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Congenital cataract and congenital glaucoma
Congenital cataract and congenital glaucoma are two eye emergencies of infancy: a significant cataract blocks the visual stimulus and requires surgery in the first weeks of life to prevent dense amblyopia, and congenital glaucoma — with watering, photophobia and a large cloudy cornea — is treated with surgery, not drops.
Why it matters in the exam
Congenital cataract: found on the red reflex test. A significant unilateral cataract is operated on by about 6 weeks of age, and a bilateral one by 8–10 weeks, because afterwards amblyopia and nystagmus are hard to reverse. Most unilateral cases are idiopathic; in bilateral cases a cause is sought: dominant inheritance, galactosaemia ("oil droplet" cataract that regresses with diet), congenital infections — mainly rubella, Down syndrome, Lowe syndrome (oculocerebrorenal, X-linked) and hypocalcaemia. The IATS study showed that in an infant under 7 months an intraocular lens is not better for vision than a contact lens, and needs more repeat operations.
After surgery: a contact lens or glasses to correct aphakia, intensive amblyopia treatment and lifelong follow-up for glaucoma, which develops in a substantial share of operated children. Primary congenital glaucoma: a triad of watering, photophobia and blepharospasm; on examination — corneal diameter above 12 mm in the first year, a cloudy cornea, horizontal breaks in Descemet membrane (Haab striae), an enlarged eye (buphthalmos) and a cupped disc. The common gene is CYP1B1, inherited recessively, so it is commoner with consanguineous marriage.
Treatment of congenital glaucoma is surgical — goniotomy or trabeculotomy — and drops only bridge to surgery. Glaucoma in children also appears as part of syndromes: Sturge-Weber (a facial port-wine stain), aniridia (the PAX6 gene, and with a chromosomal deletion — WAGR with a risk of Wilms tumour), Axenfeld-Rieger and neurofibromatosis. Watering in an infant without photophobia and with a normal cornea is usually nasolacrimal duct obstruction — not glaucoma.
Key points
- Absent red reflex → urgent referral.
- Unilateral cataract by 6 weeks; bilateral by 8–10 weeks.
- Bilateral cataract — search for a cause: TORCH, galactosaemia, genetics.
- Congenital glaucoma: watering, photophobia, blepharospasm, cornea above 12 mm.
- Treatment of congenital glaucoma: goniotomy or trabeculotomy.
Summary table
| Condition | Signs | Work-up | Treatment |
|---|---|---|---|
| Unilateral cataract | Absent red reflex in one eye | Usually idiopathic | Surgery by 6 weeks, contact lens, patching |
| Bilateral cataract | Absent red reflex in both eyes, nystagmus | TORCH, galactosaemia, calcium, genetics | Surgery by 8–10 weeks |
| Galactosaemia | "Oil droplet" cataract, jaundice, vomiting | GALT, urinary reducing substances | Galactose-free diet |
| Congenital glaucoma | Watering, photophobia, large cloudy cornea | Pressure, corneal diameter under anaesthesia | Goniotomy or trabeculotomy |
| Nasolacrimal duct obstruction | Watering and discharge, normal cornea | Clinical | Massage; probing if it persists |
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Sources
- AAO Preferred Practice Pattern: Pediatric Eye Evaluations, 2022 — Red reflex and referral
- Infant Aphakia Treatment Study (IATS) Group, 2014 — Contact lens versus intraocular lens after unilateral congenital cataract surgery
- AAO Basic and Clinical Science Course, Section 6: Pediatric Ophthalmology and Strabismus, 2024-2025 — Childhood cataract and childhood glaucoma
Sources are given for further reading; the text is written afresh and not copied from them.
Reviewed by the Examqo AI Professor · 2026-11-06. The text may contain errors; when in doubt, check the source.