Pachychoroid Spectrum Disease Research Articles

Characteristics and Classification of Choroidal Caverns in Patients with Various Retinal and Chorioretinal Diseases

To investigate the features of choroidal caverns in diverse retinal diseases with swept-source optical coherence tomography (SS-OCT). Subjects with normal eyes, retinitis pigmentosa (RP), wet age-related macular degeneration (wAMD), acute central serous chorioretinopathy (CSC), or chronic CSC were enrolled. The characteristics of choroidal caverns were evaluated with SS-OCT. The prevalence of choroidal caverns in retinal diseases and the correlations between the number, width and depth of choroidal caverns with the thickness of choroid were analyzed. Among 315 eyes of 220 subjects, choroidal caverns were found in 110 eyes (34.9%). Choroidal caverns were divided into two categories based on their location and size. Type I was small and usually lobulated, presented in the choroidal capillary and Sattler's layers. Type II was larger, usually isolated, and presented in the Sattler's and Haller's layers. The prevalence of type I in subjects with normal eyes, RP, wAMD, acute CSC, or chronic CSC was 17.4%, 19.6%, 1.6%, 32.8%, and 85.2%, respectively, while that of type II was 0%, 0%, 21.3%, 13.8%, and 53.7%, respectively. The number, width, and thickness of type II choroidal caverns correlated positively with macular choroidal thickness. Choroidal caverns could be divided into two categories. Type II choroidal caverns appeared associated with the pachychoroid spectrum and RPE atrophic diseases.

Three-dimensional choroidal vascularity index in central serous chorioretinopathy using ultra-widefield swept-source optical coherence tomography angiography.

BackgroundTo map and compare the three-dimensional choroidal vascularity index (3D-CVI) in eyes with unilateral central serous chorioretinopathy (CSC), fellow eyes and control eyes using ultra-widefield swept source optical coherence tomography (UWF SS-OCTA).MethodsIn this prospective observational study, the 3D-CVIs were measured in 9 subfields or 1 × 1 mm grids by the UWF SS-OCTA with a viewing angle of horizontal 24 × vertical 20 mm. The proportions of vortex vein anastomoses and their corresponding CVI in the central regions were compared among the CSC, fellow and control eyes. Correlations of CVI and vascular density of the large choroidal vessel layer/choriocapillaris layer/choroidal thickness (CT) were also assessed.ResultsThirty-two eyes in 32 patients with unilateral CSC and 32 normal eyes were included in the study. The mean CVI in the eyes with CSC was significantly greater than that in the fellow eyes of CSC and control eyes (41.99 ± 3.56% vs. 40.38 ± 3.855%, P = 0.003; 41.99 ± 3.56% vs. 38.93 ± 4.067%, P = 0.004, respectively). The CVIs in superotemporal, inferotemporal and inferonasal regions were significantly higher in CSC eyes than control eyes (P = 0.03, P = 0.02, P = 0.008). In CSC eyes, there was a linear positive correlation between 3D-CVI and vascular density of the large choroidal vessel layer and CT in all subfields. The proportion of vortex vein anastomoses in CSC was 25/32 (78.1%), and significantly higher in fellow and control eyes (P < 0.001). The average central CVI was significantly higher in CSC eyes with anastomoses than in CSC eyes without anastomoses (42.8 ± 5.1% vs. 38.4 ± 2.7%, P = 0.039). CVIs in superior, central, inferior, superonasal, nasal and inferonasal regions were significantly correlated with vortex vein anastomoses (P < 0.05), regardless of CSC, fellow or healthy eyes. In addition, whether there were vortex vein anastomoses, CVI in superotemporal region was significantly higher in eyes with CSC (P = 0.002) and fellow eyes (P = 0.014), compared to control eyes. No significant correlation was found between hypertension and CVIs in the three groups.ConclusionRemodeling of choroidal drainage routes by venous anastomosis between superior and inferior vortex veins may be common in CSC. The 3D-CVI could be a comprehensive parameter to evaluate the choroid vasculature and help understand the pathogenesis of pachychoroid spectrum disease.

Hydrodynamic Analysis of the Clinical Findings in Pachychoroid-Spectrum Diseases.

: PurposeWe wish to demonstrate that theorems of fluid dynamics may be employed to hydrodynamically analyze the clinical presentations seen within the pachychoroid-spectrum diseases (PSD). Methods: We employed both the Equation of Continuity Q = A · V in which Q represents blood flow volume, A the sectional area of a vessel, and V blood flow velocity as well as Bernoulli’s Principle 1/2 V2 + P/ρ = constant where V represents blood flow velocity, P static blood pressure and ρ blood density. The Equation of Continuity states that a decrease in flow volume occurs simultaneously with a decrease in the flow velocity and/or sectional area, and vice versa. Bernoulli’s Principle states that a decrease in the velocity of a fluid occurs simultaneously with an increase in static pressure, and vice versa. Results: Hyperpermeability of the choriocapillaris, as visualized on fluorescein angiography and indocyanine green angiography (ICGA), causes a fluid exudation and, therefore, a decrease in the blood flow volume Q which elicits a simultaneous decrease in the blood flow velocity V clinically observable in filling delay into the choriocapillaris on ICGA. An increase in the static blood pressure P will simultaneously occur in venules in accord with Bernoulli’s Principle. Conclusions: A decrease in the blood flow velocity in the choriocapillaris due to its hyperpermeability will hydrodynamically elicit an increase in the blood pressure in venules. This blood pressure rise may expand Sattler and Haller veins, forming pachyveins. The primary lesion of PSD can be in pigment epithelium and choriocapillaris.

A Comparative Study of Short-Term Vascular and Stromal Alterations of the Choroid Following Half-Fluence Photodynamic Therapy in Pachychoroid Neovasculopathy and Chronic Central Serous Chorioretinopathy.

Background: Central serous chorioretinopathy (CSCR) and pachychoroid neovasculopathy (PNV) are among the pachychoroid spectrum diseases (PSDs). Half-fluence photodynamic therapy (hf-PDT) is one of the effective treatment methods for both diseases. The aim of this study was to compare the effect of hf-PDT on the choroidal structure in CSCR and PNV. Methods: This study included 35 patients with chronic CSCR and 18 patients with PNV. The hf-PDT protocol was applied to all eyes. Before and 3 months after hf-PDT, enhanced-depth optical coherence tomography images were analyzed. The total choroidal area (CA), luminal area (LA), and stromal area (SA) were measured using ImageJ software. Results: Compared with baseline values, 3 months after hf-PDT, the mean CA reduced from 1.398 to 1.197 mm2 (p < 0.001) in the CSCR group and the total CA reduced from 1.050 to 1.000 mm2 (p < 0.021) in the PNV group. The mean percentage changes in CA, LA, and SA values were statistically higher in the chronic CSCR group (13.86%, 13.53%, and 14.11%, respectively) than those in the PNV group (4.61%, 4.02%, and 5.74%; p = 0.001, p = 0.002, and p = 0.031, respectively). Conclusion: CSCR and PNV are thought to be PSDs. However, they differ in choroidal morphological response after hf-PDT, which might be a result of the different structural components of the PNV lesions.

Pachychoroid Spectrum Diseases in Patients with Cushing's Syndrome: A Systematic Review with Meta-Analyses.

Cushing’s syndrome is a rare disease with an endogenous cause of excess cortisol secretion. More evidence substantially links cortisol levels to the pachychoroid spectrum diseases. In this systematic review and meta-analysis, we summarize available evidence on pachychoroid spectrum diseases in patients with Cushing’s syndrome. We performed a systematic literature search in 11 databases on 21 May 2022. Studies were considered eligible if they performed retinal examination of a consecutive group of patients with Cushing’s syndrome using optical coherence tomography (OCT) scans. We extracted data on subfoveal choroidal thickness in patients with Cushing’s syndrome compared to matched controls. We also extracted data on the prevalence of pachychoroid pigment epitheliopathy (PPE), central serous chorioretinopathy (CSC), and polypoidal choroidal vasculopathy (PCV). We identified six eligible studies with a total of 159 patients with Cushing’s syndrome. On average, patients with Cushing’s syndrome have 49.5 µm thicker subfoveal choroidal thickness compared to matched healthy individuals. Pachychoroid spectrum diseases were relatively common in these patients: PPE in 20.8%, CSC in 7.7%, and PCV in 2.8%. We conclude that there should be low threshold to recommend ophthalmic examination to patients with Cushing’s syndrome, and that a macular OCT is recommended during this examination.

Imaging Characteristics of Neovascular and Atrophic Pachychoroidal Spectrum Diseases.

BackgroundThis study qualitatively and quantitatively compared imaging characteristics between neovascular and atrophic pachychoroid spectrum disease (PSD) by optical coherence tomography (OCT), and OCT angiography (OCTA).MethodsThe subtypes of PSD were identified by multi-modality imaging approaches. Subfoveal choroidal thickness (SFCT), choroidal vascular index (CVI), and vascular density of choroidal neovascularization (CNV) were measured.ResultsThe CVI and SFCT of 174 PSD eyes were 67.6% ± 5.48% and 362.2 ± 131.88 μm, respectively. After adjustment for age, linear regression indicated that SFCT was positively associated with CVI (p < 0.001), and patched hyper-reflective lesions in choriocapillaris layers (p = 0.009). Compared with neovascular PSD eyes, atrophic PSD eyes had similar patient age (57.1 ± 16.72 years, p = 0.639), SFCT (332.0 ± 111.00 μm, p = 0.51), and CVI (67.6% ± 3.94%, p = 0.527). There were no differences between polypoidal choroidal vasculopathy (PCV) eyes with aneurysmal polypoidal lesions and PCV eyes with tangled polypoidal lesions in terms of age, CVI, SFCT, vascular density, or the occurrence of double layer signs (DLSs, all p > 0.05). Logistic regression indicated that age (p = 0.003), SFCT (p = 0.003), patched hyper-reflective lesions in choriocapillaris layers (p = 0.009), and DLSs (p < 0.001) were predictive factors for CNV progression in PSD eyes (all p < 0.05).ConclusionsOur study highlighted the similarities in SFCT and CVI between neovascular and atrophic PSD, both of which were late stage lesions. Besides, age, SFCT, patched hyper-reflective lesions in choriocapillaris layers, and DLSs were risk factors for CNV in PSD. Our results showed that atrophic PSD is an important change in the late stage of PSD disease, which is helpful for in-depth understanding of the pathological mechanism of PSD and corresponding intervention.

Case series: pachychoroid pigment epitheliopathy transformed to polypoidal choroidal vasculopathy after long-term follow-up

BackgroundPachychoroid pigment epitheliopathy (PPE), a retinal disorder that falls into the pachychoroid spectrum, is characterized by retinal pigment epithelium changes in pachychoroid eyes without existing or previous subretinal fluid or soft drusen. Previous reports have indicated that PPE may share some pathophysiologic component with other pachychoroid spectrum diseases and could transform into central serous chorioretinopathy (CSC) during follow-up. CSC transformation to PNV and PCV has also been reported, but PPE transformation to PCV has not been reported. Case presentationSeven eyes of seven patients (four male three female, aged 62.7 ± 8.4 years) who presented with PPE at baseline transformed to PCV during follow-up. All study eyes had baseline contralateral eye diagnoses of PCV. All PPE eyes reported no symptoms at baseline and were followed up regularly for the treatment of their contralateral eyes. All PPE presented as pigment epithelium detachment (PED) at baseline. The mean central macular thickness (CMT) was 217.6 ± 14.6 µm, the mean subfoveal choroidal thickness (SFCT) was 354.9 ± 94.9 µm, and the mean sub-PPE choroidal thickness was 332.3 ± 84.6 µm. The mean PPE width and height were 1326.4 ± 791.4 µm and 58.7 ± 23.6 µm, respectively, at baseline. Disruption of the ellipsoid zone (EZ) was noted in 3 eyes, while choroidal vascular hyperpermeability (CVH) was noted in 5 eyes at baseline. The follow-up period was 75.0 ± 41.1 months, and the mean transformation time was 49.6 ± 24.8 months. All study eyes received no intervention before transformation.ConclusionsPPE could transform to PCV after a long follow-up period. Regular follow-ups for a long time should be recommended for patients with PPE.

Visualization of Type-1 Macular Neovascularization Secondary to Pachychoroid Spectrum Diseases: A Comparative Study for Sensitivity and Specificity of Indocyanine Green Angiography and Optical Coherence Tomography Angiography.

Background: The aim of this study was to compare optical coherence tomography angiography (OCTA) and indocyanine green angiography (ICGA) in detecting type-1 macular neovascularization (MNV) in pachychoroid spectrum diseases. Methods: Patients with pachychoroid characteristics who had undergone ICGA and OCTA imaging at the same visit, were recruited. The diagnosis of MNV was made by a senior retina specialist using multimodal imaging techniques. Afterward, both ICGA and OCTA images were separately reviewed by a masked-independent senior retina specialist with regard to the presence of MNV. The specificity, sensitivity, positive, and negative predictive values of ICGA and OCTA were analyzed. Results: OCTA was able to detect MNV with 97.2% sensitivity, failing to detect MNV only in one eye. The sensitivity of ICGA to detect MNV was 66.76%. The negative predictive value of OCTA was 94.7%; however, this value was 60% for ICGA. Multimodal imaging and OCTA were in almost perfect agreement (kappa coefficient = 0.95). Conclusion: OCTA shows greater sensitivity when detecting type-1 MNV than ICGA in pachychoroid neovasculopathy cases. OCTA is a non-invasive and quick imaging modality that can be preferred to dye angiography in the visualization of type-1 MNV in pachychoroid neovasculopathy.

EFFICACY OF HALF-DOSE PHOTODYNAMIC THERAPY VERSUS HIGH-DENSITY SUBTHRESHOLD MICROPULSE LASER FOR TREATING PIGMENT EPITHELIAL DETACHMENTS IN CHRONIC CENTRAL SEROUS CHORIORETINOPATHY.

Comparing the effect of half-dose photodynamic therapy and high-density subthreshold micropulse laser treatment on retinal pigment epithelial detachments (PEDs) in chronic central serous chorioretinopathy. This study included data from the PLACE trial, a prospective randomized controlled trial comparing half-dose photodynamic therapy and high-density subthreshold micropulse laser treatment in chronic central serous chorioretinopathy. Main outcome measurements were changes in both the foveal PED and the highest PED within the macula at baseline compared with first and final evaluation visit. At baseline, a macular PED was detected in 76.9% of patients (123/160), and a PED within 1,500 µm from the foveal center in 37.5% of patients (60/160). In the half-dose photodynamic therapy arm (61 patients), there was a significantly larger decrease in the highest macular PED compared with the high-density subthreshold micropulse laser treatment arm (62 patients) at both first and final evaluation visits (P < 0.001 and P = 0.012, respectively). The decrease of highest foveal PED was significant at first visit (P = 0.025). Half-dose photodynamic therapy is superior to high-density subthreshold micropulse laser treatment with regard to a statistically significant reduction in the height of macular PEDs in active chronic central serous chorioretinopathy. These findings may also have implications for other diseases within the pachychoroid disease spectrum that can present with PEDs.

Choriocapillaris: Fundamentals and advancements.

The choriocapillaris is the innermost structure of the choroid that directly nourishes the retinal pigment epithelium and photoreceptors. This article provides an overview of its hemovasculogenesis development to achieve its final architecture as a lobular vasculature, and also summarizes the current histological and molecular knowledge about choriocapillaris and its dysfunction. After describing the existing state-of-the-art tools to image the choriocapillaris, we report the findings in the choriocapillaris encountered in the most frequent retinochoroidal diseases including vascular diseases, inflammatory diseases, myopia, pachychoroid disease spectrum disorders, and glaucoma. The final section focuses on the development of imaging technology to optimize visualization of the choriocapillaris as well as current treatments of retinochoroidal disorders that specifically target the choriocapillaris. We conclude the article with pertinent unanswered questions and future directions in research for the choriocapillaris.

Choroidal arteriovenous anastomoses: a hypothesis for the pathogenesis of central serous chorioretinopathy and other pachychoroid disease spectrum abnormalities.

The pachychoroid disease spectrum (PDS) includes several chorioretinal diseases that share specific choroidal abnormalities. Although their pathophysiological basis is poorly understood, diseases that are part of the PDS have been hypothesized to be the result of venous congestion. Within the PDS, central serous chorioretinopathy is the most common condition associated with vision loss, due to an accumulation of subretinal fluid in the macula. Central serous chorioretinopathy is characterized by distinct risk factors, most notably a high prevalence in males and exposure to corticosteroids. Interestingly, sex differences and corticosteroids are also strongly associated with specific types of arteriovenous anastomoses in the human body, including dural arteriovenous fistula and surgically created arteriovenous shunts. In this manuscript, we assess the potential of such arteriovenous anastomoses in the choroid as a causal mechanism of the PDS. We propose how this may provide a novel unifying concept on the pathophysiological basis of the PDS, and present cases in which this mechanism may play a role.

Pachychoroid spectrum disease and choriocapillary flow analysis in patients with Cushing disease: an optical coherence tomography angiography study.

To investigate the presence of pachychoroid spectrum disease (PSD) in patients with Cushing disease (CD) and to evaluate subfoveal choroidal thickness (SFCT) and choriocapillary flow using spectral domain OCT (SD-OCT) with the enhanced depth imaging (EDI) and optical coherence tomography angiography (OCT-A). Thirty-two patients with CD and 32 age- and sex-matched healthy volunteers were enrolled in this observational study. All participants had a complete ophthalmic examination including SD-OCT with EDI and OCT-A, and were subjected to the Perceived Stress Scale test (PSS). All patients with CD had hormone test including 24-h urinary-free cortisol (UFC) and plasma adrenocorticotropic hormone (ACTH). We compared SFCT and choriocapillary vessel density (CVD) between the two groups and evaluated the presence of PSD. We investigated the association of hormone level, SFTC, CVD with the presence of CD; the association between the hormone level, SFTC, CVD, the CD disease activity, and duration with the presence of PSD in CD patients; and the association between SFTC and CVD with the hormone level, the CD disease activity, and duration in CD patients. Higher values of SFCT and CVD were associated with CD (β: 0.028, 95% CI: 0.014; 0.041; β: 0.912, 95%CI: 0.205; 1.62, respectively). Twelve patients with CD (37.5%) reported a PSD in at least one eye, whereas no subject was found in control group (p < 0.001); in particular, 11 CD patients (34%) presented pachychoroid pigment epitheliopathy (PPE) and 1 CD patient (3%) presented polypoidal choroidal vasculopathy/aneurysmal type 1 neovascularization (PCV/AT1). In patients with CD, a significant positive association between SFCT and PSD was found (β: 0.010, 95% CI 0.001; 0.019). A chronic state of hypercortisolism may have direct implications on the choroid. Patients with CD had higher SFCT values and a significant change in the choriocapillary flow compared to healthy controls. Moreover, PSD was observed only in CD patients.

Prevalence of focal lamina cribrosa defects in eyes with pachychoroid disease spectrum.

To determine the prevalence of focal lamina cribrosa (LC) defect among patients with pachychoroid disease spectrum (PDS) in the absence of peripapillary retinoschisis. This retrospective, cross-sectional study comprised of 180 patients with PDS, including polypoidal choroidal vasculopathy (PCV), central serous chorioretinopathy, and pachychoroidal neovasculopathy. Medical records and optic nerve head evaluations conducted using spectral-domain optical coherence tomography with enhanced depth imaging were reviewed. As a control group, 236 patients who underwent ophthalmologic evaluation for vitreous floaters, without obvious ocular disease, were also included. The mean age of the PDS group, which included 118 male patients (65.6%), was 57.4±11.1y. There was no significant difference between the two groups in age (P=0.710) or sex (P=0.248). Six patients (3.3%) in the PDS group and none in the control group showed focal LC defect (P=0.318). Among the six patients with focal LC defect in the PDS group, four eyes had PCV, one eye was the fellow eye of a PCV eye, and one eye had pachychoroidal neovasculopathy. Focal LC defect can be defected in patients with PDS in the absence of peripapillary retinoschisis. However, the prevalence of focal LC defect was not different significantly between PDS patients and those who did not have PDS.

PERIPAPILLARY PACHYCHOROID SYNDROME

Available literature on peripapillary pachychoroid syndrome (PPS) is very limited. The purpose of this study is to evaluate features of PPS using multimodal imaging and shed further light on this entity. This is a retrospective case series at a tertiary eye-care center. Patients with features of pachychoroid with preferential choroidal thickening in nasal macula along with pachyvessels were identified and included for analysis. The patients underwent fundus photography, fundus autofluorescence, optical coherence tomography, fundus fluorescein angiography, and indocyanine angiography. Images were separately analyzed by two different retina specialists. A total of 27 eyes of 14 patients with mean age of 52.2 years were included. Seven patients had bilateral PPS, whereas six had unilateral; one patient had only one seeing eye. A total of 21 eyes with PPS were studied. 52.4% of the eyes revealed retinal pigment epithelium gravitational tracks, outer retinal atrophy, and serous PEDs. None of the eyes showed choroidal folds. Fundus fluorescein angiography showed late leakage at macula in 33% eyes. Fundus autofluorescence showed features of PPS to be similar to central serous chorioretinopathy in most cases. Peripapillary pachychoroid syndrome shares common findings with central serous chorioretinopathy and seems to be a subset of central serous chorioretinopathy than a separate entity in pachychoroid disease spectrum.

Central serous chorioretinopathy: Treatment.

Central serous chorioretinopathy (CSC) is a pachychoroid spectrum disease characterized by serous detachment of the neurosensory retina with subretinal fluid in young and middle-aged adults. The pathogenesis of CSC is not yet fully understood. However, it is considered a multifactorial disease that is strongly associated with choroidal dysfunction or vascular engorgement. Although there is no consensus on the treatment of CSC, photodynamic therapy has been effectively used to manage serous retinal detachment (SRD) in CSC. Moreover, micropulse diode laser photocoagulation and focal laser treatment have also been used. Recently, oral medications, including mineralocorticoid receptor antagonists, have been proposed for the management of CSC. Multimodal imaging plays a significant role in the diagnosis and treatment of CSC. Optical coherence tomography angiography (OCTA) has the advantage of detecting vascular flow in the retina and choroid layer, allowing for a better understanding of the pathology, severity, prognosis, and chronicity of CSC. In addition, early detection of choroidal neovascularization in CSC is possible using OCTA. This review article aims to provide a comprehensive and updated understanding of CSC, focusing on treatment.

Imbalanced choroidal circulation in eyes with asymmetric dilated vortex vein.

Asymmetric dilated vortex vein (ADVV) observed in eyes with pachychoroid spectrum diseases is thought to be due to congestion of choroidal blood flow. The purpose of this study was to quantitatively investigate the blood flow velocity of ADVV using laser speckle flowgraphy (LSFG). Retrospective case series. This was a retrospective case series with 23 eyes of 18 patients with ADVV on en-face OCT. A pair of choroidal veins from ADVV side (defined as ADVV vein) and non-ADVV side (defined as non-ADVV vein) was selected in each eye under the following criteria: (i) equivalent proximity to the deviated watershed, (ii) does not overlap with retinal blood vessels in the en-face OCT image, (iii) has approximately the same blood vessel diameter. Rubber bands were placed on the selected choroidal veins on the LSFG color map. Mean blur rate (MBR) values of ADVV and non-ADVV veins were statistically compared. The average MBR was 10.11 ± 1.9 in the ADVV veins and 13.49 ± 6.2 in the non-ADVV veins, showing significantly lower values in the ADVV veins (P = 0.03). The blood vessel diameter of the ADVV was 10.26 ± 3.0 and in the non-ADVV veins, 10.63 ± 2.9 pixels; not significantly different (P = 0.66). The distance from the deviated watershed to the ADVV was 53.3 ± 24.8 and to the non-ADVV veins, 46.80 ± 20.3 pixels; not significantly different (P = 0.41). In eyes with ADVV, the blood flow velocity in the ADVV veins was lower than in the non-ADVV veins, suggesting anatomical congestion of ADVV.

Pachychoroid disease spectrum: review article.

The aim of this article is to do a comprehensive literature review about the current understandings of the pachychoroid disease spectrum, describing its multimodal imaging analysis, pathophysiology, differential diagnosis, and current types of management. This comprehensive literature review was performed based on a search on the PubMed database, of relevant pachychoroid published papers according to our current knowledge. The pachychoroid disease spectrum, according to some authors, includes the following: pachychoroid pigment epitheliopathy (PPE), central serous chorioretinopathy (CSC), pachychoroid neovasculopathy (PNV), polypoidal choroidal vasculopathy (PCV)/aneurysmal type 1 neovascularization (AT1), and more recently focal choroidal excavation (FCE) and peripapillary pachychoroid syndrome (PPS). Each one of these entities will be described and discussed in this article. Significant advances in multimodal imaging have enabled a better understanding of the typical choroidal changes in pachychoroid disease spectrum. The clinical knowledge and managing options about this disease significantly increased in the last years. However, it is still unclear why some eyes with typical pachychoroid disease phenotype show no evidence of RPE damage and subretinal fluid (uncomplicated pachychoroid) while others present progressive tissue damage, neovascularization, and atrophy.

A case of bilateral pachychoroid disease: polypoidal choroidal vasculopathy in one eye and peripheral exudative hemorrhagic chorioretinopathy in contralateral eye

BackgroundWe report a case of bilateral pachychoroid disease manifesting polypoidal choroidal vasculopathy (PCV) with punctate hyperfluorescent spot (PHS) in one eye, and peripheral exudative hemorrhagic choroidal retinopathy (PEHCR) with central serous chorioretinopathy (CSC) and PHS in the contralateral eye.Case presentation: A 51-year-old healthy woman presented with complaint of blurred vision in her right eye. Corrected visual acuity was 20/20 in the right and 24/20 in the left eye. Fundus examination was normal in the left eye. In the right eye, fundus finding of an orange-red nodular lesion and optical coherence tomography (OCT) finding of polypoidal lesions led to a diagnosis of PCV. Four aflibercept intravitreal injections were performed in her right eye. After treatment, indocyanine green angiography (ICGA) confirmed residual polypoidal lesions with branching vascular networks and PHS with choroidal vascular hyperpermeability. OCT showed PHS associated with small sharp-peaked retinal pigment epithelium (RPE) elevation in peripheral fundus and small RPE elevation in posterior fundus. Based on the above findings, PCV with PHS was finally diagnosed in the right eye. Posttreatment corrected visual acuity in the right eye was 20/20. She presented again 32 months later, with complaint of vision loss in her left eye. Left corrected visual acuity was 20/20, and fundus examination showed mild vitreous hemorrhage. Vitrectomy was performed. In temporal midperipheral fundus, fluorescein angiography revealed CSC, and OCT showed pachychoroid. ICGA depicted abnormal choroidal networks and PHS in peripheral fundus. Furthermore, polypoidal lesions were confirmed by OCT. Based on the above findings, PEHCR and CSC with PHS was finally diagnosed in the left eye. Postoperative corrected visual acuity in the left eye was 20/20, and aflibercept intravitreal injection was performed for prevention of recurrence of vitreous hemorrhage.ConclusionsThis is the first case report of PCV with PHS in one eye, and PEHCR with CSC and PHS in the contralateral eye. This case suggests that PCV, PEHCR, and CSC may be linked pathologies of pachychoroid spectrum disease.

This issue of ACTA

This issue of ACTA

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.