APMPPE – December 2015

December 2015

Case Presentation

An 18-year-old male complained of intermittent flashes of blue light in both eyes over the past 3-5 days. He reported that the lights appeared worse in bright lighting conditions and were worse upon awakening. He recently suffered a concussion. He hit his head on the ceiling after waking up in a lofted bed. He is healthy with a past medical history of only asthma.

Examination

Visual acuity with myopic correction was 20/20 in both eyes. The anterior segment was within normal limits. The posterior segment exam revealed diffuse, scattered retinal white spots, symmetrical in both eyes (Figure 1 & 2). Red free photography helps highlight the lesions also (Figure 3 & 4).

Figure1
Figure 1
Figure2
Figure 2
Figure 3Figure 3
Figure 4

 

Diagnostic Testing

Fundus photographs revealed the widespread presence of yellow-white lesions in both eyes. Fundus angiography showed normal arterovenous transit. There were multiple areas of pronounced hypofluorescence in early phases with late hyperfluorescence which correspond to the lesions (Figures 5, 6 & 7).

Figure5
Figure 5
Figure6
Figure 6
Figure7
Figure 7

 

Spectral domain optical coherence tomography revealed hyper-reflectivity in the outer portion of the retina in the juxtafoveal region corresponding to the lesions on clinical examination (Figure 8).

Figure7
Figure 8

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) was thought to be the most probable diagnosis.

Differential Diagnosis

  • Serpiginous choroiditis
  • Multiple evanescent white dot syndrome (MEWDS)
  • Birdshot retinochoroidopathy
  • Multifocal choroiditis
  • Punctate inner choroidopathy
  • Acute zonal occult outer retinopathy
  • Laser pointer injury
  • Uveitis (sarcoid, syphilis, etc)

Discussion

APMPPE is a rare, bilateral inflammatory chorioretinopathy which has been classified as a White Dot Syndrome. It usually presents with decreased vision in one eye followed by the contralateral eye or with central or paracentral scotomas. It affects both women and men equally. It often occurs in otherwise healthy adults in the second to third decades of life. Approximately one third of patients experience a viral prodrome.

On clinical examination, one can see yellow, creamy, placoid lesions within the retinal pigment epithelium in the macula. Cases are often self-limited. Improvement of the fundus appearance characteristically occurs within a few weeks and there is generally very good visual prognosis. It can be difficult to differentiate this disease from early serpiginous choroidopathy which has a much less favorable visual prognosis. Therefore, close monitoring is recommended.

Of note, there have been case reports linking APMPPE to CNS vasculitis. Patients should undergo a full neurologic and systemic workup to rule out CNS vasculitis or other systemic disease.

Follow Up

Given the patient’s history of headaches, the patient was sent for CNS imaging to rule out vasculitis and was evaluated by a neurologist. The work up came back negative. The patient was started on oral prednisone to help with the recovery of his lesions although there is no clear direction from the literature on this. Ten (10) days after presentation, the patient stated he was doing much better as can be seen on these comparison photos (Figure 1 & 9; Figure 8 & 10).

Figure1
Figure 1
Figure9
Figure 9
Figure8
Figure 8
Figure10
Figure 10

References

Gass JD Acute posterior multifocal placoid pigment. Arch Ophthalmol. 1968 Aug; 80(2):177-85

Quillen DA et al. The white dot syndromes. Am J Ophthalmol. 2004 Mar; 137(3):538-50

Mirza G, Jampol L. Acute posterior multifocal placoid pigment epitheliopathy. In Ryan SJ. Retina, 5th Edition. Elsevier, 2013: 1341-1346.

O’Halloran HS et al. Acute multifocal placoid pigment epitheliopahty and central nervous system involvement: nine new cases and a review of the literature Ophthalmology. 2001 May; 108(5):861-8

Steiner S, Goldstein D. Imaging in the Diagnosis and Management of APMPPE. International Ophthalmology Clinics. 2012;52(4):211-219.