Author: David Aragon-Roca (Spain)
Co-authors: Fernando Trejo Velasco, Francesc Xavier Garrell Salat, Sandra Banderas García, Anna Boixadera, Randa Falah, José García-Arumí
Purpose
We present a case report of Acute Retinal Pigment Epitheliitis or Krill Disease, a rare inflammatory disease of the retina.
Setting/Venue
Retina Unit. Vall d´Hebron Hospital. Barcelona. Spain.
Methods
An 18-year-old male presented at the emergency room referring blurry vision and a central scotoma in the left eye (OS) of a 1-day onset. He mentions he had mild flu-like symptoms 1 week before. Best visual acuity was 20/20 in the right eye and 20/25 in OS. The anterior segment examination was unremarkable. Fundus examination in OS presented a yellowish fine pigment stippling in the macula surrounded by a hipopigmentated halo. Optical coherence tomography (OCT) in OS showed an hyperreflective lesion at the photoreceptor outer segment layer and a disrupted interdigitation zone Fundus autofluorescence showed dots of slightly increased autofluorescence in the fovea surrounded by an hiperautofluorescent halo. Multifocal electroretinogram showed changes consistent with an alteration of photoreceptors in OS.
Results
A wait and see attitude, with close follow-up visits, was adopted and 8 weeks after the onset of the symptoms, we observed a reduction in the hyperreflective lesion at the photoreceptor outer segment layer. A year after the initial consultation, the patient is asymptomatic, he has a visual acuity of 20/20 in OS, with a complete disappearance of the hyper-reflective lesion and a restoration of interdigitation zone in the OCT.
Conlusions
Acute Retinal Pigment Epitheliitis or Krill Disease is a rare, unilateral, idiopathic, self-limiting inflammatory disease of the retina that commonly affects young adults (between the age of 20 and 50 years). The etiology and pathophysiology are unknown. However, flu-like symptoms have been reported to occur at 1-2 weeks before its onset, suggesting viral infection may play a role in the pathogenesis. The clinical presentation consists in unilateral acute loss of vision or central scotoma. The diagnosis is based on typical presentation and the fundus examination. The OCT is the complementary exam of choice for diagnosis and follow-up of patients. It has a very good prognosis, with a complete resolution and recovery of visual acuity in the great majority of patients in 6 to 12 weeks.
Financial Disclosure
We declare NO conflict of interest
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