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Hydrocephalus, cervical cord lesions, and spinal deformity
Insights
This study links spinal deformities in children to central nervous system lesions like hydrocephalus and Arnold-Chiari malformation. These conditions may cause spinal issues by affecting muscle control.
Area of Science:
- Pediatric Neurology
- Neurosurgery
- Developmental Biology
Background:
- Spinal deformities, including scoliosis and kyphosis, frequently coexist with central nervous system (CNS) lesions in children.
- Conditions such as hydrocephalus and Arnold-Chiari malformation are often observed in these pediatric cases.
Purpose of the Study:
- To investigate the frequent coexistence of spinal deformity and CNS lesions in children.
- To explore the relationship between Arnold-Chiari malformation, cord cavitation (syringomyelia), and spinal deformities.
- To support theories on the developmental origin of Arnold-Chiari malformation and syringomyelia propagation.
Main Methods:
- Retrospective analysis of 26 children (4-16 years) with hydrocephalus and spinal deformity.
- Utilized metrizamide-enhanced cord CT scans and head scans for 21 and 12 children, respectively.
- Included intraoperative studies of fluid pressure transmission and intracranial pressure monitoring during spinal surgery.
Main Results:
- All children had Type I and II Arnold-Chiari malformations; 58% had cord cavitation (syringo/hydromyelia), and 67% had cord tethering.
- Anatomic studies revealed fibrosis/scarring of the upper cord/brain stem.
- Intraoperative findings demonstrated free fluid pressure wave transmission from the cord to the foramen magnum, causing increased intracranial pressure.
Conclusions:
- The study documents a frequent association between spinal deformity and CNS lesions (hydrocephalus, Arnold-Chiari malformation, cord tethering, cord cavitation).
- Findings support Arnold-Chiari malformation as a primary developmental issue and syringomyelia as a result of ischemic necrosis.
- CNS lesions likely cause spinal deformity by disrupting postural reflex mechanisms controlling spinal musculature.
Abstract:
Twenty-six children (4-16 years) had hydrocephalus and spinal deformity; 22 children had scoliosis over 50 degrees, and five children had kyphosis over 100 degrees. Twenty-one children had metrizamide enhanced cord CT scans, and 12 had additional head scans. Two children died, and one was autopsied. Three children had intracranial pressures monitored during spinal surgery. All children had Type I and II Arnold-Chiari malformations, 58% had cord cavitation (syringo/hydromyelia), 67% had cord tethering. The anatomic study showed extensive fibrosis and scarring of the upper cord and brain stem, but intraoperative studies showed that there is free transmission of fluid pressure wave from the cord across the foramen magnum with a consequent rise in intracranial pressure. This study documents frequent coexistence of spinal deformity and central nervous system lesions (hydrocephalus, Arnold-Chiari malformation, cord tethering with atresia, and cord cavitation). It supports the theory that the Arnold-Chiari malformation is a primary developmental deformity and that cord cavitation noted in these patients is primarily syringomyelia formed on the basis of ischemic necrosis within the cord. The free transmission of a fluid pressure wave from the cord to the lateral ventricle supports the Williams theory of propagation of syringomyelic cavities once they have developed. It is conjectural but likely that the CNS lesions cause spinal deformity by disrupting coordinating control of spinal musculature by the normal postural reflex mechanisms. Hydrocephalic patients who develop spinal deformity require thorough investigation for CNS lesions with head scans, CT scans, and metrizamide enhanced CT scans. NMR technology, however, may supplant techniques currently employed.