Treatment of Chiari type I malformation in patients with and without syringomyelia: a consecutive series of 66 cases

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  • Institute for Neurology and Neurosurgery, Department of Pediatric Neurosurgery, Beth Israel Medical Center, Singer Division, New York, New York
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Object

The authors describe the results of performing a standard posterior craniovertebral decompression and placement, if indicated, of a syringosubarachnoid shunt for the treatment of patients with Chiari I malformation with and without syringomyelia.

Methods

This is a retrospectively analyzed consecutive series of 66 patients (mean patient age 15 years, range 1–53 years). The uniform posterior craniovertebral decompression consisted of a small suboccipital craniectomy, a C-1 laminectomy, microsurgical reduction of the cerebellar tonsils, and dural closure with a synthetic dural graft to increase the cerebrospinal fluid space at the craniocervical junction. The presence of a large syrinx, with significant thinning of the spinal cord tissue and obliteration of the spinal subarachnoid space, particularly when combined with syrinx-related symptoms, was an indication for the placement of a syringosubarachnoid shunt.

In 32 patients Chiari I malformation alone was present, and 34 in patients it was present in combination with syringomyelia. Clinical findings included pain, neurological deficits, and spinal deformity. The presence of syringomyelia was significantly associated with the presence of scoliosis (odds ratio 74.4 [95% confidence interval 8.894–622.4]).

All patients underwent a posterior craniovertebral decompression procedure. In 22 of the 34 patients with syringomyelia a syringosubarachnoid shunt was also placed. The mean follow-up period was 24 months (range 3–95 months). Excellent outcome was achieved in 54 patients (82%) and good outcome in 12 (18%). In no patient were symptoms unchanged or worse at follow-up examination, including four patients who initially required a second operation for persistent syringomyelia. Pain was more likely to resolve than sensory and motor deficits after decompressive surgery.

Radiological examination revealed normalization of tonsillar position in all patients. The syrinx had disappeared in 15 cases, was decreased in size in 17, and remained unchanged in two.

Conclusions

Posterior craniovertebral decompression and selective placement of a syringosubarachnoid shunt in patients with Chiari I malformation and syringomyelia is an effective and safe treatment. Primary placement of a shunt in the presence of a sufficiently large syrinx appears to be beneficial. The question of if and when to place a shunt, however, requires further, preferably prospective, investigation.

Abbreviations used in this paper:

CSF = cerebrospinal fluid; MR = magnetic resonance.

Object

The authors describe the results of performing a standard posterior craniovertebral decompression and placement, if indicated, of a syringosubarachnoid shunt for the treatment of patients with Chiari I malformation with and without syringomyelia.

Methods

This is a retrospectively analyzed consecutive series of 66 patients (mean patient age 15 years, range 1–53 years). The uniform posterior craniovertebral decompression consisted of a small suboccipital craniectomy, a C-1 laminectomy, microsurgical reduction of the cerebellar tonsils, and dural closure with a synthetic dural graft to increase the cerebrospinal fluid space at the craniocervical junction. The presence of a large syrinx, with significant thinning of the spinal cord tissue and obliteration of the spinal subarachnoid space, particularly when combined with syrinx-related symptoms, was an indication for the placement of a syringosubarachnoid shunt.

In 32 patients Chiari I malformation alone was present, and 34 in patients it was present in combination with syringomyelia. Clinical findings included pain, neurological deficits, and spinal deformity. The presence of syringomyelia was significantly associated with the presence of scoliosis (odds ratio 74.4 [95% confidence interval 8.894–622.4]).

All patients underwent a posterior craniovertebral decompression procedure. In 22 of the 34 patients with syringomyelia a syringosubarachnoid shunt was also placed. The mean follow-up period was 24 months (range 3–95 months). Excellent outcome was achieved in 54 patients (82%) and good outcome in 12 (18%). In no patient were symptoms unchanged or worse at follow-up examination, including four patients who initially required a second operation for persistent syringomyelia. Pain was more likely to resolve than sensory and motor deficits after decompressive surgery.

Radiological examination revealed normalization of tonsillar position in all patients. The syrinx had disappeared in 15 cases, was decreased in size in 17, and remained unchanged in two.

Conclusions

Posterior craniovertebral decompression and selective placement of a syringosubarachnoid shunt in patients with Chiari I malformation and syringomyelia is an effective and safe treatment. Primary placement of a shunt in the presence of a sufficiently large syrinx appears to be beneficial. The question of if and when to place a shunt, however, requires further, preferably prospective, investigation.

Abbreviations used in this paper:

CSF = cerebrospinal fluid; MR = magnetic resonance.

Contributor Notes

Address reprint requests to: Karl F. Kothbauer, M.D., Pediatric Neurosurgery, Institute for Neurology and Neurosurgery, Beth Israel Medical Center, Singer Division, 170 East End Avenue, New York, New York 10128. email: kkothbau@bethisraelny.org.

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