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Augmentation Cystoplasty in the Failed Exstrophy Reconstruction
0022-534 7/88/1394-0790$2.00/0 THE JOURNAL OF UROLOGY
Vol. 139, April Printed in U.S.A.
Copyright © 1988 by The Williams & Wilkins Co.
AUGMENTATION CYSTOPLASTY IN THE FAILED EXSTROPHY RECONSTRUCTION JOHN P. GEARHART AND ROBERT D. JEFFS From the Division of Pediatric Urology, James Buchanan Brady Urological Institute, The Johns Hopkins Hospital, Baltimore, Maryland
ABSTRACT
Of the 148 patients with bladder exstrophy seen at this institution during the last 10 years 12 have ultimately required bladder augmentation. In 4 cases augmentation was performed for an inadequate bladder capacity, upper tract decompensation and dry interval of less than 1 hour after bladder neck reconstruction and epispadias repair. Likewise, 3 patients underwent augmentation for an inadequate bladder capacity and dry interval of less than 2 hours after bladder neck reconstruction and epispadias repair. Of these 7 patients 3 had undergone 2 previous bladder neck reconstructions, while 4 had undergone 1 prior repair. Five augmentations were performed for an inadequate bladder capacity before any type of continence procedure had been done. Nine patients underwent adjunctive procedures in addition to bladder augmentation, including a Young-Dees-Leadbetter procedure in 4, an artificial urinary sphincter in 3, transureteroureterostomy and psoas hitch in 1, and a Mitrofanoff procedure and bladder neck closure in 1. Of the 12 patients 11 are continent, although 9 require intermittent catheterization. There were no major complications. However, 1 artificial urinary sphincter was removed for erosion 2 years after placement. Augmentation cystoplasty has provided prolonged stability of the upper tracts and continence in these patients, and it has proved to be a successful alternative to urinary diversion in this select group of exstrophy failures. (J. Ural., 139: 790-793, 1988) The staged approach to functional closure of bladder exstrophy has gained increasing success during the last several years. 1- 3 The plan of treatment has been bladder closure with or without penile lengthening and with or without bilateral iliac osteotomy immediately after birth or as soon thereafter as possible. 2 Bladder neck reconstruction along with an antireflux procedure usually is performed when the patient is 3 years old after satisfactory bladder capacity is achieved. The epispadias repair is completed when the patient is 4 to 5 years old but it may be performed before bladder neck plasty when bladder capacity is slow to increase to acceptable size. 4 Unfortunately, not all bladders gain adequate capacity after the aforementioned reconstructive procedures. Sometimes deterioration of the upper tracts and/or urinary incontinence develops. The management options at this time include urinary diversion, a combined bladder-bowel procedure as proposed by Arap and associates, 5 an artificial urinary sphincter or a combined anti-incontinence procedure plus bladder augmentation. In the last 10 years pediatric urologists have become more aggressive in reconstructing the lower urinary tract in children with small capacity bladders. A successful outcome depends on 3 criteria: 1) the ability to achieve adequate capacity at low pressure, 2) a satisfactory dry interval and 3) the ability to empty the bladder at regular intervals. Unfortunately, not all patients achieve this success. Augmentation cystoplasty provided a means of salvage of the lower urinary tract for 12 patients during the last 10 years. Our experience with these patients is described. MATERIALS AND METHODS
Since 1976 we have treated 4 girls 1 to 17 years old and 8 boys 5 to 18 years old for small capacity bladders secondary to failed exstrophy reconstruction (see table). Of these patients 7 had initial exstrophy closure in the first week of life, 1 in the second week and 4 after 4 weeks. One of these children had undergone a colon conduit urinary diversion 2 years before undiversion and bladder augmentation for a small capacity Accepted for publication August 18, 1987.
bladder. Also, while 5 patients had only 1 previous bladder closure, 4 had 2 previous closures and 3 had undergone more than 2 closures. In addition, 4 patients had undergone 1 prior bladder neck reconstruction and 3 had undergone 2 previous bladder neck reconstructions. Five patients had had no previous bladder neck surgery. Indications for augmentation cystoplasty in these patients were an inadequate bladder capacity before bladder neck reconstruction in 5, and an inadequate bladder capacity and urinary incontinence in 7 after bladder neck reconstruction, 4 of whom demonstrated upper tract changes. The complicated history of exstrophy presentation and subsequent treatment was studied carefully and prior radiological studies were reviewed. Excretory urograms, cystograms, loopograms and endoscopy were performed as indicated. Routine urodynamic bladder evaluation was not performed, although urethral pressure profiles were obtained in 2 patients during bladder neck reconstruction. The upper urinary tracts were normal in 8 patients and hydronephrotic in 4. The creatinine clearance was greater than 40 mg./ml./1.73 m. 2 (normal 100 to 150 ml. per minute) in all patients. Bladder capacity remained at small volumes despite followup evaluation and anticholinergic medications. Early in the series bladder cycling was attempted in 3 patients with disappointing results and it was discontinued. Intermittent catheterization was discussed and taught to each child and parent in anticipation of the possible need after bladder augmentation. The augmentation of the bladder was achieved with an ileal patch in 6 patients, an ileocecal segment in 1, a sigmoid augmentation in 4 and a hindgut patch in 1. Undiversion was performed in 1 patient with a previously constructed colon conduit attached to a tubularized bladder, according to the method of Arap and associates. 5 The varied application of the bowel segments is illustrated in figure 1. Adjunctive procedures that were performed at the time of augmentation included a bladder neck plasty in 4 cases, 1 of which was a repeat, transureteroureterostomy with a psoas hitch and ureteral reimplant in 1, and a Mitrofanoff procedure 6 associated with ileal augmentation and bladder neck closure in 1.
790
AUGMENTATION CYSTOPLASTY IN FAILED EXSTROPHY RECONSTRUCTION
Findings in 12 children with exstrophy who underwent augmentation cystoplasty Surgical Repair Bowel Segment
Continence Procedure
No. Closures
Postop. Continence
1 2
Hindgut Ileocecal
Young-Dees-Leadbetter Scott*
1 2
Yes* No
3 4
Scott Arap
5
Sigmoid Sigmoid Sigmoid
1 1 1
Yes Yest Yest
6 7
Sigmoid Ileum
Young-Dees-Leadbetter
4 2
Yest Yes
8
Ileum
Scott
3
Yest
9
10
Ileum Ileum
Young-Dees-Leadbetter Young-Dees-Leadbetter
1 2
Yest Yest
11
Ileum
Closure bladder neck
2
Yest
12
Ileum
3
Yest
Pt. No.
Followup (yrs.)
Unusual Features
5
Previous Young-Dees-Leadbetter operation Prior diversion
9
4 4 8
Previous Young-Dees-Leadbetter operation, transureteroureterostomy with psoas hitch
8
Previous Young-Dees-Leadbetter operation X2 Previous Young-Dees-Leadbetter operation
2 2 2 2
Previous Young-Dees-Leadbetter operation X2 Previous Young-Dees-Leadbetter operation, Mitrofanoff procedure Previous Young-Dees-Leadbetter operation X2
1 1
* Only complication was erosion.
t Patient dry on intermittent catheterization.
In 3 patients an artificial sphincter was implanted for continence. The selected cases included an undiversion in which the sphincter was placed around the tubularized bladder and a bladder exstrophy variant in which the sphincter was placed at the upper prostatic level after removing 2 ectopic ureters from the posterior urethra. The third patient received a sphincter around a failed bladder neck plasty when he was 5 years old. All patients who underwent combined bladder augmentation and sphincter placement had complete antibiotic bowel preparations before surgery and they received intravenous broadspectrum antibiotics for 7 days postoperatively. Our experience with myelodysplastic patients who have undergone augmentation and sphincter placement simultaneously led us to continue this regimen with the exstrophy group. Since our 1 case of erosion occurred 2 years after augmentation, the open gastrointestinal tract most likely did not compromise sphincter placement.
~
.
l leal palch &. appendix with M\\rofanoff procedure
Itealpalch
s.~~
..
H\nd[lul pakh in ctoacal exstrophy
RESULTS
All 12 children have been followed postoperatively for an average of 4 years (range 1 to 9 years) with few complications. In 4 patients with upper tract changes the upper tract status has improved and in 8 it has remained stable. Vesicoureteral reflux was present in 5 patients preoperatively and it has not recurred. Also, there has been no evidence of secondary ureteral obstruction. One patient who did not have reflux previously suffered reflux after bladder augmentation and sphincter placement. This patient now has complete incontinence owing to erosion of the artificial urinary sphincter which has been removed. Of the remaining 11 patients 2 are voiding and continent, and 9 are dry on intermittent catheterization at regular intervals. Only 1 patient is wet at night. Although bacteriuria has been present occasionally in many of these patients, febrile urinary tract infections have occurred in only 2 since the bladder augmentation. All patients have been maintained on continuous prophylactic medications. There were no instances of bowel obstruction, urinary fistulas or re-diversion. Electrolyte disturbance owing to reabsorption has not occurred except in 1 patient who has received bicitrate for chronic acidosis due to the combination of a short gut and hindgut bladder augmentation in a cloaca! exstrophy. Currently, she is dry for 3 hours on intermittent catheterization and the chronic acidosis is well controlled with bicarbonate therapy. Excess mucus was a problem in some patients in the
~ $
'
* Tubutar\zed
1(.
S\!jmoid cys\oplasly Araptype
Sigmoid pakh
Y:1 ~
il-Und\verted colon condu\\ with sphincter Arap type
~t
Ileocecal clstoplasty wit spn\ncler
Fm. 1. Various applications of bowel segments to small exstrophy bladder.
immediate postoperative period but, subsequently, it has seldom required irrigation even in those using intermittent catheterization. Adequate capacity and potential continence were achieved quickly in all patients using the detubularized ileum and the sigmoid patch. The patients with an undiverted tubularized colon and a Scott sphincter also gained day and night capacity and control. The patient with a tubularized colon augmentation and an Arap urethra fashioned from the bladder was slow in gaining control, partially owing to a weak bladder neck closure and partially to bowel peristalsis. This patient now is dry for 4 hours on intermittent catheterization and she only has an occasional wet night. The patient with a tubularized ileocecal cystoplasty remains a failure owing to erosion of a sphincter that had been placed when he was young over a previously reconstructed bladder neck. This patient awaits revision.
792
GEARHART AND JEFFS DISCUSSION
The potential for the development of an adequate bladder capacity is often difficult to evaluate at birth. Because of this variable potential, strict adherence to previously recommended principles at the time of initial closure becomes important. 7 The initial bladder neck and posterior urethra should be long enough and tight enough to allow unimpeded egress of urine but not allow the anterior bladder wall to prolapse. Prolapse in our experience was a factor in 4 patients who were unable to obtain a satisfactory bladder capacity before bladder neck reconstruction. Likewise, secure pelvic ring fixation and postoperative traction maintain the closure, and prevent tension and shearing forces from altering the bladder neck and neourethra. This allows the bladder to remain deeper in the pelvis whether or not one has chosen to use paraexstrophy skin flaps. If t~e initial clos~re accomplishes the aforementioned goals, the urme can pool m the bladder during the nighttime hours and, therefore, allow the bladder to adapt gradually to larger volumes without upper tract deterioration as low pressure drainage occurs. If the initial closure does not allow for a 50 to 60 cc bladder capacity by the time the patient is 3 years old an epispadias repair can be accomplished to increase outlet re;istance and hopefully bladder capacity (fig. 2). 4 The potential for bladder capacity is decreased by each successive bladder closure and bladder neck reconstruction. The most difficult patients in this group were those who had undergone multiple bladder closures. Wound infection, bladder prolapse, bladder calculi and vesicostomy have been identified as factors that can jeopardize an initial bladder closure, and 7 of our patients had a combination of these factors. 8 Therefore, every attempt should be made at the initial operation to achieve proper closure and proper pelvic fixation to promote and ensure primary healing of the abdominal wall, bladder outlet and urethral tissue. Normally, a Young-Dees-Leadbetter reconstruction is not recommended until at least a 50 to 60 cc bladder capacity is achieved by the child. In our experience it is estimated that bladder neck reconstruction requires at least 20 cc of bladder capacity. Therefore, a small capacity bladder becomes problematic. If a bladder tends to have a borderline capacity and a substantial ".olume is used by a bladder neck operation, this procedure will only decrease bladder capacity and increase intravesical pressure with a significant risk of deterioration of the upper tracts and/or urinary leakage. A wide variety of techniques for bladder augmentation and bladder substitution have been developed. Likewise, these techniques have been applied to bladder augmentation and urinary undiversion in children. 9 - 12 However, in addition to augmentatio~, the need for ureteral reimplantation, artificial urinary sphmcter, bladder neck plasty and other adjunctive procedures must be considered carefully. Ureteral reflux or obstruction in the small bladder seems to be managed best by reimplantation with a psoas hitch13 and transureteroureterostomy combined with augmentation. However, if ureteral reimplantation is re!NEWBORN BLAODER CLOSUREj AGE 2-4
AGE 2-4
1 BLADDER CAPACITY
lI
LESS THAN 50cc
!
rEPISPADIAS REPAIRl BLADDER CAPACITY GREATER THAN 50cc
!
YOUNG DEES LEADBETTER PROCEDURE
BLADDER CAPACITY LESS THAN 50cc
~
!
50c1
BLADDER CAPACITY GREATER THAN
YOUNG DEES LEADBETTER PROCEDURE
YOUNG DEES LEADBETTER PROCEDURE AND EPISPADIAS REPAIR
1
EPISPADIAS REPAIR
YOUNG DEES LEADBETTER PROCEDURE AND BLADDER AUGMENTATION
Fm. 2. Algorithm for exstrophy management
quired in conjunction with augmentation and the bladder is not suitable for this procedure, then a detubularized sigmoid or ileocecal segment is preferred. Early in our experience nondetubularized ileocecal segment was used along with an artificial urinary sphincter. Contractions of this segment caused incontinence and, therefore, only detubularized segments currently are used. However, if ureteral reimplantation is not required then an ileal patch is preferred because of the safer ileal anastomosis, lack of contractions and ease of handling small bowel. . Obv~ously, in the child with a small bladder and no previous mcontmence procedure the choice would be a Young-DeesLeadbetter procedure in conjunction with bladder augmentation. If a~ mentioned previously, the bladder was adequate for the creation of a bladder neck but not for ureteral reimplantation, then reimplantation into a detubularized bowel segment and bladder augmentation would be preferred. Placement of an artificial urinary sphincter in a bladder exstrophy patient should likely remain as a salvage procedure after previous attempts at continence. Of our 3 patients who underwent sphincter placement 2 had undergone previously a Young-Dees-Leadbetter procedure. One sphincter eroded into the urethra 2 years after placement. Currently, sphincters should remain an option for the older child with previous bladder neck surgery who has an adequate omental pad that can be used around the previously reconstructed bladder neck. A~so, continent ~rinary diversion procedures most certainly will have a place m the reconstruction of these most problematic cases. 14 The exstrophy patient who initially undergoes closure without dehiscence or prolapse frequently can achieve a functional bladder of good capacity for subsequent bladder neck reconstruction and ureteral reimplantation. The routine use of bladder ~u~entat~on at the time of closure is not only unnecessary but it might hmder bladder adaptation to volume and bladder growth. The indications for augmentation in exstrophy patients can be divided into 3 main groups: group 1-those in whom after initial or subsequent closures and epispadias repair the bladder does not adapt to a volume of 50 cc or greater even when treated with anticholinergic medication (to which the patient should respond), 15 group 2-those who have persistent incontinence with a small bladder capacity despite what is thought to be an adequate bladder neck repair and group 3those in whom upper tract decompensation occurs with or without the onset of incontinence after bladder neck reconstruction. In our 5 group 1 patients with a small bladder capacity before a_ny incontinence operation has been done bladder augmentat10n can be performed easily with the bladder neck procedure. This initial course of management is preferred before the use of an artificial sphincter and augmentation in these patients. There were 3 group 2 patient~ with persistent leakage, 2 of whom had undergone 2 previous bladder neck procedures and 1 who had undergone a prior bladder neck reconstruction. Although the outlet resistance was thought to be adequate bladder capacity only averaged approximately 60 cc. In thi~ situation augmentation allows for a decrease in bladder pressure, and an increase in capacity and compliance. It also may allow a bladder neck with previously marginal outlet resistance to become adequate for continence. However, if a previous Young-Dees-Leadbetter procedure is deemed inadequate and the bladder capacity is judged to be adequate the choice would be a repeat Young-Dees-Leadbetter procedure before resorting to bladder .neck reconstruction and simultaneous augmentation. Likewise, if a previous Young-Dees-Leadbetter procedure is deemed inadequate and the bladder capacity is less than 50 cc and it will again be decreased by a repeat bladder neck procedure, then concomitant bladder augmentation becomes desirable.
AUGMENTATION CYSTOPLASTY IN FAILED EXSTROPHY RECONSTRUCTION
In our 4 group 3 patients who benefited from bladder augmentation upper tract decompensation occurred. This group included 1 patient who had undergone 2 previous Young-DeesLeadbetter procedures following which the bladder was small. Bladder augmentation improved and stabilized the upper urinary tract in all 4 of these patients. These 12 patients are among 148 patients seen at our institution in the last 10 years and they represent the small percentage of those who fail to achieve satisfactory function after bladder closure. Augmentation cystoplasty appears to provide prolonged stability in this group of exstrophy patients and it has proved to be a successful alternative to urinary diversion when patients and parents have accepted the adjunctive need for intermittent catheterization when necessary. REFERENCES 1. Jeffs, R. D.: Exstrophy and cloaca! exstrophy. Urol. Clin. N. Amer., 5: 127, 1978. 2. Jeffs, R. D.: Management of the exstrophy-epispadias complex and urachal anomalies. In: Campbell's Urology, 5th ed. Edited by P. C. Walsh, R. F. Gittes, A. D. Perlmutter and T. A. Stamey. Philadelphia: W. B. Saunders Co., vol. 2, sect. XIII, chapt. 43, p. 1882, 1985. 3. Jeffs, R. D., Guice, S. L. and Oesch, I.: The factors in successful exstrophy closure. J. Urol., 127: 974, 1982. 4. Oesterling, J. E. and Jeffs, R. D.: The importance of a successful initial bladder closure in the surgical management of classical bladder exstrophy: analysis of 144 patients with bladder exstrophy treated at the Johns Hopkins Hospital from 1975 to 1985.
793
J. Urol., 137: 258, 1987. 5. Arap, S., Giron, A. M. and de Goes, G. M.: Initial results of the complete reconstruction of bladder exstrophy. Urol. Clin. N. Amer., 7: 477, 1980. 6. Mitrofanoff, P.: Cystostomie continente trans-appendiculaire clans le traitement des vessies neurologiques. Chir. Ped., 21: 297, 1980. 7. Lepor, H. and Jeffs, R. D.: Primary bladder closure and bladder neck reconstruction in classical bladder exstrophy. J. Urol., 130: 1142, 1983. 8. Lowe, F. C. and Jeffs, R. D.: Wound dehiscence in bladder exstrophy: an examination of the etiologies and factors for initial failure and subsequent success. J. Urol., 130: 312, 1983. 9. Mitchell, M. E.: The role of bladder augmentation in undiversion. J. Ped. Surg., 16: 790, 1981. 10. Mitchell, M. E.: Urinary tract diversion and undiversion in the pediatric age group. Surg. Clin. N. Amer., 61: 1147, 1981. 11. Perlmutter, A. D.: Experiences with urinaryundiversion in children with neurogenic bladder. J. Urol., 123: 402, 1980. 12. Kass, E. J. and Koff, S. A.: Bladder augmentation in the pediatric neuropathic bladder. J. Urol., 129: 552, 1983. 13. Gearhart, J.P. and Woolfenden, K. A.: The vesico-psoas hitch as an adjunct to megaureter repair in childhood. J. Urol., 127: 505, 1982. 14. Mitchell, M. E. and Hensle, T. W.: Total bladder replacement in children. In: Bladder Reconstruction and Continent Urinary Diversion. Edited by L. R. King, A. R. Stone and G.D. Webster. Chicago: Year Book Medical Publishers, Inc., chapt. 21, p. 312, 1987. 15. Shapiro, E., Jeffs, R. D., Gearhart, J.P. and Lepor, H.: Muscarinic cholinergic receptors in bladder exstrophy. Insight into surgical management. J. Urol., 134: 308, 1985.
Vol. 139, April Printed in U.S.A.
Copyright © 1988 by The Williams & Wilkins Co.
AUGMENTATION CYSTOPLASTY IN THE FAILED EXSTROPHY RECONSTRUCTION JOHN P. GEARHART AND ROBERT D. JEFFS From the Division of Pediatric Urology, James Buchanan Brady Urological Institute, The Johns Hopkins Hospital, Baltimore, Maryland
ABSTRACT
Of the 148 patients with bladder exstrophy seen at this institution during the last 10 years 12 have ultimately required bladder augmentation. In 4 cases augmentation was performed for an inadequate bladder capacity, upper tract decompensation and dry interval of less than 1 hour after bladder neck reconstruction and epispadias repair. Likewise, 3 patients underwent augmentation for an inadequate bladder capacity and dry interval of less than 2 hours after bladder neck reconstruction and epispadias repair. Of these 7 patients 3 had undergone 2 previous bladder neck reconstructions, while 4 had undergone 1 prior repair. Five augmentations were performed for an inadequate bladder capacity before any type of continence procedure had been done. Nine patients underwent adjunctive procedures in addition to bladder augmentation, including a Young-Dees-Leadbetter procedure in 4, an artificial urinary sphincter in 3, transureteroureterostomy and psoas hitch in 1, and a Mitrofanoff procedure and bladder neck closure in 1. Of the 12 patients 11 are continent, although 9 require intermittent catheterization. There were no major complications. However, 1 artificial urinary sphincter was removed for erosion 2 years after placement. Augmentation cystoplasty has provided prolonged stability of the upper tracts and continence in these patients, and it has proved to be a successful alternative to urinary diversion in this select group of exstrophy failures. (J. Ural., 139: 790-793, 1988) The staged approach to functional closure of bladder exstrophy has gained increasing success during the last several years. 1- 3 The plan of treatment has been bladder closure with or without penile lengthening and with or without bilateral iliac osteotomy immediately after birth or as soon thereafter as possible. 2 Bladder neck reconstruction along with an antireflux procedure usually is performed when the patient is 3 years old after satisfactory bladder capacity is achieved. The epispadias repair is completed when the patient is 4 to 5 years old but it may be performed before bladder neck plasty when bladder capacity is slow to increase to acceptable size. 4 Unfortunately, not all bladders gain adequate capacity after the aforementioned reconstructive procedures. Sometimes deterioration of the upper tracts and/or urinary incontinence develops. The management options at this time include urinary diversion, a combined bladder-bowel procedure as proposed by Arap and associates, 5 an artificial urinary sphincter or a combined anti-incontinence procedure plus bladder augmentation. In the last 10 years pediatric urologists have become more aggressive in reconstructing the lower urinary tract in children with small capacity bladders. A successful outcome depends on 3 criteria: 1) the ability to achieve adequate capacity at low pressure, 2) a satisfactory dry interval and 3) the ability to empty the bladder at regular intervals. Unfortunately, not all patients achieve this success. Augmentation cystoplasty provided a means of salvage of the lower urinary tract for 12 patients during the last 10 years. Our experience with these patients is described. MATERIALS AND METHODS
Since 1976 we have treated 4 girls 1 to 17 years old and 8 boys 5 to 18 years old for small capacity bladders secondary to failed exstrophy reconstruction (see table). Of these patients 7 had initial exstrophy closure in the first week of life, 1 in the second week and 4 after 4 weeks. One of these children had undergone a colon conduit urinary diversion 2 years before undiversion and bladder augmentation for a small capacity Accepted for publication August 18, 1987.
bladder. Also, while 5 patients had only 1 previous bladder closure, 4 had 2 previous closures and 3 had undergone more than 2 closures. In addition, 4 patients had undergone 1 prior bladder neck reconstruction and 3 had undergone 2 previous bladder neck reconstructions. Five patients had had no previous bladder neck surgery. Indications for augmentation cystoplasty in these patients were an inadequate bladder capacity before bladder neck reconstruction in 5, and an inadequate bladder capacity and urinary incontinence in 7 after bladder neck reconstruction, 4 of whom demonstrated upper tract changes. The complicated history of exstrophy presentation and subsequent treatment was studied carefully and prior radiological studies were reviewed. Excretory urograms, cystograms, loopograms and endoscopy were performed as indicated. Routine urodynamic bladder evaluation was not performed, although urethral pressure profiles were obtained in 2 patients during bladder neck reconstruction. The upper urinary tracts were normal in 8 patients and hydronephrotic in 4. The creatinine clearance was greater than 40 mg./ml./1.73 m. 2 (normal 100 to 150 ml. per minute) in all patients. Bladder capacity remained at small volumes despite followup evaluation and anticholinergic medications. Early in the series bladder cycling was attempted in 3 patients with disappointing results and it was discontinued. Intermittent catheterization was discussed and taught to each child and parent in anticipation of the possible need after bladder augmentation. The augmentation of the bladder was achieved with an ileal patch in 6 patients, an ileocecal segment in 1, a sigmoid augmentation in 4 and a hindgut patch in 1. Undiversion was performed in 1 patient with a previously constructed colon conduit attached to a tubularized bladder, according to the method of Arap and associates. 5 The varied application of the bowel segments is illustrated in figure 1. Adjunctive procedures that were performed at the time of augmentation included a bladder neck plasty in 4 cases, 1 of which was a repeat, transureteroureterostomy with a psoas hitch and ureteral reimplant in 1, and a Mitrofanoff procedure 6 associated with ileal augmentation and bladder neck closure in 1.
790
AUGMENTATION CYSTOPLASTY IN FAILED EXSTROPHY RECONSTRUCTION
Findings in 12 children with exstrophy who underwent augmentation cystoplasty Surgical Repair Bowel Segment
Continence Procedure
No. Closures
Postop. Continence
1 2
Hindgut Ileocecal
Young-Dees-Leadbetter Scott*
1 2
Yes* No
3 4
Scott Arap
5
Sigmoid Sigmoid Sigmoid
1 1 1
Yes Yest Yest
6 7
Sigmoid Ileum
Young-Dees-Leadbetter
4 2
Yest Yes
8
Ileum
Scott
3
Yest
9
10
Ileum Ileum
Young-Dees-Leadbetter Young-Dees-Leadbetter
1 2
Yest Yest
11
Ileum
Closure bladder neck
2
Yest
12
Ileum
3
Yest
Pt. No.
Followup (yrs.)
Unusual Features
5
Previous Young-Dees-Leadbetter operation Prior diversion
9
4 4 8
Previous Young-Dees-Leadbetter operation, transureteroureterostomy with psoas hitch
8
Previous Young-Dees-Leadbetter operation X2 Previous Young-Dees-Leadbetter operation
2 2 2 2
Previous Young-Dees-Leadbetter operation X2 Previous Young-Dees-Leadbetter operation, Mitrofanoff procedure Previous Young-Dees-Leadbetter operation X2
1 1
* Only complication was erosion.
t Patient dry on intermittent catheterization.
In 3 patients an artificial sphincter was implanted for continence. The selected cases included an undiversion in which the sphincter was placed around the tubularized bladder and a bladder exstrophy variant in which the sphincter was placed at the upper prostatic level after removing 2 ectopic ureters from the posterior urethra. The third patient received a sphincter around a failed bladder neck plasty when he was 5 years old. All patients who underwent combined bladder augmentation and sphincter placement had complete antibiotic bowel preparations before surgery and they received intravenous broadspectrum antibiotics for 7 days postoperatively. Our experience with myelodysplastic patients who have undergone augmentation and sphincter placement simultaneously led us to continue this regimen with the exstrophy group. Since our 1 case of erosion occurred 2 years after augmentation, the open gastrointestinal tract most likely did not compromise sphincter placement.
~
.
l leal palch &. appendix with M\\rofanoff procedure
Itealpalch
s.~~
..
H\nd[lul pakh in ctoacal exstrophy
RESULTS
All 12 children have been followed postoperatively for an average of 4 years (range 1 to 9 years) with few complications. In 4 patients with upper tract changes the upper tract status has improved and in 8 it has remained stable. Vesicoureteral reflux was present in 5 patients preoperatively and it has not recurred. Also, there has been no evidence of secondary ureteral obstruction. One patient who did not have reflux previously suffered reflux after bladder augmentation and sphincter placement. This patient now has complete incontinence owing to erosion of the artificial urinary sphincter which has been removed. Of the remaining 11 patients 2 are voiding and continent, and 9 are dry on intermittent catheterization at regular intervals. Only 1 patient is wet at night. Although bacteriuria has been present occasionally in many of these patients, febrile urinary tract infections have occurred in only 2 since the bladder augmentation. All patients have been maintained on continuous prophylactic medications. There were no instances of bowel obstruction, urinary fistulas or re-diversion. Electrolyte disturbance owing to reabsorption has not occurred except in 1 patient who has received bicitrate for chronic acidosis due to the combination of a short gut and hindgut bladder augmentation in a cloaca! exstrophy. Currently, she is dry for 3 hours on intermittent catheterization and the chronic acidosis is well controlled with bicarbonate therapy. Excess mucus was a problem in some patients in the
~ $
'
* Tubutar\zed
1(.
S\!jmoid cys\oplasly Araptype
Sigmoid pakh
Y:1 ~
il-Und\verted colon condu\\ with sphincter Arap type
~t
Ileocecal clstoplasty wit spn\ncler
Fm. 1. Various applications of bowel segments to small exstrophy bladder.
immediate postoperative period but, subsequently, it has seldom required irrigation even in those using intermittent catheterization. Adequate capacity and potential continence were achieved quickly in all patients using the detubularized ileum and the sigmoid patch. The patients with an undiverted tubularized colon and a Scott sphincter also gained day and night capacity and control. The patient with a tubularized colon augmentation and an Arap urethra fashioned from the bladder was slow in gaining control, partially owing to a weak bladder neck closure and partially to bowel peristalsis. This patient now is dry for 4 hours on intermittent catheterization and she only has an occasional wet night. The patient with a tubularized ileocecal cystoplasty remains a failure owing to erosion of a sphincter that had been placed when he was young over a previously reconstructed bladder neck. This patient awaits revision.
792
GEARHART AND JEFFS DISCUSSION
The potential for the development of an adequate bladder capacity is often difficult to evaluate at birth. Because of this variable potential, strict adherence to previously recommended principles at the time of initial closure becomes important. 7 The initial bladder neck and posterior urethra should be long enough and tight enough to allow unimpeded egress of urine but not allow the anterior bladder wall to prolapse. Prolapse in our experience was a factor in 4 patients who were unable to obtain a satisfactory bladder capacity before bladder neck reconstruction. Likewise, secure pelvic ring fixation and postoperative traction maintain the closure, and prevent tension and shearing forces from altering the bladder neck and neourethra. This allows the bladder to remain deeper in the pelvis whether or not one has chosen to use paraexstrophy skin flaps. If t~e initial clos~re accomplishes the aforementioned goals, the urme can pool m the bladder during the nighttime hours and, therefore, allow the bladder to adapt gradually to larger volumes without upper tract deterioration as low pressure drainage occurs. If the initial closure does not allow for a 50 to 60 cc bladder capacity by the time the patient is 3 years old an epispadias repair can be accomplished to increase outlet re;istance and hopefully bladder capacity (fig. 2). 4 The potential for bladder capacity is decreased by each successive bladder closure and bladder neck reconstruction. The most difficult patients in this group were those who had undergone multiple bladder closures. Wound infection, bladder prolapse, bladder calculi and vesicostomy have been identified as factors that can jeopardize an initial bladder closure, and 7 of our patients had a combination of these factors. 8 Therefore, every attempt should be made at the initial operation to achieve proper closure and proper pelvic fixation to promote and ensure primary healing of the abdominal wall, bladder outlet and urethral tissue. Normally, a Young-Dees-Leadbetter reconstruction is not recommended until at least a 50 to 60 cc bladder capacity is achieved by the child. In our experience it is estimated that bladder neck reconstruction requires at least 20 cc of bladder capacity. Therefore, a small capacity bladder becomes problematic. If a bladder tends to have a borderline capacity and a substantial ".olume is used by a bladder neck operation, this procedure will only decrease bladder capacity and increase intravesical pressure with a significant risk of deterioration of the upper tracts and/or urinary leakage. A wide variety of techniques for bladder augmentation and bladder substitution have been developed. Likewise, these techniques have been applied to bladder augmentation and urinary undiversion in children. 9 - 12 However, in addition to augmentatio~, the need for ureteral reimplantation, artificial urinary sphmcter, bladder neck plasty and other adjunctive procedures must be considered carefully. Ureteral reflux or obstruction in the small bladder seems to be managed best by reimplantation with a psoas hitch13 and transureteroureterostomy combined with augmentation. However, if ureteral reimplantation is re!NEWBORN BLAODER CLOSUREj AGE 2-4
AGE 2-4
1 BLADDER CAPACITY
lI
LESS THAN 50cc
!
rEPISPADIAS REPAIRl BLADDER CAPACITY GREATER THAN 50cc
!
YOUNG DEES LEADBETTER PROCEDURE
BLADDER CAPACITY LESS THAN 50cc
~
!
50c1
BLADDER CAPACITY GREATER THAN
YOUNG DEES LEADBETTER PROCEDURE
YOUNG DEES LEADBETTER PROCEDURE AND EPISPADIAS REPAIR
1
EPISPADIAS REPAIR
YOUNG DEES LEADBETTER PROCEDURE AND BLADDER AUGMENTATION
Fm. 2. Algorithm for exstrophy management
quired in conjunction with augmentation and the bladder is not suitable for this procedure, then a detubularized sigmoid or ileocecal segment is preferred. Early in our experience nondetubularized ileocecal segment was used along with an artificial urinary sphincter. Contractions of this segment caused incontinence and, therefore, only detubularized segments currently are used. However, if ureteral reimplantation is not required then an ileal patch is preferred because of the safer ileal anastomosis, lack of contractions and ease of handling small bowel. . Obv~ously, in the child with a small bladder and no previous mcontmence procedure the choice would be a Young-DeesLeadbetter procedure in conjunction with bladder augmentation. If a~ mentioned previously, the bladder was adequate for the creation of a bladder neck but not for ureteral reimplantation, then reimplantation into a detubularized bowel segment and bladder augmentation would be preferred. Placement of an artificial urinary sphincter in a bladder exstrophy patient should likely remain as a salvage procedure after previous attempts at continence. Of our 3 patients who underwent sphincter placement 2 had undergone previously a Young-Dees-Leadbetter procedure. One sphincter eroded into the urethra 2 years after placement. Currently, sphincters should remain an option for the older child with previous bladder neck surgery who has an adequate omental pad that can be used around the previously reconstructed bladder neck. A~so, continent ~rinary diversion procedures most certainly will have a place m the reconstruction of these most problematic cases. 14 The exstrophy patient who initially undergoes closure without dehiscence or prolapse frequently can achieve a functional bladder of good capacity for subsequent bladder neck reconstruction and ureteral reimplantation. The routine use of bladder ~u~entat~on at the time of closure is not only unnecessary but it might hmder bladder adaptation to volume and bladder growth. The indications for augmentation in exstrophy patients can be divided into 3 main groups: group 1-those in whom after initial or subsequent closures and epispadias repair the bladder does not adapt to a volume of 50 cc or greater even when treated with anticholinergic medication (to which the patient should respond), 15 group 2-those who have persistent incontinence with a small bladder capacity despite what is thought to be an adequate bladder neck repair and group 3those in whom upper tract decompensation occurs with or without the onset of incontinence after bladder neck reconstruction. In our 5 group 1 patients with a small bladder capacity before a_ny incontinence operation has been done bladder augmentat10n can be performed easily with the bladder neck procedure. This initial course of management is preferred before the use of an artificial sphincter and augmentation in these patients. There were 3 group 2 patient~ with persistent leakage, 2 of whom had undergone 2 previous bladder neck procedures and 1 who had undergone a prior bladder neck reconstruction. Although the outlet resistance was thought to be adequate bladder capacity only averaged approximately 60 cc. In thi~ situation augmentation allows for a decrease in bladder pressure, and an increase in capacity and compliance. It also may allow a bladder neck with previously marginal outlet resistance to become adequate for continence. However, if a previous Young-Dees-Leadbetter procedure is deemed inadequate and the bladder capacity is judged to be adequate the choice would be a repeat Young-Dees-Leadbetter procedure before resorting to bladder .neck reconstruction and simultaneous augmentation. Likewise, if a previous Young-Dees-Leadbetter procedure is deemed inadequate and the bladder capacity is less than 50 cc and it will again be decreased by a repeat bladder neck procedure, then concomitant bladder augmentation becomes desirable.
AUGMENTATION CYSTOPLASTY IN FAILED EXSTROPHY RECONSTRUCTION
In our 4 group 3 patients who benefited from bladder augmentation upper tract decompensation occurred. This group included 1 patient who had undergone 2 previous Young-DeesLeadbetter procedures following which the bladder was small. Bladder augmentation improved and stabilized the upper urinary tract in all 4 of these patients. These 12 patients are among 148 patients seen at our institution in the last 10 years and they represent the small percentage of those who fail to achieve satisfactory function after bladder closure. Augmentation cystoplasty appears to provide prolonged stability in this group of exstrophy patients and it has proved to be a successful alternative to urinary diversion when patients and parents have accepted the adjunctive need for intermittent catheterization when necessary. REFERENCES 1. Jeffs, R. D.: Exstrophy and cloaca! exstrophy. Urol. Clin. N. Amer., 5: 127, 1978. 2. Jeffs, R. D.: Management of the exstrophy-epispadias complex and urachal anomalies. In: Campbell's Urology, 5th ed. Edited by P. C. Walsh, R. F. Gittes, A. D. Perlmutter and T. A. Stamey. Philadelphia: W. B. Saunders Co., vol. 2, sect. XIII, chapt. 43, p. 1882, 1985. 3. Jeffs, R. D., Guice, S. L. and Oesch, I.: The factors in successful exstrophy closure. J. Urol., 127: 974, 1982. 4. Oesterling, J. E. and Jeffs, R. D.: The importance of a successful initial bladder closure in the surgical management of classical bladder exstrophy: analysis of 144 patients with bladder exstrophy treated at the Johns Hopkins Hospital from 1975 to 1985.
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J. Urol., 137: 258, 1987. 5. Arap, S., Giron, A. M. and de Goes, G. M.: Initial results of the complete reconstruction of bladder exstrophy. Urol. Clin. N. Amer., 7: 477, 1980. 6. Mitrofanoff, P.: Cystostomie continente trans-appendiculaire clans le traitement des vessies neurologiques. Chir. Ped., 21: 297, 1980. 7. Lepor, H. and Jeffs, R. D.: Primary bladder closure and bladder neck reconstruction in classical bladder exstrophy. J. Urol., 130: 1142, 1983. 8. Lowe, F. C. and Jeffs, R. D.: Wound dehiscence in bladder exstrophy: an examination of the etiologies and factors for initial failure and subsequent success. J. Urol., 130: 312, 1983. 9. Mitchell, M. E.: The role of bladder augmentation in undiversion. J. Ped. Surg., 16: 790, 1981. 10. Mitchell, M. E.: Urinary tract diversion and undiversion in the pediatric age group. Surg. Clin. N. Amer., 61: 1147, 1981. 11. Perlmutter, A. D.: Experiences with urinaryundiversion in children with neurogenic bladder. J. Urol., 123: 402, 1980. 12. Kass, E. J. and Koff, S. A.: Bladder augmentation in the pediatric neuropathic bladder. J. Urol., 129: 552, 1983. 13. Gearhart, J.P. and Woolfenden, K. A.: The vesico-psoas hitch as an adjunct to megaureter repair in childhood. J. Urol., 127: 505, 1982. 14. Mitchell, M. E. and Hensle, T. W.: Total bladder replacement in children. In: Bladder Reconstruction and Continent Urinary Diversion. Edited by L. R. King, A. R. Stone and G.D. Webster. Chicago: Year Book Medical Publishers, Inc., chapt. 21, p. 312, 1987. 15. Shapiro, E., Jeffs, R. D., Gearhart, J.P. and Lepor, H.: Muscarinic cholinergic receptors in bladder exstrophy. Insight into surgical management. J. Urol., 134: 308, 1985.