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Idiopathic Polypoidal Choroidal Vasculopathy (PCV)

ROUTINE — Retina

Symptoms

decreased central vision (sudden or gradual)

sudden vision loss often from massive submacular hemorrhage

metamorphopsia and central scotoma

recurrent serous or hemorrhagic pigment epithelial detachments (ped)

Clinical Findings

Clinical hallmarks

  • multiple large orange-red subretinal nodules (polyps)
  • branching inner-choroidal vascular network (bvn)
  • massive sub-rpe or subretinal hemorrhage (cardinal differentiator from typical wet amd)
  • absence of large soft drusen or geographic atrophy
  • common in asian and african-american populations; occurs at a younger age than typical amd

Icg angiography (gold standard)

  • punctate, focal hyperfluorescent nodules ("salmon-pink popcorn")
  • bvn visible in early phases; polyps may show dynamic pulsation

OCT signs

  • "thumb-like" ped: sharp, dome-shaped sub-rpe elevation representing a polyp
  • double-layer sign: shallow irregular separation of rpe from bruch membrane (the bvn)
  • peaked or notched peds

Pachychoroid spectrum

  • thickened choroid with dilated haller vessels (pachyvessels)
  • related to cscr and pachychoroid neovasculopathy

Etiology

Pathogenesis

  • abnormal dilated choroidal vascular network with aneurysmal terminal dilations (polyps)
  • part of the pachychoroid disease spectrum
  • high association with systemic hypertension

Red Flags

massive subretinal or suprachoroidal hemorrhage

recurrent exudation despite anti-vegf therapy

sudden metamorphopsia in a patient with a history of hypertension and pigmented skin

unresponsive "wet amd" in asian or african-american patients

Investigations

Imaging

  • icg angiography (icga): mandatory gold standard to visualize polyps and bvn
  • ivfa: often misleadingly suggests "occult cnv"
  • oct: monitor for subretinal fluid, thumb-like peds, and double-layer sign
  • octa: non-invasive detection of bvn flow signal

Clinical exam

  • biomicroscopy for orange-red nodules and subretinal lipid/hemorrhage

Treatment

First-line therapy

  • anti-vegf monotherapy (aflibercept, faricimab, brolucizumab): effective for exudation
  • combination therapy (everest-ii/planet trials): pdt with verteporfin + anti-vegf for superior polyp closure

Hemorrhage management

  • pneumatic displacement: intravitreal tpa + gas (sf6/c3f8)
  • pars plana vitrectomy with subretinal tpa for severe bleeds

Local destruction

  • thermal laser photocoagulation for extrafoveal polyps

Clinical Pearl

In pigmented patients, "wet AMD" that does not respond to anti-VEGF is usually polypoidal choroidal vasculopathy — ICG angiography makes the diagnosis and combined PDT plus anti-VEGF is often the answer.

Differential Diagnosis

  • neovascular age-related macular degeneration (amd)
  • central serous chorioretinopathy (cscr)
  • peripheral exudative hemorrhagic chorioretinopathy (pehcr)

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