Deposits on Retinal Surface Seen on OCT in Ocular Amyloidosis.

PURPOSE

To investigate the tomographic features in patients with hereditary amyloidosis transthyretin (hATTR).

DESIGN

Retrospective case series and analysis of B-scan OCT images.

PARTICIPANTS

A total of 120 patients (240 eyes) diagnosed with hATTR.

METHODS

We performed a retrospective review of the treatment history and retrospective analysis of the OCT images of patients with hATTR. The parameters analyzed were the age at which the last OCT was performed, presence of ocular amyloidosis, history of pars plana vitrectomy (PPV), systemic treatment, and genetic mutations. Two independent evaluators evaluated the OCT images for characteristic needle-shaped pattern deposits on the retinal surface, and a third evaluator resolved any differences in their evaluations.

MAIN OUTCOME MEASURES

The frequency of characteristic needle-shaped deposits on the retinal surface seen on OCT.

RESULTS

The mean age at the time of the last OCT was 56.5 ± 14.9 years. Ninety-nine patients had gene encoding transthyretin (TTR) with the Val30Met mutation, and 21 patients had other mutations. Of 240 eyes, 128 had signs of ocular amyloidosis. Fifty of 73 eyes (68.5%) with a history of PPV for vitreous opacities exhibited characteristic deposits on OCT. Four of 31 eyes with vitreous opacity but without a history of PPV showed deposits on the retinal surface. No eyes without a history of vitreous opacities revealed the characteristic needle-shaped deposits.

CONCLUSIONS

Characteristic needle-shaped deposits on the retinal surface seen on OCT are significant because they are seen in most of the vitrectomized eyes presenting with ocular amyloidosis.