Eales syndrome is an ocular vasculitis associated with retinal damage, vitreous hemorrhage, and visual loss. The causes of the disease are as yet unclear and may correspond to a wide variety of contexts ranging from autoimmunological processes to tubercolotic phenomena. Symptomatic treatment of the syndrome encompasses medicinal and ophthalmic surgical steps.
What is Eales syndrome?
A condition called vasculitis is an inflammatory disease that affects the blood vessels. Vasculitides can occur in nearly any tissue and have different causes. Eales syndrome is a type of vasculitis and corresponds to an eye disease that results in retinal damage with loss of visual acuity and vitreous hemorrhage. The disease is named after its first author Henry Eales. The English ophthalmologist described the phenomenon already towards the end of the 19th century. In medical literature, the disease is also known as angiopathia retinae juvenilis or periphlebitis retinae. Sometimes the phenomenon is also referred to as idiopathic perivasculitis of the retina or idiopathic retinal pervasculitis. The prevalence for the ocular disease is unknown to date. Predominantly male adults are affected by Eales syndrome. The average age of manifestation is between 20 and 30 years of age.
Causes
To date, the causes of Eales syndrome are as poorly understood as the genesis of the disease. Since no familial clustering has been observed in the cases documented to date, it is probably not a hereditary disease. Common backgrounds could not be identified on the basis of the cases documented so far. Therefore, the disease remains idiopathic. The assignment to the group of vasculitides at least explains the reasons for the loss of visual acuity in the course of the syndrome. Inflammations of the supplying vessels form the initial stage of the syndrome. This inflammatory stage is accompanied by periphlebitis of the peripheral retinal areas. In the ischemic stage, there is sclerosis of the retinal veins, which in the subsequent stage of proliferation turns into vascular neoplasms with hemorrhage. These processes lead to retinal detachment. According to scientific assumptions, the syndrome has a multifactorial genesis consisting of autoimmunity, Mycobacterium tuberculosis, oxidative stress and possibly coagulopathy. In all cases, the disease occurs spontaneously and without an inflammatory or traumatic antecedent.
Symptoms, complaints, and signs
At disease onset, patients with Eales syndrome often have no manifest symptoms. Early symptoms of the disease exist, for example, with blurred vision that leads to a painless loss of vision. Visual acuity is only rarely worse than 20/200. Different symptoms refer to vascular damage: in the anterior chamber of the eye there is rubeosis iridis and in isolated cases glaucoma due to neovascularization. The posterior chamber of the eye shows vitreous hemorrhages as well as traction amotio. In later stages, proliferative retinopathy with retinal hemorrhages often occurs. Periphlebitis retinae is also among the most common symptoms. Central nervous system symptoms have been described as complications in isolated cases. These symptoms include mainly sensory disturbances, speech disorders, and loss of movement.
Diagnosis and course
Diagnosis of Eales syndrome involves differential exclusion of diseases such as sarcoidosis, retinal vein occlusion, and other proliferative retinal diseases. The diagnostic tool of choice is usually fluorescein angiography of the ocular fundus. In addition to this diagnostic test, sonography may be performed, which provides helpful information, especially in cases of retinal detachment and vitreous hemorrhage. An analysis of blood coagulation is also recommended by different authors. In general, patients with Eales syndrome have a rather favorable prognosis. Complete blindness has been documented only in isolated cases. Nevertheless, the course varies from person to person. Complete regression is conceivable, but equally conceivable is progression to retinal detachment. Recurrent complications have occurred in isolated cases.
When should one go to the doctor?
Since Eales syndrome can lead to complete blindness in the worst case, an examination by a physician should be performed in any case in this case. Self-cure does not occur with this disease. The doctor should usually be consulted if the affected person suffers from blurred vision or if there is a general deterioration of vision. This deterioration usually occurs very suddenly. Speech disorders or paralysis also indicate Eales syndrome and must be examined in any case. Similarly, Eales syndrome can cause limitations in movement, which must also be examined by a doctor. First and foremost, the ophthalmologist can be consulted for this disease. The earlier the disease is diagnosed, the higher the probability of a positive course of the disease. Since the symptoms of the disease may recur even after successful treatment, the affected person should attend regular examinations even after treatment.
Treatment and therapy
Causal therapy is not available for patients with Eales syndrome to date. The reason for this is the inadequate elucidation of causative factors. Causal therapies address the primary cause of a disease in order to stop the disease process at its root. By eliminating the cause, causal therapy steps also permanently resolve the individual symptoms. Although idiopathic Eales syndrome does not offer a causal approach, at least symptomatic treatment approaches are available. Symptomatic treatment steps depend on the symptoms in the individual case and are mainly stage-dependent in Eales syndrome. For example, in the perivasculitis stage, patients receive corticosteroids. This conservative drug treatment can correspond to a systemic or peri-ocular form of therapy. Systemic administration means the body-wide mode of action. In this context, the corticosteroids are administered perorally or via infusion and thus act on the entire organism via the bloodstream. More gentle and targeted is the peri-ocular administration at the site of the pathological event. Since speculation suggests that autoimmunological processes are probably involved in the inflammatory disease, immunosuppressants such as azathioprine or cyclosporine can be given on a trial basis. The patient’s immune system is dampened via these agents. Because of the tuberculosis factors that may be involved, some scientists recommend additional tuberculostatic therapy. However, this approach is considered controversial. In the proliferative stage, photocoagulation is the treatment option of choice. Vitreous retinal surgery may also be used, especially in cases of prolonged vitreous hemorrhage or impending adhesions with the risk of retinal detachment.
Outlook and prognosis
The course in Eales syndrome varies widely. Both regression and progression of the disease may occur. There is no causal therapy to date. It can only be treated symptomatically. Often there is a loss of visual acuity. Nevertheless, complete blindness is very rare even in untreated individuals. Problems occur when vitreous hemorrhages and retinal hemorrhages occur over a long period of time. Retinal detachments also occur. When vitreous hemorrhages last longer than three months or retinal detachment is imminent, vitreous-retinal surgery becomes necessary. Due to the constant formation of new vessels, growths develop which, in addition to retinal detachment, can also lead to glaucoma. Without treatment, the loss of eyesight is imminent. All treatment methods can alleviate the most severe symptoms, but have no influence on the course of the disease. Since the causes of Ealys syndrome are not yet fully understood, a causal therapy has not yet been found. As mentioned above, the symptoms can completely regress or progress both with and without treatment. If the disease progresses in a progressive manner, vascular degradation and new vessel formation constantly alternate with each other. During new vessel formation (proliferation), new cells are constantly being formed, resulting in proliferation. If there is a risk of retinal detachment caused by proliferation, photocoagulation is recommended as therapy to reattach the retina completely.
Prevention
To date, Eales syndrome cannot be prevented because the causes are still considered idiopathic. Only when the causative factors involved have been conclusively
ly clarified, preventive starting points will be available.
Follow-up
The measures or possibilities of aftercare are very limited in Eales syndrome. In any case, the affected person is dependent on a rapid diagnosis with subsequent treatment, as complete vision loss may occur if left untreated. This can be irreversible and thus can no longer be treated. Therefore, the main focus in Eales syndrome is early detection of the syndrome. If the first signs or symptoms of this disease appear, an ophthalmologist must be contacted immediately. In most cases, patients with Eales syndrome are dependent on taking medication to alleviate the symptoms. Attention should be paid to regular intake with a proper dosage. If there is any uncertainty or question, a physician should always be contacted first to avoid making incorrect assumptions. In the case of children, parents should check that they are taking the medication correctly. Furthermore, regular check-ups by an ophthalmologist are also very important to control the stage of Eales syndrome. In some cases, surgical intervention may also be necessary. After such a procedure, the eye should be particularly well protected, usually wearing a bandage around the eye.
What you can do yourself
Eales syndrome is a relatively rare eye disease whose causes are still largely unknown. Everyday life with this disease varies greatly depending on how severe the symptoms are. A characteristic feature of Eales syndrome is the bursting of blood vessels in the eyes. In the worst case, this leads to temporary blindness of one eye. It is very important to regularly visit the ophthalmologist, who can decide on further measures. In case of emergency, urgent action is required. This is especially true in the case of severe clouding of vision. In this case, the emergency doctor must be called. In everyday life it is very important to follow the recommendations and the instructions of the attending physician. In this way, it is often possible to lead a largely normal life for a long time. Especially in the case of rare diseases, such as Eales syndrome, it can be very helpful to exchange information with other affected persons. This can happen in a local self-help group or in an internet forum. Through the exchange with others, stressful situations can be endured more easily, experiences can be gathered and passed on, and valuable friendships can be made with similarly affected people. It does not even have to be a group in which only patients with Eales syndrome come together. Such groups are rather rare. An important contact could be the retina self-help group.
