Marc Spirn
BASICS
DESCRIPTION
• Idiopathic juxtafoveal retinal telangiectasia (IJRT) is characterized by the presence of small perifoveal or parafoveal telangiectatic retinal vessels in the absence of an identifiable systemic or ocular disease.
– Typically occurs temporal to the fovea
– May be scattered throughout the macula
• Classified on the basis of biomicroscopic and fluorescein angiographic appearance into 3 groups (1,2,3) and 2 sub-groups (A and B)
EPIDEMIOLOGY
Incidence
Rare
Prevalence
Unknown
RISK FACTORS
No clear risk factors identified
PATHOPHYSIOLOGY
• Multifactorial visual loss
– Macular edema from vascular leakage
– Retinal ischemia from capillary occlusion
– Secondary choroidal neovascularization (CNV)
ETIOLOGY
• Not well understood
– May be a result of retinal Muller cell dysfunction
COMMONLY ASSOCIATED CONDITIONS
• Most often an isolated condition
• An association exists with abnormal glucose metabolism (2).
DIAGNOSIS
HISTORY
Unilateral or bilateral disruption of central vision
PHYSICAL EXAM
• Slit-lamp examination:
– Peri- or parafoveal dilated, tortuous, and irregular capillaries seen most often in the temporal macula
– “Right-angle” veins may be seen draining the telangiectatic areas.
– Small refractile deposits on the surface of the retina
– Retinal pigment epithelial hyperplasia may develop in long-standing disease.
DIAGNOSTIC TESTS & INTERPRETATION
Imaging
• Intravenous fluorescein angiography (IVFA) demonstrates the abnormal retinal vessels and may also show:
– Areas of leakage or capillary occlusion
– Presence of CNV
• OCT shows:
– Areas of hyporeflectivity representing cystic intraretinal spaces
– Intraretinal and subretinal fluid as well as outer retinal atrophy, particularly in IJRT group 2A
Pathological Findings
Endothelial cell and pericyte degeneration with retinal capillary narrowing
DIFFERENTIAL DIAGNOSIS
• Various vascular disorders may mimic IJRT including:
– Diabetic retinopathy
– Retinal vein occlusion
– Radiation retinopathy
– Carotid-occlusive disease
– Sickle cell retinopathy
– Coats’ disease
TREATMENT
SURGERY/OTHER PROCEDURES
• Laser photocoagulation may improve the macular edema (3)[C].
– This treatment is limited in IJRT due to the proximity of the lesions to the fovea.
• For treatment of CNV:
– Anti-VEGF agents (ranibizumab and bevacizumab) and photodynamic therapy (PDT) may be beneficial, although large studies are lacking (4,5)[C].
ONGOING CARE
FOLLOW-UP RECOMMENDATIONS
• Yearly dilated fundus examinations at the minimum
– Should complications such as macular edema or CNV develop, the patient should be referred to a retinal specialist for further evaluation and management with IVFA and/or OCT.
Patient Monitoring
Patients should be counseled to return to the ophthalmologist if any changes in vision occur.
PROGNOSIS
• Vision is normal or only mildly affected in the absence of macular edema, macular ischemia, or CNV.
– The extent of vision loss is directly correlated with the development and severity of the above-mentioned complications.
COMPLICATIONS
• Macular edema
• Macular ischemia
• Choroidal neovascularization
REFERENCES
1. Gass JDM, Oyakawa RT. Idiopathic juxtafoveal retinal telangiectasis. Arch Ophthalmol 1982;100:769–780.
2. Milay RH, Klein ML, Handelman IL, et al Abnormal glucose metabolism and parafoveal telangiectasia. Am J Ophthalmol 1986;102:363.
3. Chopdar A. Retinal telangiectasis in adults: Fluorescein angiographic findings and treatment by argon laser. Br J Ophthalmol 1978;62:243–250.
4. Konstantinidis L, Mantel I, Zografos L, et al. Intravitreal ranibizumab as primary treatment for neovascular membrane associated with idiopathic juxtafoveal retinal telangiectasia. Graefes Arch Clin Exp Ophthalmol 2009;247(11):1567–1569.
5. Kovach JL, Rosenfeld PJL. Bevacizumab (avastin) therapy for idiopathic macular telangiectasia type II. Retina 2009;29:27–32.
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