CONGENITAL OPTIC-NERVE HYPOPLASIA

Congenital optic nerve hypoplasia (CONH) is an increasingly recognized cause of low vision in children. The spectrum of ophthalmoscopic and clinical presentations of CONH is described here with illustrative cas es. Some children with unilateral CONH develop a secondary, functional amblyopia that may respond to patching during visual development. Chi ldren with bilateral CONH, poor vision and nystagmus usually present e arly and have a high risk of exhibiting central nervous system (CNS) m alformations and treatable endocrine deficiencies.