Surgery and General Anaesthesia With hEDS, POTS or MCAS: How to Prepare

🏥 A date comes through for an operation. Routine, they said. And you sit with the letter knowing that you will be unconscious for the part where it matters, that the team may never have met your condition before, and that the last three clinicians you saw had not heard of it.

📄 There is a published set of perioperative recommendations for Ehlers-Danlos syndromes. It exists, it is peer reviewed, and it is free to read. Putting it in front of the people who will be in the room is something you can do, four weeks early, from your sofa.

💚 This article is about preparation and conversation, not about technique. Every clinical decision below belongs to your anaesthetist. What belongs to you is arriving early, with the right paperwork, and the right paper.

⚠️ Start with the uncomfortable part: the evidence is thin

The main reference here is a 2014 review by Wiesmann, Castori, Malfait and Wulf in the Orphanet Journal of Rare Diseases. Before anything else, read what its authors say about their own work: their literature search did not identify any data with a high level of evidence concerning anaesthetic issues in EDS, and their recommendations rest on case reports, small series and expert opinion.

That is worth sitting with for a moment, because it cuts both ways.

It means nobody can hand your anaesthetist a protocol. They will be making judgement calls. Which is precisely why the information you bring has weight: you are not arguing against a guideline, you are filling a space where there is none. It also means that anyone online telling you with total confidence what must and must not be done is overstating what is known.

🧬 Your subtype decides most of this

This is the section that matters most, and the one most often skipped. The review is unambiguous: subtype classification should be obtained before surgery, and contacting the local or regional reference centre is essential.

A word on the labels first, because the review is from 2014 and you are reading this now. The classification in current use dates from 2017 and describes thirteen types, having updated the 1997 Villefranche nomenclature. The subtype names used below, hypermobile, classical, vascular, dermatosparaxis, kyphoscoliotic and brittle cornea syndrome, are all among those thirteen, so the review's labels still map onto a present-day diagnosis. The 2014 paper writes classical as "classic", which is the same subtype. Take the wording of your own diagnosis as the reference rather than guessing.

The reason subtype matters so much is that the recommendations diverge sharply.

Vascular EDS is a different conversation entirely. It is a distinct subtype with a distinct cause: most cases result from variants in the COL3A1 gene, whereas in most people with hypermobile EDS the genetic cause is unknown. So if your diagnosis does not say vascular EDS, this paragraph is not describing you. It is still worth knowing it exists, because it is the reason generic advice about EDS and surgery is unreliable.

For that subtype, the review states that vascular EDS is associated with spontaneous visceral ruptures, and that even elective vascular, gastrointestinal and other surgery should be avoided whenever possible. It recommends avoiding neuraxial blockade, does not recommend peripheral nerve blocks, and advises avoiding invasive blood pressure measurement and central venous access wherever possible in non-emergency surgery. If this is your subtype, nothing on a general blog substitutes for a reference centre.

For the hypermobile and classical types, the picture is less restrictive but has its own specifics. POTS is described as sometimes a feature in EDS, especially those with the hypermobile variant. Tarlov cysts are noted as a feature of the classical, hypermobile and kyphoscoliotic subtypes. And one figure applies across the board: about 90% of EDS patients, all subtypes, bruise easily.

If you are not certain which category you fall into, or whether you have a formal diagnosis at all, our guide to where the line between hypermobility and hEDS actually sits is the place to start, and it is worth starting well before a surgical date.

🩸 Bleeding: raise it even if you have never had a problem

Here is the single most useful thing in the paper for a patient, and it is counterintuitive.

The review advises taking a standardized bleeding history, and says that laboratory results are usually within normal range and mostly not helpful for estimating the bleeding risk. A normal clotting screen does not reassure anybody. The history does the work the tests cannot.

Which means the useful preparation is to write yours down before you are asked, from memory, calmly, at home. Dental extractions. Previous operations. Heavy periods. Nosebleeds. Bruises that arrive without a cause. How long small cuts take to stop. None of that will be found by a blood test, and all of it changes what gets planned.

What the review says may follow from a positive history, so that you recognise the vocabulary rather than request it: crossmatching adequate amounts of red cells is advised for patients at risk of bleeding; autologous donation should be discussed in elective surgery; cell saving might be advisable for major surgery; its summary table advises using desmopressin in patients with a positive bleeding history; prophylactic tranexamic acid before surgery should be contemplated. On tourniquets, the review puts the matter in the surgeon's court rather than banning them: it lists avoidance of tourniquets among the things to discuss with the surgeon, and says the anaesthetist must discuss the benefits and drawbacks with the surgeon if a tourniquet is contemplated, noting a high risk of haematoma and compartment syndromes.

On subtypes and bleeding, the review is careful, and so should we be: it says that in some classical and dermatosparaxis EDS, and in subtypes with increased vascular fragility, patients are prone to severe bleeding and haematoma formation. Some, not all.

🫁 The mechanical details, because you will be asleep for them

Most of what the review covers under airway and positioning comes down to one idea: the usual amount of force is the wrong amount of force.

On the airway, it notes that care is required with mask ventilation to avoid temporomandibular joint luxation, that smaller endotracheal tubes may reduce mucosal damage, that cuff pressure should be checked frequently and kept as low as possible, and that airway pressure should be minimised due to the risk of pneumothorax. Where difficulty is anticipated, elective fibreoptic intubation should be considered.

On positioning and handling: careful patient positioning and mobilisation is advised to reduce the risk of joint luxation. Padding should be used to reduce shear forces and external tissue pressure. The eye should be protected, since the review notes that direct force to the eyeball increases the risk of retinal detachment and globe rupture, and flags this as especially important in kyphoscoliotic EDS and brittle cornea syndromes. Tapes and dressings should be easily removable or avoided if possible. Patient handling must be performed with maximal reduction of shear forces of the skin. The review adds that pre-operative and post-operative written documentation of neurological and visual status might be helpful.

The patient-side version of all this is a short list nobody can produce but you. Which joints dislocate, in which direction, and what position they will not tolerate. Whether your jaw subluxes. Whether adhesive tape takes your skin off. Say it at the pre-op clinic and ask for it to go in the notes, because at the point it becomes relevant you will not be able to say anything.

💓 If you have POTS: fluids, and the 24 hours afterwards

The review addresses POTS directly, and briefly: preoperative infusion of crystalloid and early use of vasopressors may be helpful, and adequate postoperative patient monitoring is recommended.

Two more of its general recommendations matter especially here. All patients, even after minor surgery, should be monitored for at least 24 hours, a line that sits in its section on ambulatory anaesthesia. Its summary table separately lists avoiding ambulatory surgery among the things to discuss with the surgeon. And early mobilisation is described as essential to prevent loss of strength, alongside a recommendation for adequate prophylaxis of postoperative nausea and vomiting.

That 24 hour line is worth noticing, because it sits awkwardly with how minor surgery is actually run. Day case lists are built around going home the same afternoon. If an overnight observation is something you want discussed, the moment to raise it is when the operation is booked, not on the morning. It is a 2014 suggestion in a paper that admits its own evidence is thin, not an entitlement, so raise it as a question rather than treating a same-day discharge as a failure.

🔥 If you have MCAS as well

The clearest material we have found on the mast cell side is a 2025 slide set by Dr Anton Krige, published by Mast Cell Action, on considerations for anaesthesia and surgery.

Be clear about what it is before you lean on it. It is written for clinicians, not for patients. It is a slide deck rather than a paper, and it carries no citations on any slide. So the right use of it is as something to put in front of your anaesthetic team, not as evidence you quote at them.

These are the points in it that are yours to act on:

• Get referred to a pre-operative assessment clinic, with allergy or immunology advice as needed. Not the morning of.

• Bring a careful history of your MCAS symptoms and triggers, and keep what the deck calls an anaesthetic logbook. It does not define the term, but a dated record of what you were given at each procedure and how you reacted is the obvious reading.

• Ask about continuing your regular MCAS medications up to the day of surgery, which is what the slides advise, and which routinely surprises people who expect to be told to stop everything.

• Ask whether premedication is appropriate for you. The slides give suggested premedication regimes including H1 and H2 receptor antagonists twelve hours and one hour before the procedure. Whether any of that applies to you is a question for the clinic, not a request to make on arrival.

• Ask about the environment, not just the drugs. The slides mention maintaining a steady environmental temperature, minimising friction and mechanical trauma such as from tape and tourniquet use, considering preoperative intravenous hydration, keeping adrenaline immediately available, and extended post-operative monitoring. They also note that reactions are more common to chlorhexidine than to povidone iodine.

The deck also contains agent-by-agent comparisons of induction agents, muscle relaxants, opioids and antibiotics. Those are written for anaesthetists, and deliberately not reproduced here, because a patient arriving with a list of drugs to refuse is a worse outcome than a patient arriving with a document. Send them the link, ahead of the date, and let the specialists read it.

Separately, make sure your own reaction plan is current and that the team has seen it. Our guide to building an MCAS emergency plan covers what that document should contain.

💉 Epidurals, spinals and nerve blocks

This is the commonest specific question, and the review answers it by subtype rather than by yes or no.

Before any neuraxial anaesthesia, it advises that scoliosis, previous spinal surgery and other spine pathology should be ruled out. For subtypes other than vascular EDS, neuraxial blockades are described as feasible, and peripheral nerve blocks can be advised on a case-by-case shared decision making basis, with ultrasound guidance recommended. For vascular EDS, as above, the review recommends avoiding both.

One thing worth knowing in advance rather than discovering afterwards: the review notes that a greater rate of post-dural puncture headache after neuraxial blockade might be expected, and adds immediately that there are no relevant studies. An expectation, then, not a finding.

If your question is about local anaesthetic not working properly at the dentist, that is a related but distinct problem, and we have covered it separately in hEDS and your teeth.

✅ Five things to settle before you are in a gown

1. Your subtype, in writing. The review says it should be obtained before surgery. If you do not have it, that is the first phone call, not the last.

2. Your bleeding history, written out. Because the tests will not find it and the appointment is too short to remember it.

3. Contact with a reference centre. The review calls this essential, and it is the step that carries the most weight with a cautious anaesthetist.

4. A pre-operative assessment appointment, booked early enough that something can still change as a result of it.

5. Your joint, skin and medication list on one page, handed over rather than recited. Our notes on preparing for an appointment apply here more than anywhere.

❓ Frequently asked questions

📚 Sources & further reading

The information in this article is drawn from the following sources. We encourage you to explore them, and to share the first two with your clinical team.

• 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.

• Dr Anton Krige, via Mast Cell Action, June 2025: MCAS: considerations for anaesthesia and surgery (clinician-facing slide set, uncited)

• MedlinePlus Genetics, National Library of Medicine: Ehlers-Danlos syndrome, for the 2017 classification and the subtype genetics