British Journal of Urology (19781, 50, 90-94

Exstrophy of the Bladder: Treatment by Trigonosigmoidostomy -Long-term Results W. GREGOIR and C. C. SCHULMAN Department of Urology, Brugmann University Hospital, University of Brussels, Brussels, Belgium

Summary-Twenty-five patients with bladder exstrophy were treated by implanting the vesical trigone into the sigmoid colon. Twenty-three have been followed for 3 to 19 years, with an average of 10 years. Taking into account the clinical status, renal function, the intravenous urogram, continence and social life, 18 patients were considered to have had an excellent result. There were 2 failures; 1 post-operative death occurred due to ruptured oesophageal varices and 1 patient developed bilateral hydronephrosis which necessitated cutaneous urinary diversion. In 4 cases the results, while satisfactory, were only fair in that 2 developed stones and 2 showed symptomatic electrolyte disturbances requiring treatment. Trigonosigmoidostomy preserves intact the ureterovesical junction which prevents colo-ureteric reflux and this probably accounts for the generally excellent long-term results. We believe the operation is preferable t o ureterosigmoidostomy in the management of bladder exstrophy.

Exstrophy of the bladder remains one of the ultimate challenges in paediatric urology. Although functional reconstruction seems the ideal solution, no operation can produce a normal bladder. The aim of the surgeon should be to have a continent child with normal social life, preserved renal function with normal upper tract, an acceptable physical appearance and ability to function sexually (Marshall and Muecke, 1970). Various methods have been used to treat the child with bladder exstrophy, including primary functional closure of the bladder (Fisher and Retik, 1969; Ezell and Carlsson, 1970; Marshall and Muecke, 1970; Cendron, 1971; Jeffs et al., 1972; Megalli and Lattimer, 1973; Williams and Keeton, 1973), ureteroilealcolostomy (Houtappel, 1963), primary ureterosigmoidostomy (Spence, 1966; King and Wendel, 1972; Bennett, 1973; Megalli and Lattimer, 1973), rectal bladder with adjacent colon pull through (Mauclaire, 1895; Heitz-Boyer and Hovelaque, 1912; Lowsley and Johnson, 1955; Duhamel, 1971; Nedelec et al., 1975), vesicoileosigmoidostomy (Hays et al., 1969; Singer and Pompino, 1966), bladder enlargement by intestinoplasty (Cukier and Ott, 1971; MartinezPineiro, 1976) and non-refluxing colon conduits (Hendren, 1976). _

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Read at the 33rd Annual Meeting of the British Association of Urological Surgeons it.) Aberdeen, June 1977.

An alternative approach in patients with bladder exstrophy consists of implanting the isolated trigone in the sigmoid colon. The method was first described by Maydl (1894) and it was widely used in the beginning of this century; Moorhead and Moorhead (1916) reviewed 152 cases. Several slight modifications of Maydl’s procedure were reported (Peters, 1901; Fowler, 1898; Moynihan, 1906; Boyce and Vest, 1952; Gregoir, 1968). Experimental studies on urinary diversion through the use of vesico-rectal anastomosis demonstrated a satisfactory preservation of renal function (Boyce, 1951). The purpose of this report is to present the results of treatment of exstrophy of the bladder by trigonosigmoidostomy in 25 patients.

Patients Twenty-five patients with classic bladder exstrophy have been treated by trigonosigmoidostomy. The series included only 2 females. The ages ranged from 2 t o 30 years (average 6 years). The follow-up period was from 3 to 19 years (average follow-up period 10 years). This operation was the primary treatment in 23 cases; in 2 cases it was performed after failure of an attempt at bladder reconstruction. The indications for operation were based on 3 factors:

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1. a normal IVP; 2. a normal competent anal sphincter; 3. the absence of reflux.

Operation Technique The operation is performed when the competence of the anal sphincter has been satisfactorily tested. In some cases it is necessary to wait until the child is 3 years old to ascertain that the sphincter is competent. Prolonged exposure of the exstrophied bladder mucosa will produce inflammatory lesions, fibrosis and damage the uretero-trigonal muscu-

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lature which may later result in reflux or squamous metaplasia and glandular adenoma in which neoplastic changes might develop (Culp, 1964). The absence of reflux can be tested easily. A small glass funnel is firmly applied on the abdominal wall over the exstrophied bladder. The funnel is attached to a T-tube and through 1 of the arms contrast medium is injected under pressure with a syringe; a manometer is attached to the other arm to measure the pressure. The operation consists of the implantation of the vesical trigone in the sigmoid colon without any cutaneous diversion; the technique has been previously described in detail (Gregoir, 1968). Results Twenty-three patients have been followed from 3 to 19 years, the average period being 10 years. One patient from another country was lost to follow-up and 1 patient died in the post-operative period from rupture of oesophageal varices. In general, patients who developed complications have deteriorated within the first years after the operation. Patients who had a normal urogram, minor or no infection, and good electrolytic balance for a few years did not deteriorate subsequently.

Renal Function The urogram has given the most valuable information in the post-operative follow-up. In 2 cases the urogram showed stones in the kidney and 1 of the stones caused ureteric obstruction. In another case bilateral hydronephrosis developed accompanied by severe parenchymal infection due to reflux. This case had had several unsuccessful attempts at reconstruction and was the first in this series; reflux had not been tested for before the operation and a cutaneous diversion had to be performed. In all other 23 cases the urogram was satisfactory at repeated intervals.

Fig. 1 Control IVP 8 years after trigonosigmoidostomy in a girl with bladder exstrophy operated at the age of 4 years. Normal renal function. No upper tract dilatation. Note that the sigmoid colon has taken the form of a “bladder”.

Electrolytic Disturbances About half of the patients showed transient elevation of the blood chloride and a lowering of bicarbonate. The remainder of the patients showed no electrolytic disturbances. These changes were insignificant in most cases and the patients remained asymptomatic. A few cases required corrective medical measures such as alkalinisation. About one-third of the infants developed

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abnormal thirst; they did not however show evident electrolytic disturbances or renal insufficiency. In all the 23 patients having a normal urogram, blood urea and creatinine were within normal limits.

Infection The rate of infection and complications was surprisingly low and antibiotic medication was administered for only a short period. Prolonged medication was not necessary. Operations f o r Complications Two patients required an additional operation; 1 for an obstructive ureteric stone, the other for hydronephrosis and reflux underwent a cutaneous diversion. Continence During the day continence was perfect in all cases. During the night however most patients were incontinent for several months after the operation. This lasted for more than 5 years in 6 cases but finally disappeared in most of them. As far as the sexual function is concerned most of our patients are still too young t o be objectively evaluated, but some adults have married recently. Overall Results

Failures ( 1 ) One death: a 30-year-old alcoholic patient had a sudden rupture of oesophageal varices on the fifth post-operative day and died. (2) One case was a complete failure as it required a secondary urinary diversion for severe pyelonephritis and hydronephrosis. Satisfactory Four cases were considered as satisfactory. Two had symptomatic electrolytic disturbances that required an alkali regime and 2 others developed stones requiring operative removal. Good Eighteen cases were considered as good. They were followed for a minimum of 3 years and for an average of 10 years. They were symptom free, the urogram remained normal, blood chemistry was within normal limits and they led a normal social life with normal life expectancy. The adults are gainfully employed and some of them

Fig. 2 Normal urograrn 6 years after trigonosigmoidostorny .

are married. They are all perfectly continent although a few cases suffered from night wetting for some years before it finally disappeared.

Discussion The basic principle that inspired Maydl (1894) to perform implantation of the trigone in the sigmoid colon in bladder exstrophy was to preserve intact the uretero-vesical junction so as to achieve better functional results than with a simple ureterosigmoidostomy. The present series seems to confirm this idea. Histological study of the uretero-vesical junction in bladder exstrophy has shown that the uretero-trigonal musculature is quite normal. The exstrophied bladder does not reflux. The normal antireflux function of the uretero-vesical junction is preserved in trigonosigmoidostomy if the trigone is normal before the operation and if its vascular and nervous supply is

EXSTROPHY OF THE BLADDER: TREATMENT BY TRIGONOSIGMOIWSTOMY-LONG-TERM RESULTS

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Fig. 3 Trigonosigmoidostomyfor bladder exstrophy-control urograms: (a) 4 years later; (b) 14 years later; (c) 19 years later.

not jeopardised during the procedure. This antireflux mechanism deteriorates and disappears if prolonged exposure of the mucosa produces inflammatory lesions, fibrosis or squamous metaplasia that destroys the uretero-trigonal musculature. Several authors emphasise that reconstructed exstrophied bladders present a high percentage of reflux. In this instance however our opinion is that reflux is secondary to fibrosis and rigidity of the reconstructed bladder which may be of reduced capacity and the seat of severe chronic infection. In about half of the cases (12) with trigonosigmoidostomy the injection of contrast medium in the rectosigmoid did not show reflux to the ureters. The others were not tested. Primary reconstruction of the exstrophied bladder will always remain the theoretical ideal objective, but even in the experienced hand of surgeons with a special interest in the problem the success rate is small, serious complications such as hydronephrosis, severe pyelonephritis and stone formations are not rare and these children may undergo multiple operations with long hospitalisation and difficult social and psychological problems. For example Williams and Keeton (1973) reviewed 36 cases in their latest series and considered that 19 were reasonable candidates for primary closure, accept-

able continence being obtained in 6. Marshall and Muecke (1970) had satisfactory results in 20% of their cases. Fisher and Retik (1969) reviewed 26 cases of which 9 were continent. Ezell and Carlsson (1970) had 19 patients of whom 9 were reconstructed and 2 achieved good continence. Cendron (1971) obtained acceptable continence in 6 of 12 children; reflux was present in 3 of these cases. Megalli and Lattimer (1973) reviewed 140 cases of exstrophy; primary closure was performed on 89 children of whom only 1 achieved successful continence, and 26 underwent subsequent diversion. Williams and Keeton (1973) consider that if functional reconstruction of the exstrophied bladder is to be considered some selection is important and that about 50% of cases may be considered as reasonable candidates for attempts at primary closure knowing that two-thirds of them will still need to be diverted. Marshall and Muecke (1970) are of the opinion that surgical closure of exstrophy of the bladder is still an investigative procedure worth consideration. It is not our intention to claim that trigonosigmoidostomy in patients with bladder exstrophy is the ideal solution, but in our opinion it represents an acceptable compromise for this major urological malformation. The results of trigonosigmoidostomy seem more favourable than those

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obtained with a conventional ureterosigmoidostomy as far as renal function, infection and clinical status are concerned (Spence, 1966; King and Wendel, 1972; Bennett, 1973; Megalli and Lattimer, 1973). In this series, 75% of the patients have achieved an excellent result after a single operation and have met all Marshall's criteria for successful management of exstrophy.

References Bennett, A. H. (1973).Exstrophyof bladder treated by ureterosigmoidostomies: long-term evaluation. Urology, 2, 165-168. Boyce, W. H. (1951). The absorption of certain constituents of urine from the large bowel of the experimental animal (dog). Journal of Urology, 65,241 -261, Boyce, W. H. (1971). A new concept concerning treatment of exstrophy of the bladder: 20 years later. Transactionsof the American Association of Cenito-Urinary Surgeons, 63, 121-134. Boyce, W. H. and Vest, S. A. (1952). A new concept concerning treatment of exstrophy of the bladder. Journal of Urology, 67, 503-5 17. Cendron, J. (1971). La reconstruction vesicale. Annales de Chirurgielnfantile, 12,371-381. Cukier, 1. and Ott, R. (1971). Resultats decevants des tentatives reconstructives dans le traitement de l'extrophie vesicale complete. Annales de Chirurgie Infantile, 12, 382-385. Culp, D. A. (1964). The histology of the exstrophied bladder. Journal of Urology, 91,538-548. Duhamel, B. (1971). Les vessies intestinales contrSlees par le sphincter anal. Techniques et resultats. Annales de Chirurgie Infantile, 12,433-442. Ezell, W. W. and Carlsson, H. E. (1970). A realistic look at exstrophy of the bladder. British Journal of Urology, 42, 197-202. Fisher, J. H. and Retik, A. B. (1969). Exstrophy of the bladder. Journal of Paediatric Surgery, 4. 620-626. Fowler, G. R. (1898). Implantation of the ureters into the rectum in exstrophy of the bladder, with a description of a new method of operation. American Journal of Medical Science, 115, 270-276. Gregoir, W. (1968). L'implantation trigono-rectale, Operation de Maydl. Urologia Internationalis, 23,4148. Hays, D. M., Powell, T. 0. and Strauss, J. (1969). Vesicoileosigmoidostomy in the treatment of exstrophy. Surgery, 66, 1103-111 1 . Heitz-Boyer, M. and Hovelaque, A. (1912). Creation d'une nouvelle vessie et d'un nouvel urbtre. Journal d'llrologie, 1, 237-258. Hendren, W. H. (1976). Exstrophy of the bladder. An alternative method of management. Journal of Urology, 115, 195-202. Houtappel, H. C. (1963). Experience with uretero-ileosigmoidostomy. British Journal of Urology, 35,277. Jeffs, R. D., Charrois, R., Many, M. and Juriansz, A. R. (1972). Primary closure of the exstrophied bladder. I n Current Controversies in Urologic Management (ed. by

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R. Scott, Jr., H. L. Gordon, F. B. Scott, C. E. Carlton and P. D. Beach). Saunders, Philadelphia, pp. 235-243. King, L. R. and Wendel, E. F. (1972). Primary cystectomy and permanent urinary diversion in the treatment of exstrophy of the urinary bladder. I n Current Controversies in Urologic Management(ed. by R. Scott, Jr., H. L. Gordon, F. B. Scott. C. E. Carlton and P. D. Beach). Saunders, Philadelphia, pp. 244-250. Lowsley, 0. S. and Johnson, T. H. (1955). A new operation for creation of an artificial bladder with voluntary control of urine and faeces. Journal of Urology, 73,83-90. Martinez-Pineiro, J. A. (1976). Long-term results of surgical treatment of bladder exstrophy. European Urology, 2, 168-174. Marshall, V. F. and Muecke, E. C. (1970). Functional closure of typical exstrophy of the bladder. Journal of Urology, 104, 205 -2 12. Mauclaire, P. (1895). De quelques essais de chirurgie experimentale applicables au traitement de I'exstrophie de la vessie et des anus contre nature complexes. Annales de Maladies des Organes Gknito-urinaires, 13, 1080-1086. Maydl, K. (1894). Uber die Radikltherapie der Ectopia vesical urinariae. WienerMedizinische Wochenschrvt,25,1I13-1115, 1169-1172, 1209-1210, 1256-1258, 1297-1301. Megalli. M. and Lattimer, J. K. (1973). Review of the management of 140 cases of exstrophy of the bladder. Journal of Urology, 109,246-248. Moorhead, J . L. and Moorhead, E. L. (1916). Exstrophy of the bladder. Journal of the American Medical Association, 66,40941 1. Moynihan, B. G. A. (1906). Extroversion of the bladderRelief by transplantation of the bladder into the rectum. Annals of Surgery, 43, 231-240. Nedelec. M., Auvigne, J., Buzelin, J. M., Devineau, G . , and Mathevel, J. (1975). De la neo-vessie rectale dans la cure des malformations uro-genitales. Journal d'Urologie et de Nephrologie, 81, 481496. Peters, G.A. (1901). Transplantation of the ureters into rectum by an extraperitoneal method for exstrophy of the bladder. British Medical Journal, 1, 1538-1542. Singer, H. and Pompino, H. (1966). Technik und Erfahrungen mit kilnstlichen Harnableitungen nach Tiersch-Tuffier. Urologia Internationalis, 23,49-53. Spence, H. M. (1966). Ureterosigmoidostomy for exstrophy of the bladder. Results in a personal series of 31 cases. British Journal of Urology, 38,3643. Williams, D. 1. and Keeton, J. (1973). Vesical exstrophy-20 years experience. British Journal of Surgery, 60,203-207.

The Authors W. Gregoir, MD, Professor of Urology. C. C. Schulman, MD, Urologist in charge of Paediatric Urology.

Requests for reprints to: Professor W. Gregoir, Brugmann University Hospital, University of Brussels, Brussels, Belgium.

Exstrophy of the bladder: treatment by trigonosigmoidostomy--long-term results.

British Journal of Urology (19781, 50, 90-94 Exstrophy of the Bladder: Treatment by Trigonosigmoidostomy -Long-term Results W. GREGOIR and C. C. SCHU...
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