Effect of the duration of immunomodulatory therapy on the clinical features of recurrent episodes in Vogt–Koyanagi–Harada disease

Purpose: To evaluate the duration of immunomodulatory therapy (corticosteroids, immunosuppressive drugs) with regard to the rate of relapses and clinical features (exudative retinal detachment or anterior uveitis) in inflammatory episodes of Vogt–Koyanagi–Harada disease. Methods: Data of all 42 patients diagnosed with acute uveitis associated with VKH disease during the period of January 2005 to December 2008 at the Pitié‐Salpêtrière Hospital or at the Lariboisière Hospital in Paris, France were extracted by chart review. Results: There were 31 patients (73.8%) with episodes of recurrence and were included in the study. At the first recurrence, 81% (13 patients) of exudative retinal detachments (ERD) were associated with an initial immunomodulatory treatment conducted ≤6 months (3.76 months ± 2.67). Conversely, an initial treatment duration of >6 months was associated with anterior uveitis signs for 66% of patients (eight patients) with anterior first recurrence (p = 0.0061). On second episode of recurrence, 75% of patients (three patients) who developed exudative retinal detachment had been managed by immunomodulatory therapy for ≤6 months with the total duration of immunomodulatory treatment ≤6 months during previous inflammatory episodes. Conversely, all 16 patients who presented anterior uveitis with additional manifestations (optic disc oedema, macular oedema, vitritis and/or ‘Sunset glow’ fundus) have been treated for more than 6 months or treated during the initial occurrence lasting more than 9 months (p = 0.0035). Conclusions: The duration of systemic corticosteroids (and/or immunosuppressive drug therapy) for ≤6 months at first and second recurrence was associated with features of further exudative retinal detachment instead of anterior uveitis in VKH disease.     

梅毒性葡萄膜炎3例

目的:评估梅毒性葡萄膜炎患者的临床表现和视力。方法:梅毒性葡萄膜炎患者3例在USM医院接受治疗。结果:患者3例被诊断为继发性梅毒性葡萄膜炎,这3例患者患病之前均不知道患有梅毒,但他们有明确的乱交史。并且每个月都伴有逐步的视力下降。其中两人伴有发热、眼痛、眼前悬浮物。视力从6/12到手动。所有患者均出现前葡萄膜炎,玻璃体炎和视神经炎。第一例患者出现了多灶性脉络膜视网膜炎,并伴有渗出性视网膜脱离。第二例患者出现渗出性视网膜脱离,而第三例患者仅出现了脉络膜视网膜炎。所有患者每周注射苄青霉素2.4MU,共4wk,其中2例患者口服多西霉素200mg2次/d,共3mo。治疗效果良好,其中2例患者有显著的视力上升,分别从6/120到6/21和6/12到6/6。其中较严重的1例患者出现逆转录酶为阳性。结论:眼梅毒作为非肉芽肿性的炎症与渗出性视网膜脱离有关。治疗后虽然视力恢复比较缓慢,但普遍有良好的效果。     

Syphilitic uveitis: report of 3 cases

AIM: To evaluate the clinical manifestations and visual outcome of syphilitic uveitis patients. METHODS: Case series of three patients with syphilitic uveitis who were managed in Hospital USM. RESULTS: Three patients were diagnosed to have uveitis secondary to Syphilis. All three patients were not known to have syphilis prior to presentation but have positive history of sexual promiscuity. All patients presented with progressive blurring of vision for average of one-month duration. Two of them have association with fever, ocular pain and floaters. Visual acuity at presentation ranges from 6/12 to hand movement. Mild anterior uveitis (non-granulomatous), vitritis and papillitis were presence in all the patients. First patient has multifocal chorioretinitis with exudative retinal detachment. The second patient presented with exudative retinal detachment while the third patient has chorioretinitis only. All the patients were treated with intramuscular benzyl-penicillin 2.4 MU weekly for 4 weeks and two of them received oral doxycycline 200mg twice daily for 3 months. The uveitis responded well to the treatment and two of them showed dramatic visual improvement from 6/120 to 6/21 and 6/12 to 6/6. The one with worse outcome was confirmed to have positive retroviral. CONCLUSION: Ocular syphilis presented here as non- granulomatous inflammation associated with exudative retinal detachment. Final visual outcome is generally good despite slow improvement after treatment.     

梅毒性葡萄膜炎3例

目的：评估梅毒性葡萄膜炎患者的临床表现和视力. 方法：梅毒性葡萄膜炎患者3例在USM医院接受治疗. 结果：患者3例被诊断为继发性梅毒性葡萄膜炎,这3例患者患病之前均不知道患有梅毒,但他们有明确的乱交史.并且每个月都伴有逐步的视力下降.其中两人伴有发热、跟痛、眼前悬浮物.视力从6/12到手动.所有患者均出现前葡萄膜炎,玻璃体炎和视神经炎.第一例患者出现了多灶性脉络膜视网膜炎,并伴有渗出性视网膜脱离.第二例患者出现渗出性视网膜脱离,而第三例患者仅出现了脉络膜视网膜炎.所有患者每周注射苄青霉素2.4MU,共4wk,其中2例患者口服多西霉素200mg 2次/d,共3mo.治疗效果良好,其中2例患者有显著的视力上升,分别从6/120到6/21和6/12到6/6.其中较严重的1例患者出现逆转录酶为阳性. 结论：眼梅毒作为非肉芽肿性的炎症与渗出性视网膜脱离有关.治疗后虽然视力恢复比较缓慢,但普遍有良好的效果.     

巩膜外垫压术治疗家族性渗出性玻璃体视网膜病变合并孔源性视网膜脱离

目的 探讨巩膜外垫压术治疗家族性渗出性玻璃体视网膜病变合并孔源性视网膜脱离的效果.方法 回顾性分析2011年1月至2012年10月间在我院住院行巩膜外垫压术（或联合环扎术）治疗的6例（6只眼）家族性渗出性玻璃体视网膜病变合并孔源性视网膜脱离患者的临床资料.本组所有患者视网膜裂孔检查明确,且位于赤道或赤道前,增生性玻璃体视网膜病变C2级以下;具有阳性家族史;荧光素眼底血管造影特征明显.其中1个裂孔者4只眼,2个裂孔者2只眼.1例硅胶海绵纵行垫压,5例硅胶轮胎环行垫压并联合环扎.所有患者术后均补充视网膜激光光凝.结果 6例患者术后视网膜均复位良好,视力改善,经激光治疗无灌注区,随访稳定.结论 对于家族性渗出性玻璃体视网膜病变合并孔源性视网膜脱离患者,选择部分病例行巩膜外垫压手术治疗,术后及时补充视网膜激光光凝,可取得很好效果.     

Vogt-Koyanagi-Harada disease after head trauma

PURPOSE: To report two cases of Vogt-Koyanagi-Harada disease after closed head trauma. METHODS: Case report. RESULTS: Two patients, one male and one female, developed headache, dysacusis, vertigo, tinnitus, and hair hypersensitivity shortly after a closed head trauma and, 10 and 18 days later, a bilateral uveitis with papillitis and exudative retinal detachment in one and a bilateral mild uveitis with macular exudative detachment in the other. The ocular lesions resolved with intravenous high-dose steroid therapy, but recurred after reduction of the dosages, requiring further steroid therapy. The course of the disease in both patients, with the appearance of fundus depigmentation and pigment clumping, and the occurrence of a concomitant ocular and auditory relapse in one, were typical of Vogt-Koyanagi-Harada disease. CONCLUSIONS: Vogt-Koyanagi-Harada disease may appear after a closed head trauma suggesting that even an indirect trauma in melanocyte-containing tissue may induce an inflammatory response within the eye.     

Rhegmatogenous retinal detachment complicating cytomegalovirus retinitis.

Six eyes in four patients with cytomegalovirus retinitis developed retinal holes and retinal detachment. Holes appeared in areas of necrosis and were typically large and round with shaggy edges. Differentiation from exudative detachment was sometimes difficult because of obscuration of the fundus by vitreous haze. Cryotherapy was successful in closing open breaks without detachment; cryotherapy and exoplant surgery were used effectively to treat the initial detachments. The occurrence of massive periretinal proliferation and late development of new areas of hole formation or retinitis complicated therapy for these detachments.     

Coats'-type retinitis pigmentosa

Coats'-like changes (i.e., retinal telangiectasia and/or exudative detachment) have been reported in as many as 1.2 to 3.6 percent of patients with retinitis pigmentosa. In severe cases this disorder may progress to total retinal detachment and visual loss in the context of longstanding retinitis pigmentosa. Forty-six cases of Coats'-type retinitis pigmentosa gathered from the literature are reviewed. Historical and epidemiological features, hereditary factors, clinical features, histopathological findings, pathogenesis, differential diagnosis, prognosis and possible treatment are discussed.     

Internal drainage for chronic macula-involving serous retinal detachment in idiopathic central serous chorioretinopathy

Conventional treatment of idiopathic central serous chorioretinopathy (ICSC) consists of argon laser, photodynamic therapy, or observation. However, in cases of atypical bullous ICSC with exudative detachment preventing any laser therapy, a surgical approach with external drainage of fluid has been performed. We present a case of ICSC with persistent macula involving exudative retinal detachment without evidence of uveitis that responded favorably to internal drainage by vitrectomy along with a scleral buckle placement. Our case, treated with internal drainage, also demonstrated successful long-term reattachment of the serous retinal detachment without any additional complications from the surgery.     

Photodynamic therapy of choroidal hemangioma in sturge-weber syndrome, with a review of treatments for diffuse and circumscribed choroidal hemangiomas

We report three new cases of patients with Sturge-Weber Syndrome and symptomatic retinal detachments from diffuse choroidal hemangiomas successfully treated with photodynamic therapy (PDT) and review medical literature on the available treatment options for choroidal hemangiomas. All patients were treated with a single session of PDT with verteporfin infused at a concentration of 6 mg/m² and treated for 83 seconds with 689-nm Zeiss laser that was delivered with total energy level of 50 J/cm² with an intensity of 600 mW/cm². The exudative retinal detachment (RD) and macular edema completely resolved in all cases by 1-4 months after PDT treatment. Visual acuity improved in all three cases with diminished tumor size in the areas of treatment. One case was followed for 5 months, another for 2 years, and the third case for 6 years, with no recurrence of exudative RD. PDT is an effective treatment option for visual deterioration from exudative retinal detachment in patients with diffuse choroidal hemangiomas.     

艾滋病并发巨细胞病毒性视网膜炎32例临床分析

【摘要】 目的 分析获得性免疫缺陷综合征（艾滋病）患者发生巨细胞病毒性视网膜炎及免疫恢复 性葡萄膜炎的疾病特征。设计 回顾性病例系列。研究对象 北京佑安医院眼科诊治的艾滋病合并巨 细胞病毒性视网膜炎患者32例。方法 对上述患者进行与艾滋病相关的免疫学检测;并进行视力、 眼压、眼前节检查、彩色眼底照相及荧光素眼底血管造影等眼科检查,并观察其临床特征。主要指 标 视力、眼压、眼底、CD4+T细胞计数。结果 32例患者的视力为光感～1.0;眼压8~16 mm Hg;眼 前节检查8例患者（4例为免疫恢复性葡萄膜炎）可见眼前节反应阳性（前房闪辉及角膜后KP）;32 例患者彩色眼底像均可见典型巨细胞病毒性视网膜炎眼底改变;8例（25%）CD4+T细胞＜10个/μL ,视力为光感～0.8,其中3例发生免疫恢复性葡萄膜炎,1例发生视网膜脱离;15例（46.88%） CD4+T细胞在10～50个/μL,视力为眼前指数～1.0,其中1例发生免疫恢复性葡萄膜炎,1例发生视 网膜脱离;9例（28.12%）CD4+T细胞＞50个/μL,无患者发生免疫恢复性葡萄膜炎及视网膜脱离。 19例患者（含4例免疫恢复性葡萄膜炎患者）合并其他全身机会性感染。结论 CD4+T细胞＜50个/μ L的患者更易患巨细胞病毒性视网膜炎,其视力预后差; CD4+T细胞
＜10个/μL的患者更易发生免疫恢复性葡萄膜炎,且眼前节反应明显,更易合并其他全身机会性感 染。（眼科, 2012, 21: 405-408）     

Management of exudative retinal detachment in choroidal melanoma

Exudative retinal detachment is the most common source of visual loss associated with malignant melanoma of the uveal tract. Management has historically been conservative, leading to irreversible visual loss from photoreceptor damage during the several months needed for post-radiation resolution. The purpose of this paper is to describe timely vitreoretinal surgical intervention for exudative retinal detachments associated with choroidal melanomas. This was an interventional case series including six consecutive patients with malignant melanoma who experienced VA reduction secondary to associated exudative retinal detachment. Patients underwent complete ophthalmic evaluation and B-scan ultrasound. Treatment included proton-beam radiation or brachytherapy, prognostic transretinal tumour biopsy with 25-gauge vitrector and surgical treatment of exudative retinal detachment, including vitrectomy and drainage of subretinal fluid at the time of irradiation. Successful management of exudative retinal detachments associated with choroidal melanomas was observed in all cases, with significant restoration of vision. Steady regression of tumour thickness was noted clinically and ultrasonographically, without extrascleral extension or metastasis, and with no recurrence of exudative retinal detachment found over follow up. In the present study, the investigators have showed effective surgical treatment of exudative retinal detachment associated with malignant melanoma. These patients had significant restoration of vision, confirming that timely intervention of exudative retinal detachment associated with malignant melanoma can reverse visual loss in these patients. These findings are in contrast to previous reports of irreversible visual loss after exudative retinal detachments, and suggest that photoreceptor atrophy might play a role in visual loss associated with chronic exudative retinal detachments.     

Case of retinochoroiditis associated with porcine ascarids diagnosed by antibodies in subretinal fluid

PURPOSE: To determine the cause of retinochorioditis in a patient with a granulomatous retinal exudate and an exudative retinal detachment. CASE: A 45-year-old man presented at another hospital with increased visual disturbances of the left eye. He was diagnosed with uveitis, and treated with topical steroids for 1 month. However, the uveitis worsened, and he was referred to our hospital. Ophthalmoscopy showed a yellowish-white granulomatous exudate, and an exudative retinal detachment in the lower peripheral retina. The retinal detachment worsened and affected the macula. Pars plana vitrectomy was performed, and the retina was reattached. During the surgery, ocular samples were collected for further examinations. The titers of antibodies against 12 kinds of ascaridis were examined, and elevated titers of specific antibodies against porcine ascarids were detected in the subretinal fluid, but not in the aqueous humor, vitreous, or serum. CONCLUSIONS: Vitrectomy with the collection of ocular samples, especially subretinal fluid, was a key procedure in the diagnosis and treatment of retinochoroiditis associated with the porcine ascarids.     

Clinical characteristics and outcomes of cytomegalovirus retinitis in persons without human immunodeficiency virus infection

PURPOSE: To describe the characteristics and outcomes of patients with cytomegalovirus (CMV) retinitis in the absence of human immunodeficiency virus (HIV) infection. DESIGN: Retrospective cohort study. METHODS: Consecutive patients with CMV retinitis in the absence of HIV infection were identified at a university hospital. Demographic and clinical characteristics were noted at the time of CMV retinitis. Outcomes were determined retrospectively. Main outcome measures were rates of second eye involvement, vision loss, rhegmatogenous retinal detachment (RD), immune recovery uveitis, progression of retinitis, and mortality. RESULTS: The clinical characteristics of CMV retinitis in 18 patients (30 eyes) without HIV infection diagnosed between January 1, 1984, and April 13, 2003, were similar to those of patients with HIV infection. The incidences of visual loss to the levels of 20/50 or worse and of 20/200 or worse were 17% per eye-year and 14% per eye-year, respectively. The observed incidence of RD was 3.7% per eye-year, and the mortality rate was 23% per person-year. Following reduction of immunosuppression, 10 patients (56%) who discontinued anti-CMV therapy remained free of retinitis progression. The incidence of immune recovery uveitis was 13% per person-year. CONCLUSIONS: In our series, CMV retinitis in patients without HIV infection had a clinical course similar to that in patients with AIDS treated with highly active antiretroviral therapy (HAART), except the incidence of RD was lower for patients without AIDS. A substantial number of patients no longer required long-term anti-CMV therapy after adjustment of immunomodulatory therapy.     

网脱术后并发渗出性视网膜脱离11例报告

目的：探讨网脱术后并发渗出性视网膜脱离的原因、临床表现及预后。方法：分析11例视网膜脱离行外路手术后的病例，裂孔性视网膜脱离6眼，锯齿缘离断性视网膜脱离3眼，巨大裂孔性视网膜脱离2眼，行硅压，环扎，外放液，冷凝手术。结果：11只眼于手术3天一10天出现了不向程度的渗出性视网膜脱离，经保守治疗视网膜下渗液吸收，视网膜复位。结论：渗出性视网膜脱离为视网膜脱离术后并发症，正确诊断、处理可以治愈。     

Treatment of cytomegalovirus retinitis with DHPG in a patient with AIDS

A 31-year-old homosexual man with AIDS, bilateral cytomegalovirus (CMV) retinitis and optic neuritis in one eye, was treated with DHPG. The drug is an acyclic nucleoside analogue of guanosine with antiviral activity. The visual acuity at the start of treatment was R.E.: no light perception and L.E.: 1.25. There was bilateral regression of retinal exudates on DHPG 5 mg/kg twice a day during 2 weeks. The visual result however was poor because of the optic nerve involvement, which did not improve during DHPG treatment. Four weeks later there was a recurrence of retinitis with the development of exudative retinal detachment in the eye with optic neuritis, despite maintenance therapy of 5 mg/kg once a day Monday through Friday. The dose was increased to 5 mg/kg twice a day, but after 1 week treatment had to be discontinued because of neutropenia. Eight days later treatment was restarted with DHPG 5 mg/kg in a single daily dose during 17 days, which led to remission of retinitis but retinal reattachment did not occur. Thereafter maintenance therapy was continued. Visual acuity remained unchanged. DHPG appears to be effective in treating cytomegalovirus retinitis but long-term suppressive therapy would be necessary to prevent recurrence of the retinitis.     

Ocular syphilis: the new epidemic

AIM: To present the clinical presentation, diagnosis, and management of syphilitic uveitis in the context of an epidemic of syphilis in the UK. METHOD: Retrospective clinical case series. RESULTS: Six new cases of syphilitic uveitis presented to the Manchester Uveitis Clinic in 2004, after a 15-fold increase in the incidence of syphilis in the UK, including 615 cases in Greater Manchester in the 5 years to 2004. Four cases had secondary syphilis, two had latent disease, two had no rash, and two were HIV positive. Ocular involvement included anterior or panuveitis, retinitis, retinal vasculitis, and papillitis. All resolved on treatment including intramuscular procaine penicillin G with oral probenecid. CONCLUSIONS: Syphilis is much more common recently and syphilitic uveitis should be considered in all patients with rash and/or headache, where there is retinitis and/or retinal vasculitis, or in any uveitis of uncertain origin. Treatment is that of neurosyphilis.     

Coats病的手术治疗

目的总结手术治疗Coats病的效果。方法对Coats病伴不同程度渗出性视网膜脱离的患者16例17只眼行巩膜外冷凝术和玻璃体手术治疗，手术后随访时间4.25～62.25个月, 平均随访时间13.10个月。结果手术治疗后8只眼视网膜完全复位(无硅油充填)，视网膜复位率47%；1只眼在硅油充填的情况下视网膜在位；8只眼手术后视网膜未能完全复位。5只眼手术后视力提高，2只眼视力稳定，7只眼视力下降。手术并发症有一过性渗出性视网膜脱离加重，视网膜前局限性增生，白内障形成，继发青光眼和玻璃体积血。结论Coats 病伴视网膜脱离经手术治疗后大多数病例视网膜可复位，部分患者视力提高。(中华眼底病杂志,2005,21:145-147)     

Tuberculous Uveitis Presenting with a Bullous Exudative Retinal Detachment: A Case Report and Systematic Literature Review

Purpose: To describe tuberculous uveitis (TU) presenting as a bullous retinal detachment (RD) and to perform a comprehensive literature review on TU with similar features.

Methods: Observational case report and systematic literature review.

Results: An 84-year-old woman presented with bilateral granulomatous uveitis and bullous RD in the left eye. The interferon gamma release assay was strongly positive, but all other tests were unremarkable. The patient was diagnosed with TU and started on anti-tubercular therapy (ATT) and systemic steroids with excellent treatment response. Twenty-six articles (32 cases) reported TU with exudative RD. Choroidal tuberculoma was the most common clinical manifestation, followed by optic disc edema and retinal exudate. Systemic steroids with ATT improved vision in more patients (78.6%) than ATT alone (50.0%) or oral steroids followed by ATT (50.0%).

Conclusion: Atypical presentations of TU make diagnosis and treatment difficult. A high level of suspicion for TU is needed to minimize ocular morbidity.     

Exudative retinal detachment due to small noncalcified retinal astrocytic hamartoma

PURPOSE: To report a case of exudative retinal detachment due to small noncalcified retinal astrocytic hamartoma and review pertinent literature. DESIGN: Case report and review of literature. METHODS: Clinical examination, fluorescein angiography, optical coherence tomography, and laser treatment were performed. RESULTS: Exudative macular detachment caused by a small noncalcified retinal astrocytic hamartoma confirmed by optical coherence tomography regressed completely after laser therapy. Visual acuity improved only slightly because lamellar macular thinning developed after subretinal fluid and macular exudates resolved. CONCLUSIONS: Small noncalcified, parafoveal retinal astrocytic hamartomas may cause macular retinal detachment. Optical coherence tomography may aid in the diagnosis of the tumor. Argon laser photocoagulation may induce tumor regression and resolution of exudative detachment. Final visual acuity may be limited in some cases.