Ehlers-Danlos syndromes and anaesthesia: what to tell your team before surgery

The NHS describes Ehlers-Danlos syndromes as a group of rare inherited conditions affecting connective tissue, with 13 types, most of them rare. Common features include hypermobile joints, stretchy skin and skin that breaks or bruises easily. Some of the rare types affect blood vessels and internal organs and can be life threatening.

Those features matter in an operating theatre. Joints that move too far can be strained by routine airway manoeuvres or positioning, fragile skin and tissue can be damaged by pressure and adhesive, and in the vascular type fragile blood vessels raise the stakes of any needle, line or surgical approach. The most useful single thing a patient can do is make sure the team knows which type of EDS they have and how it has affected them.

It is worth saying plainly that there is little UK patient-facing guidance on this topic. The detailed points below come from a published clinical review by European anaesthetists, summarised with the NHS general anaesthetic information, not from a UK national guideline, and the review itself notes that much of the evidence is based on case reports and clinical reasoning.

The NHS explains that a general anaesthetic is medicine that makes you asleep and unaware so you feel nothing during an operation. It is used when other types, such as local or regional anaesthetic or sedation, are not suitable. A pre-operative assessment, usually a few weeks before, makes sure you are well enough, may involve blood tests, an ECG or other checks and may include meeting the anaesthetist.

The NHS asks you to tell the doctor or nurse if you take any medicines, have any health conditions, have ever had an allergic reaction to a general anaesthetic or any other medicine, or have concerns about the procedure. During the anaesthetic an anaesthetist stays with you, monitors heart rate, blood pressure and oxygen levels and puts a tube into the throat to help you breathe. Side effects can include a sore throat or hoarse voice from the tube, nausea, confusion and soreness from lying still, and rare complications include damage to teeth or mouth, eyes or nerves.

For someone with EDS, several of those routine steps are exactly where the extra planning applies.

The review recommends that the preoperative assessment checks the specific EDS subtype, takes a thorough standardised bleeding history, asks about complications of EDS and assesses any difficulties with the airway. It emphasises that a detailed interview and clinical examination, and contact with the regional reference centre, are essential for estimating individual risk.

It also advises noting any history of ineffective local anaesthetics, for example during dental procedures, or failed blocks in the past. A Doppler ultrasound scan may be used to exclude cardiac problems in some types.

• Say which EDS or hypermobility diagnosis you have and who made it.

• Mention easy bruising, bleeding, slow wound healing or poor scarring from previous surgery.

• Describe any jaw, neck or other joints that dislocate easily.

• Tell them if local anaesthetic has not worked well at the dentist or elsewhere.

• Mention reactions to medicines, dressings or adhesives.

• Bring any letter from a genetics or EDS service, and ask whether a copy can go to the anaesthetic team.

According to the review, mask ventilation, intubation and laryngeal mask insertion are all generally possible, but care is needed with mask ventilation to avoid dislocation of the jaw joint. Difficult airways may occur in many forms of EDS because of jaw joint problems, early spinal wear or instability at the top of the neck, so difficulty should be anticipated more often than in the general population. Repeated intubation attempts may cause bleeding and smaller tubes may reduce damage to the lining, and fibreoptic intubation may be considered when difficulty is expected.

For positioning, the review advises padding to reduce shear forces and pressure, careful positioning and mobilisation to reduce joint dislocation, and avoiding careless positioning that could cause nerve problems such as brachial plexus neuropathy or pressure on the eye. It also suggests that the neurological and visual status can be documented before and after operations needing extreme positioning.

The review notes that because some people with EDS have muscle weakness, monitoring of neuromuscular blockade is advised before waking from anaesthesia.

Many people with EDS report that dental or other local anaesthetic wears off quickly or does not numb well. The review says ineffectiveness should be noted in the history, that tissue scarring might theoretically hamper the spread of local anaesthetic, and that topical local anaesthetics may be less effective. It recommends ultrasound guidance for regional techniques to reduce accidental vessel puncture and improve spread.

On technique, the review states that there is no clear recommendation for general versus regional or local anaesthesia, notes that peripheral regional anaesthesia may be useful in limb surgery given the frequency of difficult airways, and recommends avoiding spinal or epidural techniques in vascular EDS. For other types the decision is individual.

The NHS describes vascular EDS as a rare type often considered the most serious, with fragile blood vessels that can bulge or tear and risks to the bowel, the womb in late pregnancy and the lungs. For this reason surgery and procedures should only be undertaken after specialist discussion.

The review recommends ultrasound guidance for central venous catheters, advises discussing an open rather than a laparoscopic approach in vascular EDS because of bleeding risk and better options for controlling bleeding, and warns of upper airway bleeding with repeated intubation attempts and of pneumothorax with mechanical ventilation. If you have vascular EDS, an emergency care plan or letter from your specialist team is worth carrying, and the NHS notes that a specialist EDS diagnostic service exists in Sheffield and London.

After a general anaesthetic the NHS says effects can last around 24 hours, and advises having someone stay with you, not driving, cooking, drinking alcohol or making important decisions. It says to ask for an urgent GP appointment or get help from NHS 111 if you have recently had a general anaesthetic and are worried about side effects or they do not get better.

With EDS, also report new joint instability, a dislocation, wound opening, unusual bruising or swelling and new nerve symptoms such as numbness or weakness to the surgical team promptly, and use 999 or A&E for severe pain, sudden breathlessness or heavy bleeding.

Pre-operative assessment is part of NHS and private hospital pathways alike. Whichever you use, ask for the anaesthetist to be told about your EDS diagnosis in advance rather than on the day, and ask whether the unit has experience with connective tissue disorders. Dental work, which often uses local anaesthetic and a reclined position that strains the jaw, deserves the same advance conversation.