Sturge-Weber Syndrome Associated with Early Diffuse Choroidal Hemangiaoma： A Case Report

Purpose:To report indocyanine green angiography (ICGA) findings in one patient of diffuse choroidal hemangioma associated with Struge-Weber syndrome. Methods: Color fundus photography, fluorescein angiography (FA) and ICGA were performed in a patient with diffuse choroidal hemangioma associated with Sturge-Weber syndrome, Results: Three findings were unveiled by ICGA : rapid filling of diffuse choroidal hemangio‘s vascular network in the early stages; diffuse hyperfluorescence visual up to the late phase; no “wash-out“ phenomenon was observed in the late phase. Conclusion:Indocyanine green angiography can provide information that is not detected by clinical or fluorescence angiographic examination in the patient with Sturge-Weber syndrome. ICGA may be important and sensitive in detecting the diffuse choroidal hamangioma associated with Sturge-Weber syndrome. Eye Science 2004:20:168-170     

孤立性脉络膜血管瘤的荧光素钠吲哚青绿同步眼底血管造影

目的探讨孤立性脉络膜血管瘤荧光素钠及吲哚青绿同步眼底血管造影的影像学特征及其临床意义,为诊断及治疗监测提供依据。方法应用海德堡HRA2共焦激光扫描眼底血管造影系统对9例9眼孤立性脉络膜血管瘤进行荧光素眼底血管造影(Fundus fluorescein angiography,FFA)和吲哚青绿血管造影(Indocyanine green angiography,ICGA)同步检查,分析比较2种检查影像学特征。结果FFA检查主要影像学表现为动脉前期或动脉早期瘤体部位血管丛状或斑状高荧光;静脉期高荧光灶渗漏融合;晚期瘤体呈弥漫性强荧光。其中3例瘤体表面夹杂不规则的点片状低荧光;6例瘤体表面及周围视网膜毛细血管扩张;5例可见与瘤体周围RPE带状萎缩相应的透见荧光。ICG早期可见瘤体由不规则血管网状高荧光组成;中期瘤体呈强荧光团;晚期所有病例均有特征性的"冲洗现象"。ICGA比FFA更清晰显示瘤体范围。结论孤立性脉络膜血管瘤ICGA影像比FFA影像更具特征性,能够更清晰地显示肿瘤边界;FFA则能显示视网膜血管及RPE的继发性损伤。FFA和ICGA同步检查对孤立性脉络膜血管瘤临床诊断、治疗及疗效监测有重要的指导意义。     

Visual rehabilitation in a child with diffuse choroidal hemangioma by using aggressive amblyopia therapy with low-dose external beam irradiation.

Diffuse choroidal hemangioma is a congenital vascular hamartoma often associated with hemangiomatous lesions of the brain, orbit, and periocular skin (nevus flammeus) in the Sturge-Weber syndrome. Visual loss from diffuse choroidal hemangioma may result from chronic serous retinal detachments causing retinal pigment epithelial, photoreceptor or cystoid degeneration, and glaucomatous optic atrophy. Low-dose external beam irradiation has successfully resolved exudative retinal detachment and caused shrinkage of the choroidal hemangioma.(1-3) Visual loss in Sturge-Weber syndrome with diffuse choroidal hemangioma often begins during amblyogenic years. Visual rehabilitation may thus require not only therapeutic intervention to address organic disease but also amblyopia therapy. Though many of the previously reported cases address treatment of the diffuse choroidal hemangioma with laser or radiotherapy, none advocate or emphasize treatment of nonorganic amblyopia associated with this condition. We report the case of a child with Sturge-Weber syndrome and unilateral diffuse submacular choroidal hemangioma who developed an exudative retinal detachment that responded to the combination of low-dose external beam irradiation and aggressive amblyopia therapy.     

Indocyanine green angiography in the juvenile haemorrhagic choroidopathy

Juvenile haemorrhagic choroidopathy (JHC) is an idiopathic syndrome marked by macular choroidal neovascularization (CNV) in patients under the age of 50. We used fluorescein angiography (FA) and indocyanine green angiography (ICGA) to examine 17 patients with macular CNV and JHC. CNV was always unilateral. On ICGA examination the CNV were weakly fluorescent in 59% of cases, hyperfluorescent in the remaining 41%.ICGA showed up the following alterations: a) areas with diffuse choroidal hyperfluorescence at the posterior pole or in the peripapillary region in 11 affected eyes (65%), in the fellow eye too in 5 patients; b) areas of choroidal hypofluorescence at the posterior pole but also outside the vascular arcades in 2 affected eyes (12%).In conclusion, ICGA does not appear indispensable for detecting CNV in JHC but this method does show up diffuse choroidal alterations not detectable with FA. The pathogenetic implications of the ICGA findings are discussed.     

Optical coherence tomography (OCT) in idiopathic polypoidal choroidal vasculopathy (IPCV)

Purpose: Idiopathic polypoidal choroidal vasculopathy (IPCV) is a distinct clinical entity characterized by an edematous maculopathy and typical choroidal vascular change. The purpose of this study is to evaluate the use of optical coherence tomography (OCT) for diagnosing of this disease. Methods: 4 patients affected by IPVC (mean age 71–84 years) underwent biomicroscopy with a three mirror conctact lens, fluorescein angiography (FA), indocyanine green angiography (ICGA) and OCT (Humphrey Zeiss) scan. Results: in all 4 cases OCT tomograms acquired at the location of the typical choroidal abnormalities demonstrated by ICGA, revealed a characteristic hyper-reflectivity in the choroidal layers. Conclusion: ICGA is essential to diagnose IPVC, but OCT may be able to identify characteristic reflectivity patterns. This revised version was published online in July 2006 with corrections to the Cover Date.     

Resolution of Persistent Exudative Retinal Detachment in a Case of Sturge-Weber Syndrome with Anti-VEGF Administration

Purpose: Report the resolution of a persistent exudative retinal detachment in a patient with Sturge-Weber syndrome following intravitreal pegaptanib injection. Design: Case report. Methods: A 13-year-old male with Sturge-Weber syndrome presented with a choroidal hemangioma associated with an exudative retinal detachment that failed to resolve 6 months after external beam radiation therapy. Results: A single intravitreal pegaptanib injection resulted in 50% resolution of the detachment within 1 week and complete resolution within a month. Despite anatomic success, vision remained poor. Conclusions: VEGF likely plays a role in the pathogenesis of Sturge-Weber choroidal hemangioma-associated exudative retinal detachment and offers potential treatment.     

系统性红斑性狼疮脉络膜视网膜炎吲哚菁绿血管造影表现:随访一例

目的:用眼底荧光血管造影(fundus fluorescein angiography,FFA)和吲哚菁绿血管造影(indo-cyaninegreenangiography,ICGA)随访一例系统性红斑性狼疮(systemic lupuseryth ematosus,SLE)脉络膜视网膜炎患者。方法:回顾性研究随访1例SLE脉络膜视网膜炎患者临床和眼底造影影像学特征。结果:本病例ICGA特征包括造影早期脉络膜血管模糊弥漫性高荧光、局灶性低荧光,造影晚期点状脉络膜高荧光。经泼尼松治疗后,造影晚期点状脉络膜点状高荧光随着视网膜小血管阻塞出现而增加,当视网膜出现新生血管时点状高荧光消失。结论:ICGA可以揭示SLE脉络膜病变临床和FFA不能发现的信息。免疫复合物沉积在脉络膜毛细血管可能是SLE脉络膜炎发病机制的重要因素。     

Retinal fluorescein and indocyanine green angiography and optical coherence tomography in successive stages of Vogt-Koyanagi-Harada disease

Objective To report features of retinal fluorescein (FA) and indocyanine green (ICGA) angiography and optical coherence tomography (OCT) at successive stages of Vogt-Koyanagi-Harada (VKH) disease. Methods FA and ICGA were systematically performed in cases of VKH disease, at admission, 1 month later, and at the end of the follow-up. In addition, the most recent patients underwent OCT. In the follow-up, the clinical evolution and extent of treatment were related to the angiographic and tomographic features. Results FA and ICGA showed a diffuse delayed choroidal perfusion, including a delayed arterial, choriocapillar, and venous filling. This delayed choroidal perfusion involved the posterior pole and the hole periphery. The classically described serous retinal detachments were slowly filled from numerous pin points that could correspond to leakage through areas of damaged retinal pigment epithelium due to the choroidal ischemia. An uneven background choroidal fluorescence visible at the mid phase of ICGA was the result of multiple hypofluorescent round lesions in the choroidal stroma. Choroidal folds appeared hypofluorescent in FA and hyperfluorescent in ICGA. Some of these angiographic findings were present at the very early neurologic stage of the disease. Moreover, under treatment, a persisting altered choroidal perfusion could increase the incidence of retinal detachment relapses. Discussion Various pathological conditions are characterized by choroidal involvement and serous retinal detachment. Angiographical findings may be helpful in the diagnosis. Conclusion FA coupled with ICGA can be an useful tool in the early diagnosis of VKH disease as well as during the tapering of immunosuppressive drugs.     

Low Dose Irradiation for Diffuse Choroidal Hemangioma

Sturge-Weber Syndrome is a nonheritable congenital syndrome characterized by a “port-wine stain” on the face and angioma of the meninges. Ocular findings include diffuse choroidal hemangioma, retinal detachment, and various types of glaucoma. Management of diffuse choroidal hemangioma is aimed at preserving the affected eye and preventing glaucoma. In the past this has been challenging. Herein, we describe a case of Sturge-Weber Syndrome with diffuse choroidal hemangioma which was successfully treated with low dose lens-sparing external beam radiotherapy.     

Indocyanine green angiographic findings of obscure choroidal abnormalities in neurofibromatosis

We report two cases of choroidal neurofibromatosis, detected with the aid of indocyanine green angiography (ICGA) in patients with neurofibromatosis (NF)-1, otherwise having obscure findings based on ophthalmoscopy and fluoresceine angiography (FA). In case 1, the ophthalmoscopic exam showed diffuse bright or yellowish patched areas with irregular and blunt borders at the posterior pole. The FA showed multiple hyperfluorescent areas at the posterior pole in the early phase, which then showed more hyperfluorescence without leakage or extent in the late phase. The ICGA showed diffuse hypofluorescent areas in both the early and late phases, and the deep choroidal vessels were also visible. In case 2, the fundus showed no abnormal findings, and the FA showed weakly hypofluorescent areas with indefinite borders in both eyes. With the ICGA, these areas were more hypofluorescent and had clear borders. Choroidal involvement in NF-1 seems to occur more than expected. In selected cases, ICGA is a useful tool to be utilized when an ocular examination is conducted in a patient that has no definite findings based on the ophthalmoscope, B-scan, or FA tests.     

Sturge-Weber syndrome: medical management of choroidal hemangiomas

PURPOSE: To investigate the outcome of irradiation of complicated choroidal hemangiomas in Sturge-Weber syndrome. PATIENTS AND METHODS: The charts of 6 patients (7 eyes) with Sturge-Weber syndrome and choroidal hemangiomas were reviewed. An exudative retinal detachment was the indication for treatment in all cases. The mean age of the 6 patients was 13 years (range, 4 to 20 years). The minimum follow-up time was 1 year. Patients were checked for initial and final best-corrected visual acuity, fundus examination, fluorescein angiography, and tumor thickness on B-scan ultrasonography. The patients were treated with radiotherapy. A total dose of 20 Grays was applied to 7 eyes: 2 with a circumscribed choroidal hemangioma underwent proton therapy and 5 with diffuse hemangioma were treated by external beam irradiation. RESULTS: Complete resolution of the subretinal fluid was achieved in all cases with the tumor height decreased. Visual acuity improved to 1 line or more in 5 eyes and remained stable in 2 eyes. Two cases that underwent proton therapy developed radiation retinopathy. CONCLUSION: External beam radiation is an effective and safe option in the management of choroidal hemangiomas complicated by retinal detachment. Based on our experience, proton therapy should be reserved for sporadic circumscribed choroidal hemangioma.     

Indocyanine Green Angiographic and Multifocal Electroretinographic Features in the Diffuse and Regional Form of Bietti‘s Crystalline Retinopathy

Purpose:To observe different features of indocyanine green angiography(ICGA) and multifocal electroretinography(ERG)in the diffuse and regional type of Bieti‘s crystalline retinopathy(BCR).MEthods:ICGA and the multifocal ERG were performed in two cases of the diffuse and regional type of BCR respectively.These data were compared with fluorescein angiography(FA),Standard Ganzfeld ERG,and visual field testing.Results:In the regional case,ICGA revealed reduced perfusion of the choroidal circulation in the early phase and multiple hypofluorescent spots in the posterior pole in the late phase,due to choriocapillaris filling defect;the extent of choroiocapillaris loss was shown in early phase of ICGA and there were multifocal hyperfluorescent dots surrounding hypofluorescent spots in late phase in the diffuse case.The multifocal ERG showed that the central responses were markedly depressed,corresponding to the visual field defects,while the findings of Ganzfeld ERG were normal in the regional BCR;However,both the multifocal ERG and Ganzfeld ERG were severely subnormal in the diffuse case.Conclusions:The features of ICGA and multifocal ERG are different between the diffuse and regional BCR.In the meantime,the two tools are also useful to differentiate the type and assess the extent of evolution in BCR.     

Clinicopathological correlation in exudative age-related macular degeneration: recurrent choroidal neovascularization

Purpose: To report the pathology of surgically removed submacular tissue in recurrent choroidal neovascularization after laser photocoagulation of classic choroidal neovascularization in age-related macular degeneration. Methods: A recurrent subfoveal choroidal neovascular membrane was surgically removed in two patients. The recurrence was identified as a classic membrane on fluorescein angiography at the foveal border of the laser scar. A net was visualized in the early venous phase of the indocyanine green angiogram, with associated late hyperfluorescence. Both patients had undergone laser photocoagulation for a classic interpapillomacular choroidal neovascular membrane about 1 1/2 years earlier. The specimens were serially sectioned and stained with hematoxylin–eosin, periodic acid–Schiff, Masson trichrome and phosphotungstic acid–hematoxylin. Results: The two specimens consisted of subretinal fibrovascular tissue with fibrin exudation. Fibrovascular tissue bordered subretinal fibrous tissue adherent to Bruch’s membrane and remnants of the choroid in one patient. The fibrovascular portion most likely corresponded to the recurrence, whereas the fibrous portion represented the original membrane, being obliterated after photocoagulation. Some peripapillary tissue was additionally removed in the other patient. The latter lesion was invisible on fluorescein angiography but stained in the late phase of indocyanine green angiography and corresponded histopathologically to poorly vascularized intra-Bruch’s fibrovascular tissue. Granular deposits, periodic acid–Schiff positive and metachromatically purple on Masson trichrome stain, representing diffuse drusen (basal laminar/linear deposits), were identified in the three specimens. Conclusion: A subretinal fibrovascular membrane corresponded with the classic recurrent choroidal neovascularization. Received: 14 June 2000 Revised: 4 September 2000 Accepted: 13 September 2000     

Polypoidal choroidal vasculopathy in Caucasians

Purpose: To study the prevalence of polypoidal choroidal vasculopathy (PCV) in Caucasian patients with occult choroidal neovascularization (CNV); to study the clinical spectrum of PCV in Caucasians and the outcome after laser photocoagulation of such lesions. Methods: (1) A consecutive series of 374 eyes of Caucasian patients at least 58 years old, presenting occult CNV, presumed to have age-related macular degeneration (AMD) on fluorescein angiography (FA) were further characterized by indocyanine green angiography (ICGA) to determine the frequency of PCV. (2) The funduscopic, FA and ICGA findings in a cohort of 36 Caucasian patients with PCV were analyzed. (3) The outcome after laser photocoagulation was studied in 14 PCV eyes with a minimum follow-up of 6 months. Results: (1) Fourteen of 374 eyes (4%) presenting occult CNV in patients at least 58 years old were diagnosed as PCV by means of ICG-A. (2) A polypoidal lesion was found in the macula in 22 of 45 PCV eyes, in the peripapillary area in 16 of 45, under the temporal vascular arcade in 6 of 45 and in the midperiphery in 6 of 45. Large or soft drusen were observed in 15 of 45 eyes with PCV. (3) Regression of fundus signs without persisting polyps 6 months after laser photocoagulation was obtained in 5 of 5 treated peripapillary lesions but in only 5 of 9 treated macular or arcade lesions. Conclusion: Polypoidal choroidal vasculopathy is not rare in Caucasian patients presenting with occult choroidal neovascularization. The fundus abnormalities seen in such eyes overlap with the typical manifestations of AMD. Whereas the prognosis after photocoagulation of peripapillary polypoidal lesions appears to be relatively good, it is more guarded for macular or arcade lesions. Received: 10 January 2000 Revised: 30 March 2000 Accepted: 16 May 2000     

Retinal abnormalities associated with a mutation of the nucleotide 683 in von Hippel-Lindau disease

Background: von Hippel-Lindau disease (VHL) is a hereditary cancer syndrome in which affected individuals are at risk of developing tumors in multiple organs, including eyes, cerebellum, spinal cord, kidneys, inner ear, adrenal glands and pancreas.  Methods: We performed a fundus examination and fluorescein and indocyanine green (ICG) angiography in both eyes of a young woman affected by VHL with bilateral pheochromocytoma, retinal angioma, retinal microaneurysms and unusual alterations of the deep retinal layers. A molecular analysis of the VHL gene was carried out. Results: Ophthalmoscopy disclosed in her right eye a small retinal hemangioma, some microaneurysms in both eyes at the posterior pole and multiple, small, whitish, dome-shaped lesions scattered in the retinal pigment epithelium (RPE) of the posterior retina. Fluorescein angiograms revealed in the early phase multiple hyperfluorescent spots that showed progressive discoloration in the late phase of angiography. Some of these spots were ophthalmoscopically undetectable. The late phase of ICG angiography showed some small hyperfluorescent points located at the level of the RPE, and some of them corresponded to the hyperfluorescent spots seen on fluorescein angiography. The molecular analysis revealed the presence of a „missense” mutation of the VHL gene at nucleotide 683.  Conclusions: Alterations in the RPE have never been observed in the VHL syndrome. We describe an unusual case of VHL with a capillary hemangioma associated to diffuse alterations with the RPE of the posterior retina. The possibility exists that these lesions form part of the eye modifications in VHL. Received: 18 September 1999 Revised: 6 December 1999 Accepted: 7 December 1999     

Ocular Angiographic Findings in Patients with Neurofibromatosis Type 1

Purpose: To describe the ocular angiographic findings in patients with Neurofibromatosis type 1 (NF-1). Methods: We examined 20 consecutive patients diagnosed with NF1 by conventional ophthalmoscopy and fluorescein angiography (FA), indocyanine green angiograms (ICGA) were also obtained from 11 of the patients. Forty FAs and 25 ICGAs were also obtained from age-matched controls. Results: Fluorescein angiography revealed patchy hyperfluorescence in 30%, retinal pigment epithelial (RPE) atrophy in 30%, “corkscrew” vessels in 50%, optic disc abnormalities in 25% and choroidal nevi in 30% of the patients. All the patients investigated by ICGA (n = 11) demonstrated hypofluorescent lesions corresponding to the patchy hyperfluorescence on fluorescein angiography. None of the control patients had any of the findings described neither on FA nor on ICGA. Conclusion: Because of their high frequency (100%), the hypofluorescent lesions on ICGA should be added as a new diagnostic criterion in NF1. RPE atrophy and the high frequency of optic disc abnormalities appear to be novel findings in patients with NF-1.     

Assessment and classification of choroidal vasculitis in posterior uveitis using indocyanine green angiography

BACKGROUND: By allowing one to detect fluorescence beyond the retinal pigment epithelium, indocyanine green angiography (ICGA) has made it possible to analyse the choroidal vessels. Our aim was to characterize choroidal vasculitis in posterior uveitis using ICGA. METHODS: Charts of active posterior uveitis patients with a specific diagnosis seen in the different centers participating in the study who had undergone dual fluorescein and ICG angiography were reviewed. The type of inflammatory involvement of the choroidal circulation at entry and the treatment response on follow-up angiograms were analysed. RESULTS: A total of 129 patients were analysed. Choroidal vasculitis could be subdivided into two main patterns: (1) primary inflammatory choriocapillaropathy and (2) stromal inflammatory vasculopathy. The first pattern consisted of hypofluorescent areas up to the late phase of angiography characteristic for choriocapillaris non-perfusion and included entities such as multiple evanescent white dot syndrome (MEWDS), acute posterior multifocal placoid pigment epitheliopathy (APMPPE), multifocal choroiditis (MC), ampiginous choroidopathy and serpiginous choroidopathy. The second pattern consisted of fuzzy indistinct appearance of vessels in the intermediate angiographic phase and diffuse choroidal hyperfluorescence in the late phase indicating inflammatory vasculopathy of larger choroidal vessels. This pattern was found in all cases of active Vogt-Koyanagi-Harada disease, ocular sarcoidosis and tuberculosis and birdshot chorioretinopathy. In Beh?et's uveitis of recent onset, choriocapillaris perfusion delay and fuzzy choroidal vessels without diffuse late choroidal hyperfluorescence was found. In posterior scleritis, enlargement of vorticous veins was an additionnal ICGA sign. Stromal inflammatory vasculopathy always responded to anti-inflammatory therapy. A third group of patients with severe retinal or choroidal inflammation presented with associated secondary inflammatory choriocapillaropathy angiographically identical to the primary involvement. CONCLUSIONS: ICGA allowed the hitherto impossible characterization of inflammatory involvement of the choroidal vessels, showing either predominant inflammation of the choriocapillaris or predominant inflammation of the stromal choroidal vessels with or without secondary choriocapillaritis. ICGA will be indispensable for the correct evaluation and follow-up of posterior inflammation with suspected choroidal involvement.     

Bilateral diffuse choroidal hemangiomas with unilateral facial nevus flammeus in Sturge-Weber syndrome

PURPOSE: To report bilateral choroidal hemangiomas associated with unilateral facial nevus flammeus in Sturge-Weber syndrome. METHODS: Case report. RESULTS: A 6-year-old male with a left facial nevus flammeus and a history of a left cerebral angioma had been followed 2 years for increasing esotropia. Examination demonstrated bilateral diffuse choroidal hemangiomas with overlying exudative retinal detachments. After bilateral external beam radiotherapy, the retinal detachments resolved and vision improved. CONCLUSION: Patients with Sturge-Weber syndrome and unilateral facial nevus flammeus may harbor bilateral choroidal hemangiomas. Clinical manifestations of the Sturge-Weber syndrome are characteristically unilateral and ipsilateral to the facial nevus flammeus. Bilateral choroidal hemangiomas associated with bilateral facial nevus flammeus are rare. 1,2 We report bilateral diffuse choroidal hemangiomas associated with unilateral facial nevus flammeus in Sturge-Weber syndrome.     

Indocyanine green angiographic findings in serpiginous choroidopathy.

AIMS--Analysis of the choroidal findings in patients affected by serpiginous choroidopathy (SC). METHODS--Thirteen patients (23 eyes; 11 males and two females; age range 50-68 years; mean age 59.1 years) affected by SC were examined with fluorescein angiography (FA) and indocyanine green angiography (ICGA). The follow up period was 7-33 months. RESULTS--Using ICGA the disease could be divided into the following stages: (1) subclinical or choroidal stage (hypofluorescent lesions without FA evidence); (2) active stage (with ICGA and FA evidence); (3) subhealing stage (slight late hyperfluorescent lesions with ICGA, with no evidence on FA); (4) inactive or healed stage (hypofluorescent areas with ICGA and hyperfluorescent areas with FA). CONCLUSIONS--Although FA showed a clear distinction between active and healed stages, ICGA allowed a greater subdivision of the disease. In particular, ICGA allowed: (1) better staging of SC, revealing choroidal alterations when there was no ophthalmoscopic or FA evidence; (2) better identification of the active lesions which appear to be larger at the choroidal level in comparison with the corresponding retinal lesions; and (3) revealed a persistence of choroidal activity even when the signs of retinal activity had disappeared. Thus, ICGA should be a particularly useful clinical and therapeutic monitoring tool of SC.     

Scoring of dual fluorescein and ICG inflammatory angiographic signs for the grading of posterior segment inflammation (dual fluorescein and ICG angiographic scoring system for uveitis)

Purpose To propose a semiquantitative dual fluorescein angiography (FA) and indocyanine green angiography (ICGA) scoring system for uveitis that would assist in the follow-up of disease progression and monitoring response to treatment. Methods The scoring system was based on the FA scoring systems, the standardized ICGA protocol, and schematic interpretation of ICGA findings in posterior uveitis that have been previously published. We assigned scores to the fluorescein and ICG angiographic signs that represent ongoing inflammatory process in the posterior segment. We rated each angiographic sign according to the impact it has on our appreciation of active intraocular inflammation. In order to permit direct comparison between FA and ICGA, we multiplied the total ICGA score by a coefficient of 2 to adjust to the total score of FA. Results A total maximum score of 40 was assigned to the FA signs, including optic disc hyperfluorescence, macular edema, retinal vascular staining and/or leakage, capillary leakage, retinal capillary nonperfusion, neovascularization of the optic disc, neovascularization elsewhere, pinpoint leaks, and retinal staining and/or subretinal pooling. A total maximum score of 20 was assigned to the ICGA signs, including early stromal vessel hyperfluorescence, choroidal vasculitis, dark dots or areas (excluding atrophy), and optic disc hyperfluorescence. Conclusion The combined fluorescein and ICG angiographic scoring system proposed herein may help estimate the magnitude of retinal versus choroidal inflammation, monitor disease progression and response to treatment, and provide comparable data for clinical studies. The applicability of the proposed system needs to be tested in clinical settings, and intra- and interobserver variations need to be determined.