Learn about subglottic neurofibromatosis

Subglottic neurofibromatosis is a disease that should be considered in cases of dyspnea in children unresponsive to medical therapy. Early diagnosis and surgical treatment help to resolve the obstruction of the larynx of the child, helping the child to return to normal activities soon.

Neurofibroma is a benign tumor, formed from the fibrous sheath of the peripheral nerve. Neurofibromatosis is often divided into several groups, of which two main groups are peripheral neurofibromatosis (Neurofibromatosis 1-NF-1, also known as Von Recklinghausen's disease) and central neurofibromatosis (Neurofibromatosis 2-NF). -2). NF-1 is more common, occurring in about 1/2,500-3,3000 of the population, causing lesions present throughout the body, most commonly coffee-colored stains under the skin or tumors in the nervous system. NF-2 occurs with an incidence of 1/50,000-120,000 of the population, causing tumors in the auditory nervous system on both sides, the meninges, and the spinal cord. The disease is caused by inheritance of an autosomal dominant gene or by mutations in new chromosomes.
Neurofibromatosis arising from the larynx is very rare, especially the subglottis. To date, only about 10-15 cases of glottis glioma have been reported in the world. Subglottic neurofibromatosis can be hemangioma, granulomatous cell tumor, fibroblastoma, mixed tumor, cancer, non-Hodgkin lymphoma,... in which hemangioma is the most common form. The tumor may take the form of a solitary neurofibroma or a tangled neurofibroma. The tumor usually originates from the recurrent laryngeal nerve. Although rare, subglottic neurofibromatosis is a disease that should be considered, especially when medical treatment of dyspnea is unresponsive. .

The disease can be seen in between 2 and 60 years of age, but is mostly concentrated in children. Symptoms of subglottic neurofibromatosis are hoarseness, dyspnoea, wheezing, dysphagia, difficulty speaking... Children are often misdiagnosed as bronchial asthma and given medical treatment but do not respond. respond to treatment. The difficulty of breathing is increasing especially when lying down, the child must sit, lie with his head high or raise his neck to breathe. Children are often hospitalized with severe shortness of breath, sometimes requiring emergency tracheostomy.
To diagnose the disease, the doctor will perform laryngoscopy, computerized tomography and biopsy samples to perform pathological tests. Tests showed rhabdoid cells with wavy nuclei, collagen, and nerve fibers in them. Alternatively, a positive immunohistochemical test for S100 can be used to diagnose subglottic neurofibromatosis.

The definitive diagnosis and early surgery to remove the subglottic nerve fiber tumor is very important, because the disease occurs more often in children, and the diameter of the subglottis in children is much smaller than in humans. large, the disease can progress rapidly, causing acute difficulty breathing.
Depending on the patient's case and the physical conditions of the medical facility, the doctor will choose the appropriate surgical method, which can be open surgery or laparoscopic surgery to remove the tumor. Early surgery will help to resolve the obstruction of the larynx, reduce the rate of emergency tracheostomy, thereby reducing the sequelae of tracheostomy. After surgery, the patient can go to school, work and live normally. Patients need to have regular check-ups so that the doctor can monitor the recovery of the incision and detect the recurrence of the tumor early.

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