Choroidal caverns in pachychoroid neovasculopathy

Background. Choroid plays an important role in the pathogenesis of retinal pathology. Choroidal cavern, a recently identifi ed fi nding of optical coherent tomography (OCT), has been described in some degenerative and atrophic forms of retinal pathology. In the literature, there are only a few studies of choroidal cavers in pachychoroid neovasculopathy, newly described form of age related macular degeneration.The aim: to perform a detailed analysis of choroidal structure on OCT scans of patients with pachychoroid neovasculopathy and to reveal the frequency of choroidal caverns identifi cation.Material and methods. The data of 30 patients (30 eyes) aged 64.4 ± 5.6 years with pachychoroid neovascularization were retrospectively analyzed. The patients underwent spectral OCT and OCT-angiography (OCTA) using a Spectralis device (Heidelberg Engineering, Germany). The protocol was “Posterior Pole”, consisting of 61 scans. To assess the structure of the choroid, an enhanced image depth (EDI) module was used. OCT angiography was performed with a scan area of 6 × 6 mm. These methods were compared to identify choroidal caverns.Results. On OCT subretinal type 1 neovascularization was revealed as a fl at detachment of the pigment epithelium and visualization of blood flow on OCTA in the lesion as angled vessels (21 eyes) or a seafan (9 eyes). On OCT-EDI scans, there was diffuse or local choroidal thickening of choroid with an increase in the vessels of the Haller’s layer and thinning of the choriocapillaries. Choroidal caverns appeared on OCT and en-face OCT as areas with low optical density, round or irregular, located in different layers of the chorioid, without hyperrefl ective boundaries. A typical sign of choroidal cavern is the tail of hypertransmission after the cavern toward the sclera. Choroidal caverns were found in 4 of 30 eyes (13.3 %) and were located both near the choroidal neovascularization lesion and beyond this area.Conclusion. The prevalence of choroidal cavities, a new choroidal biomarker, in pachychoroid neovasculopathy was 13.3 %. Identification of these changes is possible with the use of modern diagnostic techniques (OCT-EDI, OCTA and en-face OCT) that allow visualization the state of the choroid. The prognostic signifi cance of choroidal cavities requires further study.

Progression of Subclinical Pachychoroid Neovasculopathy to an Active Neovascularization in the Presence of Acquired Vitelliform Lesions

We describe a unique case of bilateral acquired vitelliform lesions in a 67-year-old-female with pachychoroid associated with subretinal fluid in the right eye (OD) and a nonexudative choroidal neovascular membrane (CNVM) in the left eye (OS). Multimodal imaging performed at baseline and over the ensuing two years showed an increase in the OS vitelliform lesions with a concurrent transformation of quiescent CNVM to an exudative form. Further studies are warranted to gain better insight into the etiopathogenesis of these vitelliform lesions in pachychoroid and their potential role in instigating CNVM activation.

Analysis focusing on plasma von Willebrand factor in pachychoroid neovasculopathy and age-related macular degeneration

Pachychoroid neovasculopathy (PNV) is a new concept of macular disorder. Some cases diagnosed as age-related macular degeneration (AMD) have been re-diagnosed as PNV. However, the biological features of PNV are still uncertain. The purpose of this study was to compare PNV and AMD by analyses focusing on von Willebrand factor (VWF) and complement factor H (CFH). Ninety-seven patients who were previously diagnosed with treatment naïve AMD were enrolled in this study. They were re-classified as either PNV or AMD based on the clinical criteria and 33 patients were classified as PNV and 64 patients as AMD. We examined the clinical data, analyzed VWF multimer and two genetic polymorphisms (I62V and Y402H) in the CFH. PNV group was significantly younger than AMD group (P = 0.001). In both I62V and Y402H, there were no significant differences between PNV and AMD while the recessive homozygous (AA) was found only in PNV group in I62V. The presence of unusually large VWF multimers (UL-VWFMs) and subretinal hemorrhages were significantly higher in PNV than in AMD (P = 0.045, P = 0.020, respectively). Thus, the residual UL-VWFMs may result in platelet thrombosis and hemorrhages in the choriocapillaris of PNV. In conclusion, our results suggest the biological differences between PNV and AMD.

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.

A comparative study of type 1 neovascularization: Neovascular age-related macular degeneration versus pachychoroid neovasculopathy

Purpose: This study aimed to compare type 1 choroidal neovascularization (CNV) characteristics in eyes with pachychoroid neovasculopathy (PNV) and neovascular age-related macular degeneration (nAMD) using optical coherence tomography angiography (OCTA). Methods: Treatment naive 23 eyes of 23 patients with PNV and 24 eyes of 24 patients with nAMD were evaluated. The height of pigment epithelial detachment (PED) and the central macular thickness were determined. OCTA sensitivity, CNV area, morphological patterns, and retinal superficial capillary plexus vessel density (SCP-VD) values were compared. The frequency of quiescent CNV, subretinal hyperreflective exudation (SHE), subretinal/intraretinal fluid, serous PED, double-layer sign (DLS), and pachyvessels were noted. Results: CNV was detected on OCTA in 83.3% of nAMD eyes and 91.3% of PNV eyes ( p = 0.66). Indistinct pattern was more common (74% vs 50%) and the CNV area (mm2) was smaller in PNV (0.77 ± 0.54 vs 1.57 ± 1.43) but did not reach significant levels ( p = 0.27 and 0.33 respectively). SCP-VD was similar between the groups ( p = 0.38). Statistically significant differences were found between groups in age and subfoveal choroidal thickness ( p < 0.05). DLS and pachyvessels were found to be more frequently in PNV ( p < 0.05). However, both groups had similar rates of quiescent CNV, SHE, subretinal/intraretinal fluid, and serous PED ( p > 0.05). Conclusion: Morphological features, area, and activation findings of type 1 CNV may play a limited role in differentiating nAMD and PNV cases.

Pachychoroid neovasculopathy: A comparative review on pathology, clinical features, and therapy

There have been major changes in our understanding of choroidal diseases in the last decade owing to multiple retinal and choroidal imaging related advances. A major conceptual pivot is establishment of pachychoroid and its spectrum of clinical disorders: pachychoroid pigment epitheliopathy, central serous chorioretinopathy, pachychoroid neovasculopathy, polypoidal choroidal vasculopathy/aneurysmal type 1 neovascularization, peripapillary pachychoroid syndrome, and focal choroidal excavation. However, considerable overlaps in manifestations and therapeutics of these disorders make differentiation amongst them difficult. This review is focused on pathogenesis and clinical aspects of pachychoroid neovasculopathy (PNV). Since PNV was defined as a separate entity around 5 years ago, there have been numerous contrasting observations surrounding it. We review and summarize these studies, and also compare PNV with other disorders of the pachychoroid spectrum in detail. There are important differences between etiologies of neovascular age related macular degeneration and PNV. Yet the current treatment strategies for PNV have been extrapolated from the trials for the former. Future research needs to validate this assumption with long-term results.

Pachychoroid neovasculopathy with normal subfoveal choroidal thickness

Pachychoriod neovasculopathy and typical neovascular age-related macular degeneration often look similar, but some differences have been described both genotypically and in the level of cytokine profile, which explains the difference in response to antiangiogenic therapy. It is known that pachychoriod diseases are characterized by choroidal subfoveolar thickening. However, there is currently no data on the structural features of the choroid in the presence of pachychoriod neovasculopathy outside the fovea. Purpose. To perform a detailed analysis of choroial structure on OCT scans of patients with neovascular AMD with normal choroidal subfoveolar thickness and to reveal the frequency of pachychoriodal neovasculopathy identification. Material and methods. In retrospective study, the data of 30 treatment-naive patients (30 eyes) aged 71.5±7.6 years with CNV type 1 were analyzed, including 18 women and 12 men, with a subfeolar thickness of the choroidal membrane not exceeding 250 µm. The patients underwent OCT and OCT-angiography using a Spectralis device (Heidelberg Engineering, Germany). In the foveolar zone and under the neovascular complex, if it is located outside the fovea, the structure of the choroid was analyzed: the choroidal thickness, Haller layer and choriocapillaries / layer of Sattler vessels were measured. Results. The average thickness of the choroid in the fovea in all patients was 170.1±40 µm. The thickness of Haller vessels layer was on average 112.3±20 μm, and the layer of choriocapillaries / Sattler vessels was 57.8±12 μm. In 22 eyes, the structure of the choroid and the ratio of all layers were similar in all analyzed OCT scans. However, in 8 eyes, under the CNV located extrafoveally, a thickening of the choroid was revealed up to 210.5±51 µm, the main volume of the choroid was presented by pachyvessels – dilated and enlarged vessels of the Haller layer which thickness was 189.4 ± 48 µm, and thinning of the choriocapillary / Sattler layer to 21.1±16 µm. Conclusion. Among 30 patients with normal subfoveolar thickness of the choroid, the frequency of PNV was 26.6%. Taking into account the literature data on different responses to antiangiogenic therapy in patients with PNV and typical AMD, we can once again emphasize the importance of a detailed assessment of changes in the choroid not only in the foveolar zone, but also specifically in the area of the neovascular complex. Key words: pachychoriod neovasculopathy, choroid, OCT.

Vanishing pachy-choroid in pachychoroid neovasculopathy under long-term anti-vascular endothelial growth factor therapy

Background To investigate the diagnostic value of choroidal thickness in the definition of pachychoroid neovasculopathy (PNV), especially in eyes treated with anti-vascular endothelial growth factor (VEGF) therapy. Methods Twenty-two consecutive eyes of 11 patients with uni- or bilateral PNV were analyzed. Anti-VEGF treatment was correlated with changes in choroidal thickness on enhanced depth imaging optical coherence tomography. Results There were 14 eyes with PNV and 8 non-neovascular partner eyes. Mean age was 64.2 ± 4.0 (range: 60–72), total follow-up was 1.8 ± 0.4 (1–2) years. In PNV eyes, choroidal thickness at baseline was 400 ± 58 (269–485) μm. After two years and 13 anti-VEGF injections on average, a mean reduction of − 39 ± 10 (− 26 to − 56) % to final 241 ± 52 (162–327) μm was observed (p < 0.0001). Meanwhile, choroidal thickness in the partner eyes remained stable (p > 0.13 for all comparisons). A significant correlation of choroidal thinning and anti-VEGF injection rate was observed at year one (r = − 0.79; R2 = 0.63; p = 0.00073) and two (r = − 0.69; R2 = 0.48; p = 0.019). While 85.7% of PNV eyes exceeded a pachychoroid threshold of ≥350 μm at baseline, this figure dropped to 21.4% at year one and 0% at year two. Conclusion In PNV, choroidal thickness significantly decreases with anti-VEGF therapy, resembling a “vanishing pachy-choroid”, and thus does not represent a valid long-term diagnostic criterium, especially when differentiating PNV from nAMD.