No abstract

Contrary to some earlier reports, our findings suggest that bladder dysfunction does not always present as a large atonic bladder in the Wolfram syndrome. A low capacity, high pressure bladder with sphincteric dyssynergia is also common. The presence and duration of other syndrome manifestations do not correlate with the type of bladder dysfunction, suggesting that bladder dysfunction may also be a primary rather than secondary component of the syndrome.

Objectives To present the results of bladder autoaugmentation covered with a peritoneal¯ap in patients with bladder dysfunction. Patients and methods Thirteen patients (seven male and six female, mean age 11.9 years, range 4±25) who underwent autoaugmentation covered with a peritoneal¯ap were evaluated. Seven had different forms of myelodysplasia, four had spinal cord injury and two had Hinman syndrome as the cause of bladder dysfunction. Indications for augmentation included upper tract deterioration, urinary incontinence and recurrent urinary tract infection, despite anticholinergic therapy. Results The mean bladder capacity increased by 18.6% after surgery and the mean compliance at capacity increased from 3.4 to 5.8 cmH 2 O/mL. All patients were incontinent before surgery and continence was achieved in only six afterward. Four patients showed no clinical or urodynamic improvement and required re-augmentation using intestinal segments. Only three patients needed no anticholinergic therapy after surgery. All four patients in whom the procedure failed had capacities of <30% of that expected for their age. There were no metabolic problems. Conclusion Autoaugmentation combined with a peritoneal¯ap is an easy procedure but the clinical results are poor in some patients, especially those with a small initial bladder capacity. The need for secondary augmentation with enteric segments was common. The use of a peritoneal¯ap does not appear to increase the capacity and compliance more than is obtained with the classical technique; it may prevent adhesion to the abdominal wall and make a secondary procedure easier. As the increase in capacity and compliance is limited with this technique, a urothelium-preserving augmentation should be reserved for those bladders with a relatively good initial capacity.

The results of the BTA stat test in the presence of microscopic hematuria must be interpreted in regard to the degree of hematuria. The test is not reliable in urine samples with gross hematuria due to a high false-positive rate.