Diseases of the orbit in children

Diseases of the orbit in childhood can be associated with developmental disorders, but may also have acquired character. Children with acquired pathology of the orbit, as a rule, have complaints and symptoms, peculiar to the growth of tissue inside the orbit. Patients are concerned:

• decreased vision;
• limitation of mobility of the eyeball;
• pain and manifestations of the inflammatory process;
• exophthalmos.

In children, orbital diseases are mainly represented by structural pathology (including cysts) and neoplasms, while in adults the pathology of the orbit is inflammatory in 50% of cases, and structural disorders occur less than in 20% of cases.

When examining a child with orbital pathology, careful collection of an anamnesis and conducting an adequate examination greatly facilitate the differential diagnosis. Important factors are the age at which the disease first appeared, the location of the pathological focus and the duration of orbital problems.

Ophthalmic examination includes:

• visual acuity check;
• assessment of movements of the eyeball;
• exophthalmometry;
• research on a slit lamp;
• examination of the pupil's condition (special attention is paid to afferent pupillary reactions);
• the study of refraction in the state of cycloplegia;
• examination of the fundus;
• a general examination (of particular importance in cases of suspected neurofibromatosis, juvenile xanthogranuloma and histiocytosis of Langerhan's cells).

Most children with orbital pathology need a neuroendgenological study, which includes:

• standard radiography;
• X-ray of the paranasal sinuses;
• computed tomography (CT);
• magnetic resonance imaging (MRI).

[1], [2], [3], [4], [5], [6], [7], [8]

Inflammatory processes of the orbit

Inflammatory diseases of the orbit in children can be divided into nonspecific (previously referred to as pseudotumors) and specific, such as sarcoidosis and Wegener's granulomatosis. The frequency of their occurrence increases in the second decade of life, when the pathology of the orbit in children begins to resemble that of adults.

Nonspecific inflammatory diseases of the orbit

They are acute and subacute inflammatory processes of unknown etiology, the clinical manifestations of which depend on the localization of the inflammatory focus:

1. Idiopathic inflammation of the anterior part of the orbit. The most common form of nonspecific inflammatory process that occurs in childhood. Pathology is limited to the anterior part of the orbit and the adjacent surface of the eyeball.

Manifestations include:

• pain;
• exophthalmos;
• swelling of the eyelids;
• concomitant conjunctivitis;
• decreased vision;
• concomitant anterior and posterior uveitis.

2. Diffuse idiopathic inflammation of the orbit. Clinically it resembles an inflammation of the anterior part of the orbit, but, as a rule, has a more severe course and is characterized by:
   • more pronounced limitation of mobility of the eyeball;
   • a more significant decrease in visual acuity due to attached retinal detachment or atrophy of the optic nerve;
   • diffuse inflammatory changes in the orbit.
3. Idiopathic myositis of the orbit. This inflammatory disease is characterized by:
   • pain and limitation of mobility of the eyeball (usually there is a paresis of the muscles involved in the pathological process);
   • doubling;
   • ptosis;
   • edema of the eyelids;
   • chemosis of the conjunctiva;
   • in some cases exophthalmos.

Visualization of neurological studies reveals hypertrophy of muscles and tendons, which distinguishes this disorder from orbital diseases of thyroid origin, in which the tendons are not enlarged in volume.

4. Idiopathic inflammation of the lacrimal gland. An easily diagnosed form of the inflammatory process that manifests itself;
   • pain, swelling and soreness of the upper eyelid;
   • ptosis and "S-shaped" deformation of the upper eyelid;
   • the shift of the eyeball down and medially;
   • chemosis of the conjunctiva in the upper-temporal segment;
   • absence of concomitant uveitis.

Visualization of neurological studies reveals a focus of inflammation, localized in the lacrimal gland, while the adjacent surface of the eyeball is often involved in the process.

Virtually all forms of nonspecific orbital inflammation respond well to steroid medications, although inflammation of the anterior orbit and diffuse inflammation may require a longer course of treatment than with myositis or idiopathic inflammation of the lacrimal gland.

Specific inflammation of the orbit

1. Wegener's granulomatosis. Necrotic granulomatous vasculitis mainly affects the respiratory tract and kidneys. In children it is rare.
2. Sarcoidosis. Granulomatous inflammatory disease of unknown etiology; can cause infiltration of the optic nerve and external eye muscles, uveitis and hypertrophy of the lacrimal gland. There are isolated reports of the onset of this pathology in adolescence.
3. Thyroid orbitopathy. This disease is a common cause of exophthalmos in older children. In general, thyroid orbitopathy proceeds more easily in children than in adults. Thus, neuropathy of the optic nerve, severe corneal pathology, as well as gross changes in the external muscles of the eye in childhood are rare.

Histiocytosis, pathology of the hematopoiesis system and lymphoproliferative disorders

The pathology of the orbit and the organ of vision occurs with three forms of histiocytosis.

1. Langerhan-cell histiocytosis (histiocytosis X). A rare disease characterized by local proliferation of pathologically altered histiocytes in various tissues. The process can involve:
   • leather;
   • bones;
   • spleen;
   • liver;
   • The lymph nodes;
   • lungs.

Involvement in the pathological process of the organ of vision occurs frequently, while various structures of the eyeball are affected, including:

• the vascular membrane is most often observed in children in the first year of life;
• the optic nerve, the chiasmus, or the optic tract;
• III, IV, V and VI pairs of cranial nerves;
• orbit - usually the parietal and frontal bones are involved in the process, with the formation of destructive foci.

In a situation threatening to lose sight, prescribe depomedron, steroid drugs or radiation therapy. The choice of medicines depends on what tissues are involved in the pathological process. With an isolated lesion of one body system (for example, a skeleton), the prognosis is favorable. The outlook deteriorates sharply in the generalized or visceral form of the disease. The mortality of children under the age of two years (in which the generalized lesion develops especially often) is 50-60%. In older children, mortality is lower.

2. Other forms of histiocytosis.

Juvenile xanthogranulem is a disease of unexplained etiology, characterized by pathological proliferation. Patognomonichnym symptom is the defeat of the skin. Involvement in the process of the organ of vision is observed in less than 5% of patients and is manifested by damage to the iris, ciliary body and choroid. In typical cases, lesions of the iris have the appearance of yellowish or cream foci and are accompanied by a risk of spontaneous hyphema and secondary glaucoma. Effectively the general use of steroid preparations. Involvement of the orbital and epibulbar tissues in the inflammatory process is rare.

3. Histiocytosis of the paranasal sinuses.

Hystiocytosis of the paranasal sinuses is a disease of unexplained etiology, mainly affecting children and young people. Characterized by widespread painless lymphadenopathy in the neck and concomitant lesion of the orbit, upper respiratory tract, salivary gland, skin and skeleton.

The pathological process involves soft tissues of the orbit, without affecting its bone walls. In some cases, progressive exophthalmos and concomitant pathology of the optic nerve develop.

Assign steroid drugs in high doses, general chemotherapy and radiation therapy.

3. Leukemia.
4. Lymphoma.