3/19/2019 Pachychoroid disease Warrow et al 2013: Described pigmentary changes similar to fellow eyes of CSCR Pachychoroid Choroidal thickening Called this: pachychoroid pigment epitheliopathy “Pachy” : thick Clinical Spectrum Subsequent reports: other diseases manifest similar choroidal findings SURENDAR S. PUROHIT, MD New group of macular diseases: Pachychoroid Clinical Spectrum pachychoroid clinical spectrum Anatomy of Pachychoroid Pigment Epitheliopathy the Choroid Central Serous Chorioretinopathy Pachychoroid Neovasculopathy Haller’s layer Polypoidal Choroidal Vasculopathy Large choroidal veins Sattler’s layer Medium sized choroidal veins Choriocapillaris Fenestrated vessels Supply outer retina Bruch’s membrane Adjacent to RPE Choroidal thickness pachychoroid clinical spectrum Varies with age, ethnicity, axial length Increased choroidal thickness Normal: 191-354 μm “Pachyvessels” Thick choroid: >390 μm Dilated vessels in the outer choroid Enhanced depth imaging (EDI) OCT Compress inner choriocapillaris 1 3/19/2019 Pachychoroid Pigment Epitheliopathy Central serous chorioretinopathy ?forme fruste / precursor of CSCR RPE changes similar to CSCR First describes by Albrecht von Gaefe No subretinal fluid 1866 Asymptomatic Serous retinal detachment with or Thickened choroid without PED Appears reddish orange fundus Most commonly seen Reduced fundus tessellation in macular region RPE abnormalities / small PED Part of the spectrum of pachychoroid disease Epidemiology Risk factors Age of onset: 30-50 Type A behavior Can continue as chronic disease in elderly Elevation of catecholamine levels Older patients: Hypertension Worse vision Helicobacter pylori infection Diffuse retinal pigment epitheliopathy Obstructive sleep apnea Increased rates of CNVM Autoimmune disease Annual incidene: Familial predisposition 9.9 / 100,00 men 1.7 / 100,00 women Bilateral : 14-40% Risk factors pathophysiology Steroid use Occurs in 6% of patients receiving corticosteroids after renal transplant Multiple etiologies and mechanisms Oral, intra-articular, intranasal, topical Widespread choroidal circulation abnormality conditions associated with increased endogenous Choroidal vascular hyperpermeability cortisol production Increased hydrostatic pressure in choroid leads to breakdown of RPE Cushing’s disease barrier Pregnancy Leakage of fluid into subretinal space Steroid mechanism: Induce vasoconstriction Increased permeability of blood vessels RPE tight junction damage 2 3/19/2019 Clinical features: symptoms Clinical feature: acute Central scotoma Metamorphopsia Serous macular detachment Micropsia Yellow subretinal Blurred vision deposits in atypical Hyperopic shift cases RPE defects Usually resolves in 3-4 months Clinical features Clinical features: Chronic Tesselation: visibility of large choroidal vessels Reduced fundus tessellation Due to changes in choroidal pigmentation or to choroidal RPE degeneration thickening Shallow SRF Cystoid edema / schisis RPE degeneration in a teardrop configuration due to gravity CNVM Foveal atrophy Atypical CSCR Optical coherence tomography Inferior exudative RD Multifocal PED Choroid Large RPE tears Increased choroidal thickness Usually in patients undergoing systemic corticosteroid Increased thickness in fellow eye therapy Not a mandatory criterion Enhanced depth imaging 3 3/19/2019 OCT: choroid OCT: retinal pigment epithelium Thinning of inner choroidal layer PED Outer choroidal vessel dilation 50% Hyperreflective dots inner choroid Can be within or outside SRF Hyperreflectivity of wall of dilated vessels RPE atrophy “double layer sign” Undulated RPE layer with hyporeflective content over intact Bruch’s membrane Sign of chronicity Double Double layer sign layer sign Pachychoroid pigment epitheliopathy 0% Separation of the Pachychoroid neovasculopathy 93% irregularly elevated Chronic central serous chorioretinopathy 31% RPE from the inner layer of Bruch’s Polypoidal choroidopathy 91% membrane Fluid between basement membrane of RPE and Bruchs’s membrane OCT: subretinal space OCT: neurosensory retina Acute CSCR: Acute: retina intact SRF is serous Chronic: Hyporeflective area between retina and RPE changes in photoreceptor outer segments Chronic CSCR: Intraretinal hyperreflective dots Hyperreflective dots Intraretinal cysts or schisis May reflect photoreceptor outer segments shedding, fibrin or lipids Gradual loss of outer segments resulting in poor visual prognosis Atypical CSCR: clump or band like deposits 4 3/19/2019 Fundus autofluorescence (FAF) Fundus autofluorescence Acute CSCR Noninvasive imaging modality Hypoautofluorescence at leaking point Two types : Hypoautofluorescence in Short wavelength FAF areas of SRF Originates from lipofuscin pigment of RPE Due to masking of RPE Optic nerve and vessels hypoAF (no lipofuscin) autofluorescence by SRF Fovea hypoAF (absorption of blue light by luteal pigment) Infrared FAF Originates from melanin pigment of the choroid and RPE Optic nerve and vessels hypoAF (lack of melanin) Fovea: hyerAF (more dense melanin) Fundus autofluorescence Chronic CSCR Fundus autofluorescence: Hypoautofluorescence at leaking point long term sequelae Hyperautofluorescence Mixed FAF patters Outer retinal atrophy / loss of photoreceptors Later Hypoautofluorescence Unmasking of normal RPE In areas of RPE damage autofluorescence in pathway of SRF Granular Hyperautofluorescence Fundus Fluorescein Fundus Fluorescein Angiography: Angiography Acute CSCR Chronic CSCR Single or mulifocal point leaks Multifocal leakage Early punctate hyperfluorescence Diffuse oozing of dye Late: Diffuse RPE defect “ink blot” “smoke stack” leakage 5 3/19/2019 Indocyanine Green Optical Coherence Tomography Angiography Angiography Choroidal filling delay New noninvasive technique Dilated choroidal vessels Detects changes in blood flow choroidal hyperfluorescence in vessels focal patchy areas of choroidal No need for dye injection hyperfluorescence Segmentation of different Choroidal vasculature abnormality vascular layers more widespread than FA Commonly present in fellow eye CSCR: Dilated choriocapillaris Abnormal flow patterns suggestive of choroidal ischemia Can detect CNVM CSCR: natural history CSCR: natural history 90% improve spontaneously Irreversible vision loss: Residual metamorphopsia, scotoma RPE atrophy Reduced contrast sensitivity CNVM (6%) Chronic CSCR: Transformation into polypoidal choroidopathy Poor vision on presentation Prolonged duration of SRF 50% recurrence rate Hx of psychiatric disease Higher recurrence rate CSCR differential diagnosis CSCR differential diagnosis Optic disc pit Autoimmune / vascular disorders 45% serous macular detachment SLE ARMD Polyarteritis nodosa Inflammatory / infectious diseases Malignant HTN / Vogt-Koyanagi-Harada (VKH) disease Toxemia of pregnancy Posterior scleritis Disseminated intravascular coagulopathy Sympathetic ophthalmia Intraocular tumors Uveal effusion syndrome Choroidal hemangioma Benign reactive lymphoid hyperplasia Choroidal melanoma Choroidal metastasis 6 3/19/2019 pachychoroid clinical spectrum Pachychoroid Neovasculopathy Pachychoroid Pigment Epitheliopathy Central Serous Chorioretinopathy Form of type 1 (sub-RPE ) CNVM Pachychoroid Neovasculopathy Differences from AMD: Polypoidal Choroidal Vasculopathy Younger age Relative absence of drusen Thick choroid Pachyvessels Elderly patients can exhibit characteristics of both POLYPOIDAL CHOROIDAL VASCULOPATHY Polypoidal choroidal vasculopathy Traditionally considered a variant of wet AMD Minimal / absent drusen Thicker choroid Pachyvessels with inner choroid attenuation Shallow irregular PED’s Treatment: CSCR Treatment: CSCR Observation Argon laser photocoagulation Stop corticosteroids Micropulsed diode laser PO, topical, nasal sprays, injections Anti-VEGF therapy Lifestyle modifications Photodynamic therapy Choriocapillaris narrowing Reduction of choroidal exudation Choroidal hypoperfusion Choroidal vascular remodeling 7 3/19/2019 CSCR: corticosteroids Treatment: CSCR Elevated glucocorticoid hormone levels Caused by systemic / endogenous steroids Anticorticosteroid treatment Bind to glucocorticoid receptor & mineralcorticoid receptor Mineralocorticoid receptor antagonists MR present in RPE and endothelium of retinal / choroidal vessels Spironolactone (50 mg / day) Activation of MRs in choroidal vessels Eplerone (50 mg / day) Vessel dilation Other Leakage Rifampin Choroidal hyperpermeability Choroidal hyperpermeability can be blocked by MR antagonists 8 3/19/2019 9.