The indocyanine green findings in idiopathic uveal effusion syndrome

We report two cases clinically diagnosed as idiopathic uveal effusion syndrome, where indocyanine green angiogram suggest non-specific choroidal inflammation as the underlying cause. Treatment with non-steroidal anti-inflammatory drugs was beneficial.     

Clinical characteristics and surgical treatment of idiopathic uveal effusion syndrome

AIM: To investigate the clinical characteristics of idiopathic uveal effusion syndrome (IUES) and to identify effective surgical modalities for its treatment. METHODS: This retrospective analysis included clinical data of 33 eyes from 26 patients with IUES at Beijing Tongren Hospital. Records of eye examinations, ocular ultrasound, ocular ultrasound biomicroscopy (UBM), and follow-up surgical treatment were reviewed and analyzed. RESULTS: Of 26 patients, 17 (65.4%) were male and 9 (34.6%) were female. The average age of disease onset was 46.8y (range: 22-64y). Seven patients (26.9%) showed retinal detachment in both eyes at presentation. B-ultrasound showed the presence of retinal detachment in one eye or both eyes. All patients had binocular ciliary leakage and detachment. Eyes with retinal detachment underwent four-quadrantic partial-thickness sclerectomy and sclerostomy. Subretinal fluid resolution was achieved within 6mo. Recurrence was observed in three eyes and was resolved with re-operation. CONCLUSION: Ophthalmic ultrasound and UBM, among others, can be helpful in the diagnosis of IUES. Sclerectomy and sclerostomy are surgical modalities that can successfully treat the disease. Some patients may experience recurrence after surgery; reoperation remains safe and effective for them. Long-term follow-up is essential in such settings.     

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。     

葡萄膜渗漏综合征的研究近况

葡萄膜渗漏综合征(uveal effusion syndrome,UES)是由于涡静脉回流障碍和/或脉络膜血管通透性增加引起睫状体脉络膜脱离、浆液性视网膜脱离等一系列眼底改变为主的综合征。因以往对其认识不足,易漏诊误诊,且治疗棘手。我们对近年来的有关文献进行复习,并就UES的病因、发病机制、临床分型、诊断特点、治疗方法及预后进行综述。     

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.     

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.     

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.     

Medical therapy for uveal effusion syndrome

Purpose

To report a case series of three patients with bilateral uveal effusion syndrome (UES), treated conservatively with oral carbonic anhydrase inhibitors and topical prostaglandin analogues (PAs).

Methods

Three patients with bilateral UES were treated with the same initial therapy. Topical PA latanoprost 0.005% and acetazolamide 250 mg were administered in order to reduce intraocular pressure, improve uveoscleral outflow, and facilitate resolution of uveal effusion.

Results

The chorioretinal detachment resolved within 3 months in two reported patients while the third one underwent surgery on his left eye. After clinical improvement, further oral therapy with acetazolamide was stopped, while topical prostaglandins were continued for at least the next 3 months. All patients were free from recurrence during the follow-up period.

Conclusion

Although the usually recommended UES therapy is partial or full-thickness sclerectomy, our case series showed apparent resolution of chorioretinal detachment in two patients on medical therapy alone. Conservative therapy may be the first step before the standard recommended surgical approach, but further studies are needed to verify the effectiveness of reported therapy.     

葡萄膜渗漏综合征的诊断与治疗探讨

目的探讨葡萄膜渗漏综合征的诊断与鉴别诊断要点及手术效果。方法10例(19眼)诊断为葡萄膜渗漏综合征的患者行常规眼科检查及眼底荧光血管造影(FFA)、眼科超声波及超声生物显微镜(UBM)的检查。9例17眼行板层巩膜切除或联合巩膜全层切除术,平均随访18.4个月。结果睫状体脉络膜脱离见于全部病例。12眼有表层巩膜的血管扩张。FFA检查显示11眼有“豹斑”样改变。术后脉络膜和视网膜脱离完全复位14眼,视力稳定和提高13眼。结论眼科超声波、UBM、FFA等检查有助于本病的诊断及鉴别诊断,巩膜切除术治疗葡萄膜渗漏综合征效果良好,但需长期随访。     

Abnormal scleral findings in uveal effusion syndrome

We successfully treated a patient with uveal effusion syndrome and abnormal sclera with a partial-thickness sclerectomy. Part of the sclera was immediately cultured, and the excised sclera and the cultured cells were examined by electron microscopy. The sclera demonstrated increased glycosaminoglycan-like deposits between the scleral fibers. The cultured scleral cells showed large intracellular glycogen-like deposits, which were not seen in cells cultured from two control scleras. These findings may be the result of a metabolic defect, which causes a thick, impermeable sclera in some cases of uveal effusion.     

Efficacy of vitreous surgery for uveal effusion syndrome

PURPOSE: To evaluate the efficacy of pars plana vitrectomy for uveal effusion syndrome retrospectively. SUBJECTS AND METHODS: Six patients (six eyes) with uveal effusion syndrome(UE) underwent vitrectomy followed by internal drainage of subretinal fluid, fluid-gas exchange, and pan-retinal photocoagulation. Three of these cases with nanophthalmos underwent silicone oil injection. RESULTS: The retina became reattached in all cases. The silicone oil was not removed in two of the three cases with nanophthalmic eyes. Two cases of the three cases with nanophthalmic eyes needed to perform full thickness sclerostomy additionally, and in one case subchoroidal hemorrhage occurred. The visual acuity finally improved in five out of the six eyes. CONCLUSIONS: In cases of uveal effusion without nanophthalmic, vitrectomy hastens quick reattachment of the retina and may result in better visual outcome. But in cases of nanophthalmic eyes, it would be better to perform sclerostomy first.     

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.     

Nanophthalmic uveal effusion syndrome after prophylactic laser treatment

PURPOSE: We report a case of nanophthalmic uveal effusion syndrome (NUES) with total exudative retinal detachment (RD) after prophylactic argon laser (AL) treatment. The RD subsided and eventually resolved with i.v. steroid therapy. METHODS: A 45-year-old woman was referred to us with NUES and total exudative RD after prophylactic AL treatment for retinoschisis performed elsewhere. The patient had been scheduled for surgical intervention and was given i.v. prednisolone. RESULTS: Two days after starting i.v. prednisolone, the subretinal fluid partially resolved. Intervention had therefore been postponed. After 15 days the RD almost disappeared and vision improved to 20/400. Ten days later the macula was flat and vision was 20/200. At the six-month follow-up visit vision was 20/40. CONCLUSIONS: To our knowledge this is the first report of NUES and total exudative RD following AL treatment that resolved with i.v. steroid therapy alone. Since the AL treatment clearly seemed to play a role in the pathogenesis of the NUES and associated RD we strongly advise careful assessment of the risk/benefit ratio of prophylactic laser treatment in nanophthalmic patients. Although sclerectomy and vortex vein decompression are well-established techniques, we believe steroid therapy might be tried before proceeding to surgery.     

脉络膜渗漏综合征的眼底血管造影特征

目的 观察脉络膜渗漏综合征(UES)的眼底血管造影特征。 方法 回顾分析UES患者11例17只眼荧光素眼底血管造影（FFA）及吲哚青绿血管造影（ICGA）检查的临床资料。其中7只眼在作巩膜板层切除手术后1~28个月再行造影检查。 结果 FFA及ICGA检查显示12只眼有视网膜静脉纡曲扩张及视盘水肿，2只眼在后极部出现荧光素渗漏点，14只眼有豹纹样色素斑(豹斑)，8只眼有线条状或网状色素条纹。ICGA检查4例5只眼的斑片状强荧光一直持续至晚期，1只眼在眼底下方有三角形弱荧光区，1只眼在后极部显示地图状弱荧光区。巩膜板层切除手术后患眼睫状体脉络膜脱离逐渐好转甚至消失，脉络膜高灌注状态改善，渗漏点消失。1只眼手术2年后视网膜下液又增多。 结论视网膜静脉纡曲扩张及视盘水肿、豹斑和线条状或网状色素条纹是UES的特征性眼底血管造影表现；这些表现有助于加深对此病的理解和临床诊断。(中华眼底病杂志，2007，23：189-192)     

Nanophthalmic uveal effusion

A nanophthalmic patient with hypermetropia, shortened anterior-posterior axial length, and thickened choroid presented with a choroidal detachment and nonrhegmatogenous retinal detachment (NRRD). He underwent partial thickness, 5 x 7 mm, sclerectomies and 1-2 mm central sclerostomies 9.5 mm posterior to the limbus, specifically avoiding vortex veins. Complete resolution of the retinal and choroidal detachments occurred in spite of postoperative ultrasonograms demonstrating residual choroidal-scleral thickening. The effectiveness of this technique in our patient, in light of recent studies demonstrating histochemical and microscopic abnormalities in nanophthalmic sclera, suggests impairment of trans-scleral protein transport as the primary pathophysiologic mechanism in nanophthalmic uveal effusion.     

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.