小儿库欣病的诊断和经蝶窦显微外科手术

Diagnosis and transsphenoidal microsurgical operation on Cushing's disease in children

目的 总结小儿库欣病的诊断和经蝶窦显微外科手术经验。方法 男7例,女8例。年龄8～14岁,平均12.7岁。平均病程2.6年。14例有典型库欣综合征,高血压7例,发育迟缓6例。内分泌检查均有皮质醇增高,地塞米松抑制试验符合小剂量不能抑制、大剂量能抑制者12例,不符合者3例。MRI的阳性发现率为69％。均行经蝶窦显微外科手术。结果 随诊6个月～10年,痊愈12例,复发3例。结论 经蝶窦显微外科手术是治疗儿童库欣病的最佳方法。对于内分泌学检查符合库欣病而蝶鞍区MRI无异常,或MRI显示垂体腺瘤而内分泌学检查不支持库欣病者都应积极地进行经蝶窦手术探查。垂体腺瘤复发者可再经蝶窦手术。

Objective To discuss the diagnosis and surgical treatment for Cushing's disease in chil- dren. Methods Fifteen children (male 7, female 8) with Cushing's diseases were reviewed retrospective- ly. The age ranged from 8 to 14 years (mean 12. 7 yrs). The average duration of disease was 2. 6 years. The clinical features included typical Cushing appearance (14), hypertension (7) and developmental retar- dation (6). The high-dose dexamethasone (HdDXM) test led urine free cortisol (UFC) to suppress in 12 patients (80%). Both HdDXM and LdDXM showed no suppression of UFC in 3 cases (20%). Eleven cases were confirmed the diagnosis of pituitary adenomas by MRI. All cases underwent transsphenoidal mi- crosurgical operation. Results The patients were followed-up from 6 months to 10 years with a curative rate of 80% (12/15). Three cases had tumor replase in 2-year (n = 2) and 5-year (n = 1) after operation respectively of which 2 patients received transsphenoidal re-operation with pituitary radiotherapy, and the other one had adrenalectomy with pituitary radiotherapy. Conclusions Trannsphenoidal microsurgery is op- timal and effective procedure for children with Cushing's disease. When children present symptom with Cushing's disease while opposite results between the endocrinological data and MRI are found, the arthors recommend to explore the pituitary gland first. Transsphenoidal re-operation is the safe method for recur- rent Cushing's disease in children.