OOMC News

Authored by: Danielle Strauss, MD

CC: 33 year-old man.  Blurry vision and redness of the right eye for 1 week. Frontal headaches.

PMHX: No past medical history

Past ocular history: None

Va sc: 20/70 OD, 20/20 OS

IOP: 16 OD, 15 OS

SLE:

Cornea clear

Rare cell in anterior chamber both eyes

Iris wnl ou

No cataracts ou

DFE: see imaging below

Imaging:

Widefield Color photos:

Right eye shows unusual findings in the posterior pole.

Magnified view of posterior pole right eye shows diffuse deep creamy white lesions. There is no vitritis.

Left eye has similar lesions but not as diffuse.

SD-OCT macula right eye and left eye.

In the right eye there is disruption of the outer retinal and ellipsoid zones. There is evidence of hyperreflective material in the outer retina. There is also evidence of choroidal thickening in both eyes.

Fundus autofluorescence (FAF) right eye shows the lesions to be hypoautofluorescent with focal areas of hyperautofluorescence.

FAF left eye shows lesions are less prominent.

Fluorescein angiography (FA) of the right eye shows early blocking and late staining of the lesions in both eyes.

FA left eye:

HVF 24-2 shows paracentral scotoma of the right eye.

Differential Diagnosis:

The patient has deep creamy white placoid lesions in both eyes with mild anterior segment inflammation. The differential diagnosis includes

1. Infectious retinitis
2. White dot syndromes
3. Multiple evanescent white dot syndrome (MEWDS)
4. Punctate inner choroidopathy (PIC),
5. Acute posterior multifocal placoid pigment epitheliopathy (APMPPE),
6. Multifocal Choroiditis (MFC)
7. Vogt Koyanagi Harada Syndrome

Assessment and plan:

33 yo man with APMPPE

Based on the exam and multimodal imaging, we diagnosed the patient with APMPPE.

The symptom of rapid onset of paracentral scotoma, with deep white placoid lesions and mild anterior chamber reaction in both eyes was consistent with the diagnosis.

APMPPE is part of the spectrum of White Dot Syndromes. It is an inflammatory chorioretinopathy and is fairly uncommon. It is thought to be caused by an autoimmune process at the level of the choriocapillaris that causes ischemia to the overlying RPE. APMPPE is typically a self-limiting disease with patients typically recovering good vision within several weeks.

APMPPE has been known to be associated with other autoimmune diseases and can sometimes be preceded by a viral or flu-like illness.

APMPPE has a rare association with cerebral vasculitis. Therefore patients with the disease are recommended to have an evaluation with a neurologist and to get neuroimaging/ MRI.

Our patient was referred to a neurologist for further evaluation and neuroimaging, and to a rheumatologist for an auto-immune workup. He was advised to follow up with us in two weeks for reevaluation of the retina.