Orbital diseases in children

Orbital diseases in childhood may be associated with developmental disorders, but may also be acquired. Children with acquired orbital pathology usually have complaints and symptoms characteristic of tissue growth inside the orbit. Patients are concerned about:

• decreased vision;
• limitation of eyeball mobility;
• 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, orbital pathology in 50% of cases has an inflammatory origin, and structural disorders occur less than 20% of cases.

When examining a child with orbital pathology, careful anamnesis and adequate examination significantly facilitate differential diagnosis. Important factors include the age at which the disease first manifested itself, the location of the pathological focus, and the duration of the orbital problems.

Ophthalmological examination includes:

• visual acuity test;
• assessment of eye movements;
• exophthalmometry;
• slit lamp examination;
• examination of the pupil's condition (special attention is paid to afferent pupillary reactions);
• refraction study in cycloplegia;
• fundus examination;
• general examination (of particular importance in cases of suspected neurofibromatosis, juvenile xanthogranuloma and Langerhans cell histiocytosis).

Most children with orbital pathology require neuroradiological examination, including:

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

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Inflammatory processes of the orbit

Inflammatory diseases of the orbit in children can be divided into non-specific (previously referred to as pseudotumors) and specific, such as sarcoidosis and Wegener's granulomatosis. Their incidence increases in the second decade of life, when orbital pathology in children begins to resemble that in adults.

Non-specific 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 orbit. The most common form of non-specific inflammatory process occurring in childhood. The pathology is limited to the anterior 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 orbital inflammation. Clinically resembles anterior orbital inflammation, but is usually more severe and is characterized by:
   • more pronounced limitation of eyeball mobility;
   • a more significant decrease in visual acuity caused by additional retinal detachment or optic nerve atrophy;
   • diffuse inflammatory changes in the orbit.
3. Idiopathic orbital myositis. This inflammatory disease is characterized by:
   • pain and limited mobility of the eyeball (usually paresis of the muscles involved in the pathological process occurs);
   • double vision;
   • ptosis;
   • swelling of the eyelids;
   • conjunctival chemosis;
   • in some cases, exophthalmos.

Neurological imaging studies reveal muscle and tendon hypertrophy, which distinguishes this disorder from thyroid-related orbital diseases in which tendons are not enlarged.

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

Neurological imaging studies reveal a focus of inflammation localized in the lacrimal gland, with the adjacent surface of the eyeball often involved in the process.

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

Specific inflammations of the orbit

1. Wegener's granulomatosis. Necrotizing granulomatous vasculitis primarily affecting the respiratory tract and kidneys. Rare in children.
2. Sarcoidosis. A granulomatous inflammatory disease of unknown etiology; may cause infiltration of the optic nerve and extraocular muscles, uveitis, and hypertrophy of the lacrimal gland. There are isolated reports of this pathology occurring in adolescence.
3. Thyroid orbitopathy. This disease is a common cause of exophthalmos in older children. In general, thyroid orbitopathy is milder in children than in adults. Thus, optic neuropathy, severe corneal pathology, and gross changes in the external muscles of the eye are rarely observed in childhood.

Histiocytosis, pathology of the hematopoietic system and lymphoproliferative disorders

Pathology of the orbit and organ of vision occurs in three forms of histiocytosis.

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

Involvement of the visual organ in the pathological process is common, with various structures of the eyeball being affected, including:

• choroid - most often observed in children in the first year of life;
• optic nerve, chiasm, or 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 situations threatening vision loss, depomedron, steroids, or radiation therapy are prescribed. The choice of drugs depends on which tissues are involved in the pathological process. With isolated damage to one body system (for example, the skeleton), the prognosis is favorable. The outlook is sharply worsened by generalized or visceral forms of the disease. The mortality rate of children under two years of age (who are especially likely to develop generalized damage) is 50-60%. Mortality is lower in older children.

2. Other forms of histiocytosis.

Juvenile xanthogranuloma is a disease of unknown etiology, characterized by pathological proliferation. The pathognomonic symptom is damage to the skin. Involvement of the visual organ in the process is observed in less than 5% of patients and is manifested by damage to the iris, ciliary body and choroid. In typical cases, damage to the iris has the appearance of yellowish or cream-colored foci and is accompanied by the risk of spontaneous hyphema and secondary glaucoma. General administration of steroid drugs is effective. Involvement of the orbit and epibulbar tissues in the inflammatory process is rare.

3. Histiocytosis of the paranasal sinuses.

Histiocytosis of the paranasal sinuses is a disease of unknown etiology, mainly affecting children and young adults. It is characterized by widespread painless lymphadenopathy in the neck and associated lesions of the orbit, upper respiratory tract, salivary gland, skin and skeleton.

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

High doses of steroid drugs, general chemotherapy and radiation therapy are prescribed.

3. Leukemia.
4. Lymphoma.