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Urethral Replacement with Ureter
0022-534 7/88/1396-1282$02.00/0 Vol. 139, June Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright© 1988 by The Williams & Wilkins Co.
URETHRAL REPLACEMENT WITH URETER MICHAEL E. MITCHELL, MARK C. ADAMS AND RICHARD C. RINK From the Department of Pediatric Urology, James Whitcomb Riley Hospital for Children, Indiana University Medical School, Indianapolis, Indiana
ABSTRACT
Proximal or total urethral replacement was performed in 8 patients using distal ureter. This was accomplished by basing the ureteral segment solely on a vascular pedicle arising from the internal iliac artery. Diagnoses included classical bladder exstrophy in 4 patients, cloacal exstrophy in 3 and an imperforate anus with hypoplasia of the bladder neck and urethra in 1. Continence was achieved by a tunneled submucosal reimplantation into either the bladder (4 cases), gastric reservoir (2) or colonic reservoir (2). Although an isolated distal segment of ureter was used in each case, all segments have remained viable. With limited followup (5 to 44 months) continence with either normal voiding or intermittent catheterization has been achieved in 7 of the 8 patients. A distal ureteral segment should be considered potenti.ally useful in the construction or reconstruction of the proximal (male) or total (female) urethra in patients with congenital urethral malformations. (J. Ural., 139: 1282-1285, 1988) Effective reconstruction of the lower genitourinary tract requires the creation of an adequate reservoir that can be emptied reliably. Clean intermittent catheterization has clearly facilitated this emptying function requirement. 1- 3 Therefore, of utmost importance in any lower urinary reconstruction is the achievement of a catheterizable channel or urethra that also provides continence. In patients with congenital malformations of the bladder neck and urethra creation of such a channel can be difficult. One procedure that can be considered in such patients involves the use of an isolated, vascularized tube, such as the appendix or ureter, as described by Mitrofanoff, 4 and Duckett and Snyder. 5 They used appendix or ureter tunneled into the bladder to construct a catheterizable stoma on the lower abdomen. Likewise, a similar segment can be implanted into a bowel reservoir to provide continence using the same techniques that have been time-tested to prevent reflux. We used a distal ureteral segment in 8 patients to construct an orthotopic urethra in an effort to avoid a cutaneous stoma and preserve body image. In 4 girls we brought the ureteral segment to the perineum essentially to replace the entire urethra. In 4 boys we constructed a proximal urethra. We report our experience with these 8 cases. METHODS
Partial or total urethral replacement with ureter was performed in 8 cases. Although the technique varied depending on the anatomy of the particular case, the basic principle remained constant. The ureter is dissected from the native bladder in the same manner as for a standard ureteroneocystotomy. Care is taken to avoid grasping the ureter or in any way jeopardizing the ureteral blood supply. A 5 or 8F feeding tube sutured at the ureteral meatus is used as a handle for manipulation. All periureteral tissue is preserved with the ureter. The length of distal ureter needed for urethral reconstruction is, thereby, meticulously dissected with its blood supply, which in the pelvis enters .from the lateral aspect of the ureter. In each patient we were able to identify and preserve a sizable branch of the internal iliac artery supplying the distal ureter. Other nutrient vessels are divided only as necessary for adequate mobilization of the distal ureteral segment. At the proximal end of the intended segment we place a tight double-wrapped vessel loop Accepted for publication October 21, 1987.
or atraumatic clamp on the ureter for several minutes to interrupt from above longitudinal arterial inflow. We then are able to ensure viability of the ureteral segment before division of the ureter (fig. 1). This well vascularized ureteral segment is mobilized carefully with its pedicle to reach the perineum. Occasionally, it is necessary to rotate the ureteral segment 180 degrees after mobilization. One end of the segment is implanted into a bladder or bowel reservoir in a standard antirefluxing manner to provide a continence mechanism. The other end of the segment is brought to the perineum in the female patient as a catheterizable urethra or to the distal urethra in the male patient for anastomosis. We leave a stent through the ureteral segment during healing until intermittent catheterization is begun. Transureteroureterostomy then provides drainage for the ipsilateral kidney. We have used this method in 8 patients with a diagnosis of bladder exstrophy in 4, cloacal exstrophy in 3 and an imperforate anus with hypoplasia of the bladder neck and urethra in 1. All operations were performed by or directly supervised by the senior author (M. E. M.). An isolated segment of the right ureter was used in 6 patients and the left ureter was used in 2. Associated procedures included transureteroureterostomy in 5 cases, creation of an intestinal urinary reservoir in 4, intestinocystoplasty in 2, closure of vesicostomy in 1 and nephroureterectomy in 1. Continence was based on tunneled submucosal implantation of the isolated ureteral segment into the bladder in 4 cases, gastric reservoir in 2 and a colonic reservoir in a Leadbetter fashion in 2. 6 An antireflux procedure was required in 7 patients, which included a tunneled submucosal reimplant of ureter into bladder in 4 cases, a gastric reservoir in 2 and a colonic reservoir in 1. CASE HISTORIES
Case 1 (fig. 2). M. L., an 8-year-old girl with bladder exstrophy, had undergone urinary diversion via ureterosigmoidostomy when she was 1 year old and cystectomy when she was 3 years old. Although renal function was normal, she had experienced several recent episodes of pyelonephritis, as well as chronic diarrhea and soilage at night. In an effort to reduce infection and potential for tumor development, reconstruction was undertaken. The procedure began with a limited sigmoid colon resection,
1282
1283
URETHRAL REPLACEMENT WITH URETER Bilateral reflux
~
L Before division of ureter viability of isolated ureteral segment is tested by compression of longitudinal arterial flow from above using a vessel loop.
t,
(7\
(i ~) ::,,,d\vir,: reimplants /
,),.~~<)
~; FIG. 2. Case 1. Reconstruction consists of take-down of ureterosigmoidostomies and resection of sigmoid colon, including anastomotic eites, Segment of descending colon is used to construct urinary reservoir and isolated right distal ureteral segment is tunneled into reservoir to continent urethra.
the ureterosigmoidostomy anastomotic sites. An isolated descending colonic segment immediately proximal to the resected segment was used for creation of a urinary reservoir. The isolated segment was opened along the antimesenteric and re-configured. Having isolated the distal 8 cm. of ureter on a vascular pedicle from the right internal iliac artery, the distal end of this segment was brought to the and secured with circumferential sutures. The proxend of the segment was tunneled 3 cm. under the tenia of the reservoir for continence. Both ureters were tunneled along the tenia of the reservoir to prevent reflux. These reimplantahons were done in the manner described by Leadbetter 6 and Hendren. 7 At 10-month followup the patient was completely dry with catheterization at intervals as long as 6 hours. She has been free of infections and the upper tracts have remained stable. Case 2 3). M. a 2-year-old girl with cloacal exstrophy, had undergone abdominal wall closure, bladder closure and end as a newborn. She had a small bladder, total inconbilateral reflux. In an effort to avoid 2 stomas total reconstruction of the lower urinary tract was undertaken. A reservoir was created by re-configuration of an isolated segment of stomach brought into the pelvis on a pedicle of the left gastroepiploic artery. The distal right ureter was isolated on a pedicle from the right internal iliac artery and the diE:tal end of this segment was brought to the perineum and
.un,w.·vm.,,.,
)
"--' Ct.-..,.,. ~
perineum as vagina
FIG. 3. Case 2. Owing to deficiency of bowel urinary reservoir is constructed from gastric segment. Continence is based on segment of distal right ureter. Proximal portion is tunneled into reservoir and distal end is brought to perineum as orthotopic meatus. Native bladder is brought posterior to form vagina.
secured. The proximal end was tunneled between gastric mucosa and muscle for continence. Right-to-left transureteroureterostomy and a tunneled reimplant of the left ureter into the reservoir were performed. The native bladder was moved posterior to create an adequate vagina. At 8-month followup the patient was dry with catheterization up to every 6 hours and the upper tracts were stable. Case 3 (fig. 4). T. I., a 6-year-old boy with bladder exstrophy, had undergone abdominal wall and bladder closure as a newborn, bilateral cross-trigonal ureteral reimplantation when he was 1 year old, and a Young-Dees-Leadbetter bladder neck repair and distal urethroplasty when he was 4 years old. Optical urethrotomy was performed when the boy was 5 years old because of a proximal stricture. The patient had a small capacity bladder, left vesicoureteral reflux, a dense proximal urethral stricture and incontinence. Reconstruction included left-to-right transureteroureterostorny and a right cross-trigonal reimplant into the bladder. The native proximal urethra was divided at the bladder neck and incised longitudinally across the stricture in preparation for a patch urethroplasty. The isolated distal left ureter was tunneled submucosally at the bladder neck to provide continence. The distal end of the segment was incised and used as a patch for the urethral stricture. A sigmoid patch was used to augment the bladder for increased compliance and capacity. This patient currently is dry with catheterization every 4 hours. He had had some difficulty with catheterization 6 months postoperatively but this resolved completely after several days with an indwelling catheter. Case 4 (fig. 5). J. W., a 2-year-old boy, was born with an imperforate anus, patent urachus, massive bilateral reflux, hypoplasia of the bladder neck and urethra, and dysmorphism of the penis with 1 corporeal body. He had undergone sigmoid loop colostomy, resection of the patent urachus and creation of a vesicostomy as a newborn. Colostomy take-down and rectal pull-through had been performed when he was 1 year old. Reconstruction included left-to-right transureteroureterostomy and right tapered, cross-trigonal ureteroneocystostomy. The isolated distal left ureter was brought on its pedicle to the perineum. This segment was tapered and the distal end was brought through the urogenital diaphragm as a perinea! urethrostomy. Proximally, the ureteral segment was tunneled submucosally at the bladder neck. The vesicostomy was then closed. Meatotomy was required 9 months after reconstruction for meatal stenosis of the urethrostomy. At I-year followup the upper tracts were stable and the patient was free of infections.
1284
MITCHELL, ADAMS AND RINK DISCUSSION
Resect stricture ~
FIG. 4. Case 3. Sigmoid cystoplasty is performed. Proxi~al end ~f isolated left distal ureter is tunneled at bladder neck to provide continence. Distal end of ureteral segment is opened and used as cap for prostatic urethra to repair stricture.
FIG. 5. Case 4. Proximal urethra is constructed from isolated left distal ureteral segment. Proximally, ureteral segment is tapered and tunneled at bladder neck. Distal end of segment is brought through urogenital diaphragm to mid scrotum.
He remains dry for 3 to 4-hour intervals and he voids to completion with a good stream. RESULTS
The average followup for this group of patients is 13.5 months (range 5 to 44 months) and the average patient age is 4.7 years (range 1.7 to 8.3 years). All 8 patients had normal renal function preoperatively, which has remained unchanged during followup. In no patient has metabolic acidosis developed. Transient hydronephrosis developed in 1 child secondary to ureterovesical junction obstruction, which was treated by ureteral meatotomy. The other patients have demonstrated stable upper tracts on x-ray studies. No patient has had vesicoureteral reflux. Four patients have maintained sterile urine. Of the 4 patients with positive urine cultures 3 have been asymptomatic and 1 had lower abdominal pain. No patient has had a suspected renal infection (fever or systemic illness). Patients are considered to be continent if they stay completely dry on a reasonable schedule of catheterization or voiding. Of our patients 6 require clean intermittent catheterization and all are dry between catheterizations. One boy had had temporary difficulty with catheterization beyond the anastomosis of the distal urethra and the ureteral segment, which resolved after several days with an indwelling catheter. Recently, 1 girl suffered a false passage and presently she ~as an indwelling catheter during healing. One patient required a secondary ileal augmentation of a colonic reservoir to achieve continence. Of the 2 patients who void spontaneously with small residuals 1 is dry between voidings every 3 to 4 hours and 1 has been dry for intervals of up to 3 hours but he is not yet toilet-trained. Whether this patient will truly be continent remains to be seen.
True continence after reconstruction in patients with congenital malformations of the bladder neck and urethra has been difficult to achieve. Leadbetter's experience with his modification of the Young-Dees bladder neck procedure revealed a success rate of about 60 per cent. 8 Tanagho reported a success rate of 71.5 per cent using tubularization of anterior bladder musculature. 9 Early results with the artificial sphincter are encouraging, 10• 11 although a sphincter around a previously reconstructed urethra can lead to erosion. 11 Recently, Kropp and Angwafo reported their experience with the creation of a prox imal urethra from anterior bladder wall in the myelomeningocele population. 12 Duckett and Snyder reported the use of the appendix and distal ureter as a catheterizable segment5 as described by Mitrofanoff. 4 Our application merely is an extension of these procedures in an effort to create a continent urethra when none was previously available. Of our 8 patients 7 had exstrophy. Use of the ureter in such a manner requires a knowledge of the ureteral blood supply. Branches from the renal artery proximally and vesical arteries distally are the most consistent of the ureteral blood supply. 13 ' 14 In between, branches from the aorta, gonadal arteries, common iliac arteries, external iliac arteries and internal iliac arteries are much more variable, although almost all ureters receive at least 1 such intermediate vessel.1 3 • 14 Meigs believed that a vessel from the internal iliac artery was the most consistent and important for intermediate ureteral blood supply. 15 Also, the ureter usually is served by a rich longitudinal network of vessels within its adventitia. When preserved this network allows use of an isolated segment of ureter based on a pedicle of a single nutrient artery. Some early anatomical dissections suggest that about 10 per cent of the ureters lack a sufficient network of these collaterals, 13 and this procedure should be avoided in such ureters. Therefore, the distal ureter must be dissected from the bladder and pelvis with great care. We then test for adequate distal blood flow by compression of the upper portion of the distal ureteral segment before division. If blood supply to this segment is adequate it can be used reliably as a pedicle graft. All 8 patients are seemingly continent and 6 are dry with intermittent clean catheterization. Interestingly, the remaining 2 patients are voiding spontaneously. Both have the native bladder without augmentation. One is continent and the other has dry intervals of up to 3 hours but he is not yet toilettrained. This result suggests that voluntary continence may be achieved if the ureteral segment is brought through the pelvic diaphragm in a young child. Before use of this procedure we expect the patient to be totally reliant on intermittent catheterization to empty the bladder/reservoir. The patient and family must understand and accept this commitment if success is to be achieved. Any question about this commitment is considered a contraindication to this or any continence procedure in these patients. Although the duration of followup on our initial 8 patients is short, we believe that our results are encouraging. This technique allows another option for continent lower urinary tract reconstruction in this most difficult group of patients. REFERENCES
1. Lapides, J., Diokno, A. C., Silber, S. J. and Lowe, B. S.: Clean, intermittent self-catheterization in the treatment of urinary tract disease. J. Urol., 107: 458, 1972. 2. Lapides, J., Diokno, A. C., Gould, F. R. and Lowe, B. S.: Further observations on self-catheterization. J. Urol., 116: 169, 1976. 3. Mitchell, M. E., Kulb, T. B. and Backes, D. J.: Intestinocystoplasty in combination with clean intermittent catheterization in the management ofvesical dysfunction. J. Urol., 136: 288, 1986. 4. Mitrofanoff, P.: Cystostomie continente trans-appendiculaire dans
URETHRAL REPLACEMENT WITH URETER
5.
7.
9. 10.
le trnitement des vessies neurologiques. Chir. Ped., 21: 297, 1980. Duckett, J. W. and Snyder, H. M., III: Continent urinary diversion: variations on the Mitrofanoff principle. J. Urol., 136: 58, 1986. Leadbetter, W. F.: Consideration of problems incident to performance of uretero-enterostomy: report of a technique. J. Ural., 65: 818, 1951. Hendren, W. H.: Nonrefluxing colon conduit for temporary or permanent urinary diversion in children. J. Ped. Surg., :l.O: 381, 1975. Leadbetter, G. W., Jr.: Surgical correction of total urinary incontinence. J. UroL, 91: 261, 1964. Tanagho, K A.: Bladder neck reconstruction for total urinary incontinence: 10 years of experience. J. Urol., 125: 321, 1981. Barrett, D. M. and Furlow, W. L.: The management of severe urinary incontinence in patients with myelodysplasia by implan-
11. 12.
13.
14. 15.
1285
tation of the AS 791/792 urinary sphincter device. J. Urol., 128: 484, 1982. Mitchell, M. E. and Rink, R. C.: Experience with the artificial urinary sphincter in children and young adults. J. Ped. Surg., 18: 700, 1983. Kropp, KA. and Angwafo, F. F.: Urethral lengthening and reimplantation for neurogenic incontinence in children. J. Urol., 135: 533, 1986. Daniel, 0. and Shackman, R.: The blood supply of the human ureter in relation to ureterocolic anastomosis. Brit. J. Urol., 24: 334, 1952. Michaels, J. P.: Study of ureteral blood supply and its bearing on necrosis of the ureter following the Wertheim operation. Surg., Gynec. & Obst., 86: 36, 1948. Meigs, J. V.: The Wertheim operation for carcinoma of the cervix. Amer. J. Obst. Gynec., 49: 542, 1945.
THE JOURNAL OF UROLOGY
Copyright© 1988 by The Williams & Wilkins Co.
URETHRAL REPLACEMENT WITH URETER MICHAEL E. MITCHELL, MARK C. ADAMS AND RICHARD C. RINK From the Department of Pediatric Urology, James Whitcomb Riley Hospital for Children, Indiana University Medical School, Indianapolis, Indiana
ABSTRACT
Proximal or total urethral replacement was performed in 8 patients using distal ureter. This was accomplished by basing the ureteral segment solely on a vascular pedicle arising from the internal iliac artery. Diagnoses included classical bladder exstrophy in 4 patients, cloacal exstrophy in 3 and an imperforate anus with hypoplasia of the bladder neck and urethra in 1. Continence was achieved by a tunneled submucosal reimplantation into either the bladder (4 cases), gastric reservoir (2) or colonic reservoir (2). Although an isolated distal segment of ureter was used in each case, all segments have remained viable. With limited followup (5 to 44 months) continence with either normal voiding or intermittent catheterization has been achieved in 7 of the 8 patients. A distal ureteral segment should be considered potenti.ally useful in the construction or reconstruction of the proximal (male) or total (female) urethra in patients with congenital urethral malformations. (J. Ural., 139: 1282-1285, 1988) Effective reconstruction of the lower genitourinary tract requires the creation of an adequate reservoir that can be emptied reliably. Clean intermittent catheterization has clearly facilitated this emptying function requirement. 1- 3 Therefore, of utmost importance in any lower urinary reconstruction is the achievement of a catheterizable channel or urethra that also provides continence. In patients with congenital malformations of the bladder neck and urethra creation of such a channel can be difficult. One procedure that can be considered in such patients involves the use of an isolated, vascularized tube, such as the appendix or ureter, as described by Mitrofanoff, 4 and Duckett and Snyder. 5 They used appendix or ureter tunneled into the bladder to construct a catheterizable stoma on the lower abdomen. Likewise, a similar segment can be implanted into a bowel reservoir to provide continence using the same techniques that have been time-tested to prevent reflux. We used a distal ureteral segment in 8 patients to construct an orthotopic urethra in an effort to avoid a cutaneous stoma and preserve body image. In 4 girls we brought the ureteral segment to the perineum essentially to replace the entire urethra. In 4 boys we constructed a proximal urethra. We report our experience with these 8 cases. METHODS
Partial or total urethral replacement with ureter was performed in 8 cases. Although the technique varied depending on the anatomy of the particular case, the basic principle remained constant. The ureter is dissected from the native bladder in the same manner as for a standard ureteroneocystotomy. Care is taken to avoid grasping the ureter or in any way jeopardizing the ureteral blood supply. A 5 or 8F feeding tube sutured at the ureteral meatus is used as a handle for manipulation. All periureteral tissue is preserved with the ureter. The length of distal ureter needed for urethral reconstruction is, thereby, meticulously dissected with its blood supply, which in the pelvis enters .from the lateral aspect of the ureter. In each patient we were able to identify and preserve a sizable branch of the internal iliac artery supplying the distal ureter. Other nutrient vessels are divided only as necessary for adequate mobilization of the distal ureteral segment. At the proximal end of the intended segment we place a tight double-wrapped vessel loop Accepted for publication October 21, 1987.
or atraumatic clamp on the ureter for several minutes to interrupt from above longitudinal arterial inflow. We then are able to ensure viability of the ureteral segment before division of the ureter (fig. 1). This well vascularized ureteral segment is mobilized carefully with its pedicle to reach the perineum. Occasionally, it is necessary to rotate the ureteral segment 180 degrees after mobilization. One end of the segment is implanted into a bladder or bowel reservoir in a standard antirefluxing manner to provide a continence mechanism. The other end of the segment is brought to the perineum in the female patient as a catheterizable urethra or to the distal urethra in the male patient for anastomosis. We leave a stent through the ureteral segment during healing until intermittent catheterization is begun. Transureteroureterostomy then provides drainage for the ipsilateral kidney. We have used this method in 8 patients with a diagnosis of bladder exstrophy in 4, cloacal exstrophy in 3 and an imperforate anus with hypoplasia of the bladder neck and urethra in 1. All operations were performed by or directly supervised by the senior author (M. E. M.). An isolated segment of the right ureter was used in 6 patients and the left ureter was used in 2. Associated procedures included transureteroureterostomy in 5 cases, creation of an intestinal urinary reservoir in 4, intestinocystoplasty in 2, closure of vesicostomy in 1 and nephroureterectomy in 1. Continence was based on tunneled submucosal implantation of the isolated ureteral segment into the bladder in 4 cases, gastric reservoir in 2 and a colonic reservoir in a Leadbetter fashion in 2. 6 An antireflux procedure was required in 7 patients, which included a tunneled submucosal reimplant of ureter into bladder in 4 cases, a gastric reservoir in 2 and a colonic reservoir in 1. CASE HISTORIES
Case 1 (fig. 2). M. L., an 8-year-old girl with bladder exstrophy, had undergone urinary diversion via ureterosigmoidostomy when she was 1 year old and cystectomy when she was 3 years old. Although renal function was normal, she had experienced several recent episodes of pyelonephritis, as well as chronic diarrhea and soilage at night. In an effort to reduce infection and potential for tumor development, reconstruction was undertaken. The procedure began with a limited sigmoid colon resection,
1282
1283
URETHRAL REPLACEMENT WITH URETER Bilateral reflux
~
L Before division of ureter viability of isolated ureteral segment is tested by compression of longitudinal arterial flow from above using a vessel loop.
t,
(7\
(i ~) ::,,,d\vir,: reimplants /
,),.~~<)
~; FIG. 2. Case 1. Reconstruction consists of take-down of ureterosigmoidostomies and resection of sigmoid colon, including anastomotic eites, Segment of descending colon is used to construct urinary reservoir and isolated right distal ureteral segment is tunneled into reservoir to continent urethra.
the ureterosigmoidostomy anastomotic sites. An isolated descending colonic segment immediately proximal to the resected segment was used for creation of a urinary reservoir. The isolated segment was opened along the antimesenteric and re-configured. Having isolated the distal 8 cm. of ureter on a vascular pedicle from the right internal iliac artery, the distal end of this segment was brought to the and secured with circumferential sutures. The proxend of the segment was tunneled 3 cm. under the tenia of the reservoir for continence. Both ureters were tunneled along the tenia of the reservoir to prevent reflux. These reimplantahons were done in the manner described by Leadbetter 6 and Hendren. 7 At 10-month followup the patient was completely dry with catheterization at intervals as long as 6 hours. She has been free of infections and the upper tracts have remained stable. Case 2 3). M. a 2-year-old girl with cloacal exstrophy, had undergone abdominal wall closure, bladder closure and end as a newborn. She had a small bladder, total inconbilateral reflux. In an effort to avoid 2 stomas total reconstruction of the lower urinary tract was undertaken. A reservoir was created by re-configuration of an isolated segment of stomach brought into the pelvis on a pedicle of the left gastroepiploic artery. The distal right ureter was isolated on a pedicle from the right internal iliac artery and the diE:tal end of this segment was brought to the perineum and
.un,w.·vm.,,.,
)
"--' Ct.-..,.,. ~
perineum as vagina
FIG. 3. Case 2. Owing to deficiency of bowel urinary reservoir is constructed from gastric segment. Continence is based on segment of distal right ureter. Proximal portion is tunneled into reservoir and distal end is brought to perineum as orthotopic meatus. Native bladder is brought posterior to form vagina.
secured. The proximal end was tunneled between gastric mucosa and muscle for continence. Right-to-left transureteroureterostomy and a tunneled reimplant of the left ureter into the reservoir were performed. The native bladder was moved posterior to create an adequate vagina. At 8-month followup the patient was dry with catheterization up to every 6 hours and the upper tracts were stable. Case 3 (fig. 4). T. I., a 6-year-old boy with bladder exstrophy, had undergone abdominal wall and bladder closure as a newborn, bilateral cross-trigonal ureteral reimplantation when he was 1 year old, and a Young-Dees-Leadbetter bladder neck repair and distal urethroplasty when he was 4 years old. Optical urethrotomy was performed when the boy was 5 years old because of a proximal stricture. The patient had a small capacity bladder, left vesicoureteral reflux, a dense proximal urethral stricture and incontinence. Reconstruction included left-to-right transureteroureterostorny and a right cross-trigonal reimplant into the bladder. The native proximal urethra was divided at the bladder neck and incised longitudinally across the stricture in preparation for a patch urethroplasty. The isolated distal left ureter was tunneled submucosally at the bladder neck to provide continence. The distal end of the segment was incised and used as a patch for the urethral stricture. A sigmoid patch was used to augment the bladder for increased compliance and capacity. This patient currently is dry with catheterization every 4 hours. He had had some difficulty with catheterization 6 months postoperatively but this resolved completely after several days with an indwelling catheter. Case 4 (fig. 5). J. W., a 2-year-old boy, was born with an imperforate anus, patent urachus, massive bilateral reflux, hypoplasia of the bladder neck and urethra, and dysmorphism of the penis with 1 corporeal body. He had undergone sigmoid loop colostomy, resection of the patent urachus and creation of a vesicostomy as a newborn. Colostomy take-down and rectal pull-through had been performed when he was 1 year old. Reconstruction included left-to-right transureteroureterostomy and right tapered, cross-trigonal ureteroneocystostomy. The isolated distal left ureter was brought on its pedicle to the perineum. This segment was tapered and the distal end was brought through the urogenital diaphragm as a perinea! urethrostomy. Proximally, the ureteral segment was tunneled submucosally at the bladder neck. The vesicostomy was then closed. Meatotomy was required 9 months after reconstruction for meatal stenosis of the urethrostomy. At I-year followup the upper tracts were stable and the patient was free of infections.
1284
MITCHELL, ADAMS AND RINK DISCUSSION
Resect stricture ~
FIG. 4. Case 3. Sigmoid cystoplasty is performed. Proxi~al end ~f isolated left distal ureter is tunneled at bladder neck to provide continence. Distal end of ureteral segment is opened and used as cap for prostatic urethra to repair stricture.
FIG. 5. Case 4. Proximal urethra is constructed from isolated left distal ureteral segment. Proximally, ureteral segment is tapered and tunneled at bladder neck. Distal end of segment is brought through urogenital diaphragm to mid scrotum.
He remains dry for 3 to 4-hour intervals and he voids to completion with a good stream. RESULTS
The average followup for this group of patients is 13.5 months (range 5 to 44 months) and the average patient age is 4.7 years (range 1.7 to 8.3 years). All 8 patients had normal renal function preoperatively, which has remained unchanged during followup. In no patient has metabolic acidosis developed. Transient hydronephrosis developed in 1 child secondary to ureterovesical junction obstruction, which was treated by ureteral meatotomy. The other patients have demonstrated stable upper tracts on x-ray studies. No patient has had vesicoureteral reflux. Four patients have maintained sterile urine. Of the 4 patients with positive urine cultures 3 have been asymptomatic and 1 had lower abdominal pain. No patient has had a suspected renal infection (fever or systemic illness). Patients are considered to be continent if they stay completely dry on a reasonable schedule of catheterization or voiding. Of our patients 6 require clean intermittent catheterization and all are dry between catheterizations. One boy had had temporary difficulty with catheterization beyond the anastomosis of the distal urethra and the ureteral segment, which resolved after several days with an indwelling catheter. Recently, 1 girl suffered a false passage and presently she ~as an indwelling catheter during healing. One patient required a secondary ileal augmentation of a colonic reservoir to achieve continence. Of the 2 patients who void spontaneously with small residuals 1 is dry between voidings every 3 to 4 hours and 1 has been dry for intervals of up to 3 hours but he is not yet toilet-trained. Whether this patient will truly be continent remains to be seen.
True continence after reconstruction in patients with congenital malformations of the bladder neck and urethra has been difficult to achieve. Leadbetter's experience with his modification of the Young-Dees bladder neck procedure revealed a success rate of about 60 per cent. 8 Tanagho reported a success rate of 71.5 per cent using tubularization of anterior bladder musculature. 9 Early results with the artificial sphincter are encouraging, 10• 11 although a sphincter around a previously reconstructed urethra can lead to erosion. 11 Recently, Kropp and Angwafo reported their experience with the creation of a prox imal urethra from anterior bladder wall in the myelomeningocele population. 12 Duckett and Snyder reported the use of the appendix and distal ureter as a catheterizable segment5 as described by Mitrofanoff. 4 Our application merely is an extension of these procedures in an effort to create a continent urethra when none was previously available. Of our 8 patients 7 had exstrophy. Use of the ureter in such a manner requires a knowledge of the ureteral blood supply. Branches from the renal artery proximally and vesical arteries distally are the most consistent of the ureteral blood supply. 13 ' 14 In between, branches from the aorta, gonadal arteries, common iliac arteries, external iliac arteries and internal iliac arteries are much more variable, although almost all ureters receive at least 1 such intermediate vessel.1 3 • 14 Meigs believed that a vessel from the internal iliac artery was the most consistent and important for intermediate ureteral blood supply. 15 Also, the ureter usually is served by a rich longitudinal network of vessels within its adventitia. When preserved this network allows use of an isolated segment of ureter based on a pedicle of a single nutrient artery. Some early anatomical dissections suggest that about 10 per cent of the ureters lack a sufficient network of these collaterals, 13 and this procedure should be avoided in such ureters. Therefore, the distal ureter must be dissected from the bladder and pelvis with great care. We then test for adequate distal blood flow by compression of the upper portion of the distal ureteral segment before division. If blood supply to this segment is adequate it can be used reliably as a pedicle graft. All 8 patients are seemingly continent and 6 are dry with intermittent clean catheterization. Interestingly, the remaining 2 patients are voiding spontaneously. Both have the native bladder without augmentation. One is continent and the other has dry intervals of up to 3 hours but he is not yet toilettrained. This result suggests that voluntary continence may be achieved if the ureteral segment is brought through the pelvic diaphragm in a young child. Before use of this procedure we expect the patient to be totally reliant on intermittent catheterization to empty the bladder/reservoir. The patient and family must understand and accept this commitment if success is to be achieved. Any question about this commitment is considered a contraindication to this or any continence procedure in these patients. Although the duration of followup on our initial 8 patients is short, we believe that our results are encouraging. This technique allows another option for continent lower urinary tract reconstruction in this most difficult group of patients. REFERENCES
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URETHRAL REPLACEMENT WITH URETER
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