Management of urological dysfunction in pediatric patients with spinal dysraphism: review of the literature

Abstract

An intact, fully functional spine is the result of a complex sequence of embryological events involving both nervous and musculoskeletal system precursors. Deviations from this highly ordered system can result in congenital abnormalities ranging from clinically insignificant cosmetic changes to CNS malformations that are incompatible with life. Closure of the neural tube, which is believed to be the embryological event gone awry in these cases, is complete by just 28 days' gestation, often before pregnancy is detected. Although progress has been made to help prevent neural tube defects in the children of those attempting to conceive, these congenital deformities unfortunately continue to affect a startling number of infants worldwide each year. Furthermore, the precise mechanisms governing closure of the neural tube and how they might be interrupted remain elusive. What is known is that there are a large number of individuals who must deal with congenital spine dysraphism and the clinical sequelae on a daily basis. Bladder and urinary dysfunction are frequently encountered, and urological care is a critical, often neglected, component in the lifelong multidisciplinary approach to treatment. Although many treatment strategies have been devised, a need remains for evidence-based interventions, analysis of quality of life, and preemptive education of both caregivers and patients as they grow older. Pediatric neurosurgeons in particular have the unique opportunity to address these issues, often in the first few days of life and throughout pre- and postoperative evaluation. With proper management instituted at birth, many patients could potentially delay or avoid the potential urological complications resulting from congenital neurogenic bladder.