What is dome-shaped maculopathy (DSM)?
Dome-shaped maculopathy (DSM) is a type of myopic macular degeneration. Myopic macular degeneration (MMD) is a degeneration of the center of the retina seen in some people who are myopic (near-sighted). The cause of DSM and MMD is related to an elongation of the eye commonly seen in myopia. MMD is not the same as age-related macular degeneration, which is a common cause of vision loss in the elderly. DSM is often mistaken for central serous retinopathy.
What is the macula?
The macula is the area of the retina in the back of the eye that is responsible for seeing details in the central vision. The retina is a thin layer of delicate nerve tissue that lines the inside wall of the eye like the film in a camera. In the eye, light is focused onto the retina, which “takes the picture” and sends the image to the brain. Although macular problems affect the central vision, they do not affect peripheral vision— the ability to see objects off to the side when looking straight ahead. This means that macular problems alone do not result in total blindness.
Why is it called dome-shaped maculopathy (DSM)?
As the name suggests, in dome-shaped maculopathy, the macula assumes the shape of a dome or elevation as seen on special imaging, called optical coherence tomography (OCT). A dome-shaped macula is the result of the eye attempting to keep the eye focused at near. The elongation of the eye may result in an irregular eye shape, including a dome or ridge shape.
In the sketch above, A represents the normal eye. B shows how the eye is elongated in near-sighteness (myopia). C and D show a focal out-pouching at the back of the eye. This out pouching is called a staphyloma. At the edge of the staphyloma (green arrowhead), the tissues inside the eye are stretched. This area appears as a dome on retinal imaging.
What causes fluid leakage under the retina in DSM?
A dome-shaped macula may cause an abnormal leakage of clear fluid under the macula, which causes symptoms of blurred vision often associated with distortion or a round/oval dark spot in the central vision. Other factors may aggravate the situation and contribute to a leakage of fluid beneath the macula. Research suggests that hormones released under stress can affect the blood vessels beneath the retina. These vessels may leak an abnormal amount of fluid, which then works its way under the center of the retina. Sometimes, medical conditions (Cushing’s Syndrome, pheochromocytoma, sleep apnea), medications (pseudephedrine, prednisone, cold medication, diet pills, and medications for erectile dysfunction), and other agents (testosterone, cocaine, caffeine, amphetamines, etc.) may contribute to the development of leakage.
What is the treatment of DSM?
There is no universally effective treatment. In some cases, the symptoms will disappear with time. Over a period of weeks to months, the abnormal leakage may stop with normal healing. In other situations, treatment may be needed to recover vision. Prescription medication (spironolactone or diamox) may be helpful if fluid leakage does not go away with time alone. Sometimes, eye drops can help. Other treatment that have been tried without uniform success include thermal laser photocoagulation, Visudyne photodynamic therapy (PDT), and medicine injections. The doctor can recommend the best course of action by taking special pictures of the eye called a fluorescein angiogram. The fluorescein angiogram shows where the leak is coming from, how active the leak is, and whether laser might be helpful. Optical coherence tomography (with and without angiography) may also be helpful to guide treatment.
In rare situations, blood vessels grow beneath the retina from the choroid (choroidal neovascularization, CNV). When CNV is detected by fluorescein angiography or by optical coherence tomography, antiVEGF injections are the first time of treatment.
What will happen to my vision?
Over a period of months to a few years, the vision may remain normal or near normal under observation without treatment. Sometimes over time, dome-shaped maculopathy may cause permanent loss of central vision. More aggressive treatment may be considered in cases of persistent fluid leakage causing progressive loss of vision.
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