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: 探讨泪膜改变对 Goldmann 压平眼压计测量值的影响。

方法: 将 68 例(136 只眼) 受检者随机分为 2 组, 每组 34 例。随机选择一只眼作为实验眼, 另一只眼作为对照眼。A 组的实验眼采用右旋糖苷 70 滴眼液滴眼, B 组的实验眼采用粘弹剂 Viscoat 滴眼。采用 Goldmann 眼压计测量 2 次眼压, 比较滴眼前、后测量值的差异。

结果: A 组对照眼第 1、2 次的眼压测量值分别为(14.44±2.68) mmHg(1 mmHg = 0.133 KPa) 、(14.47 ± 2.69) mmHg, 两次眼压测量值的差异无统计学意义(t = - 0.329, P = 0.744) 。实验眼滴右旋糖苷 70 滴眼液前、后的眼压测量值分别为(14.41 ±2 .63) mmHg、(12.94 ± 2.59) mmHg, 两次眼压测量值的差异有统计学意义 (t = 13.949, P = 0.000) 。B 组对照眼第 1、2 次的眼压测量值分别为(14.29 ± 2.96) mmHg、(14.35 ± 3.12) mmHg, 两次眼压测量值的差异无统计学意义(t = - 0.466, P = 0.644) 。实验眼滴 Viscoat 前、后的眼压测量值分别为 (14.53 ± 3.13) mmHg、(11.18 ± 3.07) mmHg, 两次眼压测量值的差异有统计学意义( t = 22.126, P = 0.000) 。两组的实验眼滴眼后的眼压均呈一致性的下降。

结论: 泪膜的改变可以使 Goldmann 压平眼压计的测量值产生偏差。

Purpose: To explore the effect of tear film changes on the intraocular pressure (IOP) measurement by Goldmann applantion tonometer.

Methods: Sixty-eight normal subjects were randomly divided into two groups (Gruop A and B) , 34 in each group. One eye of each subject by random selection was given Dextran (in Group A) and Viscoat (in Group B) , respectively. And the fellow eyes were used as controls. IOP was measured in all subjects twice using Goldmann applantion tonometer. And the difference between first and second measurements was compared.

Result: In the control eyes of Group A, the average IOP of first and second measurements were (14.44 ± 2.68) mmHg (1 mmHg= 0.133 KPa) and (14.47 ± 2.69) mmHg, there was no difference between the two measurements (t =- 0.329, P = 0.744) . In eyes given Dextran, the average IOP of two measurements were (14.41 ± 2.63) mmHg and (12.94 ± 2.59) mmHg, there was significant difference between the two mea-surements (t =13.949, P= 0.000) . In control eyes of Group B, the average IOP of first and second measurements were (14.53 ± 3.13) mmHg and (11.18 ± 3.07) mmHg, and there was no difference between the two measurements (t = -0.466, P = 0.644) . In eyes given Viscoat, the average IOP of two measurements were (14.53 ± 3.13) mmHg and (11.18 ± 3.07) mmHg, and there was significant difference between the two measurements (t = 22.126, P = 0.000) . After being given Dextran or Viscoat, the IOP values decreased consistently in both Group A and Group B.

Conclusions: The change of tear film components can affect IOP values by Goldmann applantion tonometer.

睫状体髓上皮瘤是一种源自神经上皮层的恶性肿瘤，易因其伪装特性而被漏诊和误诊。本文报道了1例3岁9个月的男性患儿，眼部表现似晶状体破裂，经部分肿物切除活检，病理诊断为睫状体恶性髓上皮瘤。

Medulloepithelioma of the ciliary body is a kind of malignant tumor which arises from neuroepithelium. It is easily misdiagnosed or miss diagnosed due to its masquerade feature in the clinical practice. We report a boy with a tumor in his right eye at age of 3 years and 9 months presented first with lens rupture. Diagnosis of malignant medulloepithelioma of ciliary body was made pathologically by biopsy of the partial tumor.

睫状体髓上皮瘤是一种源自神经上皮层的恶性肿瘤，易因其伪装特性而被漏诊和误诊。本文报道了1例3岁9个月的男性患儿，眼部表现似晶状体破裂，经部分肿物切除活检，病理诊断为睫状体恶性髓上皮瘤。

Medulloepithelioma of the ciliary body is a kind of malignant tumor which arises from neuroepithelium. It is easily misdiagnosed or miss diagnosed due to its masquerade feature in the clinical practice. We report a boy with a tumor in his right eye at age of 3 years and 9 months presented first with lens rupture. Diagnosis of malignant medulloepithelioma of ciliary body was made pathologically by biopsy of the partial tumor.