Good Hope Eye Clinic

Proliferative
vitreo-retinopathies  

David Kinshuck

Norries & Familial Exudative Vitreoretinopathy

If the condition can be detected early, laser treatment at birth can prevent the retinal detachment and further visual loss see. Essentially the condition is like ROP (retinopathy of prematurity)

  • at birth the peripheral retinal has not been vascularised
  • this retina becomes ischaemic after birth
  • neovascularisation and then fibrosis develops
  • the treatment is laser of the ischaemic retina before the vascularisation....laser shortly after birth
  • Review 2011    Eye 2015
  • Coats diesase in young people is probably this condition, x linked FEVR
  • clinical
  • features: retinal fold (falciform fold),
  • needs laser to peripheral ischaemic retina  (patient very young)
  • FZD4 mutation...EVR focus; NP gene; ZNF408 etc Eye 2015

Norries disease and other proliferative vitreoretinopathies need urgent laser treatment of the ischaemic retina. Avastin may have a role.

 

Norries disease and other proliferative vitreolretinopathies need early laser of the ischaemic retina

 

findings in FEVR:

 the peripheral retina remains avascular >

becomes ischaemic   >

releases VEGF >

leads to new vessel growth

(VEGF is a growth chemical) >

 

 

 

 

  

FEVR..narrow angle between forking vessels, flaciform fold in later (untreated)  stage

 

 

 

 

 

then scaring develops (falciform fold) photo early

later

scan

 

Coats in young people

    • may be x linked FEVR
    • main area temporal, then inferior
    • aneurysmal dilatation of peripheral retinal vessels with ischaemic areas
    • ischaemic areas leak..lots of exudate
    • used to be treated with cryo, now lasered, Avastin has a role.
    • Coats disease website
    • photos
    • BJO 2013 A   AntiVEGF Retina 14

Coats in adults

IP

    • Nemo gene, X xhromosome
    • mainly girls
    • skin, siezures, teeth, learning problems
    • peripeheral vessels wander

Page partly from notes from a lecture given by Prof T Moore, 2011.