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

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