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Craniocervical instability
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=== Surgery === If non-invasive treatments for CCI fail to work, occipito-cervical fusion (OCF) can be considered.<ref name=":0" /> OCF is a surgery that aims at a biomechanical stabilization of the craniocervical junction. Patients with objective radiological findings, a clinical picture supportive of the diagnosis, a positive response to traction, and who are significantly impaired may be candidates for this surgery. A common method involves internal fixation of the upper spine to the skull by mechanical rods and screws. (However, rod-wire, rigid rod-screws, occipital hooks and cervical claws are all methods currently in use.<ref name=":16">{{Cite journal | last = Resnick|first = Daniel K. | last2 = Patel | first2 = Nirav J. | last3 = Lall | first3 = Rishi | date = 2010-11-01 | title = A Review of Complications Associated With Craniocervical Fusion Surgery | url = https://academic.oup.com/neurosurgery/article/67/5/1396/2563905|journal=Neurosurgery|language=en|volume=67|issue=5 | pages = 1396–1403|doi=10.1227/NEU.0b013e3181f1ec73|issn=0148-396X}}</ref>) During surgery, titanium hardware is used to fixate the occiput, axis and atlas (i.e., C0 to C2) while rib graft, cadaver bone graft or synthetic bone is used to help the bones fuse together. Wire methods are less biomechanically stable than rod methods and have high rates of dural laceration.<ref name=":16" /> Screw and rod fixation methods have lower complication rates and higher rates of successful fusion.<ref name=":18" /> Fusion rates across all hardware methods range from 89 to 100%.<ref name=":16" /> When cervical instability is present below C2, additional vertebrae may also be fused. ==== Outcomes, risks & complications ==== Little research on outcomes exists. In a small case study of 20 patients, the five-year outcome of OCF was generally favorable with most patients experiencing symptom relief post-surgery.<ref name=":0" /> In this study, following 20 EDS patients five years free O-2 fusion, most reported they were satisfied with the surgery and experienced significant improvements in symptoms such as vertigo, headaches, imbalance, dysarthria, dizziness, and frequent daytime urination. There was, however, only a small increase in objective outcomes such as work resumption, with 60% of patients remaining unable to work or go to school. Participants attributed this to other EDS comorbidities such as POTS, Mast Cell Activation Syndrome, and additional spinal problems.<ref name=":0" /> The complications of OCF can be serious<ref>{{Cite journal | last = Garrido|first = Ben J. | last2 = Sasso | first2 = Rick C. | date = Jan 2012 | title = Occipitocervical fusion | url =https://www.ncbi.nlm.nih.gov/pubmed/22082624|journal=The Orthopedic Clinics of North America|volume=43|issue=1 | pages = 1–9, vii|doi=10.1016/j.ocl.2011.08.009|issn=1558-1373|pmid=22082624}}</ref> and occur in an estimated 7% to 33% of patients.<ref name=":10" /><ref name=":18">{{Cite journal | last = Winegar | first = Corbett D. | last2 = Lawrence | first2 = James P. | last3 = Friel | first3 = Brian C. | last4 = Fernandez | first4 = Carmella | last5 = Hong | first5 = Joseph | last6 = Maltenfort | first6 = Mitchell | last7 = Anderson | first7 = Paul A. | last8 = Vaccaro | first8 = Alexander R. | date = Jul 2010 | title = A systematic review of occipital cervical fusion: techniques and outcomes |url =https://www.ncbi.nlm.nih.gov/pubmed/20594011|journal=Journal of Neurosurgery. Spine|volume=13|issue=1 | pages = 5–16|doi=10.3171/2010.3.SPINE08143|issn=1547-5646|pmid=20594011}}</ref><ref name=":11" /><ref>{{Cite journal | last = Ando|first = Kei | last2 = Imagama | first2 = Shiro | last3 = Ito | first3 = Zenya | last4 = Kobayashi | first4 = Kazuyoshi | last5 = Yagi | first5 = Hideki | last6 = Shinjo | first6 = Ryuichi | last7 = Hida | first7 = Tetsuro | last8 = Ito | first8 = Kenyu | last9 = Ishikawa | first9 = Yoshimoto | date = Jun 2017 | title = Minimum 5-year Follow-up Results for Occipitocervical Fusion Using the Screw-Rod System in Craniocervical Instability | url = https://www.ncbi.nlm.nih.gov/pubmed/28525489|journal=Clinical Spine Surgery|volume=30|issue=5| pages = E628–E632|doi=10.1097/BSD.0000000000000199|issn=2380-0194|pmid=28525489}}</ref><ref name=":16" /> Common complications include screw failure, wound infection, dural tear and cerebrospinal fluid leakage<ref name=":11" /> In some cases revision surgery is needed to treat infection or to remove hardware. Severe complications can include meningitis and accidental injury of the vertebral artery by misplaced screws.<ref name=":17">{{Cite journal | last = Nockels | first = Russ P. | last2 = Shaffrey | first2 = Christopher I. | last3 = Kanter | first3 = Adam S. | last4 = Azeem | first4 = Syed | last5 = York | first5 = Julie E. | date = Aug 2007 | title = Occipitocervical fusion with rigid internal fixation: long-term follow-up data in 69 patients |url =https://www.ncbi.nlm.nih.gov/pubmed/17688049|journal=Journal of Neurosurgery. Spine|volume=7|issue=2 | pages = 117–123|doi=10.3171/SPI-07/08/117|issn=1547-5654|pmid=17688049}}</ref> A meta-study of 2274 procedures across 22 studies<ref name=":16" /> found the following complication rates: {| class="wikitable" !Complication type !Prevalence rate |- |Hardware failure after fusion non-union |7% |- |Wound infection |3.8%-11% |- |Vertebral artery damage |1.3%-4.1% |- |Dural tears |0% to 4.2% |} Meta-studies place the rate of death from fusion surgery at 0-0.6%.<ref name=":16" /><ref name=":18" /> ==== Side effects ==== OCF causes a substantial reduction in the neck’s range of motion, estimated at approximately 40% of total cervical flexion–extension.<ref name=":8">{{Cite book | last = Ashafai|first = Nabeel S. | last2 = Visocchi | first2 = Massimiliano | last3 = Wąsik | first3 = Norbert | date = 2019 | editor-last = Visocchi|editor-first = Massimiliano | title = Occipitocervical Fusion: An Updated Review | url =https://doi.org/10.1007/978-3-319-62515-7_35|series=Acta Neurochirurgica Supplement|language=en|location=Cham| publisher = Springer International Publishing | pages = 247–252|doi=10.1007/978-3-319-62515-7_35|isbn=9783319625157}}</ref> ==== Cost ==== OCF is estimated to cost tens of thousands of dollars, although some insurance schemes fully cover the cost of surgery depending on the country located and neurosurgeons involved.
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