Congenital cataract

Alternative names

Cataract - congenital

Definition

A congenital cataract involves clouding of the lens of the eye that is present at birth.

Causes, incidence, and risk factors

The number of people born with cataracts is low. Possible causes of congenital cataracts include the following:

• Galactosemia
  
• Chondrodysplasia syndrome
  
• Congenital rubella syndrome
  
• Down syndrome (trisomy 21)
  
• Pierre-Robin syndrome
  
• Familial congenital cataracts
  
• Hallerman-Streiff syndrome
  
• Lowe syndrome
  
• Trisomy 13
  
• Conradi syndrome
  
• Ectodermal dysplasia syndrome
  
• Marinesco-Sjogren syndrome
  

Although many diseases and inherited disorders can lead to congenital cataracts, in most patients, no specific cause can be identified.

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 and possible blood tests or X-rays.

Treatment

The treatment involves surgical cataract removal followed by placement of an 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 be needed.

Expectations (prognosis)

Cataract 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 have extensive involvement of multiple organs and organ systems.