Retinoblastoma in children is a malignant neoplasm in the retina. The disease is often congenital in nature, however, timely detection of it is quite difficult. Basically, a retinal tumor is detected at 2 years, since it is at this age that the peak of the pathology falls.
Retinoblastoma in children is common throughout the world. Often, the disease affects both eyes at once.
Disease feature
The eye has a complex structure. The composition of a small eyeball provides protection against external negative factors, an optical system for a clear perception of objects, a structure that accepts light waves. This is a hereditary disease in children, which is formed from the cells of the retina. The tumor grows from the retinal pigment epithelium and provokes:
- vision loss;
- the spread of secondary foci to the brain and other organs;
- increased intraocular pressure.
Retinoblastoma is mainly manifested in the first 2 years of a child's life. Currently, the incidence of this disease is 1 case per 10-13 thousand newborns.
Types and forms
According to the prevalence of retinoblastoma in children, this disease is divided into species such as endophytic and exophytic. Endophytic type of pathology refers to the intraocular. With the growth of malignant cells, the eyeball is completely affected. At the same time, intraocular pressure slightly increases, the retina exfoliates and glaucoma forms.
The exophytic type of disease is characterized by the spread of malignant cells beyond the eyeball. At the same time, they cover the mucous membranes, sclera and blood vessels. Moving through the optic cells, malignant neoplasms metastasize to the nervous system and lymph nodes.
According to the clinical course of the disease, several stages are distinguished. The resting stage is characterized by the absence of pronounced clinical symptoms. Parents can only notice a small white spot that has formed. As progression loses binocular vision, the child begins to squint.
The stage of glaucoma is distinguished by more vivid symptoms. The child begins to be afraid of the light, and his eyes watery. At this stage, the iris is affected. The outflow of intraocular fluid is disturbed, the pressure rises, and very severe pains are also felt. Glaucoma begins to develop.
The stage of germination is considered the most dangerous, since the patient has a protrusion of the eyeball. Cancer cells begin to metastasize to the soft tissues that are located in the eye area. The final stage is characterized by the spread of malignant neoplasms to other organs. Cancer cells carry blood along the meninges, lymph nodes, and optic nerves. The patient experiences severe weakness, and he has other signs of pathology.
Causes of occurrence
Retinoblastoma in children can be hereditary and acquired. The hereditary form of the disease is genetic in nature. Basically, parents who have had retinoblastoma in childhood have children with the same disease. This form develops rapidly and is accompanied by other disorders and pathologies.
Retinal cancer can occur as a result of negative environmental factors and the consumption of genetically modified foods. With this form of the disease, only one eye is often affected. Such a tumor appears at an older age, but it is much easier to treat. This pathology can be one of the causes of red eyes in a child. It is on this basis that you can determine the presence of the disease.
Main symptoms
Among the main symptoms of retinoblastoma in children, it is necessary to highlight such as:
- a white spot on the pupil;
- decreased vision;
- photophobia;
- lacrimation
- change in the mucous membrane of the eye.
Pain in the area of the organs of vision can also be observed, intraocular pressure increases as a result of the outflow of fluid. This leads to the formation of secondary glaucoma. With the development of pathology in the baby, one pupil is larger than the other, which must alert parents.
The stage of germination is accompanied by a displacement of the eyeball slightly forward. This is due to the germination of the tumor in the sinuses located between the membranes of the brain. If the child has red eyes, the reasons for this may be associated with the development of retinoblastoma. In this case, you must definitely see an ophthalmologist, since in the initial stages it is much easier to get rid of an existing disease.
The stage of metastasis is characterized by the fact that metastases spread to other organs, in particular the liver, brain, bones. In this case, the patient’s well-being worsens. Symptoms of intoxication, severe weakness, and headache join in with the common signs.
Diagnostics
If the baby has one pupil more than the other, strabismus or a decrease in vision, then this may be a sign of retinoblastoma. In this case, you must definitely contact your ophthalmologist for an examination. For diagnosis, an examination of the fundus is carried out, since with ophthalmoscopy, it is quite easy to consider a neoplasm.
Children whose parents suffered the same disease must be registered in the dispensary, as there is a high risk of hereditary transmission of pathology. To clarify the diagnosis and determine the degree of the course of the disease, you need to conduct a number of diagnostic studies, in particular, such as:
- tomography of the affected area;
- brain biopsy;
- ultrasound diagnosis of the abdominal cavity to detect metastases;
- X-rays of light;
- blood and urine tests.
Due to the fact that children with retinoblasty often have pathologies and disorders in the functioning of internal organs and systems, an additional consultation of the ENT and a neurologist may be required.
Treatment features
The treatment of retinoblastoma depends primarily on the stage of the disease. Often used for therapy:
- chemotherapy;
- radiation therapy;
- surgical intervention;
- cryotherapy;
- laser coagulation;
- thermotherapy.
If you start treatment in a timely manner and select the most effective therapy, you can maintain normal vision and health of the child. With bilateral lesions, techniques are selected separately for each eye.
At the Institute of Eye Diseases, complex therapy is carried out, which implies the use of several different methods. Usually, the treatment of a tumor begins with radiation therapy, since the cancer cells of retinoblastoma are characterized by a very high degree of sensitivity to the effects of radiation. A similar technique is quite effective, however, among the side effects, the formation of cataracts and secondary tumors of bone tissue can be noted. With localized radiation therapy, the risk of side effects is significantly reduced.
At the Institute of Ophthalmology, ophthalmologists prefer conservative therapies. If the tumor has a size less than 7 mm and is located in the anterior region of the eye, cryotherapy is prescribed. If the tumor is located in the posterior regions and has a size of less than 4 mm, then photocoagulation is indicated. During thermotherapy, infrared radiation and ultrasound rays are directed to the affected area.
In the case of significant growth of tumor tissue, the attachment of secondary glaucoma, as well as the impossibility of preserving vision, an operation is performed to remove the eye, followed by the use of special artificial dentures. With the formation of metastases in the optic nerve region, chemotherapy in combination with the use of cytostatics is considered the only method of therapy.
Surgical intervention
Retinoblastoma necessarily undergoes surgery. However, maintaining normal vision of the affected eye is not always possible. In the clinic “Eye Microsurgery” named after Fedorov, treatment is carried out using modern techniques that can quickly eliminate the existing problem and improve vision.
With a small tumor size, thermotherapy is used. A similar technique involves the targeted effect of high temperatures. Cryotherapy gives a very good result when treating a small tumor that forms in the anterior portion of the retina. The procedure involves freezing the tissue of the neoplasm with a special probe, which is applied to the outer surface of the sclera.
In addition, in the presence of a small tumor, photocoagulation is prescribed. The operation is based on the destruction of blood vessels that feed the cancer cells of blood vessels. A laser beam is used for this.
The Eye Microsurgery Clinic named after Fedorov also carries out laser treatment, which helps to quickly and efficiently eliminate the existing problem in a minimally invasive way.
With a significant spread of retinoblastoma from the retina to other parts of the eye, the eyeball is completely removed, followed by implantation of an artificial lens. One of the most important aspects of the operation is the excision of the optic nerve as deep as possible.
If the tumor has gone beyond the eye, then the tumor is removed along with the bony walls of the orbit. After removal, prosthetics are performed.
Chemotherapy
In combination with the operation, chemotherapeutic treatment can be carried out, which guarantees 80-90% of the cure. Chemotherapy is required for:
- significant intraocular lesion;
- invasion of the optic nerve;
- lesions of the orbit;
- regional metastases.
Retinoblastoma is very sensitive to chemotherapy. For treatment, you can use several cytostatics at once. The best result can be obtained with a combination of drugs such as "carboplatin", "Vepesid", "Vincristine."
Chemotherapy allows to reduce the neoplasm in size, which is why a similar technique is recommended before surgery. In addition, a similar technique is intended for systemic therapy in advanced cases.
Other techniques
Treatment of a malignant neoplasm is carried out under the supervision of an ophthalmologist, oncologist and ophthalmic surgeon. The tumor is sensitive to x-rays. Treatment is carried out in several sessions, and the dosage is selected by the doctor-radiologist purely individually.
The neoplasm is irradiated remotely when the x-ray source is at some distance from the pathological focus. The contact method involves the use of applications of radioactive substances that are applied directly to the eyeball. However, in this case, the risk of complications increases, in particular, such as inflammation of the cornea, ulcer, clouding of the lens.
The introduction of antitumor drugs into the body helps prevent the spread of malignant neoplasms and reduce its size. Medications are injected directly into the tumor area or used as intravenous infusions. During the treatment, anesthesia and a special table are used to fix the patient.
Eye prostheses
If an eyeball was removed during the operation, the child is taught to wear an artificial prosthesis. His constant presence is required in order to prevent facial deformation during the growth of the child. In addition, the absence of a prosthesis can lead to eyelash wrapping inside the eye socket, which leads to mucosal irritation.
A temporary prosthesis is inserted 6 days after the operation to remove the eyeball. After the swelling of the eye sac passes, a permanent prosthesis is placed on the child. Prosthetics has 3 purposes at once, namely:
- cosmetic;
- functional;
- psychological.
Modern materials and technologies make it possible to produce prostheses that can hardly be distinguished from real eyes. Models of prostheses skillfully imitate the movements of their own eyeball.
To make the resemblance to a healthy eye even more pronounced, a lens is placed on the surface of the prosthesis that exactly repeats the shape, size and color of a healthy iris. Thanks to this, the child will not feel disabled.
Possible complications
Complications are observed if the disease becomes very aggressive, it is not diagnosed in a timely manner or treatment has not been carried out correctly. With a strong germination of retinoblastoma in the orbit, the risk of metastases increases significantly. The main complication of a malignant neoplasm is relapse after therapy.
A tumor that grows inside the eye can spread outside the organs of vision. Cancer often passes through the optic nerve into the brain. Such a complication can lead to the death of the child. In some cases, cancer passes through the walls of the eye and spreads to other internal organs throughout the body. In the presence of complications, there is a high risk of death, which is why diagnosis and treatment must be carried out in a timely manner.
Forecast and Prevention
Timely diagnosis of retinoblastoma at the initial stage makes it possible to completely cure with the help of photocoagulation, cryotherapy or radiation therapy. All these techniques are organ-preserving and less traumatic. Surgical eye removal also guarantees complete cure, however it causes discomfort and leaves a cosmetic defect. The prognosis is poor in the presence of complications such as:
- extrascleral spread of the neoplasm;
- tumor invasion deep into the optic nerve;
- involvement in the pathological process of choroidal membranes.
Prevention is very important, as this will prevent the subsequent long-term treatment and the occurrence of dangerous complications. Families with a history of hereditary retinoblastoma should consult with a geneticist. It is important to undergo examinations by an ophthalmologist, as this will determine the disease in the initial stages of development in the child and maintain vision.
Retinoblastoma is a very dangerous cancer. The sooner a diagnosis is made, the more favorable the outcome of treatment and the preservation of vision.