Browsing by Subject "Clivo-axial angle"

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    Craniocervical instability in patients with Ehlers-Danlos syndromes: outcomes analysis following occipito-cervical fusion
    (Springer, 2024-01-02) Henderson, Fraser C.; Schubart, Jane R.; Narayanan, Malini V.; Tuchman, Kelly; Mills, Susan E.; Poppe, Dorothy J.; Koby, Myles B.; Rowe, Peter C.; Francomano, Clair A.; Medical and Molecular Genetics, School of Medicine
    Craniocervical instability (CCI) is increasingly recognized in hereditary disorders of connective tissue and in some patients following suboccipital decompression for Chiari malformation (CMI) or low-lying cerebellar tonsils (LLCT). CCI is characterized by severe headache and neck pain, cervical medullary syndrome, lower cranial nerve deficits, myelopathy, and radiological metrics, for which occipital cervical fusion (OCF) has been advocated. We conducted a retrospective analysis of patients with CCI and Ehlers-Danlos syndrome (EDS) to determine whether the surgical outcomes supported the criteria by which patients were selected for OCF. Fifty-three consecutive subjects diagnosed with EDS, who presented with severe head and neck pain, lower cranial nerve deficits, cervical medullary syndrome, myelopathy, and radiologic findings of CCI, underwent open reduction, stabilization, and OCF. Thirty-two of these patients underwent suboccipital decompression for obstruction of cerebral spinal fluid flow. Questionnaire data and clinical findings were abstracted by a research nurse. Follow-up questionnaires were administered at 5-28 months (mean 15.1). The study group demonstrated significant improvement in headache and neck pain (p < 0.001), decreased use of pain medication (p < 0.0001), and improved Karnofsky Performance Status score (p < 0.001). Statistically significant improvement was also demonstrated for nausea, syncope (p < 0.001), speech difficulties, concentration, vertigo, dizziness, numbness, arm weakness, and fatigue (p = 0.001). The mental fatigue score and orthostatic grading score were improved (p < 0.01). There was no difference in pain improvement between patients with CMI/LLCT and those without. This outcomes analysis of patients with disabling CCI in the setting of EDS demonstrated significant benefits of OCF. The results support the reasonableness of the selection criteria for OCF. We advocate for a multi-center, prospective clinical trial of OCF in this population.
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    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
    (Wolters Kluwer, 2021-01-13) CreveCoeur, Travis S.; Yahanda, Alexander T.; Maher, Cormac O.; Johnson, Gabrielle W.; Ackerman, Laurie L.; Adelson, P. David; Ahmed, Raheel; Albert, Gregory W.; Aldana, Phillipp R.; Alden, Tord D.; Anderson, Richard C.E.; Baird, Lissa; Bauer, David F.; Bierbrauer, Karin S.; Brockmeyer, Douglas L.; Chern, Joshua J.; Couture, Daniel E.; Daniels, David J.; Dauser, Robert C.; Durham, Susan R.; Ellenbogen, Richard G.; Eskandari, Ramin; Fuchs, Herbert E.; George, Timothy M.; Grant, Gerald A.; Graupman, Patrick C.; Greene, Stephanie; Greenfield, Jeffrey P.; Gross, Naina L.; Guillaume, Daniel J.; Haller, Gabe; Hankinson, Todd C.; Heuer, Gregory G.; Iantosca, Mark; Iskandar, Bermans J.; Jackson, Eric M.; Jea, Andrew H.; Johnston, James M.; Keating, Robert F.; Kelly, Michael P.; Khan, Nickalus; Krieger, Mark D.; Leonard, Jeffrey R.; Mangano, Francesco T.; Mapstone, Timothy B.; McComb, J. Gordon; Menezes, Arnold H.; Muhlbauer, Michael; Oakes, W. Jerry; Olavarria, Greg; O’Neill, Brent R.; Park, Tae Sung; Ragheb, John; Selden, Nathan R.; Shah, Manish N.; Shannon, Chevis; Shimony, Joshua S.; Smith, Jodi; Smyth, Matthew D.; Stone, Scellig S.D.; Strahle, Jennifer M.; Tamber, Mandeep S.; Torner, James C.; Tuite, Gerald F.; Wait, Scott D.; Wellons, John C., III.; Whitehead, William E.; Limbrick, David D., Jr.; Neurological Surgery, School of Medicine
    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.