Congenital and childhood cataract
A cloudy lens in a baby or child blocks the image the brain needs to learn to see. Dense ones are operated on in the first weeks of life.
What it is
An opacity of the lens present at birth or appearing in childhood. Because the visual system develops during the first months and years, a dense cataract causes deprivation amblyopia that becomes permanent unless removed early. It is a leading cause of treatable childhood blindness.
Who gets it
One of the commonest causes of treatable visual impairment in children. Many cases are isolated or inherited; others come from infections in pregnancy, metabolic or chromosomal diseases.
Causes
- Inherited (often autosomal dominant)
- Infections in pregnancy (rubella, toxoplasmosis, cytomegalovirus, herpes, syphilis)
- Metabolic disease (galactosaemia)
- Chromosomal syndromes (Down syndrome)
- Trauma or steroid use in older children
- Idiopathic
Risk factors
- Family history of childhood cataract
- Maternal infection in pregnancy
- Prematurity
- Chromosomal and metabolic disorders
Pathophysiology
A disturbance in the formation or metabolism of the lens fibres makes them opaque. The blurred image reaching the retina during the critical period prevents normal development of the visual pathway, producing amblyopia and often nystagmus if both eyes are affected.
Symptoms and signs
- White or dull pupil (leukocoria) or an absent red reflex
- Poor fixation and following
- Wandering eyes (nystagmus) in bilateral dense cataract
- Strabismus
- Photophobia
Diagnosis
- Clinical
- Detected by the red reflex test at birth and at well-child visits, confirmed by ophthalmology with a dilated exam.
- Tests
- Infection serology and metabolic tests (such as for galactosaemia) for bilateral cases without a family history.
- Imaging
- Ocular ultrasound if the fundus cannot be seen, to exclude retinoblastoma and retinal detachment.
Differential diagnosis
- Retinoblastoma
- Retinopathy of prematurity
- Persistent fetal vasculature
- Coats disease
Goal of treatment
Clear the visual axis early and rehabilitate vision to prevent amblyopia.
Treatment
- Surgical
- Cataract surgery within the first weeks of life for dense cataracts; partial ones may be observed.
- Conservative
- Optical correction after surgery with contact lenses, glasses or an intraocular lens.
- Conservative
- Amblyopia treatment with patching of the better eye.
- Follow-up
- Lifelong follow-up for glaucoma after cataract surgery.
Complications
- Permanent deprivation amblyopia
- Nystagmus
- Strabismus
- Glaucoma after surgery
Prognosis
Depends on the age at surgery and adherence to optical correction and patching; early treatment gives the best vision.
Prevention
- Rubella vaccination before pregnancy
- Red reflex test at birth and every well-child visit
Test yourself
Think of the answer, then tap the question to see it.
Why must a dense congenital cataract be operated on in the first weeks of life?
To prevent deprivation amblyopia during the critical period of visual development.
Which screening test detects congenital cataract?
The red reflex test at birth and at well-child visits.
Which infection in pregnancy classically causes congenital cataract?
Rubella.
Why it happens
Connections
Educational information, not medical advice. For any symptom, see a health professional.
Sources: Pediatric Cataract (StatPearls Publishing (NCBI Bookshelf), 2026); Red Reflex (StatPearls Publishing (NCBI Bookshelf), 2023).
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Representative model, for educational purposes only.