Abstract
Myelomeningocele is a congenital pathologic condition characterized by a defect in the posterior vertebral elements, a malformation in the underlying neural elements, a related neurologic deficit, and the related multi-system consequences of the resulting paralysis. Spinal deformity of some degree will occur in most involved individuals, but unlike the deformities in most other neuromuscular diseases, its cause is variable and, in some cases, progression preventable. The spinal deformity may be congenital, or developmental due to paralysis or bony deficiency. It may also be secondary to associated abnormalities that include Arnold–Chiari malformation, hydrocephalus, spinal cord tethering, and syringomyelia. Most children with myelomeningocele will have some form of spinal cord tethering and current data supports an aggressive surgical approach to this condition when it is symptomatic. Additional unique features include frequent allergy to latex, precocious puberty, and the apparent increased incidence in mothers with folate deficiency.
Surgery is the definitive treatment of spinal deformity that either is or is predicted to become symptomatic. The procedures have a significant risk due to the anatomic abnormalities of the spine and the orthopedic, neurologic, and medical comorbidities. The preoperative evaluation must address the associated problems that may adversely affect outcomes such as urinary tract infections, poor nutrition, untreated hydrocephalus, symptomatic spinal cord tethering, and scarred or compromised skin and soft tissues. Segmental spinal instrumentation represents a significant technical improvement. It permits stable fixation of the dysplastic posterior elements, improved deformity correction, and earlier mobilization. Growth preserving technology offer another option for the treatment of significant early onset spinal deformity. Techniques that avoid midline approaches seem particularly suitable to this population.
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Karlin, L.I. (2016). Myelomeningocele. In: Akbarnia, B., Yazici, M., Thompson, G. (eds) The Growing Spine. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-48284-1_14
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