Surgical Screening Protocol for Craniocervical Instability Secondary to Ehlers-Danlos Syndrome and other Connective Tissue Disorders: Analysis of a 347 Patient Case Series

Background Evaluation of craniocervical instability (CCI) in individuals with connective tissue disorders (CTDs) remains controversial. We present a surgical screening protocol created and implemented at our institution for these individuals. Methods We reviewed patients referred to our institution for CCI secondary to CTDs, between May 2018 and April 2022 using our two-stage protocol. Stage 1 included: history, clinical questionnaire, physical examination, non-invasive provocative testing, neuroimaging with morphometric analysis, Karnofsky Performance Scale. Items 1–5 were each scored on a scale from 0 to 2. Individuals with a KPS ≤ 70 and an aggregate score ≥6 were recommended for Stage 2, which included additional neuroimaging, psychiatric evaluation, and a trial of intraoperative craniocervical traction (ICT) with clinical and morphometric scores. Individuals meeting criteria in both stages were considered surgical candidates. Postoperative outcomes were assessed. Results Of the 347 individuals who entered Stage 1, 190 progressed through Stage 2. Following advanced evaluation, 115 patients met full surgical qualification criteria, with 95 proceeding to craniocervical fusion (CCF), and 86.4% reporting satisfaction on the NASS satisfaction index. An additional 12 patients were offered surgery due to marked clinical improvement during ICT despite borderline morphometric findings, with 70% reporting satisfaction on the NASS index postoperatively. Conclusions We present a two-stage surgical evaluation protocol for CCI secondary to CTDs. ICT served as a critical diagnostic and prognostic tool, clarifying the biomechanical contribution of CCI to symptomatology and aiding in the identification of appropriate surgical candidates. Further multicenter studies are warranted to validate this approach.