OOMC News

A 45 year old man was referred for recent vision loss OD > OS.  Medical history is unremarkable.  Ocular history is significant for being told years ago of an optic nerve problem.  On examination VA was OD: 20/70 and OS: 20/40.  IOP’s were normal.  SLE was normal.  DFE showed a clear lens and vitreous.  OCT, photos, and FA are shown below:

The patient had numerous interesting preexisting ocular conditions and one new finding causing the acute vision loss.  Old findings include dramatic optic nerve drusen, angioid streaks, and macular orange pigment (peau d’orange).   The new finding consists of a macular CNVM OD and superior as well as nasal CNVM OS, both with active hemorrhage and exudation. 

There are two important considerations:  First, what is the best way to treat the exudative maculopathy, and second, are there any medical issues that need to be addressed?  Regarding the second question, the group of findings including angioid streaks, peau d’orange macular pigment, and CNVM strongly suggests the medical condition pseudoxanthoma elasticum (PXE).  This is a systemic abnormality with elastic tissue in the body which often has the above noted eye findings but can also be associated with systemic morbidity.  The most concerning systemic issue is the possibility of GI bleeding due to weakening of the blood vessel walls.  This was discussed with his medical doctor who will arrange the appropriate tests. 

Regarding the eye findings, optic nerve drusen can cause chronic optic nerve compression but are untreatable.  Most patients do not have significant optic neuropathy, but occasionally VF constriction can be progressive.  I have never seen a patient with PXE who also had optic nerve drusen, but it is possible that there is an association.  The imminent ocular concern regarding this patient is the rapid vision loss due to the bilateral CNVM’s.  The best initial treatment course is antiVEGF injections.  In older patients with CNVM due to AMD, injections are chronic and usually have no end point.  In a younger patient there is often an end point after a series of monthly injections.  We started our patient on a course of monthly avastin injections OU with dramatic improvement.  VA improved after two shots to OD: 20/20 and OS: 20/30.  The hemorrhage and exudation resolved (see below):

Unfortunately, as is common with young patients, after initial success, he disappeared for several months.  He came back with recurrent hemorrhage and exudation OU with visual reduction to OD: 20/100 and OS: 20/40.  The injections were resumed. See photos below:

He once again responded quickly with resolution of the hemorrhage and exudation (see photos below: VA improved to OU: 20/30. He will need careful monitoring and will need continued treatment if his exudation worsens.