Dany Najjar, MD

HISTORY:

A 22 year old lady previously healthy presents because of sudden blurring of vision of her left eye of one week duration, not associated with any eye redness, pain or discharge. No flashes of light reported. No history of trauma or previous similar episodes.

Past Medical History: Negative. She is on no medications.

Past Ocular History : Negative

EXAM:

VOD= 20/20 VOS=20/50 à NI with PH

TOU= 16

Pupils: Equal and reactive; No APD

A/C: +1 cells OS

Refraction: Emmetrope OU.

FUNDUS EXAM:

Fig.1: Fundus OS (click to enlarge)

 DIFFERENTIAL DIAGNOSIS:

1. Central Serous Chorioretinopathy
2. Pigment Epithelial Detachment
3. Idiopathic Choroidal Effusion
4. AMPPPE
5. Inflammatory Choroidal Disorders
6. Choroidal Tumors

 

FLUORESCEIN ANGIOGRAPHY:

OS : Click to enlarge

 

DIAGNOSIS:

Acute Multifocal Posterior Placoid Pigment Epitheliopathy (AMPPPE)

 

 

 

AMPPPE

 

Definition:

Acute multifocal posterior placoid pigmant epitheliopathy (AMPPPE) is a disorder characterized by an acute decrease in central visual acuity in association with subretinal yellow-white placoid lesions of the posterior pole followed by a spontaneous recovery.

Incidence:

It usually occurs in the 3^rd decade.

Males are equally affected as females.

There is no racial predilection.

It is often bilateral but may be unilateral at the initial presentation.

 

Pathogenesis:

Its pathogenesis remains unknown

 

Signs:

Multiple yellow patches at the level of the pigment epithelium of the choriocapillaris.

The initial lesions usually involve the posterior pole.

Serous retinal detachment seldom occurs.

It may be accompanied by episcleritis or iritis.

The lesions spontaneously resolve in 2 to 5 weeks; however improvement of visual acuity often takes weeks to months.

Systemic Findings:

AMPPPE may be associated with CNS disorders such as cerebral vasculitis, late onset meningoencephalitis, CSF pleocytosis.

Other organs may be involved as well:

0. Erythema nodosum
1. Platelet aggregation anomalies
2. Hearing loss or tinnitus
3. Acute thyroiditis
4. Sarcoidosis

GI or respiratory viral prodromes are common.

Fluorescein Angiography:

Hypofluorescence is observed early (due to irregular filling of the choriocapillaris) followed by hyperfluorescence in the late venous phase (due to compromise of the integrity of the blood retinal barrier).

 

Therapy:

There is no effective treatment for this condition.

Prognosis is usually favorable. (80% of affected eyes have a final visual acuity of 20/40 or better)

Persistent paracentral scotomas may occur.

Recurrent cases have a poorer prognosis.