Paediatric Ophthalmology Strabismus Optics and Refraction Intraocular and Orbital Tumors Retina and Vitreous Neuro-Ophthalmology Cornea and External Disease Uveitis Glaucoma Orbit and Oculoplastics

This rare case uploaded by Mr Armin Moradi . Case content generously contributed by Dr. Bernard Hurley at the University of Ottawa Eye Institute.

Clinical History

62yo male complaining of blurry vision for 6 months 'wavy' visual distortion.


Q What is the differential diagnosis? (one per line)

The differential diagnosis is as follows:

  • Macular Telangiectasia
  • Radiation retinopathy - usually involves a larger area with cotton wool spots and neovascularization
  • BRVO - area of distribution distar to an arteriolar-venular crossing
  • Tamoxifen retinopathy

Q What further investigations would you order? (one per line)

The following investigation results are available:

OCT

OCT is key. Hyporeflective *inner* retinal cavities. Inner lamellar cysts that enlarge with progressive disease. Thinning and/or disruption of the ISOS junction (photoreceptor layer).

FA

Hallmark of macular telangiectasia is telangiectatic capillaries seen on FA starting predominantly temporal to fovea. The images are sorted in order from early to late.


Q What is the most likely or definitive diagnosis?

The accepted diagnosis is Macular Telangiectasia.


Q What is the management plan for this condition? (one per line)

The management of this condition is as follows:

  • Anti-VEGF therapy for neovascular complications
  • Observation for non-neovascular mac tel

Things to look for on fundus exam: retinal loss of transparency which can make the retina look greyish, possibly crystalline deposits, retinal pigment epithelial hyperpigmentation, and perifoveal telangiectactic vessels (Ryan's Retina). Additionally, aneurysms are uncommon, but RPE plaques can be seen which are often associated with right-angled venules. Pay close attention to OCT findings of INNER retinal cavities and disruption of ISOS junction with or without RPE thinning which does not look like geographic atrophy.

Ryan's Retina 6th ed pg 1180-1187. Kanski's clinical ophthalmology 8th ed pg 627-630. MacTel Project.