目的：分析Hallermann-Streiff综合征(Hallermann-Streiff syndrome,HSS)继发性青光眼的临床表现，探讨其治疗方法。方法：采用病例系列研究与文献回顾方法，记录3例确诊为HSS继发性青光眼患者的视力、眼压、裂隙灯、超声生物显微镜、相干光断层扫描、角膜地形图、A超、B超、X线眼眶大小测量等检查结果。随访患者药物治疗、周边虹膜切除术、小梁切除术或青光眼阀植入术的疗效。结果：3例患者年龄分别为9、29和47岁，其中女性2例、男性1例。最佳矫正视力为0.04-0.5，平均屈光度为+12.1D，平均眼压为37.7 mmHg，平均角膜直径为9.1 mm，平均中央前房深度为2.43 mm，平均眼轴长度为18.13 mm，角膜地形图示平均K1值为56.97 D，平均K2值为60.65 D。眼眶水平径为28.86~31.40 mm，垂直径为30.16~32.90 mm。2例年轻患者为无晶状体眼，伴葡萄膜炎、瞳孔区纤维膜、视盘旁脉络膜萎缩弧。年长患者表现为蓝色巩膜、白内障、房角关闭，眼底表现为青光眼性视杯凹陷。3例患者平均身高143 cm，伴头发及眉毛稀疏、额头前凸、鼻子呈喙状、牙齿发育不全、下颌发育不全。术后平均随访47.7个月(范围：11~84个月)，眼压控制，视力与术前一致，无治疗相关并发症出现。结论：HSS继发性青光眼的眼部病变可表现为小眼眶、小眼球、小角膜、蓝色巩膜、无晶状体、瞳孔区纤维膜、葡萄膜炎、继发性青光眼及视盘旁脉络膜萎缩。对HSS继发性青光眼的患者，个性化地选择治疗方案，可以获得较好的治疗效果。

Objective: To demonstrate the clinical characteristics and surgical effects of glaucoma in Hallermann-Streiff syndrome(HSS). Methods: Observational case series and literature review. The results of ophthalmic examinations of three patients diagnosed as glaucoma with HSS were recorded, including visual acuity, intraocular pressure (IOP), slit-lamp microscopy, ultrasound biomicroscopy, optical coherence tomography, corneal topography, A-scan and B-scan ultrasonography, and orbital size measurement by X-ray. Peripheral iridectomy, glaucoma drainage device implantation or trabeculectomy, were performed in these patients. Results: Three HSS patients were 9, 29 and 47 years old, respectively, including 2 females and 1 male. The best corrected visual acuity was 0.04-0.5. The mean spherical equivalent refraction was +12.1 D. The average IOP was 37.7 mm Hg, and the average corneal diameter was 9.1 mm. The average central anterior chamber depth was 2.43mm. The average axial length was 18.13mm. Keratometry showed average K1 of 56.97 degrees, and K2 of 60.65 degrees. Two younger patients were aphakic bilaterally with uveitis, pupillary fibrous membrane and peripapillary choroidal atrophy. The older patient showed blue sclera, cataract, and anterior chamber angle closure. The horizontal orbital diameter was 28.76-31.40 mm, and vertical orbital diameter was 30.16-32.90 mm. All patients were proportionate nanism, with an average height of 143 cm. Craniofacial manifestations included dyscephalia and “bird-like” face, hypotrichosis, dental anomalies, and mandibular hypoplasia. They were followed up for an average of 47.7 months(range:11-84 months) after surgery. The IOPs were all controlled, and the visual acuities remained unchanged. No treatment-related complications occurred. Conclusions: HSS patients with glaucoma may present as small orbit, microphthalmia, microcornea, blue sclera, aphakia, pupillary fibrous membrane, uveitis, with atrophic chorioretinal changes. For these patients, personalized treatment may help to achieve better therapeutic effects.

目的：建立和验证一个涉及多级临床场景的白内障协作通用的人工智能(artificial intelligence,AI)管理平台，探索基于AI的医疗转诊模式，以提高协作效率和资源覆盖率。方法：训练和验证的数据集来自中国AI医学联盟，涵盖多级医疗机构和采集模式。使用三步策略对数据集进行标记: 1)识别采集模式；2)白内障诊断包括正常晶体眼、白内障眼或白内障术后眼；3)从病因和严重程度检测需转诊的白内障患者。此外，将白内障AI系统与真实世界中的居家自我监测、初级医疗保健机构和专科医院等多级转诊模式相结合。结果：通用AI平台和多级协作模式在三步任务中表现出可靠的诊断性能: 1)识别采集模式的受试者操作特征(receiver operating characteristic curve,ROC)曲线下面积(area under the curve,AUC)为99.28%~99.71%)；2)白内障诊断对正常晶体眼、白内障或术后眼，在散瞳-裂隙灯模式下的AUC分别为99.82%、99.96%和99.93%，其他采集模式的AUC均 > 99%；3)需转诊白内障的检测(在所有测试中AUC >91%)。在真实世界的三级转诊模式中，该系统建议30.3%的人转诊，与传统模式相比，眼科医生与人群服务比率大幅提高了10.2倍。结论：通用AI平台和多级协作模式显示了准确的白内障诊断性能和有效的白内障转诊服务。建议AI的医疗转诊模式扩展应用到其他常见疾病和资源密集型情景当中。

Objective: To establish and validate a universal artificial intelligence (AI) platform for collaborative management of cataracts involving multilevel clinical scenarios and explored an AI-based medical referral pattern to improve collaborative efficiency and resource coverage. Methods: The training and validation datasets were derived from the Chinese Medical Alliance for Artificial Intelligence, covering multilevel healthcare facilities and capture modes. The datasets were labelled using a three step strategy: (1)capture mode recognition; (2) cataract diagnosis as a normal lens, cataract or a postoperative eye and (3) detection of referable cataracts with respect to aetiology and severity. Moreover, we integrated the cataract AI agent with a real-world multilevel referral pattern involving self-monitoring at home, primary healthcare and specialised hospital services. Results: The universal AI platform and multilevel collaborative pattern showed robust diagnostic performance in three-step tasks: (1) capture mode recognition (area under the curve (AUC) 99.28%–99.71%), (2) cataract diagnosis (normal lens, cataract or postoperative eye with AUCs of 99.82%, 99.96% and 99.93% for mydriatic-slit lamp mode and AUCs >99% for other capture modes) and (3)detection of referable cataracts (AUCs >91% in all tests). In the real-world tertiary referral pattern, the agent suggested 30.3%  of people be ’referred’, substantially increasing the ophthalmologist-to-population service ratio by 10.2-fold compared with the traditional pattern. Conclusions: The universal AI platform and multilevel collaborative pattern showed robust diagnostic performance and effective service for cataracts. The context of our AI-based medical referral pattern will be extended to other common disease conditions and resource-intensive situations.

目的：应用Cirrus HD-OCT 5000对正常眼黄斑及视盘血流参数进行血管成像测量，评估其可重复性及再现性。方法：纳入40只正常眼进行前瞻性研究。操作者A于一周内3天的同一时段(T1，T2，T3)对正常受试者的同一眼黄斑及视盘进行3次血管成像扫描，扫描过程中均开启FastTrac^TM图像跟踪功能，操作者B在T 2时间点再次对受试者同一眼进行相同程序扫描，使用Angio PlexMetrix^TM量化软件(版本10.0)自动测量黄斑和视盘的血管长度密度(vessel length density，VD)和血管灌注密度(vascular perfusion density，PD)。应用单因素方差分析或非参数检验比较3次扫描的VD、PD是否有差异。采用组内相关系数(intraclass correlation coefficient，ICC)、变异系数(coefficient of variation，CV)评价操作者A的可重复性，采用一致性相关系数(consistent correlation coefficient，CCC)、重复性系数(repeatability coefficient，CR)、C V评价操作者间的再现性。结果：操作者A对黄斑及视盘各区域3次扫描的V D、P D之间差异无统计学意义(P >0.05)。操作者A对黄斑和视盘V D、PD 3次扫描的ICC值分别为0.260~0.517、0.362~0.898，黄斑及视盘各区域V D、P D的C V值均<8.1%。黄斑和视盘V D、P D的操作者间CCC值分别为0.3130~0.5665、0.5149~0.7801；黄斑VD和PD的CR值分别为3.2212~4.6399、0.0574~0.0832；视盘VD和PD的CR值分别为2.0675~4.0630、0.0447~0.0730。黄斑CV值均<9.0%，视盘CV值均<6.9%。结论：非同日的同一时段视盘浅层血流参数具有较好的重复性及再现性，黄斑浅层血流参数的重复性及再现性相对较差。

Objective: To evaluate the repeatability and reproducibility of quantification of macular and peri-papillary superficial capillaries in normal eyes using Cirrus HD-OCT 5000 angiography. Methods: Forty normal eyes were included in this prospective study. Angiography 3 mm × 3 mm scan protocol centered on the disc or macula of the same eye was repeated 3 times by operator A in the same period on 3 days (T1, T2, T3) within 1 week,and the FastTrac^TM image tracking function was turned on during the scan. Operator B repeated the same scan independently at T2 after operator A. Angio Plex Metrix^TM quantification software (version 10.0) was used to automatically measure the vessel length density (VD) and vascular perfusion density (PD) of macular and peri-papillary superficial capillaries. One-way analysis of variance or nonparametric test was used to compare the VD and PD measurements of the 3 scans. The intraclass correlation coefficient (ICC) and coefficient of variation (CV)were calculated to evaluate the intra-operator repeatability. Consistent correlation coefficient (CCC), repeatability coefficient (CR), and CV were calculated to evaluate the inter-operator reproducibility. Results: There was no statistically significant difference in the measurements of VD and PD between the 3 scans of the macular and peri-papillary superficial capillaries by operator A (P>0.05). The ICC values for the 3 scans of VD and PD of the macular and peri-papillary superficial capillaries by operator A were 0.260–0.517 and 0.362~0.898, respectively.The CV values of the VD and PD of the macular and peri-papillary superficial capillaries were all <8.1%. The inter-operator CCC values of the macular and peri-papillary superficial capillaries were 0.3130–0.5665 and 0.5149~0.7801,respectively; the CR values of the VD and PD of macula were 3.2212–4.6399 and 0.0574–0.0832, respectively; the CR values of the VD and PD of optic disc were 2.0675–4.0630 and 0.0447–0.0730, respectively. The CV values of macular were all <9.0%, and the CV values of optic disc were all <6.9%. Conclusion:The macular and peri-papillary superficial capillaries parameters in the same period on different days have a good repeatability and reproducibility,while the superficial macular blood flow parameters have relatively poor repeatability and reproducibility.

目的：探讨原发性慢性闭角型青光眼(chronic primary angle-closure glaucoma，CPACG)患者的视网膜血流密度(vessel density，VD)与视野缺损程度的相关性。方法：光学相干断层血管成像技术(optical coherence tomography angiography，OCTA)测量89例(112眼)视野缺损的CPACG患者的黄斑区VD、视盘旁VD，分析VD与视野缺损程度的相关性。结果：视盘旁VD与视野缺损程度成负相关(r>–0.728，P<0.05)。黄斑浅层总VD的受试者工作曲线(receiver operating characteristic，ROC)及曲线下面积(area under the curve，AUC)为0.874。在控制年龄、眼压及视力的情况下，黄斑总浅层VD每降低1%，视野平均缺损(mean deviation，MD)值增加–0.639 dB。结论：CPACG患者VD与视野缺损呈线性负相关，OCTA可以方便无创地观察青光眼患者眼底血流情况，在视野缺损前发现视网膜VD降低，从而可以作为CPACG早期诊断的参考指标。

Objective: To investigate the correlation between the retinal vessel density (VD) and the degree of visual field loss in chronic primary angle-closure glaucoma (CPACG). Methods: Eighty-nine CPACG patients (112 eyes)with different degrees of visual field loss were measured with optical coherence tomography angiography (OCTA) for macular VD and para-optic microcirculation VD, and the correlation between them and the degree of visual field defect were analyzed. Results: There was a negative correlation between the VD of the microcirculation in each zone next to the optic disc and the degree of visual field loss (r>–0.728, P<0.05). The receiver operating characteristic (ROC) and area under the curve (AUC) of the total VD of the superficial macula is 0.874. Under the condition of controlling age, intraocular pressure and vision, for every 1% decrease in the total superficial macular VD, the average visual field defect mean deviation (MD) value increases –0.639 dB. Conclusion: The VD of CPACG patients is linearly negatively correlated with visual field defects. OCTA can conveniently and non-invasively observe the blood flow of the fundus in patients with glaucoma. It is found that the retinal VD is reduced before visual field defects, which can be used as a reference index for early diagnosis of CPACG.