Unilateral buried optic nerve head drusen triggering central retinal vein occlusion in young - Discovering an eclipsed entity

Abstract

Optic nerve head (ONH) drusen are acellular calcified concretions. Buried drusen manifests as pseudopapilledema. The compressive effects of ONH drusen can rarely precipitate central retinal vein occlusion (CRVO). The superimposition of pseudopapilledema on disc edema in CRVO poses a diagnostic dilemma. A 40-year-old female without systemic comorbidities presented with resolving CRVO. An exhaustive systemic workup revealed no abnormalities. Ultrasonography demonstrated buried ONH drusen. This unusual etiology must be considered in a young patient in the absence of systemic risk factors, persistence of "nasally conspicuous" disc elevation, and presence of peripapillary hemorrhages. Ultrasonography must be incorporated in the diagnostic armamentarium in a young patient with CRVO.

Keywords: Central retinal vein occlusion; optic nerve head drusen; ultrasonography; young.

Figure 1:

Color fundus photograph of the left eye shows an elevated optic disc with blurred margins, moderate venous tortuosity, vascular sheathing, peripapillary hemorrhages (white arrow), and macular edema, consistent with resolving CRVO. CRVO: Central retinal vein occlusion

Figure 2:

Fluorescein angiography of the left eye shows venous staining, petaloid leak at the macula, late leak, and staining of the optic disc

Figure 3:

(a) Horizontal SD-OCT scan through macula in the left eye shows atrophy of inner retinal layers and intraretinal hyporeflective cystoid spaces, (b) vertical sectional image of the optic nerve head, and (c) analysis of circumpapillary RNFL using SD-OCT shows thickening of RNFL consistent with disc edema. SD-OCT: Spectral-domain optical coherence tomography, RNFL: Retinal nerve fiber layer

Figure 4:

Ultrasound B-scan image of the left eye shows increased echogenicity of ONH (white arrow) consistent with buried ONH drusen. ONH: Optic nerve head