Spinal Dysraphism

Spinal dysraphism refers to a variety of congenital nervous system anomalies that arise from failures in neural tube formation and are discussed in greater detail in Chapter 8. Myelomeningocele is the most common developmental nervous system disorder, although its incidence is decreasing, presumably bacause of prevention by the use of folic acid during pregnancy, and also due to early diagnosis leading to termination. A practical method of grouping the various forms of spinal dysraphism is into the open ('aperta') and closed ('occulta') categories. Myelomeningocele is an example of an open neural tube defect and is defined by the presence of an unclosed spinal cord that is displaced dorsally by a ventral CSF collection. The postnatal diagnosis is clear, with an obvious CSF-filled sac visibly protruding through a dorsal defect in the spine and soft tissues. If the sac ruptures, with a corresponding release of CSF, the neural tissue may spontaneously reduce into a more ventral position. In the neonatal period, the treatment goals are prevention of infection by early closure of the defect and placement of a VP shunt if hydrocephalus is present. Additional problems that require the participation of other specialties include hip dislocation, clubfeet, scoliosis and kyphosis and urinary retention.

Spina bifida occulta consists of a number of anomalies that range from minor anatomical abnormalities (e.g., fatty filum terminale) to extremely severe anomalies that can cause severe neurological compromise (e.g., lipomyelomeningoceles, spinal cord lipoma, split cord malformations and myelocystoceles). The presence of normal skin covering these anomalies means that immediate repair to prevent infection is not required. Often, repair is delayed until later in the first year of life.

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