Abstract
Methods This monocentric observational study was conducted at the French National Reference Center for TTR-FAP. Genetically confirmed TTR-FAP patients with suspected retinal and/or choroidal vascular abnormalities underwent fluorescein and indocyanine green angiography. Sensorimotor polyneuropathy (SPN) was staged with the Polyneuropathy Disability (PND) score, vegetative neuropathy was staged with the Compound Autonomic Dysfunction Test (CADT). Medical and surgical treatments were analyzed for all patients.
Highlights
Retinal amyloid angiopathy is considered to be a rare ocular manifestation of TTR-FAP
Sensorimotor polyneuropathy (SPN) was staged with the Polyneuropathy Disability (PND) score, vegetative neuropathy was staged with the Compound Autonomic Dysfunction Test (CADT)
Bilateral retinal ischemic vasculopathy was present in 8/12 patients and included retinal hemorrhages, microaneurysms, venous and arteriolar segmental staining
Summary
Retinal amyloid angiopathy is considered to be a rare ocular manifestation of TTR-FAP. Choroidal vascular abnormalities have been described in isolated case reports. The purpose of this study was to describe fluorescein and indocyanine green angiographic findings of retinal and choroidal vascular abnormalities in a series of TTR-FAP patients
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