Anaesthesia for patients with non-vascular Ehlers-Danlos syndromes: recommendations based on a descriptive cohort study of 69 patients and 127 orthopaedic operations

Anaesthetists are generally well-aware of the serious complications that can occur when anaesthetising patients with vascular Ehlers-Danlos syndrome. They are usually less familiar with complications that can occur in patients with hypermobile EDS and other non-vascular EDS (nvEDS). This study aims to identify anaesthesia-related complications in a cohort of patients with nvEDS. In a retrospective, descriptive, single-centre cohort study, we recruited adult patients diagnosed with a nvEDS who had previously undergone orthopaedic surgery on a shoulder, elbow, wrist, knee or ankle. During video calls, patients were asked a series of standardised questions about their surgery and any complications. Main outcome measures were the number and type of anesthesia-related complications occurred during and after surgery. We recruited 69 patients with nvEDS, including 55 with hypermobile EDS, who had undergone a total of 127 orthopaedic operations. The mean (standard deviation) age at the time of surgery was 25.6 ± 11.1 years, and the interviews were conducted after a median delay of 175 months (inter-quartile range 86 to 297). Most of the operations were carried out under general anaesthesia alone (82/127; 64.6%), but some involved peripheral nerves blocks (33/127; 26.0%) or spinal anaesthesia (12/127; 9.5%). Most patients reported complications, which included an increase in temporomandibular problems (34/105; 32.4%), peripheral nerves block failure (16/33; 48.5%), and persistent pain at the surgical site (82/127; 64.6%). Dislocations during surgery (5/127; 3.9%) were also reported. Anaesthesia-related complications were found to be common in patients with nvEDS, but none were serious or life-threatening. Based on our findings and the available literature, we propose practical suggestions for the anaesthetic management in hypermobile EDS. Clinicaltrials.gov identifier: NCT05137379.