Clinical features of retinal amyloid angiopathy with transthyretin Gly83Arg variant

AIM:To report on the clinical features of patients with retinal amyloid angiopathy(RAA)who were identified to be caused by the transthyretin(TTR)Gly83Arg variant.METHODS:Case series of five patients diagnosed with RAA was collected at Affiliated Hospital of Zunyi Medical University from January 2010 to December 2021.The clinical features,therapeutic strategies,and prognoses of all patients were reviewed.RESULTS:Five patients with a mean age of 52.00±7.23y were diagnosed as RAA.These patients were previously diagnosed with hereditary transthyretin amyloidosis caused by the TTR Gly83Arg variant.Vitreous opacity was found in all 10 eyes,and 7 eyes developed RAA 2 to 20y after the onset of hereditary transthyretin amyloidosis.The clinical manifestations were recurrent vitreous hemorrhage in 2 eyes(29%),neovascular glaucoma in 2 eyes(29%),and iris neovascularization in 1 eye(14%).Microangioma lesions were found in all affected eyes that underwent fundus fluorescein angiography(FFA)in this group of cases,and the incidence of the retinal non-perfusion area was 67%.Although no cases of retinal neovascularization were found,the prognosis of visual acuity was not ideal.CONCLUSION:This is the first report of RAA in patients with the TTR Gly83Arg variant.Complications such as RAA and glaucoma will seriously affect the visual prognosis of patients.Thereafter,regular ophthalmic follow-up of patients with hereditary transthyretin amyloidosis is essential.And FFA after vitrectomy is very important,which can help ophthalmologists detect RAA earlier and treat it in time.

Improved thickness measurement method for choroidal hyperpermeability in central serous chorioretinopathy

AIM:To observe choroidal thickness changes in the choroidal hyperpermeability area(CHA)in patients with central serous chorioretinopathy(CSC)after photodynamic therapy(PDT)using indocyanine green angiography(ICGA)combined with optical coherence tomography(OCT).METHODS:This was a cohort study of 17 eyes(17 patients)with CSC.In all patients,the range of CHA was determined by ICGA.The patients were divided into two groups based on CHA covered the fovea(group A)or not(group B).All patients received half-dose verteporfin PDT over CHA in ICGA.Choroidal thickness was measured by OCT before,1,and 3 mo after treatment.The choroidal thickness values of the fovea and CHAs were obtained for each measurement.Secondary outcomes were changes in the best-corrected visual acuity(BCVA)and amount of subretinal fluid(SRF).RESULTS:The differences in center choroidal thickness at baseline and at 1 and 3 mo post-PDT were statistically significant in group A and all patients(both P<0.001).There was no significant difference in group B(P=0.059).The differences of thickness of CHA and BCVA at baseline and1 and 3 mo post-PDT were statistically significant in group A,group B,and all patients(all P<0.01).All patients showed complete SRF absorption at 3 mo post-PDT.CONCLUSION:Center choroidal thickness does not accurately reflect changes in CHA of patients whose CHA does not covered the fovea center.Using CHA as the observation target can make up for this limitation,expand the scope of application,and reduce bias.