Vitrectomy techniques in late-stage Coats'-like exudative retinal detachment

Retinal telangiectasia is the hallmark of Coats' disease. In the late stages, leakage from these abnormal vessels can result in a total, bullous exudative retinal detachment with cholesterol-laden subretinal fluid. Secondary angle-closure glaucoma may result in a blind and painful eye which may require enucleation or evisceration. Surgical reattachment of the retina and destruction of the retinal telangiectasia may preserve these eyes. We have found that vitrectomy, internal drainage of subretinal fluid and cholesterol, direct treatment of the retinal telangiectasia with intraocular diathermy and intravitreal gas or silicone oil injection are effective surgical techniques for salvaging these severely damaged eyes.     

视网膜冷冻手术联合玻璃体注射曲安奈德治疗伴有渗出性视网膜脱离Coats病的初步观察

目的 观察视网膜冷冻手术联合玻璃体注射曲安奈德治疗伴有渗出性视网膜脱离Coats病的疗效.方法 前瞻性连续病例研究.21例伴有渗出性视网膜脱离的Coats病患者的21只眼纳入研究,其中,男性19例,女性2例,年龄2～18岁;部分视网膜脱离15只眼,属3A期,完全视网膜脱离6只眼,属3B期.所有患眼均接受视网膜冷冻手术联合玻璃体注射曲安奈德治疗,3只眼辅助巩膜切开引流视网膜下液.经视网膜冷冻手术联合玻璃体注射曲安奈德治疗后,4只眼针对残余异常血管再行视网膜光凝或冷冻手术.治疗后随访3～15个月,平均随访时间(7±3)个月.观察视力、眼压、眼位及眼球运动、裂隙灯、间接检眼镜及彩色眼底像,比较异常血管变化,视网膜下液及渗出的吸收情况等.以末次随访为疗效判定时间点.结果 治疗后眼压增高6只眼,均局部药物控制.随访结束时,视网膜复位19只眼,局限视网膜脱离1只眼,视网膜全脱离1只眼.视力提高3只眼,不变14只眼,下降2只眼.新发生斜视1只眼.新发生白内障或白内障加重4只眼.结论 视网膜冷冻手术联合玻璃体注射曲安奈德治疗伴有渗出性视网膜脱离Coats病,可使大部分患者视网膜复位,视功能可以有效保存.     

闭角型青光眼伴有深前房的常见原因

目的 分析闭角型青光眼伴深前房的常见原因，提供临床参考。方法 研究我院青光眼专科门诊及住院患者中深前房伴闭角型青光眼的患眼24例，进行眼压、房角镜和超声生物显微镜检查。结果 发现主要有4种类型：继发于炎症(4例)或新生血管(6例)的青光眼，伴有虹膜一睫状体囊肿的青光眼(5例)，巩膜扣带术后所致的青光眼(5例)和高褶虹膜型青光眼(4例)。结论 闭角型青光眼伴深前房是闭角型青光眼中的特殊类型，多为继发性闭角型青光眼且尽管中央前房不浅但周边前房大多变浅。     

Unusual ocular presentation of von Hippel-Lindau disease

CASE REPORT: We report a young girl who first presented with a unilateral total exudative retinal detachment diagnosed as Coats' disease. Eight years later, when she presented with classical retinal hemangioblastomas with reduced vision in the fellow eye, the diagnosis of von Hippel-Lindau (VHL) disease was confirmed. COMMENTS: This case highlights the importance of considering the possibility of VHL in atypical cases of Coats' disease and unusual sporadic cases of unexplained unilateral exudative retinal detachment. The identification of VHL mutations and subsequent screening allows early diagnosis and treatment of asymptomatic retinal or central nervous system hemangioblastomas, as well as other malignancies associated with this syndrome.     

Retinal angiomatoses

The three classical angiomatoses of the retina are described on the basis of the clinical picture. If early diagnosis is possible, the otherwise inevitable progression to exudative retinal detachment and secondary glaucoma may be avoided through extensive coagulation by laser, xenon or cryopexy. - The prognosis is best in Leber's retinitis; it in much poorer in cases of von Hippel-Lindau angiomatosis and primary Coats' disease.     

Selective photocoagulation in Coats' disease: ten-year follow-up

PURPOSE: The diagnostic hallmark of Coats' disease is development of "light bulb" telangiectasis in the retinal periphery, leading to posterior pole intraretinal and subretinal exudation. Even after complete obliteration of all abnormal vessels and resorption of all exudates, follow-up examinations are mandatory for several years. METHODS: We retrospectively analyzed the charts, pictures and/or fluorescein angiographies of a series of 32 consecutive patients, in which the diagnosis of Coats' disease was made. All patients underwent selective photocoagulation of the telangiectasis using a yellow-dye laser. Efficacy of treatment was monitored with drawings and/or fluorescein angiographies. RESULTS: All our patients had unilateral disease, with macular involvement and exudative retinal detachment. Visual acuity improved in one patient, decreased from light perception to blindness in another, and was unchanged in the remainder. CONCLUSIONS: This is the first report of anatomical benefits from treatment with a yellow-dye laser (i.e., selective photocoagulation without cryotherapy or drainage) despite the presence of a severe form of Coats' disease. Our young Coats' patients responded quickly to treatment. With successful obliteration of the abnormal vasculature, exudates began to absorb in about 4 to 8 weeks although, in some cases, 10 to 12 months were needed to obtain resolution of the exudative detachment. Poor visual outcome of 20/100 or worse was common. In Coats' patients, the key issue is continuous control and long-term follow-up.     

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)     

Peripheral retinoschisis and exudative retinal detachment in pars planitis

PURPOSE: To review and describe bullous retinoschisis and exudative retinal detachment in patients with pars planitis. METHODS: Retrospective, multicenter study of patients with pars planitis who presented with retinoschisis and exudative retinal detachments. RESULTS: The authors describe 13 eyes of 9 patients with pars planitis who presented with inferior peripheral retinoschisis and/or exudative retinal detachment. Four patients were male; five were female. Patients' ages ranged from 8 years to 35 years (median, 12 years). The follow-up interval ranged from 1 month to 10 years (median, 4 years). These peripheral retinal elevations had a tendency to remain stable, although those with telangiectatic vessels or vasoproliferative tumors occasionally demonstrated an increase in accumulation of exudate. Treatment of such eyes with cryotherapy, or low-dose plaque radiotherapy, resulted in vasoproliferative tumor and telangiectatic vessel regression, absorption of the hard exudate, and resolution of the retinal elevation in four of five eyes. One patient had spontaneous regression of the retinoschisis cavity over a 4-year period. CONCLUSION: Patients with pars planitis may present with bullous retinoschisis and/or exudative retinal detachment. These findings may be related to a Coats disease-like vascular response (telangiectatic vessels and vasoproliferative tumors) secondary to chronic inflammation. Treatment of the vascular leakage tended to result in resolution of the detachment and/or schisis.     

Acute angle-closure glaucoma resulting from spontaneous hemorrhagic retinal detachment in age-related macular degeneration: case reports and literature review

PURPOSE: Acute angle-closure glaucoma resulting from massive subretinal hemorrhage is a rare and catastrophic complication in age-related macular degeneration. Anticoagulant usage had been strongly correlated with this complication in previously reported cases. METHODS: Four patients (4 eyes), 3 men and 1 woman, developed angle-closure glaucoma with diffuse subretinal hemorrhage and total hemorrhagic retinal detachment. RESULTS: Serial funduscopic examinations and echographic studies in 2 eyes showed that the blood gradually accumulated in the subretinal space. It took more than 10 days for the bleeding to build up to bullous hemorrhagic retinal detachment and secondary glaucoma. Anti-glaucomatous agents were given and sclerotomy was performed in 3 of the 4 patients. Phthisical changes were observed subsequently in these 3 eyes. The eye that received early drainage of blood was an exception, and a small degree of residual acuity was retained. Three of the 4 patients had diabetes mellitus, and hypertension and vascular diseases were also present in the same 3 patients. CONCLUSIONS: Diabetes mellitus might be a predisposing factor for the impaired hemostasis. Anti-glaucomatous agents were of no effect in the management of intraocular pressure. Sclerotomy and drainage of blood help control intraocular pressure and relieve ocular pain. Poor final visual acuity is inevitable. However, phthisical changes might be prevented with early sclerotomy and drainage of blood.     

Bilateral Coats' disease: long-term follow up

PURPOSE: To report on the long-term follow-up of a female patient with bilateral Coats' disease, who showed marked asymmetry between the two eyes. METHODS: A five year old girl presented in 1978 with leukocoria in a blind right eye. A total exudative retinal detachment and extensive retinal telangiectasiae were noted. In the other eye, there was a localized area of retinal exudation and vascular abnormality in the supero-temporal periphery. Ultrasonography showed no evidence of intraocular tumour in the right eye and a clinical diagnosis of bilateral Coats' disease was made. RESULTS: In 1995, the area or retinal exudation in the left eye increased and laser photocoagulation was applied successfully. To date, no disease recurrences have occurred. CONCLUSION: Although Coats' disease is usually unilateral, bilateral, asymmetrical involvement may occur on rare occasions. Long-term follow-up of the least affected eye is necessary so that late complications can be identified early and treated adequately to prevent visual loss.     

A long-term follow-up study of severe variant of central serous chorioretinopathy

PURPOSE: To facilitate understanding of the long-term course and visual outcome of a severe variant of central serous chorioretinopathy. DESIGN: Consecutive observational case series. PATIENTS AND METHODS: The authors reviewed 25 patients with multifocal posterior pigment epitheliopathy and bullous retinal detachment, who had a mean follow-up time of 10.6 years (range, 6-22 years), with reference to the demographic feature, fundus changes, recurrence, and final anatomic and visual outcome. Two patients underwent optical coherence tomography. RESULTS: The patients were 21 men and 4 women, with a mean age at disease onset of 43.1 years (range, 30-63 years). Twenty-one patients were otherwise healthy, and four developed ocular disease during systemic corticosteroid therapy for metabolic or autoimmune diseases including systemic lupus erythematosus. The disease was bilateral in 21 patients (84%). Nine patients (36%) presented initially with classic central serous chorioretinopathy, followed by its severe variant 7 months to 9 years later. Active disease was characterized by multifocal exudative lesions in the posterior pole and bullous retinal detachment with shifting subretinal fluid in the inferior periphery. Optical coherence tomography of exudative lesions disclosed cloudy and fibrinous subretinal fluid. The exudative lesions were self-limited or responded to photocoagulation. During the follow-up period, 13 patients (52%) showed 1 to 5 recurrent disease, but the disease eventually became quiescent with multifocal atrophic scars in the posterior pole with or without atrophic tracts in the inferior periphery. Final best-corrected visual acuity was 2020 or better in 24 of 46 affected eyes (52%) of 25 patients and 2040 or better in 37 eyes (80.4%). CONCLUSIONS: A severe variant of central serous chorioretinopathy characterized by multifocal posterior exudations and bullous inferior retinal detachment with shifting subretinal fluid may affect otherwise healthy, middle-aged males or individuals receiving systemic corticosteroid therapy for metabolic or autoimmune diseases. Exudative chorioretinal lesions are self-limited or respond to photocoagulation. Recurrence is common, but the disease eventually becomes quiescent with favorable visual acuity unless the macula is damaged.     

小梁切开联合虹膜根切术治疗原发性闭角型青光眼

目的 观察外路小梁切开术联合虹膜周边切除治疗原发性闭角型青光眼的疗效.方法 对31例(31眼)原发性闭角型青光眼行外路小梁切开及虹膜周边切除术.其中急性闭角型青光眼22例,慢性闭角型青光眼9例.术后1周,1、3、6个月观察患者眼压和房角情况.结果 术后1个月和3个月,不用降眼压药眼压≤21 mm Hg(1 mm Hg=0.133 kPa)者分别为29例和27例,完全成功率分别为93.5%和87.1%.19例(19眼)随访6个月,不用降眼压药眼压≤21 mm Hg者16例(84.2%).术后房角检查显示上方120°范围房角开放和小梁组织切开的裂隙.术后并发症:前房出血31眼,均自行吸收,后弹力层损伤6眼、虹膜根部断离2眼、虹膜后粘连4眼.结论 外路小梁切开联合周边虹膜切除术能有效治疗原发性闭角型青光眼.     

Vitreous surgery for bilateral bullous retinal detachment in Vogt-Koyanagi-Harada syndrome

A successful surgical treatment (vitrectomy) for bilateral bullous retinal detachment in a patient with Vogt-Koyanagi-Harada (VKH) disease is reported. A 78-year-old woman had severe reduction of visual acuity in both eyes because of an extremely bullous nonrhegmatogenous retinal detachment accompanied by VKH disease. We performed lens extraction and vitrectomy on both eyes combined with systemic and topical corticosteroid therapy. The retina was reattached immediately after the surgery and her visual acuity promptly improved in both eyes. She had no recurrence of retinal detachment even after tapering the dose of corticosteroid. We suggest that vitrectomy may be an effective therapeutic option in the treatment for severe bullous retinal detachment associated with VKH disease.     

Bilateral Coats' disease with unusual presentation--a case report

Coats' disease is a well-known ocular disorder, characterized by idiopathic retinal telangiectasia, retinal exudation and retinal detachment. We report on a boy who first presented at the age of 2 years with Coats' disease and secondary neovascular glaucoma. The differential diagnosis with retinoblastoma could not be made clinically or with the help of imaging studies, such as ultrasonography or computed tomography scanning (CT-scan). The right eye was consequently enucleated for diagnostic and therapeutical purposes. Histopathologic examination confirmed the diagnosis of Coats' disease. Five years later the same boy consulted again with profound visual loss in his only functional left eye. Fundoscopy revealed lesions typical for Coats' disease. Coats' disease can present in very different ways and bilateral disease is possible, even after several years.     

多波长激光治疗伴有视网膜脱离的Ⅲ期青少年型Coats病初步观察

目的观察多波长激光治疗伴有轻中度视网膜脱离的Ⅲ期青少年型Coats病的疗效。设计回顾性病例系列。研究对象伴有轻中度视网膜脱离的青少年型Coats病(Shields等分期标准3A期)8例8眼,全部患者视网膜血管均异常扩张和存在散在的粟粒状动脉瘤样改变,周围可见无灌注区;其中交通支形成3眼;病变位于赤道部附近7眼。黄斑部均有黄色渗出,外斜视4眼,内斜视1眼。方法采取在视网膜脱离交界处使用包围式绿光光凝,Ⅲ级光斑,能量200~260 mw;视网膜脱离处使用黄光光凝,能量100~150 mw;血管异常处使用黄光光凝,能量220~240 mw。重复治疗间隔2个月,光凝3~8次。主要指标视力及视网膜脱离复位情况。结果视网膜脱离完全复位4/8眼(50%),脱离范围明显缩小3/8眼(38%),无明显变化1/8眼(13%);视网膜微血管瘤和无灌注区消失5/8眼(63%),大部分消退2/8眼(25%),无明显变化1/8眼(13%);黄斑部渗出明显吸收7/8眼(88%),无明显变化1/8眼(13%)。视力提高6/8眼(75%),无变化2/8眼(25%),无视力下降者。结论本文的小样本资料表明,多波长激光光凝治疗伴有轻中度视网膜脱离的青少年型Coats病有效,无明显视网膜前增殖,能最大限度地保存视力。     

Vitrektomie bei fortgeschrittenem Morbus Coats

BACKGROUND: Coats disease is a retinal vasculopathy of unknown cause. Untreated cases usually lead to an exudative retinal detachment and rubeosis iridis with secondary glaucoma. Photocoagulation and/or cryotherapy are generally the first interventions in treating the disease. Pars plana vitrectomy may be indicated in cases of vitreous hemorrhage or retinal detachment. METHODS: We performed pars plana vitrectomy in 9 eyes with Coats disease between 1992 and 1999. A retinal detachment was present in 3 cases, and three showed a vitreous hemorrhage. In two cases surgery was indicated because of paramacular localization of the pathological vessels with associated exudations. RESULTS: The two cases with paramacular involvement showed improvement in visual acuity of eight lines. In the remaining cases visual acuity remained within two lines compared to the initial visual acuity. All eyes except one could be saved. CONCLUSION: Pars plana vitrectomy is a useful option in treating advanced Coats disease, especially in cases associated with vitreous hemorrhage or retinal traction.     

Angle-closure glaucoma complicating ciliochoroidal detachment

Acute angle-closure glaucoma complicating ciliochoroidal detachment developed in eight eyes of six patients. The clinical presentation was uniform: extremely shallow central anterior chamber depth, flat peripheral anterior chamber, closed angle, and elevated intraocular pressure (IOP). There were three patients with uveal effusion syndrome, two with posterior scleritis, and one with an arteriovenous malformation. Cycloplegia, along with aqueous suppressants and corticosteroids, successfully resolved the acute glaucoma in all eyes. This rare, secondary glaucoma must be differentiated from primary angle-closure glaucoma, because the treatment is markedly different. Although primary angle-closure glaucoma is treated with miotics and peripheral iridectomy, such therapy may worsen the glaucoma in eyes with angle-closure glaucoma due to a ciliochoridal detachment.     

Posterior scleritis presenting as unilateral secondary angle-closure glaucoma

The following is a case report of a 19-year-old male who presented with unilateral acute angle-closure glaucoma. Posterior scleritis was diagnosed based on clinical features and ultrasound B-scan. The acute angle closure attack and exudative retinal detachment completely resolved with use of oral corticosteroids.     

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.     

Coat's disease treated with bevacizumab (Avastin)

CASE REPORT: We report a case of Coats' disease in a 10-year-old-girl who presented with a profound visual deficit, exudative retinal detachment, vascular telangiectasias and subretinal lipid, who underwent treatment with an intravitreal injection of bevacizumab (AVASTIN(TM)). Serial examinations documented an involutional response with a reduction of the subretinal fluid, exudates and macular thickness. DISCUSSION: The aetiology of Coats' disease remains uncertain, as does its optimal management. Although resolution of a case depends partially on age, and can even occur spontaneously on rare occasions, intravitreal injections of bevacizumab should be considered when planning treatment.