摘要目的分析和探讨Nelson综合征的病因、诊断标准和治疗方法.方法 对我科手术治疗的23例病例进行回顾性研究.21例经蝶窦手术,2例经额开颅,随访时间半年～9年.结果 本组23例Nelson综合征,占同期手术治疗的库欣病的7.7%.垂体腺瘤切除后皮肤色素沉着减轻,血ACTH水平下降,8例视力视野障碍术后均改善,治愈率56.5%,缓解率26.1%.结论 经蝶窦垂体ACTH腺瘤切除是预防和治疗Nelson综合征的首选方法,术后应定期随访.

abstractsObjective To discuss the etiology, diagnostic criteria and treatment of Nelson's syndrome.Methods 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.Results 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. Conclusions 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.