Dome-shaped macula (DSM) is characterized by a convex anterior protrusion (bowing towards the vitreous cavity) of the macula associated with high myopia and a posterior staphyloma.
DSM is associated with high myopia, and its presence is positively correlated with the severity of myopic maculopathy. Though initially thought to only occur with high myopia, DSM has also been noted in mildly myopic and non-myopic eyes.
Several theories have been proposed, but the exact pathophysiology of DSM remains unclear. When DSM was first observed by Gaucher in 2008, it was believed to be due to either choroidal thickening, a the posterior eye-wall collapsing, or vitreomacular traction. Subsequently, focal thickening of the subfoveal sclera has been shown to be a more likely mechanism, though the reason for the thickening is unknown. A defect in Bruch’s membrane may also create a focal relaxation in the posterior sclera, allowing it to bulge anteriorly and form a dome. Some speculate that the dome may compensate for the large axial length in high myopes, thus protecting against tractional damage to the fovea and distributing tractional forces more peripherally.
Optical Coherence Tomography (OCT) is necessary to diagnose DSM and identify the morphological type. Because the convexity can appear in different orientations, it is important to obtain both vertical and horizontal scans or radial scanning to ensure visualization.
Patients with DSM are likely to have a history of high myopia and may experience worsening visual acuity or metamorphopsia.
There is no specific abnormality in DSM that can be reliably visualized ophthalmoscopically in DSM. Ophthalmoscopy findings that may raise suspicion for DSM include an oval-shaped area of macular pigment mottling, the appearance of a ridge bridging the fovea and optic nerve, or a horizontally-oriented oval-shaped optic nerve.
Besides displaying an elevated macula on OCT, eyes with DSM may demonstrate pinpoint leakage on fluorescein angiography, a serous retinal detachment, retinal pigment epithelium elevations, a small pigment epithelial defect, or focal punctate hyperfluorescence with indocyanine green chorioangiography.
Patients may experience a decrease in visual acuity or metamorphopsia as complications of associated serous foveal detachment or choroidal neovascularization.
1. Central Round Dome (21%)
3. Vertically Oriented Oval Shaped Dome (17%)
- Central serous chorioretinopathy
- Non-pigmented neoplasm of the choroid, such as choroidal hemangioma or amelanotic choroidal melanoma
- Bilateral diffuse uveal melanocytic proliferation (BDUMP)
- Uveal effusion syndrome
- Posterior scleritis
DSM management focuses on reducing subretinal fluid, but no effective treatment for this complication has been established. Three monthly injections of aflibercept may improve best-corrected visual acuity (BCVA) and reduce central subfield thickness on OCT, but bevacizumab and ranibizumab have proven ineffective at improving BCVA or subretinal fluid. There are also limited reports of mineralocorticoid receptor antagonists successfully resolving serous foveal detachments. Photodynamic therapy trials for subretinal fluid associated with DSM have conflicted over the efficacy of this treatment. Additionally, vitrectomy may aid in the transient resolution of sub-retinal detachment in DSM patients. Due to the lack of a proven treatment, many physicians elect to observe asymptomatic or mildly symptomatic cases. Oftentimes the subretinal fluid resolves and recurs intermittently over time (Figure 1).
The most frequent complication of DSM is subretinal fluid, which may predispose to foveal serous retinal detachment (SRD). The prevalence of SRD in DSM has been shown to vary widely, occurring in 2% to 67% of patients with DSM. The cause of SRD in this context is a topic of debate. It is possible that scleral thickening under the dome obstructs choroidal fluid outflow, leading to a fluid collection. Others have found that in eyes with DSM and SRD, the submacular choroid is abnormally thick for the amount of myopia, pointing to a mechanism similar to central serous chorioretinopathy. The abrupt change in choroidal thickness in eyes with DSM may lead to retinal pigment epithelium dysfunction and SRD. SRF in DSM has also been shown to be associated with higher macula dome height, a lower degree of myopia, and vertically-oriented oval shaped domes. Besides structural factors, alterations in the mineralocorticoid pathway could also be involved. Studies conflict over whether subretinal fluid actually results in a decrease in BCVA.
Choroidal neovascularization may occur in DSM, but Zhao et. al did not find this complication more likely in DSM compared to high myopes without DSM. Choroidal neovascularization may not be a result of DSM, but associated with myopia itself.
On the other hand, some suggest that DSM may actually act as a protective factor against foveal schisis, macular holes, and choroidal neovascularization. DSM may also prevent vision-threatening macular complications after cataract surgery in highly myopic eyes.
In eyes with DSM, BCVA and central foveal thickness may remain stable for years. Dome height and retinal pigment epithelium atrophy tend to increase over time, however, and a dome height greater than 400 µm has been associated with subfoveal serous retinal detachment and decreased visual acuity.
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- Image courtesy of Danny A. Mammo MD
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