单侧肾上腺切除术治疗原发性双侧大结节性肾上腺皮质疾病的疗效和围手术期处理(附手术视频)

Effectiveness and perioperative managements of unilateral adrenalectomy as surgical treatment in curing primary bilateral macronodular adrenal hyperplasia

目的探讨单侧肾上腺切除术治疗原发性双侧大结节性肾上腺皮质疾病(PBMAH)的疗效和围手术期处理。方法云回顾性分析2008年5月至2023年3月上海瑞金医院收治的54例PBMAH患者的病例资料,男24例,女30例。年龄(55.1±7.9)岁。体质量指数(24.9±2.9)kg/m^(2)。库欣综合征特征性临床表现分别为满月脸32例,水牛背24例,紫纹9例,痤疮6例,多血质面容30例,多毛质10例。51例(94.4%)伴高血压,收缩压(159.1±15.8)mmHg(1mmHg=0.133kPa),舒张压(99.2±11.0)mmHg。所有患者术前CT影像均显示双侧肾上腺明显增大伴多发大小不等结节,呈融合团块样特征性改变。结节最大径(10.3±3.5)cm。术前8:00时血皮质醇(21.5±7.7)μg dl,24h尿游离皮质醇(442.6±300.4)μg/24h,8:00时血浆促肾上腺皮质激素(ACTH)(6.4±2.3)pg/ml。所有患者术前行2mg地塞米松抑制试验,结果均为不被抑制。48例术前行8mg地塞米松抑制试验,结果均为不被抑制。根据患者术前临床表现和实验室检查结果,分为亚临床型8例、临床型37例、危重型9例。本组54例,均选择影像学检查显示肿瘤较大侧肾上腺,行单侧肾上腺切除术,均为首次行肾上腺切除术,传统开放手术13例,普通腹腔镜手术27例,机器人辅助腹腔镜手术14例。比较不同术式的手术时间、术中出血量、住院时间。比较各型患者手术前后临床症状、血尿皮质醇浓度、ACTH变化,记录术后生化复发率。比较复发患者术前一般资料与总体患者的差异。结果与普通腹腔镜手术相比,机器人辅助腹腔镜手术的手术时间更短[(115.4±22.1)min与(95.0±19.8)min,P=0.045],术中出血量[(118.2±57.0)ml与(125.6±45.3)ml,P=0.441]和住院时间[(5.2±0.9)d与(6.4±1.2)d,P=0.279]差异无统计学意义。与微创手术相比,开放手术的手术时间更长[(134.0±34.5)min与(104.3±20.1)min,P=0.035],术中出血量更多[(305.5±85.2)ml与(122.5±44.3)ml,P=0.012],住院时间更长[(10.4±3.2)d与(5.7±1.0)d,P=0.020]。所有患者术后病理诊断均为肾上腺皮质大结节增生。本研究54例,随访(23.7±11.7)个月。16例生化复发,无复发时间(25.4±13.4)个月。54例术后3个月收缩压为(131.1±16.8)mmHg(P=0.001),舒张压为(82.2±11.1)mmHg(P=0.002),均较术前明显下降。8:00时血皮质醇为(10.2±4.0)μg/dl(P<0.01)24h尿游离皮质醇为(106.6±43.4)μg/24h(P<0.01),8:00时ACTH为(12.6±4.1)pg/ml(P=0.005),较术前均显著改善。复发患者为临床型12例、危重型4例,术前血皮质醇(24.7±8.2)μg/dl(P=0.046),尿皮质醇(522.8±234.2)μg/24h(P=0.028),均高于总体患者术前数据。16例均行挽救性对侧肾上腺切除术,术后长期补充醋酸可的松(8:00时25mg+20:00时12.5mg)。结论单侧肾上腺切除术治疗PBMAH安全有效。亚临床型患者术后无复发。复发患者术前血、尿皮质醇水平更高,应行挽救性对侧肾上腺切除术并终身补充皮质激素。

Objective To evaluate the efficacy and safety of unilateral adrenalectomy for treating primary bilateral macronodular adrenal hyperplasia(PBMAH)of different clinical types.Methods The clinical and biochemical data of 54 patients with PBMAH who underwent unilateral adrenalectomy from May 2008 to March 2023 were retrospectively collected.Preoperative CT images of all patients showed enlarged bilateral adrenal glands with multiple nodules of"fused masses".Mean preoperative blood cortisol concentration at 8am was(21.5±7.7)μg/dl,urinary free cortisol concentration was(442.6±300.4)μg/24h,and mean 8am ACTH concentration was(6.4±2.3)pg/ml.Postoperative symptoms,BMI,blood pressure,mass diameter,cortisol and ACTH concentration were recorded and analyzed.Results Compared with ordinary laparoscopic surgery,robot-assisted surgery showed shorter operation time[(115.4±22.1)min vs.(95.0±19.8)min,P=0.045];less blood loss[(118.2±57.0)ml vs.(125.6±45.3)ml,P=0.441] and shorter hospitalization time[(5.2±0.9)day vs.(6.4±1.2)day,P=0.279].Compared with laparoscopic surgery,open surgery showed longer operation time[(13434.5)min vs.(104.3±20.1)min,P=0.035];more blood loss[(305.5±85.2)ml vs.(122.5±44.3)ml,P=0.012]and longer hospitalization time[(10.4±3.2)day vs.(5.7±1.0)day,P=0.020].The average follow-up time was(23.7±11.7)months.Sixteen cases biochemically relapsed,and the average relapse-free time was(25.4±13.4)month.Mean postoperative systolic blood pressure was(131.1±16.8)mmHg(P=0.001)while diastolic blood pressure decreased to(82.2±11.1)mmHg(P=0.002).Postsurgical average blood cortisol concentration decreased to(10.2±4.0)μg/dl(P<0.01),while urine cortisol concentration decreased to(106.6±43.4)μg/24h(P<0.01).Average ACTH concentration increased to(12.6±4.1)pg/ml(P=0.005).Recurrent patients had higher preoperative blood and urine cortisol concentration(24.7±8.2)μg/dl(P=0.046),(522.8±234.2)μg/24h(P=0.028),and all of them underwent contralateral adrenalectomy.Conclusions Unilateral adrenalectomy is safe and effective for treatment of PBMAH while part of patients biochemically relapsed.Subclinical patients were observed no recurrent cases after surgery.Recurrent patients have higher preoperative blood and urine cortisol levels and should undertake contralateral adrenalectomy and supplement corticosteroids for whole life.