Neurosurgical Management of Myelomeningocele (Spina Bifida)

Mark S. Dias, MD, FAAP* 1. *Associate Professor of Neurosurgery, Pennsylvania State University College of Medicine and Milton S. Hershey Medical Center, Hershey, Pa After completing this article, readers should be able to: 1. Understand the outcomes regarding survival, cognitive abilities, ambulation capacities, and urologic functions for the child who has a myelomeningocele. 2. Appreciate the importance of folate in the pathogenesis of myelomeningoceles and the rationale for periconceptional folate supplementation in the prevention of neural tube defects. 3. Describe the initial neurosurgical care and rationale for neurosurgical treatment for the child who has a myelomeningocele. 4. Discuss the common presenting features of a shunt malfunction in the child who has a myelomeningocele. 5. Understand the significance and describe the management of the child who has signs or symptoms of shunt malfunction and no change in ventricular size on imaging studies. 6. Discuss the importance of considering shunt malfunction before initiating other neurosurgical treatments for the deteriorating child who has a myelomeningocele. 7. Describe the common signs and symptoms of spinal cord tethering, Chiari malformation, and syringomyelia, and the surgical options for each. Myelomeningocele (spina bifida) is the most common congenital neurologic malformation compatible with life. Therefore, every busy pediatric practice includes at least a few children who have spina bifida, and it is important for the primary care clinician to have a practical working knowledge about the unique and important problems that face this group of children. The incidence of myelomeningocele in the United States is between 0.41 and 1.43 per 1,000 live births (1) and is lower among African-Americans than Caucasians. Native Africans have a very low incidence of 0.1 per 1,000 live births. (2) The incidence of myelomeningocele in the United States and worldwide has declined significantly over the past 3 decades. (2) This decline has been attributed, in part, to improved maternal nutrition and periconceptional vitamin supplementation and to wider availability of prenatal diagnostic tests with a corresponding increase in the number of elective terminations. However, these …