Vitreoretinal degenerations
Vitreoretinal degenerations include the following conditions:
- Wagner syndrome
- Stickler syndrome
- Favre Goldmann syndrome
- Familial exudative vitreoretinopathy
- Erosive vitreoretinopathy
- Dominant neovascular inflammatory vitreoretinopathy
- Dominant vitreoretinochoroidopathy
Wagner syndrome
It is characterised by
- Autosomal dominant inheritance
- Choroid atrophy
- Liquefaction of vitreous with condensation of membranes
- Retina – narrow sheathed vessels with pigmented spots in periphery
- Cataract – late complication
Stickler syndrome
Also known as heriditary arthroophthalmopathy. It is characterised by:
- Autosomal dominant inheritance
- Eye changes
- Vitreous liquefaction and collapse
- Progressive myopia
- Retinal pigmentation, vascular sheathing and radial lattice like degeneration
- Bilateral retinal detachment (commonest cause of heriditary retinal detachment in childeren)
- Presenile cataract
- Ectopia lentis
- Maxillofacial features
- Flattened nasal bridge
- Hypoplastic maxilla
- Cleft palate
- High arched palate
- Joint features
- Prominent, painful, stiff, hyperextensible large joints
- Other associated features
- Deafness
- Mitral valve prolapse
Favre-Goldman syndrome
It presents in childhood as night blindness and is characterised by:
- Vitreous collapse
- Retnial pigmentation as in retinitis pigmentosa
- Retinoschisis (central, peripheral)
- Subnormal ERG
