Birdshot Retinochoroidopathy Masquerading as Intraocular Lymphoma

We present a case of a patient with bilateral posterior uveitis HLA-A29 positive, masquerading intraocular lymphoma. A 43 year-old woman presented with bilateral vitritis and chorioretinal lesions compatible with “birdshot lesions”. The patient was initially diagnosed with birdshot retinochoroidopathy and later on, during follow up, the occurrence of neurologic involvement and the lack of response to systemic immunosuppression led us to re-evaluate the diagnosis. A definite diagnosis of intraocular lymphoma with central nervous system involvement was made. This case is presented to highlight the importance of careful follow-up of patients with chronic uveitis and re-evaluation of systemic symptoms and signs, in particular when ocular findings are highly suggestive for masquerade syndrome.     

Efficacy of Anti-IL6-Receptor Tocilizumab in Refractory Cystoid Macular Edema of Birdshot Retinochoroidopathy Report of Two Cases and Literature Review

Purpose: To evaluate tocilizumab (TCZ) efficacy in severe and refractory birdshot chorioretinopathy (BSCR).

Methods: Assessment of BSCR patients refractory to conventional immunosuppressive and anti-TNF-α drugs who underwent TCZ therapy.

Results: Two HLA-A29 positive patients (man/37 years and woman/38 years; four affected eyes) with BSCR were studied. They had a chronic bilateral posterior uveitis. Patient 1 had been treated with intraocular and oral corticosteroids, cyclosporine A, and infliximab whereas Patient 2 received intravenous methylprednisolone pulses, cyclosporine A, azathioprine, and adalimumab. At TCZ onset they had macular edema (four eyes); visual acuity (VA) impairment (four eyes); vitritis (one eye); and diffuse angiographic signs of vasculitis (periphlebitis) (two eyes). Improvement of VA and OCT was observed following TCZ therapy in both patients. After a follow-up of 18 months (Patient 1) and 10 months (Patient 2), respectively, a corticosteroid sparing effect without any adverse effects was achieved in both cases.

Conclusions: TCZ was effective in two patients with BSCR refractory to anti-TNF-α agents.     

Birdshot Retinochoroidopathy: Differences in Clinical Characteristics between Patients with Early and Late Age of Onset

Purpose: To describe differences in the clinical characteristics of birdshot retinochoroidopathy (BSRC) patients diagnosed early and later in life.

Methods: This is a retrospective cohort study. Age was primarily analyzed and 50 years of age at diagnosis was selected as a cut-off point.

Results: A total of 144 patients (288 eyes) were included; 68 with early-onset and 76 with late-onset BSRC. The younger group had a statistically significant higher rate of more severe iritis (p = 0.04); an average number of non-steroidal immunosuppressants and biologic agents (NSIB) (p = 0.04); and a prolonged time to initiation of NSIB (p = 0.01). There were only four patients (3%) who had >0.5+ cells in the anterior chamber.

Conclusions: Patients with early-onset BSRC carried a higher risk for anterior segment inflammation, had a more prolonged delay to initiation of treatment with NSIB, and required a greater number of NSIBs to achieve remission.     

Long-Term Follow-Up of Patients with Birdshot Retinochoroidopathy Treated with Systemic Immunosuppression

Purpose: Birdshot retinochoroidopathy (BRC) is a rare uveitis syndrome of presumed autoimmune etiology. Therapy with systemic and periocular corticosteroids is of inconsistent efficacy, attendant with numerous potential long-term side effects. Corticosteroid-sparing strategies with agents such as cyclosporine A or azathioprine have been suggested for this disease. Methods: We retrospectively reviewed the medical charts of patients with BRC who were evaluated consecutively at a tertiary-care, referral-based North American uveitis clinic over a 15-year period. Results: Eleven Caucasian patients (22 eyes) were diagnosed with BRC, representing approximately 1% of all cases seen at the uveitis clinic. HLA-A29 was positive in all 11 patients. We elected to treat five patients with azathioprine, methotrexate, cyclosporine A, mycophenolate mofetil, and/or IvIg, as well as systemic or periocular corticosteroid injections. The median period of follow-up for the five treated patients was six years (range: 8 months–13 years). Inflammation was reduced or stabilized in five of five patients. Conclusion: Although the definitive strategy for the management of BRC is unknown, control of intraocular inflammation and preservation of vision is possible with corticosteroid-sparing immunosuppressive agents.     

Standardized Visual Field Assessment for Patients with Birdshot Chorioretinopathy

Purpose: To adapt a standardized technique for assessment of visual fields to the study of patients with birdshot chorioretinopathy and to describe visual field patterns and changes over time for affected individuals. Methods: A retrospective chart review was performed of patients with a diagnosis of birdshot chorioretinopathy who had undergone at least two automated visual field studies. A masked evaluation of each visual field was performed using standardized criteria. Subjective symptoms and visual acuity measurements were compared to visual field data from the same examination dates. Serial visual field studies were evaluated for changes in the findings over time. Results: Six patients met inclusion criteria. A total of 104 visual field studies (ranging from 2–19 studies per eye) were evaluated. Visual field abnormalities occurred in all tested eyes and were observed even in eyes with a Snellen visual acuity of 20/15. Discordant patterns were observed in the two eyes of some individual patients. On serial testing, multiple patterns of visual field abnormality were observed in the same eye over time. Conclusions: A standardized protocol for systematic evaluation of visual field measurements can be used to characterize disturbances of vision in patients with birdshot chorioretinopathy. Abnormalities of visual fields may occur even when central visual acuity is preserved.     

Systemic therapy with conventional and novel immunomodulatory agents for ocular inflammatory disease

Ocular inflammatory disease is the third leading cause of blindness in the United States. In addition to the conventional immunomodulatory agents, which include antimetabolites, alkylating agents, and antibiotics such as cyclosporine, many of which have been used in the treatment of this disease for decades, several new treatment modalities have emerged within the past 10 years. We review in detail the characteristics, safety, and efficacy of the conventional immunomodulators, the more novel agents such as the biologics, and investigational drugs that appear promising in the treatment of ocular inflammatory disease.     

Pattern electroretinographic results after photodynamic therapy alone and photodynamic therapy in combination with intravitreal bevacizumab for choroidal neovascularization in age-related macular degeneration

Purpose To evaluate the changes in pattern electroretinography (PERG) 1 month after photodynamic therapy alone and photodynamic therapy in combination with intravitreal bevacizumab for choroidal neovascularization (CNV) secondary to age-related macular degeneration (AMD). Methods This is a prospective series of 45 eyes with subfoveal CNV secondary to AMD. Twenty eyes were treated with photodynamic therapy (PDT) with verteporfin and 1.25 mg of intravitreal bevacizumab, and 25 patients were treated with PDT alone. Visual acuities and serial PERG recordings were performed both before and 1 month after therapy. Results Following the 1-month therapy period, visual acuity improved in 56% of patients in the PDT group and 76% of patients in the combination group. No significant ocular or systemic adverse effects were observed in either group. According to the PERG results, the mean P50 amplitude was 1.5 ± 0.9 μV before PDT and improved to 2.1 ± 1.1 μV at 1 month after PDT. The mean P50 amplitudes in the combination therapy group before and after therapy were 1.6 ± 0.8 μV and 2.7 ± 1.2 μV, respectively, and the difference was statistically significant between the groups. Conclusions In this small series of eyes with limited follow-up, the combined treatment of PDT with verteporfin and intravitreal bevacizumab seems to be associated with improvement in VA and pattern electroretinographic results when compared to those in the PDT group.     

The outcomes of mycophenolate mofetil therapy combined with systemic corticosteroids in acute uveitis associated with Vogt–Koyanagi–Harada disease

Purpose: To study the effectiveness of mycophenolate mofetil (MMF) as first‐line therapy combined with systemic corticosteroids in acute uveitis associated with Vogt–Koyanagi–Harada (VKH) disease. The outcomes in this group were compared with those of another group of patients with VKH disease who were treated with corticosteroid monotherapy or with delayed addition of immunomodulatory therapy. Methods: This prospective study included 19 patients (38 eyes) diagnosed with acute uveitis associated with VKH disease. Results: The mean follow‐up period was 27.0 ± 11.1 months (range 16–54 months). Corticosteroid‐sparing effect was achieved in all patients. The mean interval between starting treatment and tapering prednisone to 10 mg or less daily was 5.1 ± 1.2 months (range 3–7 months). Ten (53%) patients discontinued treatment without relapse of inflammation. The mean time observed of treatment was 17.3 ± 11.9 months (range 3–41.5 months). Visual acuity of 20/20 was achieved by 38% of the eyes in the corticosteroid group and by 74% in the corticosteroid + MMF group (p < 0.001). Recurrent inflammation of ≥3 times was reduced significantly (p = 0.0383) in the corticosteroid + MMF group (3%) as compared to corticosteroid group (18%). Development of all complications was significantly higher in the corticosteroid group (43%) compared with the corticosteroid + MMF group (8%) (p < 0.001). None of the eyes in the corticosteroid + MMF group developed ‘sunset glow fundus’. Conclusions: Addition of MMF as first‐line therapy to corticosteroids in patients with acute uveitis associated with VKH disease leads to significant reduction in recurrences of uveitis and development of late complications and significantly improves visual outcome.     

激光治疗后不同病程中心性浆液性脉络膜视网膜病变多焦视网膜电图的研究

目的观察经激光治疗后的中心性浆液性脉络膜视网膜病变(central serous chorioretinopathy,CSC)多焦视网膜电图(multifocal electroretinography,mfERG)特点。方法应用RETI Scan multifocal ERG Version3.15系统对25例CSC患者进行双眼mfERG的随访测定,61个六边形从中央到周边共分5个环,测定31°视野范围的一阶反应(first-orderkernel),分别进行双眼比较。结果3~6个月病程组N1波幅值于环2患眼与对侧眼比较差异有显著性(P<0.01),P1波幅值于环1、环2患眼与对侧眼比较差异均有显著性(P<0.01);7~20个月病程组N1波幅值于环1患眼与对侧眼比较差异有显著性(P<0.05),P1波幅值于环1、环2患眼与对侧眼比较差异有显著性(P<0.05)。潜伏期比较,不管是3~6个月病程组还是7~20个月病程组,其患眼与对侧眼比较均无明显差异(P>0.05)。结论3~6个月病程组及7~20个月病程组其黄斑中心区mfERG幅值患眼仍低于对侧眼,mfERG检查能反映其黄斑区功能变化。     

The changes of multifocal electroretinography in the early stage of photodynamic therapy for choroidal neovascularization

To investigate short-term changes in the multifocal electroretinography (ERG) recordings after photodynamic therapy (PDT) for choroidal neovascularization (CNV), 16 patients (17 eyes) with classic CNV confirmed by fluorescein angiography (FA) and indocyanine green angiography (ICGA), including 11 cases (12 eyes) of exudative age-related macular degeneration (AMD), two cases (two eyes) of pathological myopia and three cases (three eyes) of idiopathic causes, were treated using PDT with verteporfin. The multifocal ERGs of these patients were tested with VERIS Science^TM4.0 imaging system. The latencies and average response densities of all six ring retinal regions were measured and compared before PDT and 3 or 7 days after PDT. The latencies and amplitude densities of the N₁ and P₁ waves in all six rings remained unchanged at 3 or 7 days post-treatment (p>0.05). Therefore, there is no significant evidence to suggest an adverse effect from PDT for classic CNV on the outer retinal function in the early stage of treatment, with the aid of the multifocal ERG recordings.     

25G玻璃体切除联合空气填充治疗特发性黄斑裂孔

目的:观察对于特发性黄斑裂孔行25G玻璃体切除联合空气填充治疗后的解剖和视功能结果.方法:前瞻性干预性分析.27例(30眼)特发性黄斑裂孔患者接受25G经睫状体平坦部玻璃体切除术,同时行白内障超声乳化抽吸联合人工晶体置入和玻璃体腔无菌空气填充.所有患者在术前和术后3mo进行最佳矫正视力(logMAR视力)、视野检查和多焦视网膜电流图(multifocal electroretinography, mfERG)检查,使用OCT明确黄斑裂孔闭合情况.结果:首次手术后28眼的黄斑裂孔闭合,平均logMAR视力由术前的0.72±0.22提高到术后的0.29±0.18(P<0.001).中心10°视野检查中,平均偏差(mean deviation, MD)由术前的-3.59±1.83 dB减少到术后的-2.51±1.36 dB (P<0.001),平均模式标准差(pattern standard deviation, PSD)由术前的1.86±0.68 dB减少到术后的1.33±0.32 dB (P=0.001).MfERG中可见术后中心凹和旁中心凹区域的平均振幅明显升高,而4-6环区域的平均潜伏期明显延长(P<0.05).疾病病程长短(P<0.001)和1环区域的术前N1波振幅(P=0.001)对术后最佳矫正视力有预测作用.结论:特发性黄斑裂孔患者行25G玻璃体切除联合空气填充,术后保持1d的面向下体位,有很好的解剖成功率和视功能结果.     

Outcomes of 25-gauge vitrectomy with air tamponade for idiopathic macular hole repair surgery

AIM: To evaluate the anatomic and visual outcomes of 25-gauge vitrectomy combined with air tamponade for the treatment of idiopathic macular hole (IMH). METHODS: Thirty eyes of 27 patients with IMH were included in this prospective interventional study. All patients underwent 25-gauge pars plana vitrectomy (PPV) combined with phacoemulsification and air tamponade. Best corrected visual acuity [BCVA; logarithm of the minimum angle of resolution (logMAR)], perimetry and multifocal electroretinography (mfERG) were conducted before and after the operation. Anatomical changes were evaluated with optical coherence tomography (OCT). RESULTS: The macular holes closed successfully in 28 eyes after the primary vitrectomy. The mean BCVA improved from 0.72±0.22 logMAR preoperatively to 0.29±0.18 logMAR postoperatively (P<0.001). In the visual field of central 10°, the average mean deviation (MD) decreased from -3.59±1.83 dB preoperatively to -2.51±1.36 dB postoperatively (P<0.001) and the average pattern standard deviation (PSD) decreased from 1.86±0.68 dB preoperatively to 1.33±0.32 dB postoperatively (P=0.001). The retinal response densities of mfERG in the foveal and perifoveal area increased significantly, and implicit times of rings 4-6 prolonged significantly too (P<0.05). The symptom duration and baseline N1 amplitude densities at ring 1 had a significant impact on postoperative BCVA (P<0.001, P=0.001, respectively). CONCLUSION: The 25-gauge PPV and air tamponade with 1d prone positioning produced favorable anatomic and functional outcomes.     

光动力疗法联合玻璃体腔注射Bevacizumab治疗息肉状脉络膜血管病变前后多焦视网膜电图的改变

目的 应用光动力疗法（PDT）联合玻璃体腔注射Bevacizumab治疗息肉状脉络膜血管病变（PCV）,探讨治疗前后患者多焦视网膜电图（mfERG）的变化.方法 前瞻性研究.共纳入黄斑型PCV患者13例（13眼）,男8例,女5例,年龄（60.2±7.7）岁.所有患者接受吲哚菁绿眼底血管造影（ICGA）指导下6 mg/m2剂量光敏剂Verteporfin的PDT治疗83 s,3 d后行玻璃体腔注射Bevacizumab 1.5 mg（0.06 ml）.治疗前及治疗后1、3、6个月,记录视力、眼底荧光素血管造影（FFA）、mfERG各环的潜伏期和振幅密度变化及并发症情况.采用Friedman＇s检验观察视功能指标的变化情况,当检验发现显著性改变时,采用Wilcoxon检验以比较治疗前后视功能的改变.结果 仅一例患者因为FFA的持续渗漏而于治疗后3个月随访时接受了再次治疗.治疗后平均logMAR视力逐渐提高,治疗后3个月及6个月视力与治疗前比较,差异有统计学意义（Z=-2.823,P=0.005;Z=-2.620,P=0.009）.与治疗前相比,6个环的潜伏期无明显下降.治疗后3个月,1环P1波（Z=-2.271,P=0.023）及2环的N1、P1波（Z=-2.202,P=0.028;Z=-1.992,P=0.046）振幅密度明显升高.治疗后6个月,1环P1波（Z=-2.432,P=0.015）及2环的N1、P1波Z=-2.158,P=0.031;Z=-2.118,P=0.034）振幅密度的升高仍具有统计学意义.结论 PDT联合玻璃体腔注射Bevacizumab治疗PCV后病灶血管渗漏改善,视力提高,外层视网膜功能部分恢复.mfERG可以作为一种评价联合治疗后PCV患者视功能变化情况的良好指标.     

激光周边虹膜切除术治疗原发性闭角型青光眼的远期疗效观察

目的 探讨激光周边虹膜切除术(LPI)治疗原发性闭角型青光眼(PACG)的远期疗效及安全性.方法 回顾性系列病例研究.收集1992年4月至2002年10月实施LPI治疗且术后随诊时间达5年以上的PACG患者临床资料,根据患者LPI治疗前眼压、视乳头、视野、前房角等情况,将患眼重新分为3组:疑似原发性前房角关闭(PACS)组、原发性前房角关闭(PAC)组、原发性闭角型青光眼(PACG)组,分析LPI治疗后各组患者远期眼压控制、视力及并发症等情况.不同类型的原发性闭角型青光眼之间LPI治疗后眼压控制情况比较采用x~2检验.结果 共收集到符合条件的患者131例(251只眼),其中PACS组18只眼(7.2%),PAC组98只眼(39.0%),PACG组129只眼(51.4%),无法分类的6只眼(2.4%).PACS组、PAC组、PACG组患者LPI治疗后眼压控制满意率分别为88.9%(16/18)、38.8%(38/98)及10.9%(14/129),眼压控制不满意率分别为5.6%(1/18)、48.0%(47/98)及75.2%(97/129),眼压控制失败率分别为5.6%(1/18)、13.3%(13/98)及14.0%(18/129).3组患者LPI治疗后眼压控制情况的差异有统计学意义(x~2=59.08,P=0.000).251只眼中8只眼(3.2%)在LPI治疗后1周至16年发生青光眼急性发作.全部患者在随诊期间未发生大泡性角膜病变.结论 LPI治疗后PACG的眼压控制不如预期的那样好.在大多数青光眼中,LPI可以有效防止闭角型青光眼的急性发作.LPI治疗后PACG、PAC、PACS组患者均存在不同程度的眼压升高危险,需密切随诊,及时处理.     

综合疗法治疗儿童弱视远期疗效分析

目的：近年来统计结果表明,我国儿童弱视发病率高达2％～4％。报告通过用综合疗法治疗591眼儿童弱视的远期疗效,揭示医院验光配镜矫正屈光不正,除调解性斜视外用手术矫正斜视,在医生的指导下开展家庭弱视矫正训练,合理选择治疗方法及疗程,随访3～9a痊愈率达92．0％。 方法：用20g／L的阿托品眼膏散瞳,涂眼1～2次／d,连续3d,麻痹睫状肌后检影验光,在配镜的基础上,辅以家庭精细作业及闪烁红光刺激疗法等治疗。 结果：疗效评价标准：按1996年全国儿童弱视斜视工作会议讨论通过的标准进行评价。591眼痊愈544眼,痊愈率为92．0％,47眼有不同程度的视力减退,复发率为8．0％。 结论：为挽救众多弱视儿童的视力,除用眼镜矫正屈光不正外,广泛开展家庭弱视矫治训练,这是一种既简单有效,而又容易被接受的方法,是普及弱视治疗和巩固疗效的上策。     

Long-term refractive and biometric outcomes following diode laser therapy for retinopathy of prematurity.

PURPOSE: To assess the long-term refractive and biometric outcomes of diode laser-treated eyes in threshold retinopathy of prematurity (ROP). METHODS: Cycloplegic autorefraction and biometry (Zeiss IOLMaster) were performed, at a mean follow-up of 11 years, on 16 laser-treated eyes with threshold ROP and 9 comparison eyes with subthreshold untreated ROP. RESULTS: The laser-treated eyes had a mean spherical equivalent of -2.33 D with a mean astigmatic error of 1.38 D. The comparison eyes had a mean spherical equivalent of +1.07 D with a mean astigmatic error of 0.42 D. This trend toward increased myopia in treated eyes did not achieve statistical significance (p=0.08). The myopia in the laser group appeared to be slowly progressive in nature when compared with earlier refractive data for these patients. The laser-treated eyes had reduced anterior chamber depth (ACD) compared with the subthreshold eyes (p=0.02). When physiologic accommodation was inhibited by cycloplegic drops, the anterior chamber deepened by 0.13 mm in the laser-treated eyes and by 0.06 mm in the comparison eyes. This effect of accommodation on ACD did not differ significantly between the two groups (p=0.23). The laser-treated eyes and the comparison eyes did not differ significantly in terms of axial length, corneal power, corneal diameter, or lens power. However, both groups had steeper corneas, shallower anterior chambers, and shorter axial lengths when compared with historical full-term controls. CONCLUSIONS: Myopia in premature infants requiring laser treatment for ROP is associated with a shallowing of the anterior chamber and a steepening of the cornea. Physiological accommodation is not impaired by laser therapy or by severe ROP.     

视觉训练对症状性集合不足儿童疗效的持久性观察

目的探讨症状性集合不足儿童经医院内规范视觉训练（OBVT）治愈后的持久性疗效,并分析其影响因素。方法前瞻性研究。选择在门诊被诊断为症状性集合不足,且接受12周OBVT后成功治愈者40例,平均（12.4±3.4）岁。在训练前,训练结束后1周、3个月、6个月均测量双眼视功能,并进行视疲劳症状问卷评分。采用重复测量方差分析比较训练结束后1周、3个月、6个月患者的视疲劳症状问卷评分（CISS）、集合近点（NPC）、近距正融像性聚散范围值（PFV）;训练结束后6个月复发者与未复发者的训练前参数比较采用独立样本t检验或Mann-Whitney检验;训练结束后6个月CISS与训练前参数关系进行Pearson相关分析。结果训练结束后3个月,85%（34/40）患者无体征及症状;训练结束后6个月,68%（26/38）患者无体征及症状。训练结束后1周、3个月、6个月CISS评分、NPC、PFV差异均无统计学意义;训练结束后6个月复发者与未复发者之间训练前NPC、CISS及调节幅度（AA）差异有统计学意义（Z=-3.212,P＜0.01;Z=-2.461,P＜0.05;t=2.264,P＜0.05）,PFV均值差异无统计学意义;训练结束后6个月CISS评分与训练前NPC及CISS分值呈正相关（r=0.420、0.473,P＜0.01）,与训练前AA呈负相关（r=-0.361,P＜0.05）。结论大部分症状性集合不足儿童经视觉训练治愈后疗效可持续6个月;训练效果的持久性可能与训练前NPC远移程度,CISS分值大小及AA大小有关。     

激素冲击疗法治疗特发性脱髓鞘视神经病变的远期疗效

目的:探讨甲基强的松龙冲击疗法治疗特发性脱髓鞘视神经病变的远期疗效。方法:对2003-01/2005-12在我院确诊为特发性脱髓鞘视神经病变患者81例104眼进行回顾性分析,随机分为A组和B组。A组采用甲基强的松龙冲击辅助营养神经及扩血管药物治疗,B组仅采用营养神经及扩血管药物治疗,观察两组患者首次发病后1,2,5a的视力、视野、视觉诱发电位的变化及复发率等。结果:各观察时间点两组患者视力提高、视野改善、视觉诱发电位恢复,差异无统计学意义(P>0.05);自发病初5a内A组患者复发率较B组明显降低,差异有统计学意义(P<0.05)。结论:甲基强的松龙冲击疗法能有效降低特发性脱髓鞘视神经病变患者的复发率。