Hydrocephalus, cervical cord lesions, and spinal deformity

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.