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• Congenital anomaly


  Condition existing at or before birth regardless of cause
• Types of congenital conditions

  • Congenital - Present at birth
  • Infantile - Develops within the first 6 months of life
  • Developmental - Develops after birth though may be related to abnormality present at birth or appearing soon after
• Ophthalmia Neonatorum


  Conjunctivitis affecting a newborn within first month of life
• Ophthalmia Neonatorum


  • A bacterial, chlamydial or viral infection acquired during birth
  • Most commonly caused by Chlamydia trachomatis or Neisseria gonorrhoeae (sexually transmitted diseases)
  • Emergency referral required to Ophthalmologist for treatment with anti-viral / antibiotic drug
• Signs and Symptoms of Ophthalmia Neonatorum

  • Redness
  • Mucopurulent discharge
  • Lid oedema
  • Conjunctival oedema (chemosis)
  • Usually bilateral may be asymmetrical
  • Cornea may be affected by N Gonorrhoeae - may be perforated
• Orbital Cellulitis


  Infection of orbital tissue
• Orbital Cellulitis


  • Redness, swelling and tenderness of lids, pain, double vision, blurred vision, proptosis, fever, general malaise
  • Can lead to meningitis, brain abscess, cavernous sinus thrombosis
  • Emergency referral - can be sight and life threatening
• Periorbital/Preseptal cellulitis


  Infection of superficial lids and tissue around eye
• Periorbital/Preseptal cellulitis


  • Swollen and red lids, but no effects on vision and no proptosis
  • More common and less serious than orbital cellulitis
  • Can progress to orbital cellulitis
• Blocked nasolacrimal duct


  Common in babies due to incompletely developed tear duct
• Blocked nasolacrimal duct


  • Causes recurrent conjunctivitis, watery eyes (epiphora) and discharge
  • Usually self-resolves as the baby grows
  • May cause infection of lacrimal sac (dacryocystitis), requiring antibiotics (urgently in newborn)
• Allergic (Vernal) conjunctivitis

  Unusual in small babies
• Allergic (Vernal) conjunctivitis


  • Watery, red, itchy eyes
• Retinopathy of Prematurity (ROP)


  Due to birth before complete development of retinal circulation into the periphery
• Retinopathy of Prematurity (ROP)

  • Retinal hypoxia triggers release of growth factors which cause neovascularisation and tortuosity
  • Can be exacerbated by supplemental oxygen
  • May be mild and resolve spontaneously or can be severe and cause vitreous haemorrhage, scarring, and can lead to myopia, strabismus, retinal detachment, visual impairment, acute glaucoma
  • Premature babies screened regularly by Ophthalmologist
  • Treatment (if necessary) laser of peripheral retina
• Stages of Retinopathy of Prematurity (ROP)

  • Mild ROP
  • Moderate ROP
  • Advanced ROP
• Anophthalmia


  Absence of eye
• Microphthalmia


  Small eye
• Microphthalmia


  • Associated with hyperopia, cataract, persistent hyperplastic primary vitreous, retinal dysplasia
  • Causes strabismus, glaucoma, nystagmus
• Buphthalmos


  Enlarged eye
• Buphthalmos


  • Usually due to congenital glaucoma
  • Most bilateral
  • Untreated will lead to eventual blindness
  • Symptoms: hazy vision, excessive lacrimation, photophobia
  • Signs: bulging eye, increased IOP, distortion of the optic disc, corneal oedema, enlarged cornea, myopia, strabismus
• Leukocoria


  White pupillary reflex
• Causes of Leukocoria


  • Persistent hyperplastic primary vitreous
  • Congenital cataract
  • Coloboma
  • Advanced ROP
  • Coat's disease
  • Retinoblastoma
• Coloboma


  Failure of the ectodermal optic vesicle fissure to fully close
• Coloboma


  • Unilateral or bilateral
  • Anterior segment colobomas: cornea, eyelid, ciliary body, lens
  • Posterior segment colobomas: choroid, retina, (can cause leukocoria), optic nerve
  • Posterior segment coloboma associated with VF defect
• Anisocoria


  Difference in pupil size between eyes
• Causes of Anisocoria


  • Physiological anisocoria (20% population)
  • Abnormally large pupil: Aniridia, Holmes Adie's pupil, Third nerve palsy
  • Abnormally small pupil: Horner's Syndrome
  • Anomalies of shape: Iris coloboma, Ectopic pupil (pupil displaced from normal position)
• Port-wine stain


  30% develop ipsilateral glaucoma
• Persistent Pupillary Membrane


  Remnants of a foetal membrane
• Marcus Gunn jaw winking syndrome


  Branch of mandibular division of 5th nerve misdirected to levator
• Marcus Gunn jaw winking syndrome


  • Retraction ptotic lid on chewing, opening mouth, contralateral jaw movement
• Infantile esotropia


  Onset before 6 months, constant large angle esotropia, cross fixation common
• Infantile esotropia


  • Amblyopia is infrequent
• Neurogenic palsies


  • Congenital III, IV, VI nerve paresis
  • Mechanical Palsies (Brown's, Duane's)
  • Nystagmus
• Optic Disc Coloboma


  Bowl shaped excavation, may be mistaken for glaucoma
• Optic Disc Coloboma


  • Visual acuity very poor
  • Disc posterior to globe, blood vessels radiate from disc centre, core of glial tissue
  • Can cause serous macular detachment later in life
• Optic Nerve Hypoplasia


  Commonest optic nerve anomaly
• Optic Nerve Hypoplasia


  • Isolated or associated with endocrinopathies (hormone deficiencies), developmental delay, and brain malformations
  • Small grey discs, fewer axons
  • Tortuous vessels
  • VA from no light perception to near normal
  • Associated with nystagmus and strabismus
• Possible causes of a red eye in a child

  • Blocked nasolacrimal duct
  • Subtarsal foreign body
  • Conjunctivitis (allergic, bacterial, viral)
  • Keratitis
  • Dacryocystitis
• Questions to ask when a child has a red eye

  • Age - is it under 1 month (ophthalmia neonatorum)?
  • Is there discharge - mucopurulent (bacterial conjunctivitis/keratitis) or watery (allergic, viral conjunctivitis)?
  • Are the lids swollen (ophthalmia neonatorum, orbital/periorbital cellulitis, dacryocystitis)?
  • Is the child generally unwell (orbital cellulitis)?