Best Disease Treatment in Connecticut: Entrust Your Vision to Retina Experts
Best vitelliform macular dystrophy (BVMD) or simply Best disease is an inherited disease that affects the macula (the center-most part of the retina). Best disease is rare, affecting about 1 in every 10,000-20,000 people. Best disease is a progressive eye condition, making early intervention essential.
At Retina Consultants, P.C., our retinal specialists have years of hands-on experience, allowing us to diagnose and treat rare and complex retinal diseases, including Best disease. If you have concerns about your retinal health, schedule an appointment at any of our five Connecticut offices. Patients and referring physicians trust our team for our medical and surgical excellence, expert guidance, and compassion we bring to every case.
What is Best disease?
Best disease is a genetic retinal disorder named after Dr. Friedrich Best, a German ophthalmologist who described it in detail in 1905. This hereditary disease affects the macula, the central part of the retina responsible for sharp, fine details. Best disease often begins in childhood or adolescence, although many symptoms become noticeable in middle age.
The condition progresses through six stages, and symptoms do not appear until stage 4 or 5, when the egg-shaped, yellow material (vitelliform) begins to damage retinal cells. Since the disease is progressive, careful monitoring is essential to detect changes and preserve as much vision as possible over time.
What are the symptoms of Best disease?
You’ll probably discover you have Best disease during an eye exam, since you don’t usually experience symptoms. However, when symptoms appear, they include.
- Blurred vision
- Problems with central vision while side vision remains unaffected
- Difficulty reading or recognizing faces or colors
- Straight lines appearing wavy (metamorphopsia)
Vision changes in Best disease can be gradual or rapid. The final stage of the disease, choroidal neovascularization (CNV), causes growth of abnormal blood vessels at the back of the eye (neovascularization). These vessels are weak and can rupture, leading to fluid buildup in the retina. CNV affects about 20% of patients with Best disease and is the likely cause of sudden vision changes.
How do you diagnose Best disease?
Diagnosing Best disease involves looking for the characteristic egg yolk-like lesions observed in the vitelliform stage. Retina Consultants, P.C. uses state-of-the-art diagnostic tools to examine your retina at a microscopic level.
- Imaging tools, such as optical coherence tomography (OCT) and fundus photography, capture images of the retina.
- The electrophysiological (EOG) test assesses the health and activity of retinal pigment epithelium (RPE) at the back of the eye.
- Genetic testing: Alongside imaging tests, your retina specialist will recommend genetic testing as the definitive diagnostic test for Best disease.
These diagnostic tools enable our retina specialists to track disease progression and detect complications early, including fluid buildup, neovascularization, and macular holes.
What are the treatment options for Best disease?
Currently, there is no treatment available to cure Best disease or other bestrophinopathies. However, treatments are available to manage complications of Best disease.
These include:
- Observation: Many patients may only need regular monitoring, especially in the early stages.
- Anti-VEGF injections: Injecting anti-VEGF drugs, such as Avastin or Bevacizumab, into the back of the eye can help stop the growth of abnormal blood vessels (choroidal neovascularization).
- Laser therapy/ photodynamic therapy: Helps shrink or seal leaky blood vessels.
- Vitrectomy: Your doctor recommends this surgical procedure to address pronounced macular holes.
There is no one-size-fits-all treatment for Best disease. Your doctor tailors your treatment depending on the stage and severity of the disease. However, every decision is made to preserve vision and maintain quality of life.
Do you need treatment for Best disease in Connecticut?
Living with an inherited retinal disease can feel overwhelming, but you are not alone. The team at Retina Consultants, P.C. uses a compassionate, personalized approach to provide advanced retinal care. To get started, call us at
Hartford
Manchester
Cromwell
Farmington
Enfield
Best disease requires expert attention and long-term management. We are here to guide you every step of the way with expertise and personalized care.
