Recurrent Photoreceptor Loss with Spontaneous Recovery as a Presenting Sign of Syphilitic Outer Retinopathy

Jessica G Shantha,Gregg T Kokame,Lucas Kim

doi:10.2174/1874364101913010086

Abstract

A 64-year-old male presented with sudden onset of decreased vision with a central blind spot and shimmering visual symptoms without significant inflammatory findings, and was noted on optical coherence tomography to have localized loss of photoreceptors, which spontaneously resolved after two weeks. This recurred subsequently three times spaced by months of recovery with normal vision and normal photoreceptors. In the fourth episode, the patient developed an arcuate-shaped area of outer retinitis more typical for acute syphilitic posterior placoid chorioretinitis, and the diagnosis was made serologically. With subsequent treatment for syphilis, there was not any recurrence after the treatment and vision recovered to 20/20 with a normal photoreceptor layer.

Highlights

Sudden vision loss associated with localized damage to the photoreceptor layer on Optical Coherence Tomography (OCT) without significant inflammatory signs is an unusual clinical presentation, especially when there is spontaneous resolution with the recovery of normal vision and a normal photoreceptor layer on OCT
Two years after the last episode, his vision is 20/20 in both eyes with no recurrences in the left eye and recovery of a normal photoreceptor layer (Fig. 1H). His VDRL decreased from 1:64 to 1:4 after treatment. This case demonstrates the unusual finding of symptomatic vision loss without clinical signs of active inflammation and with loss of the ellipsoid layer on OCT, which rapidly and spontaneously resolved without treatment on three separate episodes with the recovery of good vision and a normal ellipsoid layer
Additional findings included choroidal thickening and decreased flow on OCT angiography isolated to the left eye

Summary

INTRODUCTION

Sudden vision loss associated with localized damage to the photoreceptor layer on Optical Coherence Tomography (OCT) without significant inflammatory signs is an unusual clinical presentation, especially when there is spontaneous resolution with the recovery of normal vision and a normal photoreceptor layer on OCT. Acute Zonal Occult Outer Retinopathy (AZOOR) is an idiopathic disease with acute damage to photoreceptors with minimal funduscopic findings. It is associated with photopsias and is usually found in young females. Acute cone photoreceptor vision loss was recently described as a presenting sign of AZOOR, but the findings persisted and were associated with decreased signal in the photoreceptor layer on OCT [1]. Differential diagnosis includes other acute inflammatory diseases, such as acute multifocal placoid pigment epitheliopathy or unilateral acute idiopathic maculopathy, and infectious diseases, such as tuberculosis and syphilis, sarcoidosis, and intraocular lymphoma

CASE REPORT

Findings

DISCUSSION