Our first “Case of the Week”!!⁠
⁠
These are colour photos and fluoroscein angiograms of a 30yo female who presents with progressive acuity loss OS. ⁠
⁠⁠
⁠⁠On examination she has Angioid Streaks (AS) radiating from the disc and is now involving her macula OS, hence the deteriorating vision.⁠
She also has Peau d’Orange appearance of the fundus (yellow stars) which is pathognomonic of Pseudoxanthoma Elasticum (PXE). ⁠
⁠
Angioid Streaks are bilateral, narrow irregular “Jagged” or “vessel-like” lines deep to retina that radiate from the ONH. They form from full thickness breaks in a weakened and calcified Bruch’s with disruption of overlying RPE. ⁠
Tend to develop in patients with certain systemic disorders marked by extensive calcification of Bruch’s elastic layer. ⁠
⁠
Complications/Visual Loss occur in over 70% of patients through either ⁠
a. CNV – Most common cause of visual loss⁠
b. Choroidal Rupture – May occur following trivial trauma eg rubbing eye ⁠
c. Foveal involvement of streak ⁠
⁠
Our favourite mnemonic for causes of AS was PEPSI MAX ⁠-
Pseudoxanthoma Elasticum ⁠
Ehler-Danlos Syndrome⁠
Paget’s Disease of bone⁠
Sickle Cell Disease and other haemoglobinopathies ⁠
Idiopathic (~50% of angioid streaks) ⁠
Myopia⁠
Acromegaly, Abetalipoproteinaemia ⁠
toXic – Lead poisoning⁠
⁠

⁠#retina #ophthalmology #vitreoretina #ophthalmologist #eyedoctor #optometry #optometrist #optometrystudent