Good Hope, Heartlands, and Solihull Eye Clinics


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




Coats in young people

Coats in adults


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


  • Young men epistaxis, constipation, headache, minor occlusions, = Eales disease search
  • Eales is related to TB in India
  • treatment      


  • Hearing loss, seizures, ischaemic retina, =susac, even optic atrophy , need immunosuppressive treatment Susac

Optic nerve