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Management of Chiari I malformations with holocord syringohydromyelia
1Department of Neurological Surgery, The Neurological Institute of New York, Columbia University College of Physicians and Surgeons, New York 10032, USA. naf6@columbia.edu
Insights
Suboccipital decompression alone effectively manages Chiari malformation Type I with syringohydromyelia in children. This surgical approach shows significant radiographic and clinical improvements, avoiding further interventions.
Area of Science:
- Neurosurgery
- Pediatric Neurology
- Radiology
Background:
- Management of Chiari malformation Type I (CM 1) with syringohydromyelia (SHM) is controversial, with various surgical techniques proposed.
- Holocord syringomyelia associated with CM 1 presents unique treatment challenges.
Observation:
- Seven pediatric patients with CM 1 and holocord SHM were treated with suboccipital decompression and duraplasty without intradural procedures.
- Postoperative MRI and clinical follow-up were conducted at regular intervals.
Findings:
- Marked reduction in syringomyelia was observed in 6 of 7 children on early postoperative MRI.
- At 1-year follow-up, syringomyelia remained collapsed in most patients, with significant size reduction in others.
- All patients demonstrated clinical improvement, with no need for further neurosurgical intervention during a mean 30-month follow-up.
Implications:
- Posterior fossa decompression alone appears to be a safe and effective treatment for CM 1 with holocord SHM in pediatric patients.
- This approach offers excellent radiographic and clinical outcomes, potentially simplifying surgical management.
- Further studies can validate these findings and refine surgical protocols for CM 1 and SHM.
Abstract:
The management of patients with Type I Chiari malformations (CM 1) with or without syringohydromyelia (SHM) has remained quite controversial, and many different surgical procedures have been advocated. Over the past several years, the authors have treated 7 children presenting with CM 1 and holocord syringohydromyelia with suboccipital decompression and duraplasty alone without intradural procedures. All children received MRI imaging at 2-4 months and 1 year postoperatively. On the early postoperative MRI examination, marked reduction in the syringohydromyelia was seen in 6 children, with minimal change in syrinx size in 1 child who was clinically improving after the operation. At 1 year, all children with early collapse remained collapsed, and the child with minimal early collapse demonstrated an approximately 50% reduction in syrinx size. Clinical follow-up (mean 30 months, range 21-50 months) showed good results in all patients: none of the children have required further neurosurgical intervention, and all have shown improvement in their preoperative function. One child with a 46 degrees scoliosis had a complete collapse of her SHM, but ultimately required spinal fusion. The presenting clinical findings, operative technique, MRI imaging and clinical outcomes will be discussed. The results from these 7 patients with CM 1 and holocord syringomyelia suggest that posterior fossa decompression alone (without intradural procedures) can provide excellent radiographic and clinical outcome.