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 0 to 2 scale. 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 entering 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 reporting an 86.4% satisfaction rate on the North American Spine Society (NASS) satisfaction index. Twelve additional patients with borderline morphometric scores were offered surgery due to marked clinical improvement during ICT; however, this Subgroup demonstrated a lower NASS satisfaction rate (70%). Conclusions: We present a two-stage surgical evaluation protocol for CCI in patients with CTDs. ICT served as a critical diagnostic tool, clarifying the biomechanical contribution of CCI to symptomatology and aiding in the identification of appropriate surgical candidates. Further multicenter studies are warranted for validation.