Vitreoretinal degenerations

Vitreoretinal degenerations include the following conditions:

  1. Wagner syndrome
  2. Stickler syndrome
  3. Favre Goldmann syndrome
  4. Familial exudative vitreoretinopathy
  5. Erosive vitreoretinopathy
  6. Dominant neovascular inflammatory vitreoretinopathy
  7. 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