Spinal Dysraphism

Frank Acosta, Jr. and Nalin Gupta Introduction

Neural tube defects (NTDs) are a group of congenital anomalies characterized by defects in dorsal midline structures, including neural tissue, dura, muscle, bone and/or skin. Spinal and cranial dysraphism refer to those anomalies affecting bony and/or nervous components of the spine or brain, respectively. The primary em-bryological defect is believed to be a failure of, or incomplete, neural tube closure. This is a process that normally occurs during the third to fourth week of fetal life. These lesions can involve any part of the spine, although they most often involve the lumbosacral spine, and range from a simple gap in the lamina of a single vertebral level to an extensive dorsal opening with an exposed spinal cord. The incidence of spinal dysraphism is estimated at 0.05 to 0.25 per 1000 live births per year.

Severe spinal dysraphic disorders constitute a major source of disability among children and adults. Management of spinal dysraphic anomalies involves a number of steps: accurate diagnosis; an assessment of the severity of the lesion; a decision whether intervention is warranted; the nature of the intervention; and educating the family of the need for lifelong medical care. Below we present a practical framework for the evaluation and treatment of spinal dysraphism that reflects current standards of management.

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