Ophthalmology: Paediatrics

• Children's eyes: Squint and Amblyopia | Eye Tumours | White Pupil and Retinoblastoma | Retinopathy of Prematurity | Other Childhood Eye Problems
• Revision: Drug Reference Table | Spot the Diagnosis | Exam-style Scenarios | Key Points | Guidelines and Further Reading

Eye Tumours

• Choroidal melanoma (uveal melanoma):
  • The commonest primary intraocular tumour in adults; often found on routine examination (pigmented mass), or blurred vision, flashes, field loss.
  • Spreads to the liver (check LFTs, liver imaging).
  • Treated at specialist centres — plaque brachytherapy, proton beam radiotherapy, or removal of the eye.
  • Metastatic disease — tebentafusp (for people with a specific HLA type).
  • Choroidal naevi are common and benign; monitored for change.
• Metastases to the eye — overall the commonest intraocular tumour (breast and lung cancer).
• Vitreoretinal lymphoma — older people with "uveitis" that doesn't respond to steroids; linked to CNS lymphoma.
• Eyelid tumours — basal cell carcinoma (most), squamous cell carcinoma, sebaceous carcinoma, melanoma, Merkel cell carcinoma.
• Conjunctival tumours — squamous cell carcinoma (HIV, sun exposure), melanoma, lymphoma (salmon-pink patch).
• Orbital tumours — lymphoma, cavernous haemangioma, meningioma, optic nerve glioma (NF1), lacrimal gland tumours, metastases. Present with proptosis, double vision or visual loss.
• Retinoblastoma — children (see below).

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Squint and Amblyopia

• Squint (strabismus) — the eyes point in different directions:
  • Esotropia (turns in), exotropia (turns out), hyper- or hypotropia (up or down).
  • Concomitant (non-paralytic) — the angle is the same in all directions; common in children; often from long sight.
  • Incomitant (paralytic) — varies with gaze; nerve palsy or restriction; needs investigation.
• Tests:
  • Corneal light reflex (Hirschberg) — reflections should be in the same place in each pupil.
  • Cover test — cover the straight eye; the squinting eye moves to take up fixation.
  • Red reflex; acuity.
• Pseudosquint — wide nasal bridge and epicanthic folds make the eyes look turned in; light reflexes are symmetrical.
• Normal — intermittent squint can be normal in the first 3 months of life.
• Amblyopia ("lazy eye"):
  • Reduced vision in an eye that is otherwise healthy, because the brain didn't get a clear image during development.
  • Causes — squint, unequal glasses prescription (anisometropia), blocked vision (cataract, ptosis).
  • Treat early — best results before age 7–8, when the visual system is still developing.
  • Treatment — glasses first; then patching the good eye or atropine drops to blur it.
• Refer any child with a persistent squint; urgently if sudden onset, with a poor red reflex, headache, or other neurological signs (tumour, raised ICP).
• Squint surgery — to straighten the eyes (cosmetic and to help binocular vision).

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White Pupil and Retinoblastoma

• Leukocoria — a white pupil (often first seen in a flash photograph) or an absent red reflex.
• Causes:
  • Retinoblastoma — must be excluded.
  • Congenital cataract.
  • Retinopathy of prematurity; Coats disease; toxocara; persistent fetal vasculature; retinal detachment.
• Retinoblastoma:
  • The commonest eye cancer of childhood — usually under 5.
  • RB1 gene (chromosome 13). Bilateral or family history = heritable (germline) form — also a lifelong risk of other cancers (osteosarcoma, soft tissue sarcomas), especially after radiotherapy.
  • Features: White pupil, new squint, red painful eye, poor vision, family history.
  • Referral (NICE NG12) — absent red reflex: refer urgently (within 2 weeks) for assessment for retinoblastoma. A new squint or change in vision: urgent if there is a family history of retinoblastoma.
  • Treatment at the national centres (London and Birmingham) — chemotherapy (IV or intra-arterial), laser, cryotherapy, plaque radiotherapy, or eye removal. Survival in the UK is over 95%.
  • Genetic testing and screening of siblings and offspring.
• Congenital cataract — found at the newborn and 6–8 week red reflex checks. Needs surgery within weeks to prevent dense amblyopia. Causes: genetic, infection (rubella, CMV, toxoplasmosis), galactosaemia, Down syndrome.

⚠️ Safety Pearl: Take a parent's concern about a "white eye in photos" seriously — examine the red reflex and refer urgently if it is absent or abnormal.

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Retinopathy of Prematurity

• What it is: Abnormal blood vessel growth in the immature retina of premature babies; can cause retinal detachment and blindness.
• Risk factors: Low gestational age and birth weight; high or fluctuating oxygen levels; sepsis.
• Screening (UK guideline): Babies born before 31 weeks or weighing under 1,501 g — dilated eye examinations on the neonatal unit from a few weeks after birth.
• Treatment: Laser to the peripheral retina, or anti-VEGF injection.
• Prevention: Careful oxygen targeting in neonatal care.
• Long-term — higher risk of short sight, squint, amblyopia; follow-up.

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Other Childhood Eye Problems

• Vision screening:
  • Newborn and 6–8 week checks — red reflex (cataract, retinoblastoma).
  • Orthoptist screening at age 4–5 — amblyopia and squint.
• Blocked tear duct (congenital nasolacrimal duct obstruction) — watery, sticky eye from birth; usually clears by 12 months; massage; probing if persistent.
• Congenital glaucoma — large eyes (buphthalmos), cloudy cornea, watering, photophobia. Urgent referral — surgery.
• Non-accidental injury — retinal haemorrhages (many, in several layers, extending to the periphery) in a baby with unexplained brain injury suggest abusive head trauma. Follow safeguarding procedures; ophthalmology examination with photographs.
• Periorbital and orbital cellulitis — see the Red Eye and Eye Emergencies chapter.
• Nystagmus in infancy — may be idiopathic or a sign of poor vision (albinism, congenital cataract, optic nerve problems) or brain disease; refer.
• Myopia in children — increasingly common; more time outdoors helps prevent it; low-dose atropine drops and special lenses can slow progression.
• Allergic eye disease and conjunctivitis — common; see the Red Eye chapter.

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Drug Reference Table

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Spot the Diagnosis

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Exam-style Scenarios

1. A mother says her 18-month-old's left eye "glows white" in photographs. The child has also developed an inward squint on that side. Answer: Suspected retinoblastoma.

• Examine the red reflex; urgent ophthalmology referral (within 2 weeks; sooner per local pathway).
• Treatment at a national retinoblastoma centre; genetic testing (RB1) and sibling screening.

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Key Points

• Anisocoria worse in the dark — small pupil abnormal (Horner); worse in light — large pupil abnormal (third nerve, Adie, drugs).
• Painful Horner syndrome = carotid dissection until proven otherwise; Horner with arm symptoms in a smoker = Pancoast tumour.
• Monocular diplopia is an eye problem (often cataract); binocular diplopia is a nerve, muscle or junction problem.
• Third nerve palsy with a dilated pupil = aneurysm until proven otherwise — CT angiography the same day.
• Fourth nerve: vertical diplopia going downstairs, head tilt; sixth nerve: can't abduct — consider raised ICP.
• INO: MS in the young, stroke in the old. Myasthenia: fatigable ptosis, normal pupils.
• Thyroid eye disease: stop smoking; radioiodine can worsen it; optic neuropathy needs IV steroids and decompression.
• Uveitis can be the first sign of HLA-B27 disease, sarcoid, Behçet's, syphilis, TB or lymphoma.
• Hydroxychloroquine: 5 mg/kg/day or less; yearly retinal screening after 5 years.
• Topiramate can cause bilateral angle closure; vigabatrin causes permanent field loss; tamsulosin affects cataract surgery.
• Amblyopia must be treated early (glasses, patching or atropine) — refer any persistent squint.
• Absent red reflex or white pupil in a child — urgent referral for retinoblastoma or cataract.
• Screen premature babies (under 31 weeks or under 1,501 g) for retinopathy of prematurity.

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Guidelines and Further Reading

• The Royal College of Ophthalmologists: Hydroxychloroquine and chloroquine retinopathy — recommendations on monitoring
• EUGOGO and British Oculoplastic Surgery Society guidance on thyroid eye disease
• NICE NG12: Suspected cancer — recognition and referral (retinoblastoma)
• RCOphth and RCPCH: UK screening guideline for retinopathy of prematurity
• Public Health England / NHS: Newborn and infant physical examination (NIPE) and child vision screening
• DVLA: Assessing fitness to drive (diplopia and visual fields)
• BNF — for current doses

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