目的：分析先天性葡萄膜外翻(Congenital ectropion uveae, CEU)继发性青光眼的临床表现、影像学特点和治疗方法。方法：回顾分析就诊于中山大学中山眼科中心诊断为CEU继发性青光眼患者的电子病历资料，收集整理患者的一般情况、视力、眼压、裂隙灯检查、房角镜照相、超声生物显微镜等检查结果，以及手术治疗方式选择和术后随访眼压情况，并结合国内外文献回顾分析总结CEU继发性青光眼的临床特点和诊疗效果。结果：报告两例CEU继发性青光眼患者年龄分别为24岁和11岁，均为男性、单眼发病慢性病程，患眼都为右眼，都不伴有全身异常，最佳矫正视力为手动/40cm-0.16，就诊时平均眼压为37 mmHg，平均中央前房深度为3.45 mm。两例患者查体表现：均角膜透明，瞳孔大小约5-5.5mm，对光反应灵敏，瞳孔缘近360°葡萄膜外翻呈棕褐色，晶状体透明，眼底检查青光眼性视杯凹陷，视网膜平伏。其中24岁患者房角镜检查全周宽角开放，小梁网有色素沉着。超声生物显微镜（ultrasound biomicroscopy, UBM）检查提示右眼虹膜稍向后凹，7点位房角关闭，其余全周房角开放。而11岁患者房角镜检查以及UBM检查均为全周房角关闭。两例患者角膜内皮镜检查均正常。两例患者均诊断为右眼先天性葡萄膜外翻继发性青光眼，眼压高，最大量降眼压药物治疗下不能控制，对24岁患者行小梁切除联合术中使用抗代谢药物、11岁患者行引流阀植入联合术中使用抗代谢药物，术中术后均无明显并发症，至今随访6个月，眼压控制良好。结论：CEU特征性改变为瞳孔缘葡萄膜外翻，常继发青光眼，可表现为开角型、房角发育异常（闭角型），降眼压药物治疗效果有限，通常需要手术治疗。由于CEU继发性青光眼人群以儿童和年轻患者为主，为难治性青光眼，因此抗青光眼术中联合使用抗代谢药物可获得较好的治疗效果。
Objective: To analyze the clinical manifestations, imaging characteristics, and treatment methods of secondary glaucoma associated with congenital ectropion uveae (CEU). Methods: Observational case series and literature review. A retrospective analysis was conducted on the electronic medical records of patients diagnosed with secondary glaucoma due to CEU at the Zhongshan Ophthalmic Center, Sun Yat-sen University, China. Data collected included demographics, visual acuity, intraocular pressure (IOP), slit-lamp examination, gonioscopy, ultrasound biomicroscopy (UBM), and surgical treatment methods. Postoperative IOP were also collected. A literature review was conducted to summarize the clinical characteristics and treatment outcomes of secondary glaucoma due to CEU. Results: Two male patients with secondary glaucoma due to CEU were included, aged 24 and 11 years, respectively. Both patients had a chronic course of disease affecting the right eye, with no systemic abnormalities. The best-corrected visual acuity was hand motion/40 cm to 0.16, and the average IOP at presentation was 37 mmHg, with an average central anterior chamber depth of 3.45 mm. Clinical examination revealed clear corneas, pupils measuring approximately 5-5.5 mm with brisk light reflexes, and nearly 360° of brownish ectropion of the uveae. The lenses were clear, and fundus examination showed glaucomatous optic disc cupping with a normal retina. In the 24-year-old patient, gonioscopy revealed a wide-open angle with trabecular meshwork pigmentation, while UBM indicated slight posterior bowing of the iris and angle closure at the 7 o'clock position. In the 11-year-old patient, both gonioscopy and UBM showed complete angle closure. Corneal endothelial microscopy was normal in both patients. Both were diagnosed with secondary glaucoma due to CEU in the right eye, with elevated IOP that was uncontrolled by maximal medications. The 24-year-old underwent trabeculectomy with intraoperative use of antimetabolites, while the 11-year-old received a drainage valve implantation with antimetabolites. No significant intraoperative or postoperative complications were observed, and IOP was well controlled over a six-month follow-up period. Conclusions: CEU is characterized by ectropion of the uveae and is frequently associated with secondary glaucoma, which may present as open-angle or angle-closure (goniodysgenesis) glaucoma. The effectiveness of IOP-lowering medications is limited, often necessitating surgical intervention. Given that secondary glaucoma due to CEU predominantly affects children and young adults, the use of antimetabolites during glaucoma surgery can yield favorable treatment outcomes.
