[Analysis of the misdiagnosis of bilateral iridocorneal endothelial syndrome].

Objective: To analyze the misdiagnosis of bilateral iridocorneal endothelial (ICE) syndrome and the possible reasons.

Methods: The patients seen from January 1998 to December 2011 at Beijing Tongren Hospital, misdiagnosed as bilateral ICE syndrome, were analyzed retrospectively. And a literature search was performed with a Wanfang computerized database website for the case studies of bilateral ICE syndrome. The history, clinical profiles and the findings of corneal, iris and anterior chamber examinations were analyzed. The clinical manifestations of patients with ICE syndrome were also compared with those of Axenfeld-Rieger syndrome, posterior polymorphous dystrophy (PPMD) and chronic iritis for differential diagnosis.

Results: Ten patients were diagnosed as bilateral ICE syndrome. However, among them, 5 patients were diagnosed as Axenfeld-Rieger syndrome, 2 patients as PPMD, 3 as chronic iritis and none as bilateral ICE syndrome. There were 9 Chinese reports of bilateral ICE syndrome in a total of 18 patients. Analysis showed that Axenfeld-Rieger syndrome needed to be ruled out in 10 patients, PPMD could not be ruled out in 2 patients while diagnosis could not be made in 6 patients for a lack of proof.

Conclusion: Despite the reporting of bilateral cases, ICE syndrome is usually unilateral. With specific micrographic features for a definite diagnosis of ICE syndrome, ICE cells may become an important feature differentiating from Axenfeld-Rieger syndrome, PPMD and chronic iritis.