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Treatment of Chiari I malformation in patients with and without syringomyelia: a consecutive series of 66 cases.
Neurosurg Focus. 2001 Jul 15; 11(1):E3.NF

Abstract

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.

Authors+Show Affiliations

Institute for Neurology and Neurosurgery, Singer Division, Department of Pediatric Neurosurgery, Beth Israel Medical Center, New York, New York 10128, USA.No affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Clinical Trial
Comparative Study
Journal Article

Language

eng

PubMed ID

16724813

Citation

Alzate, J C., et al. "Treatment of Chiari I Malformation in Patients With and Without Syringomyelia: a Consecutive Series of 66 Cases." Neurosurgical Focus, vol. 11, no. 1, 2001, pp. E3.
Alzate JC, Kothbauer KF, Jallo GI, et al. Treatment of Chiari I malformation in patients with and without syringomyelia: a consecutive series of 66 cases. Neurosurg Focus. 2001;11(1):E3.
Alzate, J. C., Kothbauer, K. F., Jallo, G. I., & Epstein, F. J. (2001). Treatment of Chiari I malformation in patients with and without syringomyelia: a consecutive series of 66 cases. Neurosurgical Focus, 11(1), E3.
Alzate JC, et al. Treatment of Chiari I Malformation in Patients With and Without Syringomyelia: a Consecutive Series of 66 Cases. Neurosurg Focus. 2001 Jul 15;11(1):E3. PubMed PMID: 16724813.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Treatment of Chiari I malformation in patients with and without syringomyelia: a consecutive series of 66 cases. AU - Alzate,J C, AU - Kothbauer,K F, AU - Jallo,G I, AU - Epstein,F J, Y1 - 2001/07/15/ PY - 2006/5/27/pubmed PY - 2006/7/13/medline PY - 2006/5/27/entrez SP - E3 EP - E3 JF - Neurosurgical focus JO - Neurosurg Focus VL - 11 IS - 1 N2 - 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. SN - 1092-0684 UR - https://www.unboundmedicine.com/medline/citation/16724813/Treatment_of_Chiari_I_malformation_in_patients_with_and_without_syringomyelia:_a_consecutive_series_of_66_cases_ L2 - https://thejns.org/doi/10.3171/foc.2001.11.1.4 DB - PRIME DP - Unbound Medicine ER -