on the right side. Vice versa, in the miillerian system, only the right duct had developed into a right fallopian tube, right unicornous uterus and a small right vaginal canal. Presumably, the right miillerian system had failed to join a degenerating or non-existent left miillerian system. This girl, who had been totally incontinent all her life, should have been seen earlier. She wore pads to manage the incontinence and did not seek help until her first sexual encounter. Surgical reconstruction: Through a vaginal approach the bladder neck was circumscribed and dissected from its connection to the vaginal vestibule (fig. 2, A). The dissection was extended posteriorly retrotrigonally until the vaginal connection with the bladder was identified. The distal end of the vaginal canal was freed all around and transected from the bladder wall (fig. 2, -A). The bladder was mobilized freely and displaced upward while the vaginal canal was also dissected, mobilized and pulled downward to replace the bladder. Its opening was sutured to the vaginal vestibule in the position previously occupied by the bladder neck (fig. 2, A). Through a suprapubic exposure the mobilized bladder was freed further and repositioned more cranially. Along the principles of a technique described previously 1 • 2 a square flap from the anterior bladder wall, just above the internal meatus, was shaped into a tube for the reconstruction of a 3½ cm. long urethral canal (fig. 2, B). The apex of the trigone was then sutured to the base of the reconstructed tube and the bladder closure was completed (fig. 2, B). Through a stab wound incision below the pubic arch and anterior to the repositioned vaginal canal, a pathway was created in which the neourethra could lie (fig. 2, B). The end of the tube was sutured vaginally to the vestibule just below the clitoris. The bladder was drained through a suprapubic cystostomy and a urethral catheter. Convalescence was uneventful except for moderate upward retraction of the external meatus and resultant stenosis after removal of the urethral catheter. Suprapubic drainage was maintained for a total of 6 weeks until the retracted external meatus was dilated and opened. After removal of the suprapubic tube the patient was completely dry and voided with an adequate stream. She was informed at that time that resumption of sexual activity was safe. When she did, a certain degree of urinary leakage occurred and the patient was then advised to abstain from sex for 3 more months. During this lapse of time she regained urinary control and resumption of sexual activity
238
'T'A.NAGHO
FIG. 1. Case 1. A, IVP shows normal left kidney, no visualization of right kidney and defects of pelvic outlet, especially pubic bones. B, anomalous anatomy of lower genitourinary tract. Solitary kidney with left hemitrigone. Ectopic vaginal opening into bladder base with right unicornous uterus and right fallopian tube. Wide bladder neck opening directly in vaginal vestibule.
Inds.ion at Bladdt'f Ned.
FIG. 2. Case 1. A, vaginal vestibule exposing single opening leading to bladder cavity and circular incision around bladder neck. Vaginal canal freed and disunited from base of bladder, which is being mobilized upward while vaginal canal is p•'!~ed downward. Vaginal canal mobilized and sutured to vaginal vestibule. B. 1 inch wide and 1 inch long anterior bladder flap is developed. Flap is turned into tube around Foley catheter. Complete closure of bladder base. Neourethra sutured to vaginal vestibule above new vaginal opening.
did not weaken the sphincteric function. The patient stays completely dry and engages in an active sexual life 2 ½ years postoperatively (fig. 3). Case 2. A 6-week-old female infant was first seen in 1968. She had had a distended lower abdomen since birth and was unable to void. A catheter, passed through a single opening into the perineum, drained a large volume of fluid and was then left in place. A large right abdominal mass was palpable. On excre-
tory urography (IVP) no right kidney was visualized and the left kidney was normal except for mild lower ureteral dilatation (fig. 4, A). Cystography via the draining catheter revealed a cavity that did not look like a bladder and was displaced posteriorly. On endoscopic observation a persistent, relatively long, urogenital sinus leading to a distended vaginal canal and a flattened cervix on top of it were seen. A urethral opening that was noted about 2 cm. proximally was catheterized, dye
URETHROSPHINCTERIC RECONSTRUCTION
FIG. 3. Case 1. A, postoperative IVP shows good function in left kidney and adequate bladder capacity. B, cystogram shows bladder cavity and catheter in urethra delineating length and course of reconstructed tube. Clamp is at level of external meatus. Note adequate urethral length. C, voiding cystourethrogram shows urethral canal, which appears narrow, although it admits 20F catheter easily.
FIG. 4. Case 2. A, IVP when patient was 6 weeks old shows no visualization of right kidney, left kidney within normal limits and large irregular filling defect in bladder base. B, enlarged view of lateral cystogram and vaginogram shows anteriorly displaced bladder, distended posterior vaginal cavity and large defect between them owing to giant ureterocele. Note short urethra opening into long urogenital sinus.
was injected and the bladder was outlined radiographically (fig. 4, B). A third cavity was situated between the anteriorly displaced bladder and the posteriorly distended vagina (fig. 4, B). This cavity later proved to be a giant ureterocele draining a dysplastic right kidney, prolapsing into the rudimentary short urethra and partially blocking the bladder. Abdominal exploration revealed the dysplastic right kidney and a bicornous uterus whose right half only was well developed, with a normal right fallopian tube and ovary (fig. 5, A). The left half of the bicornous uterus was rudimentary and had a tiny fallopian tube with a streak ovary. The rudimentary left horn, which was hardly distinguishable from the fallopian tube, joined the right uterus close to the cervix.
Surgical repair: Complete ureterocelectomy was attempted. However, because the ureterocele extended into the urethra and for fear of endangering the sphincteric mechanism, the urethral extension was only partially unroofed. Complete right nephroureterectomy was done. After reconstruction of the attenuated bladder base and trigonal area, left ureteral advancement was performed. Convalescence was uneventful but the patient remained wet constantly and had persistent lower urinary tract infection. This wetness was ascribed to vaginal voiding and continuous leakage from the vagina into the common sinus-the latter being so long that we thought a voluntary distal sphincteric mechanism might be contributing to the outlet resistance and thus increasing vaginal voiding.
240
TANAGHO
FIG. 5. Case 2. A, anatomical arrangement shows dysplastic right kidney ending in giant ureterocele that partly prolapsed in short urethra opening into long persistent urogenital sinus, leading to single opening in vaginal vestibule. B, square bladder flap developed to left side of midline because of previous cystostomy scar. Reconstruction of urethral tube from bladder flap. Anastomosis ofneourethra to vaginal vestibule.
Duplication of Uterus and Vagina
FIG. 6. Case 3. Initial anomalous anatomy includes high rectovesical fistula and obstructed right hydroureteronephrosis. Lateral view shows persistent urogenital sinus leading to duplicated tube (double vagina and double uterus) and short urethral opening directly into bladder. Rectum separated from bladder and opening into perineum. Perinea! view shows rectal opening anterior to anal sphincter dimple. Single urogenital sinus opening.
fufected urine was believed to be stagnant in the vaginal cavity rather than in the bladder cavity. An infected stone developed in the vaginal canal 1 ½ years postoperatively. Sphincterotomy and partial vaginoplasty were done to properly drain the vaginal canal. The stone was crushed and extracted. The patient continued to be wet continuously. When she was
5 years old endoscopic and urodynamic evaluations revealed a short urethra ( 1/2 cm. in length), widely open with remaining mucosal tags of the initial ureterocele. Pressure studies disclosed a zero closure pressure of this short urethral segment. Since hope for spontaneous control was unrealistic, a urethral reconstruction was planned. The Leadbetter technique• was inadvisable because of the previous existence of a giant ureterocele, attenuated bladder base and trigonal segment, in addition to the fact that the anterior bladder wall had been injured during the initial operation. The endoscopic findings were confirmed at exploration. Because of the midline scar from the previous anterior bladder wall incision, a flap developed from the bladder wall left to the midline was reconstructed into a tube around a IOF catheter and brought out to the vaginal vestibule via a stab wound incision anterior to the urogenital sinus (fig. 5, B). Suprapubic and Foley catheter drainage was maintained for 3 weeks postoperatively. Although on removal of the catheter and suprapubic tube, the patient had little urinary control it improved gradually. It has been 9 months since the operation and the child is almost completely dry except for mild stress incontinence when .the bladder is overdistended. She voids every 2 to 2 ½ hours with a good stream. External examination revealed that the external meatus had also retracted inward and was now appearing at the most distal part of the urogenital sinus. The patient and family are relieved from pre-existing tremendous tension resulting from attempts at training this unfortunate child. Case 3. A 3½-year-old girl was first seen in 1974 because of complete urinary incontinence. A single perineal opening, assumed to be a cloacal aperture draining the urinary and gastrointestinal tracts, was noted at birth. A high imperforate anus that opened into the bladder also had been noted (fig. 6). Under the protection of a transverse colostomy, the rectum had been separated from the bladder and brought down to the perineum (fig. 6). The patient remained subjected to complete urinary and fecal incontinence. Examination revealed that the rectum had been brought out anteriorly to a distinct dimple of an active anal sphincter (fig. 6). A single narrow vaginal opening also was seen after separation of underdeveloped labia. Endoscopic and radiologic examinations revealed a long com-
URETHROSPHINCTERIC RECONSTRUCTION
241
FIG. 7. Case 3. A, preoperative IVP shows right hydroureteronephrosis and poorly visualized left kidney with filling of distended right vaginal cavity. B, dotted line shows outline of left vaginal canal. C, lateral view shows large right vaginal canal behind distended bladder.
ladder Flap
Neourethra
Common Sinus
FIG. 8. Case 3. Surgical repair. Short urethra mobilized and excised from common sinus. Anterior bladder flap is developed, incorporating short urethral segment at its distal end. Reconstruction of vaginal tube and closure of bladder base. Neourethral opening sutured to vaginal vestibule just above urogenital sinus opening.
mon sinus leading to 3 orifices, 1 of which was flat, anteriorly located and led directly to a normal bladder cavity after a short ( ½ cm. long) urethral length. This orifice was rigid but patulous. The remaining 2 orifices were more posteriorly placed and led to 2 separate vaginal canals with a normal cervix on top of each. Both vaginal canals were distended moderately (fig. 7). On catheterization the left cervix proved to lead to a normal uterine cavity and fallopian tube. The right vaginal cavity was large, easily catheterized and distended. Cystography revealed the relationship of 2 cavities (fig. 7). In summary, the anatomic description of this area included a persistent urogenital sinus with a high imperforate anus, vaginal and uterine duplication, congenital absence of the urethra and congenital right lower ureteral obstruction (fig. 6). Reconstruction: Surgical repair was attempted. The bladder was mobilized suprapubically and the short urethral stump was dissected, then separated from the urogenital sinus. The urethra's opening was encroaching on the opening of the right vaginal cavity and they were closed separately. An anterior bladder flap incorporating the urethral stump was developed and formed into a tube (fig. 8). Through sharp dissection a pathway was developed anterior to the common sinus to the vaginal vestibule and, through a stab wound incision the reconstructed urethra was pulled down and its end sutured to the vaginal vestibule (fig. 8). The postoperative course was complicated by excessive urinary leakage that gradually diminished and stopped after 4 weeks when the catheter and suprapubic tubes were removed. The patient was still wet during early postoperative followup but holding variable volumes of urine and voiding frequently. IVP revealed some progression of the hydroureteronephrosis for which right ureteral reimplantation is planned. Voiding cystourethrography obtained with IVP has shown the development of a fistulous connection between the proximal segment of the reconstructed urethra and the right vaginal cavity. This fistula probably resulted from the opposing suture lines between the reconstructed urethral tube and the closure of the defect into the common sinus and right vaginal cavity. Fortunately, the fistula is distal to a competent internal meatus and urethral length seems to be adequate. The current plan is to wait for the
242
TANAGHO
repair of this iatrogenic fistula until the child is older, hoping that gradually she will develop better urinary control. Right ureteral reimplantation will be done to repair the lower ureteral obstruction. DISCUSSION
In 1969 and 1972 we reported experimental and clinical studies on a technique of sphincteric reconstruction for treatment of c...nplete urinary incontinence. 1• 2 The rationale of the surgical technique was that the anterior bladder wall above the internal meatus has abundance of transverse fibers and that a flap of this segment, when shaped into a tube, will amply provide circularly oriented fibers capable of exerting an occlusive effect on this tube and thus supply a sphincteric function. Experimental and urodynamic evaluations proved efficacy of such a rationale. In properly selected cases the operation offers a success rate of about 80 per cent. The same principle was applied in the 3 rare varieties of congenital anomalies involving the caudal end of the urogenital sinus herein reported. All 3 patients displayed complete incontinence owing to congenital
absence of urethra and lack of any sphincteric activity. The success of the operation totally depends on the reconstructed sphincteric tube without any help from either voluntary or involuntary sphincteric mechanism, as might be the case in postoperative or post-traumatic incontinence. This presentation is further proof of the soundness of the principle of sphincteric reconstruction, using the specific musculature of the anterior bladder wall. These varieties of congenital anomalies are interesting in their mechanism and embryogenesis but their extended discussion is beyond the scope of this report. REFERENCES 1. Tanagho, E. A., Smith, D. R., Meyers, F. H. and Fisher, R.:
Mechanism of urinary continence. II. Technique for surgical correction of incontinence. J. Urol., 101: 305, 1969. 2. Tanagho, E. A. and Smith, D. R.: Clinical evaluation of a surgical technique for the correction of complete urinary incontinence. J. Urol., 107: 402, 1972. 3. Leadbetter, G. W., Jr.: Surgical correction of total urinary incontinence. J. Urol., 91: 261, 1964.
Vol. 116, August
Copyright © 1976 by The Williams & Wilkins Co.
Printed in U.S.A.
URETHROSPHINCTERIC RECONSTRUCTION FOR CONGENITALLY ABSENT URETHRA EMIL A. TANAGHO* From the Division of Urology, University of California School of Medicine, San Francisco, California
ABSTRACT
Three female patients with complete urinary incontinence owing to congenital absence of the urethra associated with complex congenital anomalies involving the caudal end of the urogenital sinus are reported. A sphincteric tube constructed from a flap of the anterior bladder wall was positioned in place of the missing urethra. Abundance of circularly oriented fibers in this neourethra provided sphincteric function sufficient to maintain continence and eliminate the need for urinary diversion. Details of the congenital anomalies and reconstructive techniques are discussed. Congenital anomalies involving development of the caudal segment of the urogenital sinus sometimes result in the female subject who has an absent or markedly deficient urethra with complete lack of sphincteric function. The most common anomaly is persistence of the urogenital sinus when the urethral development usually is adequate and the sphincteric function is normal. Variation or deviation from this anomaly has been encountered in 3 female patients with total absence of the urethra or its partial or distorted development devoid of functional significance. Complete urinary incontinence was an associated factor. These cases are described and discussed herein. Surgical reconstruction of a sphincteric urethral tube as a substitute for the anatomically absent urethral canal, with restoration of sphincteric function, is presented. CASE REPORTS
Case 1. A 17-year-old girl was seen by a gynecologist because of severe pain during an unsuccessful attempt at sexual intercourse the preceding day. The external genitalia appeared normal but when the labia were spread only 1 large opening could be seen. This opening was believed to be the vaginal canal. Pelvic examination allowed insertion of 2 fingers with difficulty but 1 finger could be introduced easily into a large smooth cavity that did not feel like a vaginal canal. Endoscopic examination revealed that the opening at the depth of the vaginal vestibule was actually the bladder neck. A normal left ureteral orifice and the left hemitrigone could be seen. This left orifice was easily catheterized, filled with dye and a normal kidney was visualized radiologically. Another larger opening was seen slightly distal to the usual site of the right ureteral orifice. This orifice was also easily catheterized, filled with dye and a vaginal canal was visualized (fig. 1, A). Clearly, what initially was thought to be a golfhole right ureteral orifice was actually an ectopic vaginal opening into the base of the bladder. The gross features of the anomaly began to take shape (fig. 1, B). The urethral canal was absent completely. A normal vaginal vestibule existed, with normally developed labia majora and minora. This vaginal vestibule led directly to the bladder cavity, which in turn received an ectopically ending vaginal canal. The right kidney and ureter were absent. A hemitrigone with a solitary left kidney existed. The vaginal canal revealed a rudimentary unicornous uterus with 1 right Accepted for publication December 12, 1975. Read at annual meeting of Western Section, American Urological Association, Portland, Oregon, April 13-17, 1975. * Requests for reprints: Division of Urology, A-644, University of California, San Francisco, California 94143. 237
fallopian tube and ovary. It seems that besides the lower urogenital sinus anomaly there was complete development of the left mesonephric duct system with its ureteral bud and nephric blastema, and complete absence of such structures on the right side. Vice versa, in the miillerian system, only the right duct had developed into a right fallopian tube, right unicornous uterus and a small right vaginal canal. Presumably, the right miillerian system had failed to join a degenerating or non-existent left miillerian system. This girl, who had been totally incontinent all her life, should have been seen earlier. She wore pads to manage the incontinence and did not seek help until her first sexual encounter. Surgical reconstruction: Through a vaginal approach the bladder neck was circumscribed and dissected from its connection to the vaginal vestibule (fig. 2, A). The dissection was extended posteriorly retrotrigonally until the vaginal connection with the bladder was identified. The distal end of the vaginal canal was freed all around and transected from the bladder wall (fig. 2, -A). The bladder was mobilized freely and displaced upward while the vaginal canal was also dissected, mobilized and pulled downward to replace the bladder. Its opening was sutured to the vaginal vestibule in the position previously occupied by the bladder neck (fig. 2, A). Through a suprapubic exposure the mobilized bladder was freed further and repositioned more cranially. Along the principles of a technique described previously 1 • 2 a square flap from the anterior bladder wall, just above the internal meatus, was shaped into a tube for the reconstruction of a 3½ cm. long urethral canal (fig. 2, B). The apex of the trigone was then sutured to the base of the reconstructed tube and the bladder closure was completed (fig. 2, B). Through a stab wound incision below the pubic arch and anterior to the repositioned vaginal canal, a pathway was created in which the neourethra could lie (fig. 2, B). The end of the tube was sutured vaginally to the vestibule just below the clitoris. The bladder was drained through a suprapubic cystostomy and a urethral catheter. Convalescence was uneventful except for moderate upward retraction of the external meatus and resultant stenosis after removal of the urethral catheter. Suprapubic drainage was maintained for a total of 6 weeks until the retracted external meatus was dilated and opened. After removal of the suprapubic tube the patient was completely dry and voided with an adequate stream. She was informed at that time that resumption of sexual activity was safe. When she did, a certain degree of urinary leakage occurred and the patient was then advised to abstain from sex for 3 more months. During this lapse of time she regained urinary control and resumption of sexual activity
238
'T'A.NAGHO
FIG. 1. Case 1. A, IVP shows normal left kidney, no visualization of right kidney and defects of pelvic outlet, especially pubic bones. B, anomalous anatomy of lower genitourinary tract. Solitary kidney with left hemitrigone. Ectopic vaginal opening into bladder base with right unicornous uterus and right fallopian tube. Wide bladder neck opening directly in vaginal vestibule.
Inds.ion at Bladdt'f Ned.
FIG. 2. Case 1. A, vaginal vestibule exposing single opening leading to bladder cavity and circular incision around bladder neck. Vaginal canal freed and disunited from base of bladder, which is being mobilized upward while vaginal canal is p•'!~ed downward. Vaginal canal mobilized and sutured to vaginal vestibule. B. 1 inch wide and 1 inch long anterior bladder flap is developed. Flap is turned into tube around Foley catheter. Complete closure of bladder base. Neourethra sutured to vaginal vestibule above new vaginal opening.
did not weaken the sphincteric function. The patient stays completely dry and engages in an active sexual life 2 ½ years postoperatively (fig. 3). Case 2. A 6-week-old female infant was first seen in 1968. She had had a distended lower abdomen since birth and was unable to void. A catheter, passed through a single opening into the perineum, drained a large volume of fluid and was then left in place. A large right abdominal mass was palpable. On excre-
tory urography (IVP) no right kidney was visualized and the left kidney was normal except for mild lower ureteral dilatation (fig. 4, A). Cystography via the draining catheter revealed a cavity that did not look like a bladder and was displaced posteriorly. On endoscopic observation a persistent, relatively long, urogenital sinus leading to a distended vaginal canal and a flattened cervix on top of it were seen. A urethral opening that was noted about 2 cm. proximally was catheterized, dye
URETHROSPHINCTERIC RECONSTRUCTION
FIG. 3. Case 1. A, postoperative IVP shows good function in left kidney and adequate bladder capacity. B, cystogram shows bladder cavity and catheter in urethra delineating length and course of reconstructed tube. Clamp is at level of external meatus. Note adequate urethral length. C, voiding cystourethrogram shows urethral canal, which appears narrow, although it admits 20F catheter easily.
FIG. 4. Case 2. A, IVP when patient was 6 weeks old shows no visualization of right kidney, left kidney within normal limits and large irregular filling defect in bladder base. B, enlarged view of lateral cystogram and vaginogram shows anteriorly displaced bladder, distended posterior vaginal cavity and large defect between them owing to giant ureterocele. Note short urethra opening into long urogenital sinus.
was injected and the bladder was outlined radiographically (fig. 4, B). A third cavity was situated between the anteriorly displaced bladder and the posteriorly distended vagina (fig. 4, B). This cavity later proved to be a giant ureterocele draining a dysplastic right kidney, prolapsing into the rudimentary short urethra and partially blocking the bladder. Abdominal exploration revealed the dysplastic right kidney and a bicornous uterus whose right half only was well developed, with a normal right fallopian tube and ovary (fig. 5, A). The left half of the bicornous uterus was rudimentary and had a tiny fallopian tube with a streak ovary. The rudimentary left horn, which was hardly distinguishable from the fallopian tube, joined the right uterus close to the cervix.
Surgical repair: Complete ureterocelectomy was attempted. However, because the ureterocele extended into the urethra and for fear of endangering the sphincteric mechanism, the urethral extension was only partially unroofed. Complete right nephroureterectomy was done. After reconstruction of the attenuated bladder base and trigonal area, left ureteral advancement was performed. Convalescence was uneventful but the patient remained wet constantly and had persistent lower urinary tract infection. This wetness was ascribed to vaginal voiding and continuous leakage from the vagina into the common sinus-the latter being so long that we thought a voluntary distal sphincteric mechanism might be contributing to the outlet resistance and thus increasing vaginal voiding.
240
TANAGHO
FIG. 5. Case 2. A, anatomical arrangement shows dysplastic right kidney ending in giant ureterocele that partly prolapsed in short urethra opening into long persistent urogenital sinus, leading to single opening in vaginal vestibule. B, square bladder flap developed to left side of midline because of previous cystostomy scar. Reconstruction of urethral tube from bladder flap. Anastomosis ofneourethra to vaginal vestibule.
Duplication of Uterus and Vagina
FIG. 6. Case 3. Initial anomalous anatomy includes high rectovesical fistula and obstructed right hydroureteronephrosis. Lateral view shows persistent urogenital sinus leading to duplicated tube (double vagina and double uterus) and short urethral opening directly into bladder. Rectum separated from bladder and opening into perineum. Perinea! view shows rectal opening anterior to anal sphincter dimple. Single urogenital sinus opening.
fufected urine was believed to be stagnant in the vaginal cavity rather than in the bladder cavity. An infected stone developed in the vaginal canal 1 ½ years postoperatively. Sphincterotomy and partial vaginoplasty were done to properly drain the vaginal canal. The stone was crushed and extracted. The patient continued to be wet continuously. When she was
5 years old endoscopic and urodynamic evaluations revealed a short urethra ( 1/2 cm. in length), widely open with remaining mucosal tags of the initial ureterocele. Pressure studies disclosed a zero closure pressure of this short urethral segment. Since hope for spontaneous control was unrealistic, a urethral reconstruction was planned. The Leadbetter technique• was inadvisable because of the previous existence of a giant ureterocele, attenuated bladder base and trigonal segment, in addition to the fact that the anterior bladder wall had been injured during the initial operation. The endoscopic findings were confirmed at exploration. Because of the midline scar from the previous anterior bladder wall incision, a flap developed from the bladder wall left to the midline was reconstructed into a tube around a IOF catheter and brought out to the vaginal vestibule via a stab wound incision anterior to the urogenital sinus (fig. 5, B). Suprapubic and Foley catheter drainage was maintained for 3 weeks postoperatively. Although on removal of the catheter and suprapubic tube, the patient had little urinary control it improved gradually. It has been 9 months since the operation and the child is almost completely dry except for mild stress incontinence when .the bladder is overdistended. She voids every 2 to 2 ½ hours with a good stream. External examination revealed that the external meatus had also retracted inward and was now appearing at the most distal part of the urogenital sinus. The patient and family are relieved from pre-existing tremendous tension resulting from attempts at training this unfortunate child. Case 3. A 3½-year-old girl was first seen in 1974 because of complete urinary incontinence. A single perineal opening, assumed to be a cloacal aperture draining the urinary and gastrointestinal tracts, was noted at birth. A high imperforate anus that opened into the bladder also had been noted (fig. 6). Under the protection of a transverse colostomy, the rectum had been separated from the bladder and brought down to the perineum (fig. 6). The patient remained subjected to complete urinary and fecal incontinence. Examination revealed that the rectum had been brought out anteriorly to a distinct dimple of an active anal sphincter (fig. 6). A single narrow vaginal opening also was seen after separation of underdeveloped labia. Endoscopic and radiologic examinations revealed a long com-
URETHROSPHINCTERIC RECONSTRUCTION
241
FIG. 7. Case 3. A, preoperative IVP shows right hydroureteronephrosis and poorly visualized left kidney with filling of distended right vaginal cavity. B, dotted line shows outline of left vaginal canal. C, lateral view shows large right vaginal canal behind distended bladder.
ladder Flap
Neourethra
Common Sinus
FIG. 8. Case 3. Surgical repair. Short urethra mobilized and excised from common sinus. Anterior bladder flap is developed, incorporating short urethral segment at its distal end. Reconstruction of vaginal tube and closure of bladder base. Neourethral opening sutured to vaginal vestibule just above urogenital sinus opening.
mon sinus leading to 3 orifices, 1 of which was flat, anteriorly located and led directly to a normal bladder cavity after a short ( ½ cm. long) urethral length. This orifice was rigid but patulous. The remaining 2 orifices were more posteriorly placed and led to 2 separate vaginal canals with a normal cervix on top of each. Both vaginal canals were distended moderately (fig. 7). On catheterization the left cervix proved to lead to a normal uterine cavity and fallopian tube. The right vaginal cavity was large, easily catheterized and distended. Cystography revealed the relationship of 2 cavities (fig. 7). In summary, the anatomic description of this area included a persistent urogenital sinus with a high imperforate anus, vaginal and uterine duplication, congenital absence of the urethra and congenital right lower ureteral obstruction (fig. 6). Reconstruction: Surgical repair was attempted. The bladder was mobilized suprapubically and the short urethral stump was dissected, then separated from the urogenital sinus. The urethra's opening was encroaching on the opening of the right vaginal cavity and they were closed separately. An anterior bladder flap incorporating the urethral stump was developed and formed into a tube (fig. 8). Through sharp dissection a pathway was developed anterior to the common sinus to the vaginal vestibule and, through a stab wound incision the reconstructed urethra was pulled down and its end sutured to the vaginal vestibule (fig. 8). The postoperative course was complicated by excessive urinary leakage that gradually diminished and stopped after 4 weeks when the catheter and suprapubic tubes were removed. The patient was still wet during early postoperative followup but holding variable volumes of urine and voiding frequently. IVP revealed some progression of the hydroureteronephrosis for which right ureteral reimplantation is planned. Voiding cystourethrography obtained with IVP has shown the development of a fistulous connection between the proximal segment of the reconstructed urethra and the right vaginal cavity. This fistula probably resulted from the opposing suture lines between the reconstructed urethral tube and the closure of the defect into the common sinus and right vaginal cavity. Fortunately, the fistula is distal to a competent internal meatus and urethral length seems to be adequate. The current plan is to wait for the
242
TANAGHO
repair of this iatrogenic fistula until the child is older, hoping that gradually she will develop better urinary control. Right ureteral reimplantation will be done to repair the lower ureteral obstruction. DISCUSSION
In 1969 and 1972 we reported experimental and clinical studies on a technique of sphincteric reconstruction for treatment of c...nplete urinary incontinence. 1• 2 The rationale of the surgical technique was that the anterior bladder wall above the internal meatus has abundance of transverse fibers and that a flap of this segment, when shaped into a tube, will amply provide circularly oriented fibers capable of exerting an occlusive effect on this tube and thus supply a sphincteric function. Experimental and urodynamic evaluations proved efficacy of such a rationale. In properly selected cases the operation offers a success rate of about 80 per cent. The same principle was applied in the 3 rare varieties of congenital anomalies involving the caudal end of the urogenital sinus herein reported. All 3 patients displayed complete incontinence owing to congenital
absence of urethra and lack of any sphincteric activity. The success of the operation totally depends on the reconstructed sphincteric tube without any help from either voluntary or involuntary sphincteric mechanism, as might be the case in postoperative or post-traumatic incontinence. This presentation is further proof of the soundness of the principle of sphincteric reconstruction, using the specific musculature of the anterior bladder wall. These varieties of congenital anomalies are interesting in their mechanism and embryogenesis but their extended discussion is beyond the scope of this report. REFERENCES 1. Tanagho, E. A., Smith, D. R., Meyers, F. H. and Fisher, R.:
Mechanism of urinary continence. II. Technique for surgical correction of incontinence. J. Urol., 101: 305, 1969. 2. Tanagho, E. A. and Smith, D. R.: Clinical evaluation of a surgical technique for the correction of complete urinary incontinence. J. Urol., 107: 402, 1972. 3. Leadbetter, G. W., Jr.: Surgical correction of total urinary incontinence. J. Urol., 91: 261, 1964.