Table 1
Ocular features described in incontinentia pigmenti
| Part of the eye affected | Description |
|---|---|
| Lids | Ptosis21 |
| Nasolacrimal duct | Congenital obstruction28 |
| Control of eye movements | Strabismus1,7,17,19,21,23 |
| Nystagmus22,23 | |
| Abnormalities of globe size | Microphthalmos23 |
| Buphthalmos22 | |
| Conjunctiva | Hyperpigmentation29 |
| Sclera | Blue19,20 |
| Corneal abnormalities | See text |
| Iris | Atrophy with pigmentary irregularities23 |
| Hypoplasia and synaechiae7 | |
| Lens | Congenital cataract (usually associated with retinal detachment)1,7,22,23 |
| Vitreous | Persistent fetal vasculature30 |
| Haemorrhage | |
| Fovea | Foveal hypoplasia31 |
| Macular aneurysms8,9 | |
| Peripheral retina | Fibrovascular proliferation and traction retinal detachment15,17,22,25,31 |
| Vascular anomalies—eg, microaneurysms, arteriovenous shunts, neovascularisation associated with an avascular zone8,9,17,23,32 | |
| Optic disc | Neovascularisation33 |
| Atrophy1,7,17,32 | |
| RPE | Pigmentary changes13,15,23,29,34 |
