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.



In addition to treating routine pediatric patients in his clinical practice, above, Lawrence Tychsen, MD, has become an advocate for improving the vision of children with neurological disorders such as cerebral palsy, Down syndrome and autism.

Vision correction surgery helped Julie Lawrence to see "almost perfectly."

"These are kids who were legally blind and on whom everyone had given up."
Janice E. Brunstrom, MD

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."

As Colton Barnes watches a test pattern, a readout of his brain activity allows the physician to determine the child's visual acuity.

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."

When "good enough" is not good enough

CP advocacy to U.S. Congress: Janice E. Brunstrom, MD

When Janice E. Brunstrom, MD, opened the Washington University Pediatric Neurology Cerebral Palsy Center at St. Louis Children's Hospital in 1998, she heard the same story over and over from parents of her patients who had difficulty with their vision. The parents were often told by other physicians that their child's vision was "good enough" for a child with cerebral palsy (CP).

Brunstrom recalls one patient who had been examined by many ophthalmologists. Her parents had accepted that their daughter's vision always would be poor. Brunstrom convinced the family to see just one more ophthalmologist — Lawrence Tychsen, MD. He found that the little girl was profoundly nearsighted, but amenable to treatment.

Stories like these motivate Brunstrom to advocate for more research into cerebral palsy's causes, treatments and potential cures.

In testimony to the U.S. House Subcommittee on Labor, Health and Human Services, Education and Related Agencies — Committee on Appropriations earlier this year, Brunstrom asked the committee to allocate $10 million in 2007 to establish 10 cerebral palsy surveillance and epidemiology research sites nationwide.

The little girl Brunstrom insisted be examined by Tychsen had LASEK vision correction surgery shortly after her exam. One year later, her vision is now 20/40 in one eye and 20/70 in the other. This summer, she saw stars in the night sky for the very first time.

Janice E. Brunstrom, MD, and cerebral palsy patient Emma Price demonstrate martial arts moves for karate instructor Charlie Walton. A national seminar, led by Brunstrom, highlights a highly effective approach to managing cerebral palsy through physical education.