British Journal of Urology (1992)), 70,683-685 01992 British Journal of Urology

Results of Surgical Treatment in Children with Bladder Exstrophy A. CSONTAI, M. MERKSZ, L. PIR6T and J. T6TH Department of Urology, Heim Phl Children's Hospital, Budapest, Hungary

Summary-A series of 42 children (30 boys and 12 girls) underwent surgery for bladder exstrophy between 1972 and 1989. Primary bladder closure was performed in 1 1 patients and was successful in 6. Four of these children are about 2 years old and so it is not yet possible to assess their continence. Ureterosigmoidostomy was performed in 35 children, one of whom was converted to cutaneous ureterostomy. Follow-up ranges from 2 to 20 years (average 9 years 3 months) and 50% of the patients are symptom-free. The most frequent problems were acidosis and urinary tract dilatation. Other complications, s u c h a s hypokalaemia and pyelonephritis, were seldom seen. Although ureterosigmoidostomy has some disadvantages, o u r patients have adapted well and lead a normal life.

Management of the bladder exstrophy patient presents a great challenge to the surgeon. Closure of the bladder is the ideal solution, but reconstructive surgery cannot create completely normal anatomy and physiology. We present our experience with bladder exstrophy patients in the 1970s and 1980s. Patients and Methods Between 1972 and 1989, 42 children (30 boys and 12 girls) underwent surgery for bladder exstrophy. Their ages at the time of the first operation ranged between 7 days and 18 years and they are currently aged between 2 and 34 years. Mean follow-up is 9 years 3 months. Eight children had associated anomalies : horseshoe kidney (l), duplex kidney (l), solitary kidney (l), ectodermal dysplasia (l), heart failure (l), vertebral anomalies (l), bilateral undescended testis (5). Primary bladder closure. During the 1970s and in the early 1980s we had a strict policy for selecting Presented at the Third Annual Meeting of the European Society of Paediatric Urology in Cambridge, 20-22 March 1992

children for bladder closure. Contraindications included those with a small bladder, with infection or leukoplakia on the bladder mucosa and those whose general condition was not good. Bladder closure was performed in 11 patients (9 in this institute and 2 in other hospitals; in the latter 2 cases the procedure was unsuccessful). Bladder closure was successful in 6 cases (in 3 following repeat procedures) (Table 1). The 9 patients treated here were between 7 days and 1 month old, except for one child who was 1 year old. The ureters were always stented for 14 days and a small feeding tube was placed in the bladder and led out through the urethra or through the wound. Osteotomy was not performed after 1970 because it did not lead to improved results. In our opinion the divergent pubic bones can be brought together easily because of the mobility of the sacroiliac articulation; it is important to secure the pubic bones with nylon sutures. Urinary diversion. In children who were judged unsuitable for primary or repeat bladder closure, urinary diversion was performed; 35 children underwent this operation (24 boys and 11 girls); 24 were aged between 2 and 3 years, 10 between 3 and 6 and one boy was 18 years old. In each case a

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Table 1 Reconstructive Procedures for Bladder Closure in 6 Patients Type of surgery

Bladder closure (1 operation) Reconstruction for complete bladder dehiscence Reconstruction for partial prolapse

No. of operations

3 2 4

Table 2 Ureterosigmoidostomy : Outcome and Symp toms in 34 Patients Free of symptoms Chronic pyelonephritis Urinary tract dilatation Non-functioning kidney Impaired renal function Acidosis H ypokalaemia Hypocalcaemia Anal incontinence Mortality

sign of urinary tract dilatation or infection. Four children are now 2 years old, so their continence cannot yet be established. Two boys (6 and 14 years old) have only partial continence and can void 60 and 100 ml respectively. Urinary diversion. In all, 34 patients have ureterosigmoidostomies. All who underwent urinary diversion are alive and 17 patients (50%) are free of symptoms. Of the remaining 17 patients, 13 have some degree of urinary tract dilatation and 5 have chronic pyelonephritis for which 2 nephrectomies have been performed. Acidosis is minimal in 5 cases and moderate in 10. Hypokalaemia occurred in 4 patients, necessitating substitution. Transitory hypocalcaemia was seen in 1 child. All patients have good anal continence (Table 2).

Discussion

Several decades ago it was generally accepted that urinary diversion was the only solution to bladder exstrophy. Ureterosigmoidostomy was usually performed, but other forms of diversion appeared later and only a few bladder closures were carried out. ureterosigmoidostomy was done. Examinations The number of bladder closures increased gradually were carried out pre-operatively to establish anal but even now, when most centres agree that it continence and 8 children underwent surgery would be the most beneficial procedure, the perrelated to their bladder exstrophy. Three needed centage of diverted patients is still significant perineal/anal procedures prior to urinary diversion (O’Donnel, 1984; Connor et al., 1988; De la Hunt and this resulted in good anal continence. Five et al., 1989). In Great Britain and Ireland, 38 of 81 children had failed bladder closure; one of them patients born between 1975 and 1985 were diverted had undergone bladder closure elsewhere, followed (De la Hunt et al., 1989). Urinary diversion is often by’abdominal wall reconstruction due to eventra- necessary after bladder closure as a result of tion and later a laparotomy for ileus. Ureterosig- complications such as hydroureteronephrosis or moidostomy was followed by early complications stones. O’Donnell (1984) reported that 14/24 chilin 5 children: paralytic ileus with partial wound dren were diverted. On the other hand, in the dehiscence in 2 and acute pyelonephritis in 2. One management of patients with failed exstrophy boy developed urinary leakage from the abdomen closure (Gearhart et al., 1991) and in operative on the eighth post-operative day; this was caused techniques designed to improve continence after by bilateral dehiscence of the anastomosis between bladder closure (Gearhart et al., 1989; Hollowell et the ureter and the sigmoid and bilateral ureterocu- al., 1991), further progress has been achieved. In taneostomy was done. Late post-operative compli- the present study, 35/42 patients (83%) have cations necessitating surgery occurred in 3 cases : diverted urinary tracts. The fact that most of our in 2 children a pyelonephrotic non-functioning successful bladder closures were carried out in 1988 kidney had to be removed and in one girl, 10 years and 1989 reflects the changes in our approach to after ureterosigmoidostomy, a laparotomy was treatment and the changes in referral patterns to performed for mechanical ileus caused by strangu- this institute. In the management of bladder lation. exstrophy patients we have gained experience mainly with ureterosigmoidostomy and follow-up is sufficiently long for an opinion to be formed Results about the results. It is noteworthy that 50% of our Primary bladder closure. In all 6 children both the patients are completely free of symptoms. Those kidneys and the urinary tracts are normal, with no who do have symptoms can be managed conserva-

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tively and all have an acceptable life style. Patients aged 6 to 18years attend school, some are university students and those who have reached adulthood are all working. One patient gave birth to a child. Thus, although ureterosigmoidostomy has its disadvantages, it does enable patients to lead a normal life.

Gearhart, J. P. and Jeffs, R. D. (1991). Management of the failed exstrophy closure. J. Urol., 146,610-612. Hollowell,J. G. and Ransley, P. G. (1991). Surgical management of incontinence in bladder exstrophy. Br. J. Urol., 68, 543548. O’Donnell,B. (1984). The lesson of 40 bladder exstrophies in 20 years. J. Pediatr. Surg., 19,547-549.

References

The Authors

Connor,J. P., Lattimer, J. K., Hensle, T. W. e t d (1988). Primary closure in bladder exstrophy : long-term functional results in 137 patients. J. Pediatr. Surg., 23, 1102-1 106. DelaHunt,M.N.andO’Donnell,B.(1989). Currentmanagement of bladder exstrophy: a BAPS collective review from eight centersof 81 patients born between 1975 and 1985.J . Pediatr. Surg., 24,584-585. Gearhart, J. P. and Jeffs, R. D. (1989). State-of-the-art reconstructive surgery for bladder exstrophy at the Johns Hopkins Hospital. Am. J . Dis. Child., 143, 1475-1478.

A. Csontai, MD, Paediatric Urologist; Chief of Department of Urology. M. Merksz, MD, Paediatric Urologist. L. Pi& MD, Paediatric Urologist. J. T6th, MD, Paediatric Urologist; Chief(retired)ofDepartment of Urology. Requests for reprints to: A. Csontai, Department of Urology, Heim Pal Children’s Hospital, U11oi ut 86, H-1089 Budapest, Hungary.

Results of surgical treatment in children with bladder exstrophy.

A series of 42 children (30 boys and 12 girls) underwent surgery for bladder exstrophy between 1972 and 1989. Primary bladder closure was performed in...
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