ROP-like disorders

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Familial exudative vitreoretinopathy

Autosomal dominant (or X-linked)
Asymmetrical, bilateral
Abrupt cessation of peripheral retinal vessels at the equator
Vitreous bands in the periphery
Retinal ischaemia leads to fibrovascular membrane, retinal folds, macular ectopia (dragging of the retina temporally), retinal detachment, neovascularisation and subretinal exudation (like Coats)
Differentiated from ROP by lack of prematurity, low birth weight or oxygen therapy (otherwise can be indistinguishable)
Management: cryotherapy, vitrectomy
Tends to stabilize by adulthood.

X-linked dominant (fatal in males): 80% have deletion in NEMO gene
Affected eyes, skin and brain
Skin: bullae on extremities, hyperpigmented macules on trunk
Brain: microcephaly, seizures, LD
Eyes: can mimic ROP. Incomplete retinal vascularisation, neovascular membranes leading to tractional detachment