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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 treatment of Cushing’s disease in children under 20 and adolescence. Methods: There were 11 males and 23 females, with an average age of 15 2 years and a mean duration of 21 years. The performance of Cushing’s syndrome accounted for 91 2%. Endocrinological examination was consistent with Cushing’s disease accounted for 70 6%, CT positive rate 5 7 1%, MRI positive rate of 70%. All patients underwent transsphenoidal surgery. Postoperative pathology confirmed 28 cases of pituitary ACTH adenoma, 4 cases of pituitary hyperplasia and 2 cases of pathological negative. Results Follow-up 3 months to 10 years, the cure rate of pituitary ACTH adenoma 89 3%. The effective rate of pituitary hyperplasia and pathology negative treatment is 50%. Conclusion Transanal microsurgery is the best way to treat Cushing’s disease. When the endocrinology meets the Cushing’s disease but the pituitary adenoma is not found in the MRI of the sella, or when the pituitary adenoma is found in the sella MRI, the endocrine examination does not comply with Cushing’s disease and the pituitary exploration should be performed.