Congenital orbital tumors

The neoplasms of this group include dermoid and epidermoid (cholesteatoma) cysts, which constitute about 9% among all orbital tumors.

Their growth is accelerated by the transferred trauma, cases of malignancy are described.

[1], [2], [3]

Dermoid cyst

A dermoid cyst in more than half of the patients appears before the age of 5, but almost 40% of patients seek help only after 18 years. It grows extremely slowly, but in the puberty and during pregnancy there are cases of its rapid increase. The cyst develops from wandering epithelial cells, concentrating near the bone joints, located under the periosteum. The epithelium of the inner wall of the cyst secretes the mucous contents with an admixture of cholesterol crystals, which gives it a yellowish hue. The cyst can have short hair. Favorite location - the area of the bone seams, often the upper 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 with an arrangement in the upper leg can shift the eye downward and inside. Complaints of patients, as a rule, boil down to the appearance of a painless edema of the upper eyelid, corresponding to the location of the cyst. The skin of the eyelid in this zone is slightly stretched, but its color is not changed, the elastic, painless, immobile formation is palpated.

Up to 4% of the cyst is located deep in the orbit. This is the so-called kormokovidnaya dermoid cyst of the orbit, described by Kronlein in the late XIX century. Education consists of three parts: the head of the cyst - ampoule-like expansion - is located outside the tarzorbital fascia in the depth of the orbit, the tail of the cyst is in the temporal fossa, and the isthmus is in the frontal-zygomatic suture. This localization is characterized by a prolonged (sometimes within 20-30 years) gradual increase in exophthalmos. The tumor, shifting the eye to the side, limits the functions of the extraocular muscles, diplopia appears. The increase in exophthalmos to 7-14 mm is accompanied by constant raspiruyuschimi pains in orbit. Visual impairment can be caused by a change in refraction due to both the deformation of the eye and the developing primary atrophy of the optic nerve. A cyst-like cyst is usually diagnosed after 15 years. A long-existing cyst can spread into the cavity of the skull.

The diagnosis is confirmed by radiography of the orbit (reveals the thinning and deepening of the bone edge of the orbit in the area of the cyst location). The most informative is computerized tomography, in which not only the altered bone walls but also the cyst with the capsule are well visualized.

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

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

[4], [5], [6], [7], [8],

Cholesteatoma, or epidermoid cyst

Cholesteatoma, or epidermoid cyst, develops in the fourth decade of life, in men almost 3 times more often. Etiology and pathogenesis are unclear. Cholesteatoma is characterized by local bone tissue disintegration, which becomes soft; osteolysis sites progressively, a significant amount of yellowish soft contents, including degenerate altered cells of the epidermoid tissue, blood, cholesterol crystals, is accumulated subperiosteally. As a result of the detachment of the periosteum with the necrotic masses subject to reduction, the volume of the orbit decreases, its content shifts anteriorly and downward. Education develops, as a rule, under the upper or uppermost orbital wall of the orbit. The disease begins with a one-sided shift of the eye downward or downward. Gradually develops painless exophthalmos. Reposition is difficult. Changes on the fundus are absent, the functions of extraocular muscles are preserved in full.

Diagnosis is established based on the results of radiography and computed tomography.

Treatment of cholesteatoma is only surgical.

The prognosis for life and vision is usually favorable, but cases of malignancy of the cyst are described.

[9], [10], [11], [12], [13]

Pleomorphic adenoma

Pleomorphic adenoma (mixed tumor) is a benign variant of the primary tumor of the lacrimal gland. Age of patients from 1 to 70 years. Women fall ill almost 2 times more often. The tumor consists of two tissue components: epithelial and mesenchymal. The epithelial component forms sections of mucus-like and chondro-like structures. The stroma (mesenchymal component) is non-uniform within one node: sometimes it is loose, in some areas there are connective tissue bands, hyalinosis areas 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 a painless, non-inflammatory eyelid edema. Gradually, the eyeball shifts downward and inside. Exophthalmos occur much later and very slowly. During this period, it is already possible to palpate a stationary formation located under the upper or upper edge of the orbit. The surface of the tumor is smooth, with palpation it is painless, dense. Reposition is difficult.

When radiography reveals an increase in the cavity of the orbit due to thinning and displacement upward and outwardly its walls in the region of the lacrimal fossa. Ultrasound scanning allows to determine the shadow of a dense tumor surrounded by a capsule, and deformation of the eye. With CT, the tumor is visualized more clearly, you can trace the integrity of the capsule, the prevalence of the tumor in the orbit, the condition of its adjacent bone walls.

The 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 relapse, which can occur after 3 to 45 years. Approximately 57% of patients with the first relapse have malignant elements. The danger of malignant degeneration of pleomorphic adenoma increases as the period of remission increases.