自体肾上腺移植治疗难治性库欣病临床疗效分析

Evaluation of adrenal autotransplantation for the treatment of persistent Cushing＇s disease

目的探讨垂体肿瘤切除术后难治性库欣病行双侧肾上腺全切并带血管自体肾上腺移植的临床疗效。方法回顾性分析4例难治性库欣病患者行带血管白体肾上腺移植的远期疗效，男1例，女3例，年龄14～36岁。4例肾上腺移植前均行经鼻经蝶窦垂体肿瘤切除术，术后效果不佳；肾上腺移植术式为左侧肾上腺全切，取30％～50％肾上腺组织移植于左侧髂窝，游离腹壁下动脉并与肾上腺中央静脉吻合，游离大隐静脉并套入式吻合在肾上腺切开的包膜上，术后逐渐减少激素替代用量。分别随访1．0、1．5、8．0、10．0年，观察患者临床症状，检测血皮质醇，24h尿游离皮质醇，促肾上腺皮质激素（AcTH），调整激素替代用量。结果4例患者自体肾上腺移植患者术后库欣病症状消失，无Nelson综合征发生，仅1例皮肤轻度变黑。影像学、手术、临床表现和内分泌检查证实，4例移植肾上腺均存活，并可减少激素替代用量。移植术后1年，。肾上腺功能和激素替代用量保持稳定，存活的移植肾上腺在高ACTH刺激下无明显增生。结论双侧肾上腺全切并自体肾上腺移植远期疗效明确，可作为难治性库欣病治疗的可选方案。

Objective To evaluate the safety and efficacy of adrenal autotransplantation for the treatment of persistent Cushing＇s disease after transsphenoidal pituitary tumor resection. Methods Four patients were treated by adrenal autotransplantation with attached blood vessels after bilateral adrenalectomy for persistent Cushing＇s disease from April 1991 to March 2008 in our institute. The four patients were 3 females and 1 male. Their ages ranged from 14 to 36 years, with an average of 30 years. Right adrenalectomy was performed 1 to 3 months before the left adrenalectomy. 30%--50% of the left hyperplastic adrenal was placed in the left inguinal region with the anastomosis of the inferi or epigastric artery and the central adrenal vcin, and between the left saphenous vein and the incised adrenal envelope with the adrenal middle artery inside. The hormone replacement dosage decreased gradually after operation. They were followed up for 1.0, 1.5, 8.0 and 10. 0 years. The patient＇s symptoms, adrenal hormone （serum cortisol, urinary free cortisol and adrenocorticotroph） levels and the steroid replacement dosages were recorded and analyzed. Results The symptom of the Cushing＇s disease disappeared completely after operation and there was no Nelson＇s syndrome except one patient with slight darken skin. Through 4 ways of imaging examination, operation, clinical presentation and endocrine examination, the 4 transplanted adrenals functioned well with less steroid replacement dosage needed after operation. The 4 transplanted adrenal glands functioned steadily 1 year after the autotransplantation, and no hyperplasia was detected in the transplanted adrenals with the stimulus of high dosage adrenocorticotroph. Conclusions The long term effects of adrenal autotransplantation with attached blood vessels after bilateral adrenaleclomy is effective and safe. Adrenal autotransplantation can be a feasible option for the treatment of persistent Cushing＇s disease.