Lecture notes, cheat sheets
Eye diseases. Pathologies of the fundus (part II) (lecture notes) Directory / Lecture notes, cheat sheets Table of contents (expand) LECTURE No. 21. Pathologies of the fundus (part II) 1. Retinoblastoma Retinoblastoma is a malignant neoplasm that develops in the retina of the eye. It belongs to congenital tumors. It is detected in children aged two to four years. Retinoblastoma can be either unilateral or bilateral. The disease is detected more often in the first year of life. In approximately 30% of cases, the process is bilateral, often of a family-hereditary nature. Etiology, pathogenesis and pathological anatomy. The etiology has not been reliably established. A possible cause is considered to be a mutation of the chromosomes of germ cells due to internal and external factors. Retinoblastoma consists of neuroglia, that is, it is a glioma in its structure. Glia develops from the ectoderm and has great energy to reproduce and form new elements. Glial tissue permeates all layers of the retina and optic nerve. There are differentiated and undifferentiated forms of the tumor. The specific structures of differentiated retinoblastoma are true rosettes, which consist of a cylindrical form of embryonic-type neuroepithelial cells, sometimes there are abortive forms of the outer segment of photoreceptors. In undifferentiated forms, small cells with a large nucleus and a narrow rim of the cytoplasm are found, located diffusely or forming pseudorosettes around the vessels, surrounded by a zone of necrosis and calcifications. Mitoses are often noted in the tumor, dissemination to the vitreous body and the anterior chamber of the eyeball develops. Forms and stages of retinoblastoma. There are five stages of tumor development: 1) latent; 2) primary; 3) developed; 4) far gone; 5) terminal. The latent stage of retinoblastoma is characterized by such early indirect signs as unilateral pupil dilation, slowing or lack of its reaction to light. More pronounced is not a direct, but a friendly pupillary reflex. The depth of the anterior chamber may be reduced. These signs can be combined with slight changes in the fundus. The initial stage is characterized by the fact that ophthalmoscopy reveals a yellowish-white lesion with unclear contours in the fundus, often covered with retinal vessels. Small satellite lesions are observed around the lesion. Tumor growth leads to amaurosis (blindness). The tumor can spread into the vitreous body in the form of grayish-white, sometimes yellowish or greenish masses with blurred boundaries, on the surface of which hemorrhages are visible. Retinoblastoma is detected in lateral lighting and with the naked eye by the yellow glow of the pupil area. It is the last symptom that usually forces parents to show the child to an ophthalmologist. The process can spread across the retina (exophytic growth) and towards the vitreous body (endophytic growth). The appearance of retinal detachment makes diagnosis difficult. The advanced stage (glaucoma stage) is characterized by tumor growth and an increase in the volume of the contents of the eye, which often leads to an increase in intraocular pressure. In this case, pain in the eye, congestive injection, corneal edema, a decrease in depth and clouding of the moisture of the anterior chamber (pseudohypopion) are possible due to the fact that individual particles of the tumor come off and disperse inside the eye. The pupil is dilated and does not react to light. In some cases, implantation nodules resembling tuberculous ones are found in the iris. Advanced stage (germination stage). This stage of retinoblastoma is characterized by tumor growth beyond the eyeball, more often through the sclera, along the vessels, through the sheaths of the optic nerve, etc. Posterior growth of the tumor is manifested by exophthalmos with limited eye mobility, the spread of the process into the cranial cavity causes an expansion of the optic nerve canal, which is detected on radiograph. In the terminal stage (stage of metastases), enlarged cervical, submandibular lymph nodes are determined, further gross changes are observed from the side of the eye, up to the collapse of the membrane and the sharpest exophthalmos (the eye is dislocated). Tumor-like formations in the bones of the skull are revealed, and tuberosities of the liver, spleen, kidneys and other organs are enlarged. The process is accompanied by pain, the onset of cachexia, changes in the blood picture. Clinical picture. 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. Complications and diagnosis. A complication of retinoblastoma with a favorable outcome may be loss of vision in one or both eyes. A possible complication is the development of retinoblastoma into sarcoma (rhabdomyosarcoma), leukemia. The diagnostic criterion for retinoblastoma at the first stage of the disease is the detection of an amaurotic cat's eye. To objectively confirm the diagnosis, they resort to ophthalmoscopy, radiography, ultrasound examination of the eyeball and skull. Retinoblastoma should be differentiated from pseudoglioma, which occurs as a result of an inflammatory process, and it must also be distinguished from organized hemorrhage, proliferating retinitis, retinal detachment, Coates' disease, cysticercosis, tuberculosis, etc. Anamnesis (past infectious diseases) is important for making a diagnosis, the presence in some cases on the radiographs of the orbit of foci of calcification. Diaphanoscopy and echographic studies help in the diagnosis. 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 of retinoblastoma consists in the timely genetic examination of those families whose relatives have been treated for retinoblastoma in the past. 2. Retinal anomalies Patients do not complain. Ophthalmoscopy at the optic disc reveals white, shiny, with a silver tint smears, radially arranged, resembling white flames, somewhat passing to the optic disc. These are myelin fibers. When examining the functions of vision, no pathological changes were noted. There is an increase in the blind spot. Author: Shilnikov L.V. << Back: Pathologies of the fundus (part I) (General diagnostic issues. Pigmentary dystrophy (degeneration) of the retina (retinitis pigmentosa). Macular degeneration of the retina (degeneratio maculae luteae). Macular degeneration in Tay Sachs disease. Retrolental fibroplasia. External exudative hemorrhagic retinitis, or Coats disease (retinitis exsudativa haemorrhagica externa Acute obstruction of the central retinal artery (embolia s. thrombosis arteriae centralis retinae). Thrombosis of the central retinal vein (thrombosis venae centralis retinae)) >> Forward: Diagnosis, clinical picture and treatment of optic nerve pathologies (General diagnostic issues. Optic neuritis (neuritus nervi optici). Congestive papilla (disc) of the optic nerve (papillitis oedematosa s. oedema papillae n. optici). Optic nerve atrophy (atrophia nervi optici). Optic nerve glioma (glioma nervi optici) ) We recommend interesting articles Section Lecture notes, cheat sheets: ▪ Commercial activity. Lecture notes ▪ Advertising and promotional activities. Lecture notes ▪ Informatics and information technologies. Crib See other articles Section Lecture notes, cheat sheets. Read and write useful comments on this article. Latest news of science and technology, new electronics: The existence of an entropy rule for quantum entanglement has been proven
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