Paediatric opthalmology Flashcards
What is leukocoria?
Leukocoria:
- Means ‘white pupil’
- Concerning sign
- Seen in children
- Loss of red reflex
- Opacity in the optical media of the eye
What are the causes of leukocoria?
- Cataracts
- Retinoblastoma (must be excluded immediately)
- Vitreous opacity
- Retinal detachment
What dilating drops do we use for children when performing fundoscopy?
Cyclopentolate
What is the initial management for leukocoria?
- History and examination
- Dilate eyes using cyclopentolate
- Fundoscopy
- Refer to opthalmology
Ensure you rule out retinoblastoma as soon as possible
What is retinopathy of prematurity?
Retinopathy of prematurity (ROP)
- Proliferative retinopathy affecting premature infant of very low birth weight
- This is due to exposure of high ambient oxygen concentrations
- Retina has no blood vessels until fourth month of gestation
- At birth, it is still incompletely vascularised at the temporal periphery
- This means the retina is susceptible to oxygen damage in premature infants
- The avascular retuna produces VEGF
- In utero VEGF is the stimulus for vessel migration in the developing retina
- However, in premature infants, VEGF is down-regulated due to the relative hyperoxia, so blood vessels do not develop
- However the eye still has increasing metabolic demands
- This eventually causes excessive VEGF production resulting in neovascular complications of ROP
What is the screening for retinopathy of prematurity?
ROP screening:
- All babies born at or before 31 weeks
- Babies weighing less than 1.5kg
- Screen for ROP at around 4-7 weeks postnatally
- Only 8% of those screened require treatment
What is the treatment for retinopathy of prematurity?
Laser photocoagulation - successful in 80% of cases
If ROP is left untreated it can lead to sight-threatening complications such as vitreous haemorrhage and retinal detachment
What is retinoblastoma?
Retinoblastoma:
- Most common primary intraocular malignancy of childhood
- 3% of all childhood cancers
- Rare
- Results from malignant transformation of primitive retinal cells before differentiation
- These cells disappear within the first few years of life, so this tumour is usually seen in children less than 3
Heritability
- Can be heritable or non-heritable
- The gene predisposing to retinoblastoma (RB1) is at 13q14
1. Heritable (germline) retinoblastoma = 40%
2. Non-heritable retinoblastoma = 60% of cases - Tumour is unilateral not transmissible and does not predispose the patient to second non-ocular cancers
- If patient has a solitary retinoblastoma and no fhx, it is most likely non-heritable
- This would make the risk in siblings and offspring about 1%
Spread In both forms, the risk of metastatic disease is greater if: - Tumour is advanced - There is optic nerve invasion - Massive choroidal invasion - Anterior chamber involvement - Orbital spread
What are the treatments for retinoblastoma?
Depend on site of tumour and visual prognosis
- Chemotherapy
- Radiotherapy
- Brachytherapy
- Enucleation (removing eyeball)
What are congenital cataracts?
Congential cataracts are a cause of leukocaria
- Occur in 3 in 10000 births
- 2/3 of cases are bilateral
- Cause identified in 50% of those affected
- Most common cause is genetic mutation (autosomal dominant) Also: - Chromosomal abnormalities - Metabolic disorders - Intrauterine infections
Unilateral cataracts:
- Sporadic
- Without family history or systemic disease
- Affected infants are full-term and healthy
What conditions are associated with congenital cataract formation?
Chromosomal abnormalities:
- Down Syndrome
- Edward syndrome
Intrauterine infections
- Varicella
- Cytomegalovirus
- Rubella
How are congenital cataracts treated?
Paediatric cataract surgery is complex and is only undertaken by few centres in the UK
Timing is crucial
Depends on bilateral/unilateral
Depends on density
Bilateral dense cataracts:
- Early surgery when the child is 4–6 weeks to prevent amblyopia
Bilateral partial cataracts:
- May not require surgery until later if at all
Unilateral dense cataract:
- Urgent surgery (possibly within days) followed by aggressive anti-amblyopia therapy, despite which the results are often poor
What are some causes of leukocoria?
- Intraocular infection
- Retinoblastoma
- Coat’s disease
- Congenital cataract
How urgently should suspected retinoblastoma be discussed?
As soon as possible - telephone consultation with opthalmologist on-call
