Orbital pseudotumor is a heterogeneous group of diseases of the orbit, including primary idiopathic myositis, dacryoadenitis, local and diffuse vasculitis. Common clinical manifestations of all forms are exophthalmos, pain in the eye socket, edema and hyperemia of the eyelids. Diagnosis of orbital pseudotumor includes external examination and palpation, ophthalmoscopy, visiometry, computed tomography, histomorphological and cytological examination. The tactics of conservative therapy consists in prescribing a course of corticosteroids in the form of retrobulbar injections. In the absence of the effect of drug treatment of orbital pseudotumor, an orbitotomy is performed.
ICD 10
H05.2 Exophthalmic conditions
General information
Orbital pseudotumor are considered as a polyethological group of pathologies of the visual organ of a nonspecific inflammatory nature. The concept of “pseudotumor” was introduced in 1970 by the German scientist F. Birch-Hirschfeld. At that time, this term referred to all volumetric neoplasms of the eye socket of a non-oncological nature, leading to clinical manifestations of exophthalmos. A significant contribution to the study of such processes was made by ophthalmologists J. Kennerdel and S. Drezner, who proposed the first morphological classification of this pathology. According to statistics, the prevalence of orbital pseudotumor among all diseases of the orbit is 5-12%. The disease is most common at the age of 30-50 years, cases of development are described at a younger age (11.5%). These pathological formations are more often diagnosed in women (54%).
Causes
Orbital pseudotumor is a polyethological disease. The cause of its development may be autoimmune disorders in the patient’s body. In some cases, pathology occurs in patients with lupus erythematosus, Wegener’s granulomatosis, nodular periarteritis, Graves’ disease, Hashimoto’s or Sjogren’s disease. Pseudotumor of inflammatory nature is often a manifestation of Benier-Beck-Schauman disease. When the organ-specific allele DRB10401 is affected, sarcoidosis is clinically manifested by the formation of granulomas in the eye socket. Orbital pseudotumor can develop in patients with systemic multifocal fibrosclerosis and be one of the first symptoms of this pathology.
The lesion of arterioles and venules with orbital pseudotumor is not always associated with systemic inflammatory processes. Histological examination of the vessels of the neoplasm allowed us to conclude about the development of local vasculitis. The cause is focal tissue hypoxia. At the same time, small infiltrates of an inflammatory nature can form around the vessels of the arterial bed, which confirms this theory of development. In some cases, orbital pseudotumor occurs in the form of diffuse nonspecific inflammation of the orbit.
Symptoms
Clinically, pseudotumor orbits are divided into primary idiopathic orbital myositis, dacryoadenitis, local and diffuse vasculitis. The features of the course depend on the form of pathology, in most cases the disease is characterized by an acute onset. Common clinical manifestations for all forms of orbital pseudotumor are swelling and hyperemia of the eyelids, pain in the projection area of the orbit with irradiation to the frontal lobe. Later, exophthalmos joins, which indicates a sharp increase in the orbital pseudotumor in volume. The pathology is characterized by chemosis of the bulbar conjunctiva.
With primary idiopathic myositis, swelling of the eyelids occurs against the background of complete well-being. Patients complain of pronounced soreness with eye movements. One of the first symptoms of the disease is diplopia, after which exophthalmos appears. Within a few weeks, complications of exophthalmos such as strabismus, lagophthalmos, keratopathies develop. Due to compression of the optic nerve, a total decrease in visual acuity is possible. Pain syndrome with localization in the orbit and head does not go away on its own, it is difficult to be relieved by analgesics. With this form of orbital pseudotumor, the reposition of the eye against the background of its complete immobility is difficult.
Dacryoadenitis is characterized by inflammation of the lacrimal gland. This form of pseudotumor orbit has a less severe flow. Patients complain of edema and ptosis of the eyelids. Exophthalmos is poorly expressed. The pain is determined by pressing at the site of the projection of the lacrimal gland. Local orbital vasculitis was previously considered as granuloma or lymphogranulomatosis. The disease has local and diffuse forms, characterized by rapid progression. It is clinically manifested by hyperemia of the eyelids, redness of the bulbar conjunctiva. Against the background of a relatively weakly expressed exophthalmos, there is a limitation of the mobility of the eyeball. A decrease in visual acuity is possible with the development of orbital pseudotumor in the area of the apex of the eye socket.
As a consequence of orbital pseudotumor, most patients develop sclerotic changes in the orbit. Patients at the stage of sclerosis also have exophthalmos of varying severity. It is possible to reduce visual acuity up to complete blindness as a result of optic nerve atrophy. In rare cases, the stage of sclerosis is accompanied by exacerbations, which are manifested by swelling of the eyelids, a feeling of a foreign body in the eye socket, pain syndrome.
Diagnostics
The diagnosis of orbital pseudotumor is based on anamnestic data, the results of external examination and palpation, ophthalmoscopy, visiometry, computed tomography (CT), histomorphological and cytological examination. With the help of computed tomography, it is possible to carry out a differential diagnosis of the oncological process with a orbital pseudotumor. Expanded extraocular muscles are visualized on CT orbits, the contour irregularity along the entire length is determined. Muscle tissue, as a rule, is of increased density and is adjacent to the fiber located in the retrobulbar space. In primary idiopathic myositis, visometry makes it possible to assess the degree of visual acuity reduction. During ophthalmoscopy, a stagnant disc of the optic nerve with signs of atrophy is detected.
The method of palpatory examination in dacryoadenitis determines dense mobile tissue with a smooth surface in the projection area of the lacrimal gland. To differentiate this type of orbital pseudotumor with an epithelial tumor, it is necessary to conduct a fine-needle aspiration biopsy followed by cytological examination of the punctate. Microscopic examination of a smear in the case of dacryoadenitis visualizes lymphocytes, pleomorphic cells and fragments of fibrous tissue. CT is used to determine an increase in the density of tissues surrounding the orbital pseudotumor, together with an increase in the size of the lacrimal gland.
In the case of vasculitis as one of the manifestations of orbital pseudotumor during ophthalmoscopy, a stagnant disc of the optic nerve can be detected. At the same time, the visiometry data indicate a decrease in visual acuity. CT scans visualize areas of increased density with a clear, even contour that do not adhere to the muscle tissue and optic nerve. In the case of sclerotic changes, retrobulbar tissue has the appearance of a heterogeneous high-density tissue with fuzzy boundaries and involvement of the optic nerve in the pathological process.
Treatment
The tactics of treating orbital pseudotumor depends on the form of pathology and the severity of the process. Conservative therapy is reduced to the appointment of nonspecific anti-inflammatory therapy with the use of corticosteroids. In this case, corticosteroids are administered in the form of retrobulbar injections. Systemic or local administration of glucocorticosteroids can provoke the development of dependence on steroids, resistance to drugs of this series, swelling of the periorbital fiber. Specialists in the field of ophthalmology are recommended to systematically repeat the course of treatment in order to prevent relapses of orbital pseudotumor. Auxiliary methods are pulsed electromagnetic therapy, physiotherapy and radiation (telegammatherapy) methods of exposure. Pulsed electromagnetotherapy is indicated for patients taking glucocorticoids. Telegammatherapy is used to relieve pain in the eye socket.
Surgical treatment of orbital pseudotumor is necessary in the absence of the effect of conservative therapy, the progression of sclerotic processes with severe pain syndrome. Surgical intervention can be performed using transconjunctival, transcutaneous or bone access. Bone plastic orbitotomy is often required. The tactics of surgical intervention depends on the data obtained during computed tomography.
Prognosis and prevention
No specific measures have been developed to prevent the development of orbital pseudotumor. Patients with a history of this pathology should repeat the course of corticosteroid treatment every 6-8 months in order to prevent the development of relapses. It is also recommended to be examined by an ophthalmologist every six months. Visiometry, ophthalmoscopy and biomicroscopy are mandatory. The prognosis for life and ability to work with pseudotumor orbit in case of timely diagnosis and treatment is favorable. At the same time, pathology is prone to progression and the development of relapses, which, in the absence of adequate therapy, can lead to loss of vision and disability of the patient.
