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The use of paraexstrophy flaps for urethral construction in neonatal girls with classical Exstrophy
0022-534 7/88/1403-057 4$02.00/0 Vol. 140, September
THE JOURNAL OF UROLOGY
Printed in U.S.A.
Copyright © 1988 by The Williams & Wilkins Co.
THE USE OF PARAEXSTROPHY FLAPS FOR URETHRAL CONSTRUCTION IN NEONATAL GIRLS WITH CLASSICAL EXSTROPHY MICHAEL R. SPINDEL,* BOYD H. WINSLOW
AND
GERALD H. JORDAN
From the Eastern Virginia Medical School, Norfolk, Virginia
ABSTRACT
Primary closure of classical exstrophy was performed with paraexstrophy skin flaps for urethral lengthening in 4 female neonates. In all cases more than 2.0 cm. of urethral length were achieved. Efficacy of bladder closure and subsequent bladder neck revision were enhanced by the use of these flaps. The technique of urethral construction is described and our results are discussed. (J. Ural., 140: 574-576, 1988) The goals of urethral construction in primary closure of the classical exstrophic bladder are creation of adequate urethral length to enable the bladder to drop into the pelvis, closure of the pelvic ring if possible over the neourethra, and cosmetic and functional revision of the external genitalia with anatomically correct placement of the urethral meatus. 1 Duckett used the shiny skin at the lower lateral margins of the exstrophic bladder (paraexstrophy skin) in male subjects to bridge the gap between the bladder and neourethra after urethral plate transection, which enhances the penile lengthening phase of repair. 2 -• We now routinely use paraexstrophy skin flaps for urethral construction during primary closure of the exstrophic bladder in all children with the classical exstrophy complex. In addition to facilitating the achievement of the aforementioned goals, it also decreases the difficulty of subsequent bladder neck revision for incontinence and increases the ultimate continence zone after the revision. We used paraexstrophy skin flaps in 4 female neonates to achieve satisfactory urethral length. The advocacy of the use of these flaps in female subjects is discussed. METHOD
Primary closure was undertaken within 48 hours of birth in all 4 cases. Paraexstrophy skin was identified with lateral traction on surrounding skin and the flaps were marked (fig. 1, A). Paraexstrophy flaps approximately 2 cm. long and of maximal width were then elevated taking care to preserve their blood supply. These flaps were assessed carefully and any nonviable flap tissue was removed before urethral construction. Division of the urethral plate at the bladder neck allowed the bladder to move cephalad into a more intra-abdominal position (fig. 1, B). The flaps were then transposed medially and the posterior wall was closed with a running full thickness suture of absorbable material. In all cases 5F ureteral stents and an 8F urethral stent were brought out through the neourethra (fig. 1, C). After bladder neck closure the flaps were closed anteriorly with a running subcutaneous suture and anastomosed to the bladder neck with interrupted sutures (fig. 1, D). A suprapubic drainage catheter was placed. The pubic rami were approximated over the neourethra_ In 1 case pelvic ring approximation was achieved with a plastic cable connector as described by Hanna. 5 With good pelvic ring closure approximation of the abdominal wall was easily achieved. Clitoroplasty then joined the bifid clitoral halves. Urethral meatal skin was tacked down Accepted for publication January 12, 1988. * Current address: 3313 Peace Court, Anchorage, Alaska 99508.
below the clitoris, thus creating proper anatomical relationship (fig. 2). Ureteral stents were removed 1 week postoperatively and the urethral stent was removed on day 10. The newborns were placed in Bryant's traction for 4 weeks. The suprapubic tube was clamped at 5 weeks and removed 1 week later after residual urine volumes were documented to be minimal. RESULTS
Mean duration of followup in our patients is 2 years. In all cases the neourethra created by this process was at least 2 cm. long and the bladder was placed in an intrapelvic position. Subsequently, 1 child has had spreading of the bony closure leaving the anterior abdominal wall and bladder intact. Two infants have undergone a Young-Dees-Leadbetter bladder neck revision with concomitant augmentation cystoplasty. In these cases bladder neck reconstruction was made simpler by the significant urethral length that was achieved at initial closure. The children have been maintained free of infection with intermittent catheterization and both remain continent. In 1 infant multiple bladder stones developed 14 months after closure. Urethral calibration revealed a diameter of less than 5F and a bladder capacity of 10 cc. No ureteral reflux or upper tract dilatation has been noted. Bladder neck revision and augmentation cystoplasty will be performed when she is 2 to 3 years old. In the child with the nylon fastener closure the plastic cable connector eroded through the pubic rami and into the vagina (the urethral meatus being posterior to the erosion site) 4 months postoperatively. Transvaginal removal of the connector was accomplished without incident, and the child continues to have excellent pelvic approximation. DISCUSSION
The use of paraexstrophy skin flaps as originally described for urethral lengthening in male subjects with classical exstrophy is shown to be equally beneficial in primary closure of female infants. Duckett described the shiny skin adjacent to the exstrophied bladder as "mucosa-like" with a lack of skin appendages making it an excellent urethral substitute. 2- 4 Care must be taken to limit the flaps only to that shiny hairless skin. Likewise the flaps are random flaps and caution with length-to-width ratios must be taken. The importance of gaining enough length to enable intrapelvic positioning of the bladder has been stressed by Jeffs. 1 The majority of infants undergoing early closure of the exstrophied bladder will require bladder neck revision and possible augmen574
PARAEXSrfROPHY FLAPS FOR. UR,ETHRAL CO!>JSTRUCTION OF CLASSICAL El'~STROPHY
575
A
C
Fm. L A, identification and harvesting of paraexstrophy skin flaps. Although flaps are initially harvested as noted, they are later trimmed back to viable tissue before urethral construction. B, urethral plate divided at bladder neck with flaps elevated. C, posterior wall of urethra closed with subsequent stent placement. D, anterior urethral wall and bladder closure with suprapubic tube in place.
tation cystoplasty. Regardless of the technique used, subsequent bladder neck revision is facilitated by the increased existing urethral length and the resulting intra-abdominal bladder position if paraexstrophy flaps are used at the time of initial closure. The goal of genitourinary reconstruction in these infants should be a good cosmetic as well as functional result. With the use of paraexstrophy flaps for primary closure in female patients the cosmetic closure of the external genitalia can be essentially normal. Further procedures need to be directed only at the achievement of continence and the correction of vesicoureteral reflux. At puberty it may be necessary to rotate pubic hair-bearing flaps to the midline and vaginal introital stenosis may be corrected using a V -flap of perineal skin. 6 • 7
In the exstrophy patient bladder outlet obstruction has the same ill effects as it does with a normal bladder. 8 These obstructive phenomena, superimposed on an incompetent ureterovesical junction secondary to exstrophy, can cause rapid loss of renal parenchyma. It is imperative that the neourethra be of adequate caliber and that careful followup be maintained. Our followup includes cystoscopy when the patient is 3 to 6 months old and catheterized residuals at 6-month intervals until definitive anti-incontinence and antireflux surgery is completed. U rodynamic studies and voiding cystourethrograms are performed if residuals increase or recurrent urinary tract infections occur. Jeffs recommends staged closure with the primary closure creating an "incontinent epispadias" state. 1 While our patients are not epispadiac after closure, our aim is for incon-
576
SPINDEL, WINSLOW AND JORDAN
tinence with reflux being treated when the child is 2 to 4 years old. The infant with bladder stones had a tight urethra that most likely was secondary to scru:ring of the flaps. She has responded well to interval dilations and the urethral caliber will be augmented at bladder neck reconstruction. In the infant whose pelvic ring closure was achieved with the nylon fastener no sequelae of the vaginal erosion have been noted. We believe that these fasteners are an excellent adjunct for use in initial pelvic closure and in the future we would probably remove the device at about 6 to 8 weeks after initial closure. REFERENCES 1. Jeffs, R. D.: Exstrophy and cloaca! exstrophy. Urol. Clin. N. Amer.,
Fm. 2. Final result demonstrates satisfactory external genitalia configuration.
5: 127, 1978. 2. Duckett, J. W., Jr.: Epispadias. Urol. Clin. N. Amer., 5: 107, 1978. 3. Duckett, J. W. and Caldamone, A.: Bladder exstrophy. AUA Update Series, vol. Ill, lesson 13, 1984. 4. Duckett, J. W.: Use of paraexstrophy skin pedicle grafts for correction of exstrophy and epispadias repair. Birth Defects, 13: 175, 1977. 5. Hanna, M. K.: Approximation of pubic bones in closure of bladder exstrophy. Society for Pediatric Urology Newsletter, February 24, 1987. 6. Horton, C. E. and Devine, C. J., Jr.: Repair of hypospadias and epispadias. Society for Pediatric Urology Newsletter, December 14, 1982. 7. Sadove, R. and Horton, C.: Utilizing full thickness skin grafts for vaginal reconstruction. In preparation. 8. Hendren, W. H.: Exstrophy of the bladder. Birth Defects, 13: 207, 1977.
THE JOURNAL OF UROLOGY
Printed in U.S.A.
Copyright © 1988 by The Williams & Wilkins Co.
THE USE OF PARAEXSTROPHY FLAPS FOR URETHRAL CONSTRUCTION IN NEONATAL GIRLS WITH CLASSICAL EXSTROPHY MICHAEL R. SPINDEL,* BOYD H. WINSLOW
AND
GERALD H. JORDAN
From the Eastern Virginia Medical School, Norfolk, Virginia
ABSTRACT
Primary closure of classical exstrophy was performed with paraexstrophy skin flaps for urethral lengthening in 4 female neonates. In all cases more than 2.0 cm. of urethral length were achieved. Efficacy of bladder closure and subsequent bladder neck revision were enhanced by the use of these flaps. The technique of urethral construction is described and our results are discussed. (J. Ural., 140: 574-576, 1988) The goals of urethral construction in primary closure of the classical exstrophic bladder are creation of adequate urethral length to enable the bladder to drop into the pelvis, closure of the pelvic ring if possible over the neourethra, and cosmetic and functional revision of the external genitalia with anatomically correct placement of the urethral meatus. 1 Duckett used the shiny skin at the lower lateral margins of the exstrophic bladder (paraexstrophy skin) in male subjects to bridge the gap between the bladder and neourethra after urethral plate transection, which enhances the penile lengthening phase of repair. 2 -• We now routinely use paraexstrophy skin flaps for urethral construction during primary closure of the exstrophic bladder in all children with the classical exstrophy complex. In addition to facilitating the achievement of the aforementioned goals, it also decreases the difficulty of subsequent bladder neck revision for incontinence and increases the ultimate continence zone after the revision. We used paraexstrophy skin flaps in 4 female neonates to achieve satisfactory urethral length. The advocacy of the use of these flaps in female subjects is discussed. METHOD
Primary closure was undertaken within 48 hours of birth in all 4 cases. Paraexstrophy skin was identified with lateral traction on surrounding skin and the flaps were marked (fig. 1, A). Paraexstrophy flaps approximately 2 cm. long and of maximal width were then elevated taking care to preserve their blood supply. These flaps were assessed carefully and any nonviable flap tissue was removed before urethral construction. Division of the urethral plate at the bladder neck allowed the bladder to move cephalad into a more intra-abdominal position (fig. 1, B). The flaps were then transposed medially and the posterior wall was closed with a running full thickness suture of absorbable material. In all cases 5F ureteral stents and an 8F urethral stent were brought out through the neourethra (fig. 1, C). After bladder neck closure the flaps were closed anteriorly with a running subcutaneous suture and anastomosed to the bladder neck with interrupted sutures (fig. 1, D). A suprapubic drainage catheter was placed. The pubic rami were approximated over the neourethra_ In 1 case pelvic ring approximation was achieved with a plastic cable connector as described by Hanna. 5 With good pelvic ring closure approximation of the abdominal wall was easily achieved. Clitoroplasty then joined the bifid clitoral halves. Urethral meatal skin was tacked down Accepted for publication January 12, 1988. * Current address: 3313 Peace Court, Anchorage, Alaska 99508.
below the clitoris, thus creating proper anatomical relationship (fig. 2). Ureteral stents were removed 1 week postoperatively and the urethral stent was removed on day 10. The newborns were placed in Bryant's traction for 4 weeks. The suprapubic tube was clamped at 5 weeks and removed 1 week later after residual urine volumes were documented to be minimal. RESULTS
Mean duration of followup in our patients is 2 years. In all cases the neourethra created by this process was at least 2 cm. long and the bladder was placed in an intrapelvic position. Subsequently, 1 child has had spreading of the bony closure leaving the anterior abdominal wall and bladder intact. Two infants have undergone a Young-Dees-Leadbetter bladder neck revision with concomitant augmentation cystoplasty. In these cases bladder neck reconstruction was made simpler by the significant urethral length that was achieved at initial closure. The children have been maintained free of infection with intermittent catheterization and both remain continent. In 1 infant multiple bladder stones developed 14 months after closure. Urethral calibration revealed a diameter of less than 5F and a bladder capacity of 10 cc. No ureteral reflux or upper tract dilatation has been noted. Bladder neck revision and augmentation cystoplasty will be performed when she is 2 to 3 years old. In the child with the nylon fastener closure the plastic cable connector eroded through the pubic rami and into the vagina (the urethral meatus being posterior to the erosion site) 4 months postoperatively. Transvaginal removal of the connector was accomplished without incident, and the child continues to have excellent pelvic approximation. DISCUSSION
The use of paraexstrophy skin flaps as originally described for urethral lengthening in male subjects with classical exstrophy is shown to be equally beneficial in primary closure of female infants. Duckett described the shiny skin adjacent to the exstrophied bladder as "mucosa-like" with a lack of skin appendages making it an excellent urethral substitute. 2- 4 Care must be taken to limit the flaps only to that shiny hairless skin. Likewise the flaps are random flaps and caution with length-to-width ratios must be taken. The importance of gaining enough length to enable intrapelvic positioning of the bladder has been stressed by Jeffs. 1 The majority of infants undergoing early closure of the exstrophied bladder will require bladder neck revision and possible augmen574
PARAEXSrfROPHY FLAPS FOR. UR,ETHRAL CO!>JSTRUCTION OF CLASSICAL El'~STROPHY
575
A
C
Fm. L A, identification and harvesting of paraexstrophy skin flaps. Although flaps are initially harvested as noted, they are later trimmed back to viable tissue before urethral construction. B, urethral plate divided at bladder neck with flaps elevated. C, posterior wall of urethra closed with subsequent stent placement. D, anterior urethral wall and bladder closure with suprapubic tube in place.
tation cystoplasty. Regardless of the technique used, subsequent bladder neck revision is facilitated by the increased existing urethral length and the resulting intra-abdominal bladder position if paraexstrophy flaps are used at the time of initial closure. The goal of genitourinary reconstruction in these infants should be a good cosmetic as well as functional result. With the use of paraexstrophy flaps for primary closure in female patients the cosmetic closure of the external genitalia can be essentially normal. Further procedures need to be directed only at the achievement of continence and the correction of vesicoureteral reflux. At puberty it may be necessary to rotate pubic hair-bearing flaps to the midline and vaginal introital stenosis may be corrected using a V -flap of perineal skin. 6 • 7
In the exstrophy patient bladder outlet obstruction has the same ill effects as it does with a normal bladder. 8 These obstructive phenomena, superimposed on an incompetent ureterovesical junction secondary to exstrophy, can cause rapid loss of renal parenchyma. It is imperative that the neourethra be of adequate caliber and that careful followup be maintained. Our followup includes cystoscopy when the patient is 3 to 6 months old and catheterized residuals at 6-month intervals until definitive anti-incontinence and antireflux surgery is completed. U rodynamic studies and voiding cystourethrograms are performed if residuals increase or recurrent urinary tract infections occur. Jeffs recommends staged closure with the primary closure creating an "incontinent epispadias" state. 1 While our patients are not epispadiac after closure, our aim is for incon-
576
SPINDEL, WINSLOW AND JORDAN
tinence with reflux being treated when the child is 2 to 4 years old. The infant with bladder stones had a tight urethra that most likely was secondary to scru:ring of the flaps. She has responded well to interval dilations and the urethral caliber will be augmented at bladder neck reconstruction. In the infant whose pelvic ring closure was achieved with the nylon fastener no sequelae of the vaginal erosion have been noted. We believe that these fasteners are an excellent adjunct for use in initial pelvic closure and in the future we would probably remove the device at about 6 to 8 weeks after initial closure. REFERENCES 1. Jeffs, R. D.: Exstrophy and cloaca! exstrophy. Urol. Clin. N. Amer.,
Fm. 2. Final result demonstrates satisfactory external genitalia configuration.
5: 127, 1978. 2. Duckett, J. W., Jr.: Epispadias. Urol. Clin. N. Amer., 5: 107, 1978. 3. Duckett, J. W. and Caldamone, A.: Bladder exstrophy. AUA Update Series, vol. Ill, lesson 13, 1984. 4. Duckett, J. W.: Use of paraexstrophy skin pedicle grafts for correction of exstrophy and epispadias repair. Birth Defects, 13: 175, 1977. 5. Hanna, M. K.: Approximation of pubic bones in closure of bladder exstrophy. Society for Pediatric Urology Newsletter, February 24, 1987. 6. Horton, C. E. and Devine, C. J., Jr.: Repair of hypospadias and epispadias. Society for Pediatric Urology Newsletter, December 14, 1982. 7. Sadove, R. and Horton, C.: Utilizing full thickness skin grafts for vaginal reconstruction. In preparation. 8. Hendren, W. H.: Exstrophy of the bladder. Birth Defects, 13: 207, 1977.