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PURPOSE: 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. METHODS: A case report. RESULTS: 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. CONCLUSION: 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.

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Introduction: Nanophthalmos is characterized by a short axial length, a thick choroid, and a thick sclera. Unilateral symptomatic disc swelling in nanophthalmos presents both a diagnostic and a therapeutic challenge. Case Presentation: A healthy 59-year-old man reported a two-week-long abrupt vision reduction in his right eye. 20/100 best spectacle (+17.25 diopter) corrected visual acuity, unilateral widespread disc enlargement, central scotoma, and a slight color vision disruption without an afferent pupillary defect were among the positive findings in the right eye. Workup for neuro-ophthalmology was negative. Numerous consultations did not suggest any form of treatment for the patient. Review of the optical coherence tomography (OCT) indicated a small, crowded optic nerve head and substantial diffuse choroidal thickening with dome-shaped temporal peripapillary area with choroidal expansion. In addition to circumferential anterior four-quadrant 95%-deep sclerectomy from recti insertion to the vortices, radial nasal posterior sclerotomy reaching the optic nerve sheath was performed on the patient. After the procedure, 2 weeks later, the patient's vision returned, and it persisted until the 6-month follow-up. By OCT, the two eyes were comparable as far as disc contour and nerve fiber layer thickness. Conclusion: This form of sclerectomy, which aims at decompressing the oncotic choroidal pressure, is an effective treatment for compressive optic neuropathy in the context of nanophthalmos. Could sclerectomy assist in treating other optic neuropathies associated with peripapillary pachychoroid?

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Peripapillary pachychoroid syndrome (PPS) is a rare disease characterized by choroidal thickening around the optic disc. Visual acuity might be impaired secondary to the associated peripapillary intraretinal and/or subretinal fluids. We reported a case of a 70-year-old male patient who presented with a gradual bilateral decrease in vision. His best-corrected visual acuity was 20/60 in the right eye and 20/25 in the left eye. Dilated fundus examination showed yellowish peripapillary lesions and intraretinal fluid (IRF) surrounding the optic disc in both eyes. Optical coherence tomography showed that the outer retinal layers were disrupted, and IRF affected the nasal macula. A bilateral increase in the thickness of the choroid around the optic discs was found. The patient was successfully treated with a tapering dose of topical prednisolone acetate (1%). Three months after treatment, his vision improved to 20/25 in the right eye and 20/20 in the left eye. Topical steroid drops might be administered to treat PPS.

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Purpose: To report a case of peripapillary pachychoroid syndrome (PPS) complicated with peripapillary retinal neovascularization causing vitreous hemorrhage. Observation: A 42-year-old man, with a history of a visual loss of the right eye (RE) for 4 years, presented for ophthalmological examination. Best-corrected visual acuity was «counting fingers¼ in the RE and 20/25 in the left eye. Fundus examination showed irregular pattern of hypopigmentation of the retinal pigmented epithelium in both eyes with retinal neovessels in the peripapillary region of the RE. Swept-source optical coherence tomography demonstrated a central serous pigment epithelial detachment with intraretinal cysts and serous retinal detachment in the nasal macula extending from the temporal disc margin in the RE. Fluorescein angiography showed multiple areas of hyperfluorescence without clear distinction of retinal neovessels. Indocyanine green angiography showed patches of choroidal hyperpermeability predominant in the peripapillary region in both eyes. Optical coherence tomography angiography provided a good visualization of the papillary and retinal neovessels without signs of choroidal neovascularization. Two months after initial examination, the patient presented with vitreous hemorrhage associated to juxtapapillary preretinal hemorrhage in the RE. After vitreous injections of bevacizumab, we observed a total resolution of the vitreous hemorrhage a partial decrease of the intraretinal and subretinal fluid. Conclusions and importance: We report an unusual case of peripapillary retinal neovascularization and vitreous hemorrhage complicating a PPS. OCTA was useful to detect retinal neovessels and peripapillary retinal and choriocapillaris hypoperfusion, supporting the understanding of the pathogenic mechanism of neovascularization in PPS.

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Intraretinal or subretinal fluid in the peripapillary area can be clinically visualized in conditions such as peripapillary choroidal neovascularization, optic disc pit maculopathy, and optic nerve head tumors and granulomas. Optical coherence tomography (OCT) helps to visualize peripapillary fluid in many other chorioretinal conditions such as peripapillary pachychoroid syndrome, posterior uveitis, central retinal vein occlusion, malignant hypertension, hypotonic maculopathy as well as neuro-ophthalmological conditions such as glaucoma, microcystic macular edema and disc edema due papilledema, non-arteritic anterior ischemic optic neuropathy, neuroretinitis, and diabetic papillopathy. Often, the differential diagnosis of peripapillary fluid is a bit tricky and may lead to misdiagnosis and improper management. We describe a diagnostic algorithm for peripapillary fluid on OCT and outline the salient features and management of these conditions.

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INTRODUCTION: 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. METHODOLOGY: Literary research focused on the current view of pachychoroid spectrum diseases, including clarification of the pathophysiological theories of the formation of "pachychoroid". RESULTS: 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. CONCLUSION: Pachychoroid diseases should be included in the differential diagnosis of characteristic features observed during multimodal imaging analysis of choroidal changes.

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INTRODUCTION: 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. METHODOLOGY: Literary research focused on the current view of pachychoroid spectrum diseases, including clarification of the pathophysiological theories of the formation of "pachychoroid". RESULTS: 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. CONCLUSION: Pachychoroid diseases should be included in the differential diagnosis of characteristic features observed during multimodal imaging analysis of choroidal changes.

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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.

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Purpose: To report a case of peripapillary pachychoroid syndrome (PPS) successfully treated with navigated subthreshold micropulse laser (SML). Observations: A 65-year-old male was referred to our retina service complaining a worsening vision in the left eye (LE) over the past 6 months. A complete ophthalmological evaluation including best corrected visual acuity (BCVA) measurement, spectral-domain optical coherence tomography (SD-OCT) and fluorescein angiography (FA) was performed. SD-OCT showed a thicker nasal choroid and peripapillary intraretinal cysts in both eyes, and macular subretinal fluid (SRF) in the LE. FA illustrated a bilateral peripapillary hyperfluorescent areas, with some macular focal leaking points in the left eye. A diagnosis of PPS was made, and considering the worldwide shortage of verteporfin, Navigated 577-nm SML was performed in the LE on the leaking areas shown on the FA. At the 3- and 6- months follow-up the SRF reabsorbed and BCVA improved from 20/32 to 20/20. Conclusions and importance: SML can be considered an efficacious treatment option in patients with PPS. Prospective studies with longer follow-up in a bigger cohort are needed to confirm the optimal treatment strategy in PPS.

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PURPOSE: Peripapillary pachychoroid syndrome (PPS) is a recently described entity of the pachychoroid disease spectrum and is characterized by thickening of the nasal choroid and peripapillary fluid pockets. This case illustrates the remarkable natural history of this recently described disorder. OBSERVATION: This case report describes a patient with PPS who presented with severe cystoid macular edema (CME) that spontaneously resolved without treatment. CONCLUSIONS AND IMPORTANCE: This report indicates that PPS can exhibit significant fluctuation of CME and a favorable natural history in some cases. It also suggests that observation may be a viable option in the initial management of this finding if vision is not significantly unaffected.

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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.

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Pachychoroid spectrum disorders are characterized by a thickening of the choroid. The spectrum includes pachychoroid pigment epitheliopathy (PPE), central serous chorioretinopathy (CCS), pachychoroid neovasculopathy (PNV), polypoidal choroidal vasculopathy (PCV)/aneurysmal type 1 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 ACNV­1. The PPE, CCS, PNV and ACNV­1 were formerly regarded as independent disease entities but can be classified into four forms of a single disease family, pachychoroid macular disease.

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PURPOSE: The term "pachychoroid" refers to a newly described phenotype in which functional and structural choroidal changes are thought to play a key pathogenic role in a spectrum of related retinal disorders. A more detailed understanding of how the choroid is involved within this spectrum and a better knowledge of the most relevant clinical signs of the pachychoroid phenotype are important to differentiate these disorders from other retinal conditions. Our objectives are to provide a literature review of pachychoroid and the commonalities that may be present across pathologies included in the spectrum, and to provide details on the examination, monitoring, and management of these disorders. METHODS: We searched the PubMed web platform to identify relevant studies using the following keywords: pachychoroid, pachychoroid pigment epitheliopathy, pachychoroid neovasculopathy, aneurysmal type 1 neovascularization, focal choroidal excavation, peripapillary pachychoroid syndrome, vasculopathy pachysclera, pachychoroid geographic atrophy, and pachydrusen. We selected 157 publications and identified the most important features related to pachychoroid. RESULTS: The presence of hypertrophic or congested vessels in the choroid, not thickened choroid per se, under an area of reduced or absent choriocapillaris in the posterior pole seems to be the most salient feature of pachychoroid. However, other qualitative/quantitative features are needed to differentiate the uncomplicated pachychoroid from the pathological pachychoroid clinical spectrum, which may be associated with exudation, neovascularization, and/or retinal pigment epithelium and photoreceptor atrophy. CONCLUSIONS: The most salient feature of pachychoroid appears to be the presence of large vessels under an area of reduced or absent choriocapillaris. Knowledge of the features and pathogenesis of the different disorders in the pachychoroid spectrum may assist in the management of patients.

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Peripapillary pachychoroid syndrome (PPS) is a newly described disease entity belonging to the spectrum of pachychoroid diseases. We report on a patient with PPS.

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