Bloom L S, Libertino J A
Department of Urology, Lahey Clinic Medical Center, Burlington, Massachusetts.
Urol Clin North Am. 1989 Aug;16(3):547-65.
Cushing's syndrome represents a constellation of symptoms of various origins. In most patients, detailed endocrinologic and radiologic testing will differentiate between Cushing's disease, adrenal adenoma, adrenal carcinoma, primary bilateral nodular hyperplasia, and ectopic corticotropin-producing tumors. Although adrenal surgery affords rapid and reliable remission in patients with Cushing's syndrome, it is associated with significant morbidity and mortality. Complications can be minimized by careful perioperative preparation. The indications for adrenal surgery for Cushing's disease have been altered radically by the success and low morbidity of transsphenoidal surgery. Total adrenalectomy is indicated in patients with bilateral nodular hyperplasia and should be considered for adults who have failed selective pituitary adenectomy or hypophysectomy and in whom ectopic corticotropin secretion has been unequivocally ruled out. At the Lahey Clinic, total adrenalectomy is performed through an anterior abdominal incision. Anterior approaches are especially indicated in those patients who require abdominal exploration for other intra-abdominal pathologic conditions that require surgery. Total adrenalectomy is indicated in the very rare patient who has Cushing's syndrome caused by ectopic corticotropin production when the patient is severely ill, a primary tumor is not found, and medical therapy fails or is poorly tolerated. Small adrenal tumors are best approached through a flank incision. Larger potentially malignant tumors should be approached through a thoracoabdominal incision.
库欣综合征表现为一系列源自不同病因的症状。在大多数患者中,详细的内分泌学和放射学检查能够区分库欣病、肾上腺腺瘤、肾上腺皮质癌、原发性双侧结节性增生以及异位促肾上腺皮质激素分泌肿瘤。尽管肾上腺手术能使库欣综合征患者迅速且可靠地缓解病情,但它也伴随着显著的发病率和死亡率。通过精心的围手术期准备,并发症可降至最低。经蝶窦手术的成功及低发病率已从根本上改变了库欣病肾上腺手术的适应证。双侧结节性增生患者需行双侧肾上腺切除术,对于选择性垂体腺瘤切除术或垂体切除术失败且已明确排除异位促肾上腺皮质激素分泌的成年人,也应考虑行双侧肾上腺切除术。在拉希诊所,双侧肾上腺切除术通过腹部前切口进行。对于那些因其他需要手术的腹腔内病理状况而需要进行腹部探查的患者,尤其适合采用前路手术。对于因异位促肾上腺皮质激素分泌导致库欣综合征且病情严重、未发现原发肿瘤、药物治疗失败或耐受性差的极少数患者,应行双侧肾上腺切除术。较小的肾上腺肿瘤最好通过侧腹切口进行手术。较大的潜在恶性肿瘤应通过胸腹联合切口进行手术。