Patients with Ehlers-Danlos syndrome (EDS) may need to undergo medical procedures that require anesthesia. Here is information about the different forms of anesthesia, how the condition may complicate their use, and precautions physicians should take.
What is anesthesia?
Anesthesia involves the use of medications to prevent or reduce pain during medical procedures. The four main categories of anesthesia are local anesthesia, regional anesthesia, general anesthesia, and sedation.
Local anesthesia involves delivering medication to a very small area of the body. Stitching a wound, taking a biopsy, or filling a dental cavity are all examples when a local anesthetic might be necessary. Doctors can apply local anesthetics as injections, sprays, or ointments such as lidocaine.
In regional anesthesia, anesthesiologists apply medication to nerves to block a much larger portion of the body. Regional anesthesia is generally achieved through injections into a peripheral nerve (peripheral nerve block) or into the space around the spinal cord (epidural).
Most major surgical procedures require general anesthesia. Here, the patient is unconscious and unresponsive to pain. Doctors use intravenous (IV) injections or infusions, as well as inhalation of gases to achieve general anesthesia. They continuously monitor patients for vital signs such as heart rate, blood pressure, oxygen levels, and breathing.
In sedation, patients are usually conscious. However, they may be in different mental states, ranging from feeling drowsy to not being able later to remember what happened. Sedation is often used with a pain killer to help patients feel more comfortable during a procedure.
When might a patient with EDS need anesthesia?
Depending on disease type, EDS patients may be more likely to need certain surgeries. For example, if a patient has hypermobility, he or she may need surgery to address joint instability or to correct scoliosis. Surgery for joint debridement may also be needed, which is a procedure to clear out any debris causing irritation from inside the joints.
With vascular EDS, patients may need surgical interventions to repair blood vessels, rupture of internal organs, perforation of the intestines, or pneumothorax.
Some patients may need anesthesia for surgery to repair eye damage, such as retinal detachment, or to replace the cornea or lens.
What are possible complications of anesthesia?
Complications can occur with the use of anesthesia depending on the patient’s EDS type. People with EDS often have fragile skin that can bruise or tear easily, which makes the use of tape to hold down IVs problematic. The use of blood pressure cuffs for monitoring can also potentially lead to bruising in some patients.
Many patients have fragile blood vessels, so the use of IVs or invasive monitoring can lead to dissections (small tears in the lining of the blood vessels).
The application of regional anesthetics may also be more difficult or riskier for patients with EDS because of issues such as scoliosis and the risk for punctures or bleeding into the cerebrospinal fluid. Some forms of EDS can also cause Tarlov cysts — small sacs around the nerves of the spinal cord, which can possibly lead to complications during epidurals.
What precautions should doctors take?
Doctors strongly recommend that EDS patients have any procedures done at specialized hospital facilities with EDS experience, because of the potentially life-threatening complications of the disease.
Prior to surgery, physicians should do a thorough analysis of the patient’s medical history for previous complications. MRI or ultrasound imaging of the spine can help determine the potential risks of using regional anesthetics.
Blood pressure cuffs should be checked regularly during operations to make sure they are not applying too much pressure and causing bruising.
Ultrasound can be used during the placement of IVs to help lower the risk of tears in blood vessels. Patients with vascular EDS may experience a potential bleeding episode so physicians should have extra blood available if needed.
After anesthesia, physicians should give patients medications to reduce the possibility of nausea and vomiting as this can lead to rupture of the esophagus.
Last updated: Nov. 25, 2020
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