Occipital-Cervical Fusion and Ventral Decompression in the Surgical Management of Chiari-1 Malformation and Syringomyelia: Analysis of Data From the Park-Reeves Syringomyelia Research Consortium

Travis S CreveCoeur, Alexander T Yahanda, Cormac O Maher, Gabrielle W Johnson, Laurie L Ackerman, P David Adelson, Raheel Ahmed, Gregory W Albert, Phillipp R Aldana, Tord D Alden, Richard C E Anderson, Lissa Baird, David F Bauer, Karin S Bierbrauer, Douglas L Brockmeyer, Joshua J Chern, Daniel E Couture, David J Daniels, Robert C Dauser, Susan R Durham, Richard G Ellenbogen, Ramin Eskandari, Herbert E Fuchs, Timothy M George, Gerald A Grant, Patrick C Graupman, Stephanie Greene, Jeffrey P Greenfield, Naina L Gross, Daniel J Guillaume, Gabe Haller, Todd C Hankinson, Gregory G Heuer, Mark Iantosca, Bermans J Iskandar, Eric M Jackson, Andrew H Jea, James M Johnston, Robert F Keating, Michael P Kelly, Nickalus Khan, Mark D Krieger, Jeffrey R Leonard, Francesco T Mangano, Timothy B Mapstone, J Gordon McComb, Arnold H Menezes, Michael Muhlbauer, W Jerry Oakes, Greg Olavarria, Brent R O'Neill, Tae Sung Park, John Ragheb, Nathan R Selden, Manish N Shah, Chevis Shannon, Joshua S Shimony, Jodi Smith, Matthew D Smyth, Scellig S D Stone, Jennifer M Strahle, Mandeep S Tamber, James C Torner, Gerald F Tuite, Scott D Wait, John C Wellons, William E Whitehead, David D Limbrick, Travis S CreveCoeur, Alexander T Yahanda, Cormac O Maher, Gabrielle W Johnson, Laurie L Ackerman, P David Adelson, Raheel Ahmed, Gregory W Albert, Phillipp R Aldana, Tord D Alden, Richard C E Anderson, Lissa Baird, David F Bauer, Karin S Bierbrauer, Douglas L Brockmeyer, Joshua J Chern, Daniel E Couture, David J Daniels, Robert C Dauser, Susan R Durham, Richard G Ellenbogen, Ramin Eskandari, Herbert E Fuchs, Timothy M George, Gerald A Grant, Patrick C Graupman, Stephanie Greene, Jeffrey P Greenfield, Naina L Gross, Daniel J Guillaume, Gabe Haller, Todd C Hankinson, Gregory G Heuer, Mark Iantosca, Bermans J Iskandar, Eric M Jackson, Andrew H Jea, James M Johnston, Robert F Keating, Michael P Kelly, Nickalus Khan, Mark D Krieger, Jeffrey R Leonard, Francesco T Mangano, Timothy B Mapstone, J Gordon McComb, Arnold H Menezes, Michael Muhlbauer, W Jerry Oakes, Greg Olavarria, Brent R O'Neill, Tae Sung Park, John Ragheb, Nathan R Selden, Manish N Shah, Chevis Shannon, Joshua S Shimony, Jodi Smith, Matthew D Smyth, Scellig S D Stone, Jennifer M Strahle, Mandeep S Tamber, James C Torner, Gerald F Tuite, Scott D Wait, John C Wellons, William E Whitehead, David D Limbrick

Abstract

Background: Occipital-cervical fusion (OCF) and ventral decompression (VD) may be used in the treatment of pediatric Chiari-1 malformation (CM-1) with syringomyelia (SM) as adjuncts to posterior fossa decompression (PFD) for complex craniovertebral junction pathology.

Objective: To examine factors influencing the use of OCF and OCF/VD in a multicenter cohort of pediatric CM-1 and SM subjects treated with PFD.

Methods: The Park-Reeves Syringomyelia Research Consortium registry was used to examine 637 subjects with cerebellar tonsillar ectopia ≥ 5 mm, syrinx diameter ≥ 3 mm, and at least 1 yr of follow-up after their index PFD. Comparisons were made between subjects who received PFD alone and those with PFD + OCF or PFD + OCF/VD.

Results: All 637 patients underwent PFD, 505 (79.2%) with and 132 (20.8%) without duraplasty. A total of 12 subjects went on to have OCF at some point in their management (PFD + OCF), whereas 4 had OCF and VD (PFD + OCF/VD). Of those with complete data, a history of platybasia (3/10, P = .011), Klippel-Feil (2/10, P = .015), and basilar invagination (3/12, P < .001) were increased within the OCF group, whereas only basilar invagination (1/4, P < .001) was increased in the OCF/VD group. Clivo-axial angle (CXA) was significantly lower for both OCF (128.8 ± 15.3°, P = .008) and OCF/VD (115.0 ± 11.6°, P = .025) groups when compared to PFD-only group (145.3 ± 12.7°). pB-C2 did not differ among groups.

Conclusion: Although PFD alone is adequate for treating the vast majority of CM-1/SM patients, OCF or OCF/VD may be occasionally utilized. Cranial base and spine pathologies and CXA may provide insight into the need for OCF and/or OCF/VD.

Keywords: Chiari malformation; Clivo-axial angle; Craniovertebral junction; Occipital-cervical fusion; Syringomyelia; Ventral brainstem compression; pB-C2.

Copyright © 2020 by the Congress of Neurological Surgeons.

Figures

Graphical Abstract
Graphical Abstract
FIGURE.
FIGURE.
Flowchart depicting the study cohort.

Source: PubMed

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