摘要目的 探讨后腹腔镜手术治疗库欣病的临床疗效.方法 回顾性分析2006年2月至2017年2月天津医科大学总医院收治的38例库欣病患者的临床资料.男6例,女32例;年龄13 ～66岁,平均38岁;病程2 ～96个月,平均28.3个月.38例均有皮质醇增多症表现,内分泌检测结果示血浆皮质醇昼夜节律均消失,血皮质醇579.6～1 821.6 nmol/L (21.0 ～ 66.0 μg/dl),平均1 054.3 nmol/L(38.2 μg/dl);24 h尿皮质醇168.1 ～1 980.0 μg/24 h,平均671.3μg/24 h;血促肾上腺皮质激素(ACTH) 29.8～76.8 pmol/L(135.4 ～ 348.9 pg/ml),平均54.2 pmol/L(246.2 pg/ml),大、小剂量地塞米松抑制试验均不被抑制.肾上腺CT检查示双侧肾上腺增生.其中32例既往曾有经蝶窦垂体肿瘤手术和(或)放疗史,垂体MRI检查均示垂体术后改变,未见明显肿瘤.6例垂体MRI检查未见垂体异常,临床诊断为垂体微腺瘤.38例均行后腹腔镜手术治疗,其中8例同期行一侧肾上腺全切+对侧肾上腺次全切除术,30例行单侧肾上腺切除术.结果 所有手术均顺利完成,无中转开放和死亡病例.手术时间30 ～270 min,平均88.3 min.术中出血量10 ～200 ml,平均38.3 ml,无输血病例.术后随访2～13年,平均7年.8例行一侧肾上腺全切+对侧肾上腺次全切除术患者术后第1天24 h尿皮质醇90.35～220.84 μg/24h,平均102.83μg/24h,6例在正常范围内;术后6～11个月患者的大部分症状消失,术后1～3个月停用激素替代治疗;3例分别于术后1.0、3.5、5.0年复发,行残余肾上腺切除术.30例单侧肾上腺切除患者术后第1天24 h尿皮质醇99.80 ～550.84 μg/24h,平均372.83μg/24h,其中24例高于正常值,6例在正常范围内;术后1个月复查24 h尿皮质醇382.16 ～ 520.34μg/24h,平均461.62 μg/24h,均高于正常值,临床症状缓解不满意;其中22例术后2～7个月行对侧肾上腺大部分(80％)切除术,8例行对侧肾上腺切除及肾上腺组织自体移植术;再次手术患者术后第1天24h尿皮质醇62.58 ～ 182.34 μg/24h,平均92.83μg/24h,22例在正常范围内;术后6～9个月患者的大部分临床症状消失,术后3个月停用激素替代治疗;其中2例分别于二次手术后2.0、4.5年复发,行残余肾上腺切除术.最终共13例终生激素替代治疗,5例出现Nelson综合征.结论 后腹腔镜一侧肾上腺全切+对侧肾上腺次全切除术是治疗复杂性库欣病安全有效的治疗方法,大多分期进行,通常先行一侧肾上腺全切术,如临床症状缓解不明显,择期行对侧肾上腺次全切除术,可避免终生激素替代治疗.术后应密切随访,一旦复发,可考虑行残存肾上腺切除术.

abstractsObjective To evaluate the feasibility and clinical efficacy of retroperitoneal laparoscopic adrenalectomy for the treatment of Cushing disease.Methods Clinical data collected from 38 cases retroperitonel laparoscopic adrenalectomy for Cushing disease,from February 2006 to February 2017 were analyzed retrospectively.Among them,there were 6 males and 32 females aged from 13 to 66 years old,with an average age of 38 years old.The disease history ranged from 2 to 96 months,with an average of 28.3 months.Besides,there were 32 cases that had previous history of transsphenoidal pituitary tumor surgery and or radiotherapy.All 38 cases underwent retroperitoneal laparoscopic adrenalectomy therapy.There were 8 cases accepted right adrenalectomy and left subtotal adrenalectomy at the same time,24 cases accepted unilateral adrenalectomy,another 6 cases of pituitary tumors without lesions in medical imaging underwent unilateral adrenalectomy.Results All operations were completed sucessfully without conversion to open surgery and mortality.There was no blood transfusion during the period of operation.The operation time ranged from 30 min to 270 min (mean 88.3 min),the amount of bleeding was among the range of 10 ml to 200 ml (mean 38.33 ml).During 2 to 13 years of the follow up (mean 7 years),the 24 h urine cortisol concentration in the first day after surgery of piatients with subtotal resection was 90.35-220.84 μg/24h (mean 102.83 μg/24h),and 6 cases were in the normal range.After surgery 6-11 months,the clinical symptoms completely disappeared.The hormone replacement therapy was discontinued 1-3 months after surgery.However,There were 3 cases treated with residual adrenalectomy because of the recurrence after 1,3.5,5.0 years respectively.30 cases underwent unilateral adrenalectomy,whose 24 h urine cortisol concentration in the first day after surgery was 99.80-550.84 μg/24h (mean 372.83 μg/24h),among which 24 cases were beyond normal range,and another 6 cases were within the normal range.Reviewing 24 h urine cortisol concentration one month later after operation,it was 382.16-520.34 μg/24h (mean 461.62 μg/24h),with the results of all cases being higher than the normal range,the clinical symptoms were not relieved satisfactorily.During 2 to 7 months after surgery,there were 22 cases further underwent contralateral subtotal adrenalectomy (80％),the remaining 8 cases underwent contralateral adrenalectomy and autologous transplantation of adrenal tissue.Immediately one day after surgery,the 24 h urine cortisol concentration level of patients was ranged from 62.58 to 182.34 μg/24h (mean 92.83 μg/24h),and all 22 cases were within the normal range.The clinical symptoms completely disappeared during next 6 to 9 months after surgery,and hormone replacement therapy was discontinued 3 months after surgery.There were 2 cases received residual adrenalectomy because of the recurrence.Lifelong hormone replacement therapy after surgery occurred in 13 cases and 5 cases developed Nelson syndrome.Conclusions Retroperitoneal laparoscopic of unilateral adrenalectomy and contralateral subtotal adrenalectomy for the treatment of Cushing disease was safe and effective.The contralateral adrenal subtotal resection could be performed at an appropriate time in the case of that the clinical symptoms not obvious.By doing so,it could significantly alleviate the clinical symptoms as well as avoiding lifelong hormone replacement therapy.Once recurrence,residual adrenalectomy can be considered.