Knappe U J, Lüdecke D K
Department of Neurosurgery, University Hospital Eppendorf, Hamburg, Germany.
Neurosurgery. 1996 Sep;39(3):484-92; discussion 492-3. doi: 10.1097/00006123-199609000-00010.
Transnasal adenomectomy is the treatment of choice for Cushing's disease. We review the diagnostic peculiarities, specific surgical problems, and outcome of children and adolescents with Cushing's disease.
We report on our series of 55 consecutive children and adolescents (range, 4.2-18.9 yr [mean age, 14.4 yr]; female:male = 1.1:1.0; mean follow-up, 54.5 +/- 38.6 mo [standard deviation]) with Cushing's disease on whom we performed surgery since 1980. The indication for transsphenoidal surgery is based on endocrinological parameters and not on neuroradiological findings.
Detection rate of the tumor site was 22% using computed tomography and 33% using magnetic resonance imaging. Only 7 of 13 interpetrosal adrenocorticotropic hormone gradients obtained during inferior petrosal sinus sampling correctly lateralized the tumor site preoperatively (53.8%). In cases of incomplete sphenoid pneumatization, adequate exposure is achieved by drilling. The tumor finding rate is 98%. The remission rate is 100% when two early subsequent operations are included. The recurrence rate for 45 primary operations with follow-up of at least 1 year is 15.5%. Seven of nine subsequent operations for recurrent hypercortisolism were successful. One patient needed three more operations until hypercortisolism subsided; one patient achieved remission after additional pituitary irradiation. The surgical morbidity was low in this series, which consisted of two cerebrospinal fluid fistulas. The incidence of hypopituitarism after primary operations (10.3%) is significantly lower than after subsequent operations (45.5%).
Direct transnasal submucosal surgery for Cushing's disease is successful, and pituitary function can be preserved in most of these young patients.
经鼻腺瘤切除术是库欣病的首选治疗方法。我们回顾了儿童和青少年库欣病的诊断特点、特殊手术问题及治疗结果。
我们报告了自1980年以来对55例连续的儿童和青少年（年龄范围4.2 - 18.9岁[平均年龄14.4岁]；女性:男性 = 1.1:1.0；平均随访时间54.5 ± 38.6个月[标准差]）进行库欣病手术的情况。经蝶窦手术的指征基于内分泌参数而非神经放射学检查结果。
计算机断层扫描对肿瘤部位的检出率为22%，磁共振成像为33%。在岩下窦采样期间获得的13个岩间促肾上腺皮质激素梯度中，只有7个（53.8%）在术前正确定位了肿瘤部位。在蝶窦气化不完全的情况下，通过钻孔可实现充分暴露。肿瘤发现率为98%。若包括两次早期后续手术，缓解率为100%。45例至少随访1年的初次手术的复发率为15.5%。9例复发性皮质醇增多症的后续手术中有7例成功。1例患者需要再进行3次手术直至皮质醇增多症消退；1例患者在接受额外的垂体照射后实现缓解。本系列手术并发症发生率较低，包括2例脑脊液漏。初次手术后垂体功能减退的发生率（10.3%）显著低于后续手术（45.5%）。
直接经鼻黏膜下手术治疗库欣病是成功的，且大多数年轻患者的垂体功能可得到保留。
