Xing Bing, Ren Zuyuan, Su Changbao, Wang Renzhi, Yang Yi, Hu Yaofei
Department of Neurosurgery, Peking Union Medical College Hospital, Beijing 100730, China.
Chin Med J (Engl). 2002 Aug;115(8):1150-2.
To discuss the etiology, diagnostic criteria and treatment of Nelson's syndrome.
Twenty-three patients with Nelson's syndrome who were treated in our department over the last 19 years were analyzed retrospectively. Removal of adenoma by the transsphenoidal approach was done in 21 patients and by transfrontal craniotomy in 2. The follow-up period ranged from six months to nine years.
The incidence of Nelson's syndrome was 7.7% in a series of 300 patients with Cushing's disease treated by microsurgery in the same period. Hyperpigmentation was relieved and adrenocorticotropic hormone (ACTH) levels decreased in all patients after tumor excision. Eight patients with visual disturbance improved after surgery. The curative and remission rates were 56.5% and 26.1%, respectively.
Transsphenoidal microsurgical removal of pituitary ACTH adenoma is the first choice in the prevention and treatment of Nelson's syndrome. Regular follow-up examinations should be performed over a long time.
探讨尼尔森综合征的病因、诊断标准及治疗方法。
回顾性分析过去19年在我科治疗的23例尼尔森综合征患者。21例行经蝶窦入路腺瘤切除术,2例行经额开颅手术。随访时间为6个月至9年。
同期300例经显微手术治疗的库欣病患者中,尼尔森综合征的发生率为7.7%。所有患者肿瘤切除后色素沉着减轻,促肾上腺皮质激素(ACTH)水平下降。8例视力障碍患者术后改善。治愈率和缓解率分别为56.5%和26.1%。
经蝶窦显微手术切除垂体ACTH腺瘤是预防和治疗尼尔森综合征的首选方法。应长期进行定期随访检查。