Acute retinal pigment epithelitis in a pregnant woman with single functional eye: diagnosis and management

Author: Isabel Sendino-Tenorio (Spain)

Co-authors: Carlos Plaza-Laguardia, Amacia Mateos-Hernandez, Laura Monje-Fernandez, Esteban Pertejo-Fernandez, Borja Errazquin-Aguirre, Jorge Sanchez-Cañizal

Purpose

We describe the first case of acute retinal pigment epithelitis (ARPE) in a pregnant woman with single functional eye, remarking the challenging management because of the limiting circumstances.

Setting/Venue

Complejo Asistencial Universitario de León

Methods

Case report

Results

A 38-year-old caucasian woman presented with acute central scotoma in her left eye (OS) of 4 hours of evolution with no other associated symptoms. She was in the 12th week of pregnancy. Her ocular history was remarkable with an unique functional eye because of multiple rhegmatogenous retinal detachments due to high myopia. Visual acuity (VA) was counting fingers in the right eye (OD) and 20/63 in the OS. Funduscopy showed subfoveal yellowish lesion with hypopigmented halo located in the nasal area. Any contrast angiogram could not be performed due to her pregnancy state. In the OCT we found an hyper-reflective and homogeneous domed-shaped lesion at the photoreceptor outer segments which caused a disruption of the ellipsoid zone (EZ) and interdigitation zone (IZ) and thickening of the retinal pigment epithelium (RPE). The expected prognosis and evolution of the suspected diagnosis was explained, however she desperately wanted to be treated because of the fear to lose her only functional eye. We decided to prescribe a tapering regimen of prednisolone acetate 1% drops with a close follow-up. One month later her VA was 20/20 and no pathologic findings were visible on the OCT.

Conlusions

ARPE is a rare inflammatory disease of the outer layers of the retina. Its self-limiting nature makes it an under-diagnosed disease, with a very hard to estimate incidence. The inflammation affects the outer segments of the retina, basically, the EZ, the IZ, and the complex of RPE and Bruch’s membrane. The severity of the pathological disruption of these layers defined the VA of the patient, with a clear and lineal anatomic – functional correlation, as we could observe in our patient, recovering her VA when the EZ and the IZ got restored. The disruption of these layers play an important roll in the VA and retinal sensitivity in many retinal diseases. The OCT is the gold-standard test for diagnosing and monitoring this benign and self limited disease. The knowledge of the pathological findings of the OCT that occur in this entity makes that in patients whose conditions do not allow all the complementary tests to be carried out, we can orient the process in an optimal way with the same quality and confidence as if we performed all the tests.

Financial Disclosure

NO

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