国家神经系统疾病临床医学研究中心数据管理与共享系统

Data Management And Sharing System of China National Clinical Research Center for Neurological Diseases

《国家中心数据管理与共享系统操作说明书 —— 研究者部分》

Mechanism and surgical management of transsellar transsphenoidal encephalocele

第一作者：Zhijun Yang, Zhenmin Wang

通讯作者：Pinan Liu

Encephalocele Endoscopy Managemen Mechanism Surger

基本信息

2015-12-01

已发表文章

发表杂志：Journal of Clinical Neuroscience

发表类型: Original Article

影响因子(IF): 1.961

PMID: 26256066 Pubmed原文链接 全文下载

主要研究背景及目的

方法

We retrospectively reviewed six patients who were treated in our hospital from October 2003 to September 2013; five male patients and one female, with an average age of 10 years (range: 2 - 28). We collected data on their general condition, medical history, clinical features, and outcomes. An endoscopic transsphenoidal approach was used for five patients, and one refused surgery. All patients had similar imaging findings, and their pituitary gland could not be seen on MRI.

结论

This disease may result from pituitary dysplasia, and the symptoms develop as the patients grow. Surgical interventions can be helpful for symptom management, the optimal treatment being a transsphenoidal approach. Those patients with milder symptoms preoperatively have a better prognosis.

主要结果

Five of the patients showed gradual disease progression. The clinical symptoms were endocrine disturbance (n=6), decreased visual acuity (n=5), dyspnea (n=3) and cerebrospinal fluid rhinorrhea (n=2). Three of the patients also had a cleft palate. Two patients suffered serious symptoms of fever, tachyarrhythmia, and electrolyte disturbance postoperatively. After a long follow-up period, the symptoms remained stable or improved in all patients postoperatively, but worsened in the patient who did not have a surgical intervention. No mortalities were recorded.