摘要
目的 讨论 2 0岁以下儿童和青春期库欣病的诊断和治疗。方法 男性 11例 ,女性 2 3例 ,平均年龄 15 2岁 ,平均病程 2 1年。表现库欣综合征占 91 2 %。内分泌学检查符合库欣病占70 6 % ,CT阳性率 5 7 1% ,MRI阳性率 70 %。均行经蝶窦手术 ,术后病理证实垂体ACTH腺瘤 2 8例 ,垂体增生 4例 ,病理阴性 2例。结果 随诊 3个月至 10年 ,垂体ACTH腺瘤的治愈缓解率 89 3%。垂体增生和病理阴性的治疗有效率为 5 0 %。结论 经蝶窦显微外科手术是治疗库欣病的最佳方法。当内分泌学检查符合库欣病但蝶鞍MRI未发现垂体腺瘤时 ,或者当蝶鞍MRI发现垂体腺瘤但内分泌学检查不符合库欣病时 ,应行经蝶窦垂体探查术。
Objective To discuss the diagnosis and surgical treatment of Cushing's disease in children and adolescents(under 20 years of age).Methods 11 patients were males and 23 were females.The mean ages were 15 2 yrs.Mean history was 2 1 years.The presenting signs and symptoms were concentric hypertrophy in 91 2% cases.The high dose dexamethasone(HdDXM)test showed unequivocal suppression and low dose DXM test no suppression of urine free cortisol in 70 6%.Pituitary adenomas were diagnosed by CT in 57 1%, by MRI in 70%.Transsphenoidal surgery were performed on all the patients.Pituitary microadenomas were diagnosed by pathology in 28 cases, hyperplasia in 4 cases and negative in 2 cases.Results All the patients were followed up from 3 months to 10 years.The cure rate of pituitary ACTH adenomas was 89 3%.Conclusions The transsphenoidal microsurgery is optimal treatment in children and adolescents with Cushing's disease.If endocrinological data is unequivocal for Cushing's disease but MRI scans show no significant evidance of pituitary adenoma, or if endocrinological data do not supported the diagnosis of Cushing's disease but MRI scans show pituitary adnoma in Cushing syndrome, we recommend to explore the pituitary gland by transsphenoidal surgery.
出处
《中华神经外科杂志》
CSCD
北大核心
2002年第6期351-353,共3页
Chinese Journal of Neurosurgery
关键词
库欣病
诊断
经蝶窦手术
儿童
青春期
临床表现
Cushing's disease Diagnosis Transsphenoidal microsurgery Children and adolescents
