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Mohamed Elkadim
9/4/2020
 ↑ Thickness of choroidal veins (haller layer)
 Attenuation of choroidal capillaries (sattler layer)
 Choroidal hyperpermeabilty
 Progressive RPE dysfunction.
 ± Thick choroid
 Hypertension
 Choroidal ischemia
 Unknown
 Type A Personality, emotional stress,
 pregnancy,
 tobacco use,
 corticosteroids,
 sympathomimetics.
 herbal supplements.
 CSCR
 Pachychoroid pigment epitheliopathy
 Polypoyoid choroid vasculopathy
 Pachychoroid neovasculopathy
 Pachychoroid peripapillary vasculopathy
 Choroidal excavation
 Concave macula
 ICG : dilated choroidal vessels ( pachy vessels:
don’t taper towards the posterior pole)+ leak +
delayed arterial filling in the early phase
(ischemia)
 EDI / SS OCT
Thick choroid means > 300 um or extrafoveal
localized choroidal thickness > 50 um more than
the subfoveal choroidal thickness
 OCTA
 OCT: Thick choroid and large choroidal vessels beneath elevations on RPE
and sub-RPE drusen like deposits. ICG : Choroidal Hyperpermability and
hyperpermeability co-existent with areas of RPE change. FAF (Fundus
Autofluorescence): Granular and mixed stippled hyper-
hypoautofluorescence.
 Form fruste CSR = no SRF
 CSR = SRF +
pachychoroid
 In children:
steroid induced
 Focal or
multifocal
 Common in
middle Aged male
 RPE dysfunction
 Choroidal autoregulation problem
 H – Pylori infection
 FA: expansible dot (ink dot or smoke stalk)
 Chronic CSR: persistent fluid 3-6 m that gets thick
due to severe RPE dysfunction
 ± cystoid macular degeneration (if there is defect in
the ELM)
 Gattering : RPE changes + tracts of SRF by
gravity (chronic cases).
 Causes of retinal and SRF
• Masquarede
• Vitreous
• Retinal vascular
• Optic N
• Choroid
• CSF
1- Stop steroids / smoke / .. and stress
& observe for 4 -6 months.
2- Early treatment in one eyed/ high visual needs
3- Extrafoveal : Thermal laser to the leaking point or
micropulse laser (problem of thermal laser that it
increases the risk of CNV)
4- have dose vetovefrin photodynamic therapy: treats the
mechanism by decreasing choroidal permeabilty
5- Systemic drugs : Spironelactone (mineralocorticoid
antagonist)/ finasteride / mifepristone (glucocorticoid
antagonist)
6- Antivegf : No role in CSR except if complicated with
CNV.
 Type 1 CNV + features of pachychoroid.
 OCT : Double layer sign ( shallow irregular
separation of RPE from Bruch’s membrane ) +
pachy vessels
 DD from AMD : Absent soft drusen / reduced
fundus tesselation.
 Common in Asian patients with AMD
 A network of branching choroidal vessels
with terminal, polyp-like aneurysmal
dilations associated with serous and
hemorrhagic detachments of the retinal
pigment epithelium (RPE)
 TTT :
Intravitreal Anti-VEGF with or without
adjuvant vPTD.
Orange-reddish bulb-like lesions budding from
choroid into the subretinal space.
recurrent and significant hemorrhagic and
exudative detachments of retina and retinal
pigment epithelium (RPE).
Hard exudates are also common.
On occasions, patients may present with
break-through vitreous hemorrhage.
 Nodular (elevated) appearance of the polyp
on stereoscopic viewing / Pulsatile filling of
polyps
 Hypofluorescent halo around the nodule
 Abnormal vascular channel(s) supplying the
polyps
 Orange subretinal nodules corresponding to
the hyperfluorescent area on ICGA
 Massive submacular haemorrhage (>4 disc
areas).
 Subretinal fluid +/ intraretinal cystoid spaces
 Pigment epithlial detachment (PED) may have fluid
or hemorrhage beneath it.
 Notched PED- a high PED connected with PED/s of
lower height on the sides- wavy border of PEDs-
corrugated or bumpy appearance[ M shaped or QRS
complex shaped PEDs
 The double layer sign is seen at the margin of PED
elevation. It may be caused by either separation of
RPE from the Bruch's membrane or separation of
Bruch's membrane from the Choroid.
 PCV lesions on FA resemble occult CNVM lesions
and when submacular, they can be mistaken
for AMD. A peripheral notch at the margin of a
serous pigment epithelial detachment may give
a clue to the location of the polyp. RPE
atrophy is manifested as window defects.
Subretinal or sub-retinal pigment epithelial
hemorrhage gives block fluorescence.
 FA also helps to rule out other differentials
including CNV, microaneurysms and retinal
angiomatous proliferans.
 Localized area of choroidal excavation
without underlying staphyloma or scleral
thining.
 Moderate myope with mild blurring of vision
or metamorphopsia
 O/E : reduced fundus tesselation.
 Can complicate with optic nerve edema
Choroidal Disorders: Pachychoroid Disease and Polypoidal Choroidal Vasculopathy

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Choroidal Disorders: Pachychoroid Disease and Polypoidal Choroidal Vasculopathy

  • 2.  ↑ Thickness of choroidal veins (haller layer)  Attenuation of choroidal capillaries (sattler layer)  Choroidal hyperpermeabilty  Progressive RPE dysfunction.  ± Thick choroid
  • 3.  Hypertension  Choroidal ischemia  Unknown
  • 4.  Type A Personality, emotional stress,  pregnancy,  tobacco use,  corticosteroids,  sympathomimetics.  herbal supplements.
  • 5.  CSCR  Pachychoroid pigment epitheliopathy  Polypoyoid choroid vasculopathy  Pachychoroid neovasculopathy  Pachychoroid peripapillary vasculopathy  Choroidal excavation  Concave macula
  • 6.  ICG : dilated choroidal vessels ( pachy vessels: don’t taper towards the posterior pole)+ leak + delayed arterial filling in the early phase (ischemia)  EDI / SS OCT Thick choroid means > 300 um or extrafoveal localized choroidal thickness > 50 um more than the subfoveal choroidal thickness  OCTA
  • 7.  OCT: Thick choroid and large choroidal vessels beneath elevations on RPE and sub-RPE drusen like deposits. ICG : Choroidal Hyperpermability and hyperpermeability co-existent with areas of RPE change. FAF (Fundus Autofluorescence): Granular and mixed stippled hyper- hypoautofluorescence.  Form fruste CSR = no SRF
  • 8.  CSR = SRF + pachychoroid  In children: steroid induced  Focal or multifocal  Common in middle Aged male
  • 9.  RPE dysfunction  Choroidal autoregulation problem  H – Pylori infection
  • 10.  FA: expansible dot (ink dot or smoke stalk)  Chronic CSR: persistent fluid 3-6 m that gets thick due to severe RPE dysfunction  ± cystoid macular degeneration (if there is defect in the ELM)
  • 11.  Gattering : RPE changes + tracts of SRF by gravity (chronic cases).
  • 12.  Causes of retinal and SRF • Masquarede • Vitreous • Retinal vascular • Optic N • Choroid • CSF
  • 13. 1- Stop steroids / smoke / .. and stress & observe for 4 -6 months. 2- Early treatment in one eyed/ high visual needs 3- Extrafoveal : Thermal laser to the leaking point or micropulse laser (problem of thermal laser that it increases the risk of CNV) 4- have dose vetovefrin photodynamic therapy: treats the mechanism by decreasing choroidal permeabilty 5- Systemic drugs : Spironelactone (mineralocorticoid antagonist)/ finasteride / mifepristone (glucocorticoid antagonist) 6- Antivegf : No role in CSR except if complicated with CNV.
  • 14.  Type 1 CNV + features of pachychoroid.  OCT : Double layer sign ( shallow irregular separation of RPE from Bruch’s membrane ) + pachy vessels  DD from AMD : Absent soft drusen / reduced fundus tesselation.
  • 15.  Common in Asian patients with AMD  A network of branching choroidal vessels with terminal, polyp-like aneurysmal dilations associated with serous and hemorrhagic detachments of the retinal pigment epithelium (RPE)  TTT : Intravitreal Anti-VEGF with or without adjuvant vPTD.
  • 16. Orange-reddish bulb-like lesions budding from choroid into the subretinal space. recurrent and significant hemorrhagic and exudative detachments of retina and retinal pigment epithelium (RPE). Hard exudates are also common. On occasions, patients may present with break-through vitreous hemorrhage.
  • 17.
  • 18.  Nodular (elevated) appearance of the polyp on stereoscopic viewing / Pulsatile filling of polyps  Hypofluorescent halo around the nodule  Abnormal vascular channel(s) supplying the polyps  Orange subretinal nodules corresponding to the hyperfluorescent area on ICGA  Massive submacular haemorrhage (>4 disc areas).
  • 19.  Subretinal fluid +/ intraretinal cystoid spaces  Pigment epithlial detachment (PED) may have fluid or hemorrhage beneath it.  Notched PED- a high PED connected with PED/s of lower height on the sides- wavy border of PEDs- corrugated or bumpy appearance[ M shaped or QRS complex shaped PEDs  The double layer sign is seen at the margin of PED elevation. It may be caused by either separation of RPE from the Bruch's membrane or separation of Bruch's membrane from the Choroid.
  • 20.
  • 21.  PCV lesions on FA resemble occult CNVM lesions and when submacular, they can be mistaken for AMD. A peripheral notch at the margin of a serous pigment epithelial detachment may give a clue to the location of the polyp. RPE atrophy is manifested as window defects. Subretinal or sub-retinal pigment epithelial hemorrhage gives block fluorescence.  FA also helps to rule out other differentials including CNV, microaneurysms and retinal angiomatous proliferans.
  • 22.  Localized area of choroidal excavation without underlying staphyloma or scleral thining.  Moderate myope with mild blurring of vision or metamorphopsia  O/E : reduced fundus tesselation.
  • 23.  Can complicate with optic nerve edema