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Closed spinal dysraphism: Pathogenesis and types

Chaouki Khoury, MD, MS
Section Editor
Marc C Patterson, MD, FRACP
Deputy Editor
Carrie Armsby, MD, MPH


Neural tube defects are congenital anomalies of neural development with a spectrum of clinical manifestations. They can affect the cranium or spine and are often associated with cutaneous findings [1].

Cranial defects include anencephaly, exencephaly, and encephalocele. (See "Primary (congenital) encephalocele" and "Anencephaly".)    

Open spinal dysraphism (spina bifida aperta) is characterized by a cleft in the spinal column, with herniation of the meninges (meningocele) or meninges and spinal cord (myelomeningocele) through the defect. (See "Pathophysiology and clinical manifestations of myelomeningocele (spina bifida)" and "Overview of the management of myelomeningocele (spina bifida)".)  

Closed spinal dysraphism (also known as occult spinal dysraphism or spina bifida occulta) is characterized by failure of fusion of the vertebral bodies due to abnormal fusion of the posterior vertebral arches, with unexposed neural tissue; the skin overlying the defect is intact. The more common and least severe forms consist of isolated vertebral bony defects. However, the vertebral defects may occur in association with other more severe anomalies of the spinal cord and sacral structures, such as split spinal cord malformation or various cavitary defects of the spinal cord.

The clinical manifestations of closed (occult) spinal dysraphism (CSD) vary widely and range from benign or asymptomatic to severe neurologic, genitourinary, gastrointestinal, or musculoskeletal anomalies. The clinical presentation, evaluation, and management of these disorders are discussed in a separate topic review. (See "Closed spinal dysraphism: Clinical manifestations, diagnosis, and management".)


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Literature review current through: Sep 2016. | This topic last updated: Dec 14, 2015.
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