Paracentral acute middle maculopathy as a cause of unexplained visual loss in central retinal vein occlusion

Abstract

A 79-year-old man presented with unilateral unexplained sudden onset visual loss in the setting of central retinal vein occlusion (CRVO). Non ischemic CRVO in the right eye (RE) was confirmed on fluorescein angiography. Spectral domain optical coherence tomography (SD-OCT) showed absence of macular edema and hyperreflective band-like lesions in the middle retinal layers of the RE suggesting a diagnosis of paracentral acute middle maculopathy (PAMM). Patient was observed and after 3 months, best-corrected visual acuity in the RE spontaneously improved from 38 to 56 ETDRS letters. SD-OCT scans showed thinning of the inner nuclear layer of the RE. OCT angiography in the RE revealed a mild attenuation of the vascular flow signal in the superficial capillary plexus and patchy areas of vascular flow void in the deep capillary plexus, as compared to the fellow eye. The present case outlines the importance of recognising PAMM as a cause of unexplained visual loss. In the setting of a CRVO with sudden vision loss and absence of macular edema, clinicians should pay attention to any hyperreflectivity and/or to thinning of the middle retinal layers on SD-OCT.

Keywords: Central retinal vein occlusion; Deep capillary ischemia; Optical coherence tomography angiography; Paracentral acute middle maculopathy; spectral domain optical coherence tomography.

Fig. 1

Montage colour fundus photography (CFP) and spectral domain optical coherence tomography (SD-OCT) at presentation. CFP (A) of the right eye (RE) shows a mild venous engorgement and widespread peripheral retinal hemorrhages located in all 4 quadrants. CFP of the left eye (LE) (B) does not show any retinal abnormalities. SD-OCT scan of the macula of the RE (C) shows hyperreflective band-like lesions (arrows) in the middle retinal layers of the macula of the right eye and no abnormalities at the macula of the left eye (D).

Fig. 2

Fluorescein angiography frames of the right eye at presentation show minimal patchy areas of non perfusion at the posterior pole (A) and in the mid periphery (B–E) with absence of macular edema in the late phase of the angiogram (F).

Fig. 3

After 3 months, spectral domain optical coherence tomography (SD-OCT) scan of the right eye (A) reveals thinning of the inner nuclear layer (arrow); no abnormalities were detected in the left eye (B). OCT angiography with a scanning area of 3 × 3 mm shows mild attenuation of the vascular flow signal in the superficial capillary plexus (SCP) (C) and patchy areas of vascular flow void in the deep capillary plexus (DCP) (D) of the right eye, and normal flow in the SCP (E) and DCP (F) of the left eye.