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Referral and warning signs
When to refer
IMMEDIATE URGENT REFERRAL to Paediatric Ophthalmologist/Tertiary Eye Centre (RR) for ANY infant or child suspected of having a cataract (e.g., leukocoria, poor vision).
Red flags
- Leukocoria (white pupil).
- Absent red reflex.
- Nystagmus in an infant.
- Persistent squint.
- Obvious poor visual behaviour in an infant.
Signs and symptoms
- Leukocoria (white pupil / white reflex in photos).
- Poor visual behaviour / failure to fix and follow.
- Nystagmus (dancing eyes).
- Strabismus (squint / crossed eyes).
- Photophobia.
- Cloudy appearance of cornea/pupil (if dense cataract).
Risk factors
- Hereditary / Genetic disorders (most common identifiable cause).
- Intrauterine infections (TORCHES - Toxoplasmosis, Other(Syphilis, VZV, Parvo), Rubella, Cytomegalovirus, Herpes Simplex).
- Metabolic disorders (e.g., Galactosemia).
- Chromosomal abnormalities (e.g., Down Syndrome).
- Prematurity.
- Maternal drug use during pregnancy.
- Trauma.
- Idiopathic (unknown cause).
Diagnosis
Clinical assessment
Screening: Red Reflex test at birth and routine child checks (absence or abnormality suggests cataract/other pathology). Observation of visual behaviour, leukocoria, nystagmus, strabismus.
Investigations
- Ophthalmic Examination (Paediatric Ophthalmologist):
- - Red Reflex testing.
- - Assessment of visual behaviour.
- - Slit Lamp Examination (may require examination under anaesthesia - EUA).
- - Dilated Fundus Examination (rule out other causes like retinoblastoma).
- - Biometry (for IOL calculation if surgery planned).
- Systemic Workup (to identify cause):
- - TORCH screen.
- - Metabolic screen (e.g., urine for reducing substances - Galactosemia).
- - Genetic testing.
Diagnostic criteria
Presence of lens opacity on ophthalmic examination in a child.
Management
Non-pharmacological management
- Urgent Referral to Paediatric Ophthalmologist.
- Amblyopia Prevention/Treatment: Crucial if cataract is visually significant. May involve patching the better eye after surgery.
- Low vision aids / Rehabilitation if vision permanently impaired.
Pharmacological treatment
- No medical treatment for the cataract itself.
- Post-operative eye drops (antibiotics, steroids).
Monitoring
Requires long-term follow-up with Paediatric Ophthalmologist for visual development monitoring, amblyopia management, refractive error correction, glaucoma screening (increased risk after surgery).
Complications
- Amblyopia ('lazy eye') - irreversible vision loss if not treated early.
- Nystagmus.
- Strabismus.
- Glaucoma (secondary, especially after surgery).
- Retinal detachment.
- Posterior capsule opacification.
- Visual field defects.
Prevention
- Maternal immunization (Rubella).
- Genetic counseling for hereditary cases.
- Avoidance of teratogenic drugs during pregnancy.
- Good antenatal care.
Patient education
- (To Parents/Caregivers):
- Explanation of congenital/paediatric cataract.
- Critical importance of early surgery for dense cataracts to prevent amblyopia.
- Surgical procedure overview.
- Need for optical correction (glasses/contacts/IOL).
- Importance of long-term follow-up, patching therapy if needed.
- Genetic counseling if indicated.
- Support resources.
Uganda-specific notes
Congenital cataract is a significant cause of treatable childhood blindness. Early detection through red reflex screening is vital but may be inconsistent. Access to specialized paediatric ophthalmic surgery and post-operative care (contact lenses, amblyopia therapy) is very limited, primarily at tertiary centres.
Surgical Management
- Cataract Surgery: Timing depends on laterality and density.
- - Bilateral Dense Congenital Cataracts: Surgery usually within first 6-8 weeks of life.
- - Unilateral Dense Congenital Cataract: Surgery often within first 4-6 weeks of life.
- - Less dense/later onset cataracts: Surgery timing based on visual significance.
- Optical Correction after Surgery:
- - Intraocular Lens (IOL) implantation (often delayed in very young infants <6-12m).
- - Aphakic glasses.
- - Contact lenses (often preferred in infants).
References
- Uganda Clinical Guidelines (UCG) 2023
Chapter 20, Section 20.2.1.1