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Congenital cataract

Definition

A congenital cataract is a clouding of the lens of the eye that is present at birth. The lens of the eye is normally clear. It focuses light that comes into the eye onto the retina.

Alternative Names

Cataract - congenital

Causes

Unlike most cataracts, which occur with aging, 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:

  • An infant doesn’t seem to be visually aware of the world around him or her (if cataracts are in both eyes)
  • Gray or white cloudiness of the pupil (which is normally black)
  • The "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 removal 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.

Possible Complications

With cataract surgery there is a very slight risk of:

  • Bleeding
  • Infection
  • Inflammation

Infants who have surgery for congenital cataracts are likely to develop another type of cataract, which may need further surgery or laser treatment.

Many of the diseases that are associated with congenital cataract can also affect other organs.

When to Contact a Medical Professional

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, or if the child seems to ignore part of their visual world.

Prevention

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

References

Junk AK, Morris DA. Cataracts and systemic disease. In: Tasman W, Jaeger EA, eds. Duane's Ophthalmology. 16th ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2013:chap 41.

Heitmancik JF, Datilles M. Congenital and inherited cataracts. In: Tasman W, Jaeger EA, eds. Duane's Ophthalmology. 16th ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2013:chap 74.

Dahan E. Pediatric cataract surgery. In: Yanoff M, Duker JS, eds. Ophthalmology. 3rd ed. St. Louis, Mo: Mosby Elsevier; 2008:chap 5.13.


Review Date: 8/24/2013
Reviewed By: Franklin W. Lusby, MD, Ophthalmologist, Lusby Vision Institute, La Jolla, California. Also reviewed by David Zieve, MD, MHA, Bethanne Black, and the A.D.A.M. Editorial team.
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