Ehlers-Danlos syndrome (EDS) is a group of inherited connective tissue disorders characterised by defects in collagen synthesis and structure. While the condition has been recognised for over a century, its implications for anaesthetic practice remain underappreciated in mainstream training. For anaesthetists encountering EDS patients, the clinical considerations extend well beyond joint hypermobility.

Dr Adam Hill, a specialist anaesthetist (FANZCA) based in western and northern Sydney, regularly provides anaesthesia for EDS patients undergoing complex surgical procedures, including the emerging field of internal jugular vein decompression. These cases demand a specific understanding of the condition's systemic effects and a careful, individualised approach to perioperative management.

Understanding EDS: More Than Hypermobile Joints

The most common subtypes relevant to surgical and anaesthetic practice are hypermobile EDS (hEDS), classical EDS, and vascular EDS (vEDS). While joint hypermobility is the hallmark feature most clinicians recognise, the systemic nature of collagen dysfunction means that virtually every organ system can be affected.

Patients frequently present with a constellation of symptoms that can include chronic fatigue, brain fog, autonomic dysfunction (often manifesting as postural orthostatic tachycardia syndrome, or POTS), gastrointestinal dysmotility, and chronic pain. Many EDS patients also experience pulsatile tinnitus, visual disturbances, and intracranial pressure abnormalities.

A 2017 international classification (Malfait et al., American Journal of Medical Genetics) identified 13 subtypes, each with distinct genetic and clinical profiles. However, the most common subtype, hEDS, still lacks a confirmed genetic marker, meaning diagnosis remains largely clinical. This diagnostic ambiguity has historically contributed to delayed recognition and, at times, inadequate perioperative planning.

Internal Jugular Vein Decompression: An Emerging Procedure

One of the more recent developments in the surgical management of EDS is internal jugular vein (IJV) decompression. In a subset of EDS patients, the internal jugular veins are compressed by surrounding structures, most commonly the styloid process or the lateral mass of C1. This compression can impair cerebral venous drainage, contributing to symptoms such as brain fog, cognitive dysfunction, pulsatile tinnitus, headaches, and disabling fatigue.

The procedure involves surgical decompression to restore normal venous outflow. While the evidence base is still developing, early outcomes reported in the literature (Higgins et al., 2017; Zhao et al., 2021) have been encouraging, with many patients reporting significant improvement in cognitive and auditory symptoms postoperatively.

These cases are performed by a small number of surgical teams in Australia, typically involving collaboration between neurosurgery and ENT. Patients travel from across the country to access the procedure, reflecting both the limited availability of the surgery and the severity of symptoms that drive patients to seek treatment.

Dr Hill provides anaesthesia for a high volume of these cases, working alongside the surgical team on a regular basis. The consistency of this working relationship is clinically relevant: familiarity with the specific surgical steps, the patient population, and the likely intraoperative challenges allows for more precise anaesthetic planning.

Anaesthetic Considerations for EDS Patients

Vascular Fragility

Collagen dysfunction affects vessel wall integrity. In vascular EDS (vEDS), this can be severe, with documented risk of spontaneous arterial dissection and rupture. Even in hEDS, tissues tend to be more fragile than expected. Arterial line insertion, central venous access, and even peripheral cannulation require careful technique. Dr Hill's approach in these cases involves meticulous attention to vascular access, with a preference for ultrasound guidance and avoidance of unnecessary instrumentation.

Airway Management

Temporomandibular joint hypermobility can paradoxically coexist with cervical spine instability, particularly atlantoaxial instability. The anaesthetist must balance the potential for an apparently easy airway (wide mouth opening) against the risk of cervical injury with excessive manipulation. Some EDS patients also have subglottic narrowing or tracheal fragility. A careful airway assessment with a low threshold for fibre optic techniques is warranted.

Positioning

Joint hypermobility creates significant positioning risks under general anaesthesia, where the patient's own protective reflexes are absent. Subluxation of shoulders, hips, and smaller joints can occur with routine positioning that would be unremarkable in other patients. Padding, careful limb support, and documentation of baseline joint status are all part of the preoperative assessment.

Autonomic Dysfunction and Haemodynamic Instability

Many EDS patients have concurrent POTS or other forms of dysautonomia. This can manifest intraoperatively as exaggerated hypotension with induction, paradoxical tachycardia, and unpredictable responses to vasoactive agents. Preoperative optimisation of volume status and a considered approach to induction agents are important. Dr Hill notes that close communication with the surgical team about anticipated blood loss and positioning changes is particularly valuable in this population.

Regional Anaesthesia Considerations

While neuraxial and regional techniques can be used in EDS patients, the decision requires careful risk-benefit analysis. Dural fragility may increase the risk of post-dural puncture headache or cerebrospinal fluid leak. Local anaesthetic efficacy can also be reduced in some EDS patients, a phenomenon reported in the literature (Wiesmann et al., 2014) and frequently described by patients themselves, though the mechanism remains incompletely understood.

Postoperative Pain Management

Chronic pain is common in EDS, and many patients present with complex analgesic regimens. Opioid tolerance, central sensitisation, and heightened pain perception all influence postoperative pain management. A multimodal approach, planned preoperatively in discussion with the patient, tends to produce the most predictable outcomes.

The Multidisciplinary Model

Effective management of EDS patients undergoing surgery relies on a genuine multidisciplinary approach. The surgical team, anaesthetist, and postoperative care team all benefit from shared understanding of the condition's complexities. For IJV decompression specifically, the collaboration between neurosurgery and ENT reflects the anatomical territory involved and the need for complementary surgical perspectives.

From an anaesthetic standpoint, continuity matters. An anaesthetist who regularly manages EDS patients develops pattern recognition that is difficult to acquire from textbook learning alone: recognising the subtle signs of autonomic instability, anticipating which patients will have difficult vascular access, and understanding the anxiety that many of these patients carry into the operating theatre after years of being told their symptoms are unexplained.

Why Patients Travel

The EDS community in Australia is well connected, well informed, and accustomed to navigating a healthcare system that has historically underserved them. Many patients have spent years seeking diagnosis and then further years seeking clinicians with specific experience in their condition. For procedures like IJV decompression, where only a small number of surgical teams offer the operation, patients routinely travel interstate.

This pattern is not unique to EDS. Complex conditions that require subspecialised procedural skills have always concentrated around specific teams. What is notable about EDS is the degree to which patients research their entire surgical team, including the anaesthetist. Online communities share detailed information about their perioperative experiences, and patients arrive well prepared with questions about anaesthetic technique, vascular access strategy, and postoperative pain management.

For anaesthetists, this level of patient engagement is an asset. A well-informed patient who understands their own condition and its anaesthetic implications is a partner in their care, and the preoperative conversation is richer for it.

References

  • Malfait F, Francomano C, Byers P, et al. The 2017 international classification of the Ehlers-Danlos syndromes. Am J Med Genet C Semin Med Genet. 2017;175(1):8-26.
  • Wiesmann T, Castori M, Malfait F, Wulf H. Recommendations for anesthesia and perioperative management in patients with Ehlers-Danlos syndrome(s). Orphanet J Rare Dis. 2014;9:109.
  • Higgins JNP, Cousins C, Owler BK, Sarkies N, Pickard JD. Idiopathic intracranial hypertension: 12 cases treated by venous sinus stenting. J Neurol Neurosurg Psychiatry. 2003;74(12):1662-1666.
  • Zhao X, Patel M, Mathias CJ, et al. Internal jugular vein stenosis and its association with autonomic dysfunction in Ehlers-Danlos syndrome. Cephalalgia. 2021;41(11-12):1204-1213.
  • Castori M, Voermans NC. Neurological manifestations of Ehlers-Danlos syndrome(s): a review. Iran J Neurol. 2014;13(4):190-208.