Foreword.

Abstract

Spina bifida is one of the most common congenital birth defects in the United States, with prevalence estimated at 3 per 10,000 live births.1 Prevalence varies considerably worldwide, with some regions in China reporting rates more than 20 times as high.2 Within the United States, the highest incidence rate is among the Hispanics (4.2 per 10,000 live births), followed by non-Hispanic whites (3.2 per 10,000) and non-Hispanic African Americans (2.6 per 10,000).3 Spina bifida is caused by the failure of the neural tube to close during embryonic development; however, causal factors remain largely unknown. Genetic factors are estimated at 60% to 70%, although specific genes have not been identified.4 The primary nongenetic factor is inadequate maternal folic consumption. Other factors include anticonvulsant therapy, diabetes mellitus, and obesity. Primary prevention efforts focus on folic acid supplementation, including countrywide food fortification programs (eg, United States, Canada, Chile, Costa Rica, and South Africa). Woman of childbearing age are recommended to take 0.4 mg per day and up to 4 mg for those at high risk (eg, first-degree relative or previous pregnancy with neural tube defect).There are 4 types of spina bifida, ranging in severity. Severity is dependent on the location and size of the malformation and its effect on the spinal cord and nerves. The most common and severe subtype of spina bifida compatible with life is myelomeningocele, in which the spinal cord and nerves protrude through open vertebrae. In meningocele, a more moderate form, spinal fluid and meninges protrude through the vertebral opening, but the spinal cord remains intact. Closed neural tube defects include lipomeningocele in which a lipoma involves the spinal cord and tethered cord syndrome. Occulta, the mildest form often resulting in no impairment, occurs when there is a small gap in the spinal column but no open lesion or sac.Prenatal diagnosis of a neural tube defect can be accomplished by ultrasonic evaluation of the shape of the fetal skull and spine and measurement of α-fetoprotein in the maternal blood. Following prenatal diagnosis, woman may elect termination or surgical repair of the lesion, either in utero or postnatally. In addition to neurological deficits related to the level of the spinal cord lesion, affected children may also experience a Chiari II malformation, hydrocephalus, orthopedic, urological, and neuropsychological sequelae. Moreover, youth with spina bifida are at risk for psychosocial challenges, including depressive symptoms, lower levels of self-concept, social difficulties, and lower perceived health-related quality of life.56 Spina bifida also poses challenges to caregivers and their families and has significant economic consequences.7This issue of Topics in Spinal Cord Injury Rehabilitation includes several articles on spina bifida. The first focuses on the treatment options of hydrocephalus associated with myelomeningocele. Ventriculoperitoneal shunts as well as endoscopic third ventriculostomy with choroid plexus cauterization and conservative management of relatively stable ventriculomegaly are presented by Norkett et al. Next, Altiok et al discuss the development of scoliosis in youth with spina bifida and the effect of tethered cord release on the progression of scoliosis. Then Dr. Wasserman and colleagues investigate the common concern of attention problems and executive function deficits in children with spina bifida, examining differences in identification, diagnosis, and treatment of attention-deficit/hyperactivity disorder in children with and without spina bifida.We hope you will find this issue informative, enjoyable to read, and thought provoking. As always, we thank you for your readership and contributions to TSCIR. Please continue to contact us with your questions, reflections, and ideas for future issues.