Analysis of the Clinical Features and Misdiagnosis of 86 Cases of Iridocorneal Endothelial Syndrome

Abstract

Objective: \nTo observe and analyze the clinical features and misdiagnosis of iridocorneal endothelial (ICE) syndrome. \n \n \nMethods: \nIn this retrospective case analysis, 86 eyes of 86 patients who were diagnosed with ICE syndrome between 1993 and 2015 in Qingdao Eye Hospital. Sex, age, maincomplaints, course of disease, types, complication and misdiagnosis were recorded and analyzed. \n \n \nResults: \nEighty-six cases were unilateral. The ratio of male to female was 1:1.2, and the age ranged from 20 to 73 years, average 50.1±11.8 years. 91% of patients with progressive visual loss and red eye were the main complaints. The course of disease ranged from 0.17 to 10 years, average 2.4±2.3 years. 1% of patients complained about pupil deformation, the course of disease was 1 year. 6% of patients complained black eye foggy, the course of disease ranged from 0.5 to 2 years, average 1.4±0.6 years. 2% of patients with high intraocular pressure founding by physical examination were treated in time. 95% of patients with obvious iris atrophy were classified as primary progressive iris atrophy, Chandler and Cogan-Reese syndrome were 4% and 1%. The typical morphology of corneal endothelial cell showed irregular, cavitation, central black area and brighter edge. The atypical sign was no significant iris atrophy. 68% of patients had developed glaucoma and corneal endothelial dysfunction. Ultrasawnd biomicroscepy and angle mirror examination showed peripheral iris extensive anterior and local filamentous adhesion, angle pigment classification level was 1 to 3. 27% of patients were misdiagnosed for the first visit and 20% of these patients were misdiagnosed in other hospitals. 8% of patients were misdiagnosed as primary glaucoma, 12% of patients were misdiagnosed as Posner-Schlossman syndrome, Fuchs syndrome and uveitis. Absence of corneal endothelial cell morphology examination and atypical clinical manifestation were main causes of misdiagnosis. \n \n \nConclusions: \nProgressive visual loss is the main reason for patients with ICE syndrome, long course of disease and delay treatment. More than 50% of patients have complications at visiting time. 95% of patients are primary progressive iris atrophy. Most easily misdiagnosed as primary glaucoma and uveitis. Absence of corneal endothelial cell morphology examination and atypical clinical manifestation are main causes of misdiagnosis. \n \n \nKey words: \niridocorneal endothelial syndrome;\xa0clinical feature;\xa0misdiagnosis