Sinus cavernosus syndrome is a neurologic symptom complex caused by compression of the cerebral blood supply sinus casvernosus. The leading symptoms are paralysis of the facial nerves, such as those of the eye muscles. Therapy depends on the primary disease in which the syndrome occurs.
What is sinus cavernosus syndrome?
The cavernous sinus is one of the sinus durae matris. It is a venous blood conductor that supplies the brain. In addition to tributaries, the structure contains several cranial nerves. The oculomotor nerve (IIIrd cranial nerve), trochlear nerve (IVth cranial nerve), ophthalmic nerve (V1st cranial nerve) and maxillary nerve (V2nd cranial nerve) run in the lateral wall of the blood duct. The abducens nerve (VIth cranial nerve) passes directly through the canvernous sinus. In sinus cavernosus syndrome, these nerves are affected by deficits. The result is various paralysis symptoms in the area of the face. The syndrome is a cranial nerve compression, which can have various causes and thus be present in the context of different primary diseases. The therapeutic approach to the syndrome depends on the cause or primary disease. A special form and at the same time possible cause of the sinus cavernosus syndrome is the neurological symptom complex of Tolosa-Hunt syndrome. This variant of the syndrome manifests not only in the typical gaze paralysis, but especially in severe pain.
Causes
The symptoms of cavernous sinus syndrome are caused by compression of the cavernous sinus. This compression may be associated with tumors of this structure, for example. Septic or aseptic thrombosis of the venous blood vessel may also compress the vein. An equally conceivable cause of compression is cerebral aneurysms of the inflowing internal carotid artery. Sometimes fistulas also form at the sinus cavernosus or carotid artery, exerting pressure on the blood vessels and cranial nerve tracts located there. In addition, as a result of trauma or apoplexy, hemorrhage may occur at the blood vessel, which may also have compressive properties. A much rarer cause is the inflammatory Tolosa-Hunt syndrome. In this neurologic syndrome, compression of the cavernous sinus is caused by granulomatous inflammation in the bony skull. The definitive cause of the inflammation has not yet been conclusively determined.
Symptoms, complaints, and signs
In clinical presentation, sinus cavernosus syndrome presents as a combination of facial nerve palsies. Oculomotor nerve palsies, as well as trochlear nerve palsies and abducens palsies, are characteristic. These paralyses are predominantly ocular muscle paralyses, resulting mainly in gaze deviation, vertical gaze deviations and inability to accommodate. In most cases, sensory disturbances of the upper half of the face are also present, which are caused by a failure of various trigeminal branches on the ophthalmic nerve or maxillary nerve. The expression of the syndrome is either unilateral or bilateral. Especially in the case of a cause such as Tolosa-Hunt syndrome, sinus cavernosus syndrome patients additionally often complain of sharp eye pain or headache. Depending on the cause, pulsatile exophthalmos may also develop. In addition, heteronymous hemianopsia may be present if the optic chiasm is compressed, for example, by pituitary tumors. Nonspecific accompanying symptoms include fever and impaired consciousness.
Diagnosis and course of the disease
The diagnosis of sinus cavernosus syndrome primarily requires specification of the cause. The syndrome itself can be diagnosed as early as gaze findings. The specification requires more complex diagnostics. Neurological findings include functional testing of the cranial nerves and trigeminal pressure points. In patients with sinus cavernosus syndrome, ophthalmoplegia and a failure of the corneal reflex can be detected. In order to clarify the cause, blood is also taken from the affected person, which is examined for inflammatory parameters by laboratory diagnostics. A lumbar puncture with subsequent CSF diagnostics can provide indications of malignant or inflammatory processes in the central nervous system and thus further specify the diagnosis. Imaging techniques such as CCT, MRI, or cerebral angiography help in the causal exclusion of tumors and cysts.The prognosis for sinus cavernosus syndrome depends on the cause. Tolosa Hunt syndrome patients, for example, have a more favorable prognosis than tumor patients.
Complications
First and foremost, sinus cavernosus syndrome results in very severe headaches. This pain often spreads to neighboring regions, so that pain may also occur in the teeth or ears. Furthermore, the affected persons suffer from a gaze paralysis, so that a usual movement of the eyes or the eyelids is no longer possible without further ado. Sensory disturbances can also occur in the face and have a very negative effect on the quality of life of the affected person. Similarly, the patient often develops fever, fatigue and exhaustion. Due to the permanent pain, many affected persons suffer from sleep disorders and thus from irritability or depression. Likewise, disturbances of consciousness or further loss of consciousness may occur. The treatment of the sinus cavernosus syndrome always depends on the root cause. It usually proceeds without complications, although in some cases surgical intervention is necessary. In most cases, after treatment there is a positive course of the disease and limitation of all symptoms. The patient’s life expectancy is not negatively affected by sinus cavernosus syndrome.
When should one go to the doctor?
Sinus cavernosus syndrome must always be treated by a doctor. Serious discomfort and complications can result from this disease, which in the worst case can lead to the death of the affected person. For this reason, in the case of sinus cavernosus syndrome, a medical professional must be consulted at the first signs. The earlier the disease is detected, the better is usually the further course. A doctor should be consulted if the patient experiences severe pain in the neck and head. Also a permanent and above all strong fever can point to the sinus cavernosus syndrome and should be examined likewise. Most patients suffer from disturbances in sensitivity and various sensory disturbances, and there may also be severe visual complaints that restrict the patient’s daily life. If these complaints occur, a doctor must be consulted immediately. Sinus cavernosus syndrome can be treated relatively well by a cardiologist. In emergencies or if an epileptic seizure occurs, an emergency physician should be called or the hospital should be visited. There may be no self-healing in sinus cavernosus syndrome. It is also possible that the affected person’s life expectancy may be reduced as a result of this condition.
Treatment and therapy
As with prognosis, treatment of sinus cavernosus syndrome depends on the cause. In the case of causes such as tumors or cysts, causal therapy of the symptoms can take place. This causative therapy usually corresponds to a surgical intervention in which the surgeon removes the compressing growth. Intervention may also need to occur if the syndrome is preceded by bleeding or thrombosis. At times, however, intravenous antibiotics may help if thrombosis is present. In the case of causes such as Tolosa-Hunt syndrome, on the other hand, no causal therapy is available, since the final etiology of the phenomenon is as yet unclear. Therefore, in this symptom complex, symptomatic treatment of the sinus cavernosus syndrome is more common. In most cases, patients are given intravenous corticosteroids for this purpose, which cause symptoms to remit after an average of three to five days. If remission does not occur or, in the case of tumors and cysts, the cranial nerves have suffered permanent damage, supportive symptomatic therapy with eye movement training is given. Ideally, the damage can be compensated for by targeted movements, or the patient at least learns to cope with it. Thus, his quality of life increases again.
Prevention
For causes such as Tolosa-Hunt syndrome, sinus cavernosus syndrome cannot be prevented. If the neurologic symptom complex is preventable at all, it is exclusively in the context of thrombosis, trauma, and possibly fistulae.
Follow-up
In most cases, those affected by sinus cavernosus syndrome have no direct or specific measures of immediate follow-up available to them.Since this disease is congenital, it usually cannot be completely cured. In order to prevent the recurrence of the sinus cavernosus syndrome in the descendants, affected persons should have a genetic examination and consultation in case they wish to have children. Self-cure cannot occur with this disease, and in the first place, the affected person should see a doctor at a very early stage for early treatment. As a rule, most of the symptoms of this disease can be alleviated relatively well by taking various medications. The affected person should always ensure that the medication is taken regularly and in the correct dosage. If there are any uncertainties or questions, a doctor should be contacted first, and he or she should also be consulted in the event of severe side effects. Likewise, many of those affected are dependent on the help and also the care of their own family in their daily lives. This may also prevent depression and other psychological upsets.
This is what you can do yourself
In everyday life, due to a gaze paralysis, the risk of accidents of the affected person is significantly increased. Therefore, care must be taken to accept sufficient help and support. Already at the first health impairments, cooperation with a doctor should be sought. Self-help measures are not sufficient to achieve freedom from complaints or considerable alleviation of complaints. The complaints affect the facial area in particular. For this reason, states of emotional distress often occur in addition to the physical complaints. A healthy self-confidence should be built up in order to minimize the risks of psychological sequelae. The social environment should be informed about the disease as well as its symptoms. This can minimize unpleasant situations or misunderstandings. Since the occurrence of headaches is characteristic of the disease, stressors of all kinds should be reduced. These could otherwise contribute to a worsening of the situation. Also helpful are relaxation procedures and the use of mental techniques. In a large number of sufferers, improvement in symptoms can be achieved through the use of mental techniques. In addition, it is often reported that the handling of the disease is thereby improved. With fever it is to be made certain that sufficiently liquid is taken up. Dehydration should be prevented under all circumstances.
