Anesthesia and Surgery Safety

Why CMT changes the surgical plan

CMT will have implications for the surgical plan. There are decisions to be made, from the choice of the actual anesthetic, to specific drug choices, to how you are positioned and protected during the procedure.

The perioperative team (surgeons, anesthesiologists, nurses) will ask you at various points leading up to your procedure about your medical history. It is very important that your CMT and the baseline functioning of your feet and hands are discussed and documented beforehand.

Anesthetic choices and medication

CMT requires a very specific discussion about the anesthetic choice and the medications given throughout the perioperative period. Options include general anesthesia, neuraxial anesthesia (epidurals and spinals), regional anesthesia (nerve blocks to anesthetize one limb), intravenous sedation (also known as MAC or "twilight"), and local anesthesia. Each of these can be done safely in patients with CMT as long as there is a clear discussion and documentation of your functional status before the procedure.

With regional anesthesia, one limb is anesthetized for the operation. The nerve block is usually placed under ultrasound guidance so the team can visualize the nerves directly. There is, however, always a chance that the nerves could be additionally affected by the block itself, so talk the pros and cons through with your anesthesiologist.

As for the medications, the neurotoxic drug list is very helpful to bring with you to all of your appointments. It includes many medications used during the perioperative period, among them antibiotics, muscle relaxants, and nitrous oxide. Your anesthesiologist should be made aware of the list, with a specific focus on those categories, to make the best decision for your care.

Positioning and nerve protection

Different procedures require different positioning. In all of these cases the operative team will pad every area considered a pressure point, placing soft foam underneath so the still operating table does not put undue pressure on the nerves.

This matters especially for CMT patients, who may have altered sensation in their extremities, so make sure your surgical team knows to protect your nerves. The team should also assess and document your nerve sensation and motor function once you are awake from the procedure.

Breathing and recovery

Maintaining adequate breathing is the cornerstone of any anesthetic. A few subtypes of CMT have respiratory involvement, which warrants a lengthy discussion before the procedure. Separately, many patients also have sleep apnea.

Any and all respiratory concerns should be evaluated beforehand, and if you use a CPAP or BiPAP machine, consider bringing it with you to use in the post-operative period. Our page on breathing and respiratory health explains the warning signs worth mentioning.

A short checklist to bring

Tell the surgeon and anesthesiologist you have CMT, and name your subtype if you know it.

Ask them to avoid succinylcholine and to review your neurotoxic drug list.

Ask how they will protect your nerves during positioning.

Mention any breathing involvement, and confirm the recovery plan.

If your surgery is on the foot, hand, or spine, a surgeon who treats CMT regularly is worth seeking out. A Center of Excellence can help you find one.