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OBJECTIVE: To summarize the misdiagnosis and missed diagnosis of iridocorneal endothelial syndrome (ICE syndrome) and analyze the main reasons. METHODS: Retrospective case series. The records of 65 patients (65 eyes) who were diagnosed to suffer from ICE syndrome in Beijing Tongren Hospital during Jan. 2008 to Dec. 2011 were studied retrospectively. The international diagnostic standards were used. The situations of misdiagnosis and missed diagnosis were evaluated and the main reasons and the related clinical characters were analyzed. RESULTS: The correct preliminary diagnosis was found in 9 cases (13.8%). Misdiagnosis included primary angle closure glaucoma (PACG) in 14 cases (21.5%), primary open angle glaucoma (POAG) in 14 cases (21.5%), uveitis in seven cases (10.8%), etc. The contralateral eye received operation or laser therapy in 12 of 56 cases who didn't get the correct preliminary diagnosis. There were many atypical and special presentations in our patients. There was no or mild atrophy of iris in 54 cases (83.1%). Secondary open angle glaucoma was found in eleven cases (16.9%). The maximal intraocular pressure (IOP) was ≥ 40 mmHg (1 mmHg = 0.133 kPa) in 41 cases (63.1%). And there was no corneal edema when the IOP is lower than 30 mmHg in 48 cases (73.8%). There is some abnormal black pigment in the anterior chamber angle in some cases. CONCLUSIONS: ICE syndrome is a disease with high rate of misdiagnosis or missed diagnosis. The main reasons of misdiagnosis and missed diagnosis include that ICE syndrome has many different clinical manifestations and the doctors pay little attention of to the atypical presentations of ICE cases.
Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing Ophthalmology & Visual Sciences Key Lab, Beijing 100730, China.
9.4.2.1 Iridocorneal endothelial syndrome (ICE, incl. irisatrophy) (Part of: 9 Clinical forms of glaucomas > 9.4 Glaucomas associated with other ocular and systemic disorders > 9.4.2 Glaucomas associated with disorders of the cornea, conjunctiva, sclera)