Rett Syndrome and Anesthesia: What Parents Should Know Before Surgery

Children with Rett syndrome need ongoing care and support. In some cases, that may include surgery. Procedures such as scoliosis surgery, tonsil removal, and other operations may improve the child’s comfort and quality of life while also making daily care easier.

Major surgeries require general anesthesia to put the person in a sleep-like state. When a child is under anesthesia, they’re unable to feel any pain or discomfort. General anesthesia can also prevent reflexes that can cause them to move during surgery.

Although anesthesia makes surgery significantly easier than it would otherwise be, Rett syndrome can involve complications that might call for special planning for surgeries with anesthesia. In this article, we’ll discuss everything parents and caregivers should know about anesthesia for children with Rett syndrome.

Why Anesthesia Can Be Tricky in Rett Syndrome

Some Rett symptoms can influence how anesthesia is given, or put a child at a higher risk for complications while under anesthesia. The anesthesia team considers these symptoms and complications while planning for the surgery and plans breathing support accordingly.

Breathing Irregularities

More than 80 percent of people with Rett syndrome have breathing problems at some point, which often start between ages 6 and 11. These symptoms often disappear between 11 and 13 but can return later.

Sleep apnea, breath holding, and other common breathing issues in Rett syndrome can complicate anesthesia. Children with Rett syndrome are at an increased risk of apneas (pauses in breathing) and other breathing complications after they come out of anesthesia, so they might need longer hospital stays after surgery for observation.

If a person has obstructive sleep apnea, which is common in Rett syndrome, their throat may close while under anesthesia. They may also have trouble waking up and taking a breath once the surgery is over.

During surgeries with anesthesia, a care team monitors the person’s breathing and may insert a flexible breathing tube before the surgery to make sure the person gets enough oxygen.

Choking Risks

Swallowing problems in Rett syndrome can increase a child’s risk of choking or aspiration, especially if they start eating regular foods too soon after surgery under anesthesia.

Gastroesophageal reflux (acid reflux) in people with Rett can put them at risk of a complication called laryngospasm. Anesthesia can irritate the vocal cords, causing them to tighten up and prevent breathing and speaking.

Reduced Muscle Tone

Many children with Rett syndrome have low muscle tone. This can make general anesthesia more challenging because it may affect breathing during surgery.

Medication Sensitivities

Children with Rett syndrome can be more sensitive to some medications, including sedative drugs. The anesthesiology team accounts for this and can adjust the child’s dose accordingly. Still, a child might have a higher chance of side effects from certain types of anesthesia.

What To Share With the Surgical Team

A child’s surgical team will evaluate them in great detail leading up to surgery. Parents and caregivers should share as much information as possible with the care team, including details that might affect how they plan for and administer anesthesia.

Seizure History

Seizures and epilepsy are common features of Rett syndrome. If a child has seizures, parents and caregivers should let the surgical team know. The team might use an electroencephalogram (EEG) to check the brain for seizure-related activity before any surgery with anesthesia.

Current Medications

Parents or caregivers should keep a list of the child’s current medications that they give to the surgical team before any surgery with anesthesia. Some medications might need to be stopped or adjusted before surgery to lower anesthesia-related risks.

Breathing and Sleep Issues

Any breathing problems, including sleep apnea, should be reported before surgery with anesthesia. That way, the team can prepare for and take steps to prevent possible complications. The surgical team evaluates the child’s breathing patterns as part of the preoperative process.

Choking and Swallowing Issues

Parents and caregivers should let the surgical team know if the child is at a high risk of choking and aspiration. A history of swallowing issues can put the child at risk of choking and aspiration after surgery.

Heart Health History

Many children with Rett syndrome have an abnormal heart rhythm known as a prolonged QT interval. The QT interval is part of the heart’s electrical cycle, and when it lasts longer than normal, it can increase the risk of dangerous heart rhythm problems.

Before surgery, the surgical team may use an electrocardiogram (ECG), a test that records the heart’s electrical activity, to evaluate the child for arrhythmias associated with prolonged QT syndrome. Intubation, certain medications, and electrolyte imbalances can increase the risk of complications in children with a prolonged QT interval. These risks increase with age.

Past Experiences With Anesthesia

It’s also helpful for parents or caregivers to let the surgical team know about a child’s previous experiences under anesthesia, especially if there were any special precautions or complications.

Preparing for Surgery

Before any surgery with anesthesia, a child with Rett may need to undergo a series of tests. These tests help the surgical team identify any health concerns that may need to be addressed during surgery to prevent anesthesia-related complications. The surgical team may want to:

• Take an ECG to check the heart rhythm.
• Evaluate possible seizure activity in the brain with an EEG.
• Evaluate gastroesophageal reflux.
• Check lactic acid levels in the blood.
• Monitor the child’s breathing patterns.

Steps To Take After Surgery

Anesthesia-related complications can happen after surgery is over, during the postoperative period. The surgical team tells parents and caregivers how to monitor the child and what signs to watch out for.

Here are important points to know about care and monitoring during the postoperative period:

• Children with Rett syndrome might need mechanical ventilation (a breathing tube) for some time after surgery.
• Children with Rett might need a longer postoperative intensive care unit stay after surgery with anesthesia than children who don’t have Rett syndrome.
• It’s important to monitor the child’s breathing closely during the recovery period, even if they don’t have other breathing issues like sleep apnea.
• Caregivers should monitor for signs of seizures, aspiration pneumonia, and other complications during the postoperative period.
• Even minor surgeries with anesthesia can lead to postoperative complications in children with Rett syndrome, so monitoring is still just as important as with complex surgeries.

Parents or caregivers should follow all postoperative instructions from the surgical team closely, including giving pain management medicine after surgery and using noninvasive respiratory support such as bilevel positive airway pressure (BiPAP) machines.

Talk to Your Doctor

If you’re a parent or caregiver for someone with Rett syndrome and surgery with anesthesia has been recommended, talk to their doctor or pediatrician. They might have some extra tips or advice for preparing for the surgery and providing care during the recovery period.

Join the Conversation

On MyRettTeam, people share their experiences with Rett syndrome, get advice, and find support from others who understand.

What tips can you share for preparing children with Rett syndrome for surgery? Let others know in the comments below.