Congenital Cataracts

Treatment Options for Newborns

Carole Harvey's daughter, Rhiannon, was diagnosed with a unilateral cataract in her right eye when she was just 2 days old. Harvey, who lives in Scotland, says the first specialist she saw was not optimistic about the long-term prospects for saving Rhiannon's eye. "The first doctor was very unenthusiastic," says Harvey. "[He] said he had never seen good results from a unilateral cataract. He even suggested her eye could be removed for cosmetic purposes. Fortunately, he mentioned he had a colleague who might know more." The new doctor did know more. He explained to the Harveys that Rhiannon could have surgery immediately that might give her near normal vision. At the ripe old age of 5 days old, Rhiannon had surgery to remove her affected eye lens and had a specially made contact lens fitted to her eye. Now, at age 7, her vision is 20/30. Harvey says her eyesight is the best that has ever been recorded in a child with her condition.

Congenital Cataracts
When most people think of cataracts, they think of them as part of the aging process. What many people don't realize is that a small percentage of babies, approximately 0.4 percent of all children, are born with cataracts in one or both eyes. Of the various types of congenital cataracts, unilateral cataracts found in only one eye are by far the most common. There is no single known cause, although a family history of congenital cataracts can increase risk slightly. Bilateral cataracts those found in both eyes are often a result of a dominant gene in one parent or may accompany another condition, such as Down syndrome. It's important that unilateral cataracts be diagnosed and treated as early as possible because the strong eye can take over from the weak eye to the point that the child can become functionally blind in one eye, even though the eye itself is technically fine. Dr. Kenneth Wright, director of the Pediatric Ophthalmology and Adult Strabismus Center in Los Angeles, Calif., explains that this is because eyesight is as much a function of the brain as it is the eye. "A blurred image early in the baby's development damages the areas in the brain that are responsible for vision," says Dr. Wright. "This early blurred image is so disruptive that unless you correct it in the first few weeks of life they never develop vision." In other words, even if it's corrected later so that the eye is no longer clouded, the brain still "sees" clouds.

Correcting Cataracts in Newborns

The first, most important step in correcting the potential damage to vision that cataracts can cause is early diagnosis. Dr. Wright says all newborns should be examined for cataracts before leaving the hospital nursery. This is called the Red Reflex Test. It should then be repeated at each well-baby exam throughout the baby's first year. If a cataract is detected, surgery will generally be done as soon as possible to remove the cataract. Dr. Wright has done surgery on babies as young as 48 hours old. After the natural lens is removed, either a permanent lens is implanted, called an intraocular lens, or the baby is fitted with contact lenses or glasses. What the doctor decides to do depends on a number of factors, including the severity of the cataract and the child's age.

At some point, to strengthen the weak eye, the child will need to wear a patch over the stronger eye. This is the so-called "lazy eye" effect of the cataracts. With early diagnosis and aggressive treatment, vision is retained and, as is the case with Rhiannon Harvey, can end up being quite good. Sometimes, if the cataract doesn't seem to be interfering with vision, it will be left in until the child is older. This was the case with Shannon Purcell's daughter, Morgan, who was diagnosed with a unilateral cataract when she was a newborn but didn't have surgery until age 5. "Before surgery, Morgan's eyesight was approximately 20/60 to 20/80 in the left eye," says Purcell of Falmouth, Ky. "Now, since she has had the cataract removed and an intraocular lens implant, her vision is about 20/60. On a couple of visits, her vision has been 20/50. She tells me that she can see 'better' and it's not as 'blurry,' but she is still young. At such a young age, it's hard to determine an accurate reading. My hope is that her eyesight will continue to strengthen and improve as she grows older." It was Purcell who first noticed Morgan's cataract and brought it to the attention of her pediatrician. Dr. Deborah VanderVeen, a staff ophthalmologist at Children's Hospital Boston and instructor at Harvard Medical School, says it's important for parents to pay attention to their children's eyes, as some cataracts can be developmental or can be so small that they're not diagnosed immediately. "In our area, pediatricians are very good about screening infants, but if a parent has any concerns about infants not showing appropriate visual behavior they should talk to their pediatrician," says Dr. VanderVeen. "Also, if there's a family history of congenital cataracts, be sure to notify the doctor so they can be on the lookout for any problems."

What to Look For While all babies should be tested shortly after birth for the possibility of congenital cataracts, here are some things to watch for in your baby's first year that may indicate an undiagnosed problem:

Misaligned eyes Eyes not tracking objects appropriately Any abnormal "spots" in the eye, such as white spots Involuntary rhythmic movements of the eyes back and forth, up and down, around or mixed

If you notice any of these symptoms, contact your pediatrician immediately.

SUMMARY

What many people don't realize is that a small percentage of babies, approximately 0.4 percent of all children, are born with cataracts in one or both eyes. Of the various types of congenital cataracts, unilateral cataracts found in only one eye are by far the most common. There is no single known cause, although a family history of congenital cataracts can increase risk slightly. It's important that unilateral cataracts be diagnosed and treated as early as possible because the strong eye can take over from the weak eye to the point that the child can become functionally blind in one eye, even though the eye itself is technically fine. The first, most important step in correcting the potential damage to vision that cataracts can cause is early diagnosis. Dr. Wright says all newborns should be examined for cataracts before leaving the hospital nursery. If a cataract is detected, surgery will generally be done as soon as possible to remove the cataract. Dr. Wright has done surgery on babies as young as 48 hours old. After the natural lens is removed, either a permanent lens is implanted, called an intraocular lens, or the baby is fitted with contact lenses or glasses. What the doctor decides to do depends on a number of factors, including the severity of the cataract and the child's age. At some point, to strengthen the weak eye, the child will need to wear a patch over the stronger eye. This is the so-called "lazy eye" effect of the cataracts. Sometimes, if the cataract doesn't seem to be interfering with vision, it will be left in until the child is older. "Before surgery, Morgan's eyesight was approximately 20/60 to 20/80 in the left eye," says Purcell of Falmouth, Ky. "Now, since she has had the cataract removed and an intraocular lens implant, her vision is about 20/60. It was Purcell who first noticed Morgan's cataract and brought it to the attention of her pediatrician.

REACTION and RECOMMENDATION

It is good to have an article where newborn anomalies are being discussed. This helps us to have a better understanding to what has been going on and why these anomalies were acquired. Newborn cataract is a newborn anomaly wherein surgery is the only way to correct it. Early diagnoses are needed in order to correct this cataract as soon as possible in the first year of life of the child. This article helps every medical or non-medical field individuals to understand its root causes and how to prevent or to have correct interventions with regards to proper caring for those newborns who has got the problem. Newborn with this anomaly may lead to have a poor vision or, possibly, permanent blindness if not treated early. This article also gives hope to those parents whose their child has cataract, that they can live normally after surgey. As a nurse, we need to support the parents, empathize with them, and to comfort them before the surgery and give them assurance that after the surgery, their child will now be able to have a normal vision. Support is the only thing that we can give to the parents. By this, we can also give strength and hope that the surgery will be successful. After the surgery has been done, we must have an intensive care, while the child is still in the healing process. We need to assess for the childs activities in order to monitor its improvement after the surgery. We must advice the parents to note for any inappropriate things that the child is exhibiting and to report it to their pediatricians to monitor any after effects of surgery. We also need to advise them to return to the hospital to monitor the childs eye after the surgery for its effectiveness.

SUBMITTED BY: EMMANUEL R MARGATE JR.

SUBMITTED TO: MR. RAYMOND GO GEREA