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Optic nerve hypoplasia
Pediatric Ophthalmology
Key Points
- •ONH is the most common congenital optic disc anomaly and the leading cause of childhood visual impairment in developed nations
- •The double ring sign is pathognomonic - a small pale disc surrounded by a ring of exposed peripapillary sclera within the normal-sized scleral canal
- •MRI brain and endocrine evaluation are mandatory for all ONH patients - hypothalamic-pituitary dysfunction occurs in 60–80% and can be life-threatening (ACTH deficiency)
- •Septo-optic dysplasia (de Morsier) = ONH + absent septum pellucidum + hypothalamic-pituitary dysfunction; complete triad in 30%
- •Visual acuity is highly variable (6/9 to NLP) and poorly correlated with disc size - prognosis is non-progressive but functional improvement is limited
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Keep reading Optic nerve hypoplasia
17 more sections are included with the Clinical plan.
- Definition
- Epidemiology
- Aetiology and causes
- Pathogenesis
- Classification
- Risk factors and associations