Strabismus Eye Surgery in Kids with Rare Diseases

For some babies, misaligned eyes are just a quirk of development that resolves with time. For other children, misaligned eyes (called strabismus) can also signal developmental issues, says Karen Schultz-Shimshak, MD, a Mayo Clinic ophthalmologist whose expertise includes pediatric strabismus surgery. “The eyes are very much connected to overall development—they are not isolated as many people think,” she says.

If a child’s eyes are still turning in or out after six months, surgery may be needed. Strabismus surgery is a relatively common surgery that involves loosening or tightening a child’s eye muscles to correct their alignment. It doesn’t just provide cosmetic benefits. Alignment correction allows the two eyes to work together in unison, to see one picture. This correction can improve depth perception, correct double vision, and protect against further vision impairments and potential vision loss.

A crucial time of visual development happens from birth to two years. After that period, a child can lose the ability to use their eyes in unison for the rest of their life. “Early strabismus surgery is important,” Schultz-Shimshak says.

Understanding pediatric strabismus

The general guideline is that any child with a misalignment of the eye after six months should be evaluated by a pediatric ophthalmologist, Schultz-Shimshak says. In rare cases, strabismus isn’t present in the first few months and can instead develop a bit later in life, usually before three years.

About 5% of all children have some form of eye-crossing, says Schultz-Shimshak, who recommends early vision screening for all children. “The eye can often be a window into determining other systemic disorders,” she adds. Strabismus could be a marker of a genetic disease. In fact, the eye is often a targeted organ that’s affected by underlying genetic disorders, she says.

Megan Nolan, founder of Rare Parenting and mother to a three-year-old with FOXG1 syndrome, says when her son Domenico’s eyes began to turn in occasionally, around two months, it wasn’t initially cause for alarm. Many children’s eyes align without intervention, usually before six months of age. Many other children’s crossed or misaligned eyes can be resolved by wearing glasses or a patch.

At six months, Domenico’s eyes were still turning in intermittently. Additionally, developmental delays emerged, such as not reaching for objects, sitting up, and crawling. Pediatricians, among other doctors, pushed for a wait-and-see approach, all failing to mention genetic testing. “We knew something deeper was wrong, but we didn’t know what it was,” Nolan says. “Back then, we weren’t familiar with non-inherited genetic disorders, so we didn’t know to ask for testing,” she says.

When Domenico’s eyes didn’t straighten over time, Domenico tried glasses with an optometrist and eye patching with a behavioral ophthalmologist, both without success. If there’s no improvement after three to four months of interventions, surgery is recommended, Schultz-Shimshak says.

Domenico’s parents hoped that treating his eyes with strabismus surgery, a relatively simple procedure, might also help explain or resolve some of Domenico’s developmental delays.

Image of Domenico Sandhu after strabismus surgery, lying on a hospital bed, recovering
Credit: Megan Nolan | Domenico Sandhu

“It was us trying to figure out what was going on with our child,” Nolan says. “We weren’t getting any other answers, so this was something we felt we could do to help him.”

Domenico had strabismus surgery on both eyes at age 14 months at the Children’s Hospital of Philadelphia (CHOP). “There was great hope that maybe this was going to improve things for him in other areas, too,” Nolan says.

What to expect during strabismus surgery and recovery

A typical strabismus surgery is an outpatient procedure that lasts a little bit less than an hour. The eye muscles are very accessible around the outside of the eye and right underneath the white covering.

“If you were to touch the white part of your eye, you’re actually touching right over the eye muscle,” Schultz-Shimshak says. During the procedure, a tiny “window flap,” as Schultz-Shimshak called it, is made on that white membrane so the muscle can be accessed. If the eye is turning in, doctors loosen an inner muscle. If the eye is drifting out, doctors loosen the outer muscle.

“It’s very straightforward,” she says. “You don’t take the eye out or cut on the eyelid or things like that. Those are common questions. We can work right on the muscle, right where it is on the eye.”

After the surgery, patients use antibiotic eye drops, which can blur vision while in use, for a few days to prevent infection and can take over-the-counter pain medicine, as needed. The key is keeping them from rubbing their eyes, but kids are resilient, Schultz-Shimshak says.

“They’re usually bouncing around like not too much has happened,” she says. “I’m always amazed from day one to day three, how well the kids who come into the office are. You wouldn’t know that they had anything done. It’s pretty amazing.” 

For Domenico, the recovery from the surgery itself was relatively smooth, Nolan says. “With nonverbal children, it’s hard on the parent to give—or stop—medication when they can’t tell you how they feel,” she says. To avoid pain, Domenico was given the recommended over-the-counter pain medicine at the suggested dosage.

Two days after the surgery, Domenico had his first seizures. The family was still in Philadelphia, so they took Domenico to CHOP for a series of EEGs and a neurological evaluation. A neurologist there recommended genetic testing. Six weeks later, testing determined that Domenico has FOXG1 syndrome, a rare genetic disease caused by a mutation of the FOXG1 gene, which impacts the brain’s early life development.

What are the expected results from strabismus surgery

The outcome of strabismus surgery will vary depending on the underlying issue that is causing eye misalignment. If a neurological issue is at play, such as is the case with Domenico, the child has a higher chance of recurrent strabismus, Schultz-Shimshak says. On average, 25% to 50% of patients may need additional strabismus surgery.

“It’s not necessarily a one-and-done thing,” she says. “Part of the art of strabismus is knowing what to do and how much to do. It’s a dynamic system that evolves and changes as the child grows and as we grow as adults. I see a lot of adults who didn’t have issues after childhood surgery and, decades later, they develop issues. ”Bottom line, she says, early intervention is key to success.

Domenico Sandhu
Credit: Megan Nolan | Domenico Sandhu

Two years later, Domenico’s eyes still turn in at times, but not nearly as often, or as significantly, as before.

Strabismus surgery brought plenty of benefits. He has better eye coordination. He reaches for toys and objects more often. He makes more eye contact with his parents. His ability to look at and focus on objects is more apparent, which will set him up for better success with his use of communication devices.

If Domenico had been diagnosed with FOXG1 syndrome before the strabismus surgery, Nolan says, they would have moved forward with the surgery because of the benefits. “He may have other issues, but this is one we were able to help correct,” she says. She also knows he might need strabismus surgery again in the future, because of the nature of his disorder.

“Strabismus surgery is meant to straighten the eyes,” Nolan says. “But you can’t cure a symptom when your child has a neurological disorder like Domenico’s. You can treat and improve symptoms, but they won’t disappear completely.”

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