Lowery Aoife J, Seeliger Barbara, Alesina Pier F, Walz Martin K
Graduate Entry Medical School, University of Limerick and University Hospital Limerick, Limerick, Ireland.
Klinik für Chirurgie und Zentrum für Minimal Invasive Chirurgie, Kliniken Essen-Mitte, Evang. Huyssens-Stiftung/Knappschaft GmbH, Akademisches Lehrkrankenhaus der Universität Duisburg-Essen, Essen, Germany.
Langenbecks Arch Surg. 2017 Aug;402(5):775-785. doi: 10.1007/s00423-017-1569-6. Epub 2017 Feb 28.
The treatment of hypercortisolism for patients with bilateral adrenal disease (BAD) is controversial. Bilateral total adrenalectomy results in permanent hypocortisolaemia requiring lifelong steroid replacement. A more conservative surgical approach, with less than bilateral total adrenalectomy (leaving functional adrenal tissue either unilaterally or bilaterally), represents an alternative option; however, long-term outcome or recurrence data are limited. We report our experience with the surgical management of hypercortisolism caused by BAD.
Between 2004 and 2016, 42 patients (12 male, 30 female; mean age 58 ± 10 years) with clinical or subclinical Cushing's syndrome (CS/sCS) caused by BAD underwent adrenal surgery via the posterior retroperitoneoscopic approach. Adrenal surgery was defined as "adrenalectomy" when total gland excision was performed or "resection" when a partial or subtotal adrenal resection was performed. Clinical, radiological and biochemical parameters were evaluated preoperatively and postoperatively.
Seventy adrenal operations performed in total included unilateral resection (n = 3), unilateral adrenalectomy (n = 15), bilateral resection (n = 9), adrenalectomy and contralateral resection (n = 14) and bilateral total adrenalectomy (n = 3). Median operating time was 47.5 min (30-150) with no difference between unilateral and bilateral (synchronous included) procedures (p = 0.15). Mortality was zero. Clavien-Dindo grade of postoperative complications was I (n = 5) and IV (n = 3). All but one patient with CS and 17/31 patients with sCS received postoperative steroid supplementation for a median duration of 20 (1.5-129) months. After median follow-up of 40 months (3-129), the remission rate was 92%; 11 patients required ongoing steroid supplementation. There were three biochemical recurrences (two underwent contralateral resection); two patients with new/progressive radiological nodularity are biochemically eucortisolaemic. A significant reduction in BMI (p = 0.01) and antihypertensive requirements (p = 0.04) was observed postoperatively.
A surgical approach which facilitates the conservation of functional adrenal tissue represents a suitable strategy for hypercortisolism caused by BAD. This approach avoids the necessity for lifelong steroid replacement in the majority of cases with low rates of adrenal insufficiency and recurrence.
双侧肾上腺疾病（BAD）患者的皮质醇增多症治疗存在争议。双侧肾上腺全切术会导致永久性皮质醇减少症，需要终身进行类固醇替代治疗。一种更为保守的手术方法，即切除范围小于双侧肾上腺全切术（单侧或双侧保留功能性肾上腺组织），是一种替代选择；然而，长期疗效或复发数据有限。我们报告了我们对BAD所致皮质醇增多症手术治疗的经验。
2004年至2016年间，42例由BAD引起的临床或亚临床库欣综合征（CS/sCS）患者通过后腹腔镜途径接受了肾上腺手术。当进行全腺体切除时，肾上腺手术定义为“肾上腺切除术”；当进行部分或次全肾上腺切除时，定义为“切除术”。术前和术后评估临床、影像学和生化参数。
共进行了70例肾上腺手术，包括单侧切除（n = 3）、单侧肾上腺切除术（n = 15）、双侧切除（n = 9）、肾上腺切除术加对侧切除（n = 14）和双侧肾上腺全切术（n = 3）。中位手术时间为47.5分钟（30 - 150分钟），单侧和双侧（包括同期手术）手术之间无差异（p = 0.15）。死亡率为零。术后并发症的Clavien - Dindo分级为I级（n = 5）和IV级（n = 3）。除1例CS患者外，所有患者和31例sCS患者中的17例接受了术后类固醇补充，中位持续时间为20（1.5 - 129）个月。中位随访40个月（3 - 129个月）后，缓解率为92%；11例患者需要持续进行类固醇补充。有3例生化复发（2例接受了对侧切除）；2例有新的/进行性影像学结节的患者生化检查显示皮质醇正常。术后观察到BMI（p = 0.01）和降压药物需求（p = 0.04）显著降低。
一种有利于保留功能性肾上腺组织的手术方法是BAD所致皮质醇增多症的合适策略。这种方法在大多数情况下避免了终身类固醇替代的必要性，肾上腺功能不全和复发率较低。
