Eye
Eye
Cataract - close-up of the eye
Cataract - close-up of the eye
Rubella Syndrome
Rubella Syndrome
Cataract
Cataract

Congenital cataract

Definition:
A cataract is any clouding of the lens of the eye. a congenital cataract is an opacity of the lens that occurs in the fetus at some time during pregnancy and is present at birth.

Alternative Names:
Cataract - congenital

Causes, incidence, and risk factors:

Although there are many diseases and inherited disorders that can lead to congenital cataracts, the actual incidence of congenital cataracts is low. In most patients with congenital cataract, no specific cause can be identified.

This is a partial list of possible causes of congenital cataracts:

  • galactosemia
  • chondrodysplasia syndrome
  • congenital rubella syndrome
  • Down's syndrome (trisomy 21)
  • Pierre-Robin syndrome
  • familial congenital cataracts
  • Hallerman-Streiff syndrome
  • cerebrohepatorenal syndrome (Lowe's syndrome)
  • Trisomy 13
  • Conradi syndrome
  • Ectodermal dysplasia syndrome
  • Marinesco-Sjogren syndrome


Symptoms:
  • Opacity of the lens, often evident at birth without special viewing equipment and appearing as a whitish discoloration in an otherwise normally dark pupil.
  • Failure of an infant to show visual awareness of the world around him or her (if 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 with possible blood tests and x-rays.



Treatment:

The treatment involves surgical cataract removal followed by placement of an intraocular lens (IOL). Patching to force use of the weaker eye may be required to prevent amblyopia. If an underlying disorder is found, treatment of that disorder may be needed.



Expectations (prognosis):

Cataract surgery with IOL placement is routine and usually has excellent results.



Complications:

Many of the underlying diseases associated with congenital cataract have extensive involvement of multiple organs and organ systems.



Calling your health care provider:

Call for an appointment with your baby's health care provider if you notice that the pupil (the normally dark part in the center of the eye) of one or both eyes appears white or cloudy.



Prevention:

If you have a family history of any of the above inheritable disorders consider seeking genetic counseling.




Review Date: 6/6/2001
Reviewed By: Edward B. Feinberg, M.D., M.P.H., Department of Ophthalmology, Boston University School of Medicine and Harvard Medical School, Boston, MA. Review provided by VeriMed Healthcare Network.

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