Craniocervical Stabilization after Failed Chiari Decompression: A Case Series of a Population with High Prevalence of Ehlers-Danlos Syndrome. Craniocervical Stabilization After Failed Chiari Decompression: A Case Series of a Population with High Prevalence of Ehlers-Danlos Syndrome.Citation: World Neurosurgery. 161:e546-e552, 2022 May.PMID: 35192974Department: MedStar Georgetown University Hospital/MedStar Washington Hospital Center | Neurosurgery ResidencyForm of publication: Journal ArticleMedline article type(s): Journal ArticleSubject headings: *Arnold-Chiari Malformation | *Ehlers-Danlos Syndrome | Arnold-Chiari Malformation/dg [Diagnostic Imaging] | Arnold-Chiari Malformation/ep [Epidemiology] | Arnold-Chiari Malformation/su [Surgery] | Decompression | Ehlers-Danlos Syndrome/co [Complications] | Ehlers-Danlos Syndrome/ep [Epidemiology] | Ehlers-Danlos Syndrome/su [Surgery] | Humans | Prevalence | ResearchYear: 2022ISSN:
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BACKGROUND: In the treatment of Chiari malformation Type I (CM-I), posterior fossa decompression is achieved via suboccipital craniectomy (SOC); however, some patients continue to experience symptoms after treatment which may be due to craniocervical instability (CCI). The purposes of this study were to analyze data from patients who required an occipitocervical fusion (OCF) for the management of CCI after having previously undergone SOC for CM-I to determine if OCF is a safe and effective option and to determine any identifiable risk factors for CCI in these patients.
CONCLUSIONS: Symptomatic CCI should be recognized as a delayed postoperative complication in the surgical treatment of CM-I, with an underlying connective tissue hypermobility disorder such as EDS serving as a potential risk factor its development. CCI can be managed with OCF as a safe and effective treatment option for this patient population. Copyright ♭ 2022 Elsevier Inc. All rights reserved.
METHODS: A retrospective review was done on all patients who underwent an occipitocervical fusion (OCF) performed by the senior author between November 2013 and June 2020 after having previously undergone SOC for CM-I. Demographic, radiographic, perioperative, and outcome data were collected and clivoaxial angles (CXA) were measured pre- and post-operatively.
RESULTS: Fifteen patients were identified who developed symptomatic CCI after previously undergoing a suboccipital craniectomy for the treatment of CM-I. All 15 patients were treated by OCF with good outcome. Of these, 12 patients had a known diagnosis of Ehlers-Danlos Syndrome (EDS). Overall, the CXAs of these patients were found to be corrected to a more anatomical alignment.