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Complications of Paraexstrophy Skin Flaps in the Reconstruction of Classical Bladder Exstrophy
0022-534 7 /93/1 502 062 7$03.00/0 THE JOURNAL OF UROLOGY Copyright © 1993 by AMERICAN UROLOGICAL ASSOCIATION, INC.
Vol. 150, 627-630, August 1993 Printed in U. S. A.
Exstrop h y/Epispadias COMPLICATIONS OF PARAEXSTROPHY SKIN FLAPS IN THE RECONSTRUCTION OF CLASSICAL BLADDER EXSTROPHY JOHN P . GEARHART, * DENNIS S . PEPPAS AND ROBERT D. JEFFS From the Division of Pediatric Urology, Department of Urology, James Buchanan Brady Urological Institute, The Johns Hopkins Hospital, The Johns Hopkins University School of Medicine, Baltimore, Maryland
ABSTRACT
We reviewed the cases of the exstrophy/epispadias complex treated at our institution between July 1976 and April 1992. A total of 78 patients was identified who had paraexstrophy skin flaps used in the bladder closure, of whom 31 (40%) had a complication as a result of the flaps. The main complication encountered was a urethral stricture where the paraexstrophy skin flaps joined the urethral plate area. Multiple maneuvers were undertaken to correct these problems, including direct vision internal urethrotomy (12 cases), multiple urethral dilations (4), open revision (3) and full thickness skin grafts (5). Seven patients had such a complex stricture situation that they required either continent urinary diversion (5), colon conduit diversion (1) or cutaneous ureterostomy (1), the latter 2 patients having undergone vesicostomy elsewhere before referral. Of the remaining 24 patients who did not undergo a diversionary procedure 12 have undergone an epispadias repair and bladder neck reconstruction, 7 underwent an epispadias repair and 5 await further treatment. Freedom from complications in the initial closure of exstrophy significantly improves the chances of successful reconstruction. The avoidance of problems leading to obstruction, infection, hydrone phrosis and reflux nephropathy will provide better kidneys regardless of bladder suitability for function or augmentation. Our use of paraexstrophy flaps has decreased but when they are required, special care in design, placement and followup is advised to avoid complicating strictures and their sequelae. KEY WORDS:
bladder exstrophy, skin transplantation, surgical flaps
Since Duckett developed and popularized the use of the shiny skin at the lower lateral margins of the exstrophic bladder (paraexstrophy skin) to aid in urethral lengthening and bladder closure, many boys and girls have undergone this procedure. 1 • 2 The purpose of these flaps is to bridge the gap between the bladder and urethra after urethral plate transection to lengthen the urethral groove. Concerns about the incidence of stricture and other urethral complications have led us to review the approach to urethral reconstruction during primary bladder exstrophy closure. While others have popularized the use of paraexstrophy skin flaps in the reconstruction of bladder exstrophy, we have sig nificantly limited the use of skin flaps. 1 -4 Concerns about the sequelae of a stricture in recently closed exstrophy with a small bladder capacity and bilateral vesicoureteral reflux has led us to review the approach to urethral reconstruction during pri mary bladder closure. We discuss the use of paraexstrophy skin flaps, their complications and methods of management of these complications.
excoriation of the squamous epithelium exposed to continuous contact with urine to impassable urethral stenosis (see table). Of these cases closure was performed at our institution in 2 and elsewhere in 29. Children presented with infection most frequently (13), with pyelonephritis being seen in 9 patients. Hydronephrosis with or without hydrometer was demonstrated in 8 patients. In addition, 2 patients presented with bladder calculi, including l in acute urinary retention, and 1 with stricture and recurrent vesicoureteral reflux. Six children were completely asympto matic and were noted to have stricture at routine cystoscopy in advance of epispadias repair. In addition, 5 patients among the group had urethral pain associated with excessive desquamation of the squamous epithelial flap, and occasional ulceration and bleeding. These patients intermittently had significant dysuria. Dilation and subsequent intermittent catheterization were
MATERIALS AND METHOD S
I n a recent review o f exstrophy patients seen at the Johns Hopkins Children's Center 78 were identified in whom para exstrophy skin was used to lengthen the divided urethra. Of these 78 patients 31 (40%) had strictures related to the para exstrophy flaps, while other complications ranged from painful * Requests for reprints: Division of Pediatric Urology, Marburg 149, The Johns Hopkins Hospital, Baltimore, Maryland 2 1 205. 627
Complications of paraexstrophy skin flaps No. Pts. Stenosis leading to vesicostomy or perma nent diversion Stricture requiring patch graft or revision Stricture managed by direct vision internal urethrotomy Stricture responding to dilation Total Urethral pain with exfoliative dermatitis of urethral flaps among the aforementioned patients
7 8 12 4 :IT 5
628
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sufficient to manage the obstruction in 4 patients. Direct vision internal urethrotomy also was required in 12 patients, of whom 3 required 3 urethrotomy procedures. Major revision or patch graft urethroplasty was necessary in 8 patients to provide drainage primarily or to maintain adequate caliber in the course of subsequent epispadias or bladder neck plasty. Complex ste nosis and scarring led to vesicostomy in 2 cases, both of which were subsequently diverted. In 5 other patients a complex stricture problem precluded bladder neck plasty with urethral voiding, and continent diversion was the method of manage ment. Thus, of the 78 patients 31 (40%) had complications attrib utable to the urethral lengthening with paraexstrophy flaps. The remaining 60% had no problems with the urethra initially or after subsequent epispadias repair. Difficulty in catheteri zation because of irregularity following epispadias repair oc curred frequently but its occurrence could not be determined accurately in all patients with paraexstrophy flaps. Mean fol lowup of these 31 patients was 3.5 years. RESULTS OF STRICTURE MANAGEMENT
Diverted cases. All patients with complex stricture who
underwent continent urinary diversion are dry on intermittent catheterization, although 1 required a stomal revision second ary to scarring around the stoma of the Benchekroun repair. Of the 7 patients who underwent either continent urinary diversion or standard colonic conduit 6 regained normal upper tracts. In 1 patient, who underwent end ureterostomy when vesicostomy failed to resolve upper tract changes with infection, infection has been controlled and the upper tracts are improv ing 6 months postoperatively. Reconstructed cases. In 24 patients local management of the stricture was performed and they did not need diversion. Of 12 patients who underwent urethrotomy 3 required multiple inci sions. These 3 patients would now be treated by patch graft urethroplasty or stricture revisions earlier in the course as a result of our experience. One patient who underwent a patch graft required direct vision internal urethrotomy at the proxi mal end of the repair to provide free drainage at the bladder outlet. In the remaining patients dilations followed by periodic intermittent catheterization were sufficient to manage the blad der outlet problem. Among the 24 patients responding to stric ture treatment 12 have completed epispadias repair and bladder neck reconstruction, including 9 who have a dry interval of greater than 4 hours and are dry at night, and 2 who have a dry interval of 3 hours and are dry at night. One patient remains incontinent day and night. DISCUSSION
Clinical evidence has shown the importance of a secure initial bladder reconstruction in the staged approach to exstrophy repair.5 Formerly, multiple factors have been identified, which decrease the potential for eventual capacity in the bladder exstrophy patient, including multiple bladder closures, recur rent infections, bladder prolapse, dehiscence, bladder calculi and/or vesicostomy.6 Therefore, in addition to bladder closure and secure pelvic fixation, care must be taken to construct a bladder outlet and urethra with an adequate caliber so that problems with urinary tract infections and reflux, and their sequelae are obviated. At the time of closure patient age, size of bladder, degree of separation of the pubis, penile size and length of the urethral groove are considerations in manage ment. We use osteotomy as an adjunct to closure frequently, even in those patients presenting at birth. The penis in some male patients has length and a reasonable distance from the glans to the verumontanum so that urethral lengthening by paraexstrophy skin is not required. The poste rior placement of the bladder neck and prostate, and the release of the suspensory ligaments and anterior crural attachments to
the inferior rami are all that is required for penile management initially. When later growth has occurred and epispadias repair is undertaken, at approximately age 2 years, the Cantwell Ransley procedure will provide for additional correction of chordee.7• 8 In female patients paraexstrophy skin is almost never required in the management of the initial closure. Lengthening of the urethral groove with flaps during closure is undertaken only after careful consideration. Of 44 patients treated primarily by us, paraexstrophy flaps have been used only 12 times and 2 of these cases were treated for stricture. In addition, in our last 22 patients only 3 have had paraexstrophy flaps created.9 When the distance between the verumontanum and the glans penis is short but the corpora cavernosa have reasonable length from their attachment at the inferior rami to their termination in the glans, then lengthening of the open corpus spongiosum and urethral mucosa can be considered.10• 11 Duckett introduced the use of the shiny skin adjacent to the exstrophied bladder, which is "mucosa-like" with a lack of skin appendages. Paraexstrophy flaps of this tissue are interposed between the divided ends of the urethra distal to the open prostatic urethral plate.2 Misuse of this tissue and these flaps can result in contracture or necrosis of this tissue with devel opment of subsequent stricture and its sequelae. At the initial closure we mobilize the bladder and prostatic plate from its attachments to the rectus abdominis fascia, the separated ends of the pubis and the anterior ends of the inferior rami of the pubis. This procedure allows the bladder and prostate to assume a position posterior to the subsequent closure of the separated pubic bones and the approximation of the rectus abdominis fascia. The redundancy gained by freeing the suspensory tissue of the penis and the division of the anterior attachments of the crura of the corpora cavernosa will indicate the need for lengthening of the corpus spongiosum and the open urethra. Division of the urethra with a V incision distal to the verumontanum provides for separation of the 2 ends of the urethra to the extent that the freed corpora caver nosa will allow. The bladder and prostatic urethra are closed in the midline, establishing throughout mucosa! and muscular continuity. With an appropriately placed incision into the adjacent para exstrophy skin a rotation flap with blood supply laterally and distally can be rotated into the defect with good expectation of viability (see figure). No attempt is made to tubularize this skin. The wide (14F) prostatic opening is sutured to the rotation flap posteriorly, and laterally and anteriorly to the skin margins as the fascia!, pubic and skin closure proceeds. Stricture at the outlet is unlikely and should it occur it is superficial, easily detected and easily managed. To prevent trauma to the tissues in this area no stent or drainage catheter is allowed to exit from this prostatic urethral opening. In 20 of the 31 patients (64.5%) with complications related to the paraexstrophy flaps stenting catheters were brought out through the neourethra postoperatively. These stenting catheters along with normal postoperative swelling could cause ischemia of paraexstrophy skin flaps and its sequelae.12 The bladder outlet should be measured with a catheter or sound before the suprapubic tube is removed and again in the postoperative period.6 The initial presentation at the time of closure can be a hypoplastic penis with virtually no distance between the veru montanum and glans, and short corpora cavernosa that scarcely meet in the midline. Careful multidisciplinary consideration for possible female sex of rearing for such a male classical exstrophy patient can be given. This course of action has been used only once by the senior author (R. D. J.). In choosing to use paraexstrophy skin for urethral length ening the full thickness addition is expected to grow propor tionally to subsequent cavernous body growth. This growth does not always occur with or without testosterone stimulation because underlying scar may restrict epithelial and cavernous growth. Subsequently, when the elongated urethra is closed in
PARAEXSTROPHY SKIN FLAPS IN RECO N STRUCTI O N OF BLADDER EXSTROPHY
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U m b il t c al lissue exc i s e d
When urethral lengthening is undertaken at time of bladder closure, division of urethral plate distal to membranous urethra and verumontanum is accomplished with oblique incisions from lateral border to mid urethral line, creating point directed towards glans. Dissection, freeing corpora from prostatic plate, suspensory tissue and inferior pubic ramus will allow additional length to be obtained and corpora to be joined in mid.line. Paraexstrophy skin needed to fill defect in urethra can be judged, and with single lateral incision on each side this skin can be rotated toward midline, avoiding undue redundancy and dissection of skin while reducing chance of later complications. Ventral prepuce should not be used at this stage for urethral lengthening. Reprinted with permission. 3 epispadias repair, irregularities are frequent and catheterization for future investigations or management can be difficult or impossible. Complications related to urethral lengthening by paraexstro phy skin undertaken at the time of bladder closure include stricture, urethral irritation, chronic bleeding, diverticulum formation and inability to perform catheterization easily. If paraexstrophy flaps are poorly constructed and become devi talized the development of a stricture is almost certain to occur. An obstruction secondary to a stricture superimposed on an already incompetent ureterovesical junction can cause severe febrile urinary tract infections with rapid loss of renal par enchyma. A febrile urinary tract infection or the development of hydronephrosis at followup ultrasound strongly suggests outlet narrowing. If a stricture is suspected, cystoscopy and posterior urethral calibration should be performed. If the stricture appears soft, simple dilation followed by daily intermittent catheterization by the parents for several months should be done. If the stricture is not easily passable direct vision internal urethrot omy is performed. After direct vision internal urethrotomy an indwelling catheter is left for 2 weeks followed by daily cathe terization by the parents again for several months. If the stricture is distal we have been able to revise it by various advancement flaps into the suture line. However, if the stricture is more proximal and p atch graft is required, the interpubic bar will need to be incised to obtain adequate exposure for graft placement into the strictured area. One should try to temporize with dilations or direct vision internal urethrotomy for several months after the initial closure so that the pelvic girdle is completely healed and the closure will not be compromised. Even if the stricture is difficult to manage and requires an indwelling catheter, this is preferable to performing a vesicos tomy. In the patients referred to us who had undergone a vesicostomy the stricture became impassable and the bladder capacity almost nonexistent to the point that both required urinary diversion. Exstrophy still presents a formidable challenge to the recon structive surgeon. However, a successful result can be obtained with careful attention to detail. The complication rate of 40% with the use of paraexstrophy flaps in this predominantly
referred group of patients is high. These complications can be avoided by performing urethral lengthening only when abso lutely necessary. Special care must be taken in constructing the flaps of paraexstrophy skin if lengthening of the urethra is unavoidable. Techniques, such as internal urethrotomy, patch graft urethroplasty and continent urinary diversion, are avail able to allow either continuation of staged reconstruction or salvage of the upper tract when complications occur. REFERENCES
1. Duckett, J. W., Jr.: Epispadias. Urol. Clin. N. Amer., 5: 107, 1978. 2. Duckett, J. W.: Use of paraexstrophy skin pedicle flaps for correc tion of exstrophy and epispadias repair. Birth Defects, 1 3 : 175, 1977. 3. Gearhart, J. P. and Jeffs, R. D.: Exstrophy of the bladder, epispa dias, and other bladder anomalies. In: Campbell's Urology, 6th ed. Edited by P. C. Walsh, A. B. Retik, T. A. Stamey and E. D. Vaughan, Jr . . Philadelphia: W. B. Saunders Co., vol. 2, chapt. 46, p. 1772, 1992. 4. Spindel, M. R., Winslow, B. H. and Jordan, G. H.: The use of paraexstrophy flaps for urethral construction in neonatal girls with classical exstrophy. J. Urol., 140: 574, 1988. 5. Husmann, D. A., McLorie, G. A. and Churchill, B. M.: Closure of the exstrophic bladder: an evaluation of the factors leading to its success and its importance on urinary incontinence. J. Urol., part 2, 1 4 2 : 522, 1989. 6. Gearhart, J. P.: The failed bladder exstrophy repair: evaluation and management. Urol. Clin. N. Amer., 18: 687, 1991. 7. Ransley, P. G., Duffy, P. G. and Wollen, M.: Bladder exstrophy closure and epispadias repair. In: Operative Surgery-Paediatric Surgery, 4th ed., London: Butterworths, p. 620, 1989. 8. Gearhart, J. P., Leonard, M. P., Burgers, J. K. and Jeffs, R. D.: Cantwell-Ransley technique for repair of epispadias. J. Urol., 148: 851, 1992. 9. Jeffs, R. D., Gearhart, J. P. and Peppas, D. S.: Exstrophy without augmentation. The Hopkins experience. Presented at annual meeting of American Academy of Pediatrics, Urology Section, San Francisco, California, October 10, 1992. 10. Kelly, J. H. and Eraklis, A. J.: A procedure for lengthening the phallus in boys with exstrophy of the bladder. J. Ped. Surg., 6: 645, 1971. 11. Johnston, J. H.: Lengthening of the congenital or acquired short penis. Brit. J. Urol., 46: 685, 1974. 12. Gearhart, J. D. and Jeffs, R. D.: Complications of exstrophy and
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PARAEXSTROPHY SKIN FLAPS IN RECONSTRUCTION OF BLADDER EXSTROPHY epispadias. In: Complications of Urologic Surgery, 2nd ed., Ed ited by R. B. Smith and R. M. Ehrlich. Philadelphia: W. B. Saunders Co., chapt. 41, p. 569, 1990.
EDITORIAL COMMENT We originally reported the use of paraexstrophy skin flaps in 1976. Before then there was a high dehiscence rate with bladder closure, whether in the newborn period or later. Dividing the urethral plate and dropping the prostatic urethra with prostate and bladder neck back into the pelvis with penile lengthening at the initial stage clearly have been advantageous toward a more solid abnormal wall closure. How ever, the gap that was left between the prostatic urethra and penile lengthening needed to be filled with a bridge of tissue. The shiny skin lateral to the exstrophy was the logical substitute urethra. By taking these flaps with a broad base and making sure that they are not too long, these flaps can be extended beside the prostatic urethra and up to the bladder neck, making a wide open 12F to 14F closure. In our 29 cases using paraexstrophy skin we have had just 1 stricture that required revision. Two patients had bladder neck dysfunction, despite a wide open channel with the bladder still retaining urine, and required intermittent catheterization or a drainage procedure. This report serves well as a caveat to the potential complication of dividing and extending the urethra with paraexstrophy flaps. One must pay great attention to performing this procedure correctly. Previous drawings by the authors demonstrated the paraexstrophy flaps brought to the end of the prostatic urethra (see figure in article) . This illustrates that the prostatic urethra itself was tubularized rather than the widened bladder neck area created by extending the skin up to the sides of the prostatic urethra as we have subsequently emphasized (see figure).' This approach is most likely the reason for the misconception and has led to the errors in technique that caused strictures in the past. The figure in the current article is still confusing. The authors should be commended for emphasizing this troublesome aspect of the steps in exstrophy closure. They have clearly described ways to deal with the complication. More importantly, they have offered an alteruative technique to avoid dividing the urethral plate, the latest evolution of their substantial contributions to exstrophy management.
When done properly, we believe that paraexstrophy flaps still have a place and should not be discarded yet. John W. Duckett, Jr. Department of Pediatric Urology Children 's Hospital of Philadelphia Philadelphia, Pennsylvania 1. Duckett, J. W. and Caldamone, A. A.: Anomalies of the urinary tract: bladder and urachus. In: Clinical Pediatric Urology, 2nd ed. Edited by L. King, P. P. Kelalis and A. B. Belman. Philadel phia: W. B. Saunders Co., vol. 2, pp. 725-751, 1985.
Vol. 150, 627-630, August 1993 Printed in U. S. A.
Exstrop h y/Epispadias COMPLICATIONS OF PARAEXSTROPHY SKIN FLAPS IN THE RECONSTRUCTION OF CLASSICAL BLADDER EXSTROPHY JOHN P . GEARHART, * DENNIS S . PEPPAS AND ROBERT D. JEFFS From the Division of Pediatric Urology, Department of Urology, James Buchanan Brady Urological Institute, The Johns Hopkins Hospital, The Johns Hopkins University School of Medicine, Baltimore, Maryland
ABSTRACT
We reviewed the cases of the exstrophy/epispadias complex treated at our institution between July 1976 and April 1992. A total of 78 patients was identified who had paraexstrophy skin flaps used in the bladder closure, of whom 31 (40%) had a complication as a result of the flaps. The main complication encountered was a urethral stricture where the paraexstrophy skin flaps joined the urethral plate area. Multiple maneuvers were undertaken to correct these problems, including direct vision internal urethrotomy (12 cases), multiple urethral dilations (4), open revision (3) and full thickness skin grafts (5). Seven patients had such a complex stricture situation that they required either continent urinary diversion (5), colon conduit diversion (1) or cutaneous ureterostomy (1), the latter 2 patients having undergone vesicostomy elsewhere before referral. Of the remaining 24 patients who did not undergo a diversionary procedure 12 have undergone an epispadias repair and bladder neck reconstruction, 7 underwent an epispadias repair and 5 await further treatment. Freedom from complications in the initial closure of exstrophy significantly improves the chances of successful reconstruction. The avoidance of problems leading to obstruction, infection, hydrone phrosis and reflux nephropathy will provide better kidneys regardless of bladder suitability for function or augmentation. Our use of paraexstrophy flaps has decreased but when they are required, special care in design, placement and followup is advised to avoid complicating strictures and their sequelae. KEY WORDS:
bladder exstrophy, skin transplantation, surgical flaps
Since Duckett developed and popularized the use of the shiny skin at the lower lateral margins of the exstrophic bladder (paraexstrophy skin) to aid in urethral lengthening and bladder closure, many boys and girls have undergone this procedure. 1 • 2 The purpose of these flaps is to bridge the gap between the bladder and urethra after urethral plate transection to lengthen the urethral groove. Concerns about the incidence of stricture and other urethral complications have led us to review the approach to urethral reconstruction during primary bladder exstrophy closure. While others have popularized the use of paraexstrophy skin flaps in the reconstruction of bladder exstrophy, we have sig nificantly limited the use of skin flaps. 1 -4 Concerns about the sequelae of a stricture in recently closed exstrophy with a small bladder capacity and bilateral vesicoureteral reflux has led us to review the approach to urethral reconstruction during pri mary bladder closure. We discuss the use of paraexstrophy skin flaps, their complications and methods of management of these complications.
excoriation of the squamous epithelium exposed to continuous contact with urine to impassable urethral stenosis (see table). Of these cases closure was performed at our institution in 2 and elsewhere in 29. Children presented with infection most frequently (13), with pyelonephritis being seen in 9 patients. Hydronephrosis with or without hydrometer was demonstrated in 8 patients. In addition, 2 patients presented with bladder calculi, including l in acute urinary retention, and 1 with stricture and recurrent vesicoureteral reflux. Six children were completely asympto matic and were noted to have stricture at routine cystoscopy in advance of epispadias repair. In addition, 5 patients among the group had urethral pain associated with excessive desquamation of the squamous epithelial flap, and occasional ulceration and bleeding. These patients intermittently had significant dysuria. Dilation and subsequent intermittent catheterization were
MATERIALS AND METHOD S
I n a recent review o f exstrophy patients seen at the Johns Hopkins Children's Center 78 were identified in whom para exstrophy skin was used to lengthen the divided urethra. Of these 78 patients 31 (40%) had strictures related to the para exstrophy flaps, while other complications ranged from painful * Requests for reprints: Division of Pediatric Urology, Marburg 149, The Johns Hopkins Hospital, Baltimore, Maryland 2 1 205. 627
Complications of paraexstrophy skin flaps No. Pts. Stenosis leading to vesicostomy or perma nent diversion Stricture requiring patch graft or revision Stricture managed by direct vision internal urethrotomy Stricture responding to dilation Total Urethral pain with exfoliative dermatitis of urethral flaps among the aforementioned patients
7 8 12 4 :IT 5
628
PARAEXSTROPHY SKIN FLAPS IN RECONSTRUCTION OF BLADDER EXSTROPHY
sufficient to manage the obstruction in 4 patients. Direct vision internal urethrotomy also was required in 12 patients, of whom 3 required 3 urethrotomy procedures. Major revision or patch graft urethroplasty was necessary in 8 patients to provide drainage primarily or to maintain adequate caliber in the course of subsequent epispadias or bladder neck plasty. Complex ste nosis and scarring led to vesicostomy in 2 cases, both of which were subsequently diverted. In 5 other patients a complex stricture problem precluded bladder neck plasty with urethral voiding, and continent diversion was the method of manage ment. Thus, of the 78 patients 31 (40%) had complications attrib utable to the urethral lengthening with paraexstrophy flaps. The remaining 60% had no problems with the urethra initially or after subsequent epispadias repair. Difficulty in catheteri zation because of irregularity following epispadias repair oc curred frequently but its occurrence could not be determined accurately in all patients with paraexstrophy flaps. Mean fol lowup of these 31 patients was 3.5 years. RESULTS OF STRICTURE MANAGEMENT
Diverted cases. All patients with complex stricture who
underwent continent urinary diversion are dry on intermittent catheterization, although 1 required a stomal revision second ary to scarring around the stoma of the Benchekroun repair. Of the 7 patients who underwent either continent urinary diversion or standard colonic conduit 6 regained normal upper tracts. In 1 patient, who underwent end ureterostomy when vesicostomy failed to resolve upper tract changes with infection, infection has been controlled and the upper tracts are improv ing 6 months postoperatively. Reconstructed cases. In 24 patients local management of the stricture was performed and they did not need diversion. Of 12 patients who underwent urethrotomy 3 required multiple inci sions. These 3 patients would now be treated by patch graft urethroplasty or stricture revisions earlier in the course as a result of our experience. One patient who underwent a patch graft required direct vision internal urethrotomy at the proxi mal end of the repair to provide free drainage at the bladder outlet. In the remaining patients dilations followed by periodic intermittent catheterization were sufficient to manage the blad der outlet problem. Among the 24 patients responding to stric ture treatment 12 have completed epispadias repair and bladder neck reconstruction, including 9 who have a dry interval of greater than 4 hours and are dry at night, and 2 who have a dry interval of 3 hours and are dry at night. One patient remains incontinent day and night. DISCUSSION
Clinical evidence has shown the importance of a secure initial bladder reconstruction in the staged approach to exstrophy repair.5 Formerly, multiple factors have been identified, which decrease the potential for eventual capacity in the bladder exstrophy patient, including multiple bladder closures, recur rent infections, bladder prolapse, dehiscence, bladder calculi and/or vesicostomy.6 Therefore, in addition to bladder closure and secure pelvic fixation, care must be taken to construct a bladder outlet and urethra with an adequate caliber so that problems with urinary tract infections and reflux, and their sequelae are obviated. At the time of closure patient age, size of bladder, degree of separation of the pubis, penile size and length of the urethral groove are considerations in manage ment. We use osteotomy as an adjunct to closure frequently, even in those patients presenting at birth. The penis in some male patients has length and a reasonable distance from the glans to the verumontanum so that urethral lengthening by paraexstrophy skin is not required. The poste rior placement of the bladder neck and prostate, and the release of the suspensory ligaments and anterior crural attachments to
the inferior rami are all that is required for penile management initially. When later growth has occurred and epispadias repair is undertaken, at approximately age 2 years, the Cantwell Ransley procedure will provide for additional correction of chordee.7• 8 In female patients paraexstrophy skin is almost never required in the management of the initial closure. Lengthening of the urethral groove with flaps during closure is undertaken only after careful consideration. Of 44 patients treated primarily by us, paraexstrophy flaps have been used only 12 times and 2 of these cases were treated for stricture. In addition, in our last 22 patients only 3 have had paraexstrophy flaps created.9 When the distance between the verumontanum and the glans penis is short but the corpora cavernosa have reasonable length from their attachment at the inferior rami to their termination in the glans, then lengthening of the open corpus spongiosum and urethral mucosa can be considered.10• 11 Duckett introduced the use of the shiny skin adjacent to the exstrophied bladder, which is "mucosa-like" with a lack of skin appendages. Paraexstrophy flaps of this tissue are interposed between the divided ends of the urethra distal to the open prostatic urethral plate.2 Misuse of this tissue and these flaps can result in contracture or necrosis of this tissue with devel opment of subsequent stricture and its sequelae. At the initial closure we mobilize the bladder and prostatic plate from its attachments to the rectus abdominis fascia, the separated ends of the pubis and the anterior ends of the inferior rami of the pubis. This procedure allows the bladder and prostate to assume a position posterior to the subsequent closure of the separated pubic bones and the approximation of the rectus abdominis fascia. The redundancy gained by freeing the suspensory tissue of the penis and the division of the anterior attachments of the crura of the corpora cavernosa will indicate the need for lengthening of the corpus spongiosum and the open urethra. Division of the urethra with a V incision distal to the verumontanum provides for separation of the 2 ends of the urethra to the extent that the freed corpora caver nosa will allow. The bladder and prostatic urethra are closed in the midline, establishing throughout mucosa! and muscular continuity. With an appropriately placed incision into the adjacent para exstrophy skin a rotation flap with blood supply laterally and distally can be rotated into the defect with good expectation of viability (see figure). No attempt is made to tubularize this skin. The wide (14F) prostatic opening is sutured to the rotation flap posteriorly, and laterally and anteriorly to the skin margins as the fascia!, pubic and skin closure proceeds. Stricture at the outlet is unlikely and should it occur it is superficial, easily detected and easily managed. To prevent trauma to the tissues in this area no stent or drainage catheter is allowed to exit from this prostatic urethral opening. In 20 of the 31 patients (64.5%) with complications related to the paraexstrophy flaps stenting catheters were brought out through the neourethra postoperatively. These stenting catheters along with normal postoperative swelling could cause ischemia of paraexstrophy skin flaps and its sequelae.12 The bladder outlet should be measured with a catheter or sound before the suprapubic tube is removed and again in the postoperative period.6 The initial presentation at the time of closure can be a hypoplastic penis with virtually no distance between the veru montanum and glans, and short corpora cavernosa that scarcely meet in the midline. Careful multidisciplinary consideration for possible female sex of rearing for such a male classical exstrophy patient can be given. This course of action has been used only once by the senior author (R. D. J.). In choosing to use paraexstrophy skin for urethral length ening the full thickness addition is expected to grow propor tionally to subsequent cavernous body growth. This growth does not always occur with or without testosterone stimulation because underlying scar may restrict epithelial and cavernous growth. Subsequently, when the elongated urethra is closed in
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U m b il t c al lissue exc i s e d
When urethral lengthening is undertaken at time of bladder closure, division of urethral plate distal to membranous urethra and verumontanum is accomplished with oblique incisions from lateral border to mid urethral line, creating point directed towards glans. Dissection, freeing corpora from prostatic plate, suspensory tissue and inferior pubic ramus will allow additional length to be obtained and corpora to be joined in mid.line. Paraexstrophy skin needed to fill defect in urethra can be judged, and with single lateral incision on each side this skin can be rotated toward midline, avoiding undue redundancy and dissection of skin while reducing chance of later complications. Ventral prepuce should not be used at this stage for urethral lengthening. Reprinted with permission. 3 epispadias repair, irregularities are frequent and catheterization for future investigations or management can be difficult or impossible. Complications related to urethral lengthening by paraexstro phy skin undertaken at the time of bladder closure include stricture, urethral irritation, chronic bleeding, diverticulum formation and inability to perform catheterization easily. If paraexstrophy flaps are poorly constructed and become devi talized the development of a stricture is almost certain to occur. An obstruction secondary to a stricture superimposed on an already incompetent ureterovesical junction can cause severe febrile urinary tract infections with rapid loss of renal par enchyma. A febrile urinary tract infection or the development of hydronephrosis at followup ultrasound strongly suggests outlet narrowing. If a stricture is suspected, cystoscopy and posterior urethral calibration should be performed. If the stricture appears soft, simple dilation followed by daily intermittent catheterization by the parents for several months should be done. If the stricture is not easily passable direct vision internal urethrot omy is performed. After direct vision internal urethrotomy an indwelling catheter is left for 2 weeks followed by daily cathe terization by the parents again for several months. If the stricture is distal we have been able to revise it by various advancement flaps into the suture line. However, if the stricture is more proximal and p atch graft is required, the interpubic bar will need to be incised to obtain adequate exposure for graft placement into the strictured area. One should try to temporize with dilations or direct vision internal urethrotomy for several months after the initial closure so that the pelvic girdle is completely healed and the closure will not be compromised. Even if the stricture is difficult to manage and requires an indwelling catheter, this is preferable to performing a vesicos tomy. In the patients referred to us who had undergone a vesicostomy the stricture became impassable and the bladder capacity almost nonexistent to the point that both required urinary diversion. Exstrophy still presents a formidable challenge to the recon structive surgeon. However, a successful result can be obtained with careful attention to detail. The complication rate of 40% with the use of paraexstrophy flaps in this predominantly
referred group of patients is high. These complications can be avoided by performing urethral lengthening only when abso lutely necessary. Special care must be taken in constructing the flaps of paraexstrophy skin if lengthening of the urethra is unavoidable. Techniques, such as internal urethrotomy, patch graft urethroplasty and continent urinary diversion, are avail able to allow either continuation of staged reconstruction or salvage of the upper tract when complications occur. REFERENCES
1. Duckett, J. W., Jr.: Epispadias. Urol. Clin. N. Amer., 5: 107, 1978. 2. Duckett, J. W.: Use of paraexstrophy skin pedicle flaps for correc tion of exstrophy and epispadias repair. Birth Defects, 1 3 : 175, 1977. 3. Gearhart, J. P. and Jeffs, R. D.: Exstrophy of the bladder, epispa dias, and other bladder anomalies. In: Campbell's Urology, 6th ed. Edited by P. C. Walsh, A. B. Retik, T. A. Stamey and E. D. Vaughan, Jr . . Philadelphia: W. B. Saunders Co., vol. 2, chapt. 46, p. 1772, 1992. 4. Spindel, M. R., Winslow, B. H. and Jordan, G. H.: The use of paraexstrophy flaps for urethral construction in neonatal girls with classical exstrophy. J. Urol., 140: 574, 1988. 5. Husmann, D. A., McLorie, G. A. and Churchill, B. M.: Closure of the exstrophic bladder: an evaluation of the factors leading to its success and its importance on urinary incontinence. J. Urol., part 2, 1 4 2 : 522, 1989. 6. Gearhart, J. P.: The failed bladder exstrophy repair: evaluation and management. Urol. Clin. N. Amer., 18: 687, 1991. 7. Ransley, P. G., Duffy, P. G. and Wollen, M.: Bladder exstrophy closure and epispadias repair. In: Operative Surgery-Paediatric Surgery, 4th ed., London: Butterworths, p. 620, 1989. 8. Gearhart, J. P., Leonard, M. P., Burgers, J. K. and Jeffs, R. D.: Cantwell-Ransley technique for repair of epispadias. J. Urol., 148: 851, 1992. 9. Jeffs, R. D., Gearhart, J. P. and Peppas, D. S.: Exstrophy without augmentation. The Hopkins experience. Presented at annual meeting of American Academy of Pediatrics, Urology Section, San Francisco, California, October 10, 1992. 10. Kelly, J. H. and Eraklis, A. J.: A procedure for lengthening the phallus in boys with exstrophy of the bladder. J. Ped. Surg., 6: 645, 1971. 11. Johnston, J. H.: Lengthening of the congenital or acquired short penis. Brit. J. Urol., 46: 685, 1974. 12. Gearhart, J. D. and Jeffs, R. D.: Complications of exstrophy and
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PARAEXSTROPHY SKIN FLAPS IN RECONSTRUCTION OF BLADDER EXSTROPHY epispadias. In: Complications of Urologic Surgery, 2nd ed., Ed ited by R. B. Smith and R. M. Ehrlich. Philadelphia: W. B. Saunders Co., chapt. 41, p. 569, 1990.
EDITORIAL COMMENT We originally reported the use of paraexstrophy skin flaps in 1976. Before then there was a high dehiscence rate with bladder closure, whether in the newborn period or later. Dividing the urethral plate and dropping the prostatic urethra with prostate and bladder neck back into the pelvis with penile lengthening at the initial stage clearly have been advantageous toward a more solid abnormal wall closure. How ever, the gap that was left between the prostatic urethra and penile lengthening needed to be filled with a bridge of tissue. The shiny skin lateral to the exstrophy was the logical substitute urethra. By taking these flaps with a broad base and making sure that they are not too long, these flaps can be extended beside the prostatic urethra and up to the bladder neck, making a wide open 12F to 14F closure. In our 29 cases using paraexstrophy skin we have had just 1 stricture that required revision. Two patients had bladder neck dysfunction, despite a wide open channel with the bladder still retaining urine, and required intermittent catheterization or a drainage procedure. This report serves well as a caveat to the potential complication of dividing and extending the urethra with paraexstrophy flaps. One must pay great attention to performing this procedure correctly. Previous drawings by the authors demonstrated the paraexstrophy flaps brought to the end of the prostatic urethra (see figure in article) . This illustrates that the prostatic urethra itself was tubularized rather than the widened bladder neck area created by extending the skin up to the sides of the prostatic urethra as we have subsequently emphasized (see figure).' This approach is most likely the reason for the misconception and has led to the errors in technique that caused strictures in the past. The figure in the current article is still confusing. The authors should be commended for emphasizing this troublesome aspect of the steps in exstrophy closure. They have clearly described ways to deal with the complication. More importantly, they have offered an alteruative technique to avoid dividing the urethral plate, the latest evolution of their substantial contributions to exstrophy management.
When done properly, we believe that paraexstrophy flaps still have a place and should not be discarded yet. John W. Duckett, Jr. Department of Pediatric Urology Children 's Hospital of Philadelphia Philadelphia, Pennsylvania 1. Duckett, J. W. and Caldamone, A. A.: Anomalies of the urinary tract: bladder and urachus. In: Clinical Pediatric Urology, 2nd ed. Edited by L. King, P. P. Kelalis and A. B. Belman. Philadel phia: W. B. Saunders Co., vol. 2, pp. 725-751, 1985.