Tumors of the optic nerve in children

Gliomas (astrocytomas) can affect the anterior section of the optic pathway.

It is also possible to develop gliomas in the orbit and the cranial cavity.

Glioma of the optic nerve (orbital part of it)

In childhood, these tumors manifest themselves in a variety of symptoms:

• axial exophthalmos;
• decreased vision due to optic atrophy;
• strabismus with or without mobility of the eyeball;
• congestive optic nerve disc;
• formation of optociliary shunts.

The characteristic signs of this pathology in the radiographic study are:

• the tortuosity of the optic nerve directly in the retrobulbar area;
• expansion of the optic nerve canal.

Gliomas of the optic nerve refer to slowly growing piloid astrocytomas. Tactics of treatment so far remains controversial. In many cases, the functions remain unchanged, and the situation is stable for a long period.

Glioma Chiasma

A more common growth than the glioma of the orbit. Clinical manifestations usually include:

• bilateral decreased vision;
• nystagmus (possible mimic spasmus nutans);
• strabismus;
• bilateral atrophy of the optic nerve;
• stagnant nipple;
• delay in development;
• Exophthalmos are rare if the optic nerve is not involved in the pathological process.

The tactics of treatment remain controversial, as in many patients the situation remains stable for a long time. The mandatory components of treatment are endocrinological examination and correction of identified endocrinological disorders, as well as, if necessary, hydrocephalus treatment. In patients with progressive reduction in vision, the use of radiation and chemotherapy is justified.

Meningioma

Very rarely occurs in children, but occurs in adolescence.

Shelling meningioma of the optic nerve

It shows itself:

• loss of vision;
• slight exophthalmos;
• strabismus and double vision;
• atrophy of the optic nerve;
• presence of opticiliary shunts.

Loss of vision can occur in the early stages of the disease and be complete if the tumor is located inside the optic nerve canal. Treatment measures are usually impractical, although sometimes surgical removal of the tumor and the appointment of radiation therapy. This pathology often accompanies NF2.

Extraorbital meningioma

In this pathology, the wing of the sphenoidal bone, the area above the Turkish saddle or the olfactory furrow are involved in the process. Reduction of vision is associated with compression of the anterior part of the optic pathway. Rarely found in childhood.

Rhabdomyosarcoma

Primary malignant neoplasms of the orbit in children are extremely rare, the most common of these is rhabdomyosarcoma. In most cases, the tumor develops at the age of 4-10 years, but there are data on the appearance of this pathology in infants. Family cases are described that are attributed to a mutation of the p53 gene located on the short arm of the 17 chromosome.

Clinical manifestations of the disease include:

• exophthalmos, which can appear unexpectedly, for several days, and tends to increase;
• erythema and edema of the eyelid;
• ophthalmoplegia;
• ptosis;
• neoplasm in the thickness of the eyelid, accessible by palpation.

Rhabdomyosarcoma is actively spread due to invasive growth, in some cases, involvement of the anterior or middle cranial fossa, pterygoid fossa or nasal cavity is observed.

CT in typical cases demonstrates a homogeneous neoplasm, with fuzzy boundaries. To clarify the diagnosis, a biopsy is necessary.

The histological classification of rhabdomyosarcomas includes the following three groups:

1. anaplastic;
2. monomorphic;
3. mixed.

The longevity of these patients has increased due to early diagnosis of the disease and modern methods of treatment. The management tactic involves biopsy or partial removal of the tumor in combination with radiation and chemotherapy. Because of the possibility of serious complications associated with early irradiation of the orbit and eyeball, preference in choosing the method of exposure is given to prolonged chemotherapy.

Orbital Dysplasia

Fibrous dysplasia of the orbit is a rare disorder of unexplained etiology, in which the replacement of normal bone tissue by a porous fibrous tissue occurs. It appears, as a rule, in childhood. Clinical symptoms depend on which wall of the orbit was initially involved in the pathological process.

1. The upper wall of the orbit:
   • exophthalmos;
   • displacement of the eyeball and the orbit down.
2. Upper jaw:
   • displacement of the eyeball upwards;
   • persistent epiphany.
3. The sphenoid bone: atrophy of the optic nerve when the optic nerve channel is involved in the pathological process.
4. Turkish saddle: compression of the chiasma followed by atrophy of the optic nerve.

Fibrous dysplasia is easily diagnosed by X-ray examination. It looks like a thickened bone with areas of sclerosis and cysts.

The tactic of conducting is to prevent vision loss due to atrophy of the optic nerve and to resection of affected areas of the bone.