Drexel University College of Medicine

Diseases and Conditions

Congenital cataract

Definition

A congenital cataract is clouding of the lens of the eye that is present at birth. The lens of the eye is normally a clear structure, which focuses light received by the eye onto the retina.

Alternative Names

Causes, incidence, and risk factors

The number of people born with cataracts is low. In most patients, no specific cause can be found. Possible causes of congenital cataracts include the following:

• 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

• Cloudiness of the lens that looks like a white spot in an otherwise normally dark pupil -- often obvious at birth without special viewing equipment
• Failure of an infant to show visual awareness of the world around him or her (if cataracts present in both eyes)
• Nystagmus (unusual rapid eye movements)

Signs and tests

A complete eye examination by an ophthalmologist will readily diagnose congenital cataract. The search for a possible cause may require examination by a pediatrician experienced in hereditary disorders and possible blood tests or x-rays.

Treatment

In some cases, congenital cataracts are mild and not visually significant, and these cases require no treatment. Moderate to severe cataracts that affect vision will require surgical cataract removal, followed by placement of an artificial intraocular lens (IOL). Patching to force the child to use the weaker eye may be required to prevent amblyopia.

Treatment for any underlying disorder may also be needed.

Expectations (prognosis)

Cataract removal surgery with placement of an artificial intraocular lens (IOL) is routine, and usually has excellent results.

Complications

Many of the underlying diseases associated with congenital cataract involve many organs to a great degree.

Calling your health care provider

Call for an urgent appointment with your baby's health care provider if you notice that the pupil of one or both eyes appears white or cloudy.

Prevention

If you have a family history of inheritable disorders that could cause congenital cataracts, consider seeking genetic counseling.

References

Guercio JR, Martyn LJ. Congenital malformations of the eye and orbit. Otolaryngol Clin North Am. 2007 Feb;40(1):113-40, vii.

Olitsky SE, Hug D, and Smith LP. Abnormalities of the lens. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. St. Louis, Mo: WB Saunders; 2007; chap 627.

Physician Reference

International Classification of Diseases, 9^th Revision (ICD9)

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