Dome-shaped maculopathy: a review

Abstract

First described by Gaucher and associates in 2008 in eyes with high myopia, dome-shaped maculopathy (DSM) is an anterior convex protrusion of the macula towards the vitreous cavity observable on OCT. This seems to be related to a localized scleral thickness, which might be the result of regional variation in the scleral bio-mechanical properties and the process of emmetropization causing asymmetric scleral growth. The presence of DSM can be associated with an increased risk of complications. The clinical spectrum ranges from being asymptomatic to metamorphopsia and mild-to-moderate gradual visual loss over years. Visual impairment in DSM results from retinal pigment epithelial changes, sub-foveal serous detachment, retinoschisis and myopic choroidal neovascularization. In this review, we compile and review the available information on the pathophysiology, nomenclature, classification, clinical features including imaging, differential diagnosis, complications associated with DSM and the gaps in our understanding of this entity thus far.

Fig. 1. A multi-averaged vertical section OCT through the fovea obtained with swept-source OCT machine in a high myope with dome-shaped macular configuration with the parameters associated.

At the fovea, three parameters are measured: retinal thickness (red arrow), choroidal thickness (green arrow) and sclero-choroidal thickness (white arrow). The height of the forward convexity of the retinal pigment epithelium (orange arrow) was measured above the tangent plane (yellow line) at the bottom of the posterior pole. Blue dashed line indicates approximated outer scleral border (colour figure online).

Fig. 2. Optical coherence tomography scan of horizontal and vertical sections through the fovea in a high myope using swept-source technology.

The left images show the posterior pole fundus photographs with tessellated background. The middle image representing the horizontal section shows a relatively flat macular contour. The right image representing the vertical section shows a convex forward bowing of the foveal contour with normal retinal anatomy overlying it.

Fig. 3. Optical coherence tomography scan of horizontal and vertical sections through the fovea in a high myope using swept-source technology.

The left images show the posterior pole fundus photographs with tessellated background with peri-papillary atrophy and retinal pigment epithelial changes encroaching fovea. The transfoveal horizontal line scan in the middle image shows a convex forward bowing of the foveal contour. The corresponding vertical line scan on the right hand side shows a relatively flat macular contour. The overlying retina looks grossly normal with mild irregularity of the photo-receptors.

Fig. 4. Optical coherence tomography scan of horizontal and vertical sections through the fovea in a high myope using swept-source technology.

The left images show the posterior pole fundus photographs with tessellated background with peri-papillary atrophy encroaching fovea. The middle image representing the horizontal section shows a convex forward bowing of the foveal contour. The right image representing the vertical section shows a shallow forward convexity of the macular. The overlying retina looks thinned out.

Fig. 5. (Left) Optical coherence tomography scan of vertical transfoveal line scan showing a convex forward protrusion of the central macula, a shallow sub-foveal neurosensory detachment at the apex of the dome.

(Middle) Magnified image of another line scan showing retinal pigment epithelial irregularity, choroidal thinning and elongation of the photo-receptor outer segments overlying it. (Right) OCT-angiogram of the outer retinal layer slab confirms the absence of Choroidal neo-vascular membrane.

Fig. 6. (Left) Fundus photograph of an eye with high myopia showing peri-papillary atrophy, tessellations and a small dirty grey membrane with a speck of sub-retinal haemorrhage noted at fovea.

(Right) Optical coherence tomogram, vertical transfoveal line scan showing grossly thinned choroid and forward protrusion of the sclera, choroid and retinal pigment epithelium at the fovea. An ill-defined hyper-reflective material was noted in the sub-retinal space at the fovea, suggestive of choroidal neo-vascular membrane.