Congenital cataract is a lens opacity that is present at birth or shortly after birth.
Congenital cataracts may be sporadic, or they may be caused by chromosomal anomalies, metabolic disease (eg, galactosemia), intrauterine infection (eg, rubella), or other maternal disease during pregnancy. Congenital cataracts may also be an isolated familial anomaly that is commonly autosomal dominantly inherited.
Cataracts may be located in the center of the lens (nuclear), or they may involve the lens material underneath the anterior or posterior lens capsule (subcapsular or cortical). They may be unilateral or bilateral. They may not be noticed unless the red reflex is checked or unless ophthalmoscopy is done at birth. As with other cataracts, the lens opacity obscures vision. Cataracts may obscure the view of the optic disc and vessels and should always be evaluated by an ophthalmologist.
Cataracts are removed by aspirating them through a small incision. In many children, an intraocular lens may be implanted. Postoperative visual correction with eyeglasses, contact lenses, or both is usually required to achieve the best outcome.
After a unilateral cataract is removed, the quality of the image in the treated eye is inferior to that of the other eye (assuming the other eye is normal). Because the better eye is preferred, the brain suppresses the poorer-quality image, and amblyopia develops. Thus, effective amblyopia therapy is necessary for the treated eye to develop normal sight. Some children are unable to attain good visual acuity because of accompanying structural defects. In contrast, children with bilateral cataract removal in which image quality is similar in both eyes more frequently develop equal vision in both eyes.
Some cataracts are partial (posterior lenticonus) and opacify during the first 10 yr of life. Eyes with partial cataracts will have a better visual outcome.