Paeds · ophthalmology
Congenital cataract and glaucoma
Also known as Infantile cataract · Congenital cataract · Primary congenital glaucoma · Buphthalmos · Aphakic glaucoma · Childhood glaucoma · Leukocoria
Fellowship topic on congenital cataract and glaucoma: why a lens opacity or a raised intraocular pressure in the first weeks of life is an emergency for the developing visual system. Covers deprivation amblyopia and the critical-period surgical window for dense unilateral cataract (by about 6 weeks) and bilateral cataract (by 6 to 10 weeks); the Childhood Glaucoma Research Network classification of paediatric glaucoma into primary congenital glaucoma (PCG), glaucoma with non-acquired ocular anomalies (Peters, aniridia, Axenfeld-Rieger), glaucoma with non-acquired systemic syndromes (Sturge-Weber, NF1), and acquired secondary glaucoma including glaucoma following cataract surgery; the classic PCG triad of epiphora, photophobia and blepharospasm with buphthalmos and Haab striae; the red-reflex (Brückner) test and the leukocoria differential including retinoblastoma; examination under anaesthesia with intraocular pressure, corneal diameter and disc cupping; the Infant Aphakia Treatment Study evidence on aphakia versus primary intraocular lens; the surgical ladder for PCG (goniotomy, 360-degree trabeculotomy, trabeculectomy with mitomycin C, drainage device, cycloablation); the drugs to avoid in the infant eye including brimonidine; and lifelong glaucoma surveillance after cataract surgery.
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Target exams
Red flags
- A white pupil reflex (leukocoria) or an absent, dark or asymmetric red reflex in any infant — refer urgently to paediatric ophthalmology; retinoblastoma and a dense congenital cataract are the diagnoses not to miss
- The triad of epiphora, photophobia and blepharospasm with corneal clouding or an enlarged eye (buphthalmos) in an infant — primary congenital glaucoma; untreated it causes irreversible blindness
- Glaucoma developing weeks to years after infantile cataract surgery (aphakic glaucoma) — it is often silent; every postoperative cataract child needs lifelong intraocular-pressure surveillance
- A child with unilateral cataract, microphthalmia and a retrolental mass — persistent fetal vasculature; the retina may be detached, and surgery carries a high risk of glaucoma and haemorrhage
- Sporadic aniridia — screen for Wilms tumour with renal ultrasound, because a contiguous 11p13 deletion may include the WT1 gene (WAGR syndrome)
Life stages
Care settings
Clinical exam formats
Board mappings
- Recognise leukocoria and an abnormal red reflex; arrange urgent ophthalmology referral; understand deprivation amblyopia and the surgical time-window
- Distinguish primary congenital glaucoma from secondary aphakic glaucoma; interpret corneal diameter, Haab striae and intraocular pressure; counsel on genetics and prognosis
- Newborn and infant red-reflex screening; buphthalmos and the epiphora-photophobia-blepharospasm triad as red flags; timing of cataract surgery
- Long case of a child with congenital cataract: amblyopia management, patching, aphakia correction and postoperative glaucoma surveillance
- Newborn and infant eye examination; recognition of vision-threatening eye disease; the retinoblastoma differential of leukocoria
- Causes of leukocoria; congenital glaucoma triad; inherited cataract syndromes; red-reflex technique
- Short case of an abnormal red reflex or buphthalmos; communication station breaking the news of cataract and patching
- Newborn discharge eye examination; cataract as a cause of deprivation amblyopia; recognition of primary congenital glaucoma
- Newborn examination competency; urgent referral for leukocoria and abnormal red reflex; amblyopia principles
- Structured oral on the differential of leukocoria, the management of congenital glaucoma, and counselling for congenital cataract
A baby is born with a visual system that is wired only in rough draft. Over the first months and years of life the brain refines that wiring, keeping the connections that carry a sharp, matching picture from each eye and pruning the ones that do not. Anything that blurs the picture during this critical and sensitive period — a cataract, or the corneal oedema and optic-nerve damage of glaucoma — permanently weakens the pathway from that eye. That permanent weakness is amblyopia, and it is the reason these two conditions are time-critical and the reason a general paediatric clinician sits at the front door of both of them. Most cases are found, or missed, at the red-reflex examination. [11]
This topic covers both diseases together because they share that single clock, because glaucoma is itself the commonest serious complication of cataract surgery, and because an examiner who asks about one will follow with the other. The cataract half is about recognising an abnormal red reflex and operating before amblyopia is locked in; the glaucoma half is about recognising the tearing, light-averse infant and re-establishing aqueous outflow before the globe is damaged. [3]
Overview & Definition
A cataract is any opacity of the crystalline lens. In an adult it dims vision slowly over years; in an infant it blocks picture brain needs to finish wiring the eye, so the harm accrues in weeks. A congenital cataract is present at birth or recognised in the first months, and an infantile cataract appears within the first year; the distinction matters less than the density and the timing, because both demand urgent decisions about surgery. [1]
Glaucoma in a child means sustained damage to the optic nerve from raised intraocular pressure (IOP), and in infancy the pressure does something it never does in an adult: because the infant sclera is elastic, the eye enlarges under pressure. That enlarged, tearing, light-hating eye is buphthalmos ("ox eye"), and it is the hallmark of primary congenital glaucoma (PCG), the commonest glaucoma of infancy, caused by maldevelopment of the anterior-chamber angle. [2]
Two definitions carry the whole topic. Deprivation amblyopia is the permanent cortical blindness that follows when a sharp image is withheld from an immature eye — the engine of urgency in cataract. And aphakic glaucoma (more precisely, glaucoma following cataract surgery) is the delayed, often silent rise in pressure that can blind a child who did well from their cataract operation — the reason every postoperative cataract child is a glaucoma patient for life. [3]
References12ShowHide
- [1]Haargaard B; Wohlfahrt J; Fledelius HC; Rosenberg T; Melbye M A nationwide Danish study of 1027 cases of congenital/infantile cataracts: etiological and clinical classifications. Ophthalmology, 2004.PMID 15582089
- [2]Biglan AW Glaucoma in children: are we making progress? J AAPOS, 2006.PMID 16527674
- [3]Rabiah PK Frequency and predictors of glaucoma after pediatric cataract surgery. Am J Ophthalmol, 2004.PMID 14700641
- [4]Mataftsi A; Haidich AB; Kokkali S; et al Postoperative glaucoma following infantile cataract surgery: an individual patient data meta-analysis. JAMA Ophthalmol, 2014.PMID 24921712
- [5]Freedman SF; Lynn MJ; Beck AD; et al Glaucoma-Related Adverse Events in the First 5 Years After Unilateral Cataract Removal in the Infant Aphakia Treatment Study. JAMA Ophthalmol, 2015.PMID 25996491
- [6]Solebo AL; Rahi JS; British Congenital Cataract Interest Group Glaucoma following cataract surgery in the first 2 years of life: frequency, risk factors and outcomes from IoLunder2. Br J Ophthalmol, 2020.PMID 31586948
- [7]Repka MX; Sutherland DR; Hatt SR; et al Effects of Age at Surgery and Laterality of Cataract on Visual Acuity 5 Years after Surgery in Infants Left Aphakic. Ophthalmology, 2025.PMID 40582417
- [8]Abbas J; Haider F; Arooj H; et al Comparison of 360-Degree Trabeculotomy Versus Traditional Angle Surgery in Primary Congenital Glaucoma: A Systematic Review and Meta-Analysis. J Glaucoma, 2026.PMID 41875194
- [9]Thau A; Lloyd M; Freedman S; et al New classification system for pediatric glaucoma: implications for clinical care and a research registry. Curr Opin Ophthalmol, 2018.PMID 30096087
- [10]Ngo AM; Kraus CL Postoperative guidelines following pediatric cataract and glaucoma surgeries: a survey of preferred surgeon instructions. J AAPOS, 2025.PMID 39863265
- [11]Toli A; Perente A; Labiris G Evaluation of the red reflex: An overview for the pediatrician. World J Methodol, 2021.PMID 34631483
- [12]Anderson J Don't Miss This! Red Flags in the Pediatric Eye Examination: Abnormal Red Reflex. J Binocul Vis Ocul Motil, 2019.PMID 31329054