Pediatric retinoblastoma
Introduction
Introduction to pediatric retinoblastoma Retinoblastoma (retinoblastoma) is the most common intraocular tumor in children. It is more common in infants and young children. 2/3 is under 3 years old, and less than 5% is over 5 years old. This tumor not only harms children's vision, but also threatens children. life. basic knowledge The proportion of illness: 0.002% Susceptible people: children Mode of infection: non-infectious Complications: glaucoma, anterior chamber hemorrhage
Cause
Pediatric retinoblastoma etiology
(1) Causes of the disease
The etiology of retinoblastoma is unknown. The risk factors for individual genetic retinoblastoma are older parents, mother (Derkinderen et al., 1990) and father employed in metal factories (Bunin et al., 1990), about 1/10 Patients with lateral retinoblastoma carry a susceptibility gene for retinoblastoma, so the risk of unilateral lesions in the next generation is 1 in 20.
(two) pathogenesis
Although the tissue origin of retinoblastoma is still controversial, recent studies have suggested that the tumor originates from the neuroepithelial and can be classified as primitive neuroectodermal tumors (PNETs), which often occur in the posterior part of the retina. The tumor cells are small and round, with little cytoplasm and dense, often forming rosettes.
Reese-Ellsworth's tumor staging is as follows:
Stage I: The prognosis is very good, single or multiple, the tumor is less than 4 disk diameter (disk diameter = 1.5 ~ 1.75mm) located in or after the equator.
Stage II: good prognosis, single or multiple tumors, 4 to 10 disc diameters, located or after the equator.
Stage III: The prognosis is uncertain, the lesion is located before the equator; the single tumor is larger than 10 disc diameter, located behind the equator.
Stage IV: poor prognosis, multiple tumors, some larger than 10 disc diameters; any lesions spread to the retinal jagged line.
Stage V: The prognosis is very poor, and the huge tumor invades more than half of the retina; vitreous planting.
If a large tumor spreads to the choroid, it can cause blood to spread. For example, the tumor penetrates the sieve plate, and the central nervous system can be invaded along the optic nerve. Because these tumors are rarely metastasized, effective visual acuity can often be preserved because of the primary tumor.
Prevention
Pediatric retinoblastoma prevention
Consultation on genetic diseases should be done well. Patients with bilateral retinoblastoma are mostly hereditary. Therefore, children of both eyes and children with family history should be highly alert to the occurrence of tumors for early detection, early diagnosis and early treatment. .
Complication
Pediatric retinoblastoma complications Complications Glaucoma anterior chamber hemorrhage
Causes secondary glaucoma, anterior chamber hemorrhage, intraocular inflammation, can be combined with other malformations, long arm loss syndrome of chromosome 13, anal atresia, perineal and thumb dysplasia, mental retardation, growth retardation, cleft palate and multiple fingers ( Toe) deformity and so on.
Symptom
Symptoms of retinoblastoma in children Common symptoms Eyeball prominent eyelid drooping pseudo anterior chamber empyema alpha-fetoprotein increased neck short head lymph node enlargement visual impairment
There are familial, infants and young children, the incidence of 5 years old or older is reduced, the eye performance varies depending on the origin and development direction of the tumor, such as the tumor originating from the inner layer of the retina, growing in the direction of the vitreous, forming a mass, clinically often It is characterized by a special yellow-white light reflection (commonly known as cat's eye) and visual impairment after the pupil. Many children have a medical treatment. The tumor originates from the outer nuclear layer and grows into the subretinal space. It shows progressive retinal detachment in the fundus. The white-gray discolored area can be seen, because the bulge is not significant, so it is easy to be missed. The diffuse invasive tumor from the periphery of the retina can affect the iris, ciliary body, and produce symptoms similar to iridocyclitis; Retinal plane expansion, accompanied by retinal vasodilation, can be mistaken for retinal angiomatosis. If left untreated, the tumor continues to grow, the retinal tissue is completely destroyed, and the tumor cells cause secondary glaucoma due to obstruction of the anterior chamber, generally from the beginning of the tumor. From the development of the intraocular pressure to about 1 year; glaucoma period lasts about half a year, and small tumors in the posterior pole of the eye can cause strabismus; iris surface blood vessels The rupture can cause blood in the anterior chamber. Tumors, such as the optic nerve or optic nerve sheath, can spread to the brain, such as through the scleral aqueduct to the ankle.
Full of eyelids, eyeballs protruding, or formation of ulcerated giant pieces protruding in the eyelids, a small number of cases can cause intraocular inflammation due to tumor necrosis, but the performance of endophthalmitis or total ocular inflammation, advanced cases of blood transfer, as much as the liver Or bone, 5% of children with other malformations, such as long-arm loss syndrome with chromosome 13 reported, manifested as microcephaly, wide and significant nasal forehead bone, small eyes, wide eye distance, internal epithelium, The eyelids sag, the upper incisors protrude, the small jaw, the neck short and wrinkled on both sides, deformed and rotating ears, anal atresia or perineal palsy and thumb dysplasia or absence, some malformations also complicated by retinoblastoma including mental retardation, Growth retardation, cleft palate and multi-finger (toe) malformations have two important implications for these other malformations:
1 If the child has retinoblastoma and microcephaly, it is necessary to do chromosome analysis and test to exclude intrauterine infection after development.
2 pediatricians diagnosed with the above-mentioned malformations and mental retardation in children should be identified with or without retinoblastoma.
Examine
Examination of pediatric retinoblastoma
Children with yellow-white light reflection after pupil, vision loss accompanied by strabismus, unexplained anterior chamber blood, single-eye pupil dilation or glaucoma, family history of retinoblastoma, should be in-depth examination of bilateral fundus and vitreous, check should be Under general anesthesia, dilated pupils, indirect ophthalmoscope to find the extent of intraocular lesions, scleral depression and retina before the latitude, for advanced intraocular tumors, especially recurrence or distant metastasis should be done The following checks:
1. Cerebrospinal fluid examination to check for the presence or absence of malignant tumor cells.
2. Blood test serum alpha-fetoprotein increased, serum alpha-fetoprotein can be decreased after surgical removal of tumor, tumor recurrence is increased again.
3. Bone marrow examination Note that there are no tumor cells.
4. Pathological examination of lymph nodes can be done lymph node biopsy.
1. Fundus examination of the bilateral dilated pupils to carefully examine the fundus, positive findings of both eyes help the diagnosis and treatment of the program and prognosis.
2. Cranial radiographs can be seen in the cerebral X-ray examination. For example, the optic nerve hole is enlarged, and intracranial spread may be considered.
3. Ultrasound examination Ultrasound and CT examination can show tumor lesions in the eye and in the eyelids.
A super-peak peaks of pathological echoes appear in the flat section of the vitreous, usually connected with the wall of the eye. The pathological peaks of severe cases can occupy all the vitreous chambers, and the sound attenuation of different parts is inconsistent.
B-ultrasound shows that the boundary of the eyeball is clear to the vitreous cavity, which is spherical or irregularly shaped. The internal reflection echoes the intensity of the light spots, the distribution is uneven, the lesions are necrotic, and cystic darkness may occur. Area image.
4. CT scan examination CT examination can show the tumor lesions in the eye and in the eyelids. It can be seen that there are localized masses with uneven density increase in the vitreous cavity, often with calcification plaques. In the advanced cases, the optic nerve is thickened and the optic nerve pores are enlarged.
5. Fundus fluorescein angiography is a valuable auxiliary diagnostic method for choroidal malignant melanoma.
Diagnosis
Diagnosis and diagnosis of pediatric retinoblastoma
Early should be associated with congenital cataract, embryonic vascular residual proliferative choroidal metastasis, choroidal hemangioma, choroidal hemorrhage, secondary glaucoma or pseudo anterior chamber empyema, should be associated with choroidal junction, exudation Endophthalmitis, total ocular inflammation and other phase differentiation, this disease and retinal hamartoma differentiation, is a benign tumor of the retina, relatively rare, combined with other malformations should be noted that the identification is caused by intrauterine infection or caused by chromosomal abnormalities.
