Horner’s syndrome is a symptom complex caused by a lesion of the sympathetic nervous system, manifested by ophthalmological disorders, disorders of sweating and vascular tone on the side of the lesion. The clinical picture is represented by ptosis, myosis, endophthalmos in combination with hyperemia and dyshydrosis of half of the face. To make a diagnosis, a test with oxamphetamine, biomicroscopy of the eye, CT of the eye sockets, a test to study the reaction of the pupils is used. Therapeutic tactics are reduced to the appointment of electrical neurostimulation. Correction of cosmetic defects with the help of plastic surgery is possible.
G90.2 Horner’s syndrome
Horner’s syndrome (Bernard-Horner syndrome, oculosympathetic syndrome) is a secondary pathology that develops against the background of other diseases. In 75% of cases, the occurrence of the acquired form is associated with the blockade of the brachial plexus by supraclavicular access when using a large volume of anesthetic. Sometimes the appearance of the syndrome indicates severe lesions of the neck or chest (malignant neoplasms of the lungs, mediastinitis). The disease was first described by the Swiss ophthalmologist Johann Friedrich Horner in 1869. Pathology occurs with the same frequency among men and women.
Causes of horner’s syndrome
There are congenital or acquired forms of the disease. The congenital variant occurs due to birth trauma. Cases of inheritance of oculosympathetic syndrome by autosomal dominant type are described, while there may be no signs of involvement of the autonomic nervous system in the pathological process. The main reasons for the development of the acquired variant:
- Iatrogenic intervention. Horner’s syndrome is a frequent complication of incorrect drainage by Bulau. Symptoms also appear after the intersection of nerve fibers, blockage of the cervical plexus or stellate ganglion in the chest or neck.
- Traumatic injuries. The disease is often caused by a blunt trauma to the base of the neck with a lesion of the thoracic or cervical sympathetic nervous chain. Clinical manifestations are observed on the side opposite from the injury zone.
- Otogenic pathologies. The disease can occur as a result of the spread of infection from the middle ear cavity with otitis media. This is facilitated by perforation of the eardrum and a history of labyrinthitis.
- Malignant neoplasms. Compression of nerve fibers by a tumor leads to the appearance of clinical symptoms of Horner’s syndrome. The most common causes are a Pancost tumor or bronchogenic carcinoma of the apex of the lung.
- Neurological diseases. The development of pathology may be associated with lateral medullary syndrome, Dejerin-Klumpke paralysis or multiple sclerosis. Less often, syringomyelia becomes an etiological factor.
- Vascular pathologies. A common cause of Horner’s syndrome is an aortic aneurysm. Local changes at the level of the arteries supplying the affected area can also act as a trigger.
- Diseases of the thyroid gland. Oculosympathetic syndrome may occur against the background of thyroid-cervical venous dilation or goiter. There is often a relationship between the manifestations of the disease and thyroid hyperplasia.
The development of the disease is based on a central, pre- or post-ganglionic lesion of sympathetic nerve fibers. Due to a violation of the innervation of the upper tarsal muscle or the Muller muscle, a classic drooping of the upper eyelids or inverted ptosis occurs. The severity of the ciliospinal reflex decreases. Pathological pigmentation of melanocytes forming the basis of the iris leads to heterochromia. The conjunctiva looks hyperemic, which is caused by vasodilation in paralysis of smooth muscles or pathology of sympathetic vasoconstrictor reaction. Paralysis or paresis of the orbital muscle of the eye leads to enophthalmos. The symptoms of the disease develop from the opposite side of the affected area.
Symptoms of horner’s syndrome
The pathology is characterized by a one-sided course. Patients complain of drooping of the upper eyelid or slight elevation of the lower one. Anisocoria caused by narrowing of one of the pupils is visually determined. On the affected side of the face, there is a violation of sweating and hyperemia. The eye looks set deep into the eye socket, the severity of endophthalmos is insignificant. Clinical symptoms are aggravated by narrowing of the ocular slit. The allocation of tears is sharply difficult, in rare cases the eye seems “wet”. Due to persistent miosis, dark adaptation worsens, patients note that they have become worse at dusk, although visual acuity persists. The development of the disease in childhood often leads to the fact that the iris has a lighter shade from the side of the lesion.
Violation of innervation is a predisposing factor to the development of inflammatory diseases of the anterior segment of the eye (conjunctivitis, keratitis, blepharitis). Due to the altered topography of the eyeball in relation to the lacrimal gland, its dysfunction occurs, this potentiates the development of xerophthalmia. The occlusion of the eye promotes the penetration of infectious agents into the orbit. In severe cases, the formation of phlegmon or subperiosteal abscess of the orbit is possible. Most patients develop secondary hemeralopia, which does not respond to classical methods of treatment.
Diagnostics of horner’s syndrome
The diagnosis is based on the results of physical examination and special research methods. Examination reveals myosis, heterochromia and occlusion of the eyeball on the affected side. The reaction of the pupils to light is sluggish or not determined. Basic diagnostic methods:
- Oxamphetamine test. It is used to determine the cause of myosis. If the third neuron is not involved, the use of amphetamine leads to the appearance of persistent mydriasis. In conclusion, they indicate at what interval the nerve pathway is damaged.
- Biomicroscopy of the eye. There is an injection of conjunctival vessels. The transparency of the optical media of the eye is not reduced, the structure of the iris is heterogeneous. There may be an uneven distribution of pigment.
- A test to determine the delay in pupil dilation. In Horner’s disease, ptosis is combined with a narrowed pupillary opening. When the oculomotor nerve is affected, the study reveals a combination of drooping eyelid with mydriasis.
- CT orbits. The study is carried out to determine the etiology of the disease. Computed tomography makes it possible to visualize the volumetric formations of the eye socket and traumatic damage to the organ of vision.
Differential diagnosis is carried out with anisocoria of various genesis and Pti syndrome. If anisocoria is detected, patients are subject to further examination, since this symptom can also be observed in violation of cerebral circulation, anomalies in the development of the eye. Pti disease is often referred to as reverse oculosympathetic syndrome. In this pathology, mydriasis, exophthalmos, dilation of the eyelid folds, ophthalmohypertension are determined, which are not characteristic of the classical oculosympathetic syndrome.
The disease is difficult to treat. A certain effectiveness of the use of neurostimulation has been proven. Due to low-amplitude electrical impulses, the affected muscles are stimulated, this contributes to the improvement of trophic and full or partial restoration of lost functions. If the disease occurred against the background of hormonal imbalance, hormone replacement therapy is indicated. If the effectiveness of the method is low, prompt elimination of the cosmetic defect is recommended. The use of plastic surgery techniques allows for the correction of ptosis and enophthalmos. The possibility of using kinesiotrapy in the treatment of the disease is being studied. The essence of the technique is to perform a massage to stimulate the affected areas.
Prognosis and prevention
The outcome of Horner’s syndrome may be unfavorable in prognostic terms if it is impossible to eliminate the cause of damage to nerve fibers. If the etiological factor is hormonal dysfunction, the appointment of substitution therapy allows you to stop all manifestations of the disease. Specific preventive measures have not been developed. Nonspecific prevention is reduced to the timely diagnosis and treatment of diseases of the ENT organs and thyroid gland, hormonal correction. If the clinical picture of the disease is associated with volumetric formations of the eye socket, patients should be registered with an ophthalmologist at the dispensary. CT of orbits in dynamics is recommended.