Inversion of Asymmetric Vortex Vein Dilatation in Pachychoroid Spectrum Diseases.

Purpose: Intervortex venous anastomosis is widely recognized as compensating for vortex vein congestion in pachychoroid spectrum diseases. However, determining the blood flow direction within the compensated drainage route is often challenging. Herein, we investigated the morphological patterns of vortex veins in eyes showing retrograde pulsatile vortex venous flow. Design: Retrospective observational case series. Subjects: Six hundred eighty-nine consecutive eyes with treatment-naive central serous chorioretinopathy, pachychoroid neovasculopathy, or polypoidal choroidal vasculopathy. Methods: We reviewed the clinical records of patients with these pachychoroid spectrum diseases. Multimodal images including indocyanine green angiography (ICGA) and en face OCT were analyzed. Main Outcome Measures: Intervortex venous anastomosis between superotemporal and inferotemporal vortex veins and the dominant site of dilated temporal vortex veins were determined in the eyes with retrograde pulsatile vortex venous flow in the temporal vortex veins. Results: Twenty-two eyes with retrograde pulsatile vortex venous flow in the temporal vortex veins were identified utilizing early phase ICGA videos. In 9 eyes, retrograde pulsatile flow was detected in the superotemporal vortex veins, which were connected to the inferotemporal vortex veins via intervortex venous anastomoses. Among these cases, contralateral inferotemporal vortex vein dilatation was dominant in 7 eyes (77.8%), while superotemporal and inferotemporal vortex veins were symmetrically dilated in the other 2 eyes (22.2%). On the other hand, in 13 eyes, the retrograde pulsatile flow was detected in the inferotemporal vortex veins, which were linked to the superotemporal vortex veins via intervortex venous anastomoses. In these eyes, contralateral superotemporal vortex vein dilatation was dominant in 10 eyes (76.9%). Superotemporal and inferotemporal vortex veins were symmetrically dilated in 2 eyes (15.4%), while mainly inferotemporal vortex veins were dilated in 1 eye (7.7%). Conclusions: In pachychoroid spectrum diseases, there are cases wherein congested venous blood might drain into the contralateral vortex veins via intervortex anastomoses. Overloaded contralateral vortex veins may, as a consequence, become more dilated than the primary congested vortex veins. Inversion of asymmetric vortex vein dilatation might thereby develop in pachychoroid spectrum diseases. Financial Disclosures: Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Predicting treatment outcomes of intravitreal brolucizumab for polypoidal choroidal vasculopathy through noninvasive assessment of polypoidal lesion blood flow with optical coherence tomography angiography.

We investigated the assessment of blood flow within polypoidal lesions using optical coherence tomography angiography (OCTA) to determine intravitreal brolucizumab (IVBr) efficacy for treating polypoidal choroidal vasculopathy (PCV). We retrospectively studied 46 eyes with PCV that completed 1-year IVBr treatment. Blood flow signals within polypoidal lesions were evaluated using OCTA after loading-phase treatment, and 1-year outcomes were compared between eyes in which blood flow signals disappeared versus persisting. After loading-phase treatment, blood flow signals within polypoidal lesions disappeared in 31 eyes and persisted in 15. In the former group, visual acuity improved significantly throughout the year (P < 0.01), while in the latter there was no significant difference between baseline and after 1 year. The total number of injections was significantly lower with than without disappearance of blood flow signals (6.0 vs. 6.9, P < 0.01). The intended injection interval at the last visit was significantly longer in the former than in the latter group (15.7 weeks vs. 12.5 weeks, P < 0.01). These results indicate that PCV cases showing disappearance of blood flow signals within polypoidal lesions by OCTA after loading-phase treatment had favorable 1-year outcomes of IVBr. Therefore, evaluating blood flow within polypoidal lesions by OCTA may allow noninvasive prediction of PCV treatment outcomes.

One-year results of treat-and-extend regimen with intravitreal faricimab for treatment-naïve neovascular age-related macular degeneration.

PURPOSE: To evaluate 1-year outcomes of loading phase treatment followed by maintenance therapy using a treat-and-extend (TAE) regimen with intravitreal faricimab for neovascular age-related macular degeneration (nAMD). STUDY DESIGN: Retrospective, interventional case series. METHODS: We retrospectively studied 40 eyes of 38 consecutive patients with treatment-naïve nAMD, assessing best-corrected visual acuity (BCVA), foveal thickness, central choroidal thickness (CCT), total number of injections over 1 year, and intended injection interval at the last visit. RESULTS: Thirty eyes (75.0%) had completed the 1-year intravitreal faricimab treatment. Their BCVA showed significant improvement, with significant reductions in foveal thickness and CCT. The total number of injections during the 1-year treatment period was 6.6 ± 0.7. The intended injection interval at the last visit was 12.7 ± 3.3 weeks. Of the 10 eyes (25.0%) failing to complete the 1-year faricimab treatment, 1 eye developed intraocular inflammation after the loading phase treatment but showed no recurrence of exudative changes, and no further treatment was required. Moreover, 5 eyes switched to intravitreal brolucizumab injection due to persistent exudative changes with an 8-week interval of faricimab injections. The remaining 4 eyes either dropped out or the patient died. CONCLUSIONS: A loading phase treatment followed by a TAE regimen with intravitreal faricimab appears to be generally safe and effective for improving visual acuity and ameliorating exudative changes in eyes with nAMD. However, there might be cases in which exudative changes cannot be adequately controlled with injections of faricimab every 8 weeks in the maintenance phase.

Attenuation of irradiated choroid and its regional vortex veins in central serous chorioretinopathy after photodynamic therapy.

We retrospectively studied 12 eyes of 12 patients with central serous chorioretinopathy (CSC) to investigate choroidal thickness changes following half-fluence photodynamic therapy (PDT) using widefield choroidal thickness maps obtained by optical coherence tomography (OCT). Additionally, we assessed the relationship between choroidal thickness changes and the regional vortex veins as visualized on widefield en face OCT of the choroid. Pre-treatment en face images of the choroidal vasculature were superimposed on subtracted choroidal thickness maps before and 3 months after half-fluence PDT. The choroidal thickness decreased mainly in the irradiated macular area and in the region of vortex veins which function as drainage for the macula in all eyes. Eleven eyes (91.7%) showed choroidal thinning in the nasal area which overlapped with the nasal vortex vein distribution. Moreover, in 10 (90.9%) of those eyes, we observed intervortex venous anastomosis across the vertical watershed zone. Quantitative analysis revealed that the reduction in choroidal thickness was most pronounced in the macular area. Furthermore, the choroidal thickness reduction in the area with macular drainage vortex veins was significantly greater than that in the area without such vortex veins. These results suggest that half-fluence PDT might decrease choroidal thickness due to choriocapillaris occlusion in the irradiated macula, possibly leading to diminished venous drainage from the macula to regional vortex veins. Moreover, venous blood flow through the anastomotic vessels from the macular drainage vortex veins into the nasal vortex veins might be reduced post-treatment.

Short-term outcomes of intravitreal faricimab for treatment-naïve neovascular age-related macular degeneration.

PURPOSE: To investigate the efficacy and safety of loading phase treatment with 3 monthly intravitreal injections of faricimab for neovascular age-related macular degeneration (nAMD). METHODS: We retrospectively analyzed 16-week outcomes of 40 consecutive eyes of 38 patients with treatment-naïve nAMD. Three monthly injections of faricimab were administered to all eyes as a loading phase treatment. Best-corrected visual acuity (BCVA), foveal thickness, central choroidal thickness (CCT), and dry macula achievement were all assessed every 4 weeks. Moreover, the regression of polypoidal lesions was evaluated after the loading phase. RESULTS: BCVA was 0.33 ± 0.41 at baseline and showed significant improvement to 0.22 ± 0.36 at week 16 (P < 0.01). Foveal thickness was 278 ± 116 µm at baseline, decreasing significantly to 173 ± 48 µm at week 16 (P < 0.01). CCT was 214 ± 98 µm at baseline, decreasing significantly to 192 ± 89 µm at week 16 (P < 0.01). Dry macula was achieved in 31 eyes (79.5%) at week 16. Indocyanine green angiography after the loading phase revealed complete regression of polypoidal lesions in 11 of 18 eyes (61.1%) with polypoidal lesions. One eye (2.5%) developed vitritis without visual loss at week 16. CONCLUSION: Loading phase treatment with intravitreal faricimab appears to generally be safe and effective for improving visual acuity and reducing exudative changes in eyes with nAMD.

Quadrant laser photocoagulation trial to ameliorate choroidal congestion in central serous chorioretinopathy.

PURPOSE: To investigate the efficacy and safety of quadrant laser photocoagulation to ameliorate the choroidal congestion in central serous choroidopathy (CSC). STUDY DESIGN: Historically controlled study. METHODS: We prospectively studied 20 eyes with acute CSC in the quadrant laser group, in which laser photocoagulation was applied to the macular leakage point(s) as well as the quadrant of the fundus showing vortex vein dilatation. Central choroidal thickness (CCT), vertical diameter of dilated vortex vein, resolution rate of serous retinal detachment (SRD), and visual field were evaluated post-treatment. We also compared the results with those of 18 retrospectively analyzed eyes with acute CSC in an external control group, in which laser photocoagulation had been applied only to the macular leakage point(s). RESULTS: In the quadrant laser group, 2 eyes were excluded from data analysis due to choroidal neovascularization (CNV). CCT was significantly reduced in both groups, but more significantly in the quadrant laser group. The vertical diameter of the dilated vortex vein was significantly decreased only in the quadrant laser group. The resolution rate of SRD was similar in the two groups. In the quadrant laser group, 8 eyes (44.4%) showed mild deterioration of the visual field, consistent with the area subjected to quadrant laser photocoagulation. CONCLUSION: Quadrant laser photocoagulation can have limited efficacy for ameliorating vortex vein congestion in CSC. When laser photocoagulation to the macular area is combined with quadrant laser photocoagulation, attention must be paid to the possible development of CNV and visual field deterioration.

Two-year outcomes of treat-and-extend regimen with intravitreal brolucizumab for treatment-naïve neovascular age-related macular degeneration with type 1 macular neovascularization.

We previously reported one-year results of a treat-and-extend (TAE) regimen with intravitreal brolucizumab for 68 eyes with treatment-naïve neovascular age-related macular degeneration (nAMD) associated with type 1 macular neovascularization (MNV). In the current study, we evaluated second-year results of the brolucizumab TAE therapy in 45 eyes with type 1 MNV that had completed the first-year treatment. Forty-three eyes (95.6%) received brolucizumab TAE treatment during a period of 96 weeks. The significant improvement of best-corrected visual acuity in the first year was maintained in the second year. Moreover, the significant foveal thickness and central choroidal thickness reductions in the first year were maintained in the second year. The total number of injections over the 96-week study period was 10.0 ± 1.4, with 6.4 ± 0.6 in the first year and 3.6 ± 1.0 in the second year. The intended injection interval at week 96 was 8 weeks in 9 eyes (20.9%), 12 weeks in 3 eyes (7.0%), and 16 weeks in 31 eyes (72.1%), with an average injection interval of 14.0 ± 3.3 weeks. No eyes developed brolucizumab-related intraocular inflammation (IOI) during the second-year treatment. These results indicate that the TAE regimen with intravitreal brolucizumab for treatment-naïve nAMD associated with type 1 MNV effectively maintained the improved visual acuity and the diminished exudative changes in the second year. Moreover, intravitreal brolucizumab has the potential to reduce the treatment burden of nAMD. The risk of developing brolucizumab-related IOI appeared to be very low during the second year of this TAE regimen.

Comparison of the 2-Year Results of Photodynamic Therapy with Aflibercept and Aflibercept Monotherapy for Polypoidal Choroidal Vasculopathy.

Purpose: To compare the efficacies of photodynamic therapy (PDT) combined with intravitreal aflibercept (IVA) injections and IVA monotherapy using a treat-and-extend regimen (TAE) for treatment-naïve polypoidal choroidal vasculopathy (PCV). Patients and Methods: One hundred and nine eyes treated with PDT combined with IVA (PDT+IVA group: 51 eyes) or IVA monotherapy (IVA group: 58 eyes) were assessed for 2 years. The main outcome measures included best-corrected visual acuity (BCVA), central macular thickness (CMT), central choroidal thickness (CCT), number of IVA injections, and macular atrophy (MA). Polypoidal lesions before and after the loading phase were assessed using indocyanine green angiography. Results: In both groups, BCVA significantly improved after the loading phase and was maintained for 2 years. CMT and CCT were significantly reduced in both groups, without significant differences after 2 years between the groups (P=0.2708). The mean number of IVA injections in the IVA and PDT+IVA groups during the 2 years were 13.2±3.3 and 12.7±1.8, respectively, without a significant difference (P=0.06). The frequencies of MA expansion in the IVA and PDT+IVA groups during the 2 years were 25.9% and 33.4%, respectively, with no significant difference in the incidence (odds ratio: 1.40, P=0.4253). The ratios of polyp regression after the loading phase in the IVA and PDT+IVA groups were 55.2% and 94.1%, respectively, with a significant difference (P<0.0001). Conclusion: PDT combined with IVA injections using a TAE regimen is effective for anatomical and visual function improvement, without a significant difference as compared to IVA monotherapy. It can facilitate complete regression of polyps with higher odds.

Vortex vein congestion in the monkey eye: A possible animal model of pachychoroid.

PURPOSE: To create vortex vein congestion in the monkey eye as a possible pachychoroid model. METHODS: We ligated superotemporal and inferotemporal vortex veins at the surface of the sclera in monkey eyes. Optical coherence tomography (OCT) and indocyanine green angiography (ICGA) were performed before and 2, 7, and 28 days after the vortex vein ligations to investigate changes in vortex vein morphology and alterations in choroidal blood flow. RESULTS: Before the vortex vein ligations, en face OCT and ICGA images showed well organized vortex veins as well as horizontal and vertical watershed zones. Two days after the vortex vein ligations, dilatation of the superotemporal and inferotemporal vortex veins as well as intervortex venous anastomoses were seen on en face OCT and ICGA images. B-mode OCT images showed choroidal thickening associated with dilatation of the outer choroidal vessels. Moreover, video ICGA revealed choriocapillaris filling delay and pulsatile flow in the dilated vortex veins. At 7 and 28 days after we ligated the vortex veins, these findings were reduced, except for the intervortex venous anastomoses. CONCLUSIONS: We created a monkey model of vortex vein congestion by ligating two vortex veins. This animal model demonstrated pachychoroid-related findings, indicating that vortex vein congestion is involved in the pathogenesis of pachychoroid. However, remodeling of the choroidal drainage route via intervortex venous anastomosis appeared to compensate for the vortex vein congestion created in this model.

Intravitreal Aflibercept versus Brolucizumab for Treatment-Naive Neovascular Age-Related Macular Degeneration with Type 1 Macular Neovascularization: Comparison of Short-Term Outcomes.

INTRODUCTION: The purpose of this study was to evaluate the outcomes of loading-phase treatment with intravitreal aflibercept (IVA) or brolucizumab (IVBr) for treatment-naïve neovascular age-related macular degeneration (nAMD) associated with type 1 macular neovascularization (MNV). METHODS: We retrospectively studied 108 consecutive eyes undergoing IVA and 103 consecutive eyes administered IVBr. Best-corrected visual acuity (BCVA), central macular thickness (CMT), central choroidal thickness (CCT), dry macula achievement, polypoidal lesion regression, and development of intraocular inflammation (IOI) were all assessed. RESULTS: During the loading-phase treatment, IOI was detected in 18 eyes (17.5%) in the IVBr but none of those in the IVA group (p < 0.01). The loading-phase treatment was completed in 101 eyes in the IVA and 85 eyes in the IVBr group. In those cases, BCVA improved significantly, and CMT and CCT showed significant reductions after the loading-phase treatment in both groups (all p < 0.01). The CCT reduction was significantly greater with IVBr than with IVA (32 ± 28 µm vs. 40 ± 25 µm, p < 0.01). The dry macula achievement rate was significantly higher in the IVBr than in the IVA group (71.3 vs. 85.9%, p < 0.05). We observed complete regression of polypoidal lesions significantly more frequently with IVBr than with IVA (47.5 vs. 77.3%, p < 0.01). DISCUSSION/CONCLUSION: Loading-phase treatment with IVA or IVBr for treatment-naïve nAMD with type 1 MNV effectively improved BCVA and diminished exudative changes. The CCT reduction, dry macula achievement, and polypoidal lesion regression rates were all significantly greater in the IVBr than in the IVA group. The incidence of IOI was significantly higher with IVBr than with IVA.

One-year results of treat-and-extend regimen with intravitreal brolucizumab for treatment-naïve neovascular age-related macular degeneration with type 1 macular neovascularization.

We evaluated 1-year outcomes of loading phase treatment followed by maintenance treatment using a treat-and-extend (TAE) regimen with intravitreal brolucizumab for neovascular age-related macular degeneration (nAMD) associated with type 1 macular neovascularization (MNV). We analyzed 68 eyes of 65 consecutive patients with treatment-naïve nAMD associated with type 1 MNV. Forty-five eyes (66.2%) completed the 1-year treatment with intravitreal brolucizumab. In those cases, best-corrected visual acuity (BCVA) showed significant improvement, while there were significant reductions in foveal thickness and central choroidal thickness, after the initial brolucizumab injection, which were maintained until the last visit. The average total number of injections over 1 year was 6.4 ± 0.6. The average intended injection interval at the last visit was 14.0 ± 2.9 weeks. Moreover, 17of 23 eyes (73.9%) with polypoidal lesions showed complete regression of these lesions after the loading phase treatment. Although intraocular inflammation (IOI) was observed in 15 of 68 eyes (22.1%) within 1 year, amelioration in response to combination therapy with topical and subtenon injection of steroids, without visual decline, was obtained. These results indicate that loading phase treatment followed by the TAE regimen with intravitreal brolucizumab might improve BCVA and ameliorate exudative changes in eyes with treatment-naïve nAMD associated with type 1 MNV. Moreover, intravitreal brolucizumab can potentially reduce the treatment burden of nAMD. Prompt steroid therapy might be efficacious for ameliorating brolucizumab-related IOI without visual decline.

Clinical characteristics and pachychoroid incidence in Japanese patients with neovascular age-related macular degeneration.

The phenotypes of neovascular age-related macular degeneration (nAMD) are recognized as differing between Caucasian and Asian patients. Pachychoroid is thought to be more prevalent in Asians than in Caucasians, and may be involved in the development of nAMD in Asian patients. Therefore, we investigated the clinical characteristics and pachychoroid incidence in Japanese patients with nAMD. We retrospectively analyzed 385 eyes of 370 consecutive Japanese patients with treatment naïve nAMD. According to the nAMD nomenclature, type 1 macular neovascularization (MNV) was observed in 132 eyes (34.3%), polypoidal choroidal vasculopathy (PCV) in 137 (35.6%), mixed type 1 and type 2 MNV in 32 (8.3%), type 2 MNV in 43 (11.2%), and type 3 MNV in 41 (10.6%). Pachychoroid was seen in 58.3% of type 1 MNV, 75.2% of PCV, 34.4% of mixed type 1 and type 2 MNV, 14.0% of type 2 MNV, and 0% of type 3 MNV. Compared to nAMD patients without pachychoroid (188 eyes), those who had nAMD with pachychoroid (197 eyes) were significantly younger, had a higher proportion of males, greater central choroidal thickness, and a higher frequency of macular vortex vein anastomoses (all P < 0.001). Furthermore, drusen subtypes differed significantly between the two groups (P < 0.001). These results suggest that most Japanese nAMD patients might have type 1 MNV or PCV. Moreover, in approximately half of patients, nAMD might be associated with pachychoroid, and choroidal congestion may be involved in the development of MNV in these cases.

Chronic choriocapillaris ischemia in dilated vortex vein region in pachychoroid neovasculopathy.

We evaluated choroidal congestion using multimodal imaging in pachychoroid neovasculopathy (PNV). In a retrospective case series of 100 eyes of 99 treatment-naïve PNV patients, their clinical records were reviewed and the corresponding multimodal imaging studies were analyzed. We assessed areas of choriocapillaris filling delay which overlapped with dilated outer choroidal vessels, choroidal neovascularization (CNV), and retinal pigment epithelium (RPE) atrophy. The study subjects were 78 men (78.8%) and 21 women (21.2%). The mean patient age was 67.5 ± 10.5 years. On indocyanine green angiography, all eyes showed choriocapillaris filling delay in the early phase. Dilated outer choroidal vessels were demonstrated in all eyes by en face optical coherence tomography. The areas of choriocapillaris filling delay overlapped extensively with that of dilated outer choroidal vessels. All eyes showed CNV localized within the sites of choriocapillaris filling delay. RPE atrophy was noted in 71 eyes (71.0%), and 68 of these (95.8%) had RPE atrophy within the areas showing choriocapillaris filling delay. These findings indicate that chronic choriocapillaris ischemia secondary to vortex vein congestion may lead to CNV development as well as RPE atrophy in eyes with PNV.

Pulsation of anastomotic vortex veins in pachychoroid spectrum diseases.

Accumulating evidence points to pachychoroid possibly being caused by vortex vein congestion which results in remodeling of choroidal drainage routes via intervortex vein anastomosis. This hypothesis prompted us to investigate vortex vein hemodynamics by studying videos of indocyanine green angiography (ICGA) in a retrospective case series of 295 eyes with pachychoroid spectrum diseases. In the early phase of the video-ICGA, pulsatile vortex venous flow was observed in 76 eyes (25.8%) at the vortex veins connected with anastomosis between superior and inferior vortex veins. The patients with pulsatile vortex venous flow were significantly older than those without pulsatile vortex venous flow (67.8 ± 13.2 vs. 63.9 ± 14.5 years, P < 0.05). Pulsatile vortex venous flow was 1.84 times more common in the inferior quadrants than in the superior quadrants. Interestingly, 14 of 76 eyes (18.4%) with pulsatile vortex venous flow showed retrograde pulsatile blood flow in the vortex veins. This retrograde pulsatile blood flow was 2.50 times more common in the inferior than in the superior quadrants. These findings indicate altered vortex vein hemodynamics due to vortex vein congestion in pachychoroid spectrum diseases.

Short-term outcomes of intravitreal brolucizumab for treatment-naïve neovascular age-related macular degeneration with type 1 choroidal neovascularization including polypoidal choroidal vasculopathy.

We evaluated the efficacy and safety of loading phase treatment with intravitreal brolucizumab for neovascular age-related macular degeneration (nAMD) with type 1 choroidal neovascularization (CNV). We analyzed consecutive 42 eyes of 40 patients with treatment-naïve nAMD associated with type 1 CNV. Three monthly injections of brolucizumab were completed in 36 eyes (85.7%). In those cases, best-corrected visual acuity (BCVA) was 0.24 ± 0.27 at baseline and improved significantly to 0.12 ± 0.23 after 3 months (P < 0.001). Central macular thickness was 301 ± 110 µm at baseline and decreased significantly to 160 ± 49 µm after 3 months (P < 0.001). Dry macula was achieved in 34 eyes (94.4%) after the loading phase. Central choroidal thickness was 264 ± 89 µm at baseline and decreased significantly to 223 ± 81 µm after 3 months (P < 0.001). Indocyanine green angiography after the loading phase revealed complete regression of polypoidal lesions in 15 of the 19 eyes (78.9%) with polypoidal lesions. Non-infectious intraocular inflammation (IOI) was observed in 8 of 42 eyes (19.0%) during the loading phase, while showing amelioration in response to combination therapy with topical and subtenon injection of steroids. In these eyes, BCVA after 3 months had not deteriorated as compared to that at baseline. These results indicate that loading phase treatment with intravitreal brolucizumab might be effective for improving visual acuity and reducing exudative changes in eyes with nAMD associated with type 1 CNV. Moreover, polypoidal lesions appear to frequently regress after this treatment. However, we must monitor patients carefully for brolucizumab-related IOI, and administer steroid therapy promptly.

Variation of vortex veins at the horizontal watershed in normal eyes.

PURPOSE: To assess the vascular pattern of choroidal vortex veins at the horizontal watershed zone in normal eyes using optical coherence tomography (OCT). METHODS: We retrospectively studied 207 normal eyes of 207 patients whose fellow eyes were diagnosed with unilateral retinal diseases without choroidal involvement. Venous anastomosis between the superior and inferior vortex veins and deviation of the horizontal watershed zone were evaluated using 12 × 12-mm en face OCT images. Central choroidal thickness (CCT) was measured on B-mode OCT images. RESULTS: Vortex vein anastomosis was observed in 92 eyes (44.4%) at the horizontal watershed zone. Superior or inferior deviation of the horizontal watershed was ascertained in 69 eyes (33.3%). The frequency of the anastomosis and deviation did not differ significantly between age groups (P = 0.56 and 0.96, respectively). Mean CCT of all eyes was 221 ± 80 µm. CCT was significantly greater in eyes with anastomosis than in those without (233 ± 73 µm vs 210 ± 83 µm, P < 0.05). However, CCT did not differ significantly between eyes with and without deviation of the horizontal watershed zone (223 ± 74 µm vs 219 ± 82 µm). CONCLUSIONS: Venous anastomosis at the horizontal watershed zone as well as superior or inferior deviation of the zone were frequently observed in normal eyes. CCT was greater in eyes with than in those without anastomosis, suggesting subclinical vortex vein congestion.

Choroidal congestion mouse model: Could it serve as a pachychoroid model?

Pachychoroid spectrum diseases have been described as a new clinical entity within the spectrum of macular disorders. "Pachychoroid" is defined as choroidal thickening associated with dilated outer choroidal vessels often showing retinal pigment epithelium (RPE) degeneration. Although various clinical studies on the pachychoroid spectrum diseases have been conducted, the pathophysiology of pachychoroid has yet to be fully elucidated. In this study, we attempted to establish a mouse model of pachychoroid. We sutured vortex veins in eyes of wild type mice to imitate the vortex vein congestion in pachychoroid spectrum diseases. Fundus photography and ultra-widefield indocyanine green angiography showed dilated vortex veins from the posterior pole to the ampulla in eyes after induction of choroidal congestion. Optical coherence tomography and tissue sections presented choroidal thickening with dilatation of choroidal vessels. The RPE-choroid/retina thickness ratios on the tissue sections in the treated day 1 and day 7 groups were significantly greater than that in the control group (0.19±0.03 and 0.16±0.01 vs. 0.12±0.02, P<0.05 each). Moreover, immunohistochemistry using RPE flatmount revealed focal RPE degeneration in the treated eyes. Furthermore, inflammatory response-related genes were upregulated in eyes with choroidal congestion induction, and macrophages migrated into the thickened choroid. These results indicated that vortex vein congestion triggered some pachychoroid features. Thus, we have established a choroidal congestion mouse model by suturing vortex veins, which would potentially be useful for investigating the pathophysiology of pachychoroid spectrum diseases.

Quantitative measures of vortex veins in the posterior pole in eyes with pachychoroid spectrum diseases.

Pachychoroid spectrum diseases have attracted increasing attention, though their pathophysiology has yet to be fully elucidated. In this study, we assessed the vascular diameters of vortex veins in pachychoroid spectrum diseases such as central serous chorioretinopathy (CSC), pachychoroid neovasculopathy without polypoidal lesions (PNV), and pachychoroid neovasculopathy with polypoidal lesions (polypoidal choroidal vasculopathy: PCV). In a retrospective case series of 94 eyes with CSC, 60 eyes with PNV and 57 with PCV, we binarized en face optical coherence tomography (OCT) images of choroidal vortex veins and analyzed the mean diameter of vortex veins. The presence of anastomosis between the superior and inferior vortex veins and central choroidal thickness (CCT) were also evaluated using OCT images. CSC showed significantly larger mean diameter of vortex veins than PCV (P < 0.05). Anastomosis between superior and inferior vortex veins was observed in over 90% of eyes with each pachychoroid spectrum disease. The patients with CSC were the youngest, followed by PNV patients, and then patients with PCV. The largest CCT values were observed in CSC eyes, followed by PNV eyes, and then PCV eyes. CCT correlated with the mean diameter of vortex veins (rs = 0.51, P < 0.01). These findings suggest that congestion of vortex veins might show gradual amelioration corresponding to the development of anastomosis between the superior and inferior vortex veins during the course of progression of pachychoroid spectrum diseases. Moreover, the mean diameter of vortex veins can be used as a parameter indicating choroidal congestion.

Vortex Vein Anastomosis at the Watershed in Pachychoroid Spectrum Diseases.

PURPOSE: To evaluate the vascular changes in vortex veins at the posterior pole in pachychoroid spectrum diseases, including central serous chorioretinopathy (CSC), pachychoroid neovasculopathy without polypoidal lesions (PNV), and pachychoroid neovasculopathy with polypoidal lesions (polypoidal choroidal vasculopathy [PCV]). DESIGN: Retrospective case-control study. PARTICIPANTS: Ninety-two eyes of 89 patients with CSC, 61 eyes of 59 patients with PNV, 63 eyes of 61 patients with PCV, and 25 healthy control eyes of 25 age- and gender-matched participants for each pachychoroid spectrum disease. METHODS: Clinical records of patients with pachychoroid spectrum diseases and healthy controls were reviewed. Multimodal images of each group were analyzed. Swept-source OCT was performed to obtain B-mode and en face images in patients with pachychoroid spectrum diseases and healthy controls. All patients underwent fluorescein angiography, indocyanine green angiography, and OCT. MAIN OUTCOME MEASURES: Vortex vein anastomosis at the watershed, determined using en face OCT, and central choroidal thickness (CCT), measured using B-mode OCT, were examined in patients and healthy controls. Patient ages also were taken into consideration. RESULTS: Patients with CSC were the youngest, followed by patients with PNV, and then those with PCV (P < 0.01, CSC vs. PNV and PNV vs. PCV), whereas CSC eyes showed the highest CCT values, followed by the PNV and then the PCV eyes (P < 0.01, CSC vs. PNV; P < 0.05, PNV vs. PCV). Central choroidal thickness was significantly greater in pachychoroid spectrum diseases than in healthy controls. No significant CCT differences were found among healthy controls. Anastomosis between superior and inferior vortex veins was observed in more than 90% of eyes with pachychoroid spectrum diseases, making this finding significantly more frequent than in healthy controls (P < 0.01, each pachychoroid spectrum disease vs. control). Polypoidal choroidal vasculopathy showed a significantly higher rate of anastomosis than CSC (P < 0.05). CONCLUSIONS: Anastomosis between superior and inferior vortex veins was found to be a common feature in pachychoroid spectrum diseases. Longstanding vortex vein congestion may lead to the development of pachychoroid spectrum diseases. Choroidal congestion may be compensated for by new drainage routes formed via vortex vein anastomosis.

Scedosporium apiospermum infectious scleritis following posterior subtenon triamcinolone acetonide injection: a case report and literature review.

BACKGROUND: Ubiquitous fungi of the Scedosporium apiospermum species complex (SASC) cause various opportunistic infections. Posterior subtenon triamcinolone acetonide (STTA) injection is a standard therapy for intraocular inflammation and macular edema. We report a case of Scedosporium apiospermum infectious scleritis after a posterior STTA injection. CASE PRESENTATION: A 75-year-old man received a posterior STTA injection to treat macular edema in his left eye. After 3 months, he complained of ocular pain and hyperemia in his left eye. Examination showed a subtenon abscess in the site corresponding with the STTA injection. After incising the abscess, the smear revealed numerous conidia-like structures. Although we suspected fungal infection and started topical voriconazole (VRCZ) and levofloxacin, the inflammation of the eye worsened. Fungal culture revealed filamentous fungus growth. Subsequently, we added systemic VRCZ and performed surgical debridement of the infected sclera and Tenon's capsule. Pathology of the sclera showed fungal hyphae. The antifungal susceptibility test revealed low minimum inhibitory concentrations for micafungin, VRCZ and miconazole (0.06, 0.25 and 0.5 µg/mL, respectively). After 2 months, the ciliary injection subsided and VRCZ therapy was stopped. However, subtenon abscess recurred 1 month after discontinuation of topical VRCZ. Surgical debridement and topical VRCZ were resumed, with the eye finally improving after 5 months of management. The fungal species was identified as Scedosporium apiospermum sensu stricto morphologically and by DNA sequencing. CONCLUSIONS: This case was successfully treated by topical and systemic VRCZ and repeated surgical debridement. Infectious scleritis caused by SASC rarely develops after posterior STTA. SASC can produce conidia in the enclosed subtenon space. Late-onset infectious scleritis after a posterior STTA injection suggests the presence of a fungal infection, including SASC, thereby requiring extensive and prolonged medical and surgical treatment.