Peripheral Exudative Hemorrhagic Chorioretinopathy is the reason some healthy eyes are nearly removed for a cancer that was never there.

It produces a dark, elevated mass in the far periphery of the fundus, packed with blood and lipid, that looks unnervingly like a choroidal melanoma.

Yet it is a degenerative, self-limiting condition of the elderly retina, not a tumour.

For clinicians, recognizing Peripheral Exudative Hemorrhagic Chorioretinopathy (PEHCR) matters because the alternative—an unnecessary enucleation—is catastrophic and avoidable.

Any peripheral haemorrhagic lesion in an older patient deserves the question: is this a melanoma, or is it PEHCR?

Peripheral Exudative Hemorrhagic Chorioretinopathy (PEHCR): Optos ultra-widefield colour fundus of the left eye showing an elevated dark peripheral chorioretinal lesion simulating a choroidal tumour

What Is Peripheral Exudative Hemorrhagic Chorioretinopathy?

PEHCR is a degenerative disorder of the peripheral retina and choroid characterized by sub-retinal and sub-RPE haemorrhage and exudation.

Its defining features are:

  • An elevated, often dark peripheral lesion, usually temporal
  • Sub-retinal or sub-RPE haemorrhage with lipid exudation
  • A strong association with age-related macular degeneration
  • A tendency to resolve, leaving pigmentary and fibrotic scarring

It is best understood as a peripheral counterpart of neovascular AMD, driven by abnormal choroidal vessels far from the macula.

Epidemiology

The condition is a disease of the older eye.

  • Typically affects patients in their seventies and eighties
  • More common in women
  • Bilateral in roughly a third of cases
  • Frequently associated with macular AMD in the same or fellow eye

Because the lesions sit in the far periphery, many are found incidentally on routine or widefield examination.

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Pathophysiology

The mechanism parallels that of neovascular macular disease.

  • Abnormal peripheral choroidal neovascular or polypoidal vessels develop, often on a background of RPE and Bruch membrane degeneration
  • These fragile vessels leak and bleed beneath the RPE and retina
  • Blood and lipid accumulate to form an elevated, tumour-like lesion
  • Over time the haemorrhage organizes into fibrosis and pigment

It is this combination of elevation, dark blood, and lipid that so convincingly imitates a pigmented tumour.

Clinical Presentation

Symptoms

Most patients have few or no symptoms.

  • Often completely asymptomatic and found incidentally
  • Painless visual loss if haemorrhage or exudate reaches the macula
  • Floaters or sudden loss if the lesion bleeds into the vitreous

Examination Findings

The appearance is dramatic but characteristic.

  • An elevated peripheral lesion, usually temporal, with dark sub-retinal blood
  • Lipid exudation and sub-retinal fluid around the lesion
  • Sub-RPE haemorrhage that may appear almost black
  • Associated drusen and RPE changes at the macula
  • Occasionally vitreous haemorrhage obscuring the view

Peripheral Exudative Hemorrhagic Chorioretinopathy (PEHCR): widefield fundus montage showing a peripheral lesion with sub-retinal haemorrhage, lipid exudation and pigment, mimicking a choroidal melanoma

A peripheral haemorrhagic lesion with lipid, in an elderly eye that also shows macular AMD, is far more likely to be PEHCR than melanoma.

Why PEHCR Is So Often Mistaken for Melanoma

The confusion is understandable and follows a familiar pattern.

  • The lesion is elevated, dark, and dome-shaped, exactly like a pigmented tumour
  • It sits in the periphery, where it is hard to examine and image
  • Sub-RPE blood can look solid and mass-like on cursory examination
  • Fear of missing a melanoma pushes clinicians toward aggressive assumptions

In a large series, a substantial number of eyes referred as suspected choroidal melanoma were in fact PEHCR—underlining how often the two are confused.

Diagnostic Evaluation

Imaging is what separates a benign haemorrhage from a true tumour.

Ultrasonography

  • B-scan typically shows haemorrhage and a dome of sub-RPE blood rather than a solid, acoustically hollow mass
  • Melanoma classically shows low internal reflectivity and choroidal excavation, which PEHCR lacks

Angiography and OCT

  • Fluorescein and indocyanine green angiography may reveal peripheral polypoidal or neovascular lesions
  • Blocked fluorescence from blood, rather than a vascularized tumour, supports PEHCR
  • OCT of the lesion, where reachable, shows haemorrhagic RPE detachment

The combination of a haemorrhagic, non-solid lesion on ultrasound and bilateral peripheral disease strongly favours PEHCR.

Differential Diagnosis

Several elevated peripheral lesions enter the differential:

  • Choroidal melanoma — the critical distinction, favouring a solid, acoustically hollow mass
  • Choroidal melanocytoma and other pigmented lesions
  • Choroidal metastasis and choroidal haemangioma
  • Retinal vasoproliferative tumour
  • Peripheral neovascular AMD and Coats disease

Bilaterality, coexisting macular AMD, and haemorrhage without a solid mass are the features that point away from melanoma.

Management

Most lesions need confidence rather than intervention.

  • Observation is appropriate for asymptomatic peripheral lesions that are not threatening the macula
  • Intravitreal anti-VEGF is used when there is macular exudation or fluid
  • Laser photocoagulation or cryotherapy can treat active peripheral leakage
  • Vitrectomy is reserved for non-clearing vitreous haemorrhage
  • The essential step is to confirm the diagnosis and avoid enucleation

Once melanoma has been excluded, the natural tendency of PEHCR to resolve makes conservative management the rule.

Prognosis

The outlook is generally favourable.

  • Peripheral lesions often resolve with pigmentary and fibrotic scarring
  • Central vision is preserved unless the macula is involved
  • The main threat is diagnostic—an eye lost to a mistaken cancer diagnosis

In practice, the whole value of knowing PEHCR is protecting a comfortable, seeing eye from unnecessary removal.

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References

  1. Shields CL, Salazar PF, Mashayekhi A, Shields JA. “Peripheral Exudative Hemorrhagic Chorioretinopathy Simulating Choroidal Melanoma in 173 Cases.” Ophthalmology. 2009;116(3):529–535.
  2. Annesley WH Jr. “Peripheral Exudative Hemorrhagic Chorioretinopathy.” Transactions of the American Ophthalmological Society. 1980;78:321–364.
  3. Mantel I, Uffer S, Zografos L. “Peripheral Exudative Hemorrhagic Chorioretinopathy: A Clinical, Angiographic, and Histologic Study.” American Journal of Ophthalmology. 2009;148(6):932–938.
  4. Reese AB, Jones IS. “Hematomas Under the Retinal Pigment Epithelium.” Transactions of the American Ophthalmological Society. 1962;60:43–79.
  5. American Academy of Ophthalmology. “Peripheral Exudative Hemorrhagic Chorioretinopathy.” EyeWiki.

Test yourself

A few questions straight from this article.

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  1. Question

    Peripheral exudative hemorrhagic chorioretinopathy (PEHCR) is best defined as which kind of disorder?

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    Answer

    B

    PEHCR is a degenerative disorder of the peripheral retina and choroid marked by sub-retinal and sub-RPE haemorrhage with lipid exudation, not a tumour.