Glaucoma
Primary Congenital (Infantile) Glaucoma
Also known as: congenital glaucoma, infantile glaucoma, PCG, buphthalmos, Haab striae, baby with watery cloudy eye
Filed as: Urgent
Source-cited from the record's own evidence list. Clinical content programme led by Dr Ankit Mathur, PhD, Grad Cert Ocu Thera, B.S. Optom. How the clinical content is governed
Clinical decision support only
Overview
Primary congenital (infantile) glaucoma is a treatable cause of childhood blindness caused by a developmental abnormality of the trabecular meshwork, presenting with raised intraocular pressure in infancy. Most cases (about 80%) present within the first year of life and 70-80% are bilateral. It is a surgical disease — the optometrist's role is early recognition and urgent referral, because optic-nerve cupping is reversible in the elastic infant eye if treated early, whereas untreated disease progresses to irreversible blindness.
Recognition
Symptoms: what patients report
- Excessive watering / tearing (epiphora) in an infant.
- Light sensitivity (photophobia) — the baby turns away from light or buries the face.
- Eyelid squeezing / spasm (blepharospasm).
- A cloudy or hazy cornea, or an eye that looks larger than the other.
Signs: what the examination shows
- Corneal oedema / haze (present in essentially all affected newborns, diffuse or localised).
- Enlarged corneal diameter / buphthalmos (>11.5 mm at birth, >12 mm under 1 year, >13 mm over 1 year; normal newborn ~9.5-10 mm).
- Haab striae — horizontal breaks in Descemet's membrane; a defining sign of congenital glaucoma.
- Globe enlargement.
- Raised intraocular pressure (>21 mmHg; normal in children ~12 mmHg).
- Progressive optic-nerve cupping (reversible if treated early).
- Dull, irregular, or absent red reflex.
What OptoGuide™ covers for primary congenital (infantile) glaucoma
- Pattern reasoning: what this combination of findings points to, and its differentiators
- Don't-miss risks and escalation triggers
- Management tiers with linked Australian therapeutics
- Referral urgency, specialty, and letter drafting
Sources
- Primary Congenital Glaucoma — EyeWiki
- Prevalence of FOXC1 Variants in Individuals With a Suspected Diagnosis of Primary Congenital Glaucoma. Siggs OM, Souzeau E, Pasutto F, et al. JAMA Ophthalmology. 2019;137(4):348-355.
- Approach to Childhood Glaucoma: A Review. Karaconji T, Zagora S, Grigg JR. Clinical & Experimental Ophthalmology. 2022;50(2):232-246.
- Ocular Disorders in the Newborn. Mehner LC, Singh JK. NeoReviews. 2021;22(7):e461-e469.
Standard texts: Kanski's Clinical Ophthalmology: A Systematic Approach, The Wills Eye Manual, Oxford Handbook of Ophthalmology, Oxford American Handbook of Ophthalmology, Signs in Ophthalmology: Causes & Differential Diagnosis.