A Comparative Study between Occlusive and Non-occlusive Retinal Vasculitis: Data from a Referral Center in Tunisia, North Africa.

PURPOSE: To compare clinical profile and visual outcomes of occlusive versus non-occlusive retinal vasculitis (RV). METHODS: A retrospective comparative study. RESULTS: 284 patients were enrolled, including 124 patients with occlusive RV (ORV) and 160 patients with non-occlusive RV (NORV). Patients with ORV were older (p ≤ 10-3), predominantly male (p ≤10-3), with less bilateral involvement (31.5% vs 53,4%; p ≤ 10-3). Infectious RV was more frequently diagnosed in the ORV group than in the NORV group (48.8% vs 32.9%, p = .006). Behçet disease and ocular tuberculosis were the leading causes of ORV. Idiopathic RV, Behçet disease, and sarcoidosis were the most common causes of NORV. Independent predictive factors of poor visual outcome were worse baseline visual acuity in both groups (p = .006 and p ≤ 10-3, respectively), and retinal hemorrhages (p = .048) and optic atrophy (p = .040) in the ORV group. CONCLUSION: Occlusive and non-occlusive RV have distinctive clinical and etiological profile.

Rickettsial disease: An underestimated cause of posterior uveitis.

Rickettsioses are worldwide distributed infectious disease caused by intracellular small Gram-negative bacteria transmitted to humans by the bite of contaminated arthropods, such as ticks. Systemic disease typically consists of a triad of high fever, headache, and skin rash. It usually has a self-limited course, but severe, life-threatening complications can sometimes occur. It may be clinically difficult to differentiate rickettsial diseases from other febrile illnesses. Rickettsial infection has been largely underestimated as a cause of infectious uveitis for long decades in the past. Conversely, recent data show that ocular involvement is much more common than previously thought, with retinitis, retinal vasculitis, and neuroretinitis being the most typical and frequent findings. Early clinical diagnosis of rickettsial disease, while awaiting laboratory test results, is essential for prompt initiation of appropriate antibiotic treatment to prevent systemic and ocular morbidity. The prevention remains the mainstay of rickettsial infection control.

Ocular Manifestations of Emerging Arthropod-Borne Infectious Diseases.

PURPOSE: To review the clinical features, diagnosis, treatment modalities, and prognosis of arthropod-borne infectious diseases. METHODS: This is a narrative review on arthropod-borne infectious diseases including general and ophthalmological aspects of these infectious diseases. A comprehensive literature review between January 1983 and September 2020 was conducted in PubMed database. Epidemiology, clinical features, diagnosis, treatment, and prognosis of arthropod-borne infectious diseases were reviewed. RESULTS: Emergent and resurgent arthropod-borne infectious diseases are major causes of systemic morbidity and death that are expanding worldwide. Among them, bacterial and viral agents including rickettsial disease, West Nile virus, Dengue fever, Chikungunya, Rift valley fever, and Zika virus have been associated with an array of ocular manifestations. These include anterior uveitis, retinitis, chorioretinitis, retinal vasculitis, and optic nerve involvement. Proper clinical diagnosis of any of these infectious diseases is primarily based on epidemiological data, history, systemic symptoms and signs, and the pattern of ocular involvement. The diagnosis is confirmed by laboratory tests. Ocular involvement usually has a self-limited course, but it can result in persistent visual impairment. Doxycycline is the treatment of choice for rickettsial disease. There is currently no proven specific treatment for arboviral diseases. Prevention remains the mainstay for arthropod vector and zoonotic disease control. CONCLUSIONS: Emerging arthropod vector-borne diseases should be considered in the differential diagnosis of uveitis, especially in patient living or with recent travel to endemic countries. Early clinical diagnosis, while laboratory testing is pending, is essential for proper management to prevent systemic and ocular morbidity.

Hypopyon: Is-it Infective or Noninfective?

Hypopyon usually corresponds to the sedimentation of white blood cells, and it signifies severe intraocular inflammation. This key clinical sign may occur in association with a wide variety of infectious, inflammatory, and neoplastic conditions that may be sight- and, occasionally, life-threatening. A careful history and thorough clinical examination are the cornerstones for orienting the differential diagnosis, identifying the causative agent, and initiating prompt and appropriate treatment. This review outlines the clinical characteristics and management of hypopyon in relation with the underlying causative infectious or noninfectious ocular or systemic diseases.

Multimodal imaging in IRVAN syndrome presenting with Branch Retinal Artery Occlusion.

PURPOSE: To describe multimodal imaging findings in a patient with idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome presenting with branch retinal artery occlusion (BRAO). CASE DESCRIPTION: A 33-year-old woman presented with acute BRAO in the right eye. A diagnosis of underlying IRVAN syndrome was made based on the presence of arteriolar aneurysms on the optic disc and along major arterioles and faint retinal hard exudates in both eyes. Eight months later, best-corrected visual acuity was 20/25 in the right eye and 20/20 in the left eye. The hard exudates had increased, and there were extensive areas of peripheral retinal capillary nonperfusion without new vessels. Optical coherence tomography (OCT) showed a localized retinal thinning corresponding to the prior BRAO. Fundus autofluorescence showed nodulo-linear periarterial hypoautofluorescence. OCT angiography (OCTA) showed localized ischemic changes, mainly involving the deep capillary plexus, corresponding to the area of resolved BRAO. It also clearly delineated the optic disc aneurysms. The patient received bilateral scatter laser photocoagulation directed to areas of peripheral capillary nonperfusion. Over a 6-month follow-up period, visual acuity remained unchanged, and there was no evidence of disease progression. CONCLUSION: Multimodal imaging, including fundus autofluorescence, OCT, and OCTA can provide additional valuable information in the evaluation of IRVAN syndrome complicated with BRAO.

Changing pattern of clinical manifestations of Behçet's disease in Tunisia: comparison between two decades.

OBJECTIVES: To investigate the changes over time in extraocular and ocular manifestations of Behçet's disease (BD) in Tunisian patients. MATERIAL AND METHODS: Retrospective study of 246 patients divided into two groups: group 1 (147 patients examined from 1995 to 2005) and group 2 (99 patients examined from 2006 to 2017). RESULTS: Active or scarred genital ulcers observed by physician at presentation were significantly less frequent in group 2 (47.2% vs. 29.6%; p = 0.007), as were articular involvement (50.3% vs. 34.7%; p = 0.016) and erythema nodosum (18.4% vs. 8.1%; p = 0.024). One hundred-seven patients (43.5%) developed ocular manifestations during the 23-year study period. Intermediate uveitis was significantly more frequent in group 2 than in group 1 (11.7% vs. 28.4%; p = 0.003), and posterior uveitis less frequent in group 2 than in group 1 (34.2% vs. 19.7%; p = 0.016). Patients from group 2 were more likely to have macular edema (19.8% vs. 45.6%; p = 0.001). However, better visual prognosis, with a lower rate of legal blindness, was noted in group 2. CONCLUSIONS: Changes over time included a decrease in the rate of articular involvement and cutaneous involvement. There was an increase in the rate of intermediate uveitis and a decrease in the rate of posterior uveitis over time. Despite an increase in the rate of macular edema, there was an improvement in visual prognosis, with less legal blindness over time.

Update on Bartonella neuroretinitis.

PURPOSE: To review the clinical features, diagnosis, treatment modalities, and prognosis of Bartonella-associated neuroretinitis. METHODS: This is a narrative review on Bartonella-associated neuroretinitis including general and ophthalmological aspects of the disease. A comprehensive literature review between January 1950 and September 2018 was conducted in PubMed database. Epidemiology, clinical features, diagnosis, treatment, and prognosis of Bartonella neuroretinitis were reviewed. RESULTS: Cat scratch disease (CSD) is a worldwide distributed systemic infectious disease caused by a bacterium, Bartonella henselae (B. henselae) which is usually transmitted to humans through contact with infected cats. Ocular manifestations of CSD are diverse, with neuroretinitis and superficial retinal infiltrates being the most common and typical manifestations. Neuroretinitis typically presents as optic disc edema with a partial or complete macular star in association with mild vitritis. Macular star may be absent at the initial presentation, becoming evident 1-2 weeks after the onset of optic disc edema. Diagnosis of CSD is confirmed by reliable laboratory tests. Neuroretinitis usually has a self-limited course. Antibiotic therapy is required for severe systemic disease and vision-threatening ocular involvement. The adjunctive use of oral corticosteroids may further improve the visual outcome. CONCLUSIONS: The diagnosis of Bartonella-associated neuroretinitis is based on typical clinical findings and positive serology. The prognosis is usually favorable in immunocompetent individuals.

Swept source-OCT and swept source-OCT angiography findings in posterior microphthalmos.

PURPOSE: To describe swept source-OCT (SS-OCT) and swept source-OCT angiography (SS-OCTA) findings in eyes with posterior microphthalmos (PM). METHODS: Twelve eyes (six patients) with PM were evaluated using SS-OCT and SS-OCTA. Structural changes, subfoveal choroidal thickness (SFCT), and perifoveal capillary changes with qualitative and quantitative assessments were analyzed. Twenty eyes served as control group. RESULTS: SS-OCT findings included elevated retinal papillo-macular fold (75%), retinal pigment epithelium folds (83%), macular cystoid spaces (42%), subretinal fluid (17%), and increased visibility of posterior vitreous cortex and hyaloid (42%). Mean SFCT in PM and in control eyes were 430.33 ± 157.48 µm and 290.05 ± 52.87 µm, respectively (p = 0.004). Perifoveal capillary changes on SS-OCTA included foveal avascular zone (FAZ) remodeling (100%), vessel tortuosity (67%), disorganization of the deep capillary network (67%), intraretinal cystoid spaces (42%), and areas of signal voids in the choriocapillaris (33%). FAZ area was significantly smaller in eyes with PM than in the control group in both the superficial (p < 0.001) and deep capillary plexuses (p = 0.001). Capillary vessel density (CVD) was significantly lower in the PM than in the control group in the deep capillary plexus (p = 0.004). Log MAR BCVA correlated negatively with axial length (r = - 0.929, p < 0.001), FAZ area in both the superficial (r = - 0.637, p < 0.001) and deep capillary plexus (r = - 0.561, p = 0002), and CVD in the deep capillary plexus (r = - 0.450, p = 0.016). CONCLUSIONS: Combined SS-OCT and SS-OCTA allow the detection of various retinal and choroidal structural and microvascular changes in eyes with PM. These findings can provide new insights onto this blinding ocular condition.

Atypical white dot syndrome with choriocapillaris ischemia in a patient with latent tuberculosis.

BACKGROUND: White dot syndromes (WDS) are a group of idiopathic multifocal inflammatory conditions that can be recognized and distinguished by lesion morphology, other specific clinical features, imaging findings, and disease course. Our purpose is to describe an atypical case of WDS with choriocapillaris ischemia shown by multimodal imaging including swept-source OCT angiography (OCTA) in a 30-year-old woman with latent tuberculosis. FINDINGS: At presentation, visual acuity in the left eye was 20/500. Clinical findings included macular granularity, diffuse outer retinal discoloration with satellite yellow-white dots, and disc margin blurring. Fluorescein angiography showed early confluent areas of choroidal hypofluorescence and late perifoveal punctate hyperfluorescence. There was choroidal hypofluorescence in a geographic configuration throughout the indocyanine green angiography sequence. OCTA showed confluent geographic areas of loss of signal in the choriocapillaris. Work-up revealed latent tuberculosis. The patient received corticosteroids and prophylactic anti-tubercular treatment. Nine months later, visual acuity had improved to 20/20, and there were some residual retinal pigment epithelium changes. CONCLUSION: Atypical WDS associated with choriocapillaris hypoperfusion may show features of multiple evanescent white dot syndrome and acute posterior multifocal placoid pigment epitheliopathy melting together.

Acute multifocal retinitis: a retrospective review of 35 cases.

BACKGROUND: Acute multifocal retinitis is a rare condition that has been considered to be often idiopathic. The purpose of this study was to analyze clinical features and causes of acute multifocal retinitis. RESULTS: This study is a retrospective review of the charts of 35 patients with acute multifocal retinitis. Patients with three or more retinal lesions in at least one eye, with at least one lesion of less than 500 µm in size were included. All patients had complete ophthalmological examination, fundus photography, and fluorescein angiography. Twelve patients (34.3%) had optical coherence tomography. An extensive work-up was performed including a detailed comprehensive medical history, examination by an internist and an infectious disease specialist, a chest X-ray, Mantoux test, and laboratory tests for syphilis, human immunodeficiency virus, Bartonella, and Rickettsia. Of the 35 patients, 25 (71.4%) had bilateral involvement and 10 (28.6%) had unilateral involvement (total number of eyes: 60). Mean best-corrected visual acuity (BCVA) was 20/25 (range, 20/1000-20/20). Retinal lesions ranged from 3 to more than 20 in number in at least 1 eye, and from 150 to 1500 µm in size. Associated findings included mild anterior chamber inflammation in 5 eyes (8.3%), mild vitritis in 46 eyes (76.7%), optic disc swelling in 9 eyes (15%), macular star in 4 eyes (6.7%), exudative retinal detachment in 6 eyes (10%), and branch retinal artery occlusion in 6 eyes (10%). Acute multifocal retinitis was found to be caused by Rickettsia conorii infection in 20 patients (57.1%), Rickettsia typhi infection in 4 patients (11.4%), cat-scratch disease in 8 patients (22.9%), and syphilis in 1 patient (2.9%). It was idiopathic in two patients (5.7%). Retinal lesions resolved without scarring in 3 to 12 weeks in all but three eyes (5%), in which residual retinal pigment epithelial changes were noted. CONCLUSION: Rickettsial disease was the most common cause of acute multifocal retinitis. Other identified causes included cat-scratch disease and syphilis, and a very small subset of patients was diagnosed with idiopathic multifocal retinitis.

Anterior chamber aqueous flare, pseudoexfoliation syndrome, and glaucoma.

The purpose of this study is to evaluate anterior chamber aqueous flare (ACAF) in Tunisian patients with pseudoexfoliation (PEX) syndrome with or without associated glaucoma. This is a prospective, cross-sectional, comparative study including 53 patients (88 eyes) with PEX syndrome, 48 patients with PEX glaucoma (86 eyes), and 53 healthy sex-and age-matched control subjects (106 eyes). All patients underwent a complete ophthalmic examination and laser flare photometry. Mean ACAF was significantly higher in the PEX syndrome group in comparison with the control group (17.96 ± 10.05 vs 7.06 ± 2.95 ph/ms; p = 10(-4)), in patients with PEX glaucoma compared to PEX syndrome without associated glaucoma (27.99 ± 15.45 vs 17.96 ± 10.05 ph/ms; p = 10(-4)), in the PEX glaucoma group in comparison with control group (27.99 ± 15.45 vs 7.06 ± 2.95 ph/ms; p = 10(-4)), and in patients with unilateral PEX syndrome in comparison with contralateral-unaffected eyes (25.72 ± 14.88 vs 8.58 ± 3.45 ph/ms; p = 0.000). For patients with PEX syndrome, a high ACAF might be a predictor for the development of glaucoma. Further investigations are needed to clarify the role of laser flare photometry in predicting the risk of glaucoma in patients with PEX syndrome.

Clinical and multimodal imaging characteristics of acute Vogt-Koyanagi-Harada disease unassociated with clinically evident exudative retinal detachment.

The purpose of this study was to describe the clinical and multimodal imaging findings in acute Vogt-Koyanagi-Harada (VKH) disease without clinically evident exudative retinal detachment (ERD). We retrospectively reviewed the charts of 18 patients (36 eyes), diagnosed with acute VKH disease without clinically evident ERD. All patients underwent complete ophthalmic examination, fundus photography, optical coherence tomography (OCT), B-scan ultrasonography, fluorescein angiography (FA), and indocyanine green angiography (ICGA). Of 18 patients, twelve (66.7 %) were female and 6 (33.3 %) were male. Mean age was 39 years (range, 23-60). Ten patients had been referred with an erroneous diagnosis of primary optic nerve disorder (8; 44.4 %) or isolated anterior uveitis (2; 11.1 %). Anterior chamber or vitreous inflammatory reaction was noted in 22 eyes (61.1 %), each. Fundus findings included optic disc swelling in 30 eyes (83.3 %), retinal striae in 20 eyes (55.5 %), and yellowish deep lesions in 3 eyes (8.3 %). OCT showed a shallow, localized subclinical ERD in 18 eyes (50 %), and retinal pigment epithelial folds in 23 eyes (63.9 %). B-scan ultrasonography showed diffuse, low- to medium-reflective choroidal thickening in all eyes. FA disclosed delayed choroidal perfusion in at least one eye of all patients (100 %), mild pinpoint leakage in 21 eyes (58.3 %), optic disc hyperfluorescence in 35 eyes (97.2 %) and choroidal folds in 13 eyes (36.1 %). ICGA findings included delayed choroidal perfusion in 24 eyes (66.7 %), decrease in the number of large choroidal vessels in 36 eyes (100 %), fuzzy choroidal vessels in 35 eyes (97.2 %), and hypofluorescent dark dots in 28 eyes (77.8 %). The association of bilateral optic disc edema with retinal striae and intraocular inflammatory reaction highly suggests acute VKH disease. A multimodal imaging approach including fundus photography, OCT, B-scan ultrasonography, FA, and ICGA provides important clues for the definite diagnosis and help differentiate VKH disease from primary optic nerve disorders.

The spectrum of presumed tubercular uveitis in Tunisia, North Africa.

The purpose of this study was to analyze the spectrum of presumed tubercular uveitis in Tunisia, North Africa. We retrospectively reviewed the clinical records of 38 patients (65 eyes) diagnosed with presumed tubercular uveitis at two referral centers in Tunisia, between January 2009 and December 2011. Mean age at presentation was 42.7 years. Twenty-four patients were women (63.2%) and 14 (36.8%) were men. Twenty-three eyes (35.4%) had posterior uveitis, 21 eyes (32.3%) had intermediate uveitis, 13 eyes (20%) had panuveitis, and 8 eyes (12.3%) had anterior uveitis. Ocular findings included vitritis in 67.7% of eyes, posterior synechiae in 47.7%, multifocal non-serpiginoid choroiditis in 23.1%, multifocal serpiginoid choroiditis in 21.5%, periphlebitis in 21.5%, and mutton-fat keratic precipitates in 20%. Anti-tubercular treatment was prescribed in 33 patients (86.8%) and was associated with systemic corticosteroids in 20 patients (52.6%) and periocular injections of corticosteroids in four patients (10.5%). After a mean follow-up of 14.2 months (range, 10-58), inflammation was controlled, with a significant improvement in visual acuity (VA) (p = 0.028). However, recurrences developed in two patients (5.3%). Final VA was better than 20/40 in 27 eyes (41.5%) and less than 20/200 in five eyes (7.7%). In Tunisia, all anatomic types are possible in tuberculosis-associated uveitis, but posterior and intermediate uveitis are more frequent. Vitritis, posterior synechiae, multifocal serpiginoid or non-serpiginoid choroiditis, and periphlebitis are the most common manifestations.

Optic nerve head drusen: a comparative study of 10 MHz and 20 MHz ultrasound probes.

The aim of this study was to evaluate and compare 10-MHz and 20-MHz ultrasonography in the assessment of patients with optic nerve head drusen (ONHD). The design of the study was prospective, comparative and cross-sectional. Ultrasonographic examination with a 10 and 20 MHz probe was performed in 45 eyes with suspected ONHD. The 20 MHz probe showed drusen in 43 eyes (95.5 %), while the 10 MHz probe revealed drusen in only 33 eyes (73.3 %, p = 0.0001). The 10 MHz probe showed surface drusen in 10 eyes (22.2 %), while the 20 MHz probe showed surface drusen in 14 eyes (31.1 %) (sensitivity 71.4 %; 95 % CI [47.6-95.1 %]). The 10 MHz probe showed buried drusen in 23 eyes (23.1 %), while the 20 MHz probe showed buried drusen in 29 eyes (64.4 %) (sensitivity 79.3 %; 95 % CI [56.6-86.2 %]). The sensitivity was 76.7 % with 10 MHz probe compared to a 20 MHz as gold standard. The use of 20 MHz probe increased the sensitivity of buried disc drusen by 1.5 times and surface disc drusen by nearly 2 times. Using the 10 MHz probe alone the false negative error rate was 83.3 %. The 20 MHz probe has shown itself to be an excellent method for the diagnosis of ONHD; it is more sensitive and reliable than 10 MHz probe and should be considered in the management of patients with clinical evidence of ONHD.

Ocular involvement and visual outcome of herpes zoster ophthalmicus: review of 45 patients from Tunisia, North Africa.

BACKGROUND: Ocular complications of herpes zoster ophthalmicus (HZO) may lead to substantial visual impairment. The purpose of this study was to characterize and analyze ocular involvement and visual outcome of HZO in patients from Tunisia, North Africa. This study is a retrospective chart review of 51 eyes of 45 patients with HZO. RESULTS: Mean age was 44.5 years. Thirty patients (66.7%) were aged over 50 years. Twenty-four patients (53.3%) were male and 21 patients were female (46.7%). There was no statistically significant difference in gender distribution. Initial mean best corrected visual acuity (BCVA) was 20/50. Ocular manifestations included adnexal involvement (58.8%), keratitis (31.4%), keratouveitis (31.4%), isolated anterior uveitis (AU) (29.4%), intraocular pressure elevation (23.5%), oculomotor nerve palsy (5.8%), and optic neuritis (1.9%). Isolated AU (p < 0.001), isolated keratitis (p = 0.001), and intraocular pressure elevation (p = 0.013) were more likely to be concomitant to HZO active skin disease, while keratouveitis occurred more likely more than 1 month after HZO eruption (p < 0.001). AU and keratouveitis were more likely to be associated with age ≥ 50 years (p = 0.001 and p = 0.02, respectively). Ocular complications included neurotrophic keratopathy (1.9%), corneal opacity (5.9%), secondary glaucoma (7.8%), optic atrophy (1.9%), and postherpetic neuralgia (13.3%). Mean follow-up was 12 months. Mean final BCVA was 20/32; it was ≥ 20/40 in 78.4% of the eyes. CONCLUSIONS: Our study provided epidemiologic and clinical data of HZO in a Tunisian population. AU and keratitis were the most common ocular complications. Neurotrophic keratopathy was scarce. The overall visual outcome is good, with about three quarters of the treated patients maintaining VA of 20/40 or better.

Herpesvirus detection and cytokine levels (IL-10, IL-6, and IFN-γ) in ocular fluid from Tunisian immunocompetent patients with uveitis.

Human herpesviruses (HHVs) are involved in the pathogenesis of different types of uveitis. Cytokine response plays an important role in virus-induced immunopathology. This study aimed to investigate the incidence of HHVs in aqueous humor samples of immunocompetent patients with suspected viral uveitis and cytokine (IL-10, IL-6, and IFN-γ) expression profiling. Forty-seven aqueous humor samples were collected from immunocompetent patients with viral uveitis. Samples were assayed for HHV-1 to HHV-8 by in-house real-time polymerase chain reactions. IL-6, IL-10, and IFN-γ were quantified with a cytometric bead array. Relations between viral detection, cytokine profiles, and clinical data were studied. At least one viral genome was detected in 21 aqueous humor samples analyzed. Varicella-zoster virus (VZV) was detected in 14 of the positive samples, cytomegalovirus (CMV) in 8, HSV-1 in 1, Epstein-Barr virus (EBV) in 4, and HHV-6 in 2. More than one viral genome was detected in seven aqueous humor samples. Aqueous humor samples positive for HHV-DNA contained significant levels of IL-6, IL-10, and IFN-γ, compared to HHV-DNA negative samples. High levels of IL-6 were detected in patients with CMV-DNA in their aqueous humor samples. Significantly higher levels of IL-10 and IFN-γ were found in positive samples for VZV, EBV, and HHV-6 DNA than in negative aqueous humor ones. VZV was the principal etiologic agent of uveitis in this Tunisian series, with CMV the second most common agent. Knowledge of immunoregulatory interactions and dynamic changes in viral uveitis may be a key to understand the pathogenesis leading to more-effective treatments.

Indocyanine green angiography findings in patients with nonfamilial amyloidosis.

PURPOSE: The purpose of this study is to assess indocyanine green angiographic findings in patients with nonfamilial amyloidosis. METHODS: The method used was a prospective study including seven patients (14 eyes) with nonfamilial amyloidosis. All patients underwent detailed ophthalmic clinical examination, fundus photography, and indocyanine green angiography (ICGA). Fluorescein angiography (FA) was performed in four patients. RESULTS: Of the seven patients, four (57.1 %) were male. Mean age was 49.5 years. Six patients had renal amyloidosis and one patient had systemic amyloidosis. Mean best-corrected visual acuity was 20/25. Fundus and FA findings included cotton-wool spots (28.5 %), retinal hemorrhages (14.3 %), retinal pigment epithelial changes (21.4 %), serous retinal detachment (7.1 %), optic disk edema or staining (7.1 %), area of peripheral retinal capillary non-perfusion (7.1 %), disseminated peripheral punctiform hyperfluorescence (21.4 %), and subretinal pooling (7.1 %). Fundus examination results were unremarkable in eight eyes (57.1 %). ICGA showed abnormal findings in all eyes. These included diffuse or focal/multifocal choroidal vascular staining appearing at the late phase and prevailing in peripheral fundus (100 %), hyperfluorescent fleecy lesions appearing at the late phase and also prevailing in peripheral fundus (28.5 %), hypofluoresent areas of variable sizes (85.7 %), and pinpoints (71.4 %). CONCLUSIONS: Our results show that a subclinical, fairly typical choroidal involvement, detectable only by ICGA, is common in patients with nonfamilial amyloidosis. ICGA may be useful in better understanding the pathogenesis of amyloidosis choroidopathy and in establishing a diagnosis of amyloidosis in atypical or incomplete clinical presentations.