Congenital tumors of the eye socket

Neoplasms of this group include dermoid and epidermoid (cholesteatoma) cysts, which make up about 9% of all orbital tumors.

Their growth is accelerated by trauma, and cases of malignancy have been described.

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Dermoid cyst

Dermoid cysts appear in more than half of patients before the age of 5, but almost 40% of patients seek help only after the age of 18. It grows extremely slowly, but during puberty and pregnancy, there are cases of its rapid enlargement. The cyst develops from wandering epithelial cells, concentrated near bone sutures, located under the periosteum. The epithelium of the inner wall of the cyst secretes mucous contents with an admixture of cholesterol crystals, which gives it a yellowish tint. Short hair may be found in the cyst. The favorite localization is the area of bone sutures, most often the upper inner quadrant of the orbit. Up to 85% of dermoid cysts are located near the bone edge of the orbit and do not cause exophthalmos, but when located in the upper outer section, they can displace the eye downwards and inwards. Complaints of patients, as a rule, are reduced to the appearance of painless edema of the upper eyelid in accordance with the location of the cyst. The skin of the eyelid in this area is slightly stretched, but its color is not changed; an elastic, painless, immobile formation is palpated.

Up to 4% of cysts are located deep in the orbit. This is the so-called cat-shaped dermoid cyst of the orbit, described by Kronlein at the end of the 19th century. The formation consists of three parts: the head of the cyst - an ampulla-shaped expansion - is located outside the tarso-orbital fascia deep in the orbit, the tail of the cyst is in the temporal fossa, and the isthmus is in the area of the frontozygomatic suture. This localization is characterized by a long-term (sometimes for 20-30 years) gradual increase in exophthalmos. The tumor, displacing the eye to the side, limits the functions of the extraocular muscles, diplopia appears. An increase in exophthalmos to 7-14 mm is accompanied by constant bursting pain in the orbit. Visual impairment can be due to a change in refraction due to both eye deformation and developing primary atrophy of the optic nerve. Cat-shaped cysts are usually diagnosed after 15 years. A long-standing cyst can spread into the cranial cavity.

The diagnosis is confirmed by X-ray of the orbit (revealing thinning and deepening of the bone edge of the orbit in the area of the cyst). The most informative is computed tomography, which visualizes not only the altered bone walls, but also the cyst itself with the capsule.

Treatment of dermoid cyst is surgical: subperiosteal orbitotomy is indicated.

The prognosis for life and vision is favorable. Relapse should be expected with significant changes in the underlying bones or the appearance of a postoperative fistula. Recurrent dermoid cysts may become malignant.

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Cholesteatoma, or epidermoid cyst

Cholesteatoma, or epidermoid cyst, develops in the fourth decade of life, almost 3 times more often in men. The etiology and pathogenesis are unclear. Cholesteatoma is characterized by localized fraying of bone tissue, which becomes soft; areas of osteolysis progress, a significant amount of yellowish soft content accumulates subperiosteally, including degeneratively altered cells of the epidermoid tissue, blood, cholesterol crystals. As a result of detachment of the periosteum with underlying necrotic masses, the volume of the orbit decreases, its contents shift forward and downward. The formation develops, as a rule, under the upper or upper-outer wall of the orbit. The disease begins with a unilateral displacement of the eye downward or downward inward. Painless exophthalmos gradually develops. Reposition is difficult. There are no changes in the fundus, the functions of the extraocular muscles are fully preserved.

The diagnosis is established based on the results of X-rays and computed tomography.

Treatment of cholesteatoma is only surgical.

The prognosis for life and vision is generally favorable, however, cases of malignant transformation of the cyst have been described.

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Pleomorphic adenoma

Pleomorphic adenoma (mixed tumor) is a benign variant of the primary tumor of the lacrimal gland. The age of patients ranges from 1 to 70 years. Women get sick almost 2 times more often. The tumor consists of two tissue components: epithelial and mesenchymal. The epithelial component forms areas of mucus- and chondroitin-like structures. The stroma (mesenchymal component) is heterogeneous within one node: sometimes it is loose, in some areas there are connective tissue strands, areas of hyalinosis are noted. The tumor develops slowly. More than 60% of patients consult a doctor 2-32 years after the onset of clinical symptoms. One of the first is painless, non-inflammatory edema of the eyelids. Gradually, the eyeball shifts downwards and inwards. Exophthalmos occurs much later and increases very slowly. During this period, it is already possible to palpate a motionless formation located under the upper outer or upper edge of the orbit. The tumor surface is smooth, painless and dense upon palpation. Reposition is difficult.

Radiography reveals an enlargement of the orbital cavity due to thinning and upward and outward displacement of its wall in the area of the lacrimal fossa. Ultrasound scanning allows one to determine the shadow of a dense tumor surrounded by a capsule and the deformation of the eye. Computer tomography visualizes the tumor more clearly, one can trace the integrity of the capsule, the spread of the tumor in the orbit, the condition of its adjacent bone walls.

Treatment of pleomorphic adenoma is only surgical.

The prognosis for life and vision is favorable in most cases, but the patient should be warned about the possibility of a relapse, which may occur in 3-45 years. Approximately 57% of patients have malignant transformation elements at the first relapse. The risk of malignant transformation of pleomorphic adenoma increases as the period of remission increases.