Long-term outcome analysis of low pressure rectal reservoirs in 33 children with bladder exstrophy

Abstract

Purpose: The augmented valved rectum and double folded rectosigmoid bladder represent 2 modifications of ureterosigmoidostomy. Both procedures improve continence by lowering the reservoir pressure. We present the outcome of these techniques on the upper urinary tract, continence, metabolic profile, linear growth and bone density.

Materials and methods: Between March 1987 and May 1997, 22 boys and 11 girls with bladder exstrophy underwent urinary diversion to a low pressure rectal reservoir. Patient age at surgery ranged from 2 to 13 years (mean 5.4). The augmented valved rectum technique was used in 18 cases and the double folded rectosigmoid bladder method was used in 15. Serial followup ultrasounds were obtained and voiding proctography was performed 1 year postoperatively in all patients. Supine height was measured at last followup in all cases. Serum electrolytes, arterial blood gases, the results of which were plotted on an acid base nomogram, and bone density using dual energy x-ray absorptiometry were measured in all patients. Prophylactic alkalization was administered to all patients.

Results: Mean followup is 66 months (range 24 to 148). All patients are continent during the daytime with an emptying frequency of 3 to 5 times, and all are continent at night. No patient experienced pyelonephritis or clinical acidosis. The upper urinary tract was either improved or stabilized in 64 of 66 renal units. No patient had reflux to the upper urinary tract. All patients had normal serum creatinine, sodium, potassium, calcium and phosphorus. Hyperchloremia was noted in 19 of the 33 patients (57%), and arterial blood gases showed subclinical metabolic acidosis in 18 (55%). Supine height measurements at last followup revealed that 19 of the 33 patients (57%) had decreased linear growth (below 3rd percentile). All patients had significant reduction in bone density, and mean for age corrected bone density was 70% (standard deviation 10.9%). Both groups (augmented valved rectum and double folded rectosigmoid bladder) were comparable in regard to age, sex and followup duration. There was no statistically significant difference between the groups in any parameter measured.

Conclusions: The augmented valved rectum and double folded rectosigmoid bladder provide preservation of the upper urinary tract with excellent continence rates. However, prophylactic alkalization and functional isolation of the reservoir do not prevent the long-term metabolic consequences. Subclinical metabolic acidosis and decreased linear growth are to be anticipated in more than 50% of patients. Moreover, significant bone demineralization is to be expected in all of these patients.