SCIENTIFIC POSTER_Ines Casal_3

Introduction:

Familial amyloidosis TTR V30M is an autosomal dominant inherited disorder caused by extracellular amyloid deposition in nerves, solid organs and in the eye. Several ocular manifestations were reported such as abnormal conjunctival vessels, keratoconjunctivitis sicca, amyloid deposits at the pupillary margin, glaucoma, deposition of amyloid at the anterior capsula of the lens, vitreous opacities and retinal amyloid angiopathy.

Purpose:

To report the use of intravitreal ranibizumab followed by laser photocoagulation in a patient with familial amyloidosis and neovascular glaucoma.

Methods:

A 52-year-old patient with familial amyloidotic polyneuropathy associated with TTR V30M mutation that underwent a liver transplantation was referred to the ophthalmology emergency department with pain of the eye and decreased visual acuity. She presented with rubeosis iridis and neovascular glaucoma in the left eye as well as other ocular manifestations of the disease such as keratitis and opacification of the anterior capsule of the lens. Apart from the clinical examination, fluorescein and indocyanine green angiography were carried out.

Results:

Best-corrected visual acuity was 0.05 (Snellen) and intraocular pressure was 42 mmHg (applanation tonometry) before anti-VEGF injection. Fluorescein showed vascular occlusion in the retinal periphery, microaneurysms and neovascularizations. Indocyanine green angiography showed hyperfluorescent spots alongside the choroidal vasculature. Intravitreal ranibizumab was injected, and 48 hours after the procedure, improvement of the clinical situation was observed. Then laser photocoagulation was performed. Best-corrected visual acuity improved to 0.4 (Snellen) after 2 years of follow-up, and the intraocular pressure was lower than 18 mmHg under topical treatment with timolol. There were not any visible new vessels in the iris or the retina. A stable ophthalmologic situation was preserved until the patient died a few years later due to early dementia as a result of cerebral amyloid angiopathy.

Conclusion:

The use of intravitreal ranibizumab can be a potential treatment for amyloid angiopathy and neovascular glaucoma.