Congenital Cataract

Congenital Cataract

The term congenital cataract refers to a lens opacity present at birth. Congenital cataracts cover a broad spectrum of severity: whereas some lens opacities do not progress and are visually insignificant, others can produce profound visual impairment.

Congenital cataracts may be unilateral or bilateral. 

Causes

Unlike most cataracts, which occur with age, congenital cataracts are present at birth.

Congenital cataracts are rare. In most patients, no cause can be found.Congenital cataracts often occur as part of the following birth defects:

Chondrodysplasia syndrome

Congenital rubella

Conradi syndrome

Down syndrome (trisomy 21)

Ectodermal dysplasia syndrome

Familial congenital cataracts

Galactosemia

Hallerman-Streiff syndrome

Lowe syndrome                                                 

Marinesco-Sjogren syndrome

Pierre-Robin syndrome

Trisomy 13

Symptoms

Congenital cataracts usually look different than other forms of cataract.

Symptoms include:

Gray or white cloudiness of the pupil (which is normally black)

Infant doesn't seem to be able to see (if cataracts are in both eyes)

"Red eye" glow of the pupil is missing in photos, or is different between the two eyes

Unusual rapid eye movements (nystagmus)

Exams and Tests

To diagnose congenital cataract, the infant should have a complete eye examination by an ophthalmologist. The infant may also need to be examined by a pediatrician who is experienced in treating inherited disorders. Blood tests or x-rays may also be needed.

Treatment

If congenital cataracts are mild and do not affect vision, they may not need to be treated, especially if they are in both eyes.

Moderate to severe cataracts that affect vision, or a cataract that is in only one eye, will need to be treated with cataract surgery. In most (noncongenital) cataract surgeries, an artificial intraocular lens (IOL) is inserted into the eye. The use of IOLs in infants is controversial. Without an IOL, the infant will need to wear a contact lens.

Patching to force the child to use the weaker eye is often needed to prevent amblyopia.

The infant may also need to be treated for the inherited disorder that is causing the cataracts.

Outlook (Prognosis)

Removing a congenital cataract is usually a safe, effective procedure. The child will need follow-up for vision rehabilitation. Most infants have some level of "lazy eye" (amblyopia) before the surgery and will need to use patching.