葡萄膜渗漏综合征的临床特征和治疗效果

背景葡萄膜渗漏综合征临床上较少见,充分了解其临床特征有助于正确诊断和及时治疗,对挽救患者的视力有极其重要的意义。目的探讨葡萄膜渗漏综合征的诊断、分型及治疗要点。方法回顾性分析在北京协和医院眼科确诊的葡萄膜渗漏综合征患者10例14眼的临床资料,包括患者的眼科检查,眼部B型超声检查、眼部超声生物显微镜（UBM）检查、荧光素眼底血管造影（FFA）、吲哚青绿血管造影（ICGA）各项辅助检查,治疗经过及预后。结果本组患者直接检眼镜检查均可见随体位变化的球形视网膜脱离。眼部B型超声检查显示视网膜及脉络膜脱离。UBM检查均可见环形周边睫状体脉络膜脱离;FFA检查显示眼底呈“豹斑”样改变,但未发现荧光素渗漏。ICGA检查显示早期脉络膜即出现弥漫性颗粒状高荧光,荧光强度不断增强,直到造影晚期仍持续存在。本组患者1例1眼行巩膜瓣下巩膜切除术,2例4眼行巩膜全层切除术,术后视网膜脱离解剖复位,随访1年均未复发。结论术前眼部B型超声、MRI及CT检查有助于对葡萄膜渗漏综合征患者进行准确分型并制定合理的治疗方案。     

Uveal effusion syndrome complicated by anterior ischemic optic neuropathy

We report on a case of idiopathic uveal effusion syndrome complicated by AION. To our knowledge such an association hasn't been previously described. We suggest that scleral thickening caused obstruction of vortex veins followed by uveal effusion and compression of posterior ciliary arteries within their intrascleral tract, leading to AION. Nevertheless it can't be excluded that AION was the result of mechanical compression on ciliary vessels of optic disc by choroidal detachment.     

葡萄膜上腔积液与原发性闭角型青光眼关系探讨

目的 探讨葡萄膜上腔积液在原发性闭角型青光眼中的关系状况.方法 对60例120只眼,其中急性闭角型青光眼病人39例78只眼,慢性闭角型青光眼病人21例42只眼,采用超声生物显微镜检查并进行前房深度测量.结果 急性闭角型青光眼病人中急性发作眼有10例f25.6%),对侧眼4例(10%)出现葡萄膜上腔积液(x2=9.47,P＜0.05);慢性闭角型青光眼病人中6例(14.3%)出现葡萄膜上腔积液;其中伴葡萄膜上腔积液眼前房深度为(1.76±0.42)mm,不伴葡萄膜上腔积液眼为(2.12±0.24)mm,差异有显著性(P＜0.05).结论 葡萄膜上腔积液是原发性闭角型青光眼发病中的一个特殊的特征性改变;葡萄膜上腔积液多伴有浅前房的发生.     

Uveal Effusion Syndrome

The terms uveal effusion, choroidal effusion, ciliochoroidal effusion, ciliochoroidal detachment, and choroidal detachment have been used interchangeably in the literature. These labels all describe an abnormal collection of fluid that expands the suprachoroidal space, producing internal elevation of the choroidal. There are various inflammatory and hydrostatic conditions that can cause uveal effusion, but in some cases no obvious cause exists. In this setting, patients are thought to have a distinct, primary abnormality of the choroid or sclera, called uveal effusion syndrome (UES). UES may be idiopathic, or associated with hypermetropia, and should be considered a diagnosis of exclusion. Histological studies show amorphous glycosaminoglycan-like material filling the interfibrillary spaces of excised scleral tissue, with disruption of collagen fibers. In some patients there may be reduced macromolecular diffusion that interferes with the normal transscleral egress of albumin out of the eye, perhaps causing choroidal fluid retention due to altered osmotic forces. An alternative, and perhaps complementary hypothesis, is that swollen sclera compresses the transscleral vessels with resulting fluid retention. Patients with UES are most typically middle-aged men who have a relapsing remitting clinical course. There is often co-existing, shifting subretinal fluid that may involve the macula. Chronic disease may lead to secondary retinal pigment epithelial (leopard spot) changes and permanently reduced visual acuity. Treatment with systemic steroids does not appear to be effective. Surgical decompression of the vortex veins as they pass through the sclera has been described, but the most common treatment is full-thickness sclerectomies to provide an exit for choroidal fluid. The largest case series suggests that this produces an anatomic improvement in approximately 83% of treated eyes after a single procedure and in about 96% after one or two procedures. Final visual acuity improves by two or more lines in 56% of the eyes, is stable in 35%, and worsens in 9%. Although extremely rare, UES is a serious condition that is difficult to treat and can lead to severe and permanent visual loss in both eyes.     

Two cases of uveal effusion syndrome

PURPOSE: To report a case of uveal effusion syndrome associated with hypotony and a case of uveal effusion syndrome in nanophthalmos. METHODS: The first case was a 25-year-old man who presented with decreased visual acuity in the left eye and hypotony. Fundus examination revealed choroidal effusion and retinal detachment with a thickened eyeball. Partial thickness sclerotomy and sclerectomy were performed. The second case was a 13-year-old boy who had uveal effusion syndrome with a nanophthalmic eye. RESULTS: In the patient with hypotony, intraocular pressure was well maintained following partial thickness sclerotomy and sclerectomy, and choroidal effusion and retinal detachment were reduced. The visual acuity of the nanophthalmic patient was well maintained during a 3-year follow-up period without treatment. CONCLUSIONS: appropriate treatment modalities should be considered depending on the ophthalmic condition of the individual patient.     

原发性闭角型青光眼合并葡萄膜渗漏的研究进展

原发性闭角型青光眼小梁切除术后葡萄膜渗漏是临床常见并发症,对患者的预后产生一定影响,然而,闭角型青光眼在小梁切除术前也可以合并葡萄膜渗漏,其发生机制尚存在争议,目前依靠超声检查方法可以明确诊断。葡萄膜渗漏是否增加手术风险？怎样避免葡萄膜渗漏的发生？以及如何进行治疗？本文将围绕原发性闭角型青光眼合并葡萄膜渗漏的发生机制、诊断、治疗及预后进行一一阐述,为临床研究提供思路。     

Nanophthalmos with uveal effusion

A typical case of nanophthalmos with uveal effusion syndrome is reported. Clinical, pathological and therapeutic aspects of uveal effusion syndrome are discussed. A surgical treatment consisting of lamellar sclerectomies is proposed.     

Choroidal metastasis simulating uveal effusion syndrome: case report

We report a case of choroidal metastasis simulating uveal effusion syndrome. This patient with uveal metastasis from lung carcinoma presented with decreased vision in the left eye. Fundoscopic examination revealed pigmentary clumping, multiple choroidal nodules and serous retinal detachment. Fluorescein angioraphy revealed a leopard spots pattern similar to uveal effusion syndrome. Ultrasound revealed the presence of choroidal nodules with moderate internal reflectivity, choroidal thickening and serous retinal detachment. Optical coherence tomography showed retinal pigment epithelium tear and hyper-reflective nodules within the choroid and areas of serous retinal detachment. Image investigation demonstrated the presence of a solitary pulmonary nodule at the left apical lung. Choroidal metastasis can lead to a scenario that simulates uveal effusion syndrome. This possibility must be considered when having an atypical uveal effusion symptomatoloy and a systemic screening for systemic cancer should be performed.     

Uveal effusion and ultrasonic imaging: a clinical series

PURPOSE: To establish the diagnostic role of imaging by ultrasound for the diagnosis of uveal effusion syndrome. METHODS: Using ultrasonic imaging features as the main criteria for evaluation, the recorded data from 16 patients with presumed uveal effusion seen between 1994 and 2000 were reviewed and analysed for clinical manifestations and current diagnostic suggestions. Eventually, as cumulating experience suggested that ultrasound is capable of indicating uveal effusion, the study set-up became in part more prospective. RESULTS: The two main ultrasonic features found were: (1) extended multi-layered detachment patterns by B-scan, often kinetically mobile; (2) a spongy oedematous ciliary body region, often with interstitial lacunae, as demonstrated by high-resolution ultrasound biomicroscopy (50 Mhz). CONCLUSIONS: In patients with visual loss and ambiguous detachment-like fundus pathology, ultrasonic features are often of practical guidance for diagnosing uveal effusion syndrome.     

Measurement of scleral thickness in uveal effusion syndrome

PURPOSE: To measure scleral thickness in patients with and without uveal effusion syndrome using ultrasound biomicroscopy (UBM) and magnetic resonance imaging (MRI). DESIGN: Prospective case-control study. METHODS: UBM was used to measure scleral thickness in five subjects with uveal effusion syndrome and five matched controls. We also used MRI to measure scleral thickness in three subjects. RESULTS: The mean thicknesses for eyes with uveal effusion syndrome versus control eyes were 0.65 +/- 0.08 mm and 0.55 +/- 0.05 mm, respectively (mean difference 0.10, P value = .13). MRI measurements of three subjects showed abnormally thick sclera but were imprecise. CONCLUSIONS: UBM can be used to measure scleral thickness, and our results support the finding that patients with uveal effusion syndrome have abnormally thick sclera. Compared with MRI, UBM may be a more accurate and precise method of measuring scleral thickness. UBM can be a useful adjunctive test in the management of uveal effusion syndrome.     

Uveal effusion syndrome and scleral thickening

The uveal effusion syndrome associates bullous no rhegmatognous retinal detachment with shifting subretinal fluid, choroidal detachment and elevated spinal fluid protein. This syndrome could result of nonophthalmos, scleral thickening and/or vortex vein attenuation. The author describes a case of uveal effusion syndrome with isolated scleral thickening. Theoretical explanations of this syndrome are emphasized on the basis of literature and clinical and surgical guidelines are given.     

Full-thickness Sclerotomy for Uveal Effusion Syndrome

To report the surgical outcome of full-thickness sclerotomy in five cases of uveal effusion syndrome (UES). Full-thickness sclerotomy without sclerectomy was performed on five eyes of four patients with UES with or without nanophthalmos. In four of the eyes, exudative retinal detachment associated with UES resolved after the sclerotomy. The subretinal fluid in one eye, which had a normal axial length, was relieved after undergoing three sclerotomy procedures. Full-thickness sclerotomy without vortex vein decompression or sclerectomy is an effective surgical option for the management of significant UES.     

Scleral window surgery and topical mitomycin C for nanophthalmic uveal effusion complicated by renal failure: case report

Purpose To describe the case of a 16-year-old patient with nanophthalmic uveal effusion associated with renal failure that was treated with scleral window surgery and topical administration of mitomycin C (MMC). Methods Case report. Results Total uveal effusion was restored in the nanophthalmic eyes. Partial-thickness scleral flap with deep sclerostomy was performed and topical MMC was administered to one quadrant of the equatorial sclera. The subretinal fluid resorbed gradually. Conclusions Topical MMC might relieve the blocked transscleral outflow of intraocular fluid in the small area of a sclerostomy in young patients with nanophthalmos or in patients with renal failure in whom uveal effusion occurs repeatedly. The authors have no proprietary interest in any aspect of this report.     

Uveal effusion syndrome--case report

The history and clinical findings of 60-year old man with effusion syndrome are presented. The uveal effusion syndrome typically affects healthy middle age men and causes recurrent, spontaneous, serous retinal and cilio-choroidal detachments which, often results in significant visual impairment. In addition, to the general clinical examinations, indirect ophthalnoscopy, fundus fluorescein angiography, ultrasonography and MRI are used to make definitive diagnosis. Annular cilio-choroidal detachment, shifting non-rheumatogenous retinal detachment, unremarkable inflammation in the anterior segment and normal intraocular pressure are the key features of the idiopathic uveal effusion syndrome. The fundus change is characterized by the "leopard-spot".     

Choroidal detachments: what do optometrists need to know?

Choroidal detachments occur when there is an accumulation of fluid or blood in the suprachoroidal space, a potential space situated between the choroid and the sclera. They are an uncommon ocular pathology. The most common cause of choroidal detachment is secondary to trabeculectomy; however, there are other causes such as trauma and inflammation. Clinically, choroidal detachments may vary in presentation from asymptomatic, to very poor vision, severe ocular pain, vomiting and nausea. Ocular findings associated with choroidal detachments include serous retinal detachment, secondary angle closure, and a very shallow anterior chamber. Optometrists, as primary eye care providers, need to be aware of the clinical signs and symptoms associated with choroidal detachments and ensure that appropriate and timely management, with a referral to an ophthalmologist, is instigated for optimal visual outcomes. In this review, the pathophysiology, detection, and associated risk factors for choroidal detachments are discussed, and evidence‐based management recommendations in an optometric context are provided. The characteristics and management of uveal effusion syndrome are also reviewed, as this can cause idiopathic exudative choroidal detachments distinct from classical choroidal detachment.     

Sclerotomy in uveal effusion syndrome

PURPOSE: To report visual and anatomic outcomes after modified scleral surgery in patients with uveal effusion syndrome with retinal and choroidal detachment. METHODS: In five eyes of four patients with retinal and choroidal detachment and uveal effusion syndrome due to nanophthalmos, we performed pars plana full-thickness unsutured sclerotomies without sclerectomy. RESULTS: In all cases, uveal effusion with choroidal and retinal detachment resolved within 3 weeks, and all patients showed improved vision. The functional and anatomic results remained stable for over 2 years. CONCLUSIONS: Uveal effusion syndrome that is refractory to medical treatment (high-dose systemic corticosteroids) can be managed effectively by pars plana full-thickness unsutured sclerotomy without sclerectomy.     

Spontaneous primary uveal effusion syndrome

CLINICAL CASE: This was a 73 year-old male patient who developed an apparent uveal effusion syndrome in his right eye one year after cataract surgery. Once possible associated conditions were discarded, a diagnosis of spontaneous uveal effusion syndrome was reached. With appropriate systemic steroid therapy, a favourable response occurred. DISCUSSION: The diagnosis of uveal effusion may sometimes be difficult to establish. In order to diagnose and manage these patients, a detailed clinical examination along with fluorescein angiography, ultrasonography, ultrasound biomicroscopy (UBM) and magnetic resonance imaging (MRI) tests, must be carried out.     

Leopardenmuster in der Fluoreszenzangiographie

The uveal effusion syndrome is a rare disease characterized by serous choroidal detachment. The pathogenesis of idiopathic uveal effusion syndrome has not yet been conclusively established. One hypothesis is an abnormality of diffusion of extravascular proteins in the choroid leading to decompensation of the pigment epithelium pumping capacity. Fluid then accumulates in the subretinal space leading to retinal detachment which results in loss of visual acuity. It typically affects males and hypermetropia is another risk factor. When looking at the fundus a circular serous detachment of the choroid and choroidal puckering is typical. The fluorescein angiography shows hyperfluorescence in the form of a leopard-spot pattern. Space-occupying lesions have to be excluded with the help of ultrasound or magnetic resonance tomography. The uveal effusion syndrome is a diagnosis by exclusion. Treatment varies because of the different hypotheses for the pathogenesis. An intraocular tamponade in combination with laser coagulation may for example be an effective treatment.     

Idiopathic Serous Detachment of the Choroid,Ciliary Body,and Retina (Uveal Effusion Syndrome)

Nine patients with bilateral idiopathic serous detachment of the peripheral choroid, ciliary body, and retina (uveal effusion syndrome)are presented. The following features are emphasized: (1) dilationof episcleral vessels, (2) vitreous cells, (3) characteristic leopardspot pigment epithelial changes, and (4) ultrasonic and angiographic findings. One patient presented with recurrent serous macular detachment and was misdiagnosed as idiopathic central serous choroidopathy. Although the pathogenesis of this syndrome is uncertain, the available clinical and histopathologic findings suggest that obstruction of the venous outflow of the uveal tract occurring predominantly in nanophthalmic or normal size eyes with abnormally thick sclera and vortex vein anomalies may be the primary cause of the uveal and retinal detachment.     

Uveal effusion in Hunter's syndrome. Evidence that abnormal sclera is responsible for the uveal effusion syndrome

A patient with Hunter's Syndrome (systemic mucopolysaccharidosis type II) was evaluated for bilateral uveal effusion syndrome. The right eye showed a circular peripheral choroidal detachment for 360 degrees. The left eye showed a larger circular peripheral choroidal detachment and an exudative retinal detachment. Evaluation of the sclera during combined sclerectomies and sclerostomies of the left eye revealed markedly thickening sclera and a reduced number of vortex veins. The surgery resulted in complete resolution of the choroidal detachment and exudative retinal detachment of the left eye. The presence of uveal effusion in Hunter's Syndrome, in which the sclera has been histologically demonstrated to be abnormally thickened, supports the recently proposed pathophysiology of the uveal effusion syndrome.