[Clinical characteristics of 114 patients with iridocorneal endothelial syndrome].

Objective: To investigate the clinical features in patients with iridocorneal endothelial (ICE) syndrome. Methods: A retrospective case series study. Data of clinical manifestations of patients with ICE syndrome including clinical subtypes, presenting visual acuity, clinical features and secondary glaucoma were collected from January 2014 to May 2020 in the Eye Hospital, School of Ophthalmology and Optometry, Wenzhou Medical University. The Wald's Chi-square test of generalized estimating equations was performed to analyze the differences in three clinical subtypes. Results: A total of 127 eyes of 114 subjects (64 females and 50 males) were included. Mean±SD age at presentation was (49±13) years. There were 53 patients (46.5%) with Chandler's syndrome (CS), 36 patients (31.6%) with progressive iris atrophy (PIA), 24 patients (21.0%) with Cogan-Reese syndrome (CRS) and one patient (0.9%) with an undetermined subtype. And 101 patients (88.6%) had uniocular ICE syndrome. Approximately 81.7% (49/60), 56.1% (23/41) and 41.7% (10/24) of eyes presented visual acuity <0.3 in patients with CS, PIA and CRS, respectively. Corneal edema was most common in CS (52.5%, 32/61), followed by PIA (29.3%, 12/41) and CRS (20.8%, 5/24). Corectopia was found in 95.8% (23/24) of eyes with CRS, 95.1% (39/41) of eyes with PIA and 78.7% (48/61) of eyes with CS. Polycoria was observed in 29.3% (12/41) of eyes with PIA, 3.3% (2/61) of eyes with CS and 8.3% (2/24) of eyes with CRS. Ectropion uvea was most common in CRS (54.2%, 13/24), followed by 16.4% (10/61) in CS and 12.2% (5/41) in PIA. Glaucoma was found in 94 eyes (74.0%, 94/127). Among them, 60.7% (37/61) of CS, 80.5% (33/41) of PIA and 95.8% (23/24) of CRS had secondary glaucoma. The difference of presenting visual acuity, corneal edema, corectopia, polycoria, ectropion uveae and secondary glaucoma in three clinical subtypes all had statistical significance (Wald χ2=13.87, 10.77, 965.78, 11.45, 15.00, 222.04; all P<0.05). And 86.2% of eyes (81/94) had glaucoma surgeries and 41 eyes (43.6%, 41/94) had the intraocular pressure well controlled with various interventions. Conclusions: ICE syndrome is mostly uniocular and more common in middle-aged patients. CS is the most common clinical subtype with poor presenting visual acuity. About 3/4 of eyes have secondary glaucoma, and the majority of them require surgical interventions, but prognoses are discouraging.目的： 分析虹膜角膜内皮（ICE）综合征患者的临床特征。 方法： 回顾性病例系列研究。收集2014年1月至2020年5月在温州医科大学附属眼视光医院诊断为ICE综合征患者的临床资料。记录患者的临床类型、生活视力、临床特征及继发性青光眼情况。采用广义估计方程中的Wald χ²检验等方法对3种临床类型的临床特征进行比较。 结果： 共纳入114例（127只眼）ICE综合征患者，其中女性64例，男性50例；就诊年龄为（49±13）岁。Chandler综合征（CS）53例（46.5%），进行性虹膜萎缩（PIA）36例（31.6%），Cogan-Reese综合征（CRS）24例（21.0%），无法明确临床类型1例（0.9%）。101例（88.6%）患者为单眼发病。在CS、PIA及CRS中分别有81.7%（49/60）、56.1%（23/41）及41.7%（10/24）患眼的生活视力低于0.3；角膜水肿在CS中最多见（52.5%，32/61），其次是PIA（29.3%，12/41）以及CRS（20.8%，5/24）；瞳孔异位在CS、PIA、CRS中分别占78.7%（48/61）、95.1%（39/41）、95.8%（23/24）；多瞳在PIA中最多见（29.3%，12/41），CS与CRS中分别占3.3%（2/61）及8.3%（2/24）。葡萄膜外翻多见于CRS（54.2%，13/24），CS与PIA中分别占16.4%（10/61）、12.2%（5/41）。74.0%（94/127）的患眼合并继发性青光眼，其中CS、PIA及CRS患眼继发青光眼比例分别为60.7%（37/61）、80.5%（33/41）、95.8%（23/24）。不同临床类型ICE综合征在生活视力、角膜水肿、瞳孔异位、多瞳、葡萄膜外翻以及继发性青光眼比例上的差异均有统计学意义（Wald χ²=13.87，10.77，965.78，11.45，15.00，222.04；均P<0.05）。86.2%（81/94）患眼进行了抗青光眼手术治疗，43.6%（41/94）患眼在各种辅助降眼压治疗下眼压得到控制。 结论： ICE综合征以单眼发病、中青年发病为主，CS是主要的临床类型，且该类患者视力损伤最为严重。约3/4的患眼合并继发性青光眼，大部分患者需要进行手术干预，但眼压预后不佳。.