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Natural history of familial retinal arterial macroaneurysms syndrome

Session Details

Session Title: Vascular Diseases and Diabetic Retinopathy

Session Date/Time: Friday 18/09/2015 | 11:00-12:30

Paper Time: 11:00

Venue: Calliope

First Author: : H.Alkuraya SAUDI ARABIA

Co Author(s): :                  

Abstract Details

PURPOSE:To describe the ocular and systemic clinical findings based on long term follow up of patients with Familial Retinal Arterial Macroaneurysms (FRAM) syndrome.


retrospective clinical study


This is a retrospective clinical study including complete ophthalmologic and systemic evaluation of 22 patients with FRAM


There were 12 females and 10 males included in our study. Age at first presentation ranged from 1 to 34 years (average 10.27 years). The classical presentation of macroaneurysms and vascular beading involving the retinal artery was seen in all patients. Other retinal abnormalities included coats disease-like lesions (36%), vascular sheathing (36%), submacular gliotic mass (23%) and extensive exudative retinal detachment (18%). Follow up period ranged from 3 years to 14 years during which recurrent bleeding and leaking episodes from macroaneurysms occurred in 54%. Management of this complication involved Argon laser photocoagulation (10 patients) as well as pars plana vitrectomy for treatment of vitreous hemorrhage with subsequent improvement of visual acuity (6 patients). All patients who underwent echocardiography (n=16) showed a unique phenotype of supravalvular pulmonary stenosis, often necessitating surgical correction (44%). Two patients died in the course of the study secondary toof complications of pulmonary stenosis and cerebral hemorrhage.


FRAM is an autosomal recessive syndrome characterized by both ocular manifestations (progressive retinal arterial macroaneurysms and beading) and systemic manifestations (supravalvular pulmonary stenosis). Retinal arterial macroaneurysms and beading could be complicated by recurrent episodes of leakage and bleeding which lead to visual loss. Laser photocoagulation for the treatment of leaking macroaneurysms, and pars plana vitrectomy for treatment of vitreous hemorrhage lead to improvement of best corrected visual acuity. The systemic manifestations of this syndrome could be life threatening, and can lead to death in some cases.

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