Punctate Inner Choroidopathy Lesions Research Articles

PUNCTATE INNER CHOROIDOPATHY FOLLOWING PARS PLANA VITRECTOMY FOR HIGH MYOPIC FULL THICKNESS MACULAR HOLE.

To describe a case of punctate inner choroidopathy (PIC) and secondary or epiphenomenon multiple evanescent white dot syndrome (EpiMEWDS) following surgery for high myopic full-thickness macular hole (FTMH). Case report. A 57-year-old high myopic female was diagnosed with cataract and FTMH in the left eye. Her initial best-corrected visual acuity (BCVA) was 20/20 in her right eye and 20/80 in the left eye. She underwent routine combined phacoemulsification and 25-gauge pars plana vitrectomy (PPV) with the inverted internal limiting membrane (ILM) technique and twice-repeated epiretinal membrane (ERM) and ILM staining in the left eye. Two weeks postoperatively, the patient reported significant visual decline and photopsia in her left eye. BCVA decreased to counting fingers. Anterior segment examination was unremarkable. Ophthalmoscopic examination showed multiple whitish-yellow lesions in the macular region compatible with PIC lesions in the left eye. Optical coherence tomography (OCT), blue-light fundus autofluorescence (BAF), fluorescein angiography, and indocyanine green angiography were performed and confirmed the diagnosis. The patient underwent oral steroid therapy for PIC treatment. One week after treatment initiation, BAF showed the occurrence of EpiMEWDS. After one month, all lesions resolved and BCVA improved to 20/100. We report a rare case of PIC and EpiMEWDS development following surgery for FTMH. We hypothesize that several causes, including individual susceptibility (high myopia and female gender), post-surgical inflammation, and/or dye toxicity due to repeated staining could have amplified this inflammatory chorioretinal response. Larger studies are needed to better understand the potential triggers of PIC development after surgery.

Punctate Inner Choroidopathy (PIC) disease recurrence with inflammatory choroidal neovascular membrane (iCNVM) post-COVID-19 vaccine.

To report a recurrence of punctate inner choroidopathy (PIC) with an inflammatory choroidal neovascular membrane (iCNVM) after the Pfizer-BioNTech COVID-19 vaccine. Case report. A 38-year-old female with a history of myopia and previous episodes of PIC and iCNVM presented with distorted vision in her right eye, seven days after receiving the first dose of the Pfizer-BioNTech COVID-19 vaccine. The patient exhibited active PIC lesions with iCNVM confirmed on multimodal imaging. Treatment with a combination of oral corticosteroids and intravitreal anti-VEGF injection led to disease resolution. Subsequent COVID-19 vaccinations, administered while the patient was immunosuppressed, did not lead to disease relapse. However, relapse occurred following the fourth COVID-19 vaccine, when the patient was not immune suppressed. This case highlights the potential risk of PIC disease relapse following COVID-19 vaccination. Further research is needed to investigate the relationship between COVID-19 vaccination and PIC exacerbation, as well as to determine optimal management strategies in this population, including close observation and consideration of prophylactic immune suppression at the time of COVID-19 vaccine for high-risk individuals.

Spectrally resolved autofluorescence imaging in posterior uveitis

Clinical discrimination of posterior uveitis entities remains a challenge. This exploratory, cross-sectional study investigated the green (GEFC) and red emission fluorescent components (REFC) of retinal and choroidal lesions in posterior uveitis to facilitate discrimination of the different entities. Eyes were imaged by color fundus photography, spectrally resolved fundus autofluorescence (Color-FAF) and optical coherence tomography. Retinal/choroidal lesions’ intensities of GEFC (500–560 nm) and REFC (560–700 nm) were determined, and intensity-normalized Color-FAF images were compared for birdshot chorioretinopathy, ocular sarcoidosis, acute posterior multifocal placoid pigment epitheliopathy (APMPPE), and punctate inner choroidopathy (PIC). Multivariable regression analyses were performed to reveal possible confounders. 76 eyes of 45 patients were included with a total of 845 lesions. Mean GEFC/REFC ratios were 0.82 ± 0.10, 0.92 ± 0.11, 0.86 ± 0.10, and 1.09 ± 0.19 for birdshot chorioretinopathy, sarcoidosis, APMPPE, and PIC lesions, respectively, and were significantly different in repeated measures ANOVA (p < 0.0001). Non-pigmented retinal/choroidal lesions, macular neovascularizations, and fundus areas of choroidal thinning featured predominantly GEFC, and pigmented retinal lesions predominantly REFC. Color-FAF imaging revealed involvement of both, short- and long-wavelength emission fluorophores in posterior uveitis. The GEFC/REFC ratio of retinal and choroidal lesions was significantly different between distinct subgroups. Hence, this novel imaging biomarker could aid diagnosis and differentiation of posterior uveitis entities.

Optical Coherence Tomography Findings of Underlying Choroidal Neovascularization in Punctate Inner Choroidopathy.

Purpose: To analyze findings on optical coherence tomography (OCT) suggestive of choroidal neovascularization (CNV) in lesions of punctate inner choroidopathy (PIC).Methods: In this multi-center retrospective study, clinical data of patients with PIC were retrospectively analyzed. Quantitative data (height, width, and volume of PIC lesions), and qualitative data (disruption of ellipsoid zone (EZ)/Bruch's membrane (BM), outer retinal fuzziness, and choroidal back-shadowing) were compared between CNV+ and CNV– groups using Mann–Whitney U-test and Fischer's exact test.Results: In total, 35 eyes (29 patients; 21 women; mean age: 33.3 ± 6.5 years) were selected for analysis. Of the 35 PIC lesions studied, 17 had underlying CNV. Lesions with CNV+ had larger height, width, and volume (p < 0.001) and several distinctive features, such as disruption of EZ and BM, outer retinal fuzziness, and hypo-reflective back-shadowing (p < 0.001) compared with CNV—lesions.Conclusions: Quantitative and qualitative OCT analysis can aid in the prediction of an underlying CNV in the eyes with PIC.

Characteristics of punctate inner choroidopathy complicated by choroidal neovascularisation on Multispectral Imaging in comparison with other imaging modalities

ABSTRACT Purpose To describe the characteristics seen on multispectral imaging (MSI) in patients with punctate inner choroidopathy (PIC) and choroidal neovascularisation (CNV) and compare the findings with current standard multimodal imaging techniques. Methods This is a retrospective observational case series of 10 patients with PIC complicated by CNV that underwent multimodal retinal imaging examinations. Results Twelve eyes of 10 patients were included. CNV was identified in 11 of the 12 eyes (91.7%) by MSI with nodular or trunk-like hyperreflectance on retinal oxy/deoxyhemoglobin map. MSI revealed choroidal vasculature around CNV in 91.7% eyes and pathological changes including retinal pigment epithelial atrophy and melanin disruption of punctate lesions in all eyes. Conclusion MSI helps in noninvasively detecting CNV in PIC patients and observing associated changes in choroidal vasculature. This imaging technique is also a promising tool for better tracking pathological changes of PIC lesions complementary to current standard multimodal imaging modalities.

Presence or absence of choroidal hyper-transmission by SD-OCT imaging distinguishes inflammatory from neovascular lesions in myopic eyes.

To compare the characteristics of choroidal transmission in punctate inner choroidopathy (PIC) with or without choroidal neovascularization (CNV) and myopic CNV (mCNV) using spectral domain optical coherence tomography (SD-OCT). This retrospective observational case series includes 22 consecutive myopic patients (22 eyes) recruited from April 2016 until April 2018 who complained of acute blurring of vision and showed evidence of hyper-reflective material on SD-OCT imaging. Each patient underwent a comprehensive eye examination and imaging with fundus fluorescein angiography (FFA), SD-OCT, and SD-OCT angiography (SD-OCTA). Based on the results of SD-OCTA imaging and the color fundus imaging, the patients were divided into 2 groups: a group with myopic choroidal neovascularization (mCNV group, n = 10 eyes) and a group with PIC and no evidence of CNV at baseline (PIC group, n = 12 eyes). Four eyes diagnosed with PIC developed secondary PIC-CNV during follow-up. The characteristics of choroidal transmission in these eyes using SD-OCT imaging were compared. At baseline, none of the PIC lesions showed any evidence of blood flow within the lesions on OCTA imaging. However, all of the eyes with mCNV showed flow signals within the subretinal neovascularization on SD-OCTA and subretinal or intra-retinal fluid on SD-OCT imaging. These eyes with mCNV showed subretinal hyper-reflectivity associated with choroidal hypo-transmission accompanied by retinal pigment epithelium (RPE) and ellipsoid zone (EZ) disruption. In eyes with PIC inflammatory lesions, disruption of both the RPE and EZ were observed in 33% of the inflammatory lesions, and disruption of the EZ alone was observed in 67% of the lesions at the baseline. They all showed a hyper-reflective subretinal lesion located above RPE. Three cases (25%) showed evidence of choroidal hyper-transmission at the baseline, while the remaining had normal transmission within the first month after onset. Hyper-transmission then developed in all the lesions as the disease progressed. Four cases of PIC (33%) developed PIC-related CNV that showed choroidal hypo-transmission along with hyper-transmission with disruption of the RPE and EZ. In cases with PIC-related CNV, evidences of neovascularization on SD-OCTA imaging were all detected afterwards. No intra-retinal fluid was detected before secondary CNV occurred. SD-OCT imaging can noninvasively differentiate and track the progression of inflammatory lesions and myopic CNV by using the presence of choroidal hyper-transmission as a sign of just an inflammatory lesion and the presence hypo-transmission as a sign of a secondary CNV, which provides a convenient strategy for diagnosis and treatment of these lesions.

Treatment of Punctate Inner Choroidopathy with Choroidal Neovascularization Using Corticosteroid and Intravitreal Ranibizumab.

Background To evaluate the treatment outcomes of patients with punctate inner choroidopathy (PIC) and secondary choroidal neovascularization (CNV). Methods This is a retrospective study of 24 eyes in 22 patients suffering from PIC with CNV. Patients were treated with intravitreal ranibizumab monotherapy (14 eyes) or combined oral corticosteroid and intravitreal ranibizumab therapy (corticosteroid-ranibizumab group, 10 eyes). Mean follow-up duration was 24.0 months. We evaluated best-corrected visual acuity (BCVA), fundus autofluorescence, fluorescein angiography, indocyanine green angiography, and optical coherence tomography, before and after treatment. The following variables were compared between groups: number of intravitreal ranibizumab injections, BCVA, recurrence of CNV, and change in PIC lesions. Results The ranibizumab monotherapy group received an average of 3 intravitreal ranibizumab injections; mean logMAR visual acuity improvement was 0.34, and 8 eyes developed recurrent CNV during follow-up. The corticosteroid-ranibizumab group received an average of 1.9 intravitreal ranibizumab injections; mean logMAR visual acuity improvement was 0.61, and there was no recurrence of CNV. Combined corticosteroid-ranibizumab therapy also resulted in better resolution of PIC lesions and fewer new PIC lesions. Conclusion Both corticosteroid-ranibizumab treatment and ranibizumab monotherapy could significantly improve the vision of PIC patients with CNV. Combined corticosteroid and intravitreal ranibizumab treatment appeared to reduce CNV recurrence and development of new PIC lesions compared with ranibizumab monotherapy.

The Fundus Autofluorescence Spectrum of Punctate Inner Choroidopathy.

Purpose. To investigate the fundus autofluorescence (FAF) spectrum of punctate inner choroidopathy (PIC). Methods. This is a retrospective observational case series of 27 consecutive patients with PIC admitted from October 2013 to March 2015, who underwent short-wavelength- (SW-) and near-infrared- (NIR-) FAF imaging, spectral domain optical coherence tomography (SD-OCT), fluorescein angiography (FA), and indocyanine green angiography (ICGA). Results. There were three primary findings on the FAF imaging of patients with PIC. First, active PIC lesions revealed hypoautofluorescent spots with hyperautofluorescent margin. After the lesions regressed, the hyperautoflurescent margin faded. Second, subclinical and most of the atrophic PIC lesions appeared to be hypoautofluorescent spots. But subclinical PIC lesions were more distinctive on NIR-FAF imaging than on SW-FAF imaging. Third, hypoautofluorescent spots of PIC lesions coexisted with hyperautofluorescent patches on SW-FAF imaging. These hyperautofluorescent patches were demonstrated to be multiple evanescent white dot syndrome (MEWDS) or acute zonal occult outer retinopathy (AZOOR) lesions by subsequent multimodal imaging and faded during follow-up examinations. Conclusion. FAF imaging helps in noninvasively tracking the evolution of PIC lesions and identifying the combined MEWDS or AZOOR lesions, complementary to SD-OCT and angiographic studies.

Characterization of Punctate Inner Choroidopathy Using Enhanced Depth Imaging Optical Coherence Tomography

To perform qualitative and quantitative analyses of retinal and choroidal morphology in patients with punctate inner choroidopathy (PIC) using enhanced depth imaging optical coherence tomography (EDI-OCT). Cross-sectional, consecutive series. A total of 2242 patients attending 2 tertiary referral uveitis clinics at Moorfields Eye Hospital were screened; 46 patients with PIC diagnosis were identified, and 35 eyes (35 patients) had clinically inactive PIC had EDI-OCT images that met the inclusion criteria. Punctate inner choroidopathy lesions were qualitatively assessed for retinal features, such as (1) focal elevation of the retinal pigment epithelium (RPE), (2) focal atrophy of the outer retina/RPE, and (3) presence of sub-RPE hyperreflective deposits and choroidal features: (a) presence of focal hyperreflective dots in the inner choroid and (b) focal thinning of the choroid adjacent to PIC lesions. Quantitative analyses of the retina, choroid, and choroidal sublayers were performed, and associations with clinical and demographic data were examined. Prevalence of each lesion pattern and thickness of retinal and choroidal layers. A total of 90 discrete PIC lesions were captured; 46.6% of PIC lesions consisted of focal atrophy of the outer retina and RPE; 34.4% consisted of sub-RPE hyperreflective deposits; and 18.8% consisted of localized RPE elevation with underlying hyporeflective space. Focal hyperreflective dots were seen in the inner choroid of 68.5% of patients, with 17.1% of eyes presenting focal choroidal thinning underlying PIC lesions. By excluding high myopes, patients with "atypical" PIC had reduced retinal thickness compared with patients with "typical" PIC (246.65±30.2 vs. 270.05±24.6 μm; P = 0.04), and greater disease duration was associated with decreases in retinal thickness (r = -0.53; P = 0.01). A significant correlation was observed between best-corrected visual acuity and foveal retinal thickness (r = -0.40; P = 0.03). In a large series of patients with clinically inactive PIC, one fifth of the lesions analyzed revealed RPE elevation with underlying hyporeflective space, described before as a sign of activity and suggesting subclinical inflammation. Retinal thickness seems to be associated with disease type and duration of disease in non-highly myopic eyes. Improved visualization of the inner choroid using EDI-OCT may allow noninvasive assessment of inflammatory status.

Spectral-Domain Optical Coherence Tomographic Findings at Each Stage of Punctate Inner Choroidopathy

To describe the natural course of punctate inner choroidopathy (PIC) using spectral-domain optical coherence tomography (SD-OCT). Prospective observational case series. Forty-two consecutive patients (60 eyes) with PIC with at least 3 months of follow-up. Serial SD-OCT images were obtained from both eyes of each participant at each visit. The morphologic characteristics of each stage of PIC lesions observed by SD-OCT. Continued stage progression of PIC lesions was observed in 27 eyes (45%), among which choroidal thickness changes were observed in 8 eyes (30%). Stage I lesion showed a normal appearance or slight irregularities in the outer nuclear layer. Stage II lesion appeared as a focal elevation of the retinal pigment epithelium (RPE) with corresponding disruption of the inner and outer segments of the photoreceptor interface. Stage III lesion broke through the RPE, forming a hump-shaped chorioretinal nodule with moderate reflectivity beneath the outer plexiform layer (OPL), generally with subsequent disruption of Bruch's membrane. Nodules occasionally invaded the inner retina, causing segmental retinal phlebitis in 2 eyes. Stage IV lesion regressed in a retrograde manner with tissue loss from the photoreceptor layer and inner choroid, finally leaving a V-shaped hernia of the OPL and inner retina into the choroid. Stage V lesion gradually eliminated the photoreceptors around the lesion; this process was accompanied by RPE proliferation at multiple levels, which reduced retinal herniation. Parafoveal stage V lesions caused late occult macular atrophy in 4 eyes. Choroidal thickness increased throughout the active phase and reached a peak at stage III; this parameter then significantly decreased at stage IV and gradually reached a minimum that was lower than the initial value at stage I. Punctate inner choroidopathy is a chorioretinitis rather than an inner choroidopathy. Spectral-domain OCT characterized a 5-stage evolution of PIC lesions: choroidal infiltration, formation of sub-RPE nodules, and then chorioretinal nodules, regression, and retinal herniation.

Characterization of macular lesions in punctate inner choroidopathy with spectral domain optical coherence tomography

PurposePunctate inner choroidopathy (PIC) is an ocular inflammatory disease. Spectral domain optical coherence tomography (SD-OCT) allows detailed visualization of retinal and choroidal structures. We aimed to describe the retinal changes on SD-OCT associated with PIC lesions localized in the macula.MethodsRetrospective case series: PIC lesions not associated with choroidal neovascularization (CNV) and captured by macular SD-OCT scans were identified and characterized.ResultsTwenty-seven PIC lesions from seven patients (eight eyes) were identified and classified into four categories according to disease activity and temporal changes. Among clinically inactive patients, two main patterns were noted on OCT: (1) retinal pigment epithelium (RPE) elevation with sub-RPE hyper-reflective signals and (2) localized disruption of outer retinal layers with choroid and Bruch's membrane (BM) generally spared. Clinically active patients demonstrated lesions with intact BM with RPE elevation that fluctuated with disease activity and sub-RPE hyper-reflective signals. Photoreceptor-associated bands on SD-OCT (PRs) were not visible during active disease, but returned to normal visibility when lesions were clinically stable. Seven lesions in patients without clinically detected activity demonstrated alteration of RPE elevation.ConclusionSD-OCT can provide detailed structural characteristics of PIC lesions. RPE elevation is noted in many lesions while BM and choroid are spared. Photoreceptor-associated bands on SD-OCT appear compressed during clinically active stages and are visible during stabilization. OCT may provide information on activity not detected clinically.Electronic supplementary materialThe online version of this article (doi:10.1007/s12348-011-0054-6) contains supplementary material, which is available to authorized users.

CLINICAL FEATURES OF PUNCTATE INNER CHOROIDOPATHY IN CHINESE PATIENTS

To characterize the clinical features of punctate inner choroidopathy (PIC) in Chinese patients. A retrospective chart review of all patients admitted with PIC from June 1999 to October 2009. Of the 75 patients (112 eyes) diagnosed with PIC, 72% were women, 80% were myopic, and 49% exhibited bilateral involvement. The mean age at presentation was 32 years. Blurred central vision (64%) and scotoma (40%) were the most common symptoms. Multifocal PIC lesions were mostly restricted to the posterior pole (95%). The acute lesions were yellow and demonstrated hyperfluorescence (61%) or mild hyperfluorescence (36%) on fluorescein angiography, mostly (75%) <200 μm in diameter and <10 in number. Indocyanine angiography showed subclinical hypofluorescent spots in 32% of the affected eyes. Choroidal neovascularization developed in 63% of affected eyes. Papilledema (3%) and segmental retinal phlebitis (2%) were rare. Intact follow-up data of 27 patients (43 eyes) were available. Acute lesions turned into punched-out atrophic lesions within 3 months. New visible lesions developed in 5 eyes within 1 year after symptom onset. New choroidal neovascularization developed in five eyes. The mean best-corrected visual acuity significantly improved at last follow-up (P = 0.039). Punctate inner choroidopathy in Chinese is not rare and primarily affects young myopic women. It features multifocal, small, yellow lesions that develop within a short period, principally in the posterior pole, with subsequent atrophy. Complicated choroidal neovascularization is frequent. General visual prognosis is moderately good.

Clinical features of punctate inner choroidopathy in Chinese patients

Objective To characterize the clinical features of punctate inner choroidopathy (PIC) in Chinese patients. Methods The clinical data of 75 PIC patients (112 eyes) attending this center from June 1999 to October 2009 were reviewed retrospectively. All patients received routine examination and fluorescein fundus angiography (FFA). Twenty-eight patients also received indocyanine green angiography (ICGA). VISUPAC 3.3 software was used to determine the size of lesions in early image of FFA at the artery stage. Results Of the 75 PIC patients (112 eyes), 54 patients (72%) were female, 37 patients (49%) were bilateral cases. Sixty patients (80%) were myopic, including eight patients (7%) with mild myopia, 22 patients (20%) with moderate myopia, and 57 patients (51%) with high myopia. The mean age at presentation was 32 years (range: 17 - 61). Multifocal PIC lesions (1-56 lesions) were mostly restricted to posterior pole of affected eyes (95%). Eighty-four eyes (75%) had 10 PIC lesions. Thc active lesions were yellow-white and butter-like, 20-500pm in diameter. FFA showed that most acute lesions were early hyperfluorescence, and stained or slightly leaked on late period. The atrophic lesions were pouched-out, 50-2000 μm in diameter, with irregular pigmentation. Choroidal neovascularization developed in 70 eyes (63%). Papilledema (three eyes, 3%), staining of optic disc on late-phase fluorescein angiography (three eyes, 3%), and segmental retinal phlebitis (two eyes, 2%) were rare. Conclusions PIC primarily affects young women with moderate or high myopia. It is featured by multifocal small yellow creamy lesions and/or atrophic punched-out lesions principally in the posterior pole. Choroidal neovascularization is the most common complication. Key words: Choroiditis/diagnosis; Fluorescein angiography; Disease attributes

Punctate Inner Choroidopathy

To describe the clinical features and outcomes of a large group of patients with a spectrum of clinical appearances and diagnosed as having punctate inner choroidopathy (PIC). In a retrospective consecutive case series, patients seen during a 16-year period at Moorfields Eye Hospital who were diagnosed as having PIC and had a minimum of 12 months' follow-up were included. Patients were classified as having typical PIC or atypical PIC (larger, presumed ocular histoplasmosis syndrome-like lesions). Main outcome measures included development of choroidal neovascularization, development of new PIC lesions, and final visual acuity. A total of 136 patients (271 eyes) were included. The average age was 32 years, 126 patients (93%) were female, and the mean refraction was -4.6 diopters. The overall mean follow-up was 6.2 years. Among 63 normal fellow eyes, 56 (88%) remained unchanged, 3 (5%) developed PIC lesions, and 4 (6%) developed choroidal neovascularization. Eyes with PIC lesions remained unchanged in 49 of 74 cases (66%), with 9 (12%) developing new PIC lesions and 16 (22%) developing choroidal neovascular membrane. In eyes with choroidal neovascularization, the mean logMAR visual acuity was 0.63 at study entry, 0.63 at 12 months, 0.61 at 2 years, and 0.71 at final review (mean, 6.1 years). Overall, 40 eyes with PIC-related choroidal neovascular membrane (26%) had final visual acuity less than 6/60. No differences were observed between typical and atypical PIC eyes in any of the outcome measures or in any of the subgroup analyses. Punctate inner choroidopathy-related choroidal neovascularization was not visually benign. No differences were observed between eyes with typical and atypical choroidal lesions, supporting the notion that they represent a spectrum of a single disease, PIC.