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Eye diseases. Clinical picture and treatment of retinoblastoma (the most important)

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33. Clinical picture and treatment of retinoblastoma

Retinoblastoma can be unilateral or bilateral. Bilateral tumors are often combined with microcephaly, cleft palate and other embryonic developmental defects. The onset of the disease usually goes unnoticed. The first clinical symptom may be decreased visual acuity. Sometimes an early clinical sign of a unilateral lesion may be strabismus. Then there will be a change in eye color (yellowish reflex), observed in the depths of the eyeball and visible through the pupil (the so-called amaurotic cat's eye). This phenomenon occurs due to the presence of gliomatous nodes on the retina and forces the child’s parents to consult a doctor. The tumor is characterized by slow growth.

In the clinic of retinoblastoma, with an objective examination of the fundus, four stages are distinguished.

At the first stage, retinal whitish-pink lesions with smooth or uneven relief, newly formed vessels and gray areas of calcifications are visible. Their dimensions do not exceed one quadrant of the fundus, the surrounding tissues are not changed.

In the second stage, intraocular dissemination occurs. Whitish inclusions of various sizes are already observed in the vitreous body, precipitate-like deposits are noted in the angle of the anterior chamber of the eyeball and on the posterior surface of the cornea. Secondary glaucoma, buphthalmos (hydrophthalmos) appear. When a tumor grows into the retina, the accumulation of exudate under it leads to retinal detachment. Vision is reduced to complete blindness.

The third stage is characterized by extraocular spread of the tumor. Accompanied by exophthalmos, tumor growth into the cranial cavity, most often along the optic nerve, gives symptoms of brain damage (headache, nausea, vomiting). Germination of retinoblastoma in the choroid leads to hematogenous dissemination and an unfavorable outcome. As a result of tumor necrosis, toxic uveitis may develop.

In the fourth stage, symptoms due to metastasis join the eye symptoms. Metastasis occurs to lymph nodes, bones, brain, liver and other organs.

Treatment and prevention. At the first and second stages of the disease, organ-preserving cryoapplication is possible. At later stages, treatment consists in the timely surgical removal of the tumor (enucleation of the affected eye in a unilateral process or both eyes in a bilateral process). Enucleation of the eyeball is performed with resection of the optic nerve at a distance of 1015 cm from the posterior pole of the eyeball. In the future, radiation therapy is carried out in combination with chemotherapy or alone. Photocoagulation may also be performed. Cyclophosphamide, prospedin, methotrexatebeve are used as antitumor drugs.

Prevention. Timely genetic examination of those families whose relatives were treated for retinoblastoma in the past.

Author: Shilnikov L.V.

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