Cataract is opacification of lens. Congenital cataract is diagnosed at birth. If a cataract goes unnoticed in infancy, permanent visual loss may occurs. Not all cataracts are visually significant. If a lenticular opacity is in the visual axis, it is considered visually significant and may lead to blindness.

 

Unilateral cataracts are usually isolated sporadic in incidence. They are associated with eye abnormalities (posterior lenticonus, persistent hyperplastic primary vitreous, posterior pole tumors), trauma, or intrauterine infection (rubella).

Bilateral cataracts are mostly inherited and associated with other diseases. The common causes are hypoglycemia, trisomy (eg, Down, Edward, and Patau syndromes), myotonic dystrophy, infectious diseases (eg, toxoplasmosis, rubella, cytomegalovirus, and herpes simplex [TORCH]), and prematurity.

Causes

• Intrauterine infections- toxoplasmosis, rubella, cytomegalovirus, and herpes simplex [TORCH]

Other causes

• Galactosemia
• Chondrodysplasia syndrome
• Down syndrome
• Edward syndrome
• Pierre-Robin syndrome
• Familial congenital cataracts
• Lowe syndrome
• Sjögren syndrome

Signs and symptoms

• Congenital cataracts are present at birth but are mostly being identified in later in life.
• Some cataracts are static (anterior polar cataract and nuclear), but some are progressive (lamellar, sutural, posterior lenticonus)
• Irregular red reflex is hallmark of visual problems
• White reflex (leukocoria) can be presenting sign of cataract
• Cloudiness of eyes

Clinical types

• Sutural cataract
• Lamellar cataract
• Zonular cataract
• Total  cataract

Complications

• Loss of vision even after surgical and optical treatment
• Ambylopia
• Glaucoma
• Strabismus
• Retinal detachment

Tests and diagnosis

For unilateral cataract- TORCH titers and VDRL test.

For bilateral cataract

• Fundoscopy and slit lamp examination
• Complete blood test
• Blood urea nitrogen and serum creatinine
• TORCH titers and VDRL test
• Urine for reducing substance and amino acids
• Hearing tests

Frequent eye examinations and glaucoma screening is required.

Treatment

• Cataract surgery is the treatment of choice and should be performed when child is younger than 17 weeks to ensure minimal or no visual deprivation.
• Extracapsular cataract extraction with primary posterior capsulectomy and anterior vitrectomy is the procedure of choice.
• To treat the underlying cause