Congenital Cataracts

By Marilyn Haddrill; contributions and
review by Charles Slonim, MD

If you are told that your newborn baby has a congenital cataract, this means that the eye's natural lens is cloudy instead of clear. Vision could be hampered to the extent that cataract surgery may be required for removal of your child's natural lens (that is, the cataract).

In about 0.4 percent of all births, congenital cataracts are found or soon develop.* Not all congenital cataracts require surgical removal, but many do. Cataracts that cloud only the peripheral portion of the lens may not need removal, because central vision remains unimpeded. Very small cataracts, too, may be considered too insignificant to require surgery.

When Should My Child Have Cataract Surgery?

Opinions vary about when cataract surgery should be performed on an infant, because of concerns about complications such as development of high internal (intraocular) eye pressure (IOP) known as secondary glaucoma. High IOP can occur if cataract surgery damages the fluid outflow structure (trabecular meshwork) inside the eye. Also, the use of anesthesia for surgery involving very young infants can be cause for safety concerns.

On the other hand, cataract surgery may need to be performed as soon as possible to ensure that vision is clear enough to allow normal development of your baby's vision system. Some experts say the optimal time to intervene and remove a visually significant congenital cataract from an infant's eye is between the age of six weeks and three months. If your baby has a congenital cataract, discuss any concerns you have about timing of cataract surgery with your eye surgeon.

Once the cataract is removed, it is absolutely vital that your child's eye be corrected with a surgically implanted lens (intraocular lens), contact lens or eyeglasses. Without vision correction following cataract surgery, the eye will have poor vision, and normal infant vision development will be impeded.

Opinions also vary about whether an artificial lens should be surgically inserted in a baby's eye following cataract surgery because of concerns that normal eye growth and development might be hampered. IOLs also may need to be changed out as your child's eyes grow and change, not because of any difference in eye size but because refractive or vision errors often change.

In some cases, contact lenses fitted on the eye's surface (cornea) may be used to help restore vision after the natural lens is removed during cataract surgery. Also, eyeglasses may be prescribed to aid vision in lieu of an inserted artificial lens or contact lens.

Because it can be tough to convince a very young child to wear contact lenses or eyeglasses, you might try some of these strategies:

• Apply contact lenses while your child is sleeping. If you use extended wear contacts, this process will be needed only weekly or monthly.
• If your child wears eyeglasses, make sure you and other family members frequently make complimentary remarks about appearance.
• If you are a parent or guardian and don't need eyeglasses, consider wearing clear lenses in frames to inspire your child to follow your example.
• Ask your optician about children's frames with adjustable temples that fit snugly on the back of the ear, along with sturdy pediatric frames, to make sure your child's eyeglasses aren't damaged, lost or easily removed.

What Causes Congenital Cataracts?

Cataracts clouding the eye's natural lens usually are associated with aging processes. But congenital cataracts occur in newborn babies for many reasons that can include inherited tendencies, infection, metabolic problems, diabetes, trauma, inflammation or drug reactions.

As an example, tetracycline antibiotics used to treat infections in pregnant women have been shown to cause cataracts in newborn babies. Congenital cataracts also can occur when, during pregnancy, the mother develops infections such as measles or rubella (the most common cause), rubeola, chicken pox, cytomegalovirus, herpes simplex, herpes zoster, poliomyelitis, influenza, Epstein-Barr virus, syphilis, and toxoplasmosis.

Older babies and children also can be diagnosed with cataracts, known as pediatric cataracts, for similar reasons. However, trauma associated with events such as a blow to the eye is the underlying cause in 40 percent of cases of cataracts in older children. In 33 percent of cases of pediatric cataracts, children were born with congenital cataracts that may initially have been overlooked.**

In inherited and other forms of congenital cataracts, abnormalities may occur in the formation of proteins essential for maintaining transparency of the eye's natural lens.

This congenital cataract should be removed, since it impedes central vision.

(Photo: National Eye Institute, National Institutes of Health)

Types of Congenital Cataracts

• Anterior polar cataracts are well defined, located in the front part of the eye's lens and thought to be commonly associated with inherited traits. These types of cataracts often are considered too small to require surgical intervention.
• Posterior polar cataracts also are well defined, but appear in the back portion of the eye's lens.
• Nuclear cataracts appear in the central part of the lens and are a very common form of congenital cataracts.
• Cerulean cataracts usually are found in both eyes of infants and are distinguished by small, bluish dots in the lens. Typically, these types of cataracts do not cause vision problems. Cerulean cataracts appear to be associated with inherited tendencies.

Congenital Cataracts and Other Vision Problems

Without early intervention, congenital cataracts cause "lazy eye" or amblyopia. This condition then can lead to other eye problems such as nystagmus, strabismus and inability to fix a gaze upon objects.

Such problems can profoundly impact learning ability, personality and even appearance, ultimately affecting a child's entire life. For these and many other reasons, make sure your child's eyes are examined regularly and as soon as possible after your baby is born.

Resources:

*Congenital and juvenile cataracts. Ophthalmology, 3rd ed.. 2008.

**Pediatric cataracts. Textbook of Family Practice, 7th ed. 2007.

[Page updated November 2009]