Claude Bernard-Horner syndrome is a symptom complex associated with a violation of the nervous system. The ailment was named after the Swiss ophthalmologist Horner, who described him in 1869. However, other doctors have previously described the symptom complex. For this reason, in the medical circles of Italy and France, the disease was named after two doctors Bernard and Horner.
What it is
In fact, Claude Bernard-Horner syndrome is in most cases a secondary pathology that appears against the background of another disease. It was found that in 75% of cases the syndrome appears due to blockade of the brachial plexus, against the background of the use of a large number of anesthetic agents.
Oculosympathetic syndrome often appears in the presence of malignant neoplasms in the lungs or with mediastinitis, with other severe lesions of the chest or neck.
The disease is equally common in both sexes and appears in the eye area. As a result, eyeballs, muscle tissue of compounds can suffer. In children, the syndrome is often manifested by multi-colored irises, on one of which it is lighter. Fortunately, this symptom complex is a rare type of pathology.
Causes
In fact, there are many more reasons for the appearance of the syndrome. To date, even congenital anomaly is isolated. Usually pathology occurs due to birth injury. There are even descriptions of the hereditary form, which appeared in an autosomal dominant manner. But still, the disease is most often acquired.
The most common causes of Claude Bernard-Horner syndrome:
- Iatrogenic intervention. It may appear against the background of blockade of the cervical plexus or with incorrect placement of drainage according to Bulau.
- Injury from blunt objects to the base of the neck or chest.
- Otogenic pathologies or the spread of infection to neighboring organs and tissues with otitis media.
- Vascular pathology. Often, an aortic aneurysm becomes a provocateur.
- Neurological pathology. For example, multiple sclerosis or Dejerine-Klumpke paralysis.
- Thyroid disease.
Often, the pathology appears after an ischemic stroke.
The main external manifestations of the syndrome
The presence of the disease is often very simple to notice, it manifests itself in the form of myosis - a narrowing of the pupil. Normally, the pupil width should be in the range 2.75-4.75 mm, that is, on average it is 3.5 mm. If the pupil is narrowed to 2.5, then we can talk about miosis, although in elderly people and in newborns they are always in this state. Men have less pupils than women. Most often, the pathology is observed on one pupil.
The second characteristic symptom is ptosis. This is a prolapse or prolapse of the upper eyelid. And the third symptom is enophthalmos or retraction of the eyeball.
In the presence of all three symptoms that are observed simultaneously, with almost a 100% guarantee, we can talk about the presence of the syndrome. Most often, symptoms are observed on one half of the face, which is a consequence of the effect of the triad.
Other symptoms
But it is possible to observe in the patient not only ptosis of the upper eyelid. The reasons for this are because the syndrome is characterized by paresis of the muscles of the eyelid, orbital muscle. Therefore, quite often the patient has a violation in the adaptive ability of the pupil, that is, the patient’s eye is not able to adapt to different degrees of illumination.
In addition, the patient may have a swelling in the affected nerve, there may be a change in eye color (heterochrony). In some patients, Petit syndrome is observed, that is, the blood vessels of the eye expand as much as possible, which causes redness of the eye.
However, in comparison with the symptom of retraction of the eyeball, the above manifestations are quite rare, since they are not the main manifestations of the syndrome.
Classification
In addition to idiopathic (pathology independent of other diseases) and secondary type (symptom complex acts as a consequence of another disease), the syndrome is classified by the degree of damage to the neural chain. If the first neuron is affected, then most likely the disease arose against the background of damage to the spinal cord. If it is possible to establish that the second neuron is affected, then we can say that compression of the sympathetic nerve occurs against the background of an increase in the neoplasm. The defeat of the third neuron often occurs against the background of damage to the motor fibers.
Diagnostic measures
For diagnosis, a physical examination is performed and special diagnostic techniques are used. Without fail, the causes of ptosis of the upper eyelid, narrowing of the pupil are analyzed, because such symptoms can also speak of another disease. First of all, the doctor conducts a cocaine test. Special drops that contain cocaine sulfate are injected into the eyes. If the syndrome is absent, then the pupils dilate almost instantly, but if there is a disease, mydriasis will not.
The causes of myosis are ascertained using an oxamphetamine test. This substance should cause persistent dilatation of the pupil, but if the third neuron is damaged, then no changes will be observed. Then we can talk about the presence of the syndrome.
The test with differences in lighting allows you to find out the time it takes the patient’s eyes to adapt. Computed tomography makes it possible to visualize volumetric formations of the orbit, to find out whether there were injuries or other damage to the eyes. In some cases, other diagnostic techniques may be recommended. For example, an x-ray, which will determine the presence or absence of a neoplasm.
Therapy
Admittedly, Claude Bernard-Horner Syndrome is more of a cosmetic defect, but can bring a number of inconveniences to the patient. However, if the syndrome appeared against the background of any disease, then first of all it is necessary to treat it.
Drug treatment of the syndrome gives good results. But the appointment of eye drops should be done solely by the doctor.
When prescribing, the doctor will take into account the symptoms of Claude Bernard-Horner syndrome, the duration of the disease and the causes. Ophthalmic preparations are designed to stimulate the functioning of muscles and nerves, to correct vision and compensate for excessive narrowing of the pupil. Among the most common drugs are Apralonidine, Cytoflavin, Clonidine, and Procain.
With the hormonal nature of the origin of the disease, hormone replacement therapy is used.
Plastic surgery
In the presence of Claude Bernard-Horner syndrome, it is advisable to resort to plastic surgery if the manifestations of the disease are too pronounced. The operation allows you to restore the normal shape of the eyelid and palpebral fissure.
As a result, the patient is saved from ptosis and a cosmetic defect is eliminated, which radically changes the patient's appearance for the better. However, surgery is indicated in the late, advanced stages of the disease.
Other techniques
Claude Bernard-Horner syndrome can be treated with kinesitherapy along with neurostimulation. The essence of the technique is the effect of electric shock on the affected nerves and muscles. At the same time, it is possible to act on the transverse and smooth muscles. This type of treatment can improve the circulation of lymph and blood, activate the metabolism. However, one should know that such a technique is rather painful, which the doctor must warn the patient about. The doctor himself, who carries out such procedures, must have a very high qualification, since even one incorrect movement can lead to aesthetic and cosmetic injuries on the face.
Kinesitherapy is a special therapeutic load (stimulating massage) on the organs of vision.
Possible complications
Every person diagnosed with the syndrome should understand the degree of risk if untreated. First of all, the disease is dangerous because an inflammatory process can begin in the organs of vision: blepharitis, conjunctivitis or keratitis. Sinking of the eyeball contributes to the increased penetration of infectious agents into the organ of vision. In severe cases, even an orbital abscess develops or phlegmon forms.
There is a certain risk of xerophthalmia due to changes in the topography of the eyeball.
Congenital syndrome, which is quite rare, often leads to the development of heterochromia.
One of the most common complications is secondary hemeralopia. Such a disease is difficult to treat with traditional methods of treatment.
Prevention and prognosis
The syndrome is regarded as a unique disease, therefore, it is rather difficult to name specific measures to prevent the development of symptom complex. To date, in medical practice there are no special preventive measures.
However, the slightest manifestations of the syndrome should be an occasion to contact a medical institution to determine the cause of the disease. Only timely treatment and the correct determination of the root cause can prevent the development of serious consequences.
The prognosis largely depends on how timely the disease was detected. The risk of an adverse outcome increases if the cause of the syndrome cannot be eliminated.