Spinal Dysraphism (Continued)


A far more devastating variant of spina bifida aperta is myelomeningocele, in which the spinal cord or nerve roots, or both, protrude through the posterior bony and cutaneous defects due to failed closure of the posterior neuropore. The severity of deficits from a myelomeningocele correlates with its location along the spinal canal, with increasing deficits occurring with more rostral lesions. The neurologic deficits are due to abnormal in utero development throughout the entire CNS. Postnatal closure of the myelomeningocele in the term infant is performed within a few days of birth to minimize the risk of meningitis, and is associated with low morbidity. Prenatal fetal closure may be an option for a very select group of patients and is associated with complications, including preterm delivery.


Prenatal folate supplementation has markedly decreased both the incidence of infants born with myelomeningocele and lesion severity. Infants with sacral and low lumbar lesions often achieve some degree of ambulation, and approximately 80% can achieve social bladder and bowel continence. Approximately half of infants with a lumbar or sacral myelomeningocele will develop hydrocephalus that requires surgical treatment (see Plate 1-7). Most of these infants will have an associated Chiari II malformation, with displacement of the cerebellar vermis into the cervical canal, but only a few percent will become symptomatic at any point. Children are at risk for developing the tethered cord syndrome as the myelomeningocele scar adheres to the repair site while the spine grows. All repaired myelomeningoceles will appear adherent to some degree on MRI, and the diagnosis of a tethered cord in this population is made clinically. Although multidisciplinary care is needed throughout the life span of children born with a myelomeningocele, many will become independent productive adults with a good quality of life.


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Sep 2, 2016 | Posted by in NEUROLOGY | Comments Off on Spinal Dysraphism (Continued)

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