Neurogenic bladder and bowel dysfunction : Clinical aspects in children with spinal dysraphism

Sammanfattning: Spinal dysraphism (SD) is a congenital malformation that to a varying extent, often severely, affects the life of the child and the family. Most individuals with SD suffer from neurogenic bladder and bowel dysfunction—with the risk of urinary tract infections, renal deterioration, urinary and fecal incontinence—that affects social participation and quality of life negatively. In newborns with SD, early detection of neurogenic bladder dysfunction and determination of post-void residual urine are required to determine the need of clean intermittent catheterization (CIC) and follow-up.The non-invasive method of four-hour voiding observation with provocation test (VOP) was used to evaluate bladder function in 50 newborn children with SD. Voiding patterns for the children were described and compared with those of 50 healthy newborns evaluated with VOP in an earlier study. Comparison revealed significant differences among several variables. In particular, leakage at provocation test and not voiding with a stream were common in newborns with SD but did not occur in healthy newborns. VOP is a non-invasive standardized method to determine residual urine in newborns with SD. It also adds information on voiding pattern, frequency, voiding with a stream and leakage at provocation.Findings in neonatal VOP of the same cohort of newborns with SD were then related to radiology, presence of urinary tract infections during the first year, and urodynamic findings and use of CIC at the age of one year. It was found that, in children with SD, not voiding with a stream may have a predictive value for the need of CIC at the age of one year, followed probably by lifelong CIC. Despite this, the presence of an open SD per se has stronger predictive value, and each child needs to be evaluated individually while considering a number of factors. The main value of VOP may be as a structured non-invasive screening method to uncover neurogenic bladder-sphincter dysfunction in the newborn. Studies with a larger number of subjects than the present are needed to evaluate the potential of VOP in newborns with closed spinal dysraphism in whom the neurological consequences vary.A retrospective analysis detected renal damage on DMSA scintigraphy in 5 of 41 children with SD who were followed according to a proactive national program with minimal use of surgery. Median follow-up time was 10 years. High baseline pressure was confirmed as a risk factor for renal damage. Compliance with treatment and follow-up is likely to be an important factor for renal health. Therefore, efforts to support children and their families are crucial. A questionnaire-based study of 107 children with SD (age 6–16y) in Sweden and Norway examined aspects of treatment for neurogenic bowel dysfunction focusing on incontinence, independence, general satisfaction and quality of life. It was found that transanal irrigation (TAI) and antegrade colonic enemas (ACE) are effective treatments, but are time-consuming and difficult to perform independently. The majority of children using TAI (72%) and ACE (63%) never went to the toilet alone to empty their bowels. As children achieving independence on the toilet reported higher quality of life, efforts to support independence are beneficial.Continent, self-managing children with healthy kidneys enjoy high quality of life and contribute more fully to society. Therefore, further research is required to investigate and develop existing and new technologies and methods that mitigate the problems related to SD, and to make them accessible to all children with spinal dysraphism.