Peripheral Exudative Hemorrhagic Chorioretinopathy (PEHCR)

Key points at a glance

Key Points at a Glance

• PEHCR is a hemorrhagic and exudative lesion in the temporal peripheral retina of elderly individuals, first reported by Annseley in 1980 with 27 cases.
• More than 75% of lesions occur on the temporal side, and vitreous hemorrhage complicates 25–100% of cases.
• Many cases are asymptomatic and tend to regress spontaneously.
• It is considered part of the pachychoroid spectrum, similar to age-related macular degeneration and polypoidal choroidal vasculopathy.
• Because it presents as an elevated pigmented lesion, differentiation from choroidal melanoma is the most important clinical challenge.
• Peripheral type 1 CNV (choroidal neovascularization) has been histologically proven as the underlying pathology.
• Hypertension and anticoagulant use are major risk factors, with anticoagulant use rates as high as 61–73%.

1. What is Peripheral Exudative Hemorrhagic Chorioretinopathy (PEHCR)?

Peripheral Exudative Hemorrhagic Chorioretinopathy (PEHCR) is a disease that causes exudative and hemorrhagic lesions in the peripheral retina and choroid of elderly individuals. The disease concept was established in 1980 when Annseley first reported 27 cases ¹⁾.

This disease has long been treated as an independent disease entity, but in recent years it is being reclassified as part of the pachychoroid spectrum ⁴⁾. It shares similar pathology with age-related macular degeneration (AMD) and polypoidal choroidal vasculopathy (PCV).

Epidemiologically, it is common in the elderly, and because its course is slow, it is not uncommon for large lesions to have already formed at the time of discovery. Since it forms elevated pigmented mass-like lesions, there is a risk of misdiagnosis as choroidal melanoma, making differentiation the most important clinical challenge ²⁾.

Q How rare is PEHCR?

A

Reported cases are still few; the initial report by Annesley in 1980 described 27 cases ¹⁾. Although it is a rare disease, it is thought that there are also asymptomatic cases that go unnoticed.

2. Main Symptoms and Clinical Findings

Subjective Symptoms

Most cases of PEHCR are asymptomatic and are often discovered incidentally during health checkups or examinations for other diseases ²⁾.

• Asymptomatic: The majority of cases lack subjective symptoms. This is because the lesion is confined to the peripheral fundus.
• Visual impairment: Occurs when the lesion extends to the macula or when massive vitreous hemorrhage complicates.
• Floaters: May be noticed when vitreous hemorrhage occurs.
• Photopsia and metamorphopsia: Rarely observed.

Vitreous hemorrhage (VH) is observed in 25–100% of cases ¹⁾. Depending on the amount of bleeding, fundus examination may become difficult.

Clinical Findings

Distribution of Lesions

Temporal predominance: More than 75% of lesions occur in the temporal peripheral fundus. The temporal-to-nasal ratio has been reported as 31:14 ¹⁾.

Peripheral localization: Lesions form on the peripheral side of the equator.

Morphology of Lesions

Hemorrhagic and exudative elevation: Lesions elevate beneath the choroid and RPE, containing blood and exudate. They appear as white to orange-yellow elevated lesions ²⁾.

Serpiginous RPE tear: Characteristic serpiginous RPE tears may form due to traction from fibrovascular tissue ³⁾.

• Vitreous hemorrhage: Observed in 25–100% of cases ¹⁾. Massive bleeding makes fundus examination difficult.
• Retinal pigment epithelium (RPE) tear: Characteristically serpentine in shape, reflecting the fibrovascular nature ³⁾.
• Exudative retinal detachment: May occur adjacent to the lesion.

Q Does PEHCR occur bilaterally?

A

Most cases are unilateral, but bilateral cases have also been reported. If found in one eye, examination of the fellow eye is also important.

3. Causes and Risk Factors

The pathogenesis of PEHCR is not fully understood, but age-related degeneration of the peripheral choroid is thought to be the underlying cause ²⁾.

• Advanced age: The greatest risk factor. It predominantly occurs in older individuals.
• Hypertension: Thought to be involved as a background factor promoting vascular changes ^{1, 3)}.
• Use of anticoagulants or antiplatelet agents: Confirmed in 61–73% of cases ^{1, 3)}, potentially contributing to the expansion and prolongation of bleeding.
• Age-related choroidal degeneration: Underlying peripheral choroidal vascular abnormalities against a background of pachychoroid ⁴⁾.

Precautions in Daily Life

• If you are taking anticoagulants or antiplatelet agents, please see an ophthalmologist promptly if you experience sudden vision changes or worsening floaters.
• Regular fundus examinations may detect the condition at an asymptomatic stage.
• Management of hypertension is important for the prevention of ocular vascular diseases in general.

4. Diagnosis and Examination Methods

The most important differential diagnosis in PEHCR is choroidal melanoma. Because the appearance of a raised pigmented lesion is similar, a detailed examination combining multiple modalities is essential ³⁾.

B-scan ultrasonography

This examination plays a central role in differentiating PEHCR from melanoma.

The following three points are the main findings that differentiate PEHCR from melanoma ³⁾:

Finding	PEHCR	Melanoma
Choroidal excavation	Negative	Positive
Tumor pulsation	Negative	Positive
Internal echo	With gaps	Homogeneous, low brightness

The absence of choroidal excavation and the presence of internal space echoes are considered characteristic of PEHCR ³⁾. Intratumoral pulsation, which is characteristic of melanoma, is not observed in PEHCR ³⁾.

Indocyanine Green Angiography (ICGA)

A pachychoroid pattern (dilation of large choroidal vessels and atrophy of the inner choroid) and peripheral polypoidal lesions may be observed ³⁾. This finding is important as it supports the presence of peripheral lesions similar to PCV.

OCT / OCTA

• OCT: Allows detailed evaluation of sub-RPE fluid accumulation, fibrovascular elevation, and RPE tear morphology ²⁾.
• OCTA: Enables non-invasive assessment of CNV presence and vascular architecture ²⁾. It is particularly useful for visualizing type 1 CNV.

Fluorescein Angiography (FA)

It is used to evaluate exudation and leakage patterns, and for comparison with age-related macular degeneration and PCV.

Q How is differentiation from melanoma performed?

A

B-scan ultrasonography is most important, confirming the presence or absence of choroidal excavation and intratumoral pulsation ³⁾. Pachychoroid pattern on ICGA and sub-RPE fluid accumulation or fibrovascular changes on OCT also support PEHCR. If diagnosis is difficult, further examination at a specialized facility is recommended.

5. Standard Treatment

There is no established standard treatment for PEHCR, and since many cases show spontaneous regression, observation is the basic approach ²⁾.

Observation

Spontaneous regression: Many cases show a tendency for the lesion to shrink or regress spontaneously. Regular fundus examination and imaging evaluation should be continued ²⁾.

Pharmacotherapy

Anti-VEGF therapy: Performed for cases with confirmed CNV activity. Administered as an intravitreal injection into the vitreous cavity ¹⁾.

Photodynamic therapy (PDT): Performed for choroidal vascular abnormalities and polypoidal lesions ^{1, 3)}.

Surgical Treatment

Transscleral drainage: A surgical option for massive subretinal or sub-RPE hemorrhage ¹⁾.

Intravitreal resection: A new approach reported for refractory cases ²⁾.

Transscleral Drainage

This is a surgical method reported for cases with massive hemorrhagic lesions causing vision loss.

Kuraishi T et al. (2024) performed transscleral drainage in a PCV-associated PEHCR case (70-year-old male) by performing 25G PPV, then choroidal puncture with an argon laser (200 mW) 11 mm posterior to the corneal limbus, and draining fluid under IOP of 25 mmHg compression ¹⁾. The procedure involved moving the hemorrhage from the posterior pole to the periphery using perfluorocarbon liquid (PFCL), then draining via choroidal puncture, and finishing with C3F8 gas tamponade.

Intravitreal Resection

This is a surgical technique aimed at direct removal of subretinal fibrovascular tissue, reported in recent years.

Kase S et al. (2025) performed intravitreal resection using 25G PPV for the first time in a 76-year-old female ²⁾. After posterior capsulectomy, tissue removal was performed using diathermy and forceps, and postoperative pathological examination contributed to elucidating the pathogenesis of this disease.

Precautions in Treatment

• Surgery during anticoagulant therapy increases bleeding risk. Preoperative medication management is important.
• Many cases stabilize with observation alone, but prompt specialist evaluation is necessary when vision loss or increased bleeding occurs.
• There is no established treatment protocol; the choice of treatment is based on the severity and complications of each individual case.

6. Pathophysiology and Detailed Mechanisms

Role of Peripheral Type 1 CNV

The fundamental pathology of PEHCR is thought to be peripheral type 1 choroidal neovascularization (CNV).

Kase S et al. (2025) performed immunohistochemical staining of excised tissue and confirmed AE1/AE3-positive RPE cells and CD34/α-SMA-positive vascular wall components ²⁾. This provided the first histopathological evidence of peripheral type 1 CNV in PEHCR.

Type 1 CNV forms beneath the RPE and proliferates insidiously without destroying the RPE. In the periphery, fluid and blood leaking from these new vessels accumulate under the RPE and retina, forming elevated lesions ²⁾.

Transluminal Pressure Difference and Bleeding Mechanism

When the transluminal pressure difference between the inside and outside of peripheral choroidal vessels increases, bleeding from fragile new vessels is more likely to occur ²⁾. Use of anticoagulants is thought to amplify this bleeding tendency.

Relationship with the Pachychoroid Spectrum

PEHCR is considered to have a background of pachychoroid pathology and is positioned within the following spectrum ⁴⁾.

Disease	Features	Location
CSC	Sub-RPE fluid accumulation	Macula
PCV	Polypoidal choroidal vasculopathy	Macula to posterior pole
PEHCR	Peripheral CNV and hemorrhage	Periphery

In pachychoroid, the large choroidal vessels (Haller layer) are dilated, and the inner choroid (Sattler layer and choriocapillaris) becomes atrophic. This change is thought to lead to inner layer ischemia and stress on the RPE, promoting CNV formation ⁴⁾.

In the case by Maitray A et al. (2021), ICGA confirmed pachyvessels and peripheral polyps ³⁾, supporting the association between pachychoroid and PEHCR.

Mechanism of RPE tear formation

Serpentine-shaped RPE tears are characteristic of PEHCR. It is thought that traction from fibrovascular tissue and increased pressure from sub-RPE fluid act together to cause RPE rupture ³⁾. The presence of polypoidal lesions on ICGA may contribute to this tear formation ³⁾.

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7. Latest research and future perspectives (research-stage reports)

For patients: Please read

The following content is currently at the research stage or under clinical trial and is not standard treatment available at general hospitals. It is reference information for professionals regarding future medical developments.

Histopathological elucidation via vitrectomy

The report by Kase S et al. (2025) is the world’s first record of vitreous cavity resection for PEHCR and also the first paper to histologically prove peripheral type 1 CNV using excised tissue ²⁾. Immunostaining for AE1/AE3, CD34, and α-SMA confirmed RPE cells and new blood vessel walls, providing new insights into the understanding of the pathology.

Technical advances in transscleral drainage

For refractory cases with massive sub-RPE and subretinal hemorrhage, transscleral drainage is becoming established as an effective means of visual function recovery ¹⁾. Standardization of drainage technique using argon laser choroidal puncture combined with PPV is a future challenge.

Clinical significance of serpentine RPE tear

Maitray A et al. (2021) suggested that serpentine RPE tears originate from fibrovascular tissue ³⁾, emphasizing the role of the fibrovascular component in PEHCR lesions. This finding may have future implications for determining the indication of anti-VEGF therapy and PDT.

Reorganization of disease classification

Shroff D et al. (2021) positioned PEHCR as a peripheral type of the pachychoroid spectrum ⁴⁾. If this classification is established, systematic application of treatment strategies (anti-VEGF, PDT) common to age-related macular degeneration and PCV will become possible, contributing to the construction of treatment evidence.

Q What kind of specialized institution can diagnose and treat PEHCR?

A

Consultation at an ophthalmology facility specializing in retinal and choroidal diseases is recommended. Since differentiation from melanoma requires extensive experience, consultation with a specialized facility handling ocular tumors may also be useful.

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8. References

1. Kuraishi T, Kawamura H, Saito I, Sakurai T. Transscleral drainage to treat peripheral exudative hemorrhagic chorioretinopathy caused by retinal pigment epithelial hemorrhage. Am J Ophthalmol Case Rep. 2024;33:101977.
2. Kase S, Shimizu A, Ishida S. Transvitreal endoresection of peripheral exudative hemorrhagic chorioretinopathy: a clinicopathological study. BMC Ophthalmol. 2025;25:233.
3. Maitray A, Kohli P, Babu N. Serpentine retinal pigment epithelial tear. Taiwan J Ophthalmol. 2021;11:321-324.
4. Shroff D, Sharma M, Chhablani J, et al. Peripheral exudative hemorrhagic chorioretinopathy—a new addition to the spectrum of pachychoroid disease. Retina. 2021;41:1518-1525.