- Maybe asymptomatic
- Blurry vision
- Typically located inferonasally
- Appears as notches in iris or "keyhole pupils"
- May or may not be associated with chorioretinal coloboma
- Absence of zonules in the area of a ciliary body coloboma results in lens notching (coloboma)
- Other associated syndromes may include:
- Autosomal dominant uveal coloboma
- Cat eye syndrome
- Edwards' syndrome
- Patau's syndrome
- Linear nevus sebaceous syndrome.
- Supportive with sunglasses, tinted contact lens or contact lens with artificial pupil
- Surgical iridoplasty in patients with severe and intolerable symptoms