Fissura-orbitalis-superior syndrome is characterized by the failure of several cranial nerves responsible for supplying various eye muscles as well as sensory innervation in the eye area. The clinical picture is very complex and is caused by space-occupying processes.
What is fissura orbitalis superior syndrome?
Fissura orbitalis superior syndrome represents a complex clinical picture due to the failure of several cranial nerves at once in the area of the fissura orbitalis superior. The fissura orbitalis superior is a large gap between the small and large sphenoid bone (Os sphenoidale). Through this cleft space, the middle cranial fossa (fossa cranii media) is connected to the orbit (orbita). The superior orbital fissure serves as an opening for various structures. Among them are the cranial nerves oculomotor nerve (nerve III), trochlear nerve (nerve IV), abducens nerve (nerve VI), and a branch of the trigeminal nerve (nerve V). The trigeminal nerve is a sensitive nerve divided into four branches. Of these, only the branch ophthalmic nerve (nerve V1) passes through this fissure. Furthermore, the superior orbital fissure is still crossed by the blood vessels ramus orbitalis arteriae meningeae mediae and superior ophthalmic vein. Therefore, the fissura orbitalis superior also gives the syndrome its name.
Causes
Space-occupying developments in the area of the fissura orbitalis superior are usually responsible for the fissura orbitalis superior syndrome. These can be tumors, aneurysms, or thrombosis. However, inflammatory processes can also impair the function of the corresponding cranial nerves. As a result of tumor growth or other space-occupying processes, displacement of nerves III, IV, VI and the partial branch of nerve V1 may occur. Nervae III, IV, and VI are motor cranial nerves and innervate entire groups of muscles around the eyes. Nervus ophthalmicus (V1) belongs to the sensory nerves and mediates sensory perceptions. In the context of fissura orbitalis superior syndrome, all nerves passing through the fissura orbitalis superior are often damaged. For example, oculomotor nerve (nerve III) innervates four of six external eye muscles, two internal eye muscles, and the eyelid elevator. Depending on which eye muscles are paralyzed, very complex disorders occur. In addition to restricted eye movement and squinting, eyelid elevation may be impaired and pupils may be frozen. Images are seen double and close-up vision is unsuccessful. The trochlear nerve (IV nerve) also innervates an external eye muscle. In case of its damage, strabismus also results in combination with double vision. Here, the eye deviates inward and upward. If the abducens nerve (nerve VI) is damaged, a so-called inward strabismus occurs. This nerve is responsible for the movement of the eye to the side. When the innervation of the rectus lateralis muscle is disturbed, the opposing rectus medialis muscle predominates. Finally, the ophthalmic nerve (V1 nerve) is responsible for perceptions in the eye area, which are disturbed when it fails.
Symptoms, complaints, and signs
The symptoms of fissura orbitalis superior syndrome are varied and complex because a whole range of muscles and muscle groups are affected. In addition, sensory innervation is also impaired. Massive movement disorders of the eyes occur. In extreme cases, there is complete paralysis of the eye muscles (ophthalmoplegia). The eyelid may droop partially or completely (ptosis). The near vision is disturbed (accommodation), whereby near objects are perceived only blurred. Furthermore, pupillary rigidity often occurs. The pupil no longer reacts to light because the sphincter pupillae muscle, which is responsible for the constriction of the pupil (miosis) when exposed to light, is also paralyzed. Very typical is the occurrence of massive headache. Sometimes the eyeball also protrudes due to the space-occupying processes (exophthalmos). Sometimes there is also a loss of corneal sensitivity. Due to the lack of tear flow, the eyes dry out. Superficial lesions develop, which lead to even larger defects. Superinfections can develop on these lesions. As a result, gradual and extremely painful corneal ulcers develop.
Diagnosis
For the diagnosis of fissura orbitalis superior syndrome, imaging methods such as magnetic resonance imaging and cranial computed tomography are used in addition to strabological examination methods. The strabological methods are intended to verify the squinting of the eyes. Differential diagnosis must exclude orbital apex syndrome with additional optic nerve (II nerve) involvement and sinus cavernosus syndrome.
Complications
In most cases, fissura orbitalis superior syndrome results in ocular complications and discomfort. Because the syndrome is relatively multifaceted, different restrictions can occur on the individual muscles of the eye area. In most cases, however, the patient can barely move the eyes, if at all. Vision is not reduced, but everyday life is made very difficult because the eyes are no longer mobile. Due to this immobility, the patient with fissura orbitalis superior syndrome also suffers from blurred images and can usually also only see objects in the vicinity in a blurred manner. As a result, most patients also suffer from dizziness and severe headaches. This can also lead to sleep disturbances and massive restrictions in everyday life. Operating machines or driving vehicles is usually not possible with fissura orbitalis superior syndrome. Since the flow of tears is also prevented, the eyes often dry out, which can lead to pain. Quality of life is greatly reduced by fissura orbitalis superior syndrome. Usually, the tumors responsible for fissura orbitalis superior syndrome can be removed. This requires either surgery or chemotherapy. The further success or complications depend strongly on the previous development of the disease. With a complete cure, life expectancy is not reduced.
When should you go to the doctor?
A doctor should be seen as soon as problems or changes occur in the eye area. If there is an inability to move the eye independently, there is cause for concern. A medical examination should be initiated to determine the cause and initiate treatment. Decreases in vision, changes in color perception, or a dry feeling in the eyes, should be evaluated by a physician. If itching occurs due to eye dryness, the risk of open sores increases. Since germs can enter the organism via this route and trigger further diseases, a doctor should be consulted. If internal pressure develops in the eye sockets or headaches occur, a visit to the doctor is necessary. If the affected person suffers from emotional tension in addition to the physical problems, these should be discussed with a doctor. Anxiety, panic or a depressive phase must be treated therapeutically. If the eyeball protrudes from the eye socket, this is considered unusual. In order not to trigger further illness or damage, a doctor should be consulted as soon as possible. If there are problems with vision in normal light conditions, a visit to the doctor is necessary. In the case of pain, disturbances in the perception of sharpness or infections of the eye, a doctor is needed to clarify the causes.
Treatment and therapy
Treatment of fissura orbitalis superior syndrome can only be successful if the causative disease is treated. Despite its complexity, the syndrome cannot be considered a disease in its own right. It always represents only one symptom or a symptom complex. Further diagnostic investigations must be performed to verify the cause. Often, in the case of tumors, surgery is the only possible last step. This applies equally to benign and malignant tumors. A benign tumor should be operated on if the impairments caused by the space-occupying processes become too severe. Malignant tumors and aneurysms pose a lethal threat even in the absence of fissura orbitalis superior syndrome and should therefore always be surgically removed if possible. Follow-up treatment of malignant tumors usually requires radiation therapy, chemotherapy, or a combination of both. If surgery is no longer possible, symptomatic treatment by administration of glucocorticoids may be attempted.
Outlook and prognosis
The prognosis of fissura orbitalis superior syndrome is tied to the patient’s causative disease.Since the syndrome occurs only as a consequence of an existing health disorder, the main cause must be found and clarified. In most patients, tumors, thromboses or aneurysms can be found as the cause. These cause damage to the cranial nerves, triggering problems with vision. In addition, the stage of the underlying disease is crucial for making an overall prognosis. In many cases, complex impairments are present that are difficult to treat adequately. In the case of tumor disease, for example, the time of diagnosis and the location of the tumor are fundamental to the success of treatment. If the causative disorder is completely cured, the patient has a good chance of experiencing an improvement in the symptoms of the fissura orbitalis superior syndrome. Nevertheless, freedom from symptoms is only possible in rare cases. In most cases, impairments of varying degrees remain, since the activity of the cranial nerves does not always regenerate completely. In addition, treatment of the underlying disease is often associated with complications and sequelae. Long-term therapy may be required or, as a follow-up treatment to cancer, therapies are used which, for the purpose of a cure, cause further damage to healthy tissue in the first step. This must be taken into account when making a prognosis.
Prevention
Fissura orbitalis superior syndrome cannot be prevented because it is caused by space-occupying processes. If benign brain tumors such as meningiomas exist, regular examination for possible growth is necessary. If there is a long-term risk that it will displace nerves, surgery should be considered.
Follow-up care
In most cases, there are no special or direct measures or options for follow-up care available to the affected person with fissura orbitalis superior syndrome. In this regard, the affected person is primarily dependent on the diagnosis and treatment of the disease, as this is the only way to prevent further complications and discomfort. In general, early diagnosis with early treatment has a very positive effect on the further course of the fissura orbitalis superior syndrome. In most cases, the affected person must undergo surgery to remove the tumor in the case of fissura orbitalis superior syndrome. In any case, bed rest should be observed after the operation so that the affected person can relax and rest. In any case, the patient should refrain from exertion and other stressful activities. In many cases, patients are also dependent on the support of friends and family in order to cope with everyday life. Since the tumor can spread to other parts of the body due to the fissura orbitalis superior syndrome, regular examinations should be performed to prevent this or to detect another tumor at an early stage. In most cases, therefore, the life expectancy of the affected person is also reduced by this disease.
This is what you can do yourself
Impairment of vision very often triggers a strong experience of anxiety in the affected person. Patients of the fissura orbitalis superior syndrome should therefore adopt various coping strategies to experience a good quality of life in everyday life despite the existing discomfort of the disease. Maintaining a zest for life, optimism and a positive basic attitude are important in order to cope well with the challenges of everyday life. A reorganization of leisure activities should take place so that the ill person can experience a good balance. Relaxation techniques can be used to reduce stress in everyday life. Yoga, meditation or autogenic training are offered in many sports centers and can also be used independently. They strengthen mental power and help to achieve an inner balance. To reduce the increased risk of accidents, the environment of the ill person should be adapted to his or her needs and health conditions. Self-confidence should be stabilized by building a sense of achievement. If the eye problems represent a reduction in well-being for the sufferer due to the optical blemish, glasses can be worn to conceal the area. The sufferer is well advised to be open about his or her condition in everyday life. To avoid headaches, rest periods and sufficient rest are important.Sleep hygiene should be optimized to allow for restful sleep.
