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Enterovesical Cystoplasty for Bladder Closure in Cloacal Exstrophy
0022-534 7/87 /1375-0948$02.00/0 Vol. 137, May Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright© 1987 by The Williams & Wilkins Co.
ENTEROVESICAL CYSTOPLASTY FOR BLADDER CLOSURE IN CLOACAL EXSTROPHY KEVIN A. BURBIGE AND CHARLES LIBBY From the Department of Urology, J. Bently Squier Urological Clinic, Babies Hospital Division, Columbia-Presbyterian Medical Center, New York, New York
ABSTRACT
Successful bladder closure was accomplished in 4 infants with cloacal exstrophy by enterocystoplasty. Two patients were operated upon as newborns, and 2 were 3 and 8 months old, respectively. Three patients are alive and all are incontinent awaiting secondary bladder neck reconstruction. The surgical technique and results are described. The treatment of infants with cloacal exstrophy presents a difficult challenge for the pediatric urologist. Owing to concerns regarding the ultimate survival of these infants, most reports have emphasized management of the bowel abnormality with cystectomy and urinary diversion advocated as a secondary procedure. 1• 2 Improvement in the over-all survival of infants with cloacal exstrophy has resulted in a greater effort to correct the urological deformity by applying the techniques and principles of classical bladder exstrophy closure. It has been the consensus of most recent reports that whenever technically possible the exstrophic bladder should be closed soon after birth with a staged approach to provide later continence. 3 Occasionally, the surgeon will encounter infants with a small exstrophic bladder plate or infants whose initial closure has been delayed, resulting in damage to the exposed mucosa. Traditionally, these exstrophic bladders have been deemed unfit for closure and urinary diversion has been advocated. Recently, we treated such patients with enterocystoplasty using the rudimentary hindgut if adequate to facilitate bladder closure. MATERIALS
Between 1982 and 1985 the exstrophic bladder of 3 female and 1 male infants with cloacal exstrophy was closed primarily by enterocystoplasty. In 2 newborns the bladder plates were judged to be too small for standard functional closure and in 2 infants, 3 and 8 months old, bladder closure had been delayed because of associated anomalies. All 4 patients had normal renal function before closure. Excretory urography (IVP) was normal in 3, and in 1 a dilated right collecting system and left pelvic kidney were demonstrated.
mesenteric border (fig. 1, C). The intestinal segment then is anastomosed to the exstrophic bladder plate in 2 layers with absorbable suture (fig. 1, D). The closed augmented bladder is placed back into the abdomen. In the newborn the pubis is reapproximated with heavy suture material and the abdominal wall is closed in the midline. In the older infants we have not attempted to reapproximate the pubis, which has required development of lateral musculocutaneous flaps to close the abdominal wall defect. RESULTS
Followup ranges from 1 to 4 years for 3 patients who are well and have stable renal function. The male patient had associated myelomeningocele and he died in the newborn period of overwhelming meningitis. All patients await secondary bladder neck reconstruction and repair of the external genitalia, and they are totally incontinent. CASE HISTORY
A female infant had been born with cloacal exstrophy, and an associated omphalocele and myelomeningocele. She had undergone closure of the omphalocele, diverting ileostomy and repair of the myelomeningocele during the newborn period. Bladder closure was delayed subsequently until she was 8 months old, at which time the bladder plates were unfit for closure secondary to traumatic damage to exposed vesical mucosa.
SURGICAL TECHNIQUE
Reconstruction of these patients was based on a combination of established surgical techniques for exstrophic bladder closure and intestinal bladder augmentation. 4 • 5 After division of the intestinovesical fistula and diversion of the fecal stream, the 2 hemibladder plates are freed sharply from their skin attachments (fig. 1, A) and the hindgut is separated from the bladder plates. The fibrotic band of tissue that connects the 2 hemibladders is excised and the 2 bladder plates are joined in the midline with absorbable sutures (fig. 1, B). The ureters are intubated with small caliber pediatric feeding tubes to aid in identification and to provide postoperative urinary drainage. A wide urethral orifice then is created by tubularizing the inferior portion of the exstrophic bladder in a limited Young-Dees procedure. The hindgut is mobilized, care being taken not to injure its single anomalous mesenteric blood supply, and it is opened longitudinally along its antiAccepted for publication December 22, 1986. 948
A
B
~
~ D
FIG. 1. A, hemibladder plates are freed sharply from skin attachments. B, after separating hindgut 2 bladder plates are joined in midline. C, hindgut is opened along its antimesenteric border. D, closure is completed by anastomosing hindgut to bladder.
-
ENTEROVESICAL CYSTOPLASTY FOR BLADDER CLOSURE IN CLOACAL EXSTROPHY
An IVP revealed nonobstructive dilatation of the right kidney and a left pelvic kidney (fig. 2). Adequate drainage of both kidneys was documented by a diethylenetriaminepentaacetic acid furosemide renogram. Closure of the hypertrophic bladder plates was done with hindgut to augment capacity and the abdominal wall defect was closed. An IVP 18 months postoperatively was stable (fig. 3, A) and revealed adequate bladder capacity (fig. 3, Band C).
949
DISCUSSION
The successful treatment of children with exstrophic lesions of the bladder is a difficult and long-term process. Advances in surgical and anesthetic techniques, and the advent of modern antibiotics have improved dramatically the over-all prognosis and quality of life. The first step in this process is functional bladder closure performed as soon after birth as possible. It is always distressing to see a neonate either with a small bladder plate or a young infant whose closure has been delayed, resulting in a hypertrophic thick-walled bladder, successful closure of which becomes more difficult. Primary enterocystoplasty allows for closure of the exstrophic bladder in children who would otherwise be diverted for life or whose subsequent reconstruction after cystectomy is fraught with difficulty. Given the known hazards of long-term urinary diversion in young children, primary closure becomes more attractive. 6 Primary enterocystoplasty is not indicated nor applicable in all infants with cloacal exstrophy. Certainly infants with adequately sized bladder plates can undergo closure with urothelium alone. In those infants with severe associated anomalies, that is a giant omphalocele or severe spinal dysraphism, major reconstruction of the urinary tract should be delayed. Likewise, infants with poor gastrointestinal absorptive capacity secondary to foreshortening of the foregut may require re-establishment of the continuity of the hindgut to improve nutritional status. In newborns the length of foregut can be assessed at the initial laparotomy and a judgment can be made in regard to the need for incorporation of the hindgut into the fecal stream. If there is any doubt as to the adequacy of foregut present, enterocystoplasty should be delayed until the nutritional status is established. Primary enterocystoplasty should be reserved for infants judged to be vigorous on newborn examination and older infants whose associated anomalies have been corrected and whose nutritional status is secure. The final results of this type of treatment for the difficult exstrophy patient will have to bear the test of time in a larger group of patients. Our short-term results are encouraging, although preservation of renal function, ultimate continence and voiding ability must be followed and evaluated critically. REFERENCES
1. Fonkalsrud, E. W. and Linde, L. M.: Successful management of
FIG. 2. Preoperative IVP demonstrates dilatation of right collecting system and left pelvic kidney.
vesicointestinal fissure: report of two cases. J. Ped. Surg., 5: 309, 1970. 2. Welch, K. J.: Cloaca! exstrophy (vesicointestinal fissure). In: Pe-
FIG. 3. A, IVP 18 months after exstrophy closure is stable. Anteroposterior (B) and lateral (C) views from postoperative IVP demonstrate good capacity bladder (arrow).
950
BURBIGE AND LIBBY
diatric Surgery. Edited by M. M. Ravich, K. J. Welch, C. D. Benson, E. Aberdeen and J. G. Randolph. Chicago: Year Book Medical Publishers, vol. 2, chapt. 78, pp. 802-808, 1979. 3. Jeffs, R. D. and Lepor, H.: 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. 3, sect. XIII, chapt. 43,p. 1882, 1986.
4. Jeffs, R. D., Guice, S. L. and Oesch, I.: The factors in successful exstrophy closure. J. Urol., 127: 974, 1982. 5. Mitchell, M. E.: The role of bladder augmentation in undiversion. J. Ped. Surg., 16: 790, 1981. 6. Shapiro, S. R., Lebowitz, R. and Colodny, A. H.: Fate of 90 children with ilea! conduit urinary diversion a decade later: analysis of complications, pyelography, renal function and bacteriology. J. Urol., 114: 289, 1975.
THE JOURNAL OF UROLOGY
Copyright© 1987 by The Williams & Wilkins Co.
ENTEROVESICAL CYSTOPLASTY FOR BLADDER CLOSURE IN CLOACAL EXSTROPHY KEVIN A. BURBIGE AND CHARLES LIBBY From the Department of Urology, J. Bently Squier Urological Clinic, Babies Hospital Division, Columbia-Presbyterian Medical Center, New York, New York
ABSTRACT
Successful bladder closure was accomplished in 4 infants with cloacal exstrophy by enterocystoplasty. Two patients were operated upon as newborns, and 2 were 3 and 8 months old, respectively. Three patients are alive and all are incontinent awaiting secondary bladder neck reconstruction. The surgical technique and results are described. The treatment of infants with cloacal exstrophy presents a difficult challenge for the pediatric urologist. Owing to concerns regarding the ultimate survival of these infants, most reports have emphasized management of the bowel abnormality with cystectomy and urinary diversion advocated as a secondary procedure. 1• 2 Improvement in the over-all survival of infants with cloacal exstrophy has resulted in a greater effort to correct the urological deformity by applying the techniques and principles of classical bladder exstrophy closure. It has been the consensus of most recent reports that whenever technically possible the exstrophic bladder should be closed soon after birth with a staged approach to provide later continence. 3 Occasionally, the surgeon will encounter infants with a small exstrophic bladder plate or infants whose initial closure has been delayed, resulting in damage to the exposed mucosa. Traditionally, these exstrophic bladders have been deemed unfit for closure and urinary diversion has been advocated. Recently, we treated such patients with enterocystoplasty using the rudimentary hindgut if adequate to facilitate bladder closure. MATERIALS
Between 1982 and 1985 the exstrophic bladder of 3 female and 1 male infants with cloacal exstrophy was closed primarily by enterocystoplasty. In 2 newborns the bladder plates were judged to be too small for standard functional closure and in 2 infants, 3 and 8 months old, bladder closure had been delayed because of associated anomalies. All 4 patients had normal renal function before closure. Excretory urography (IVP) was normal in 3, and in 1 a dilated right collecting system and left pelvic kidney were demonstrated.
mesenteric border (fig. 1, C). The intestinal segment then is anastomosed to the exstrophic bladder plate in 2 layers with absorbable suture (fig. 1, D). The closed augmented bladder is placed back into the abdomen. In the newborn the pubis is reapproximated with heavy suture material and the abdominal wall is closed in the midline. In the older infants we have not attempted to reapproximate the pubis, which has required development of lateral musculocutaneous flaps to close the abdominal wall defect. RESULTS
Followup ranges from 1 to 4 years for 3 patients who are well and have stable renal function. The male patient had associated myelomeningocele and he died in the newborn period of overwhelming meningitis. All patients await secondary bladder neck reconstruction and repair of the external genitalia, and they are totally incontinent. CASE HISTORY
A female infant had been born with cloacal exstrophy, and an associated omphalocele and myelomeningocele. She had undergone closure of the omphalocele, diverting ileostomy and repair of the myelomeningocele during the newborn period. Bladder closure was delayed subsequently until she was 8 months old, at which time the bladder plates were unfit for closure secondary to traumatic damage to exposed vesical mucosa.
SURGICAL TECHNIQUE
Reconstruction of these patients was based on a combination of established surgical techniques for exstrophic bladder closure and intestinal bladder augmentation. 4 • 5 After division of the intestinovesical fistula and diversion of the fecal stream, the 2 hemibladder plates are freed sharply from their skin attachments (fig. 1, A) and the hindgut is separated from the bladder plates. The fibrotic band of tissue that connects the 2 hemibladders is excised and the 2 bladder plates are joined in the midline with absorbable sutures (fig. 1, B). The ureters are intubated with small caliber pediatric feeding tubes to aid in identification and to provide postoperative urinary drainage. A wide urethral orifice then is created by tubularizing the inferior portion of the exstrophic bladder in a limited Young-Dees procedure. The hindgut is mobilized, care being taken not to injure its single anomalous mesenteric blood supply, and it is opened longitudinally along its antiAccepted for publication December 22, 1986. 948
A
B
~
~ D
FIG. 1. A, hemibladder plates are freed sharply from skin attachments. B, after separating hindgut 2 bladder plates are joined in midline. C, hindgut is opened along its antimesenteric border. D, closure is completed by anastomosing hindgut to bladder.
-
ENTEROVESICAL CYSTOPLASTY FOR BLADDER CLOSURE IN CLOACAL EXSTROPHY
An IVP revealed nonobstructive dilatation of the right kidney and a left pelvic kidney (fig. 2). Adequate drainage of both kidneys was documented by a diethylenetriaminepentaacetic acid furosemide renogram. Closure of the hypertrophic bladder plates was done with hindgut to augment capacity and the abdominal wall defect was closed. An IVP 18 months postoperatively was stable (fig. 3, A) and revealed adequate bladder capacity (fig. 3, Band C).
949
DISCUSSION
The successful treatment of children with exstrophic lesions of the bladder is a difficult and long-term process. Advances in surgical and anesthetic techniques, and the advent of modern antibiotics have improved dramatically the over-all prognosis and quality of life. The first step in this process is functional bladder closure performed as soon after birth as possible. It is always distressing to see a neonate either with a small bladder plate or a young infant whose closure has been delayed, resulting in a hypertrophic thick-walled bladder, successful closure of which becomes more difficult. Primary enterocystoplasty allows for closure of the exstrophic bladder in children who would otherwise be diverted for life or whose subsequent reconstruction after cystectomy is fraught with difficulty. Given the known hazards of long-term urinary diversion in young children, primary closure becomes more attractive. 6 Primary enterocystoplasty is not indicated nor applicable in all infants with cloacal exstrophy. Certainly infants with adequately sized bladder plates can undergo closure with urothelium alone. In those infants with severe associated anomalies, that is a giant omphalocele or severe spinal dysraphism, major reconstruction of the urinary tract should be delayed. Likewise, infants with poor gastrointestinal absorptive capacity secondary to foreshortening of the foregut may require re-establishment of the continuity of the hindgut to improve nutritional status. In newborns the length of foregut can be assessed at the initial laparotomy and a judgment can be made in regard to the need for incorporation of the hindgut into the fecal stream. If there is any doubt as to the adequacy of foregut present, enterocystoplasty should be delayed until the nutritional status is established. Primary enterocystoplasty should be reserved for infants judged to be vigorous on newborn examination and older infants whose associated anomalies have been corrected and whose nutritional status is secure. The final results of this type of treatment for the difficult exstrophy patient will have to bear the test of time in a larger group of patients. Our short-term results are encouraging, although preservation of renal function, ultimate continence and voiding ability must be followed and evaluated critically. REFERENCES
1. Fonkalsrud, E. W. and Linde, L. M.: Successful management of
FIG. 2. Preoperative IVP demonstrates dilatation of right collecting system and left pelvic kidney.
vesicointestinal fissure: report of two cases. J. Ped. Surg., 5: 309, 1970. 2. Welch, K. J.: Cloaca! exstrophy (vesicointestinal fissure). In: Pe-
FIG. 3. A, IVP 18 months after exstrophy closure is stable. Anteroposterior (B) and lateral (C) views from postoperative IVP demonstrate good capacity bladder (arrow).
950
BURBIGE AND LIBBY
diatric Surgery. Edited by M. M. Ravich, K. J. Welch, C. D. Benson, E. Aberdeen and J. G. Randolph. Chicago: Year Book Medical Publishers, vol. 2, chapt. 78, pp. 802-808, 1979. 3. Jeffs, R. D. and Lepor, H.: 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. 3, sect. XIII, chapt. 43,p. 1882, 1986.
4. Jeffs, R. D., Guice, S. L. and Oesch, I.: The factors in successful exstrophy closure. J. Urol., 127: 974, 1982. 5. Mitchell, M. E.: The role of bladder augmentation in undiversion. J. Ped. Surg., 16: 790, 1981. 6. Shapiro, S. R., Lebowitz, R. and Colodny, A. H.: Fate of 90 children with ilea! conduit urinary diversion a decade later: analysis of complications, pyelography, renal function and bacteriology. J. Urol., 114: 289, 1975.