A year ago, a 27-year-old Zhang Xiaoshuai (pseudonym) from Huaihua, Hunan suddenly suffered from bilateral tinnitus, hearing loss, and unstable walking. He searched for doctors to no avail. On April 8 this year, Zhang Xiaoshuai was in the brain department of Hunan Province. The second neurosurgery ward of the hospital (Hunan Second People's Hospital) was diagnosed with neurofibromatosis type 2, a rare disease with a global incidence of one in 40,000. On August 29, Zhang Xiaoshuai came to the hospital again to prepare for the second operation.
A few months ago, as the size of the tumor gradually increased, Zhang Xiaoshuai showed obvious head pain, listlessness, and weight loss. After admission, the doctor formulated a detailed surgical plan for Zhang Xiaoshuai.
"The formulation of a treatment plan for NF2 acoustic neuroma is complicated. The timing of surgery, the size and severity of the tumor, and the patient's hearing status before surgery, etc. will affect the formulation of the treatment plan." Li Xin, Second Department of Neurosurgery Director introduced.
On April 12, the right acoustic neuroma was resected under general anesthetic nerve electrophysiological monitoring. The operation was very smooth. After the operation, Zhang Xiaoshuai's right hearing gradually improved. On August 29, Zhang Xiaoshuai came to the hospital again to remove the tumor on the left side.
Both operations are extremely challenging for neurosurgeons. The bilateral acoustic neuroma will cause complete damage to the bilateral auditory nerves. Leaving severe hearing impairment, and possibly even deafness, will deal a huge blow to Zhang Xiaoshuai's life. Therefore, in view of the characteristics of Zhang Xiaoshuai's tumor, Li Xin, director of the second ward of neurosurgery, organized a multidisciplinary joint diagnosis and treatment, and formulated a prudent and individualized surgical plan. After a few hours of operation, the effort paid off and the operation was a complete success.
Li Xin introduced that in 1988, the National Institutes of Health classified neurofibromatosis into type 1 (NF1) and type 2 (NF2). The main damage of NF1 is the skin, which has a greater impact on appearance. NF2 bilateral acoustic neuroma is a autosomal dominant genetic disease , which is caused by the loss of autosomal 22q12.2. About 50% of patients with NF2 have a family history. According to foreign reports, the incidence of NF2 is 1/33 000～1 /40 000,Its clinical manifestations are diverse, mainly with bilateral acoustic neuromas as its typical type
"NF2 patients mostly develop onset in childhood or adolescence, and the disease progresses rapidly, initially manifesting as hearing impairment on one side, and eventually hearing loss on both sides. If it is not treated, as the tumor grows, it will directly cause brain herniation and cause death. But because of its low incidence, the clinical diagnosis rate is not high." Director Li Xin of the second ward of neurosurgery said, "Therefore, the improvement of type 2 The understanding of neurofibromatosis, selection of reasonable and effective examination methods, early detection and selection of reasonable treatment options are very important to the prognosis and life of the patient. Once hearing loss occurs, it is necessary to seek medical treatment in time and choose a reasonable treatment plan to avoid delays. The best time for surgery."
Correspondent Jiang Pengxiaoxiang Morning News reporter Xia Sheng
.
