Vol. 114, July
THE JOURNAL OF UROLOGY
Printed in U.S.A.
Copyright © 1975 by The Williams & Wilkins Co.
LONG-TERM RESULTS OF URETEROSIGMOIDOSTOMY IN CHILDREN WITH BLAbDER EXSTROPHY JOSEPH W. SEGURA*
AND
PANAYOTIS P. KELALIS
From the Mayo Clinic and Mayo Foundation, Rochester, Minnesota
ABSTRACT
The records of 87 children with bladder exstrophy seen between 1912 and 1935 are reviewed. We believe that ureterosigmoidostomy with cystectomy can provide a satisfactory solution to the problem of bladder exstrophy. Furthermore, ureterosigmoidostomy appears suitable from the standpoint of preservation of upper tracts and provides a socially acceptable solution to the question of disposal of the diverted urine. The best treatment for exstrophy of the bladder remains a subject of debate. Although primary closure of the bladder would seem to be the ideal solution, efforts in this direction have been frustrated by reflux, ascending pyelonephritis, dehiscence of the anterior closure and complete urinary incontinence in the majority of patients. 1 Others have thought that cystectomy followed by urinary diversion yields a more satisfactory result in terms of preserved upper tracts and creation of a socially acceptable individual. 2 • 4 The ideal form of urinary diversion has yet to .be devised but Spence presented a good case for the use of ureterosigmoidostomy as the preferred method of diversion in children with bladder exstrophy. 5 We thought that a review of the long-term results in patients treated in this manner many years ago might assist us in assessing the usefulness of ureterosigmoidostomy.
urethral twist, for reasons that are not clear in the record. The right ureteral colonic anastomosis was performed first. The left anastomosis was performed 7 to 10 days later and 10 days after that cystectomy was done. The Coffey I technique was used in all of these cases.• The mean age of patients at the time of operation was 5.6 years. Hospital mortality for those undergoing ureterosigmoidostomy with or without cystectomy was 10 per cent and mostly caused by uncontrollable infection. Of these patients 3 were lost to followup, leaving 51 children who underwent bilateral ureterosigmoidostomy with or without cystectomy as the basis for this report. Followup information on this group was obtained by correspondence with patients and their physicians as well as by evaluation at the Mayo Clinic.
METHOD
RESULTS
Of the 51 patients 25 (50 per cent) are known to The records of 87 children (64 boys and 23 girls), 15 years old or less, who were evaluated between be dead at the time of this writing (table 2). Renal 1912 and 1935 because of bladder exstrophy were disease included recurrent infection, uremia and reviewed. Of these patients 68 had undergone some ureteral obstruction. The 2 patients who died of surgical procedure, and all except 1 had l:ieen 2 accidents had normal urinary tracts at the time of years old or more at the time of operation (table 1). autopsy. There were 26 patients who were known to be Cystectomy and bilateral ureterosigmoidostomy were performed as a 3-stage procedure in two- alive at their last followup (between 1940 and 194t, thirds of the group. Ureterosigmoidostomy without for 2, 1948 and 1950 for 3, 1957 and 1963 for 8, and cystectomy was done in 13 patients for various 1971 and 1974 for 13). Further tracing was impossireasons, although most of these patients eventually ble for the 13 patients who were last heard from in had their bladders removed. The last 2 stages were the years 1940 to 1963. Of these patients 2 were not completed in 4 children because of death in 2, known to be uremic and 1 had been converted to subsequent left nephrectomy in 1 and unknown bilateral cutaneous ureterostomies 22 years postreasons in 1. Two patients had transplantation of operatively. Urograms were available for 4 of the the trigone into the rectum and 1 had only a remaining patients, of whom 3 had normal upper tracts at 39, 17 and 17 years postoperatively and 1 Accepted for publication January 10, 1975. had a normal right kidney but decreased function Read at annual meeting of North Central Section, on the left side. The other patients were asymptoAmerican Urological Association, Columbus, Ohio, Sepmatic at the last followup. tember 18-21, 1974. The remaining 13 patients were 42 to 70 years old * Requests for reprints: Mayo Clinic, Rochester, Minnesota 55901. at followup and had been followed from 38 to 57 138
139
URETEROSICMOIDOSTOMY IN CHILDREN WITH BLADDER EXSTROPHY TABLE
1. Operations performed in 87 children with
exstrophy No.Pts.
Operation Bilat. ureterosigmoidostomy and cystectomy Bilat. ureterosigmoidostomy Rt. ureterosigmoidostomy only Trigonal transplantation Primary closure Urethral twist None
TABLE
"
47 13 4 2
1 1 19
2. Cause of death in 25 patients known dead Cause of Death
No.
Yrs. Postop.
Renal Probable renal Accident Ruptured appendix Polio Cerebrovascular accident Cancer of colon Unknown
2 1 1 1 1 2
14 and 25 8 29 50 49 12 and 56
la
Mean, 15
years postoperatively. Of these patients 8 have survived 45 years or longer and, with 3 of the deceased patients previously mentioned, 11 have ' survived 45 years or longer (table 3). However, their urinary tracts have suffered. In half, the left kidneys either have been removed or are not functioning. The right sides have fared better, " although 1 patient required a nephrostomy. Of the 3 deaths, 2 were from non-renal causes. The patient who died of carcinoma of the colon had a normal excretory urogram (IVP) shortly before his death. Electrolyte values were normal in 2 of this group and 1 had slight hyperchloremic acidosis. A huge urinary calculus had formed in the rectum of 1 patient. . The 3 remaining survivors required urinary di" version (conversion to conduits in 2 and bilateral nephrostomy in 1).
and ureter could no longer be positioned in an ideal relationship. Also, urine is known to pool in the loop between the 2 anastomoses, and reflux of this infected urine and feces may have been a factor in the much greater renal morbidity found on the left side compared to the right side. Perhaps the most important cause of failure was the use of the Coffey technique of ureterointestinal anastomosis. This technique has long been known to produce the problems that characterize the history of this group-scarring with obstruction and reflux of contaminated matter. Much better results have been achieved by the combined technique of Leadbetter: two-thirds of Spence's pediatric patients in whom this anastomosis was used had normal upper tracts. 5 We are not attempting to ignore the problems attendant on ureterosigmoidostomy. It is obvious that only a few of these patients have lived their lives without significant sequelae directly related to the fact that ureterosigmoidostomy was done. However, we do think that many of these problems would have been avoidable had the surgeons used the combined technique, in a single-stage procedure, with the other adjunctive measures and knowledge now available. The other major consideration in this group is social adjustment of these patients. It is agreed that anything is better than the untreated exstrophy. The constant exposure of urine produces a social pariah and, as time passes, the threat of adenocarcinoma of the bladder becomes more and more real. Few of the 17 patients who died early of renal causes could be said to have done well. Relieved of the burden of their bladders they spent their average of 15 years of life suffering the symptoms of pyelonephritis and then died of uremia. If it is true that the majority of untreated TABLE
3. Summary of 11 patients surviving 45 years or
longer
COMMENT
It seems apparent that ureterosigmoidostomy in these circumstances has the capacity to carry the patient over the long haul and has done so in 25 per 'i cent of those who survived the operation. Why did it not do so in more children and, particularly, why did a third of the group die of renal causes at an ,1. average of 15 years postoperatively? The following considerations may answer these questions. No antibiotics or effective urinary antiseptics were available at the time of these operations. The hyperchloremic acidosis and other electrolyte changes that occur as sequelae to this operation were unknown. Initial followup was inadequate by . present standards, although one wonders what ·' more could have been done, in that era, for these children. Anastomosis was always done first on the right side. One suspects that its location and -, orientation were ideal and that the left anastomosis then was placed wherever it seemed possible, in a previously operated upon field where the sigmoid
Survival (yrs. postop.)
Upper Tracts Other Right
Left
57
Normal
Normal
56
Nephrectomy
?
56
Nephrectomy
Mild hydronephrosis
55 53
Nephrectomy Nephrectomy
Normal Normal
50
Mild hydronephrosis
Moderate hydronephrosis
49
Normal
Normal
48
Moderate hydro- Normal nephrosis NoIVP Non-function ·1 Nephrostomy Non-function Mild hydronephrosis
46 46 45
Normal electrolytes Died ofunknown cause Chloride increased and mild acidosis Normal electrolytes Died of cerebravascular accident Died of cancer of colon Rectal calculus Asymptomatic
140
SEGURA AND KELALIS
patients die by the age of 21 of ascending pyelonephritis, then little has been lost in this group. 7 However, examination of the lives of the surviving patients reveals what appears to be a satisfactory quality of life. The women have married and have had children; 1 woman had 6 children. Two patients are attorneys, 1 is a rancher and all have made their way in the world including a 58-yearold man who, when last heard from 55 years postoperatively, was traveling around the Pacific Ocean on a freighter. REFERENCES
1. Marshall, V. F. and Muecke, E. C.: Functional closure
of typical exstrophy of the bladder. J. Urol., 104: 205, 1970.
2. Megalli, M. and Lattimer, J. K.: Review of the management of 140 cases of exstrophy of the bladder. J. Urol., 109: 246, 1973. 3. Nisonson, I. and Lattimer, J. K.: How well can the exstrophied bladder work? J. Urol., 107: 664, 1972. 4. Arnarson, 0. and Straffon, R. A.: Clinical experience with the ilea! conduit in children. J. Urol., 102: 768, 1969. 5. Spence, H. M.: Ureterosigmoidostomy for exstrophy of the bladder. Results in a personal series of thirtyone cases. Brit. J. Urol., 38: 36, 1966. 6. Harvard, B. M. and Thompson, G. J.: Congenital exstrophy of the urinary bladder: late results of treatment by the Coffey-Mayo method of ureterointestinal anastomosis. J. Urol., 65: 223, 1951. 7. Campbell, M. F. and Harrison, J. H.: Urology, 3rd ed. Philadelphia: W. B. Saunders Co., 1970.
THE JOURNAL OF UROLOGY
Printed in U.S.A.
Copyright © 1975 by The Williams & Wilkins Co.
LONG-TERM RESULTS OF URETEROSIGMOIDOSTOMY IN CHILDREN WITH BLAbDER EXSTROPHY JOSEPH W. SEGURA*
AND
PANAYOTIS P. KELALIS
From the Mayo Clinic and Mayo Foundation, Rochester, Minnesota
ABSTRACT
The records of 87 children with bladder exstrophy seen between 1912 and 1935 are reviewed. We believe that ureterosigmoidostomy with cystectomy can provide a satisfactory solution to the problem of bladder exstrophy. Furthermore, ureterosigmoidostomy appears suitable from the standpoint of preservation of upper tracts and provides a socially acceptable solution to the question of disposal of the diverted urine. The best treatment for exstrophy of the bladder remains a subject of debate. Although primary closure of the bladder would seem to be the ideal solution, efforts in this direction have been frustrated by reflux, ascending pyelonephritis, dehiscence of the anterior closure and complete urinary incontinence in the majority of patients. 1 Others have thought that cystectomy followed by urinary diversion yields a more satisfactory result in terms of preserved upper tracts and creation of a socially acceptable individual. 2 • 4 The ideal form of urinary diversion has yet to .be devised but Spence presented a good case for the use of ureterosigmoidostomy as the preferred method of diversion in children with bladder exstrophy. 5 We thought that a review of the long-term results in patients treated in this manner many years ago might assist us in assessing the usefulness of ureterosigmoidostomy.
urethral twist, for reasons that are not clear in the record. The right ureteral colonic anastomosis was performed first. The left anastomosis was performed 7 to 10 days later and 10 days after that cystectomy was done. The Coffey I technique was used in all of these cases.• The mean age of patients at the time of operation was 5.6 years. Hospital mortality for those undergoing ureterosigmoidostomy with or without cystectomy was 10 per cent and mostly caused by uncontrollable infection. Of these patients 3 were lost to followup, leaving 51 children who underwent bilateral ureterosigmoidostomy with or without cystectomy as the basis for this report. Followup information on this group was obtained by correspondence with patients and their physicians as well as by evaluation at the Mayo Clinic.
METHOD
RESULTS
Of the 51 patients 25 (50 per cent) are known to The records of 87 children (64 boys and 23 girls), 15 years old or less, who were evaluated between be dead at the time of this writing (table 2). Renal 1912 and 1935 because of bladder exstrophy were disease included recurrent infection, uremia and reviewed. Of these patients 68 had undergone some ureteral obstruction. The 2 patients who died of surgical procedure, and all except 1 had l:ieen 2 accidents had normal urinary tracts at the time of years old or more at the time of operation (table 1). autopsy. There were 26 patients who were known to be Cystectomy and bilateral ureterosigmoidostomy were performed as a 3-stage procedure in two- alive at their last followup (between 1940 and 194t, thirds of the group. Ureterosigmoidostomy without for 2, 1948 and 1950 for 3, 1957 and 1963 for 8, and cystectomy was done in 13 patients for various 1971 and 1974 for 13). Further tracing was impossireasons, although most of these patients eventually ble for the 13 patients who were last heard from in had their bladders removed. The last 2 stages were the years 1940 to 1963. Of these patients 2 were not completed in 4 children because of death in 2, known to be uremic and 1 had been converted to subsequent left nephrectomy in 1 and unknown bilateral cutaneous ureterostomies 22 years postreasons in 1. Two patients had transplantation of operatively. Urograms were available for 4 of the the trigone into the rectum and 1 had only a remaining patients, of whom 3 had normal upper tracts at 39, 17 and 17 years postoperatively and 1 Accepted for publication January 10, 1975. had a normal right kidney but decreased function Read at annual meeting of North Central Section, on the left side. The other patients were asymptoAmerican Urological Association, Columbus, Ohio, Sepmatic at the last followup. tember 18-21, 1974. The remaining 13 patients were 42 to 70 years old * Requests for reprints: Mayo Clinic, Rochester, Minnesota 55901. at followup and had been followed from 38 to 57 138
139
URETEROSICMOIDOSTOMY IN CHILDREN WITH BLADDER EXSTROPHY TABLE
1. Operations performed in 87 children with
exstrophy No.Pts.
Operation Bilat. ureterosigmoidostomy and cystectomy Bilat. ureterosigmoidostomy Rt. ureterosigmoidostomy only Trigonal transplantation Primary closure Urethral twist None
TABLE
"
47 13 4 2
1 1 19
2. Cause of death in 25 patients known dead Cause of Death
No.
Yrs. Postop.
Renal Probable renal Accident Ruptured appendix Polio Cerebrovascular accident Cancer of colon Unknown
2 1 1 1 1 2
14 and 25 8 29 50 49 12 and 56
la
Mean, 15
years postoperatively. Of these patients 8 have survived 45 years or longer and, with 3 of the deceased patients previously mentioned, 11 have ' survived 45 years or longer (table 3). However, their urinary tracts have suffered. In half, the left kidneys either have been removed or are not functioning. The right sides have fared better, " although 1 patient required a nephrostomy. Of the 3 deaths, 2 were from non-renal causes. The patient who died of carcinoma of the colon had a normal excretory urogram (IVP) shortly before his death. Electrolyte values were normal in 2 of this group and 1 had slight hyperchloremic acidosis. A huge urinary calculus had formed in the rectum of 1 patient. . The 3 remaining survivors required urinary di" version (conversion to conduits in 2 and bilateral nephrostomy in 1).
and ureter could no longer be positioned in an ideal relationship. Also, urine is known to pool in the loop between the 2 anastomoses, and reflux of this infected urine and feces may have been a factor in the much greater renal morbidity found on the left side compared to the right side. Perhaps the most important cause of failure was the use of the Coffey technique of ureterointestinal anastomosis. This technique has long been known to produce the problems that characterize the history of this group-scarring with obstruction and reflux of contaminated matter. Much better results have been achieved by the combined technique of Leadbetter: two-thirds of Spence's pediatric patients in whom this anastomosis was used had normal upper tracts. 5 We are not attempting to ignore the problems attendant on ureterosigmoidostomy. It is obvious that only a few of these patients have lived their lives without significant sequelae directly related to the fact that ureterosigmoidostomy was done. However, we do think that many of these problems would have been avoidable had the surgeons used the combined technique, in a single-stage procedure, with the other adjunctive measures and knowledge now available. The other major consideration in this group is social adjustment of these patients. It is agreed that anything is better than the untreated exstrophy. The constant exposure of urine produces a social pariah and, as time passes, the threat of adenocarcinoma of the bladder becomes more and more real. Few of the 17 patients who died early of renal causes could be said to have done well. Relieved of the burden of their bladders they spent their average of 15 years of life suffering the symptoms of pyelonephritis and then died of uremia. If it is true that the majority of untreated TABLE
3. Summary of 11 patients surviving 45 years or
longer
COMMENT
It seems apparent that ureterosigmoidostomy in these circumstances has the capacity to carry the patient over the long haul and has done so in 25 per 'i cent of those who survived the operation. Why did it not do so in more children and, particularly, why did a third of the group die of renal causes at an ,1. average of 15 years postoperatively? The following considerations may answer these questions. No antibiotics or effective urinary antiseptics were available at the time of these operations. The hyperchloremic acidosis and other electrolyte changes that occur as sequelae to this operation were unknown. Initial followup was inadequate by . present standards, although one wonders what ·' more could have been done, in that era, for these children. Anastomosis was always done first on the right side. One suspects that its location and -, orientation were ideal and that the left anastomosis then was placed wherever it seemed possible, in a previously operated upon field where the sigmoid
Survival (yrs. postop.)
Upper Tracts Other Right
Left
57
Normal
Normal
56
Nephrectomy
?
56
Nephrectomy
Mild hydronephrosis
55 53
Nephrectomy Nephrectomy
Normal Normal
50
Mild hydronephrosis
Moderate hydronephrosis
49
Normal
Normal
48
Moderate hydro- Normal nephrosis NoIVP Non-function ·1 Nephrostomy Non-function Mild hydronephrosis
46 46 45
Normal electrolytes Died ofunknown cause Chloride increased and mild acidosis Normal electrolytes Died of cerebravascular accident Died of cancer of colon Rectal calculus Asymptomatic
140
SEGURA AND KELALIS
patients die by the age of 21 of ascending pyelonephritis, then little has been lost in this group. 7 However, examination of the lives of the surviving patients reveals what appears to be a satisfactory quality of life. The women have married and have had children; 1 woman had 6 children. Two patients are attorneys, 1 is a rancher and all have made their way in the world including a 58-yearold man who, when last heard from 55 years postoperatively, was traveling around the Pacific Ocean on a freighter. REFERENCES
1. Marshall, V. F. and Muecke, E. C.: Functional closure
of typical exstrophy of the bladder. J. Urol., 104: 205, 1970.
2. Megalli, M. and Lattimer, J. K.: Review of the management of 140 cases of exstrophy of the bladder. J. Urol., 109: 246, 1973. 3. Nisonson, I. and Lattimer, J. K.: How well can the exstrophied bladder work? J. Urol., 107: 664, 1972. 4. Arnarson, 0. and Straffon, R. A.: Clinical experience with the ilea! conduit in children. J. Urol., 102: 768, 1969. 5. Spence, H. M.: Ureterosigmoidostomy for exstrophy of the bladder. Results in a personal series of thirtyone cases. Brit. J. Urol., 38: 36, 1966. 6. Harvard, B. M. and Thompson, G. J.: Congenital exstrophy of the urinary bladder: late results of treatment by the Coffey-Mayo method of ureterointestinal anastomosis. J. Urol., 65: 223, 1951. 7. Campbell, M. F. and Harrison, J. H.: Urology, 3rd ed. Philadelphia: W. B. Saunders Co., 1970.
