Innovative surgery corrects vision in kids with neurological disorders

Janice Brunstrom, M.D., assistant professor of neurology and pediatrics at Washington University School of Medicine in St. Loius and a neurologist at St. Louis Children's Hospital, saw firsthand how her patients' poor vision interfered with every aspect of their daily lives. Having cerebral palsy herself and wanting to help reverse the isolation that many of these children endure because of their poor vision, she approached pediatric ophthalmologist Lawrence Tychsen, M.D., to help devise some solutions.

He did. Tychsen, professor of ophthalmology and visual sciences, of pediatrics and of neurobiology and ophthalmologist in chief at St. Louis Children's Hospital, developed specialized testing and now does vision correction, or refractive surgery, on children with cerebral palsy, Down syndrome and neurobehavioral disorders such as autism. To date, St. Louis Children's Hospital is one of the only U.S. medical centers performing refractive surgery on these children and has the highest volume, operating on about 60 special-needs children a year.

“We work with the most profoundly impaired children who are the most difficult to examine,” says Tychsen, also professor of anatomy and neurobiology and of pediatrics. “So we tend to have the most grateful parents, too.”

When Brunstrom talked with Tychsen about repairing the vision in these children, he readily agreed and made room for them in his busy clinical schedule.

“These are kids who were legally blind and whom everyone had given up on,” Brunstrom says. “One by one, he has restored their sight by going through every detail and figuring out what is wrong and what he can fix. He is willing to tackle situations that used to be considered impossible or not worth the time.”

The children who are the best candidates for vision correction surgery are those who cannot or will not wear glasses and have blunted social interactions or fearfulness because of their visual impairment. Tychsen says that these children suffer a kind of “visual autism.” About 80 percent of children with severe neurological disorders have some kind of vision impairment. About 10 percent have a severe impairment.

Tychsen and his staff perform laser-assisted subepithelial keratectomy, or LASEK, in which the cornea is reshaped with a laser. This technique doesn't require a surgical flap to be cut in the eye and is safer for children, who will inevitably rub their eyes after surgery. In addition, the LASEK technique is able to correct much higher degrees of myopia, or nearsightedness, than the LASIK technique (laser-assisted in situ keratomileusis) commonly used on adults. The surgeons can also correct extreme farsightedness.

For children with focusing defects so large that they are beyond the range of laser correction, Tychsen uses other surgical techniques. One of these is implantation of a phakic intraocular lens, leaving the natural lens in place. The other is a lens extraction technique, in which the natural eye lens is removed and replaced with another type of implant. These techniques can improve vision in a child with profound nearsightedness, such as 20/1,500, to nearly 20/20, Tychsen says.

Most of these children also have other factors affecting their vision, such as crossed eyes, wiggling eyes or a structural anomaly. In those cases, refractive surgery alone can't correct their vision to 20/20, but when combined with eye muscle surgery or other procedures, it can considerably improve vision.

Because many of the children Tychsen treats are unable to communicate clearly or are uncooperative, he and his team use several noninvasive, electronic techniques to measure eyesight and determine the success of surgery. A computer-recording method measures the improvements that can be achieved in the visual brain while the child is awake. Other instruments take precise measurements before surgery while a child is under anesthesia.

Although the surgeries can make significant improvements in the child's vision and overall quality of life, most laser-treated children will see mild regression in their vision over time and about 10 percent require repeat surgery, Tychsen says. But for most parents, the decision to have their child go through the surgery is relatively simple.

“For special-needs children, there is often no alternative,” Tychsen says. “They cannot or will not wear glasses and are unsuitable for contacts. When contemplating what it could mean to the overall development of the child, most parents opt for surgery.”

Julie Lawrence is one of Tychsen's patients whose life has changed significantly since she had vision correction surgery about three years ago, says her mother, Greta Lawrence. Julie has Angelman Syndrome, a chromosomal disorder, and was extremely nearsighted with astigmatism.

Because of her autistic tendencies and poor vision, Julie would withdraw into herself. Tychsen often reminded the Lawrences that Julie needed vision correction to become interested in things and engaged in the world. However, Greta Lawrence says some of Julie's other doctors discouraged them from having the surgery.

“Because she can't read or do academics, some doctors said it wasn't worth it,” Greta Lawrence says tearfully. “But Dr. Tychsen always treated her like she was important and thought it would be worthwhile.”

The Lawrences eventually decided to have Tychsen perform the vision correction surgery and noticed changes in Julie right away.

“When we drove home from hospital, she started noticing all the cars around her,” Greta Lawrence says. “She didn't realize that when you are on the road there are other cars around. Although she doesn't talk, she was laughing and looking at everything.”

Greta Lawrence says since the surgery, which corrected Julie's vision to “almost perfect,” Julie can now recognize her family from across the room, she can walk and go up and down stairs better and is less restless when in public. “She's more content to sit and watch what people are doing. If she couldn't see, she wouldn't be doing that.”