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Exstrophy of the Bladder—an Alternative Method of Management
Vol. 115, February Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright© 1976 by The Williams & Wilkins Co.
EXSTROPHY OF THE BLADDER-AN ALTERNATIVE METHOD OF MANAGEMENT W. HARDY HENDREN From the Division of Pediatric Surgery, Massachusetts General Hospital and the Department of Surgery, Harvard ]Medical School, Boston, l\llassachusetts
ABSTRACT
Sixteen bladder m"" t- ~~n the urine into a non-refluxing colon conduit. There vvere 4 new, cases and 12 had been diverted earlier ileal conduit. 11 conduits were detached from the abdominal wall and end-to-side to the colon. This method has 0v.,,.,o.,u" to date but long-term assessrnent will be needed. This ~u""''''°·J diversion appears to be a v,my to manage new who have been diverted an ilea! conduit. From our colon conduits we conclude also that it is: 1) a better method than ileal for 2) indicated for with an ilea! who are not well and 3) useful >J~oc,,u,,c, undergoing anterior providing the of joining it to the rectosigmoid if there is no recurrent disease or radiation Exstrophy of the bladder is one of the most serious congenital malformations of the urinary tract for patient and surgeon. At the outset the patient is usually well except when there are other major anomalies, such as cloaca! exstrophy. The upper tracts are usually normal although the ureters are sometimes slightly dilated from angulation owing to prolapse of the exstrophied bladder. The surgeon's goal in treating these children should be to have a child with dry pants who is still well and whose upper tracts remain normal. Additionally, a good cosmetic appearance and ability to function sexually are of great importance. Kidney infection, hydronephrosis, stone formation, and long and social failure should be avoided. All of these complications are seen too often after an operation for exstrophy of the bladder. Many methods have been used to treat these children, including primary ureterosigmoidostomy, ,_, primary functional closure of the bladder,,_, ureteroileal colostomy,• rectal bladder with adjacent colon pull through, 9 vesicocolostomy with proximal colostomy, 10 permanent ilea! conduit diversion, vesicoileosigmoidostomy 11 and so forth. None of these methods has had consistent success. My own experience in a personal review of a large series of patients treated by primary ureterosigmoidostomy, later reported by Bennett, 2 disclosed an impressive variability in results. In some patients long-term result showed great success with normal upper tracts, while in others there was progressive renal deterioration with ultimate death or permanent urinary diversion. The majority of those patients had been treated by a tunneled ureterosigmoidostomy with simple dunking of the tip of the ureter into the colon. Today, a preferred technique is a primary anastomotic union of the ureter to the colon mucosa and a tunnel as described by Leadbetter and Clarke. 12 • 13 An inflammatory pseudopolyp with obstruction commonly resulted from simple dunking of the ureteral tip, requiring a secondary operation. Children whose ureterosigmoidostomy had been done early, at ages 1 to 2 years, often had several years of combined fecal and urinary incontinence, a sometimes difficult social problem. Children whose ureterosigmoidostomy had been deferred until they were old enough to have stool sometimes sustained had unUrological Association, IV.Iiarni
repaired genitalia. Serious upper tract damage occurred soon in those children with a technical problem "'""u,,u,u to primary ureterosigmoidostomy. Our own relatively small experience with primary closure of bladder exstrophy, plus observation of some of those patients closed elsewhere, and a review of the literature on this approach 3 - 7 have led us to conclude that the likelihood of an optimal result in an individual child is small (that is normal continence, normal upper tracts and freedom from infection) even in the hands of surgeons with a special interest in that method of management. For example Megalli and Lattimer reported 89 nn,n1,,rs1 of which only 1 female subject achieved excellent control, 25 were lost to and 26 underwent diversion. 3 Williams and Keeton reported that about half of their recent cases were considered reasonable candidates for primary closure and that 6 of 19 had acceptable continence in their latest series. 4 Marshall and Muecke had satisfactory results in 20 per cent of their cases. 5 These results in experienced hands should certainly discourage anyone to attempt the operation who does not deal frequently with exstrophy patients. Herein we present an alternative approach that we have used in patients with bladder exstrophy since March 1971. It consists of performing an isolated colon conduit with nonrefluxing ureterosigmoid anastomoses followed by anastomosis of the conduit to the rectosigmoid colon, thereby accomplishing a staged ureterosigmoidostorny. To date 16 patients were managed in this fashion. Of these 16 patients 12 were treated originally by ilea! loop urinary diversion before we began using the colon for a conduit. After prolonged observation in 8 of the 12 cases the conduit was taken down and anastomosed to the colon and 2 other cases will be completed in the near future. The remaining 2 patients will likely continue with a nonrefluxing colon conduit because the upper tracts were already so dilated as to preclude risking subsequent anastomosis to the colon and possible ascending infection. Four new, unoperated cases of bladder exstrophy were treated. A colon conduit was made when the patient was approximately 1 year old and the genitalia were repaired about 6 months later. Of these 4 children 3 underwent anastomosis of the colon conduit to the were 4 to 5 years old and bowel trained. The is yet too young to consider this. Thus, of 16 beer. 3 will be hooked ;.ip within the
HENDREN
196
next year and 2 will likely remain with a colon conduit because they had advanced upper tract dilatation before this approach was started. Even the 2 who will remain with a colon conduit should be improved for there is much evidence that the long-term result of an ileal loop in young patients is complicated by a high rate of progressive deterioration. 14- 16 Richie and associates showed in a laboratory study a high incidence of pyelonephritis from reflux with an ileal loop but a low incidence in colon conduit with non-refluxing anastomoses. 17 • 18 TECHNIQUE OF OPERATION
Figures 1 to 5 show the technique used in performing a non-refluxing conduit from the sigmoid colon. In 1 case the sigmoid was unsatisfactory and a segment of transverse colon was used instead, based on middle colic blood supply. A tunneling implantation was performed with 27 of 32 ureters. Figure 6 shows management for a dilated ureter. It should be tapered before implantation into the colon. 19 Nine of the 32 ureters were tapered. Ureteral dilatation was formerly a major contraindication to ureterosigmoidostomy. However, that has not been the case when the ureters are tapered and implanted into a defunctioned conduit with later implantation into the colon. In some cases a ureter was too short to permit 2 satisfactory
ureterocolic anastomoses. This finding was managed by implanting the better ureter by a non-refluxing technique into the colon together with transureteroureterostomy for the other ureter (fig. 7). This technique was used for 2 ureters. Figure 8 shows the Mathisen nipple ureterosigmoidostomy. 20 It was used for 3 ureters in this series when the ureter was too short for a satisfactory tunneling implantation. This technique is preferred by many European surgeons. Figure 9 shows the second stage, that is implantation of the colon conduit at a later date. This was accomplished in 11 of the 16 cases to date. CASE REPORTS
The 6 cases herein reported will illustrate certain aspects of the problem of staged ureterocolic diversion of urine in patients with bladder exstrophy. Case 1 (fig. 10). A 10-year-old boy was first seen in 1965 with unoperated exstrophy of the bladder. An ileal conduit was performed. Except for encrustation of the stoma with bleeding, the patient did well and was free of urinary infection. In July 1973, when he was 18 years old, the ileal loop was removed and a non-refluxing colon conduit was constructed. The ureters were only slightly dilated and were of ample length for long, tunneling anastomoses without tapering. Postoperative excretory urogram (NP) remained normal. Resting pressure in the loop measured 4 cm. water. A loopogram showed no low pressure reflux but slight reflux on the left side with pressures higher than 35 cm. water. In April 1974 the conduit was anastomosed to the rectosigmoid. Convalescence was uneventful with satisfactory control of urine by rectum and no clinical evidence of urinary infection. An IVP 1 year postoperatively was normal. Blood chemical values included sodium 142 mEq. per 1., potassium 4.1, chloride 104, carbon dioxide 25, blood urea nitrogen (BUN) 29 and creatinine 0.7. Comment: The upper tract had remained normal with an ileal loop and did not change when converted to a colon conduit. There are many patients with bladder exstrophy like this, that is with normal upper tracts and an ileal loop, who could be managed in the same way. It has improved the patient's life style greatly to be rid of the appliance, which has not been accompanied by renal deterioration. Case 2 (fig. 11). A female newborn was seen in 1963 with bladder exstrophy. An ileal conduit was performed in October 1964. In June 1965 cystectomy with epispadias repair was performed. An IVP showed some hydronephrosis on the right side and revision of the stoma was performed to correct stenosis. Dilatation persisted. In June 1967 the stoma was revised a second time. Urinary infection occurred and the child was maintained on long-term antimicrobial therapy. In June 1971 a colon conduit was performed. Because the right ureter was slightly short the butt of the loop was sewn into the right gutter to achieve a satisfactory right tunneling anastomosis. The left ureter was of ample length. Neither was large enough to require tapering. Postoperative IVP showed dilatation from edema 1 week later but repeat IVP was normal 1 month later. Urine was consistently sterile. A loopogram showed no reflux at pressures up to 60 cm. water. Resting pressure in the loop Colon segment was 10 cm. with the patient in the supine position and 15 cm. in the sitting position. In August 1972 the conduit was anastofor conduit mosed to the colon. The patient has been well subsequently, FIG. 1. Segment of sigmoid is selected with appropriate blood supply. Bowel must be thoroughly prepared. Usually splenic flexure is on no medication and leading a normal life. Rectal control of mobilized to permit end-to-end restoration of continuity without urine is satisfactory. IVP remains normal. Serum chemistry tension of descending colon to rectosigmoid. We prefer 2 layers of studies included sodium 144 mEq. per 1., potassium 4.1, interrupted arterial silk with atraumatic needles for anastomosis. chloride 106, carbon dioxide 22 and BUN 16. Proximal end of conduit is closed with 2 inverting layers of chromic Comment: The upper tract improved greatly by getting rid catgut. Non-absorbable suture material should not be used lest a of the refluxing ilea! loop in exchange for a non-refluxing colon suture work its way into lumen of conduit and cause stone formation. Length of sigmoid should be amply long for 2 reasons: 1) it sometimes conduit. The patient had had many episodes of bacilluria contracts and shortens while one is working and 2) if conduit is to be originally, which cleared after the colon conduit diversion. The attached back to colon later this allows the surgeon to resect and discard stoma and that length which was in abdominal wall and upper tract remains normal almost 3 years after joining the perform rejoining anastomosis with segment of fresh bowel wall. conduit to the colon.
197
EXSTROPHY OF BLADDER
B
Distal end of
\\,
FIG. 2. A, conduit is rotated clockwise 180 degrees, anchoring proximal end at aortic bifurcation with several sutures. If ureters are too short, as in some patients with prior ilea! loop, it may be anchored at higher level or in left or right gutter instead of midline. In 1 case sigmoid conduit was not possible, and so we used segment of transverse colon based on blood supply of mid colic artery. B, conduit anchored and traps closed. Mesentery of conduit lies superiorly. Note proximity of ureters to base of conduit. When ureters are mobilized, all of periureteral adventitia should be spared, for it is important with respect to good blood supply of ureter.
A
B
C
II
End of ureter to colon mucosa
with interrupted 5-0 or 6-0 chromics
Line of
incision
Mucosa
6-0 tocking suture
FIG. 3. A, infiltration with saline facilitates making tunnel. B, undermining seromuscular layer by blunt dissection sparing many small bridging vessels. Separation of mucosa can be facilitated using small peanut gauze sponge. C, ureter placed beneath bridging vessels which arise from mesentery. Ureter is possibly better supported in this position, although we have placed ureter on top of bridging vessels in some cases, which requires lesser degree of mobilization of colon wall. I do not know which position is better but theoretically I prefer beneath the vessels.
Case 3 (fig. 12). A 2-year-old boy was referred in 1961 with severe bilateral hydronephrosis and recurrent urinary infection following primary closure of bladder exstrophy done elsewhere during infancy. There had been 8 previous hospitalizations during the first 2 years of life. Ileal loop urinary diversion was performed. The ureters, which had been dilated before performing the ileal loop diversion, remained dilated. The stoma was tight and required revision in 1962 when the patient was 3 i12 years old. There was chronic bacilluria. In 1966 both ureters were tapered, 1 side at a time, to improve their emptying. Although the patient did well clinically, there was intermittent chronic bacilluria requiring long-term antimicrobial treatment. In May 1973, when the patient was 15 years old, the ileal loop was removed and a non-refluxing colon conduit was performed. The ureters were too short for tunneling anastomoses. The left ureter was anastomosed to the colon by Mathisen's method and right-to-left transureteroureterostomy was performed. The patient has been completely well clinically since we performed the colon conduit. Urine cultures have remained consistently sterile. The colon conduit stoma has shown no tendency to stenosis. The patient expressed a marked preference for the colon conduit stoma compared to the
ileal stoma, which required dilatation, revision and sometimes bled during athletics. A loopograrh showed no reflux even at a high pressure of 60 cm. water. Although the patient has been well clinically the upper tracts remain too dilated to consider implanting the colon conduit into the colon. It is likely that this youngster will remain with a permanent colon conduit for life. Comment: It is said frequently that there is little to lose by primary closure of the exstrophied bladder and that one can proceed later to diversion if that does not succeed. In this case the urinary tract was originally normal. Primary closure resulted in immediate hydronephrosis and severe urinary infection ensued with permanent damage to both upper tracts. This damage did not improve with prolonged ileal loop urinary diversion but likely progressed. If a non-refluxing colon conduit had been used when the patient was seen originally, this course might have been altered. At present further change in the drainage status seems unlikely. This case emphasizes that an attempt at functional bladder closure can damage the upper tracts to a degree that only permanent diversion will be feasible. Case 4 (fig. 13). A 19-year-old male patient was referred in
198
HENDREN
B
C
FIG. 4. Details of anastomosis of ureter to colon. Note traction suture partially transecting ureter, leaving a bit attached for traction to minimize handling ureter with forceps. This bit is resected before last few sutures are placed. Note that ureter is not spatulated unless it is particularly small, for carefully performed anastomosis with interrupted suture material does not generally stenose. Anastomosis is performed with 5 and 6-zero chromic catgut. Opening in colon mucosa should not be too large. This is inverting anastomosis with all knots on inside except for last 3 sutures, which are placed before tying them and which have knots on outside. Drainage stent catheter is not used unless ureter is tapered.
December 1973 with an ilea! conduit and gradually deteriorating upper tracts. When he was 2 years old the bladder was anastomosed to the colon with resultant vesicocolic cutaneous fistula. Partial cystectomy and closure of the fistula were performed. In 1957 bilateral ureterosigmoidostomy was performed followed by repair of epispadias in 1958. In 1962 ilea! loop urinary diversion was performed. There were multiple revisions of the stoma and revision of both ureteroileal anastomoses in 1970. The patient was maintained on long-term antimicrobial therapy for chronic urinary infection. In December 1973 the ilea! loop was removed and a sigmoid colon conduit was constructed. Both ureters were dilated, tapered and tunneled. Postoperatively, the patient did well with consistently sterile urine and marked improvement in the upper tracts. A loopogram showed no low pressure reflux but moderate reflux on the left side with pressures higher than 40 cm. water. In May 1975 the conduit was anastomosed to the rectosigmoid. This patient is the most recently completed case. Comment: The appearance of the upper tracts and the
amount of ureteral dilatation present when the patient was first seen in 1973 made it seem unlikely that he would be a candidate for anything but continuing permanent diversion. The switch from a refluxing ilea! loop to a non-refluxing colon conduit resulted in marked improvement in the appearance of the upper tracts and consistently sterile urine. Should there be any recurrence of upper tract dilatation or clinical evidence of infection following implantation of the conduit into the colon, it would be brought back to the surface. This point has been stressed to each of these patients before implanting the colon conduit into the colon. Case 5 (fig. 14). A 6-month-old female infant was referred with exstrophy and extensive hemangioma involving the lower abdomen, bladder and perineum. An IVP was normal. On observation there was spontaneous regression of the hemangioma. In July 1971 a colon conduit was performed with an uneventful course. A loopogram months later showed no low pressure reflux but moderate bilateral reflux at pressures more than 40 cm. water. Epispadias repair and cystectomy were performed in February 1972. Repeat loopogram in October 1973 showed reflux at pressures higher than 50 cm. water. Bowel control was reasonably good. The conduit was anastomosed to the colon in November 1973. The control of liquid by rectum during the next 6 months was poor at first but it improved gradually and, when the patient was 5 years old, control was satisfactory with wetting only when she was tired. An IVP was normal. Serum electrolytes included sodium 142 mEq. per I., potassium 3.8, chloride 103, carbon dioxide 23 and BUN 23. This child is an essentially normal 5 year old who attends school and plays with her peers. She does not suffer recurrent urinary infection and is dry except for occasional accidents when she is overly tired. Comment: Each of the 4 children treated in this way has had a remarkably normal early childhood. They have not spent much time in the hospital and have not been forced to tolerate the onus of being wet all the time, a cause for ridicule and shame. Considering the complexity of the exstrophy anomaly, the children have required only infrequent visits to a physician. Case 6 (fig. 15). A male newborn was first seen in 1963 with bladder exstrophy. Ilea! loop urinary diversion was performed when he was 2 years old. In April 1966 cystectomy and epispadias repair were done, and a stoma revision was performed in August 1967. There was progressive dilatation of both upper tracts yet an open stoma and no loop residual, thought secondary to a chronically spastic loop. In December 1968 the first loop was removed and a new ilea! conduit was fashioned. The upper tract remained dilated. In June 197 4 the ilea! loop was removed and a non-refluxing colon conduit with bilateral ureteral tapering was constructed. Postoperatively the right upper tract returned to normal and the left side showed more dilatation than before. Despite this, the patient was completely well clinically with consistently sterile cultures and on no medication. A left percutaneous antegrade pyelogram disclosed marked obstruction at the tip of the left ureter (fig. 15, B). Cystoscopy of the loop was performed. The right ureterocolic anastomosis could be seen easily and it effluxed intravenous indigo carmine promptly. The left orifice could not be seen. A small catheter was inserted into the left kidney percutaneously followed by a drip containing indigo carmine into the kidney, which helped identify the stenotic left orifice. A Fogarty embolectomy arterial catheter was passed, inflating the balloon with 1 cc air. With malleable retractors to visualize the interior of the conduit, traction on the balloon catheter pulled the stenotic orifice into view. Meatotomy was performed. Comment: It is inevitable that in performing ureterosigmoid anastomoses there will be occasional technical failures, particularly if the ureter is dilated and requires tapering. This is the first case that we encountered in performing 26 colon conduits for bladder exstrophy, meningomyelocele and sarcoma of the
199
EXSTROPHY OF BLADDER
A
B I
I I
I
'
Closed
para median seromuscular layer and mucoso
incision
FIG. 5. A, completed conduit. Ureters lie parallel and at same level of conduit. There is good blood supply of strip of bowel between ureters because medial flap of each tunnel was not mobilized. Seromuscular closure over ureter is performed with colored non-absorbable suture material to permit easy identification of location of ureters in future in cases in which conduit will be taken down and anastomosed into colon. Stoma can be placed in right lower quadrant at site of prior ilea! loop stoma in midline or in left lower quadrant according to particulars of given case. B, completed conduit. MATHISEN
FOR DILATED URETER
URETEROSIGMOIDOSTOMY
longitudinal
resection
FIG. 6. Management of dilated ureter by appropriate tapering. It is important to preserve vascularity of ureter by not making it too narrow. Number 5 or 8 plastic feeding catheter is used for temporary drainage for 10 to 12 days until longitudinal suture line is healed. Catheter is sutured to stoma bud. IF ONE
Left to right
URETER IS TOO SHORT Right to left
FIG. 8. Technique of ureterosigmoidostomy described by Mathisen, which we have used when ureter did not seem well suited for usual long ureterosigmoid tunnel (too short or too thick walled). It is best to fashion periureteral flap of colon a little at a time as anastomosis progresses, in order not to make it too narrow. Flap must be wide enough to wrap around ureter without compressing it. FIG. 7. Transureteroureterostomy can be used if only 1 ureter is suitable for anastomosis to colon.
prostate. Although this ureter was obstructed, there was no problem with urinary infection and ascending pyelonephritis. If this ureterosigmoid anastomosis had been into a functioning segment of colon filled with feces there would have undoubtedly been immediate pyelonephritis and probably severe
damage to the kidney. Because this stenosis was limited to the tip of the ureter it could be managed in an unusual way, that is working down inside the loop via the stoma, avoiding laparotomy. The tunnel length still looks adequate even after meatotomy. If subsequent x-rays show that obstruction is relieved and there is no reflux, the conduit will be implanted into the colon. If there is continuing obstruction, an open
200
HENDREN
operation will be performed. There would be the option of primary ureterosigmoidostomy, those patients who had asredoing that anastomosis or performing left-to-right trans- cending infection usually began to show it relatively soon. There is no way that one can be absolutely certain that no ureteroureterostomy. bacteria reached the upper tracts in these cases, except by needle puncture aspiration of the kidney. This does not seem POSTOPERATIVE RESULTS AND COMPLICATIONS justified in a patient who is well and whose IVP is stable. No Non-refluxing colon conduit was performed in 26 patients bowel gas has been noted in the upper tracts of any patient. from March 1973 through May 1975. This total included 16 patients with bladder exstrophy, 9 with neurogenic bladder and 1 with prostatic sarcoma. There was 1 obstructed ureter (case 6, fig. 15). Postoperative intestinal obstruction requiring laparotomy with lysis of adhesions occurred in 4 patients, 3 of whom had exstrophy. No low pressure reflux was found on the loopogram study in any case. Reflux could be produced in some patients by filling colon conduits with pressures higher than 35 cm. water. There was no evidence of postoperative l-'Yelonephritis or upper tract deterioration, except in the 1 case with ureteral obstruction. Recurrent bacilluria present in some patients with ileal loop preoperatively ceased when they were converted to a non-refluxing colon conduit. In 11 of the 16 exstrophy patients undergoing the second stage, that is implantation of the colon conduit into the colon, the longest is almost 3 years postoperatively and there is no evidence of pyelonephritis clinically or upper tract change by roentgenographic evaluation. Obviously, long-term observation of these cases will be needed. However, in our previous experience with
, I' I
' \
,,- ..... , \
,,\~:
\
', .... _.,,
Closed stoma site
',
'I I
I
,I
\
,,,,
,, :,"
,, I/ I
\
End to side conduit to colon anastomosis
--- I / ,._,;·,
FIG. 9. Subsequent end-to-side anastomosis of conduit to colon after enough time has passed to be certain that upper tracts remain normal, there is no low pressure reflux and child can retain liquid rectal content,
FIG. 11. Case 2-female patient with prior ilea! loop and mild upper tract dilatation; converted to colon conduit and later implanted into colon. A, IVP December 1970 at age 7 years, 6 years after ilea! loop was performed. Note right ureteral dilatation. B, IVP June 1971, 10 days after colon conduit operation. Note edema around tunneled right ureter (arrows) and bilateral hydronephrosis, often present for several weeks postoperatively. In functional colon this could result in pyelonephritis. It has not caused a problem with infection in defunctioned conduit. IVP 1 month later was normal. C, loopogram December 1971, 6 months after colon conduit. No reflux even at high pressure of 60 cm. water. D, IVP September 1974, more than 2 years after anastomosis of conduit to colon. Note delicate upper tracts, now normal size right ureter and long right ureteral sigmoid tunnel (small arrows). Note anastomosis of conduit to rectosigmoid (large arrow in right lower quadrant).
FIG. 10. Case 1-10-year-old boy with prior ilea! loop and normal upper tracts; converted to colon conduit and later implanted into colon. A, IVP July 1973 at age 18 years shows normal upper tract with ilea! loop present for 8 years. B, IVP February 1974, 8 months.after conversion to non-refluxing colon conduit. Normal IVP. Conduit joined to colon April 1974. C, IVP October 1974, 6 months after joining colon conduit to colon. Patient remains well,
EXSTROPHY OF BLADDER
201
The colon conduit stoma is larger and more bulky than an ilea! loop stoma but it has been singularly free of complications. None has developed stenosis requiring dilatation or revision. There has been no encrustation or stomatitis. All patients with a prior ilea! loop stoma have expressed a preference for the colon conduit stoma. There has been no significant postoperative hyperchloremic acidosis in this small group of patients. A generous fluid intake is encouraged with scheduled voiding every 3 hours to reduce solute reabsorption. Patients with ureterosigmoidostomy absorb chloride and secondarily lose bicarbonate, which can lead
FIG. 14. Case 5-new case of bladdeT exstrophy in 6-month-old female infant: colon conduit at 17 months and implantation of conduit into colon at age 3 years months. IVP January 1974, 3 months after anastomosis of conduit to colon. IVP March 1975, 17 months after anastomosis of conduit to colon. Note conduit filled with dye (arrow).
FIG. 12. Case 3-2-year-old boy with primary bladder closure leading to upper tract deterioration and then ilea! loop diversion; later, colon conduit using Mathisen nipple and transureteroureterostomy; probably unsuited for implantation of conduit into colon. A, retrograde study preoperatively as infant shows normal upper tracts. B, cystogram 2 years after primary bladder closure shows massive bilateral hydronephrosis and hydroureter. Recurrent severe pyelonephritis. C, loopogram December 1966, 4 years after ilea! loop diversion. Right ureter has been tapered and left ureter still dilated. Subsequently tapered. D, IVP February 1973, 11 years after ilea! loop was performed. Patient well clinically but chronic bacilluria requiring therapy. E, retrograde examination inserting cystoscope into colon conduit and catheterizing left ureter implanted by Mathisen nipple technique. Note right-to-left transureteroureterostomy. Drainage films showed prompt emptying. F, IVP November 1974, 18 months after colon conduit operation. Patient is well with stable renal function and no longer has bacilluria, but this degree of upper tract dilatation precludes anastomosis of conduit to colon. This case emphasizes that attempted primary closure of bladder can result in severe upper tract damage which may preclude any alternative approach except permanent diversion.
FIG. 15. Case 6-male patient with ilea! loop with deterioration; converted to colon conduit with bilateral ureteral tapering and stenosis of 1 ureteral anastomosis. A, IVP 9 months after colon conduit operation. Right side normal and hydronephrosis on left side. B, percutaneous left antegrade study April 1975, defining point of obstruction at tip of ureter (arrow). Note dilated segment of ureter in tunnel. Ureteral meatotomy performed through stoma with technical help of Fogarty embolectomy catheter. If this complication had occurred with implantation of ureter into fecal stream, severe pyelonephritis probably would have resulted.
F1G. 13. with deterioration and mega.ureter; converted to non-refluxing colon conduit with bilateral ureterai tapering and subsequent implantation conduit into colon. IVP February 1973 at age 19 years, 11 years after ilea! tract dilatation despite multiple revisions of loop. B, •v••,-,vto·''-"' shows massive reflux, and dilated and tortuous 16 months after colon conduit with bilateral extensive tapering. Normal upper tract, sterile urine 2.nd implanted into ,:olon.
202
HENDREN
to secondary acidosis with volume retraction. If the serum carbon dioxide decreases to 20 mEq. per I. (normal 22 to 28 mEq. per I.) we prescribe bicarbonate in a dosage of 2 to 4 mEq. per kg. body weight. A 600 mg. tablet of sodium bicarbonate contains 7.8 mEq. bicarbonate. Patients with ureterosigmoidostomy can handle the solute reabsorption and its re-excretion if they have reasonable renal function and enough fluid intake. It is important to emphasize to them the need to come for temporary intravenous fluid maintenance during an intercurrent illness when fluid intake is poor, such as gastroenteritis with vomiting or diarrhea. Otherwise, these patients can become uremic quickly despite good renal function. The use of sigmoid colon for a urinary conduit is not new, having been reported more than 20 years ago by Gross. 21 Mogg prefers using the colon conduit in children needing diversion for myelomeningocele. 22 Almost 50 years ago Coffey emphasized that an intramural tunnel improves the success of ureterosigmoidostomy. 23 Leadbetter and Clarke showed that meticulous anastomosis of the end of the ureter to the colonic mucosa, together with an intramural tunnel gives improved results. 12 • 13 The Mathisen nipple also has proved effective. 20 We believe that this staged method of ureterosigmoid diversion in patients with bladder exstrophy is preferred to a primary ureterosigmoid anastomosis for several reasons: 1) If primary ureterosigmoidostomy is performed on a small infant there is usually combined fecal and urinary incontinence, which is malodorous for several years. These children do not usually gain excellent socially acceptable control of liquid by rectum until they are about 5 years old or sometimes even older. Thus, we are opposed to performing diversion of the urine into the colon until a child has a reasonable chance of being continent of liquid in the rectum. This can be important for social reasons. 2) In former years many of these children were allowed to reach ages 4 to 5 years without any surgical correction, which is equally bad from the psychological standpoint. They are well aware of their genital anomaly at about age 2 years. 3) Performing a non-refluxing conduit when the child is about 1 year old provides a method to get the patient dry, albeit with an appliance, and then allows early repair of the genitalia, an important concern to the parents and child. The patient then spends the next several years dry with satisfactory appearance, free of urinary infection and not in and out of the hospital for recurrent infection, repeated operations to achieve continence and so forth. 4) This method permits anastomosing the ureters to a clean conduit and prevents patients from being bathed in fecal material while healing. The early postoperative IVP after a technically successful ureteral reimplantation into the bladder or ureteral anastomosis to the colon may show edema with temporary upper tract dilatation (fig. 11, B). In a colon filled with fecal material this can result in immediate pyelonephritis. Conversely, the 1 patient in this series with an obstructed ureter (fig. 15) had no clinical evidence of infection almost certainly because the ureterosigmoidostomy was in a clean isolated conduit. Also, we have seen reflux demonstrated in patients by a cystogram done early after successful ureteral reimplantation while edema persists, yet disappear as healing progresses. It is likely that this is true with a new ureterosigmoidostomy, that is some weeks must pass before the tunneled implant is competent to prevent reflux of colon content into the lower ureter. 5) This method permits prolonged observation of the ureterosigmoid anastomoses by IVP and loopogram to make certain that there is no obstruction or low pressure reflux before taking the step of implanting the conduit into the colon. We anticipate that this approach, although requiring an extra operation, will minimize the complication of ureterosigmoidostomy. In these 11 exstrophy cases and a child with anterior pelvic exenteration for sarcoma there has been no late complication to date. The primary non-refluxing colon conduit is a somewhat longer and more technically exacting operation than a classical
ilea! loop procedure. 24 Whereas the ilea! loop generally takes 2 ½ to 3 hours to perform, a non-refluxing colon conduit generally requires at least 1 to 2 hours longer, especially if ureteral tapering is required. Converting a patient from an ilea! conduit to a non-refluxing conduit takes even longer. In 1 patient ( case 4) with many previous intra-abdominal operations and bilateraily dilated, tortuous ureters, the procedure lasted for 11 hours. Present day excellent anesthesia, intraoperative monitoring, effective antibiotics and so forth make this possible, giving the surgeon time to do what is required, which was clearly not always possible years ago. REFERENCES
1. Spence, H. M.: Ureterosigmoidostomy for exstrophy of the bladder. Results in a personal series of thirty-one cases. Brit. J. Urol., 38: 36, 1966. 2. Bennett, A. H.: Exstrophy of bladder treated by ureterosigmoidostomies: long-term evaluation. Urology, 2: 165, 1973. 3. Megalli, M. and Lattimer, J. K.: Review of the management of 140 cases of exstrophy of the bladder. J. Urol., 109: 246, 1973. 4. Williams, D. I. and Keeton, J. E.: Further progress with reconstruction of the exstrophied bladder. Brit. J. Surg., 60: 203, 1973. 5. Marshall, V. F. and Muecke, E. C.: Functional closure of typical exstrophy of the bladder. J. Urol., 104: 205, 1970. 6. Chisholm, T. C.: Exstrophy of the urinary bladder. In: Pediatric Surgery, 2nd ed. Edited by W. T. Mustard, M. M. Ravitch, W. H. Snyder, K. J. Welch and C. D. Benson. Chicago: Year Book Medical Publishers, vol. 2, chapt. 73, 1970. 7. Fisher, J. H. and Retik, A. B.: Exstrophy of the bladder. J. Pediat. Surg., 4: 620, 1969. 8. Houtappel, H. C.: Experience with uretero-ileosigmoidostomy. Brit. J. Urol., 35: 277, 1963. 9. Lowsley, 0. S. and Johnson, T. H.: A new operation for creation of an artificial bladder with voluntary control of urine and feces. J. Urol., 73: 83, 1955. 10. Boyce, W. H.: A new concept concerning treatment of exstrophy of the bladder: 20 years later. J. Urol., 107: 476, 1972. 11. Hays, D. M., Powell, T. 0. and Strauss, J.: Vesicoileosigmoidostomy in the treatment of exstrophy: re-evaluation. Surgery, 66: 1103, 1969. 12. Leadbetter, W. F.: Consideration of problems incident to performance of uretero-enterostomy: report of a technique. J. Urol., 65: 818, 1951. 13. Leadbetter, W. F. and Clarke, B. G.: Five years' experience with uretero-enterostomy by the 'combined' technique. J. Urol., 73: 67, 1955. 14. Retik, A. B., Perlmutter, A. D. and Gross, R. E.: Cutaneous ureteroileostomy in children. New Engl. -J. Med., 277: 217, 1967. 15. Ray, P. and De Domenico, I.: Intestinal conduit urinary diversion in children. Brit. J. Urol., 44: 345, 1972. 16. Smith, E. D.: Follow-up studies on 150 ilea! conduits in children. J. Pediat. Surg., 7: 1, 1972. 17. Richie, J.P., Skinner, D. G. and Waisman, J.: The effect ofreflux on the development of pyelonephritis in urinary diversion: an experimental study. J. Surg. Res., 16: 256, 1974. 18. Richie, J.P. and Skinner, D. G.: Urinary diversion: the physiological rationale for non-refluxing colonic conduits. Brit. J. Urol., 47: 269, 1975. 19. Hendren, W. H.: Operative repair of megaureter in children. J. Urol., 101: 491, 1969. 20. Mathisen, W.: A new method for ureterointestinal anastomosis; preliminary report. Surg., Gynec. & Obst., 96: 255, 1953. 21. Gross, R. E.: Urinary and fecal incontinence of neurogenic origin. In: The Surgery of Infancy and Childhood: Its Principles and Techniques. Philadelphia: W. B. Saunders Co., chapt. 56, p. 740, 1953. 22. Mogg, R. A.: Some observations on urinary diversion. Ann. Roy. Coll. Surg. Engl., 46: 251, 1970. 23. Coffey, R. C.: Transplantation of the ureters into the large intestine. Surg., Gynec. & Obst., 47: 593, 1928. 24. Hendren, W. H_: Nonrefluxing colon conduit for temporary or permanent urinary diversion in children. J. Pediat. Surg., 10: 381, 1975.
THE JOURNAL OF UROLOGY
Copyright© 1976 by The Williams & Wilkins Co.
EXSTROPHY OF THE BLADDER-AN ALTERNATIVE METHOD OF MANAGEMENT W. HARDY HENDREN From the Division of Pediatric Surgery, Massachusetts General Hospital and the Department of Surgery, Harvard ]Medical School, Boston, l\llassachusetts
ABSTRACT
Sixteen bladder m"" t- ~~n the urine into a non-refluxing colon conduit. There vvere 4 new, cases and 12 had been diverted earlier ileal conduit. 11 conduits were detached from the abdominal wall and end-to-side to the colon. This method has 0v.,,.,o.,u" to date but long-term assessrnent will be needed. This ~u""''''°·J diversion appears to be a v,my to manage new who have been diverted an ilea! conduit. From our colon conduits we conclude also that it is: 1) a better method than ileal for 2) indicated for with an ilea! who are not well and 3) useful >J~oc,,u,,c, undergoing anterior providing the of joining it to the rectosigmoid if there is no recurrent disease or radiation Exstrophy of the bladder is one of the most serious congenital malformations of the urinary tract for patient and surgeon. At the outset the patient is usually well except when there are other major anomalies, such as cloaca! exstrophy. The upper tracts are usually normal although the ureters are sometimes slightly dilated from angulation owing to prolapse of the exstrophied bladder. The surgeon's goal in treating these children should be to have a child with dry pants who is still well and whose upper tracts remain normal. Additionally, a good cosmetic appearance and ability to function sexually are of great importance. Kidney infection, hydronephrosis, stone formation, and long and social failure should be avoided. All of these complications are seen too often after an operation for exstrophy of the bladder. Many methods have been used to treat these children, including primary ureterosigmoidostomy, ,_, primary functional closure of the bladder,,_, ureteroileal colostomy,• rectal bladder with adjacent colon pull through, 9 vesicocolostomy with proximal colostomy, 10 permanent ilea! conduit diversion, vesicoileosigmoidostomy 11 and so forth. None of these methods has had consistent success. My own experience in a personal review of a large series of patients treated by primary ureterosigmoidostomy, later reported by Bennett, 2 disclosed an impressive variability in results. In some patients long-term result showed great success with normal upper tracts, while in others there was progressive renal deterioration with ultimate death or permanent urinary diversion. The majority of those patients had been treated by a tunneled ureterosigmoidostomy with simple dunking of the tip of the ureter into the colon. Today, a preferred technique is a primary anastomotic union of the ureter to the colon mucosa and a tunnel as described by Leadbetter and Clarke. 12 • 13 An inflammatory pseudopolyp with obstruction commonly resulted from simple dunking of the ureteral tip, requiring a secondary operation. Children whose ureterosigmoidostomy had been done early, at ages 1 to 2 years, often had several years of combined fecal and urinary incontinence, a sometimes difficult social problem. Children whose ureterosigmoidostomy had been deferred until they were old enough to have stool sometimes sustained had unUrological Association, IV.Iiarni
repaired genitalia. Serious upper tract damage occurred soon in those children with a technical problem "'""u,,u,u to primary ureterosigmoidostomy. Our own relatively small experience with primary closure of bladder exstrophy, plus observation of some of those patients closed elsewhere, and a review of the literature on this approach 3 - 7 have led us to conclude that the likelihood of an optimal result in an individual child is small (that is normal continence, normal upper tracts and freedom from infection) even in the hands of surgeons with a special interest in that method of management. For example Megalli and Lattimer reported 89 nn,n1,,rs1 of which only 1 female subject achieved excellent control, 25 were lost to and 26 underwent diversion. 3 Williams and Keeton reported that about half of their recent cases were considered reasonable candidates for primary closure and that 6 of 19 had acceptable continence in their latest series. 4 Marshall and Muecke had satisfactory results in 20 per cent of their cases. 5 These results in experienced hands should certainly discourage anyone to attempt the operation who does not deal frequently with exstrophy patients. Herein we present an alternative approach that we have used in patients with bladder exstrophy since March 1971. It consists of performing an isolated colon conduit with nonrefluxing ureterosigmoid anastomoses followed by anastomosis of the conduit to the rectosigmoid colon, thereby accomplishing a staged ureterosigmoidostorny. To date 16 patients were managed in this fashion. Of these 16 patients 12 were treated originally by ilea! loop urinary diversion before we began using the colon for a conduit. After prolonged observation in 8 of the 12 cases the conduit was taken down and anastomosed to the colon and 2 other cases will be completed in the near future. The remaining 2 patients will likely continue with a nonrefluxing colon conduit because the upper tracts were already so dilated as to preclude risking subsequent anastomosis to the colon and possible ascending infection. Four new, unoperated cases of bladder exstrophy were treated. A colon conduit was made when the patient was approximately 1 year old and the genitalia were repaired about 6 months later. Of these 4 children 3 underwent anastomosis of the colon conduit to the were 4 to 5 years old and bowel trained. The is yet too young to consider this. Thus, of 16 beer. 3 will be hooked ;.ip within the
HENDREN
196
next year and 2 will likely remain with a colon conduit because they had advanced upper tract dilatation before this approach was started. Even the 2 who will remain with a colon conduit should be improved for there is much evidence that the long-term result of an ileal loop in young patients is complicated by a high rate of progressive deterioration. 14- 16 Richie and associates showed in a laboratory study a high incidence of pyelonephritis from reflux with an ileal loop but a low incidence in colon conduit with non-refluxing anastomoses. 17 • 18 TECHNIQUE OF OPERATION
Figures 1 to 5 show the technique used in performing a non-refluxing conduit from the sigmoid colon. In 1 case the sigmoid was unsatisfactory and a segment of transverse colon was used instead, based on middle colic blood supply. A tunneling implantation was performed with 27 of 32 ureters. Figure 6 shows management for a dilated ureter. It should be tapered before implantation into the colon. 19 Nine of the 32 ureters were tapered. Ureteral dilatation was formerly a major contraindication to ureterosigmoidostomy. However, that has not been the case when the ureters are tapered and implanted into a defunctioned conduit with later implantation into the colon. In some cases a ureter was too short to permit 2 satisfactory
ureterocolic anastomoses. This finding was managed by implanting the better ureter by a non-refluxing technique into the colon together with transureteroureterostomy for the other ureter (fig. 7). This technique was used for 2 ureters. Figure 8 shows the Mathisen nipple ureterosigmoidostomy. 20 It was used for 3 ureters in this series when the ureter was too short for a satisfactory tunneling implantation. This technique is preferred by many European surgeons. Figure 9 shows the second stage, that is implantation of the colon conduit at a later date. This was accomplished in 11 of the 16 cases to date. CASE REPORTS
The 6 cases herein reported will illustrate certain aspects of the problem of staged ureterocolic diversion of urine in patients with bladder exstrophy. Case 1 (fig. 10). A 10-year-old boy was first seen in 1965 with unoperated exstrophy of the bladder. An ileal conduit was performed. Except for encrustation of the stoma with bleeding, the patient did well and was free of urinary infection. In July 1973, when he was 18 years old, the ileal loop was removed and a non-refluxing colon conduit was constructed. The ureters were only slightly dilated and were of ample length for long, tunneling anastomoses without tapering. Postoperative excretory urogram (NP) remained normal. Resting pressure in the loop measured 4 cm. water. A loopogram showed no low pressure reflux but slight reflux on the left side with pressures higher than 35 cm. water. In April 1974 the conduit was anastomosed to the rectosigmoid. Convalescence was uneventful with satisfactory control of urine by rectum and no clinical evidence of urinary infection. An IVP 1 year postoperatively was normal. Blood chemical values included sodium 142 mEq. per 1., potassium 4.1, chloride 104, carbon dioxide 25, blood urea nitrogen (BUN) 29 and creatinine 0.7. Comment: The upper tract had remained normal with an ileal loop and did not change when converted to a colon conduit. There are many patients with bladder exstrophy like this, that is with normal upper tracts and an ileal loop, who could be managed in the same way. It has improved the patient's life style greatly to be rid of the appliance, which has not been accompanied by renal deterioration. Case 2 (fig. 11). A female newborn was seen in 1963 with bladder exstrophy. An ileal conduit was performed in October 1964. In June 1965 cystectomy with epispadias repair was performed. An IVP showed some hydronephrosis on the right side and revision of the stoma was performed to correct stenosis. Dilatation persisted. In June 1967 the stoma was revised a second time. Urinary infection occurred and the child was maintained on long-term antimicrobial therapy. In June 1971 a colon conduit was performed. Because the right ureter was slightly short the butt of the loop was sewn into the right gutter to achieve a satisfactory right tunneling anastomosis. The left ureter was of ample length. Neither was large enough to require tapering. Postoperative IVP showed dilatation from edema 1 week later but repeat IVP was normal 1 month later. Urine was consistently sterile. A loopogram showed no reflux at pressures up to 60 cm. water. Resting pressure in the loop Colon segment was 10 cm. with the patient in the supine position and 15 cm. in the sitting position. In August 1972 the conduit was anastofor conduit mosed to the colon. The patient has been well subsequently, FIG. 1. Segment of sigmoid is selected with appropriate blood supply. Bowel must be thoroughly prepared. Usually splenic flexure is on no medication and leading a normal life. Rectal control of mobilized to permit end-to-end restoration of continuity without urine is satisfactory. IVP remains normal. Serum chemistry tension of descending colon to rectosigmoid. We prefer 2 layers of studies included sodium 144 mEq. per 1., potassium 4.1, interrupted arterial silk with atraumatic needles for anastomosis. chloride 106, carbon dioxide 22 and BUN 16. Proximal end of conduit is closed with 2 inverting layers of chromic Comment: The upper tract improved greatly by getting rid catgut. Non-absorbable suture material should not be used lest a of the refluxing ilea! loop in exchange for a non-refluxing colon suture work its way into lumen of conduit and cause stone formation. Length of sigmoid should be amply long for 2 reasons: 1) it sometimes conduit. The patient had had many episodes of bacilluria contracts and shortens while one is working and 2) if conduit is to be originally, which cleared after the colon conduit diversion. The attached back to colon later this allows the surgeon to resect and discard stoma and that length which was in abdominal wall and upper tract remains normal almost 3 years after joining the perform rejoining anastomosis with segment of fresh bowel wall. conduit to the colon.
197
EXSTROPHY OF BLADDER
B
Distal end of
\\,
FIG. 2. A, conduit is rotated clockwise 180 degrees, anchoring proximal end at aortic bifurcation with several sutures. If ureters are too short, as in some patients with prior ilea! loop, it may be anchored at higher level or in left or right gutter instead of midline. In 1 case sigmoid conduit was not possible, and so we used segment of transverse colon based on blood supply of mid colic artery. B, conduit anchored and traps closed. Mesentery of conduit lies superiorly. Note proximity of ureters to base of conduit. When ureters are mobilized, all of periureteral adventitia should be spared, for it is important with respect to good blood supply of ureter.
A
B
C
II
End of ureter to colon mucosa
with interrupted 5-0 or 6-0 chromics
Line of
incision
Mucosa
6-0 tocking suture
FIG. 3. A, infiltration with saline facilitates making tunnel. B, undermining seromuscular layer by blunt dissection sparing many small bridging vessels. Separation of mucosa can be facilitated using small peanut gauze sponge. C, ureter placed beneath bridging vessels which arise from mesentery. Ureter is possibly better supported in this position, although we have placed ureter on top of bridging vessels in some cases, which requires lesser degree of mobilization of colon wall. I do not know which position is better but theoretically I prefer beneath the vessels.
Case 3 (fig. 12). A 2-year-old boy was referred in 1961 with severe bilateral hydronephrosis and recurrent urinary infection following primary closure of bladder exstrophy done elsewhere during infancy. There had been 8 previous hospitalizations during the first 2 years of life. Ileal loop urinary diversion was performed. The ureters, which had been dilated before performing the ileal loop diversion, remained dilated. The stoma was tight and required revision in 1962 when the patient was 3 i12 years old. There was chronic bacilluria. In 1966 both ureters were tapered, 1 side at a time, to improve their emptying. Although the patient did well clinically, there was intermittent chronic bacilluria requiring long-term antimicrobial treatment. In May 1973, when the patient was 15 years old, the ileal loop was removed and a non-refluxing colon conduit was performed. The ureters were too short for tunneling anastomoses. The left ureter was anastomosed to the colon by Mathisen's method and right-to-left transureteroureterostomy was performed. The patient has been completely well clinically since we performed the colon conduit. Urine cultures have remained consistently sterile. The colon conduit stoma has shown no tendency to stenosis. The patient expressed a marked preference for the colon conduit stoma compared to the
ileal stoma, which required dilatation, revision and sometimes bled during athletics. A loopograrh showed no reflux even at a high pressure of 60 cm. water. Although the patient has been well clinically the upper tracts remain too dilated to consider implanting the colon conduit into the colon. It is likely that this youngster will remain with a permanent colon conduit for life. Comment: It is said frequently that there is little to lose by primary closure of the exstrophied bladder and that one can proceed later to diversion if that does not succeed. In this case the urinary tract was originally normal. Primary closure resulted in immediate hydronephrosis and severe urinary infection ensued with permanent damage to both upper tracts. This damage did not improve with prolonged ileal loop urinary diversion but likely progressed. If a non-refluxing colon conduit had been used when the patient was seen originally, this course might have been altered. At present further change in the drainage status seems unlikely. This case emphasizes that an attempt at functional bladder closure can damage the upper tracts to a degree that only permanent diversion will be feasible. Case 4 (fig. 13). A 19-year-old male patient was referred in
198
HENDREN
B
C
FIG. 4. Details of anastomosis of ureter to colon. Note traction suture partially transecting ureter, leaving a bit attached for traction to minimize handling ureter with forceps. This bit is resected before last few sutures are placed. Note that ureter is not spatulated unless it is particularly small, for carefully performed anastomosis with interrupted suture material does not generally stenose. Anastomosis is performed with 5 and 6-zero chromic catgut. Opening in colon mucosa should not be too large. This is inverting anastomosis with all knots on inside except for last 3 sutures, which are placed before tying them and which have knots on outside. Drainage stent catheter is not used unless ureter is tapered.
December 1973 with an ilea! conduit and gradually deteriorating upper tracts. When he was 2 years old the bladder was anastomosed to the colon with resultant vesicocolic cutaneous fistula. Partial cystectomy and closure of the fistula were performed. In 1957 bilateral ureterosigmoidostomy was performed followed by repair of epispadias in 1958. In 1962 ilea! loop urinary diversion was performed. There were multiple revisions of the stoma and revision of both ureteroileal anastomoses in 1970. The patient was maintained on long-term antimicrobial therapy for chronic urinary infection. In December 1973 the ilea! loop was removed and a sigmoid colon conduit was constructed. Both ureters were dilated, tapered and tunneled. Postoperatively, the patient did well with consistently sterile urine and marked improvement in the upper tracts. A loopogram showed no low pressure reflux but moderate reflux on the left side with pressures higher than 40 cm. water. In May 1975 the conduit was anastomosed to the rectosigmoid. This patient is the most recently completed case. Comment: The appearance of the upper tracts and the
amount of ureteral dilatation present when the patient was first seen in 1973 made it seem unlikely that he would be a candidate for anything but continuing permanent diversion. The switch from a refluxing ilea! loop to a non-refluxing colon conduit resulted in marked improvement in the appearance of the upper tracts and consistently sterile urine. Should there be any recurrence of upper tract dilatation or clinical evidence of infection following implantation of the conduit into the colon, it would be brought back to the surface. This point has been stressed to each of these patients before implanting the colon conduit into the colon. Case 5 (fig. 14). A 6-month-old female infant was referred with exstrophy and extensive hemangioma involving the lower abdomen, bladder and perineum. An IVP was normal. On observation there was spontaneous regression of the hemangioma. In July 1971 a colon conduit was performed with an uneventful course. A loopogram months later showed no low pressure reflux but moderate bilateral reflux at pressures more than 40 cm. water. Epispadias repair and cystectomy were performed in February 1972. Repeat loopogram in October 1973 showed reflux at pressures higher than 50 cm. water. Bowel control was reasonably good. The conduit was anastomosed to the colon in November 1973. The control of liquid by rectum during the next 6 months was poor at first but it improved gradually and, when the patient was 5 years old, control was satisfactory with wetting only when she was tired. An IVP was normal. Serum electrolytes included sodium 142 mEq. per I., potassium 3.8, chloride 103, carbon dioxide 23 and BUN 23. This child is an essentially normal 5 year old who attends school and plays with her peers. She does not suffer recurrent urinary infection and is dry except for occasional accidents when she is overly tired. Comment: Each of the 4 children treated in this way has had a remarkably normal early childhood. They have not spent much time in the hospital and have not been forced to tolerate the onus of being wet all the time, a cause for ridicule and shame. Considering the complexity of the exstrophy anomaly, the children have required only infrequent visits to a physician. Case 6 (fig. 15). A male newborn was first seen in 1963 with bladder exstrophy. Ilea! loop urinary diversion was performed when he was 2 years old. In April 1966 cystectomy and epispadias repair were done, and a stoma revision was performed in August 1967. There was progressive dilatation of both upper tracts yet an open stoma and no loop residual, thought secondary to a chronically spastic loop. In December 1968 the first loop was removed and a new ilea! conduit was fashioned. The upper tract remained dilated. In June 197 4 the ilea! loop was removed and a non-refluxing colon conduit with bilateral ureteral tapering was constructed. Postoperatively the right upper tract returned to normal and the left side showed more dilatation than before. Despite this, the patient was completely well clinically with consistently sterile cultures and on no medication. A left percutaneous antegrade pyelogram disclosed marked obstruction at the tip of the left ureter (fig. 15, B). Cystoscopy of the loop was performed. The right ureterocolic anastomosis could be seen easily and it effluxed intravenous indigo carmine promptly. The left orifice could not be seen. A small catheter was inserted into the left kidney percutaneously followed by a drip containing indigo carmine into the kidney, which helped identify the stenotic left orifice. A Fogarty embolectomy arterial catheter was passed, inflating the balloon with 1 cc air. With malleable retractors to visualize the interior of the conduit, traction on the balloon catheter pulled the stenotic orifice into view. Meatotomy was performed. Comment: It is inevitable that in performing ureterosigmoid anastomoses there will be occasional technical failures, particularly if the ureter is dilated and requires tapering. This is the first case that we encountered in performing 26 colon conduits for bladder exstrophy, meningomyelocele and sarcoma of the
199
EXSTROPHY OF BLADDER
A
B I
I I
I
'
Closed
para median seromuscular layer and mucoso
incision
FIG. 5. A, completed conduit. Ureters lie parallel and at same level of conduit. There is good blood supply of strip of bowel between ureters because medial flap of each tunnel was not mobilized. Seromuscular closure over ureter is performed with colored non-absorbable suture material to permit easy identification of location of ureters in future in cases in which conduit will be taken down and anastomosed into colon. Stoma can be placed in right lower quadrant at site of prior ilea! loop stoma in midline or in left lower quadrant according to particulars of given case. B, completed conduit. MATHISEN
FOR DILATED URETER
URETEROSIGMOIDOSTOMY
longitudinal
resection
FIG. 6. Management of dilated ureter by appropriate tapering. It is important to preserve vascularity of ureter by not making it too narrow. Number 5 or 8 plastic feeding catheter is used for temporary drainage for 10 to 12 days until longitudinal suture line is healed. Catheter is sutured to stoma bud. IF ONE
Left to right
URETER IS TOO SHORT Right to left
FIG. 8. Technique of ureterosigmoidostomy described by Mathisen, which we have used when ureter did not seem well suited for usual long ureterosigmoid tunnel (too short or too thick walled). It is best to fashion periureteral flap of colon a little at a time as anastomosis progresses, in order not to make it too narrow. Flap must be wide enough to wrap around ureter without compressing it. FIG. 7. Transureteroureterostomy can be used if only 1 ureter is suitable for anastomosis to colon.
prostate. Although this ureter was obstructed, there was no problem with urinary infection and ascending pyelonephritis. If this ureterosigmoid anastomosis had been into a functioning segment of colon filled with feces there would have undoubtedly been immediate pyelonephritis and probably severe
damage to the kidney. Because this stenosis was limited to the tip of the ureter it could be managed in an unusual way, that is working down inside the loop via the stoma, avoiding laparotomy. The tunnel length still looks adequate even after meatotomy. If subsequent x-rays show that obstruction is relieved and there is no reflux, the conduit will be implanted into the colon. If there is continuing obstruction, an open
200
HENDREN
operation will be performed. There would be the option of primary ureterosigmoidostomy, those patients who had asredoing that anastomosis or performing left-to-right trans- cending infection usually began to show it relatively soon. There is no way that one can be absolutely certain that no ureteroureterostomy. bacteria reached the upper tracts in these cases, except by needle puncture aspiration of the kidney. This does not seem POSTOPERATIVE RESULTS AND COMPLICATIONS justified in a patient who is well and whose IVP is stable. No Non-refluxing colon conduit was performed in 26 patients bowel gas has been noted in the upper tracts of any patient. from March 1973 through May 1975. This total included 16 patients with bladder exstrophy, 9 with neurogenic bladder and 1 with prostatic sarcoma. There was 1 obstructed ureter (case 6, fig. 15). Postoperative intestinal obstruction requiring laparotomy with lysis of adhesions occurred in 4 patients, 3 of whom had exstrophy. No low pressure reflux was found on the loopogram study in any case. Reflux could be produced in some patients by filling colon conduits with pressures higher than 35 cm. water. There was no evidence of postoperative l-'Yelonephritis or upper tract deterioration, except in the 1 case with ureteral obstruction. Recurrent bacilluria present in some patients with ileal loop preoperatively ceased when they were converted to a non-refluxing colon conduit. In 11 of the 16 exstrophy patients undergoing the second stage, that is implantation of the colon conduit into the colon, the longest is almost 3 years postoperatively and there is no evidence of pyelonephritis clinically or upper tract change by roentgenographic evaluation. Obviously, long-term observation of these cases will be needed. However, in our previous experience with
, I' I
' \
,,- ..... , \
,,\~:
\
', .... _.,,
Closed stoma site
',
'I I
I
,I
\
,,,,
,, :,"
,, I/ I
\
End to side conduit to colon anastomosis
--- I / ,._,;·,
FIG. 9. Subsequent end-to-side anastomosis of conduit to colon after enough time has passed to be certain that upper tracts remain normal, there is no low pressure reflux and child can retain liquid rectal content,
FIG. 11. Case 2-female patient with prior ilea! loop and mild upper tract dilatation; converted to colon conduit and later implanted into colon. A, IVP December 1970 at age 7 years, 6 years after ilea! loop was performed. Note right ureteral dilatation. B, IVP June 1971, 10 days after colon conduit operation. Note edema around tunneled right ureter (arrows) and bilateral hydronephrosis, often present for several weeks postoperatively. In functional colon this could result in pyelonephritis. It has not caused a problem with infection in defunctioned conduit. IVP 1 month later was normal. C, loopogram December 1971, 6 months after colon conduit. No reflux even at high pressure of 60 cm. water. D, IVP September 1974, more than 2 years after anastomosis of conduit to colon. Note delicate upper tracts, now normal size right ureter and long right ureteral sigmoid tunnel (small arrows). Note anastomosis of conduit to rectosigmoid (large arrow in right lower quadrant).
FIG. 10. Case 1-10-year-old boy with prior ilea! loop and normal upper tracts; converted to colon conduit and later implanted into colon. A, IVP July 1973 at age 18 years shows normal upper tract with ilea! loop present for 8 years. B, IVP February 1974, 8 months.after conversion to non-refluxing colon conduit. Normal IVP. Conduit joined to colon April 1974. C, IVP October 1974, 6 months after joining colon conduit to colon. Patient remains well,
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The colon conduit stoma is larger and more bulky than an ilea! loop stoma but it has been singularly free of complications. None has developed stenosis requiring dilatation or revision. There has been no encrustation or stomatitis. All patients with a prior ilea! loop stoma have expressed a preference for the colon conduit stoma. There has been no significant postoperative hyperchloremic acidosis in this small group of patients. A generous fluid intake is encouraged with scheduled voiding every 3 hours to reduce solute reabsorption. Patients with ureterosigmoidostomy absorb chloride and secondarily lose bicarbonate, which can lead
FIG. 14. Case 5-new case of bladdeT exstrophy in 6-month-old female infant: colon conduit at 17 months and implantation of conduit into colon at age 3 years months. IVP January 1974, 3 months after anastomosis of conduit to colon. IVP March 1975, 17 months after anastomosis of conduit to colon. Note conduit filled with dye (arrow).
FIG. 12. Case 3-2-year-old boy with primary bladder closure leading to upper tract deterioration and then ilea! loop diversion; later, colon conduit using Mathisen nipple and transureteroureterostomy; probably unsuited for implantation of conduit into colon. A, retrograde study preoperatively as infant shows normal upper tracts. B, cystogram 2 years after primary bladder closure shows massive bilateral hydronephrosis and hydroureter. Recurrent severe pyelonephritis. C, loopogram December 1966, 4 years after ilea! loop diversion. Right ureter has been tapered and left ureter still dilated. Subsequently tapered. D, IVP February 1973, 11 years after ilea! loop was performed. Patient well clinically but chronic bacilluria requiring therapy. E, retrograde examination inserting cystoscope into colon conduit and catheterizing left ureter implanted by Mathisen nipple technique. Note right-to-left transureteroureterostomy. Drainage films showed prompt emptying. F, IVP November 1974, 18 months after colon conduit operation. Patient is well with stable renal function and no longer has bacilluria, but this degree of upper tract dilatation precludes anastomosis of conduit to colon. This case emphasizes that attempted primary closure of bladder can result in severe upper tract damage which may preclude any alternative approach except permanent diversion.
FIG. 15. Case 6-male patient with ilea! loop with deterioration; converted to colon conduit with bilateral ureteral tapering and stenosis of 1 ureteral anastomosis. A, IVP 9 months after colon conduit operation. Right side normal and hydronephrosis on left side. B, percutaneous left antegrade study April 1975, defining point of obstruction at tip of ureter (arrow). Note dilated segment of ureter in tunnel. Ureteral meatotomy performed through stoma with technical help of Fogarty embolectomy catheter. If this complication had occurred with implantation of ureter into fecal stream, severe pyelonephritis probably would have resulted.
F1G. 13. with deterioration and mega.ureter; converted to non-refluxing colon conduit with bilateral ureterai tapering and subsequent implantation conduit into colon. IVP February 1973 at age 19 years, 11 years after ilea! tract dilatation despite multiple revisions of loop. B, •v••,-,vto·''-"' shows massive reflux, and dilated and tortuous 16 months after colon conduit with bilateral extensive tapering. Normal upper tract, sterile urine 2.nd implanted into ,:olon.
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HENDREN
to secondary acidosis with volume retraction. If the serum carbon dioxide decreases to 20 mEq. per I. (normal 22 to 28 mEq. per I.) we prescribe bicarbonate in a dosage of 2 to 4 mEq. per kg. body weight. A 600 mg. tablet of sodium bicarbonate contains 7.8 mEq. bicarbonate. Patients with ureterosigmoidostomy can handle the solute reabsorption and its re-excretion if they have reasonable renal function and enough fluid intake. It is important to emphasize to them the need to come for temporary intravenous fluid maintenance during an intercurrent illness when fluid intake is poor, such as gastroenteritis with vomiting or diarrhea. Otherwise, these patients can become uremic quickly despite good renal function. The use of sigmoid colon for a urinary conduit is not new, having been reported more than 20 years ago by Gross. 21 Mogg prefers using the colon conduit in children needing diversion for myelomeningocele. 22 Almost 50 years ago Coffey emphasized that an intramural tunnel improves the success of ureterosigmoidostomy. 23 Leadbetter and Clarke showed that meticulous anastomosis of the end of the ureter to the colonic mucosa, together with an intramural tunnel gives improved results. 12 • 13 The Mathisen nipple also has proved effective. 20 We believe that this staged method of ureterosigmoid diversion in patients with bladder exstrophy is preferred to a primary ureterosigmoid anastomosis for several reasons: 1) If primary ureterosigmoidostomy is performed on a small infant there is usually combined fecal and urinary incontinence, which is malodorous for several years. These children do not usually gain excellent socially acceptable control of liquid by rectum until they are about 5 years old or sometimes even older. Thus, we are opposed to performing diversion of the urine into the colon until a child has a reasonable chance of being continent of liquid in the rectum. This can be important for social reasons. 2) In former years many of these children were allowed to reach ages 4 to 5 years without any surgical correction, which is equally bad from the psychological standpoint. They are well aware of their genital anomaly at about age 2 years. 3) Performing a non-refluxing conduit when the child is about 1 year old provides a method to get the patient dry, albeit with an appliance, and then allows early repair of the genitalia, an important concern to the parents and child. The patient then spends the next several years dry with satisfactory appearance, free of urinary infection and not in and out of the hospital for recurrent infection, repeated operations to achieve continence and so forth. 4) This method permits anastomosing the ureters to a clean conduit and prevents patients from being bathed in fecal material while healing. The early postoperative IVP after a technically successful ureteral reimplantation into the bladder or ureteral anastomosis to the colon may show edema with temporary upper tract dilatation (fig. 11, B). In a colon filled with fecal material this can result in immediate pyelonephritis. Conversely, the 1 patient in this series with an obstructed ureter (fig. 15) had no clinical evidence of infection almost certainly because the ureterosigmoidostomy was in a clean isolated conduit. Also, we have seen reflux demonstrated in patients by a cystogram done early after successful ureteral reimplantation while edema persists, yet disappear as healing progresses. It is likely that this is true with a new ureterosigmoidostomy, that is some weeks must pass before the tunneled implant is competent to prevent reflux of colon content into the lower ureter. 5) This method permits prolonged observation of the ureterosigmoid anastomoses by IVP and loopogram to make certain that there is no obstruction or low pressure reflux before taking the step of implanting the conduit into the colon. We anticipate that this approach, although requiring an extra operation, will minimize the complication of ureterosigmoidostomy. In these 11 exstrophy cases and a child with anterior pelvic exenteration for sarcoma there has been no late complication to date. The primary non-refluxing colon conduit is a somewhat longer and more technically exacting operation than a classical
ilea! loop procedure. 24 Whereas the ilea! loop generally takes 2 ½ to 3 hours to perform, a non-refluxing colon conduit generally requires at least 1 to 2 hours longer, especially if ureteral tapering is required. Converting a patient from an ilea! conduit to a non-refluxing conduit takes even longer. In 1 patient ( case 4) with many previous intra-abdominal operations and bilateraily dilated, tortuous ureters, the procedure lasted for 11 hours. Present day excellent anesthesia, intraoperative monitoring, effective antibiotics and so forth make this possible, giving the surgeon time to do what is required, which was clearly not always possible years ago. REFERENCES
1. Spence, H. M.: Ureterosigmoidostomy for exstrophy of the bladder. Results in a personal series of thirty-one cases. Brit. J. Urol., 38: 36, 1966. 2. Bennett, A. H.: Exstrophy of bladder treated by ureterosigmoidostomies: long-term evaluation. Urology, 2: 165, 1973. 3. Megalli, M. and Lattimer, J. K.: Review of the management of 140 cases of exstrophy of the bladder. J. Urol., 109: 246, 1973. 4. Williams, D. I. and Keeton, J. E.: Further progress with reconstruction of the exstrophied bladder. Brit. J. Surg., 60: 203, 1973. 5. Marshall, V. F. and Muecke, E. C.: Functional closure of typical exstrophy of the bladder. J. Urol., 104: 205, 1970. 6. Chisholm, T. C.: Exstrophy of the urinary bladder. In: Pediatric Surgery, 2nd ed. Edited by W. T. Mustard, M. M. Ravitch, W. H. Snyder, K. J. Welch and C. D. Benson. Chicago: Year Book Medical Publishers, vol. 2, chapt. 73, 1970. 7. Fisher, J. H. and Retik, A. B.: Exstrophy of the bladder. J. Pediat. Surg., 4: 620, 1969. 8. Houtappel, H. C.: Experience with uretero-ileosigmoidostomy. Brit. J. Urol., 35: 277, 1963. 9. Lowsley, 0. S. and Johnson, T. H.: A new operation for creation of an artificial bladder with voluntary control of urine and feces. J. Urol., 73: 83, 1955. 10. Boyce, W. H.: A new concept concerning treatment of exstrophy of the bladder: 20 years later. J. Urol., 107: 476, 1972. 11. Hays, D. M., Powell, T. 0. and Strauss, J.: Vesicoileosigmoidostomy in the treatment of exstrophy: re-evaluation. Surgery, 66: 1103, 1969. 12. Leadbetter, W. F.: Consideration of problems incident to performance of uretero-enterostomy: report of a technique. J. Urol., 65: 818, 1951. 13. Leadbetter, W. F. and Clarke, B. G.: Five years' experience with uretero-enterostomy by the 'combined' technique. J. Urol., 73: 67, 1955. 14. Retik, A. B., Perlmutter, A. D. and Gross, R. E.: Cutaneous ureteroileostomy in children. New Engl. -J. Med., 277: 217, 1967. 15. Ray, P. and De Domenico, I.: Intestinal conduit urinary diversion in children. Brit. J. Urol., 44: 345, 1972. 16. Smith, E. D.: Follow-up studies on 150 ilea! conduits in children. J. Pediat. Surg., 7: 1, 1972. 17. Richie, J.P., Skinner, D. G. and Waisman, J.: The effect ofreflux on the development of pyelonephritis in urinary diversion: an experimental study. J. Surg. Res., 16: 256, 1974. 18. Richie, J.P. and Skinner, D. G.: Urinary diversion: the physiological rationale for non-refluxing colonic conduits. Brit. J. Urol., 47: 269, 1975. 19. Hendren, W. H.: Operative repair of megaureter in children. J. Urol., 101: 491, 1969. 20. Mathisen, W.: A new method for ureterointestinal anastomosis; preliminary report. Surg., Gynec. & Obst., 96: 255, 1953. 21. Gross, R. E.: Urinary and fecal incontinence of neurogenic origin. In: The Surgery of Infancy and Childhood: Its Principles and Techniques. Philadelphia: W. B. Saunders Co., chapt. 56, p. 740, 1953. 22. Mogg, R. A.: Some observations on urinary diversion. Ann. Roy. Coll. Surg. Engl., 46: 251, 1970. 23. Coffey, R. C.: Transplantation of the ureters into the large intestine. Surg., Gynec. & Obst., 47: 593, 1928. 24. Hendren, W. H_: Nonrefluxing colon conduit for temporary or permanent urinary diversion in children. J. Pediat. Surg., 10: 381, 1975.