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Neurofibromatosis is a rare, dominantly inherited disease. Most cases of neurofibromatosis are benign, often affecting aesthetics, only a few large tumors compress organs and affect organ functions.1. Overview of neurofibromatosis
Neurofibromatosis is a rare, inherited disease characterized by changes in the nerves, bones, and skin. The exact cause of the disease has not yet been found.The disease has 2 out of 7 common types of neurofibromatosis, including:
Type 1: accounting for 85% of cases, patients have many neurofibromas ranging in size from a few mm to a few centimeters in diameter, with many small dots. , a small button-like lump on the skin, when lightly pressed with the hand, it feels concave but when the hand is removed, the tumor remains the same, with or without nerve damage, iris glioma. Type 2: Less commonly, this form presents with auditory or central neurofibromatosis, usually bilateral auditory neuromas. When suffering from neurofibromatosis, the patient will appear a series of tumors, these tumors are characterized by continuous growth and running along the nerve. Tumors are very common under the skin, anywhere with nerves can be seen, it can also appear in the eyelids, making the patient may not be able to see for a long time because it is covered by the tumor.
More serious are tumors appearing in the spinal cord, causing spinal cord compression, spinal deformity, affecting nerve conduction. Or the disease appears in the gastrointestinal tract, on the optic nerve affecting the ability to see. However, neurofibromatosis is mostly benign, tumors appear on the skin, affecting aesthetics, in a few cases, it is dangerous.
Bệnh u xơ thần kinh là một bệnh lý di truyền, hiếm gặp
2. Neurofibromatosis and its treatment
Currently, there is no specific treatment for neurofibromatosis, treatment for neurofibromatosis is mainly symptomatic treatment with neurofibromatosis drugs, rehabilitation or surgery.In addition, neurofibromatosis can affect learning ability: For children with low IQ, a special learning program is needed. Treatment of mental and neurological disorders in children, if any, including attention deficit hyperactivity disorder, epilepsy.
Surgery is a treatment for neurofibromatosis indicated in severe and complicated cases that must be performed many times to remove all fibroids. Surgery is indicated when:
When the tumor affects vital functions such as a large tumor, in places that compress the spinal cord, brain or other organs in the body. Tumors under the skin, in the face cause cosmetic loss, the patient needs to be removed. Tumors under the skin are too large, affecting the patient's ability to live and work. Correction of skeletal deformities or curvature of the spine Cancer.
Phẫu thuật là cách điều trị bệnh u xơ thần kinh được chỉ định trong những trường hợp nặng
The vast majority of neurofibromatosis cases are treated conservatively, and follow-up is the best method to assess disease progression. The disease has a risk of progressing to cancer, so it is necessary to examine and detect early signs of cancer. In addition, neurofibromatosis is also inherited, so when planning to have children, it is necessary to consult a specialist before birth and the newborn needs to be monitored and evaluated for neurological function after birth.
Neurofibromatosis has no specific treatment, only supportive treatment measures, surgery if it affects life function and aesthetics, causing loss of confidence. Early detection, regular progress monitoring is the best way to detect abnormalities caused by neurofibromatosis early. If you have unusual symptoms, you should be examined and consulted with a specialist.
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