Carol L. Shields

BASICS

DESCRIPTION

• Vasoproliferative tumors of the retina (VPT) are benign vascular lesions located in the sensory retina.

• They are nonfamilial.

• In a series of 103 patients, Shields and associates reported that the patients were classified as follows (2):

– Primary or idiopathic (74%)

– Secondary (26%)

• In the same series, they may also be subclassified as follows:

– Solitary (78%)

– Multiple (15%)

– Diffuse (7%)

• Other synonyms for VPT are as follows:

– Presumed acquired retinal hemangioma

– Angiomatous mass

– Peripheral retinal telangiectasia

– Angioma/hemangioma-like mass

– Reactionary retinal glioangiosis

EPIDEMIOLOGY

• Predominantly affects older patients (mean age = 40 years)

• No gender or racial predilection

Incidence

• Represent 1% of all pseudomelanomas

• True incidence is unknown

Prevalence

Unknown

RISK FACTORS

Unknown

Genetics

No definite genetic association

PATHOPHYSIOLOGY

• Precise pathogenesis is presently unclear.

• Current hypothesis is reactive pigment epithelial and vascular proliferation or reactive gliosis secondary to a variety of underlying disease processes.

ETIOLOGY

• Primary cases are idiopathic.

• Secondary cases are associated with the following:

– Intermediate uveitis (28%)

– Retinitis pigmentosa (21%)

– Toxoplasmic retinitis (7%)

– Toxocariasis (7%)

– Retinochoroidal coloboma (7%)

– Traumatic chorioretinopathy (7%)

COMMONLY ASSOCIATED CONDITIONS

• Systemic hypertension (14%)

• Hypercholesterolemia (6%)

• Neurofibromatosis (2%)

DIAGNOSIS

HISTORY

• Blurred vision and/or floaters are common presenting symptoms.

• Photopsias and visual field defects are other possible initial symptoms.

• The disease may be asymptomatic (19%).

PHYSICAL EXAM

• Visual acuity and laterality:

– Better than 20/40 in up to 50%

– Most cases are unilateral

– Can be bilateral in 40% of secondary cases

• Appearance:

– Yellowish to yellow-red in color

– Mostly solitary and small (<5 mm)

– Non- or mildly dilated, nontortuous feeding artery and vein

• Location:

– Mostly inferotemporal

– Located between equator and ora serrata

• Associated slit lamp findings:

– Elevated intraocular pressure

– Anterior vitreous cells

– Posterior synechiae

– Cataract

• Associated vitreoretinal findings:

– Intraretinal/subretinal exudation

– Intraretinal/subretinal blood

– Vitreous hemorrhage

– Retinal pigment epithelial proliferation

– Preretinal fibrosis

– Macular edema

– Epiretinal membrane

– Exudative retinal detachment

DIAGNOSTIC TESTS & INTERPRETATION

Lab

Initial lab tests

• Genetic testing is generally not indicated unless von Hippel–Lindau (VHL) disease is considered.

• Toxoplasma titers (Ig G and Ig M)

• Toxocara titers (Ig G and Ig M)

• Systemic work-up to rule out other causes of uveitis

• Lipid profile (cholesterol, HDL, LDL, triglycerides)

Follow-up & special considerations

Positive genetic testing results for VHL should prompt systemic evaluation for associated CNS or kidney disease.

Imaging

Initial approach

• Patients with associated retinitis pigmentosa need additional visual field testing and electroretinogram (ERG).

• Fluorescein angiography:

– Rapid early filling, fine intrinsic capillary network, and late leakage

• Ultrasonography:

– Difficult due to peripheral location

– Medium to high internal reflectivity

– Acoustic solidity without choroidal excavation

• Indocyanine green angiography:

– intratumoral vascularization and late diffuse hyperfluorescence

Follow-up & special considerations

• Asymptomatic patients with exudates or retinal detachment threatening vision should be followed closely and offered treatment.

• Early treatment is needed for symptomatic patients.

• Frequency of follow-up is determined by severity of the condition and degree of visual impairment.

Diagnostic Procedures/Other

Systemic evaluation and genetic testing for VHL disease can be performed when diagnosis is in doubt.

Pathological Findings

Irvine and co-workers reported on the histopathological findings of patients who underwent an excisional biopsy of VPT lesions describing the presence of benign glial cell proliferation and secondary vasoproliferation, with perivascular lymphocytes, and adjacent fibrinous, lipid, and serous exudate (6).

DIFFERENTIAL DIAGNOSIS

• Retinal hemangioblastoma (RHB)

• Choroidal melanoma

• Peripheral exudative hemorrhagic chorioretinopathy (PEHCR)

• Retinal cavernous hemangioma (RCH)

• Choroidal granuloma

TREATMENT

MEDICATION

See “General measures” below.

ADDITIONAL TREATMENT

General Measures

• Asymptomatic patients are observed (1,2)[C].

• Symptomatic patients with macular edema, retinal detachment, or exudation threatening vision are generally treated.

• Cryotherapy is preferred since most are peripherally located (1,2)[C].

• Laser photocoagulation or photodynamic therapy can be considered when posterior in location (1,2)[C].

• Plaque radiotherapy can be used for advanced cases and large tumors measuring more than 2.5 mm in thickness, as reported by Cohen and colleagues (3)[C].

• The role of antivascular endothelial growth factors is not known, but this class of medications is often used.

• Intravitreal or subtenon’s triamcinolone is used to reduce macular edema.

Issues for Referral

Treatment is usually performed by a retina specialist or an ocular oncologist.

Additional Therapies

• Retinal detachment surgery for more extensive involvement (1,2)

• Enucleation for blind painful eyes from neovascular glaucoma (2)

COMPLEMENTARY & ALTERNATIVE THERAPIES

• Photodynamic therapy and transpupillary thermotherapy are recent modalities shown to be beneficial for some patients (4,5)[C].

• Adjunctive intravitreal bevacizumab (Avastin) injection is used in certain cases to reduce exudation and leakage (3)[C].

• Intravitreal or subtenon’s triamcinolone has also been used in association with cryotherapy in cases with concomitant macular edema.

SURGERY/OTHER PROCEDURES

Local resection may be performed in selected cases, although technically difficult to perform [C].

IN-PATIENT CONSIDERATIONS

Outpatient management

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

• Outpatient care for photocoagulation, photodynamic therapy, or cryotherapy

• In-patient or outpatient for plaque radiotherapy, vitrectomy, or enucleation

Patient Monitoring

• 6-month follow-ups are recommended for most patients.

• Follow more closely with lesions threatening vision.

DIET

No restrictions

PATIENT EDUCATION

• Advice control of blood pressure and cholesterol for primary cases

• Individualized patient education for secondary cases based on cause

• Use of the Amsler grid for self-monitoring of visual changes for early diagnosis and treatment

PROGNOSIS

Good, patients maintain functional vision in majority of cases.

COMPLICATIONS

Loss of vision or globe

Stay updated, free articles. Join our Telegram channel

Join