Seeing Things Differently
Some say it's not worth it to treat the eyes of children with severe neurological disorders. Others see an opportunity to make a difference in kids' lives.
"These are kids who were legally blind and on whom everyone had given up."
Julie Lawrence, a young woman with a big smile and shiny dark hair, was once considered legally blind. Angelman Syndrome, a genetic disorder that can cause a jerky gait, absent speech, excessive laughter and seizures, made her extremely nearsighted with astigmatism. When coupled with her autistic tendencies, her poor vision caused her to withdraw into herself.
Her mom, Greta Lawrence, says some of Julie's physicians discouraged them from having vision correction surgery. Because Julie can't read or do academics, "it wasn't worth it," she recalls them saying.
But Lawrence Tychsen, MD, thought otherwise.
"Dr. Tychsen thought it would be worthwhile," says Greta. "He always treated Julie like she was important."
Three years ago, Julie's parents decided to have Tychsen, professor of ophthalmology and visual sciences, perform the vision correction surgery. They noticed changes in their daughter right away.
"When we drove home from the hospital, she started noticing all the cars around her," Greta says. "She hadn't realized that there were other cars on the road. Even though she doesn't talk, she was laughing and looking at everything."
Julie Lawrence has been a patient of Tychsen's since she was 5 years old. Glasses were prescribed for her for several years, but because she dislikes anything on her head or face, she would remove them and throw them, breaking pair after pair. Eventually, Julie refused to wear glasses at all.
Tychsen, professor of pediatrics and of neurobiology and ophthalmologist-in-chief at St. Louis Children's Hospital, told Julie's parents that she needed corrected vision to become interested and engaged in the world. He had previously surgically corrected a muscular problem in Julie's eyes and suggested the additional surgery.
Since the surgery, which corrected Julie's vision to "almost perfect," she now can recognize her family from across the room, she walks and uses stairs without assistance, and she is less restless and insecure when in public.
"She's more content to sit and watch what people are doing," says her mom, Greta. "All she has to occupy herself with is what she can look at. If she couldn't see, she wouldn't be doing that."
Many children with neurological disorders have extremely poor eyesight and are blind without correction (worse than 20/200). The ability of these children to read, pick up objects and see the world is so impaired and complicated to treat that many go untreated.
Janice E. Brunstrom, MD, a Washington University 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 Tychsen to devise some solutions.
Tychsen established a laboratory for specialized testing of children's vision 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 only a few U.S. hospitals performing such operations, averaging about 60 special-needs children each year.
"We work with the most profoundly impaired children who are the most difficult to examine," says Tychsen.
Brunstrom, assistant professor of neurology, of pediatrics, and of cell biology and physiology, says when she 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 on whom everyone had given up," 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."
The children who are the best candidates for vision correction surgery are those who cannot or will not wear glasses, like Julie, and have blunted social interactions or fearfulness because of their visual impairment. Tychsen says these children suffer from a kind of "visual autism."
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 also can 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 an intraocular lens, leaving the natural lens in place.
The other is a lens extraction technique, in which the natural 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 (nine times worse than legally blind), to nearly 20/20, Tychsen says.
Most of these children also have other factors affecting their vision, such as strabismus (crossed eyes), nystagmus (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 their vision.
To overcome the child's communication difficulties, Tychsen and his team use several noninvasive electronic techniques to measure eyesight before, during and after surgery. A computer allows precise measurement of visual acuity and eye tracking, which can help determine whether a child has a problem with sensory input or motor output.
Although the surgeries can make significant improvements in the child's vision and overall quality of life, most laser-treated children see some regression in their vision over time, and about 10 percent require repeat surgery. 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. "When contemplating what it could mean to the overall development of the child, most parents opt for surgery."