Clinical Features and Differential Diagnosis of Acute Idiopathic Blind Spot Enlargement Syndrome

Xiaocui Liu， Bing Chen， Maonian Zhang， Houbin Huang

Department of Ophthalmology， Chinese PLA（People's Liberation Army） General Hospital， Beijing 100853，China

Introduction

Materials and methods

General data

Ocular symptoms

Table 1 Basic clinical data of 6 patients with acute idiopathic blind spot enlargement syndrome

Examination methods

Results

Fundus condition

Figure 1 A．Fundus imaging of case 3 revealed mild white opacity in the peripapillary retina （OS）； B．Fundus imaging of case 6 showed slight optic disc swelling （OS）； C．Fundus imaging of case 5 revealed peripapillary ring-shaped and temporal topical scars（OD）； D．Fundus imaging of case 1 showed mild optic disc swelling （OS） and yellow-white macular lesions．

Visual field examination

Fundus angiography

OCT and mfERG

Figure 2 A．Perimetry of case 5 revealed blind spot enlargement； B．A visual field defect was observed within the region from the temporal side to beneath the nose （case 2， OS）； C．A 60°visual field revealed a large scotoma at the temporal side involving with the area within central 5°and outside central 50°from the temporal side （case 2）．

Figure 3 A．FFA of case 6 revealed peripapillary fluorescence leakage during early stage．B．ICGA of case 6 showed a bulk of weak fluorescence spots adjacent to the optic disc during late stage．C．FAF of case 6 revealed peripapillary autofluorescence enhancement； D．FAF of case 1 showed peripapillary autofluorescence enhancement； E．ICGA of case 1 revealed peripapillary diffusive weak fluorescence spots and macular scars during late stage； F．The onset time of case 2 was 1．5 months； FAF revealed mild peripapillary autofluorescence enhancement； G．FFA of case 2 revealed a large quantity of fluorescence spots within the region surrounding the optic disc to the subretinal upper posterior polar area； H．The onset time of case 4 was 4 months and no autofluorescence abnormality was observed．

Discussion

Figure 4 A．The onset time of case 6 was 2 weeks．OCT showed IS／OS defects； B．The onset time of case 1 was 1 month．OCT revealed IS ／OS defects，a bulk of mass was observed between the outer layer of temporal photoreceptors and RPE and partial Bruch＇s membrane rupture； C．The onset time of case 2 was 1．5 months．OCT revealed peripapillary retinal IS／OS and COST defects， outer membrane and outer nuclear layer atrophy； D．The onset time of case 4 was 4 months．OCT only showed macular COST defects．

Figure 5 The mfERG of case 5 revealed a lower macular intensity of response in the right eyes than in the left eyes．

Fundus pathological changes during early stage AIBSES

Table 2 Special test results of 6 patients diagnosed with acute idiopathic blind spot enlargement syndrome

Auxiliary eye examination

Diagnosis and differential diagnosis

Clinical staging and prognosis