Retinoblastoma in children
Oncology / / May 17, 2016
Retinoblastoma - a malignant tumor of the retina.It occurs usually in young children.The first description of the disease in the 3-year-old child belongs to the XVI century.However, some authors believe that this clinical picture could be observed in Kaposi's orbit and that the first description of retinoblastoma appeared in the XVIII century: in 15-month-old baby's parents noticed an unusual glow of both pupils.In spite of the therapy, the tumor continued to grow and the child died at the age of 3 years from the metastatic lesion of the brain.
Retinoblastoma registered worldwide.This is a relatively rare disease.However, suffering from eye diseases in children, it occurs in 1.9-2.6% of cases.Of all malignant tumors Retinoblastoma accounts for 16.1%.
very difficult to determine the onset of tumors.We observed cases of retinoblastoma immediately after birth or at the 1st month of life.Described natal development of retinoblastoma.All this gives grounds to consider it innate tumor that manifests itself
Currently, there is no doubt the possibility of transmission of retinoblastoma is inherited.The first descriptions of familial cases of the disease are the beginning of XVIII century.In families where there are 2 or more of patients with retinoblastoma, we can expect that on average 50% of the children in this family will be amazed by the tumor.Most cases of retinoblastoma in healthy relate only to the child.
success of the treatment of retinoblastoma, and therefore, the prognosis for life is largely dependent on the early detection of tumors and treated early.Sometimes parents notice the appearance of the child has strabismus, the unusual expansion of the pupil, the pupil glow white or light yellow.Such complaints should alert any health professional and provide a strong basis for expeditious referral of the child to the eye doctor.These signs of retinoblastoma occur relatively early in the localization, even small tumor nodes in the posterior segment of the eye.
emergence of the child's signs of inflammation or secondary glaucoma (photophobia, lacrimation, redness of the mucous membrane, cataract), and restless behavior indicate an advanced stage of the disease.Unfortunately, due to the low age of the patients nor the doctors nor the parents are unable to timely detect such important symptoms such as reduced visual acuity and visual field change.Therefore, we understand the importance of early preventive examinations of newborns and children 1 year of life with a careful study of the fundus under the enlarged pupil.
4 stage retinoblastoma
The clinical course distinguish 4 stages of retinoblastoma.
I stage - initial characterized intraocular localization process.The size and shape of the eye is not changed, the intraocular pressure is normal.The first clinical signs of the tumor - the appearance of the retina light foci with blurred contours, which can be detected only by careful systematic examination of the fundus.The growing tumor is sealed and transformed into the vitreous.Perhaps the development of retinal detachment.Sometimes there are multiple components of various shapes and sizes.Depending on their localization reduced visual acuity, develops.The decay of the tumor tissue in the vitreous screenings tumors arise.
II stage - complicated, characterized in that the growing tumor, filling the cavity of the eyeball, causing the phenomenon of secondary glaucoma: increased intraocular pressure, congestive injection, swelling of the epithelium of the cornea, pupil dilation, pain.The decay of the tumor tissue can be toxic iridocyclitis, accompanied by photophobia, lacrimation, a change in color of the iris, a decrease in intraocular pressure.
III stage - manifested output tumor outside of the eyeball through the membranes and the optic nerve.In the initial period, it can be set only by histological examination.Later in the orbit can be formed relatively large tumor nodules.In the propagation of tumors forward appear foci of necrosis of the cornea or sclera.
IV stage - metastasis, characterized generalization of the process, the proliferation of the tumor through the lymph and blood vessels in the brain and spinal cord lesions of the nasal sinuses and skull base.The most frequent metastasis to the parotid gland and neck, flat skull, long bones of the lower limbs, liver, lungs.There is also damage to the central nervous system.
Before treatment conducted research body of the child and clinical examination.Small age of the patient, it is often restless behavior during the inspection require special endurance and skill of the physician and nursing staff.Approximately (depending on age) determine visual acuity, condition and response of the pupils.In order to obtain more reliable information about the child's state of much of the research conducted during the depth of the physiological or narcotic sleep.Performs fundus examination with dilated maximum.In identifying the fundus hearth tumors determined its location, size, presence of hemorrhage.Measured intraocular pressure.
various clinical and laboratory research methods are widely used for the diagnosis of retinoblastoma.One of the most important is the method of ultrasonography, which allows to differentiate retinoblastoma from a number of other intraocular pathological processes.X-ray study of the orbits of the skull and detect calcifications in the eyeball, specific to this tumor, and at stages III and IV - darkening and enlargement of the orbit, the destruction of its walls, expansion and destruction of the optic nerve canal, tumor metastasis in the bones of the skull.In some cases, strictly on the evidence held radioisotope research using radioactive phosphorus.
child with retinoblastoma should be examined by a pediatrician, dentist, neurologist, otolaryngologist, he performed blood tests, urine tests, X-rays of the chest.A compulsory examination of the mother and child to chronic infections.To exclude metastatic lesions of other organs and systems of a child is sent to the oncologist.
Treatment of retinoblastoma is a combination comprising a surgical stage, radiation, chemotherapy and phototherapy.Crucial to the choice of methods and consistency of treatment of patients is histological examination of the remote eye, as well as the fact of unilateral or bilateral process.
When a unilateral lesion produce the removal of the patient's eyes, when bilateral - the worst eye.During the operation, you need to cross the optic nerve as far as possible from its exit from the eyeball, 8-10 mm from the sclera to provide greater probability of clipping optic nerve within the healthy tissue.Spiking the cavity of organic or synthetic materials to form mobile stump is contraindicated, as it would greatly complicate further control cavity condition.
day after surgery produce a bandage.The duties of a procedural nurse includes 3 meals cavity lavage solutions of antibiotics, sulfonamides.At 5-6 days after enucleation of the prosthetic cavity, and then processing proceeds cavity, and once a day procedural nurse removes the prosthesis in order to more rigorous sanitation of the conjunctival cavity and the prosthesis.
In combination with surgery and chemotherapy radiation therapy.Radiotherapy should start 2-3 days after surgery, with mandatory preliminary study of peripheral blood.To ensure correct and complete exposure a child during the procedure should be able to sleep.If necessary, a normal physiological sleep deepened by a sedative or narcotic drugs.
In order to prevent the development of radiation keratitis and cataract conjunctival cavity irradiated eyes before each session of radiotherapy nurse digs in 2% solution of freshly prepared cysteine or 1% solution taufona 6 times at intervals of 15 minutes.
for the prevention and treatment of radiation burns the skin of eyelids and temporal region regularly 1-2 times a day after irradiation lubricated, sea buckthorn, Dog rose, olive, corn or sunflower oil and other ointments that improve the nutrition of the skin, promotes regeneration and relieves inflammatory response.
After treatment, children with retinoblastoma should be under constant medical observation ophthalmologist.Inspections are recommended 1 time in 3 months.during the first 2 years, 1 every 6 months.over the next 3 years, after 1 time per year.On examination, we investigate visual acuity and fundus necessarily with a wide pupil, state of the conjunctival cavity ud lennogo eyes.
under medical supervision should be young children born in families where there are patients with retinoblastoma.Inspection of these children during the first 3 years of life should be at least 1 time in 3 months, and in the next 2-3 years -. 1 every 6 months.
It must be remembered that due to the existence of a hereditary form of retinoblastoma patient risk birth increases if its one parent.