CAJ | 학술논문

目的探讨库欣病经蝶术后无效的诊断和治疗.方法男2例,女10例,平均年龄36(21 ～46)岁.据典型库欣综合征表现、内分泌学和影像学检查诊断为库欣病而行经蝶手术.所有患者术后症状和体征均未缓解,术后3个月复查血、尿皮质醇仍高于正常.5例行肾上腺次全切除辅以垂体放疗,4例行单纯肾上腺次全切除,2例行单纯垂体放疗,1例行双侧肾上腺全切并自体肾上腺种植.结果肾上腺次全切除辅以垂体放疗者随访20年未出现复发和Nelson综合征;单纯肾上腺次全切除者术后2～9年症状复发;单纯垂体放疗者放疗1～2年后症状缓解,随访5年未出现复发;双侧肾上腺全切并自体肾上腺种植者,随访2年种植肾上腺未存活,出现Nelson综合征.结论经蝶术后无效库欣病的治疗应在正确诊断的前提下,结合术中垂体探查情况、术后病理结果及影像学资料,并充分考虑各种治疗方案对患者的利弊,制定个体化的治疗方案.
목적 탐토고흔병경접술후무효적진단화치료.방법 남2례,녀10례,평균년령36(21 ～46)세.거전형고흔종합정표현、내분비학화영상학검사진단위고흔병이행경접수술.소유환자술후증상화체정균미완해,술후3개월복사혈、뇨피질순잉고우정상.5례행신상선차전절제보이수체방료,4례행단순신상선차전절제,2례행단순수체방료,1례행쌍측신상선전절병자체신상선충식.결과 신상선차전절제보이수체방료자수방20년미출현복발화Nelson종합정;단순신상선차전절제자술후2～9년증상복발;단순수체방료자방료1～2년후증상완해,수방5년미출현복발;쌍측신상선전절병자체신상선충식자,수방2년충식신상선미존활,출현Nelson종합정.결론 경접술후무효고흔병적치료응재정학진단적전제하,결합술중수체탐사정황、술후병리결과급영상학자료,병충분고필각충치료방안대환자적리폐,제정개체화적치료방안.
Objective To discuss the diagnosis and treatment of Cushing's disease without remission after transsphenoidal surgery.Methods 12 patients of Cushing's disease without remission after transsphenoidal surgery were investigated in this retrospective study.There were 2 males and 10 females with an average age of 36 (21-46) years old.Cushing's disease was diagnosed with the typical symptoms and signs of Cushing's syndrome,endocrinological examination and CT or MRI.Transsphenoidal surgery was performed in all patients.None of the patients achieved remission after operation.Serum and urinary free cortisol (UFC)were still elevated 3 months after operation.Subtotal adrenalectomy combined with pituitary radiotherapy were performed in 5 patients,single subtotal adrenalectomy in 4,pituitary radiotherapy in 2 and bilateral adrenalectomy with adrenal autotransplantation in 1 patient.Results Those receiving subtotal adrenalectomy combined with pituitary radiotherapy had no recurrence or Nelson's syndrome during 20 years of follow-up,but 4 patients receiving single subtotal adrenalectomy recurred after 2 to 9 years after operation.2 patients achieved remission with pituitary radiotherapy 1 to 2 years later without recurrence during 5 years of follow-up.One patient who underwent bilateral adrenalectomy with adrenal autotransplantation needed steroid replacement and Nelson's syndrome occurred during 2 years of follow-up because transplanted adrenal gland had no function.Conclusion The diagnosis of Cushing's disease should be reconfirmed for patients without remission after transsphenoidal surgery and the treatment should be individualized based on the results of operation,pathology and imaging.