1) What types of images are shown above?
2) Describe the abnormalities seen?
3) What is the diagnosis?
Answers:
1) Colour fundus images R and L eyes, FFA frames, Oct images
2) FFA shows temporal foveal telangiectatic vessels that leak in later stages.
OCT 1 shows large cystic fluid and oedema, the second resolution of fluid after treatment with anti-VEGF, fundus images on the OCT show exudates temporal to the macular
3) Idiopathic macular telangiestasia 1
Discussion:
Retinal telangiectasia: irregular capillary dilation and connecting arterioles and venules
Differential diagnosis of retinal capillary telangiectasia include:
Primary: Congenital: Coat's and MacTel1, Acquired: MacTel2
Secondary: BRVO, diabetic retinopathy, radiation retinopathy, ROP, Eales' disease, Sickle cell, ocular ischaemic syndrome
Mac tel 1 - Aneurysmal
Telangiectasia
- Congenital
- > unilateral
- Males
- Variant of Coats disease
- Microaneurysms
- Confined to one area temporal to the macula
- CMO
- Lipid exudates
- VA loss - serous and lipid exudation at the macula
- Treatment - M.As with laser photocoagulation
Mac tel 2 - Perifoveal
Telangiectasia
- Acquired and bilateral.
- Middle-aged or older patients
- Patho-physiology - ? secondary to a retinal neuronal dysfunction/ neurodegenerative disease of the central retina disruption of pericytes, ectatic vessels, dilated venules, RPE hyperplasia
- Associations - Hypertension, diabetes
- Symptoms - metamorphopsia, scotoma.
- Vision loss - Progressive, due to CNV, disciform scar, full-thickness macular hole
- FFA - Temporal foveal telangiectatic vessels - leak in later stages,Venules diving at a right angle into deeper retinal layers
- OCT - Temporal foveal pit enlargement secondary to loss of outer nuclear layer and ellipsoid zone. Progress into large cysts involving all retinal layers. ILM Drape: only ILM is left
- Treatment - No randomized clinical study, Anti-VEGF for CNV