Peripapillary Pachychoroid Syndrome Research Articles

Peripapillary pachychoroid syndrome treated with dexamethasone implant: A case report.

To describe a case of peripapillary pachychoroid syndrome (PPS) in a diabetic patient with cystoid macular edema (CME), treated with intravitreal dexamethasone implant (IDI) injection. This report also illustrates the history of the disease after repeated IDI and dexamethasone topical treatment. A case report. A 77-year old male patient with PPS and good diabetic control was treated with dexamethasone implant for CME. After an initial morphofunctional improvement associated with a first IDI, the disease relapsed after the second dexamethasone implant injection. This was associated with a significant increase in both intraretinal fluid and choroidal thickness, with subsequent visual acuity (VA) decrease. At this point, a topical dexamethasone treatment was performed and, despite a morphological improvement, VA worsened compared with baseline, likely because of anatomical damage. In this report, the importance of the recognition of PPS is underlined and the possible occurrence of a "rebound" effect due to repeated IDI is described.

SUCCESSFUL TREATMENT OF SEVERE PERIPAPILLARY PACHYCHOROID SYNDROME WITH ANTI-VASCULAR ENDOTHELIAL GROWTH FACTOR THERAPY.

To describe two cases of severe peripapillary pachychoroid syndrome successfully managed with monthly intravitreal aflibercept therapy. Medical and imaging records were retrospectively reviewed. Patients were imaged with ultra-widefield fluorescein, indocyanine green angiography, and fundus autofluorescence. Spectral-domain optical coherence tomography was performed to evaluate macular edema and choroidal thickness. Optical coherence tomography angiography excluded macular neovascularization. This report summarizes two cases of peripapillary pachychoroid syndrome complicated by very severe bilateral macular edema. In all four eyes, the diffuse intraretinal and subretinal fluid remarkably improved or completely resolved after monthly intravitreal aflibercept injections with commensurate improvement of visual acuity. Multimodal imaging documented the significant improvement of fluid and the reduction in choroidal thickening in response to anti-vascular endothelial growth factor therapy in each case. Severe cases of peripapillary pachychoroid syndrome associated with vision loss can be successfully treated with intravitreal aflibercept therapy.

Prednisolone eye drops as a potential treatment in non-neovascular pachychoroid related diseases.

To investigate the functional and structural outcomes after treatment with prednisolone eye drops in the following pachychoroid related diseases: chronic central serous chorioretinopathy (cCSC), pachychoroid pigment epitheliopathy (PPE) and peripapillary pachychoroid syndrome (PPS). In this retrospective study, 54 eyes of 48 patients with pachychoroid related disease were treated with prednisolone acetate 1% eye drops for 3 times a day. Change in macular volume and retinal central subfield thickness on optical coherence tomography was measured. In addition, foveal or complete resolution of fluid and the change in visual acuity (VA) were studied. The follow-up visit was at a mean of 41.2 ± 14.5 days. In the 44 eyes with cCSC, a significant reduction in retinal central subfield thickness (p < 0.001) and macular volume (p < 0.001) was observed. Foveal intra- or subretinal fluid resolved completely in 22% of the eyes. In the 8 PPS eyes, a reduction in the nasal retinal thickness was observed (p = 0.025). One of the 2 PPE eyes showed structural improvement. No significant change in VA was observed in any of the pachychoroid spectrum diseases. In cCSC, PPS and PPE patients, anatomical improvement was observed after therapy with prednisolone eye drops. VA did not change significantly.

NOn-Pachychoroid PEripapillary Schisis (NOPPES) of the Retina: A New Phenotype and its Differential Diagnosis.

The presence of peripapillary intraretinal fluid (IRF) has a broad differential diagnosis, including several types of neovascular and pachychoroid-related diseases. However, the clinician may encounter cases without signs of neovascular or pachychoroid disease, or any other previously described diagnosis. For these patients, we propose the term NOn-Pachychoroid PEripapillary Schisis (NOPPES) of the retina, and we discuss the differential diagnosis. A retrospective chart study set in a tertiary referral center for retinal diseases in Amsterdam, the Netherlands. Using multimodal imaging, cases suspected of peripapillary pachychoroid syndrome were reviewed. Cases without signs of neovascular or pachychoroid disease were included in this study. These cases were discussed in a group of senior retinal specialists to establish a diagnosis, and if there was no evidence for any previously described diagnostic entity, these cases were categorized as NOPPES. Four cases of NOPPES were identified, 3 female patients and 1 male patient, aged between 58 and 75 years. Two patients were myopic, and 1 patient had a mild hyperopia. Three out of 4 cases showed unilateral peripapillary IRF, and 1 case had bilateral IRF. No improvement was seen after intravitreal bevacizumab or aflibercept, nepafenac eye drops, oral acetazolamide, vitrectomy with internal limiting membrane peeling, or surgery for carotid stenosis. One case showed a reduction in IRF after starting prednisolone eye drops. We describe NOPPES, a new form of peripapillary schisis-like IRF. NOPPES seems relatively therapy-resistant. More research is needed to delineate the clinical spectrum of NOPPES and its pathogenesis and treatment.

CURRENT VIEW OF THE SPECTRUM OF PACHYCHOROID DISEASES. A REVIEW.

The term "pachychoroid" (greek pachy- [παχύ] - thick) was first used by Warrow et al. in 2013. It is defined as an abnormal and permanent increase in choroidal thickness ≥ 300 μm, which is caused by dilatation of the choroidal vessels of the Haller's layer, thinning of the Sattler's layer and the choriocapillaris layer. Literary research focused on the current view of pachychoroid spectrum diseases, including clarification of the pathophysiological theories of the formation of "pachychoroid". It is assumed that "pachychoroid" disease has an autosomal dominant type of heredity. Depending on the further activity of various exogenous and/or endogenous factors, pachychoroid diseases may appear. According to the current knowledge, the spectrum of pachychoroid disease covers six clinical entities: pachychoroid pigment epitheliopathy, central serous chorioretinopathy, pachychoroid neovasculopathy, polypoid choroidal vasculopathy, focal choroidal excavation and peripapillary pachychoroid syndrome. In this study, we describe the clinical symptoms and objective findings of focal choroidal excavation and peripapillary pachychoroid syndrome. The current pathophysiological theory of pachychoroid diseases is based on impaired venous outflow from the choroid ("venous overload choroidopathy") and thickening of the sclera in the eyes of affected patients. Pachychoroid diseases should be included in the differential diagnosis of characteristic features observed during multimodal imaging analysis of choroidal changes.

POTENTIAL TREATMENT FOR PERIPAPILLARY PACHYCHOROID SYNDROME.

The purpose of this study was to describe the effect of topical prednisolone on intraretinal fluid in patients with peripapillary pachychoroid syndrome. We selected 11 consecutive patients (17 eyes) with a diagnosis of peripapillary pachychoroid syndrome, who were treated with topical prednisolone (Pred Forte [PF] 10 mg/mL) three times daily for 4 weeks. We tapered off PF among patients who demonstrated a reduction of intraretinal fluid. Of the included 17 eyes, the average follow-up before PF treatment ranged from 6 to 192 months, during which patients experienced no apparent reduction of intraretinal fluid. The baseline mean best-corrected VA (BCVA) was 0.6 (20/33) Snellen. The median subfoveal and peripapillary choroidal thickness were 430 µm and 202 µm, respectively. All patients showed an initial reduction of intraretinal fluid after 4 weeks of topical prednisolone. Six patients (35%) experienced a prolonged reduction of intraretinal fluid when the dosage was reduced to once daily. On tapering off PF, four eyes (24%) experienced a recurrence of intraretinal fluid. Four eyes (24%) experienced elevated intraocular pressure above 26 mmHg. In two eyes, PF was discontinued, on which intraretinal fluid reappeared. The BCVA seemed to be improved in 9 eyes (53%) and remained equal in 4 eyes (24%). In this case series of patients with peripapillary pachychoroid syndrome, we observed a reduction of peripapillary intraretinal fluid after treatment with topical prednisolone for 4 weeks in all 17 eyes. The disappearance of intraretinal fluid seemed to concede with a slight improvement in BCVA for some cases. Thus, topical prednisolone may prove to be a viable treatment option in peripapillary pachychoroid syndrome.

Pachychoroid Spectrum Disorders: An Updated Review.

Pachychoroid disease spectrum is a recent term that has been associated with an increasing number of phenotypes. This review discusses updated findings for each of the typical pachychoroid entities (central serous chorioretinopathy, pachychoroid pigment epitheliopathy, pachychoroid neovasculopathy, polypoidal choroidal vasculopathy, peripapillary pachychoroid syndrome, and focal choroidal excavation), as well as two relatively new additions (peripapillary pachychoroid neovasculopathy and peripheral exudative hemorrhagic chorioretinopathy). Here, we discuss the potential pathogenic mechanisms for these diseases and relevant imaging updates. Finally, we argue for a consistent classification scheme for these entities.

Pachychoroid Disease

Pachychoroid spectrum disorders include uncomplicated pachychoroid, pachychoroid pigment epitheliopathy, central serous chorioretinopathy, pachychoroid neovasculopathy, polypoidal choroidal vasculopathy/aneurysmal type 1 neovascularisation, focal choroidal excavation and peripapillary pachychoroid syndrome. They are characterized by a thickened and hyperpermeable choroid and thinning of the choriocapillaris. The disorders are being diagnosed with increasing frequency and differentiation due to the advancement of multimodality imaging. Current understanding of the development, course, possible complications and treatment of these diseases is growing rapidly, but not all mechanisms have yet been elucidated. A correct diagnosis is important, especially the differentiation between the presence of active neovascularisation or a purely exudative stage, in order to initiate a therapy. It is also not yet clear why patients have a thickened choroid and why some of these patients develop pathological changes such as subretinal fluid, RPE changes or neovascularisation.

ACQUIRED VITELLIFORM LESIONS: A Novel Finding in Eyes with Peripapillary Pachychoroid Syndrome.

To describe a novel finding of acquired vitelliform lesions in eyes with peripapillary pachychoroid syndrome. A retrospective, multicenter, observational case series including seven patients with peripapillary pachychoroid syndrome and concomitant acquired vitelliform lesions. Medical records and multimodal imaging findings were comprehensively reviewed. Six of seven patients were men, with a mean age of 72.6 years. Mean baseline best-corrected visual acuity was 0.11 ± 0.07 (Snellen equivalent 20/25), and all patients presented with baseline best-corrected visual acuity of 20/30 or better. Mean follow-up time was 24.4 months (range 1-54 months). At last follow-up, best-corrected visual acuity was stable in 13/14 eyes, and deteriorated in one eye because of subfoveal atrophy.Fundus examination revealed peripapillary retinal pigment epithelium alterations and pigment migration in all eyes. Fundus autofluorescence showed mottled hyperautofluorescence and hypoautofluorescence in the peripapillary region, and hypoautofluorescent lesions corresponding to the pigmentary changes. Optical coherence tomography showed nasal choroidal thickening and pachyvessels with overlying retinal pigment epithelium irregularity. All eyes presented with peripapillary intraretinal fluid. Subretinal fluid was present in five eyes of three patients. Acquired vitelliform lesions were present in 11 eyes. The novel association between peripapillary pachychoroid syndrome and acquired vitelliform lesions is characterized by the classic findings of both entities, and visual prognosis is accordingly good.

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.

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.

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.

Venous overload choroidopathy: A hypothetical framework for central serous chorioretinopathy and allied disorders.

In central serous chorioretinopathy (CSC), the macula is detached because of fluid leakage at the level of the retinal pigment epithelium. The fluid appears to originate from choroidal vascular hyperpermeability, but the etiology for the fluid is controversial. The choroidal vascular findings as elucidated by recent optical coherence tomography (OCT) and wide-field indocyanine green (ICG) angiographic evaluation show eyes with CSC have many of the same venous patterns that are found in eyes following occlusion of the vortex veins or carotid cavernous sinus fistulas (CCSF). The eyes show delayed choroidal filling, dilated veins, intervortex venous anastomoses, and choroidal vascular hyperpermeability. While patients with occlusion of the vortex veins or CCSF have extraocular abnormalities accounting for the venous outflow problems, eyes with CSC appear to have venous outflow abnormalities as an intrinsic phenomenon. Control of venous outflow from the eye involves a Starling resistor effect, which appears to be abnormal in CSC. Similar choroidal vascular abnormalities have been found in peripapillary pachychoroid syndrome. However, peripapillary pachychoroid syndrome has intervortex venous anastomoses located in the peripapillary region while in CSC these are seen to be located in the macular region. Spaceflight associated neuro-ocular syndrome appears to share many of the pathophysiologic problems of abnormal venous outflow from the choroid along with a host of associated abnormalities. These diseases vary according to their underlying etiologies but are linked by the venous decompensation in the choroid that leads to significant vision loss. Choroidal venous overload provides a unifying concept and theory for an improved understanding of the pathophysiology and classification of a group of diseases to a greater extent than previous proposals.

Pachychoroid diseases

The aim of this study is to present our knowledge about pachychoroid diseases using case reports, literature review and our own clinical experiences. A summary flow chart of treatment options for the subgroups was prepared, too. Pachychoroid diseases include the following: central serous chorioretinopathy (CSCR), pachychoroid pigment epitheliopathy (PPE), pachychoroid neovasculopathy (PNV), polypoidal choroidal vasculopathy (PCV), peripapillary pachychoroid syndrome (PPS), focal choroidal excavation (FCE). A common feature of pachychoroid diseases is the quantitative or qualitative abnormality of the choroidea, which is often associated with subretinal fluid accumulation. The disease group does not currently have a standard treatment protocol; some of the multiple treatments prove to be more effective, however, there are significant differences between the subgroups. We summarize which subgroup benefits from eplerenone tablet therapy, micropulse laser therapy, verteporfin photodynamic therapy or intravitreal anti-VEGF injection therapy. Orv Hetil. 2020; 162(20): 770-781.

Uniform classification of the pachychoroid spectrum disorders

Pachychoroid spectrum disorders are characterized by athickening of the choroid. The spectrum includes pachychoroid pigment epitheliopathy (PPE), central serous chorioretinopathy (CCS), pachychoroid neovasculopathy (PNV), polypoidal choroidal vasculopathy (PCV)/aneurysmal type1 choroidal neovascularization (ACNV-1), focal choroidal excavation (FCE) and peripapillary pachychoroid syndrome (PPS). If the choroid is thickened and there is a pathological alteration in the retinal pigment epithelium, the diagnosis is PPE; if the thickened choroid is accompanied by subretinal fluid, the diagnosis is CCS; if choroidal neovascularization is present, the diagnosis is PNV; if accompanied by aneurysms, the diagnosis is ACNV‑1. The PPE, CCS, PNV and ACNV‑1 were formerly regarded as independent disease entities but can be classified into four forms of asingle disease family, pachychoroid macular disease.

Photodynamic therapy as a treatment option for peripapillary pachychoroid syndrome: a pilot study.

To investigate the anatomical and functional results in eyes with peripapillary pachychoroid syndrome (PPS) undergoing photodynamic therapy (PDT). A total of 25 eyes from 23 patients with PPS treated with PDT were retrospectively evaluated in this multicentric study. Main outcome measure was the proportion of eyes that achieved treatment success, defined as a decrease in both subretinal fluid (SRF) height and central subfield thickness (CST), at 3 months after PDT compared to baseline. Secondary outcomes were the change in CST, SRF, and best-corrected visual acuity (BCVA) 3 months after treatment and predictive factors for treatment success. When available, data between 3 and 12 months were also reviewed. Treatment success was achieved in 16 eyes (64%). In the total cohort, CST decreased significantly from 356 ± 118 µm at baseline to 282 ± 90 µm and 270 ± 91 µm at 1 and 3 months, respectively (p < 0.001). Maximal SRF height decreased significantly from 102 ± 83 µm at baseline to 38 ± 46 µm and 32 ± 42 µm at 1 and 3 months, respectively (p < 0.001), and remained stable at month 6 (29 ± 44 µm) and month 12 (23 ± 35 µm). BCVA improved significantly from baseline to month 3 (p = 0.021). PDT can be considered an efficacious treatment option in patients with PPS. Prospective data with longer follow-up in a bigger cohort are needed in order to determine the optimal treatment algorithm in this relatively novel disease.

A case of peripapillary pachychoroid syndrome treated with anti-vascular endothelial growth factor injections

Peripapillary pachychoroid syndrome (PPS) is a novel variant in the pachychoroid disease spectrum in which pachychoroid features are more prominent around the optic nerve head and are associated with intraretinal and/or subretinal fluid. The choroidal thickness in PPS is characterized by its atypical distribution with nasal macular choroid being thicker than the temporal counterpart. There are a few case reports of PPS in the literature that describe the morphological features of PPS but the management protocols are yet to be established. We hereby describe the clinical course of a 54-year-old gentleman who came to us with complaints of a gradual decrease in vision. On basis of multimodal imaging, a diagnosis of PPS was made. Since the patient was symptomatic, he was treated with intravitreal aflibercept which showed a favorable anatomical and functional outcome.

Long-term visual and anatomic outcomes of patients with peripapillary pachychoroid syndrome

Background/AimsTo analyse the long-term anatomic and visual outcomes of patients with peripapillary pachychoroid syndrome (PPS), a recently described entity in the pachychoroid disease spectrum.MethodsThis study retrospectively included patients from several...

INTERVORTEX VENOUS ANASTOMOSIS IN Pachychoroid-RELATED DISORDERS.

To evaluate the choroidal vascular patterns of patients with pachychoroid-related diseases in eyes images with wide-field indocyanine green angiography. Retrospective study of wide-field indocyanine green angiographic images of patients with pachychoroid, peripapillary pachychoroid syndrome, central serous chorioretinopathy, and pachychoroid-associated neovascularization that were evaluated for anastomoses between vortex vein systems, which are ordinarily separated by a watershed zone. There were 21 subjects with a mean age of 57.4 years and 15 were male. Among the 42 eyes evaluated, central serous chorioretinopathy was found in 24 eyes (57.1%), peripapillary pachychoroid syndrome in 5 (11.9%), pachychoroid associated neovascularization in 7 (16.7%), and pachychoroid in 6 (14.3%). Every eye showed anastomosis between the superonasal, superotemporal, and inferotemporal vortex vein systems. The inferonasal vortex vein system was less likely to demonstrate anastomosis except for peripapillary pachychoroid syndrome, which showed anastomosis in all eyes. The anastomotic connections were prominent in the central macula in the central serous chorioretinopathy and pachychoroid-associated neovascularization cases, and around the nerve in the peripapillary pachychoroid syndrome cases. Although the large choroidal veins were particularly prominent in the neovascular cases, the number was fewer in the macular region than in other pachychoroid-related diseases in this series. Compared with a control group of nine eyes, the inferotemporal-superotemporal-superonasal anastomotic connections were more common in the case group (P < 0.001) and inferonasal quadrant (P = 0.023 right eye; P = 0.01, left eye). Intervortex venous anastomosis is common in pachychoroid, central serous chorioretinopathy, peripapillary pachychoroid syndrome, and pachychoroid-associated neovascularization. This finding has important implications concerning pathogenesis and classification of disease.

CHORIOCAPILLARIS VASCULAR PARAMETERS IN NORMAL EYES AND THOSE WITH PACHYCHOROID WITH AND WITHOUT DISEASE.

To evaluate the vascular characteristics of the choriocapillaris in eyes with pachychoroid as compared with normal controls. Eyes with pachychoroid disease were defined as those with a history of central serous chorioretinopathy or peripapillary pachychoroid syndrome. Pachychoroid without disease was defined as eyes with no history of disease with a subfoveal choroidal thickness ≥ the age-adjusted 95th percentile thickness. Frame-averaged optical coherence tomography angiography images of the choriocapillaris obtained with a Zeiss Plex Elite were binarized, skeletonized, and evaluated for vascular branching parameters. There were 7 normal control subjects, 10 subjects with pachychoroid without disease, and 17 pachychoroid disease subjects. Mean choriocapillaris vessel segment length was 12.19 µm in eyes with pachychoroid disease as compared with 11.48 µm in normal controls and 11.62 µm in pachychoroid without disease (P = 0.003 and P = 0.006, respectively). The branches per square millimeter were fewer in pachychoroid disease (1,215), as compared with normal controls (1,471) or pachychoroid without disease (1,384; P < 0.001, and P = 0.002, respectively). The choriocapillaris vessel diameter was larger, but the fractal dimension was smaller in pachychoroid disease eyes as compared with normal eyes or pachychoroid without disease eyes. There was no statistically significant difference between normal controls and pachychoroid without disease for any measured vascular parameter of the choriocapillaris. Choriocapillaris vascular parameters suggest that pachychoroid is not necessarily pathologic. It is possible that choroidal thickening is an epiphenomenon, and there are more significant vascular parameters that are related to disease. These concepts may help guide future prospective studies.