Imaging characteristics of idiopathic scleroma: a retrospective case series and review of the literature.

BACKGROUND: Idiopathic scleroma (previously coined solitary idiopathic choroiditis or focal scleral nodule) is an innocuous lesion affecting the sclera with intraocular manifestations. It is often the basis of many misdiagnoses such as amelanotic choroidal melanoma, osteoma or metastatic lesions. Patients are often asymptomatic and the course is benign. With increasing use of community based imaging, more of such cases are being identified. This paper is a retrospective case series investigating the multi-modal imaging findings of idiopathic scleroma. METHODS: A retrospective analysis of prospectively collected data were analysed. Over the course of January 2008-January 2022, 44 patients diagnosed with idiopathic scleroma and imaged with wide-field colour fundus photography, fundus autofluorescence, ocular coherence tomography (OCT) and B-scan ultrasonography. Due to a poor image, only 43 images were included for OCT review. We also reviewed our patient's demographics, symptoms and baseline ophthalmic characteristics upon presentation. RESULTS: The mean age was 52 years (range 32-79) and there was no predilection towards gender. All lesions were post equatorial with the most common location being inferotemporal (n = 16, 36%); 32 lesions (73%) were yellow on fundus photography. 82% (n = 36/44) of lesions exhibited hyperautoflourescence and 43 lesions (98%) showed hyperechogenicity on B-scan ultrasonography. 100% of lesions originated from the sclera with no lesions showing active inflammation. 20 (47%) lesions had associated blood vessels overlying them on OCT. DISCUSSION: Idiopathic scleroma is a yellow, hyperautofluorescent, hyperechogeneic scleral lesion that has no signs of active inflammation. These characteristics help define them from other more sinister cause of amelanotic fundal lesions.

The Use of Optical Coherence Tomography Angiography in the Diagnosis of Inflammatory Type 1 Choroidal Neovascularization Secondary to Tuberculosis: A Case Report.

Purpose: To report a case of choroidal neovascularization (CNV), which was not obvious with dye angiography but was detected with optical coherence tomography angiography (OCTA).Methods: A 50-year-old female presented with decreased vision in her right eye. Funduscopic examination revealed a swollen hyperemic disc, peripapillary exudation, and choroidal infiltration.Results: Optical coherence tomography revealed intraretinal cysts, subretinal fluid in the macular region, and subretinal hyperreflective material in the papillomacular area. Fluorescein angiography revealed early hypofluorescence in the areas of choroidal infiltrations, and indocyanine green angiography (ICGA) also showed hypocyanescence corresponding to these infiltrations and revealed a faint hypocyanescence in the papillomacular region, further corresponding to the subretinal hyperreflective material on OCT scan. Two weeks after the initiation of systemic therapy with the diagnosis of tuberculosis, OCTA scans detected Type 1 CNV in the peripapillary area.Conclusion: OCTA may be superior to ICGA in the detection of CNV secondary to inflammatory situations.

LONGITUDINAL FOLLOW-UP OF TUBERCULAR SERPIGINOUS-LIKE CHOROIDITIS USING OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY.

PURPOSE: To analyze and describe serial follow-up of choriocapillaris alterations in tubercular serpiginouslike choroiditis (SLC) using optical coherence tomography angiography (OCTA) and to compare it with multimodal imaging. METHODS: In this prospective cohort study, patients with active tubercular SLC underwent OCTA using Optovue RTVue XR Avanti and other imaging techniques including enhanced-depth imaging OCT (EDI-OCT) (Heidelberg Spectralis; Heidelberg Engineering, Heidelberg, Germany), fundus autofluorescence, fluorescein angiography, and indocyanine green angiography. Serial imaging was performed for a total follow-up of 3 months. Choriocapillaris alterations at the site of lesions were analyzed on OCTA imaging, and their mean lesion areas were calculated. RESULTS: Twenty-six eyes (26 patients; 20 males; mean age: 32.68 ± 10.56 years) were included. Fourteen eyes had active multifocal lesions (n = 39), whereas 12 eyes had active placoid lesions (n = 12). At baseline, OCTA showed hyporeflective flow deficit lesions corresponding to the hypofluorescent lesions on indocyanine green angiography in all eyes. In the multifocal type of SLC, the mean lesion area decreased in all eyes compared with baseline, and lesions with a lesion area less than 0.1 mm2 on OCTA showed near-complete resolution with minimal choriocapillaris atrophy. In comparison, all eyes with a placoid type of SLC showed no significant reduction in the lesion area and showed extensive choriocapillaris atrophy. CONCLUSION: Optical coherence tomography angiography has the unique ability to demonstrate pathological flow impairment at the level of choriocapillaris in active tubercular SLC. Serial OCTA analysis reveals that large tubercular SLC lesions result in choriocapillaris atrophy as the lesions heal, whereas smaller multifocal lesions show resolution of choriocapillaris hypoperfusion with minimal atrophy.

[Fundus imaging features of purified protein derivative and T-spot positive tubercular serpiginous-like choroiditis].

Objective: To summarize the clinical and fundus imaging features of purified protein derivative and T-spot positive tubercular serpiginous-like choroiditis (PTP-SLC) patients. Methods: This retrospective study consecutively enrolled 13 PTP-SLC patients (21 eyes) in Beijing Tongren Hospital from November 2015 to November 2017. There were 8 males and 5 females with an average age of (45.2±12.1) years. Medical history and results of systemic and ophthalmological examinations, such us fundus autofluorescence photography, optical coherence tomography (OCT), fluorescein fundus angiography (FFA) and indocyanine green angiography, were evaluated. Results: Eight patients had binocular disease with an average interval time of (8.4±7.9) years. The average visual acuity of all patients was 0.3, and 4 patients had a clear history of exposure to tuberculosis. The active lesions in the PTP-SLC patients were homogeneous and creamish-yellow with unclear boundaries. Fundus autofluorescence showed an ill-defined, diffuse hyperautofluorescent zone. OCT showed punctate hyperreflexes between the choroidal stroma, destruction of the outer retinal structure with intraretinal edema and discrete vitreal hyper-reflective spots. FFA showed hypofluorescence in the active lesion at early stage and diffuse hyperfluorescence with leakage. Indocyanine green angiography showed persistent hypofluorescence. Conclusions: PTP-SLC fundus lesions are mainly manifested as homogeneous creamish-yellow lesions with unclear boundaries and high in autofluorescence. The involvement of the choroid and the outer layer of the retina can be observed on OCT. FFA can find more retinal vascular inflammatory changes. It is difficult to distinguish PTP-SLC from serpiginous choroiditis simply based on clinical and epidemiological characteristics. The pathogenic examination of tuberculosis is still the key to differential diagnosis (Chin J Ophthalmol, 2020, 56: 914-919).

Imaging in Tubercular Choroiditis: Current Concepts.

PURPOSE: Ocular tuberculosis has protean clinical manifestations. Because of its varied clinical presentation, multimodal imaging is very important to characterize the disease activity, presence of inflammation, determining therapeutic response, and detection of complications. METHODS: Narrative review. RESULTS: In this review, various imaging modalities employed in the management of ocular tuberculosis including fluorescein angiography (FA), indocyanine green angiography (ICGA), optical coherence tomography (OCT), and optical coherence tomography angiography (OCTA) have been reviewed. Not only do these imaging tools complement each other in providing a comprehensive assessment of the pathology, they also help in gaining valuable insights regarding the evolution of the disease. CONCLUSIONS: Fundus imaging plays a vital role in the diagnosis and management of patients with posterior uveitis due to tuberculosis. Fundus imaging may have a useful role in defining clinical endpoints for ocular tuberculosis in the future.

'Solitary idiopathic choroiditis' or a tumour of scleral origin: A case report based hypothesis.

We here hypothesize the scleral origin of 'Solitary Idiopathic Choroiditis', a yellowish, nodular lesion that resembles other choroidal tumors. Systemic investigations were done to rule out causes like tuberculosis and sarcoidosis. Multimodal imaging was done for better characterization and to distinguish it from other choroidal lesions. Fundus imaging revealed a yellowish-orange, well-defined nodular lesion resembling solitary idiopathic choroiditis inferotemporal to the disc. Swept source optical coherence tomography (SSOCT) showed a nodular lesion arising from the sclera with compression of overlying choroid. Indocyanine green angiography (ICGA) showed hypofluorescence in all phases of angiogram with displacement of major choroidal vessels along the walls of the mass and loss of overlying choriocapillaries. The same was evident on Swept Source optical coherence tomography angiography (SSOCTA) as well. Fundus autofluorescence (FAF) showed central hypofluoresence with surrounding hyperfluorescence. Ultrasound (USG) B scan revealed a high reflective echo at the apex of the mass with moderate internal reflectivity without any evidence of calcification. Enhanced Infrared (IR) Reflectance imaging revealed a central intense hyerreflective area suggestive of a fibrotic mass. Magnetic reasonance imaging (MRI) revealed a focal hypointense nodular lesion on T2-weighted image. The central white area at the apex with corresponding hypoautofluorescence, a high spike on ultrasound, intense hyperreflectivity on enhanced Infrared reflectance, lack of vascularity and displacement of choroidal vessels is suggestive of a scleral origin of the mass and likely fibrotic nature.

Imageology features of different types of multifocal choroiditis.

BACKGROUND: Multifocal choroiditis (MFC) is multi-inflammatory lesions that occur in the retinal pigment epithelium (RPE) and the choriocapillaris. Optical examinations are the major diagnostic methods to diagnose the disease. OBJECTIVE: To examine patients with different types of MFC by multiple imageological methods. To summarize the imageology features of different types of MFC to make a medical examination guideline for clinician practices. METHOD: All of the patients who included in the study received examinations of fundus color photography, infrared fundus photography, fundus auto fluorescence (FAF), fluorescein fundus angiography (FFA), and optical coherence tomography (OCT), respectively. Finally, imageology features of different types of multifocal choroiditis were summarized. RESULTS: A total of 51 eyes from 28 patients with diagnosed MFC were included in the study. These patients consisted of 10 males and 18 females aged from 31 to 49 (mean age: 41.5 ± 0.8). 23 patients had MFC on both eye whilst 5 had monocular disease. The MFC lesions were classified as active inflammatory lesions, inactive inflammatory lesions, inflammatory lesions secondary active choroidal neovascularization (CNV) and inflammatory lesions secondary inactive CNV according to literature reports and comprehensive fundus imaging examinations. CONCLUSION: Examinations via fundus color photography, infrared fundus photography, FAF, FFA and OCT indicate typical imageological signals of different types of MFC. These imageology tests can greatly assist the clinicians to identify the MFC and provide proper therapies.

Optical coherence tomography angiography in unilateral multifocal choroiditis and panuveitis: A case report.

RATIONALE: Optical coherence tomography angiography (OCT-A) has the advantage to visualize the microvascular structure of the retina in vivo and was utilized clinically in various neovascular retinal diseases. The OCT-A has also been used to examine the lesion in multifocal choroiditis and panuveitis (MCP). This study aimed to describe a case of MCP and present the disease process of a punched-out lesion in the chorioretina with neovascular activity using OCT-A. PATIENTS CONCERNS: A 32-year-old female Caucasian patient presented with a 2-week history of progressive blurred vision in her right eye with photophobia and a diminished temporal visual field. On presentation, her best corrected visual acuity was 6/60 in the right eye with a prominent anterior uveitis seen under slit lamp examination. DIAGNOSES: Dilated fundus examination of the right eye showed vitritis and multiple, punched-out yellowish-white lesions over the peripheral retina. Additional multimodal imaging (MMI) were done including fluorescein angiography (FA), indocyanine green angiography (ICGA) and fundus autofluorescence (FAF), which all revealed characteristic findings of MCP. In general, the diagnosis of unilateral MCP was made. Furthermore, one of the punched-out lesions in the right eye was particularly selected and examined under OCT and OCT-A, which revealed a subretinal elevated lesion with high flow signal under OCT-A. INTERVENTIONS: Treatment with oral prednisolone at 30 mg daily with topical prednisolone acetate 1% every 2 hours were prescribed, which were gradually tapered down within a 2-month course. OUTCOMES: The patient's best corrected visual acuity of the right eye returned to 6/6 at 2 months after the diagnosis. The flow signal in the OCT-A study of the punched-out lesion had also resolved after steroid treatment. LESSONS: The MCP is an uncommon uveitis with multiple inflammatory chorioretinal lesions. Using multimodal imaging technique, physicians can better differentiate these lesions for diagnosis and for further monitoring. Our results demonstrated that these chorioretinal lesions in MCP may display neovascular activities that might not be seen easily using conventional FA or ICGA study. With OCT-A, ophthalmologists could identify and monitor subtle choroidal neovascularization (CNV) changes over these punched-out lesions.

Macular serpiginous choroiditis - case report.

Serpiginous choroiditis represents an inflammation, often asymmetric, situated at the level of the inner choroid, which is related to the retinal pigment epithelium and the choriocapillaris. It is known as a primarily idiopathic, but there are authors who consider it an autoimmune process. Many times, fundus autofluorescence is the investigation which is guiding the diagnosis, the management, also the follow-up together with the determination of the progressive visual prognosis. Local treatment is proven to be sometimes an adjunctive treatment often effective. Abbreviations: SC = Serpiginous choroiditis; RPE = Retinal pigment epithelium.

Optical Coherence Tomography Features of Tuberculous Serpiginous-like Choroiditis and Serpiginous Choroiditis.

OBJECTIVE: To investigate optical coherence tomography (OCT) characteristics of tuberculous serpiginous-like choroiditis (Tb-SLC) and serpiginous choroiditis (SC) and to perform OCT to differentiate between these conditions. METHODS: This retrospective, case-control study examined consecutively enrolled patients with active Tb-SLC or SC. Patients underwent comprehensive ocular examinations and imaging (OCT, color fundus photography, autofluorescence imaging, fluorescein angiography, and indocyanine green angiography). Findings were examined and compared between eyes with SC and Tb-SLC. RESULTS: Nine patients with active Tb-SLC (14 eyes) and 8 with active SC (12 eyes) were included. The following OCT findings were observed significantly more often in the Tb-SLC group than in the SC group: vitreal hyper-reflective spots [5 Tb-SLC eyes (36%), no SC eyes; P = 0.02], intraretinal edema [11 Tb-SLC eyes (79%), 3 SC eyes (25%); P = 0.01], sub-retinal pigment epithelium (RPE) drusenoid deposits [11 Tb-SLC eyes (79%), 2 SC eyes (17%); P < 0.01], and choroidal granulomas [8 Tb-SLC eyes (57%), 2 SC eyes (17%); P = 0.03]. A hyporeflective, wedge-shaped band was observed more often in the SC group [5 Tb-SLC eyes (36%), 9 SC eyes (75%); P = 0.045] than in the Tb-SLC group. The incidence of other OCT signs did not differ between the groups and included outer nuclear layer hyper-reflection, outer retinal tabulation, and choriocapillaris point-like hyper-reflection. CONCLUSION: Vitreal hyper-reflective spots, intraretinal fluid, sub-RPE drusenoid deposits, and choroidal granulomas on OCT images may indicate Tb-SLC. Additionally, a hyporeflective, wedge-shaped band may indicate SC. Therefore, OCT is likely helpful in differentiating between Tb-SLC and SC.

Need for Quantitative Measurement Methods for Posterior Uveitis: Comparison of Dual FA/ICGA Angiography, EDI-OCT Choroidal Thickness and SUN Vitreous Haze Evaluation in Stromal Choroiditis.

BACKGROUND/PURPOSE: Quantitative methods for posterior uveitis are necessary for precise appraisal and follow-up of inflammation in practice and in clinical trials. The aim of this study was to assess fluorescein angiography (FA), indocanine green angiography (ICGA), and enhanced depth imaging optical coherence tomography choroidal thickness (EDI-OCT CT) in two stromal choroiditis entities, birdshot retinochoroiditis (BRC), and Vogt-Koyanagi-Harada disease (VKH), as well as to determine (1) disease patterns, (2) respective response to therapy, and (3) their potential utility in clinical trials in comparison to vitreous haze, the present standard outcome used in clinical trials. METHODS: This retrospective study included newly diagnosed patients with BRC and VKH, seen at the Centre for Ophthalmic Specialized Care, Lausanne, Switzerland. Angiographic signs were quantified using an established dual FA/ICGA scoring system for uveitis at presentation and on follow-up. FA/ICGA score ratios were compared between diseases to determine disease patterns. EDI-OCT CT was determined using a spectral domain instrument. Vitreous haze was determined using the SUN (Standardization of Uveitis Nomenclature) method. RESULTS: Among 1872 uveitis patients seen from 1995 to 2016, 8 newly diagnosed BRC patients (16 eyes) and 6 newly diagnosed VKH patients (12 eyes) had sufficient data for study inclusion. Patients with BRC and VKH at initial onset had mean FA scores of 16.1 ± 7.0 vs. 4.6 ± 2.1 (p < 0.0001), respectively, while mean ICGA scores were similarly high in the two diseases, 18.9 ± 3.6 (BRC) vs. 20.8 ± 7.5 (VKH). After therapy, FA and ICGA scores decreased significantly for both entities (- 60% of FA score and 55% of ICGA score in BRC vs. - 72% of FA score and - 87% for ICGA score in VKH). EDI-OCT CT decreased significantly in the two entities. Vitreous haze was almost absent in VKH and low in BRC. CONCLUSION: Dual FA/ICGA scoring showed the diverse disease patterns of BRC and VKH; both the retina and choroid were involved at onset in BRC, whereas VKH was a pure choroidal disease with later spillover into the retina. Dual FA/ICGA allowed for the precise measurement of inflammation at onset and upon follow-up. EDI-OCT CT responded to therapy in both diseases but was found to be of limited use in this early/subacute disease phase because it lacked sensitivity to detect subclinical recurrences and was therefore only useful for long-term follow-up. Vitreous haze was low in both entities and thus useless as an inflammatory parameter.

Bilateral choroiditis as the only sign of persistent Mycobacterium intracellulare infection following haematogenous spread in an immunocompromised patient.

An immunocompromised patient had positive blood cultures for Mycobacterium intracellulare and no identifiable organ seeding was started on treatment. One month later, the patient was clinically well with negative blood cultures but drug-induced myelotoxicity had developed. Ocular fundus examination at this time revealed bilateral choroidal granulomas which changed patient management.

IDIOPATHIC MULTIFOCAL CHOROIDITIS PRESENTING WITH A TRANSIENT PERIPAPILLARY WHITE RING.

PURPOSE: We describe with multimodal imaging the presentation and follow-up for a patient with idiopathic multifocal choroiditis and a transient peripapillary white ring. METHODS: Case report. RESULTS: A 39-year-old Asian woman was initially seen for an evaluation of lattice degeneration in 2015. Her medical history included Graves disease and psoriasis. Best-corrected visual acuity was 20/25 in her right eye and 20/25 in her left eye. Ultra-widefield fundus autofluorescence imaging showed a curvilinear hyperautofluorescent line in her right eye. One year later, the patient returned complaining of floaters in her right eye for 1 month. Her visual acuity was unchanged. Funduscopic examination showed new inflammatory yellowish lesions in the right eye corresponding to hyperreflective sub-retinal pigment epithelium lesions on structural spectral domain optical coherence tomography. Fluorescein angiography showed corresponding late staining of these active lesions. Late-phase indocyanine green angiography showed multiple nummular hypocyanescent dots. Ultra-widefield fundus autofluorescence showed large areas of hyperautofluorescence. The patient was started on a 60-mg oral prednisone taper and demonstrated subsequent regression of the inflammatory lesions. Ten months later, the patient returned emergently with complaints of floaters in both eyes for 2 days and a new temporal scotoma in her left eye. Funduscopic examination demonstrated a white ring around the optic nerve of the left eye corresponding to a hyperautofluorescent lesion. Ultra-widefield fundus autofluorescence showed new areas of hyperautofluorescence in both eyes. Structural spectral domain optical coherence tomography showed new sub-retinal pigment epithelium inflammatory lesions and a disruption of the ellipsoid zone in both eyes. The patient was again treated with a 60-mg oral prednisone taper and demonstrated subsequent restoration of the ellipsoid zone. CONCLUSION: To our knowledge, this is the first report of a transient annular white ring occurring in a case of multifocal choroiditis. There was marked restoration of the disrupted ellipsoid zone after treatment with oral corticosteroids.

SERPIGINOUS CHOROIDITIS IMAGED BY OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY.

PURPOSE: To report a case of serpiginous choroiditis imaged with optical coherence tomography angiography (OCT-A). METHODS: Case report. RESULTS: Multimodal imaging and OCT-A were performed to investigate serpiginous choroiditis in a 65-year-old patient. Comparison between OCT-A images and intermediate and late phase indocyanine green angiography revealed that the hypofluorescent areas on indocyanine green angiography corresponded topographically to the dark areas seen in the choriocapillaris layer of OCT-A. CONCLUSION: The OCT-A was shown to be able to detect vascular nonperfusion at the level of the choriocapillaris and could be helpful, especially for follow-up purposes, in clinical practice for choriocapillaritis such as serpiginous choroiditis.

Optical Coherence Tomography Angiography Findings in Punctate Inner Choroidopathy.

BACKGROUND AND OBJECTIVE: Punctate inner choroidopathy (PIC) is an inflammatory chorioretinopathy characterized by multiple punctate, hypopigmented fundus lesions. Optical coherence tomography angiography (OCTA) is an emerging imaging modality that enables high-resolution visualization of the retinal and choroidal vasculature. The objective of this study is to examine the OCTA findings in PIC. PATIENTS AND METHODS: Observational, retrospective review of five patients with PIC. RESULTS: OCTA revealed several features in PIC lesions: distinctly visible choroidal neovascular membranes when present, as well as flow voids within the choroid and choriocapillaris layers that correspond with hypocyanescent lesions on indocyanine green angiography. CONCLUSION: OCTA may serve as a useful adjunctive imaging modality in diagnosing and monitoring patients with PIC. [Ophthalmic Surg Lasers Imaging Retina. 2017;48:786-792.].

Multi-Modal Imaging Including Optical Coherence Tomography Angiography in Patients With Posterior Multifocal Placoid Pigment Epitheliopathy.

BACKGROUND AND OBJECTIVE: New imaging methods provide novel insights into the pathogenesis of acute posterior multifocal placoid pigment epitheliopathy (APMPPE). PATIENTS AND METHODS: Four patients (eight eyes) in acute, subacute, and late phases of the disease were analyzed with multi-modal imaging methods including optical coherence tomography angiography (OCTA), structural OCT, fundus photography, and fundus autofluorescence. One patient was observed during the entire disease course. RESULTS: In acute and subacute phases of the disease, an early blockage in fluorescein angiography was found. OCTA showed perfusion defects in choriocapillaris and choroid slabs. During the course of disease, perfusion deficits observed in OCTA imaging resolved first in the choroid and then in the choriocapillaris slab. CONCLUSION: Multi-modal imaging including OCTA supports the thesis that the underlying pathology of APMPPE is an inflammatory impairment of the choroidal circulation with secondary damage to the outer retina. OCTA might be able to replace fluorescein and indocyanine angiography in the diagnostic work-up of APMPPE. [Ophthalmic Surg Lasers Imaging Retina. 2017;48:727-733.].

Multimodal Imaging Including Optical Coherence Tomography Angiography in Serpiginous Choroiditis.

PURPOSE: To report multimodal imaging in patients with serpiginous choroiditis. METHODS: A 60-year-old woman with active serpiginous choroiditis in the right eye was evaluated during the disease course with multimodal imaging, which included fluorescein angiography, swept-source optical coherence tomography (SS-OCT), OCT angiography, fundus autofluorescence (FAF), and retromode imaging. RESULTS: The patient had subretinal, yellowish lesion on the macula at presentation. The margin of the lesion was remarkable in FAF and retromode imaging. SS-OCT scans revealed slightly thickened hyporeflective space and tiny hyperreflective spots at the choriocapillaris level. OCT angiography demonstrated decreased vascularity on the choriocapillaris. Although the area was partially replaced with irregular capillaris, the photoreceptor defect persisted following systemic corticosteroid therapy. CONCLUSIONS: Multimodal imaging shows that inflammation of the choriocapillaris is a main pathology of serpiginous choroiditis. The destruction of the choriocapillaris may lead to the photoreceptor disruption, resulting in permanent visual loss in serpiginous choroiditis.

OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY SHOWS INNER CHOROIDAL ISCHEMIA IN ACUTE POSTERIOR MULTIFOCAL PLACOID PIGMENT EPITHELIOPATHY.

PURPOSE: To describe multimodal imaging findings of an evolving case of acute posterior multifocal placoid pigment epitheliopathy occurring in a young healthy male. METHODS: Case report of a patient with acute posterior multifocal placoid pigment epitheliopathy including comprehensive systemic and ocular examinations. Ultra-widefield autofluorescence, fluorescein angiography, indocyanine green angiography, and serial optical coherence tomography angiography were performed. RESULTS: A 34-year-old male presented with acute vision loss in his left eye for 2 weeks. His best-corrected visual acuity was 20/20 in his right eye and 20/200 in his left eye. Dilated funduscopic examination revealed multiple creamy white deep retinal lesions showing macular involvement of the left eye with a diffuse area of pigmentary changes. The presence of multiple areas of hypoperfusion of the inner choroid were demonstrated with fluorescein and indocyanine green angiography. Serial optical coherence tomography angiography showed multiple evolving areas of decreased flow at the level of the inner choroid. CONCLUSION: Although the pathogenesis of acute posterior multifocal placoid pigment epitheliopathy remains unknown, there is growing evidence of a primary choroidal involvement with secondary damage to the overlying retinal pigment epithelium and the outer retinal layers. Optical coherence tomography angiography may provide valuable information for the diagnosis and follow-up of this condition avoiding invasive angiographic procedures.