Infantile Cataracts are a Blind Spot

Three days after Canyon Brown’s birth, the attending pediatrician discovered a problem.

During a standard red reflex test, in which a red light from an ophthalmoscope shines into the pupil, the boy’s right eye, unlike his left, does not reflect light back.

The cataract present in Canyon Brown’s right eye at birth was removed when she was 6 weeks old.

People can be born with cataracts. They can form them at any time in life. The clouding of the lens is not just an artifact of aging. According to the American Academy of Ophthalmology, the incidence of infantile cataracts is 3 to 4 per 10,000 live births per year. They are responsible for 5 to 20 percent of childhood blindness worldwide.

An even lesser known fact is that cataracts are much more serious in a baby than in an adult.

“People always think, ‘Oh my grandpa had his cataracts removed and it was in and out of surgery in 20 minutes and he never saw a surgeon again, no problem,’ but with kids it’s the opposite,” said Jolene Rudell , MD, PhD, pediatric ophthalmologist at Shiley Eye Institute at UC San Diego Health.

Rudell removed Canyon’s cloudy lens when he was 6 weeks old. This was considered emergency surgery for several reasons, each of which differentiates between infantile cataracts and the adult kind.

“One of the things we’re always worried about is cancer,” said Rudell, explaining that retinoblastoma can sometimes look like a congenital cataract, but if it’s discovered, it could be “radiation, chemotherapy, and possibly even the removal of the eye” require treatment.

“That was scary for us,” said Canyon’s mother Rochelle Gaudette. “We have a newborn baby who may have this life-threatening disease?”

In Canyon’s case, no tumor was detected. His cataract was due to an unexplained underdevelopment of the eye in the womb. (If Canyon developed cataracts in both eyes, that could be a sign of a more serious problem, such as an infection or genetic syndrome that can affect a child’s development and health.)

Whatever the cause, treating cataracts is also much more urgent for infants than it is for adults, as the brain begins to shut down the view of a poorly performing eye immediately after birth. That’s why, like most people who develop cataracts in childhood, Canyon also has amblyopia (better called lazy eye).

“There’s a very short period of time in which we can operate, which is four to six weeks, to have a chance of saving any vision in the eye,” says Rudell, who regularly performs these surgeries, “because, from the first day, the brain prefers the eye without cataracts.”

Finally, the treatment of cataracts in infants is much more complicated because cloudy lenses cannot be immediately replaced with artificial ones. Eyes continue to grow and change shape, according to Rudell. So Canyon has to wear a contact lens, or very thick glasses, to have some focusing power in his problem eye until he’s a bit older, when his parents have the option to give him an intraocular transplant or continue indefinitely. the contact lens.

Canyon Brown, pictured with his mother when he was 18 months old, has to cover his unaffected eye with a band-aid for 3 to 6 hours a day to strengthen his affected eye.

“When he wakes up, you have to clean the contact lens and then try to pry his eyeball open and put it in without him knocking your hand away or yelling or writhing,” Gaudette said. “And the glasses don’t work because he doesn’t wear his glasses anymore. He pulls them away.”

Because of his amblyopia, Canyon also has to wear a patch over his unaffected eye for three to six hours a day to strengthen his problem eye.

“It’s really changed our whole family’s routine,” Gaudette said, “and with every step things come up.”

Rudell calls correcting cataracts in babies “a lifelong process.”

“At any point after surgery, from days to decades later, there are many potential problems that can arise, and likely additional surgery to correct them,” she said. “Even if their surgeries go well and the right things are done at the right time, children with underdeveloped eyes are at greater risk for glaucoma and other forms of blindness. Some of them may eventually lose their sight.”

And the cost of this treatment could break the bank of the average family. Canyon’s lensectomy alone cost $35,000 as billed to his insurance company, which denied the claim. Fortunately, a cash pay scheme for Gaudette and her husband cost just $8,200, all of which was paid for by generous friends and family through a GoFundMe campaign.

Many families eventually decide that saving the vision in one eye isn’t worth all the effort, Rudell said.

“It can be a difficult decision to put a six-week-old under anesthesia for something they won’t die from,” Rudell said. “After all, you can still legally ride with just one eye, and Canyon will still be able to do what other kids can do.”

Jolene Rudell, MD, PhD, is a pediatric ophthalmologist at UC San Diego Health’s Shiley Eye Institute.

The problem, Rudell said, is if anything ever happens to the unaffected eye.

“When a child grows up and develops macular degeneration that gets worse in their good eye, they essentially become blind,” she said. “That’s why we always try to maximize every vision we can, when we can.”

Rudell says more research is needed on congenital cataracts.

“We have options to treat patients like Canyon, but we still don’t have great solutions, and so many questions remain about the best way to treat congenital cataracts,” she said. “Unfortunately, it has not been well studied. But I hope that more research alone can help improve visual outcomes for children with eye diseases, including cataracts.”

For now, Canyon is healthy and developing normally at 18 months old, which is what his parents are targeting.

“He gets along well with others and his language is developing well,” said Gaudette. “I almost think he has an edge in some respects.” She paused, adding, “It’s not like this is a walk in the park, but just the fact that you can treat it makes us just want to go for it and hope for the best.”

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