Primary congenital glaucoma
Raised eye pressure in infancy from an abnormal drainage angle: a watery, light-sensitive baby with large, cloudy corneas. Surgery is needed early.
What it is
A glaucoma of infancy caused by abnormal development of the drainage angle. The infant's eye is elastic, so the raised pressure stretches it: the cornea enlarges and becomes cloudy. Unlike adult glaucoma it is treated mainly with surgery, and delay causes permanent damage to the optic nerve.
See it in 3D
Who gets it
Rare. Usually diagnosed in the first year of life, more often in boys, and in both eyes in most cases; more frequent where consanguinity is common.
Causes
- Developmental anomaly of the trabecular meshwork and angle
- Inherited forms (often autosomal recessive)
Risk factors
- Family history
- Parental consanguinity
Pathophysiology
The angle structures fail to mature, so aqueous humour cannot drain. Pressure rises and stretches the young eye: the cornea enlarges, breaks in Descemet membrane let fluid into the cornea (cloudiness, horizontal lines) and the optic nerve is damaged.
Symptoms and signs
- Excessive tearing
- Light sensitivity (photophobia)
- Squeezing of the eyelids (blepharospasm)
- Large or cloudy cornea
- Large-looking eyes (buphthalmos)
Diagnosis
- Clinical
- Suspected clinically and confirmed by ophthalmology, usually under anaesthesia: corneal diameter, eye pressure, gonioscopy and optic disc exam.
- Tests
- Measurement of corneal diameter and axial length; genetic counselling when inherited.
- Imaging
- Ocular ultrasound for axial length when the view is poor.
Differential diagnosis
- Congenital nasolacrimal duct obstruction (tearing without photophobia or corneal changes)
- Megalocornea
- Birth trauma to the cornea
- Congenital corneal dystrophies
Goal of treatment
Lower the eye pressure surgically as early as possible and treat amblyopia.
Treatment
- Surgical
- Angle surgery (goniotomy or trabeculotomy) as the first-line treatment.
- Medical
- Pressure-lowering drops as a bridge before surgery or as an adjunct.
- Conservative
- Refraction, glasses and amblyopia treatment.
- Follow-up
- Lifelong pressure and optic nerve follow-up.
Complications
- Permanent optic nerve damage and blindness
- Corneal scarring
- Amblyopia and high myopia
- Retinal detachment in very large eyes
Prognosis
Good pressure control in many children with early surgery; visual outcome depends on corneal clarity and amblyopia treatment.
Prevention
- Early recognition at well-child visits
- Genetic counselling for affected families
Test yourself
Think of the answer, then tap the question to see it.
What is the classic triad of primary congenital glaucoma?
Tearing, light sensitivity and squeezing of the eyelids, with an enlarged or cloudy cornea.
What is the first-line treatment?
Angle surgery (goniotomy or trabeculotomy).
Why does the infant's eye enlarge?
Because the young eye wall is elastic and stretches under raised pressure.
Why it happens
Connections
Educational information, not medical advice. For any symptom, see a health professional.
Sources: Primary Congenital Glaucoma (StatPearls Publishing (NCBI Bookshelf), 2024).
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Representative model, for educational purposes only.