Retinoblastoma is a malignant type of eye cancer that predominantly affects children, and is most common in infants and young children. This tumor originates in the retina, the light-sensitive layer located at the back of the eye. Although it is a serious condition, advances in medicine have allowed many cases to be treated successfully, saving both the lives and vision of affected children.
In this post, we will explore in detail how retinoblastoma can be treated in babies, covering the different treatment options, the importance of early diagnosis and the crucial role of medical follow-up.
What is Retinoblastoma?
Retinoblastoma is a malignant tumor that develops from immature retinal cells. It can affect one or both eyes and, in some cases, may be associated with an inherited genetic mutation. It is estimated that around 40% of retinoblastoma cases are hereditary, with the mutation being passed from a parent to the baby. In the remaining 60% of cases, the mutation occurs sporadically, with no family history.
Symptoms of retinoblastoma can be subtle at first and often go unnoticed by parents. Some of the signs include a white reflex in the pupil (leukocoria), which can be seen in flash photographs, strabismus (misaligned eyes), redness and eye pain. Given the severity of the condition, it is essential that any suspicion be investigated immediately by a health professional.
The Importance of Early Diagnosis
Early diagnosis is the key to successful treatment of retinoblastoma. The earlier the tumor is identified, the greater the likelihood of successful treatment, preserving not only the life of the child, but also their vision.
The Universal Newborn Eye Screening, performed in the first days of a baby's life, is one of the most effective tools for early detection of retinoblastoma. This test allows a detailed view of the retina and can identify abnormalities that indicate the presence of a tumor even before symptoms become apparent.
Once the tumor has been identified, the pediatric ophthalmologist, along with a multidisciplinary team that may include oncologists and radiologists, will determine the stage of the retinoblastoma and plan the most appropriate treatment. The tumor’s stage, location, size, and whether it has affected one or both eyes are crucial factors in determining the course of treatment.
Treatment Options for Retinoblastoma in Infants
Treatment of retinoblastoma in infants is highly individualized, taking into account the severity of the disease and the specific characteristics of the tumor. The main treatment options available are described below:
1. Chemotherapy
Chemotherapy is one of the most common forms of treatment for retinoblastoma, especially in cases where the tumor is large or there are multiple tumors in the eyes. It can be administered in several ways:
Systemic Chemotherapy: Given intravenously, systemic chemotherapy involves the use of drugs that circulate throughout the body to target and destroy cancer cells. This treatment is used when there is a need to reduce the size of the tumor before other treatments, such as cryotherapy or brachytherapy, are performed. In more advanced cases, systemic chemotherapy can also be used to treat tumors that have spread beyond the eye.
Intra-Arterial Chemotherapy: In this newer method, chemotherapy is administered directly into the artery that feeds the affected eye. This allows for a higher concentration of drugs at the tumor site, reducing systemic side effects. Intra-arterial chemotherapy is generally used in cases where the tumor is resistant to other treatments or when the goal is to preserve as much vision as possible.
Intravitreal Chemotherapy: This method involves injecting drugs directly into the vitreous humor of the eye, a fluid-filled area located between the lens and the retina. It is mainly used to treat tumors that have spread within the eye, avoiding the need for more invasive treatments.
Chemotherapy, regardless of the method, is usually given in cycles, allowing for recovery periods between sessions. Side effects can include nausea, vomiting, hair loss and increased susceptibility to infections, but these are managed by the medical team.
2. Cryotherapy
Cryotherapy is a technique that uses extremely low temperatures to freeze and destroy tumor cells. This treatment is particularly effective for small tumors located on the periphery of the retina. During the procedure, a cryogenic probe is applied directly to the surface of the eye, freezing the tumor over several sessions.
Cryotherapy is an outpatient procedure, which means that the baby does not need to be hospitalized. It is common for and several sessions are required to completely destroy the tumor. Although it is an effective treatment for small tumors, cryotherapy is usually used in combination with other treatments, such as chemotherapy, to ensure that the cancer cells are completely eliminated.
3. Brachytherapy
Brachytherapy, or plaque radiation therapy, is a form of internal radiation therapy used to treat retinoblastomas that are large or located in areas that are difficult to reach with other treatments. In this procedure, a small radioactive plaque is temporarily implanted on the outside of the eye, close to the tumor. The radiation emitted by the plaque destroys the cancer cells over several days.
Brachytherapy is effective in treating tumors without significantly damaging the healthy structures around them. After the treatment period, the plaque is removed. Like any form of radiation therapy, brachytherapy can have side effects, such as inflammation or damage to the retina, but it is a valuable option for preserving vision in complex cases.
4. Thermotherapy
Thermotherapy uses heat to destroy tumor cells. Heat is applied to the tumor using an infrared laser or microwave, and is effective for small to medium-sized tumors. Heat therapy is often used in combination with chemotherapy to increase its effectiveness.
This treatment is less invasive than brachytherapy or surgery and has the advantage of causing less damage to healthy eye tissue. However, heat therapy may be limited in its ability to treat larger tumors or those located in critical areas of the retina.
5. Surgery (Enucleation)
In cases where the tumor is very large or does not respond to other treatments, enucleation, or removal of the eye, may be necessary. Although it is a difficult decision for parents, enucleation is often the best option to save the child's life in cases of advanced retinoblastoma.
During surgery, the affected eye is removed, and a prosthetic eye is implanted for cosmetic purposes. Although the loss of an eye is a significant impact, most children adapt well to wearing a prosthetic eye and can lead full and active lives.
Enucleation may also be followed by additional treatments, such as chemotherapy, to ensure that any remaining cancer cells are destroyed, preventing the cancer from recurring.
Follow-up and Rehabilitation
After initial treatment, regular follow-up care is crucial to ensure that the retinoblastoma does not return and to monitor the child’s overall health. The pediatric ophthalmologist will perform regular eye exams, using imaging techniques to check for any new tumor growth. The frequency of these visits may decrease over time if the child remains cancer-free.
In addition, if the child has lost partial or complete vision in one or both eyes, it is important to begin vision rehabilitation programs. These programs help the child develop adaptive skills and maximize the use of his or her remaining vision. In cases of complete loss of vision in one eye, the child will learn to rely on the healthy eye and develop strategies for safety and mobility.
Emotional support for the child and family is also an essential part of the recovery process. Support groups, psychological counseling, and expert guidance can help parents cope with the stress and anxiety related to the diagnosis and treatment of retinoblastoma.
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