Choroidal Vasculopathy: Clinical Presentation, Diagnostic Evaluation, Management, and Outcomes – A Case Report

Abstract

Choroidal vasculopathy, commonly referred to as polypoidal choroidal vasculopathy (PCV), is a retinal disorder characterized by abnormal branching vascular networks and aneurysmal dilatations arising from the choroidal circulation beneath the retinal pigment epithelium. The condition is considered a subtype of neovascular age-related macular degeneration and is an important cause of vision loss, particularly in middle-aged and elderly individuals. Patients may present with blurred vision, metamorphopsia, recurrent subretinal hemorrhage, or sudden visual deterioration.

We present the case of a 64-year-old male who presented with progressive diminution of vision and distortion of images in the left eye. Multimodal retinal imaging including optical coherence tomography and indocyanine green angiography revealed features consistent with polypoidal choroidal vasculopathy. The patient underwent intravitreal anti-vascular endothelial growth factor therapy with significant anatomical and visual improvement during follow-up.

This case highlights the importance of early diagnosis, multimodal imaging, and timely retinal intervention in preventing irreversible visual loss associated with choroidal vasculopathy.

Introduction

Polypoidal choroidal vasculopathy is a chronic retinal vascular disorder involving abnormal choroidal blood vessels located beneath the retinal pigment epithelium. The disease is characterized by branching vascular networks with terminal aneurysmal or polyp-like dilatations that may leak fluid or bleed, leading to retinal damage and progressive visual impairment.

The condition most commonly affects individuals above 50 years of age and is more prevalent in Asian and African populations. Although the exact pathogenesis remains incompletely understood, several contributing factors have been identified, including:

• Age-related degeneration
• Choroidal vascular abnormalities
• Hypertension
• Smoking
• Genetic predisposition
• Chronic vascular endothelial dysfunction

Polypoidal choroidal vasculopathy may clinically resemble neovascular age-related macular degeneration, but it differs in disease behavior, imaging characteristics, and treatment response.

Patients may present with:

• Blurred central vision
• Metamorphopsia
• Scotomas
• Recurrent retinal hemorrhage
• Sudden visual loss

Without treatment, repeated leakage and hemorrhage can result in fibrosis, retinal scarring, and permanent vision loss. Early identification using advanced retinal imaging plays an important role in preserving visual function.

Case Report

Patient History

A 64-year-old male presented to the ophthalmology outpatient department with:

• Progressive blurring of vision in the left eye for 3 months
• Distortion of straight lines
• Difficulty reading
• Mild central visual shadow

The patient denied ocular trauma, redness, or pain.

Past medical history revealed:

• Hypertension for 12 years
• Type 2 diabetes mellitus
• Chronic smoking history
• Dyslipidemia

There was no previous history of retinal surgery or glaucoma.

Clinical Examination

General Examination

• Blood pressure: 150/90 mmHg
• Pulse rate: 84/min
• Afebrile
• Systemically stable

Ophthalmic Examination

Visual Acuity

• Right eye: 6/9
• Left eye: 6/36

Anterior Segment Examination

• Mild age-related cataract changes
• No active anterior segment inflammation

Fundus Examination

Left eye demonstrated:

• Orange-red subretinal nodular lesions
• Subretinal hemorrhage near the macula
• Pigment epithelial detachment
• Retinal pigment epithelium alterations

Right eye fundus was largely unremarkable.

Clinical Evaluation

Differential Diagnosis

Based on the clinical presentation, the following conditions were considered:

• Neovascular age-related macular degeneration
• Central serous chorioretinopathy
• Choroidal neovascular membrane
• Retinal angiomatous proliferation
• Polypoidal choroidal vasculopathy

The presence of subretinal hemorrhage and nodular vascular lesions strongly suggested polypoidal choroidal vasculopathy.

Investigations

Routine Laboratory Tests

• Complete blood count: Normal
• Blood glucose: Mildly elevated
• Renal function tests: Normal
• Lipid profile: Elevated cholesterol levels

Optical Coherence Tomography (OCT)

OCT revealed:

• Pigment epithelial detachment
• Subretinal fluid accumulation
• Irregular retinal pigment epithelium elevation
• Hyperreflective polypoidal lesions

Fundus Fluorescein Angiography

Findings included:

• Areas of occult leakage
• Irregular choroidal vascular pattern
• Late staining near the macular region

Indocyanine Green Angiography (ICGA)

ICGA demonstrated:

• Branching vascular network
• Multiple polypoidal dilatations
• Active choroidal leakage

These findings confirmed the diagnosis of polypoidal choroidal vasculopathy.

Diagnosis

Based on clinical examination and retinal imaging findings, the diagnosis of Polypoidal Choroidal Vasculopathy with Active Choroidal Neovascular Activity was established.

Management and Outcome

Initial Management

The patient was advised:

• Strict blood pressure control
• Smoking cessation
• Glycemic optimization
• Regular retinal follow-up

Definitive Treatment

The patient underwent:

• Intravitreal anti-VEGF injections

Therapy was administered under aseptic precautions without procedural complications.

Follow-Up and Clinical Course

At 1 Month

• Reduction in subretinal fluid
• Improvement in visual distortion
• Visual acuity improved to 6/24

At 3 Months

• Significant regression of polypoidal lesions
• Minimal residual hemorrhage
• Stable retinal anatomy

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At 6 Months

• Visual acuity improved to 6/18
• No fresh hemorrhage
• Improved reading ability and daily functioning

Discussion

Pathophysiology

Polypoidal choroidal vasculopathy results from abnormalities in the inner choroidal vasculature leading to aneurysmal vascular dilatations beneath the retinal pigment epithelium.

Important mechanisms include:

• Choroidal vascular remodeling
• Increased vascular permeability
• Endothelial dysfunction
• Chronic inflammation
• Degenerative retinal pigment epithelial changes

Repeated episodes of leakage and hemorrhage contribute to progressive retinal damage and visual decline.

Epidemiology

Key epidemiological features include:

• More common in individuals above 50 years
• Higher prevalence among Asian populations
• Male predominance in some studies
• Strong association with hypertension and smoking
• Important cause of severe central vision loss

Clinical Manifestations

Patients may present with:

• Blurred central vision
• Metamorphopsia
• Scotomas
• Reduced contrast sensitivity
• Sudden visual loss due to hemorrhage

Massive submacular hemorrhage may occur in advanced disease.

Diagnostic Considerations

Diagnosis requires multimodal retinal imaging.

Important diagnostic tools include:

1. Fundus examination
2. Optical coherence tomography
3. Fundus fluorescein angiography
4. Indocyanine green angiography
5. OCT angiography

ICGA remains the gold standard for identifying polypoidal lesions and branching vascular networks.

Treatment Modalities

Medical Management

Conservative measures include:

• Control of systemic hypertension
• Smoking cessation
• Glycemic control
• Monitoring for recurrence

Interventional Therapy

Definitive treatment options include:

• Intravitreal anti-VEGF therapy
• Photodynamic therapy
• Combination therapy
• Laser photocoagulation in selected cases

Anti-VEGF therapy has become the primary treatment modality because of favorable anatomical and visual outcomes.

Complications

Potential complications include:

• Recurrent subretinal hemorrhage
• Retinal fibrosis
• Pigment epithelial tears
• Persistent macular edema
• Severe visual loss
• Retinal scarring

Delayed diagnosis may lead to irreversible retinal damage and permanent impairment of central vision.

Prognosis

Prognosis depends on:

• Early diagnosis
• Extent of hemorrhage
• Macular involvement
• Treatment response
• Frequency of recurrence
• Baseline visual acuity

Prompt retinal intervention significantly improves long-term visual outcomes and reduces retinal damage.

In this case, early multimodal imaging and anti-VEGF therapy resulted in substantial anatomical recovery and improvement in visual acuity.

Conclusion

Polypoidal choroidal vasculopathy is an important retinal vascular disorder that can lead to progressive and irreversible visual loss if untreated. Early recognition of symptoms such as blurred vision, metamorphopsia, and subretinal hemorrhage is essential for timely diagnosis.

This case highlights the critical role of multimodal retinal imaging, particularly indocyanine green angiography and optical coherence tomography, in establishing an accurate diagnosis and guiding treatment decisions. Early intervention with intravitreal anti-VEGF therapy can significantly improve retinal anatomy, preserve vision, and reduce disease recurrence.

Comprehensive long-term follow-up and management of systemic vascular risk factors remain important components of successful patient care.

References

1. Yannuzzi LA, Wong DWK, Sforzolini BS, et al. Polypoidal choroidal vasculopathy and neovascularized age-related macular degeneration. https://pubmed.ncbi.nlm.nih.gov/11425670/
2. Cheung CMG, Lai TYY, Ruamviboonsuk P, et al. Polypoidal choroidal vasculopathy: definition, pathogenesis, diagnosis, and management. https://pubmed.ncbi.nlm.nih.gov/28650920/
3. Koh A, Lee WK, Chen LJ, et al. EVEREST study: efficacy and safety of verteporfin photodynamic therapy in polypoidal choroidal vasculopathy. https://pubmed.ncbi.nlm.nih.gov/22133795/
4. Ciardella AP, Donsoff IM, Huang SJ, et al. Polypoidal choroidal vasculopathy. https://pubmed.ncbi.nlm.nih.gov/15177963/
5. Wong CW, Yanagi Y, Lee WK, et al. Age-related macular degeneration and polypoidal choroidal vasculopathy in Asians. https://pubmed.ncbi.nlm.nih.gov/28237963/
6. American Academy of Ophthalmology. Polypoidal Choroidal Vasculopathy Overview https://www.aao.org/education/topic-detail/polypoidal-choroidal-vasculopathy-pcv--asia-pacifi

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