Impact of Solar Eclipses on Vision: Insights from Optical Coherence Tomography and Optical Coherence Tomography Angiography Analysis.

PURPOSE: Solar retinopathy, resulting from solar eclipse exposure, poses risks to visual health. This study explores acute and chronic phase findings using clinical examinations and optical coherence tomography (OCT) and optical coherence tomography angiography (OCT-A) with a focus on longitudinal assessment. METHODS: Seven eyes with a history of unprotected solar eclipse exposure were included. Clinical examination, fundus photography, OCT, and OCT-A imaging were performed at initial assessment, as well as at one-month and six-month follow-up intervals. Data analysis included descriptive statistics. RESULTS: The cases, exposed without protection, underwent assessments, revealing variable visual acuity, outer retinal layer, and Henle fiber layer changes during follow-up. Regression of hyperreflectivity within the outer retinal and Henle fiber layers was observed over time in all eyes, although persistent microdefects within the outer retinal layer were noted in specific cases. OCT-A imaging revealed a larger foveal avascular zone, which persisted over a six-month period in select cases. Additionally, affected eyes exhibited a decrease in superficial vascular density, with subsequent improvement noted during the six-month period. CONCLUSION: Solar retinopathy can result in visual impairment, accompanied by alterations observed in the Henle fiber layer using OCT. Additionally, OCT-A findings indicate possible vascular involvement. This study underscores the significance of adopting protective measures during solar eclipses and emphasizes the value of employing longitudinal multimodal imaging techniques to comprehend the pathophysiology of the condition.

Multimodal Imaging for Subacute Sclerosing Panencephalitis: Including Anterior Segment.

Subacute sclerosing panencephalitis (SSPE) is an irreversible and progressive neurological disorder. A 20-year-old woman with SSPE presented with a decline in visual acuity. Anterior segment was evaluated with slit lamp and in vivo confocal microscopy, revealing the presence of dendritiform keratic precipitates and +2 cells in the anterior chamber. In fundus examination, white-yellowish lesions involving the macula and periphery were observed, which gradually progressed into atrophy over time. Optical coherence tomography (OCT) imaging showed progression to atrophy of moth-eaten shaped cavities including all retinal layers due to necrotizing retinitis. OCT angiography (OCTA) further revealed reduced vessel densities and flow void areas. Notably, to the best of our knowledge, this is the first case documenting anterior segment findings in SSPE in detail. [Ophthalmic Surg Lasers Imaging Retina 2024;55:344-348.].

Evaluation of Microvascular Complications in Kidney Recipients with Post-Transplant Diabetes Mellitus.

CONTEXT: Limited data on microvascular complications in patients with post-transplant diabetes (PTDM) is an obstacle to developing follow-up algorithms. OBJECTIVES: To evaluate diabetic microvascular complications in patients with long-standing PTDM. METHODS: This study included patients with at least a five-year history of PTDM and age-matched renal transplant recipients without PTDM (NDM). Diabetic peripheral neuropathy (PN) was evaluated using the Michigan Neuropathy Screening Instrument (MNSI), the CASE IV device, and in vivo corneal confocal microscopy (CCM). Cardiac autonomic neuropathy (CAN) tests were performed using the heart rate variability. Nephropathy (DN) screening was assessed using spot urine albumin/creatinine ratio and eGFR calculation. Diabetic retinopathy (DR) was evaluated by fundus examination and photography, and optical coherence tomography. RESULTS: This study included 41 patients with PTDM and 45 patients in the NDM group. The median follow-up was 107.5 months in the PTDM group. PN was significantly higher in the PTDM group than in the NDM group (p = 0.02). In the PTDM patients with PN, the corneal nerve fiber density examined by CCM was significantly lower than in the PTDM patients without neuropathy (p = 0.001). Parasympathetic involvement was observed in 58.5% of the PTDM group and 22% of the NDM group (p = 0.001). Sympathetic involvement was present in 65.9% of the PTDM group and 29.3% of the NDM group (p = 0.001). Retinopathy was observed in 19.5% of patients in the PTDM group, while none were in NDM patients (p < 0.001). Renal functions were similar between the study groups. CONCLUSIONS: CAN and DR can affect patients with PTDM at a high rate. DR was found to be a threat to the vision of PTDM patients. Diabetic PN can be detected early in PTDM patients by CCM.

Recovery course of persistent posterior subretinal fluid after successful repair of rhegmatogenous retinal detachment.

PURPOSE: To investigate best corrected visual acuity (BCVA), subretinal fluid (SRF) absorption time or ellipsoid zone (EZ) restoration time and various variables in patients with persistent SRF after successful primary repair of rhegmatogenous retinal detachment (RRD). METHODS: This retrospective multicenter study allowed independent analysis of the healing pattern by two observers based on composite of serial cross-sectional macular optical coherence tomography (OCT) scans. Univariate and multivariate analyses were implemented. RESULTS: One hundred and three cases had persistent SRF after pars plana vitrectomy, scleral buckling, or pneumatic retinopexy. By univariate analysis, SRF resolution time correlated positively with the number of retinal breaks (p < 0.001) and with increased myopia (p = 0.011). Using multivariate analysis, final BCVA (log MAR) correlated positively with age, duration of RRD, initial BCVA (OR = 3.28; [95%CI = 1.44-7.47]; p = 0.015), and SRF resolution time (OR = 0.46 [95%CI 0.21-1.05]; p = 0.049). EZ restoration time was longer with increasing number of retinal tears (OR = 0.67; [95%CI 0.29-1.52]; p = 0.030), worse final BCVA, and presence of macula-off RRD (OR = 0.26; [95%CI 0.08-0.88]; p = 0.056). SRF resolution time correlated marginally with prone position. CONCLUSIONS: Residual posterior SRF is more common in eyes with multiple breaks or in myopic eyes. Final BCVA is better in younger subjects and in eyes with shorter duration of RRD. Persistent SRF is a self-limited disorder with a mean resolution of 11.2 months with good visual prognosis improving from a mean baseline logMAR of 1.08 to 0.25 at one year.

Short-term effect of macular edema on the peripapillary retinal nerve fiber layer in patients with wet age-related macular degeneration and diabetic macular edema: A comparative study.

BACKGROUND: The combined presence of glaucoma and age-related macular degeneration (ARMD), or glaucoma and diabetes mellitus (DM), occur fairly frequently, especially in elderly patients. This study was intended to compare the effect of resolving macular edema due to DM and wet ARMD on peripapillary retinal nerve fiber layer (RNFL) thickness. METHODS: This cross-sectional study included 76 patients with macular edema secondary to DM (n = 40, 52.6%) or wet ARMD (n = 36, 47.4%). The control group was comprised of 34 age and sex-matched healthy subjects. All study participants underwent evaluation of central macular thickness (CMT) and the peripapillary RNFL using spectral domain-optical coherence tomography (SD-OCT). Data from eyes that received an anti-VEGF injection were obtained one month after the procedure and were compared with pre-injection data. RESULTS: The average initial thickness of the global peripapillary RNFL was 98.9 ± 16.7 (61-163) µm in the macular edema group and 92.0 ± 16.0 (84-115) µm in the control group (p = 0.045). The post-injection global peripapillary RNFL thickness was 97.3 ± 19.0 (61-163) µm in the macular edema group and 92.2 ± 18.0 (81-126) µm in the control group (p = 0.187). In the DM group, the changes in global RNFL thickness, as well as central and temporal quadrant thicknesses, were found to correlate significantly with the change in CMT (r = 0.356, p = 0.024; r = 0.545, p < 0.001, respectively). CONCLUSIONS: Macular edema in wet ARMD appeared not to affect RNFL thickness. Differences in the etiology of macular edema can have varied effects on peripapillary RNFL. It is recommended that peripapillary RNFL thickness be evaluated cautiously in DM patients receiving intravitreal anti-VEGF therapy.

Spontaneous hyphema in juvenile idiopathic arthritis uveitis.

BACKGROUND: Juvenile idiopathic arthritis (JIA) is a rheumatic disease that may be associated with ocular involvement in childhood. Classical findings of JIA uveitis are cells and flare; hyphema, bleeding in the anterior chamber of the eye, is a rare finding. CASE: An 8-year-old girl presented with 3+ cells and a flare in the anterior chamber. Topical corticosteroids were started. A follow-up examination 2 days later revealed hyphema in the affected eye. There was no history of trauma or drug use, and the laboratory test results did not suggest any hematological disease. Systemic evaluation resulted in the diagnosis of JIA by the rheumatology department. The findings regressed with systemic and topical treatment. CONCLUSIONS: The most common cause of hyphema in childhood is trauma, but it can rarely be seen with anterior uveitis. This case highlights the importance of recognizing JIA-related uveitis in the differential diagnosis of hyphema in childhood.

Successful management of rhabdomyolysis with triheptanoin in a child with severe long-chain 3-hydroxyacyl-coenzyme A dehydrogenase (LCHAD) deficiency.

Early-onset long-chain 3-hydroxyacyl-coenzyme A dehydrogenase (LCHAD) deficiency is a fatty acid ß-oxidation disorder with a poor prognosis. Triheptanoin, an anaplerotic oil with odd-chain fatty acids can improve the disease course. The female patient presented here was diagnosed at the age of 4 months, and treatment was started as fat restriction, frequent feeding, and standard medium-chain triglyceride supplementation. In follow-up, she had frequent rhabdomyolysis episodes (∼8 per year). At the age of six, she had 13 episodes in 6 months, and triheptanoin was started as part of a compassionate use program. Following unrelated hospital stays due to multisystem inflammatory syndrome in children and a bloodstream infection, she had only 3 rhabdomyolysis episodes, and hospitalized days decreased from 73 to 11 during her first year with triheptanoin. Triheptanoin drastically decreased the frequency and severity of rhabdomyolysis, but progression of retinopathy was not altered.

Report of 2 pediatric cases with atypical Cogan's syndrome and a systematic review.

Cogan's syndrome (CS) is a rare inflammatory disease characterized by interstitial keratitis or uveitis, vestibular impairment, and progressive hearing loss, commonly bilateral. Although glucocorticoids are fundamental treatment options, in most cases, hearing loss gradually worsens. Herein we report 2 pediatric cases of CS who were treated with corticosteroids and methotrexate. One patient had a cochlear implant, and the hearing of the other patient improved with treatment. Also, a systematic literature review was conducted for articles including pediatric CS patients. In the literature, 34 articles describing 44 pediatric patients with CS were identified. Sudden hearing loss (95.3%) and ocular symptoms (92.5%) were the most common manifestations in these patients. Also, aortic involvement was present in 19.5% of patients in the literature. Otorhinolaryngologists, ophthalmologists, and pediatricians should collaborate to diagnose and manage CS to prevent progressive hearing loss and eye involvement.

Expert recommendations for the management of diabetic macular edema with intravitreal dexamethasone implant: A Turkish Delphi study.

PURPOSE: To provide consensus on the clinical use of intravitreal dexamethasone (DEX) implant for the treatment of diabetic macular edema (DME) in Turkey. METHODS: A panel of 22 retina experts prepared 77 statements of recommendation, and 80 retinal specialists practicing in Turkey were chosen to vote either in support or against each one. A Delphi-based method was used through which the ophthalmologists were able to view all of the results anonymously after two rounds and modify their subsequent answers. The survey was conducted via a mini website, and statements without consensus were resent to the specialists with the latest vote results a week later. RESULTS: A total of 72 ophthalmologists answered the first and second round questions. After the first stage, consensus was achieved on 55 of the statements, leaving 22 without agreement. After the second stage, consensus was reached on 11 of the remaining statements. Strong consensus was achieved on statements regarding the etiopathogenesis of DME and the first-line indications and safety of the DEX implant procedure. The panel recommended the use of DEX implant for patients with an arterial thromboembolic event in the last three months and also agreed that pro re nata DEX implant treatment not only provides better outcomes for DME patients but also reduces the treatment burden for those who could not receive an adequate number of anti-vascular endothelial growth factor (VEGF) injections. CONCLUSION: This study provides clinical consensus and recommendations about the use of DEX implant in the clinical practice of DME management in Turkey.

Multimodal Imaging of Severe Oxalate Retinopathy in a 20-Month-Old Boy.

Primary hyperoxaluria type 1 (PH1) is a rare disease due to alanine glyoxylate aminotransferase enzyme deficiency caused by mutations in AGXT gene. Increased oxalate causes crystalline deposits in the eye particularly in conjunctiva, cornea, iris, retina, and choroid. A 20-month-old boy was referred with a diagnosis of infantile PH1. There were numerous yellowish crystalline deposits at the posterior pole and hyperpigmented lesions were detected around the macula. Enhanced depth imaging optical coherence tomography (EDI-OCT) revealed dome-shaped retinal pigment epithelium elevations, subretinal and intraretinal hyperreflective material and also choroidal hyperreflective lesions. To the best of our knowledge, this is the first case to document choroidal deposition by EDI-OCT at such an early period of life. [Ophthalmic Surg Lasers Imaging Retina 2022;53:697-700.].

Investigation of anterior segment structures of the eye in Behçet's disease using in vivo confocal microscopy.

PURPOSE: We sought to investigate alterations in the corneal subbasal nerve plexus and endothelium in patients with Behçet's disease (BD). METHODS: This cross-sectional study included 64 patients with BD and 30 age- and gender-matched healthy control subjects. Those with BD were classified as having ocular or non-ocular disease. All subjects underwent a corneal endothelial and subbasal nerve density evaluation using in vivo confocal microscopy (IVCM). The differences among groups were analyzed using the Kruskal-Wallis test followed by Dunn's multiple comparison procedure. RESULTS: The mean age of study participants was 35.7 ± 10.2 years (16-58) in the ocular BD group, 39.6 ± 14.9 years (11-66) in the non-ocular BD group, and 34.1 ± 11.2 years (21-55) in the control group. No statistical significance was found in terms of age (p = 0.259) or sex (p = 0.560) between groups. The mean endothelial cell density determined with IVCM was 2124.9 [Formula: see text] 417.4 cells/mm2 (1811-3275) in the ocular group and 2546 [Formula: see text] 335 cells/mm2 (1798-3280) in the control group (p = 0.000). In the ocular group, the mean density of the subbasal nerve plexus was significantly lower (p = 0.004), and nerve tortuosity was significantly higher (p = 0.002). CONCLUSIONS: Ocular BD could be responsible for changes in the corneal layers, especially endothelial and corneal nerve structures. Nerve density and tortuosity differences could be inflammatory indicators for BD.

Ranibizumab therapy for predominantly hemorrhagic neovascular age-related macular degeneration.

Objective: Predominantly hemorrhage represents one of the possible manifestations of choroidal neovascularisation (CNV) in eyes with age-related macular degeneration (AMD). The purpose of this study is to evaluate the effecte of ranibizumab treatment in patients with predominantly hemorrhagic CNV secondary to AMD. Methods: Twenty-five patients with predominantly hemorrhagic choroidal neovascularization due to AMD with at least three ranibizumab injections and followed up for at least 12 months were included in the study. The months of follow-up were recorded (baseline, 3rd, 6th, and 12th months). The change in central macular thickness (CMT) on optical coherence tomography, visual acuity (VA) in ETDRS letters, and lesion size on fundus fluorescein angiography were evaluated. Results: The mean age of the patients was 68.1±5.7 (range: 63-82) years, the mean follow-up was 19.9±14.5 (range: 12-67) months, and the mean number of injections was 4.0±1.4 (range: 3-15). The initial VA was 39.3±17.9 (range: 1-65) letters, CMT was 272.7±104 (range: 164-587) µm, and the initial lesion width was 11.4±10.5 (range: 1.3-45.7) mm2. The VA was 41.4±20.1 (range: 5-75) and 36.9±21.8 (range: 4-80) letters (p=0.150), CMT was 270.7±110 (range: 159-570) and 230.4±108 (range: 109-667) µm (p=0.009) and the lesion width was 10.9±11.5 (range: 1.1-39.7) and 10.4±11.6 (range: 1.2-44.3) mm2 at 6th and 12th month, respectively. No factor was found to be associated with final CMT. Conclusion: Although the final visual outcome is limited by the progression of the disease, hemorrhagic lesions treated with ranibizumab have stable anatomical outcome.

Assessment of systemic and ocular inflammation in juvenile idiopathic arthritis via choroidal vascularity index.

We aimed to evaluate the retina and the choroid in children with juvenile idiopathic arthritis (JIA) employing optical coherence tomography (OCT). This cross-sectional study, carried out between June 2017-December 2019, included JIA patients with (JIAU; n = 28) and without (JIAN; n = 65) uveitis and age-matched healthy controls (HC) (n = 102). Laboratory and demographic information of the children were obtained from hospital records. Activity of the disease was evaluated by the Juvenile Arthritis Disease Activity Score-71 (JADAS-71). Choroidal scans were obtained with spectral domain-OCT in enhanced-depth imaging (EDI)-OCT mode to assess choroidal thickness (ChT) at five locations (under the fovea, at 750 and 1500 µm nasal and temporal sections), luminal area (LA), stromal area (SA), total subfoveal choroidal area (TCA) and CVI (choroidal vascularity index). Central foveal thickness (CFT) and 1-mm diameter foveal thickness (FT) were calculated automatically through macular volume scan analysis. The choroid was significantly thicker in JIAU and JIAN patients than in HC at the subfoveal and at the 750N, 750T, 1500T points (p < 0.001, p = 0.009, p < 0.001, and p < 0.001, respectively). The CVI was lower in JIAU patients than in JIAN patients and HC (p = 0.02). Conversely, CFT was greater in JIAU patients as compared to the JIAN patients and HC (p = 0.02). Changes in chorioretinal OCT parameters in the absence of uveitis in JIA patients may reflect subclinical choroidal inflammation in these patients. Ophthalmologic examination, including choroidal imaging in a larger cohort, may clarify this aspect.

Clinical characteristics of full thickness macular holes that closed without surgery.

PURPOSE: To ascertain the anatomic factors that help achieve non-surgical sealing in full thickness macular hole (FTMH). METHODS: Retrospective collaborative study of FTMH that closed without surgical intervention. RESULTS: A total of 78 patients (mean age 57.9 years) included 18 patients with blunt ocular trauma, 18 patients that received topical or intravitreal therapies and 42 patients with idiopathic FTMH. Mean±SD of the initial corrected visual acuity (VA) in logMAR improved from 0.65±0.54 to 0.34±0.45 (p<0.001) at a mean follow-up of 33.8±37.1 months. FTMH reopened in seven eyes (9.0%) after a mean of 8.6 months. Vitreomacular traction was noted in 12 eyes (15.8%), perifoveal posterior vitreous detachment in 42 (53.8%), foveal epiretinal membrane in 10 (12.8%), cystoid macular oedema (CME) in 49 (62.8%) and subretinal fluid (SRF) in 20 (25.6%). By multivariate analysis, initial VA correlated to the height (p<0.001) and narrowest diameter of the hole (p<0.001) while final VA correlated to the basal diameter (p<0.001). Time for closure of FTMH (median 2.8 months) correlated to the narrowest diameter (p<0.001) and the presence of SRF (p=0.001). Mean time for closure (in months) was 1.6 for eyes with trauma, 4.3 for eyes without trauma but with therapy for CME, 4.4 for eyes without trauma and without therapy in less than 200 µm in size and 24.7 for more than 200 µm. CONCLUSION: Our data suggest an observation period in new onset FTMH for non-surgical closure, in the setting of trauma, treatment of CME and size <200 µm.

Familial Mediterranean fever associated frosted branch angiitis, retinal vasculitis and vascular occlusion.

OBJECTIVES: To analyse the entity of retinal vasculitis, including frosted branch angiitis (FBA), or retina vascular occlusion in patients with familial Mediterranean fever (FMF). METHODS: Retrospective collaborative case series using invitation by email to uveitis specialists around the Mediterranean basin. This series was combined with a literature review. Exclusion criteria included infectious diseases, Behçet's disease or other autoimmune diseases. RESULTS: A total of 16 patients (21 eyes) had FMF and retinal vasculitis (FBA 11 patients, mild retinal vasculitis 5 patients). The mean age at onset of vasculitis was 29.5 ± 13.4 (range 9-62) with a female to male ratio of 9 to 7. In 19 eyes treated with various forms of corticosteroid and/or immunosuppression, the mean initial spectacle-corrected visual acuity improved from 6/194 to 6/10.5 at the last mean follow-up of 29.0 ± 34.9 months (p < 0.001). The most common FEVR mutations were M680I and M694V. In addition, retinal vascular occlusions included one case of central retinal artery occlusion and one case of branch retinal artery occlusion. CONCLUSION: FBA and milder forms of retinal vasculitis are associated with FMF. Therapy involves an increase in colchicine dosage in early cases, a long period of oral corticosteroid, intravitreal dexamethasone implant or periocular corticosteroid in select cases, and combination therapy with systemic immunosuppression in severe cases. FMF needs to be included in the differential diagnosis of retinal vasculitis.

Prevalence of cerebrotendinous xanthomatosis in cases with idiopathic bilateral juvenile cataract in ophthalmology clinics in Turkey.

PURPOSE: To determine the prevalence of cerebrotendinous xanthomatosis (CTX) in patients with idiopathic bilateral juvenile cataract in Turkey. METHODS: In this multicenter, epidemiologic observational study, patients with idiopathic bilateral juvenile cataract (aged ≥1 year at study entry) were identified from the records of 31 ophthalmology clinics. The Mignarri suspicion index determined in all potential CTX patients; blood samples were collected, and genetic testing for CYP27A1 gene mutation was performed. Cholestanol levels of the patients were measured, and those with a level of ≥3.75 µg/mL (threshold value) underwent genetic testing for mutations in the CYP27A1 gene. RESULTS: CTX was diagnosed in 7 of 452 patients (1.55%) with bilateral juvenile cataract. There was no significant difference between patients with a Mignarri score of <100 (n = 8) and those with a score of ≥100 (n = 19) with respect to cholestanol levels and genetic test results. Genetic testing was undertaken in 27 patients based on elevated cholestanol levels. Of these 27, 7 (26%) had CYP27A1 gene mutations. Of these 7 patients with genetically confirmed CTX, 5 (71%) were in the group with higher Mignarri score (≥100). CONCLUSIONS: CTX is a treatable condition, and early detection is crucial for avoiding irreversible neurological manifestations. Screening using the Mignarri suspicion index and cholestanol blood levels can be helpful in identifying suspected cases of CTX.

An Algorithm for the Diagnosis of Behçet Disease Uveitis in Adults.

Purpose: To develop an algorithm for the diagnosis of Behçet's disease (BD) uveitis based on ocular findings.Methods: Following an initial survey among uveitis experts, we collected multi-center retrospective data on 211 patients with BD uveitis and 207 patients with other uveitides, and identified ocular findings with a high diagnostic odds ratio (DOR). Subsequently, we collected multi-center prospective data on 127 patients with BD uveitis and 322 controls and developed a diagnostic algorithm using Classification and Regression Tree (CART) analysis and expert opinion.Results: We identified 10 items with DOR >5. The items that provided the highest accuracy in CART analysis included superficial retinal infiltrate, signs of occlusive retinal vasculitis, and diffuse retinal capillary leakage as well as the absence of granulomatous anterior uveitis or choroiditis in patients with vitritis.Conclusion: This study provides a diagnostic tree for BD uveitis that needs to be validated in future studies.

Prepapillary vascular loop-a new classification.

BACKGROUND/OBJECTIVES: To analyze the ophthalmic characteristics of congenital prepapillary vascular loop (PVL) and to propose a new morphologic classification dividing the loops into six types. SUBJECTS/METHODS: Collaborative multinational multicentre retrospective study of PVL cases. RESULTS: There was a total of 49 cases (61 eyes), 37 unilateral (75.5%) and 12 bilateral (24.5%), 32 arterial type (65.3%) and 18 venous type (36.7%) (one patient had either kind in each eye). The mean number of loops per eye was 2.7 (range, 1-7). The loops were asymptomatic in 42 cases (85.7%). Other findings included: the presence of cilioretinal artery (14 cases), retinal vascular tortuosity (26 cases), amaurosis fugax (1 case), branch retinal artery occlusion (1 case) and vitreous haemorrhage (3 cases). Six morphologic loop types could be discerned based on elevation (flat vs. elevated), shape (figure of 8 or corkscrew with hyaline sheath), number (multiple or single), location (central or peripheral), lumen size (arterial vs. arteriolar) and presence of vascular tortuosity or vitreous traction. CONCLUSIONS: PVL are usually asymptomatic and can be divided into six morphologic types with different pathogenesis during early embryogenesis.

Choroidal vascularity index as a biomarker of systemic inflammation in childhood Polyarteritis Nodosa and adenosine deaminase-2 deficiency.

BACKGROUND/PURPOSE: To assess EDI-OCT (enhanced depth imaging optical coherence tomography) of choroid for inflammatory signs in children with polyarteritis nodosa (PAN) and adenosine deaminase-2 deficiency (DADA-2). METHODS: In this cross-sectional study conducted between June 2017 and September 2018, we evaluated children diagnosed with PAN (n = 11) and DADA-2 (n = 4) and an age- and sex-matched control group (n = 15). Demographic and laboratory data were retrospectively analyzed from patient charts. Disease activity was assessed using the pediatric vasculitis activity score (PVAS). Choroidal images were obtained with spectral domain-OCT to measure choroidal thickness (ChT) at 5 points (750 and 1500 µm from the foveal center in the temporal and nasal quadrants and beneath the fovea), and to calculate the total subfoveal choroidal area (TCA), luminal area (LA), stromal area (SA), and the choroidal vascularity index (CVI). RESULTS: The median (min-max) age was 8 (4-16) years in PAN patients, 6 (5-16) years in DADA-2 patients and 8 (8-10) years in control group at the OCT visit (p = 0.214). The ChT at 3 points and the TCA, LA, and SA were higher in children with both PAN and DADA-2 patients compared to those of the control group (p < 0.0001, p = 0.049, p = 0.007, p = 0.007, p = 0.006, p = 0.033, respectively). The CVI was similar in both groups. No association was observed between the OCT findings, PVAS, and the erythrocyte sedimentation rate, and serum leukocyte and C-reactive protein levels. CONCLUSION: Similar CVI scores were obtained from PAN and DADA2 patients under treatment and from healthy controls. Increased subfoveal ChT without any other signs of ocular involvement may suggest choroidal thickening as a sign of mild subclinical inflammation.