Pachychoroid neovasculopathy (PNV) is defined by which combination of findings?

Type 1 choroidal neovascularisation in a thickened choroid PNV sits within the pachychoroid spectrum and is defined by type 1 CNV arising in eyes with a thickened choroid, pachyvessels and RPE changes.

Type 2 neovascularisation with large soft drusen

Retinal angiomatous proliferation with intraretinal cysts

Peripapillary neovascularisation with a thin choroid

Which mechanism drives neovascularisation in pachychoroid neovasculopathy (PNV)?

Dilated Haller vessels compressing the choriocapillaris Dilated outer choroidal vessels compress the overlying choriocapillaris, producing hypoxia and RPE stress that promote type 1 CNV.

Vitreous traction on the foveal photoreceptors

Occlusion of a branch retinal arteriole

Autoimmune destruction of the retinal pigment epithelium

Which subfoveal choroidal thickness on OCT supports pachychoroid neovasculopathy (PNV)?

Greater than 300 micrometres Increased subfoveal choroidal thickness above about 300 micrometres, with pachyvessels, is a core OCT criterion for pachychoroid disease.

Which feature separates pachychoroid neovasculopathy from neovascular age-related macular degeneration?

Drusen are absent or minimal in PNV Unlike typical AMD, PNV eyes show absent or minimal drusen, which points to a different pathogenic mechanism.

Subretinal fluid is present in PNV only

Visual acuity is always severely reduced

Fluorescein angiography shows no leakage at all

Which finding argues against polypoidal choroidal vasculopathy in an eye with pachychoroid features?

Absence of polypoidal lesions on angiography Pachychoroid features without the polyps seen in polypoidal choroidal vasculopathy favour a diagnosis of PNV.

Increased subfoveal choroidal thickness

Choroidal hyperpermeability on ICGA

Irregular elevation of the pigment epithelium

What is the first-line treatment for pachychoroid neovasculopathy (PNV)?

Intravitreal anti-VEGF therapy Intravitreal anti-VEGF agents such as ranibizumab, aflibercept or bevacizumab are first line and usually give anatomical and visual improvement.

Oral carbonic anhydrase inhibitors

Pars plana vitrectomy with membrane peel

Systemic corticosteroid therapy

When is photodynamic therapy considered in pachychoroid neovasculopathy (PNV)?

In chronic or recurrent disease, or inadequate anti-VEGF response PDT is reserved for chronic or recurrent cases and where anti-VEGF monotherapy is insufficient, and may be combined with anti-VEGF.

As the initial treatment in every new case

Only when the fellow eye becomes involved

Whenever choroidal thickness exceeds normal limits

Which imaging modality best delineates the neovascular network in pachychoroid neovasculopathy (PNV)?

OCT angiography OCT angiography demonstrates the neovascular network in the normally avascular outer retina and is particularly helpful in defining the CNV complex.

Standard colour fundus photography

Which demographic profile is typical for pachychoroid neovasculopathy (PNV)?

Men aged 50 to 70 with unilateral symptoms PNV is usually seen in middle-aged to elderly patients between 50 and 70, with a male predominance and typically unilateral presentation.

Children with a strong family history

Highly myopic women in their twenties

Women over 80 with bilateral disease

What is the visual prognosis of pachychoroid neovasculopathy (PNV) treated early?

Generally favourable, though recurrence is possible Most patients respond well to anti-VEGF with reduced subretinal fluid and stable or improved acuity, but recurrence and chronic RPE damage with fibrosis can occur.

Uniformly poor with rapid central blindness

Complete cure with no need for follow-up

Unaffected by whether treatment is given

Pachychoroid Neovasculopathy: A Deep Dive into a Choroidal Disease

Case Study

A 63-year-old male presented with complaints of gradual central vision distortion and blurred vision in his left eye over the past four months.

He denied any history of systemic diseases or previous ocular trauma. His best-corrected visual acuity was 20/30 in the affected eye.

Fundus examination revealed pigmentary changes at the macula with subretinal fluid. Optical coherence tomography (OCT) showed pachychoroid features, including increased choroidal thickness and dilated Haller’s layer vessels.

Fundus fluorescein angiography (FFA) and indocyanine green angiography (ICGA) revealed type 1 choroidal neovascularization (CNV).

A diagnosis of Pachychoroid Neovasculopathy (PNV) was made. The patient received intravitreal anti-VEGF therapy with noticeable anatomical and functional improvement.

Disease Entity

Pachychoroid Neovasculopathy (PNV) is a relatively recent and distinct clinical entity within the pachychoroid disease spectrum.

It is characterized by type 1 choroidal neovascularization (CNV) occurring in eyes with a thickened choroid and other features typical of pachychoroidopathies, such as pachyvessels, attenuation of the choriocapillaris, and retinal pigment epithelium (RPE) changes.

Test your knowledge 10 questions from this article

Pathophysiology

PNV arises due to chronic choroidal thickening and congestion that leads to RPE stress and subsequent breakdown of Bruch’s membrane.

This chronic stress promotes the development of type 1 CNV beneath the RPE.

The hallmark of pachychoroid spectrum diseases is the presence of dilated outer choroidal vessels (Haller’s layer), which compress the overlying choriocapillaris, potentially leading to hypoxia and neovascularization.

Unlike typical age-related macular degeneration (AMD), drusen are absent or minimal in PNV, suggesting a different pathogenic mechanism.

Epidemiology

PNV is most commonly seen in middle-aged to elderly individuals, often between the ages of 50 and 70.

There is a male predominance, and patients typically present with unilateral symptoms, although bilateral involvement can occur.

It may evolve from other pachychoroid disorders, such as chronic central serous chorioretinopathy (CSCR).

Clinical Features

Patients with PNV often present with:

Central vision distortion (metamorphopsia)
Blurred vision
Relative preservation of visual acuity initially

On fundus examination:

Subretinal fluid
RPE changes
Absence of soft drusen

OCT imaging is critical and typically reveals:

Increased subfoveal choroidal thickness (>300 µm)
Pachyvessels
Type 1 CNV
Irregular RPE elevation
Subretinal fluid

ICGA may show hyperpermeability and dilated choroidal vessels, while FFA reveals leakage from CNV.

Examination Findings

Visual Acuity: Mildly to moderately reduced
OCT: Subretinal fluid, pachychoroid features, flat, irregular pigment epithelial detachment
FFA: Late leakage suggestive of CNV
ICGA: Dilated choroidal vessels with hyperpermeability
OCT-A: Demonstrates the neovascular network in the avascular outer retina

Differential Diagnosis

Neovascular (wet) AMD
Polypoidal choroidal vasculopathy (PCV)
Chronic central serous chorioretinopathy (CSCR)
Idiopathic CNV
Pattern dystrophies

Distinguishing features include the absence of drusen and the presence of pachychoroid features without the polyps seen in PCV.

Diagnosis

The diagnosis is based on multimodal imaging findings that include:

Type 1 CNV
Pachychoroid features on OCT (increased choroidal thickness, pachyvessels)
No or minimal drusen
ICGA showing choroidal hyperpermeability

OCT-Angiography is particularly helpful in delineating the CNV complex.

Management

Intravitreal Anti-VEGF Therapy:

First-line treatment (e.g., ranibizumab, aflibercept, bevacizumab)
Typically results in anatomical and visual improvement

Photodynamic Therapy (PDT):

Considered in chronic or recurrent cases or when anti-VEGF monotherapy is insufficient
Can be used in combination with anti-VEGF for a synergistic effect

Monitoring:

Regular follow-up with OCT to monitor CNV activity
Adjust treatment interval based on response (treat and extend approach)

Prognosis

With early and appropriate treatment, the prognosis of PNV is generally favorable. Many patients respond well to anti-VEGF therapy, with a reduction in subretinal fluid and stabilization or improvement in visual acuity.

However, recurrence is possible, and some patients may develop chronic disease with RPE damage and fibrosis.

Prevention

As PNV is not associated with traditional AMD risk factors like smoking or drusen, there are no established preventive measures. However:

Regular follow-up for patients with pachychoroid features
Prompt treatment of CSCR
Avoidance of corticosteroid use when not necessary (due to CSCR risk)

References

Pang CE, Freund KB. Pachychoroid neovasculopathy. Retina. 2015 Jan;35(1):1-9.
Dansingani KK, Balaratnasingam C, Freund KB. Understanding aneurysmal type 1 neovascularization (polypoidal choroidal vasculopathy): A lesson in the taxonomy of “expanded spectra” – A review. Clin Exp Ophthalmol. 2018;46(2):189–200.
Miyake M, Ooto S, Yamashiro K, et al. Pachychoroid neovasculopathy and age-related macular degeneration. Sci Rep. 2015;5:16204.
Cheung CMG, Lee WK, Koizumi H, Dansingani K, Lai TYY, Freund KB. Pachychoroid disease. Eye. 2019;33(1):14–33.
Matsumoto H, Hiroe T, Imai H, et al. Clinical characteristics of pachychoroid neovasculopathy. Invest Ophthalmol Vis Sci. 2015;56(9):620–627.

Test yourself

A few questions straight from this article.

1 / 10 0 correct

Pachychoroid neovasculopathy (PNV) is defined by which combination of findings?
PNV sits within the pachychoroid spectrum and is defined by type 1 CNV arising in eyes with a thickened choroid, pachyvessels and RPE changes.
Which mechanism drives neovascularisation in pachychoroid neovasculopathy (PNV)?
Dilated outer choroidal vessels compress the overlying choriocapillaris, producing hypoxia and RPE stress that promote type 1 CNV.
Which subfoveal choroidal thickness on OCT supports pachychoroid neovasculopathy (PNV)?
Increased subfoveal choroidal thickness above about 300 micrometres, with pachyvessels, is a core OCT criterion for pachychoroid disease.
Which feature separates pachychoroid neovasculopathy from neovascular age-related macular degeneration?
Unlike typical AMD, PNV eyes show absent or minimal drusen, which points to a different pathogenic mechanism.
Which finding argues against polypoidal choroidal vasculopathy in an eye with pachychoroid features?
Pachychoroid features without the polyps seen in polypoidal choroidal vasculopathy favour a diagnosis of PNV.
What is the first-line treatment for pachychoroid neovasculopathy (PNV)?
Intravitreal anti-VEGF agents such as ranibizumab, aflibercept or bevacizumab are first line and usually give anatomical and visual improvement.
When is photodynamic therapy considered in pachychoroid neovasculopathy (PNV)?
PDT is reserved for chronic or recurrent cases and where anti-VEGF monotherapy is insufficient, and may be combined with anti-VEGF.
Which imaging modality best delineates the neovascular network in pachychoroid neovasculopathy (PNV)?
OCT angiography demonstrates the neovascular network in the normally avascular outer retina and is particularly helpful in defining the CNV complex.
Which demographic profile is typical for pachychoroid neovasculopathy (PNV)?
PNV is usually seen in middle-aged to elderly patients between 50 and 70, with a male predominance and typically unilateral presentation.
What is the visual prognosis of pachychoroid neovasculopathy (PNV) treated early?
Most patients respond well to anti-VEGF with reduced subretinal fluid and stable or improved acuity, but recurrence and chronic RPE damage with fibrosis can occur.