腹腔镜单侧肾上腺切除术治疗8例原发性双侧肾上腺大结节样增生患者的疗效随访

Follow-up of the rapeutic efficacy of laparoscopic unilateral adrenalectomy for treatment of 8 patients with primary bilateral macronodular adrenal hyperplasia

目的探讨行腹腔镜单侧肾上腺切除术的8例原发性双侧肾上腺大结节样增生(PBMAH)患者的治疗效果。方法回顾性分析香港大学深圳医院2015年1月至2020年12月收治的8例确诊为PBMAH患者的临床资料。其中男性5例,女性3例,平均年龄50(34~61)岁。定性诊断有4例为促肾上腺皮质激素(ACTH)非依赖性库欣综合征(CS),另外4例为ACTH非依赖性亚临床库欣综合征(SCS)。完善内分泌、生物化学、病理及影像学检查。所有患者术前均分别行多学科讨论,手术先切除增生明显或者增生体积较大的一侧肾上腺,围手术期和术后均予以激素替代治疗。术后定期随访患者血常规、肝功能、肾功能、血钾、肾上腺相关激素水平与功能试验、垂体及肾上腺MRI/CT等。结果8例均行后腹腔途径的腹腔镜单侧肾上腺切除术治疗,右侧5例,左侧3例,手术顺利,均无并发症发生。术前肾上腺增强CT提示7例患者双侧肾上腺弥漫性增大且伴有多发结节,1例患者6年前切除左侧肾上腺故表现为右侧肾上腺弥漫性增大伴结节。病理结果提示肾上腺皮质结节状增生5例,肾上腺皮质腺瘤3例。平均随访30.5个月,2例典型CS临床表现和体征的患者,症状得到缓解。8例患者术后的血压和体重指数(BMI)较术前有明显改善,24 h的尿皮质醇水平及血钾均正常,双侧肾上腺均被切除的1例患者术后行终身激素替代治疗。结论腹腔镜单侧肾上腺切除术治疗PBMAH安全有效,能改善患者的CS临床症状,推荐为临床一线治疗方案。若行双侧肾上腺全切术,术后需要终身激素替代治疗。

Objective To investigate the therapeutic efficacy of laparoscopic unilateral adrenalectomy in the treatment of primary bilateral macronodular adrenal hyperplasia(PBMAH).Methods Clinical data of 8 PBMAH patients treated during Jan.2015 and Dec.2020 were retrospectively analyzed,including 5 males and 3 females,average age 50 years(range:34 to 61 years).Adrenocorticotropic hormone(ACTH)-independent Cushing s syndrome(CS)was diagnosed in 4 cases,and ACTH-independent subclinical Cushing s syndrome(SCS)was diagnosed in the other 4 cases.All patients received endocrine,bioche-mical,pathological and imaging examinations.The adrenal gland with obvious hyperplasia or the larger size was removed firstly.Perioperative and postoperative hormone replacement therapies were administered according to the results of preoperative multidisciplinary discussion.Regular follow-up after surgery included complete blood count,serum potassium,liver and renal function tests,endocrine examinations,MRI and CT scan of pituitary and adrenal gland.Results All of the 8 patients underwent laparoscopic unilateral adrenalectomy by retroperitoneal approach,including 5 cases in the right side,and 3 cases in the left side.All operations were successful without complications.Perioperative adrenal enhanced-CT scan showed 7 cases had bilateral adrenal diffuse enlargement of multiple nodules and 1 case who had received adrenalectomy in the left side due to PBMAH 6 years ago had right adrenal diffuse enlargement of multiple nodules.Pathological results showed cortical nodular hyperplasia in 5 cases and adrenal cortical adenoma in 3 cases.During the mean follow-up of 30.5 months(range:19 to 48 months),24 h urine cortisol levels and serum potassium returned normal,2 patients with CS were improved,and blood pressure and body mass index(BMI)were significantly improved in all patients.However,the patient with bilateral adrenalectomies had to receive lifelong hormone replacement therapy.Conclusion Laparoscopic unilateral adrenalectomy is a safe and effective treatment for PBMAH,which can improve the symptoms of CS.Lifelong hormone replacement therapy is required if bilateral adrenalectomies are performed.