经眶额蝶联合入路显微手术切除大型,巨大型垂体腺瘤

目的：介绍一种新联合入路及其在４１例大型、巨大型垂体腺瘤显微手术的运用经验，并与经蝶入路、经颅入路及经额下—蝶窦入路进行比较。方法：作一个眶额骨瓣，于鞍结节处将经额入路（于额叶下方经鞍膈）及经蝶入路（经蝶骨平板及蝶窦前壁）联成一体。结果：全切２５例（６１％），１例术后死亡（２．４％）。结论：此入路适用于巨大型垂体腺瘤切除术。     

Contribution of MRI to the diagnosis of ACTH-secreting pituitary adenomas. Exploration of 9 patients with Cushing's disease

Between June 1987 and October 1989, nine patients with suspected Cushing's disease were explored by magnetic resonance imaging (MRI), using an 0.35 or 0.5 Tesla magnet. All these patients underwent transsphenoidal surgery and radio-surgical correlations could be established. MRI showed a lesion in 5 cases; it was negative in 2 cases and doubtful in 2 cases. Surgery was positive for pituitary adenoma in 7 cases and negative in 2 cases (including 1 patient with subsequently discovered paraneoplastic syndrome). Using sagittal and coronal planes and combined with gadolinium enhancement, MRI proved to be the most sensitive and specific of all exploratory methods for the diagnosis of ACTH-secreting pituitary adenomas.     

儿童和青春期库欣病的诊断和治疗

目的 讨论20岁以下儿童和青春期库欣病的诊断和治疗。方法 男性11例，女性23例，平均年龄15.2岁，平均病程2.1年，表现库欣综合征占91.2%，内分泌学检查符合库欣病占70.6%。CT阳性率57.1%。MRI阳性率70%，均行经蝶窦手术，术后病理证实垂体ACTH腺瘤28例。垂体增生4例，病理阴性2例。结果 随诊3个月至10年，垂体ACTH腺瘤的治愈缓解率89.3%。垂体增生和病理阴性的治疗有效率为50%。结论 经蝶窦显微外科手术是治疗库欣病的最佳方法，当内分泌学检查符合库欣病但蝶鞍MRI未发现垂体腺瘤时，或者当蝶鞍MRI发现垂体腺瘤但内分泌学检查不符合库欣病时，应行经蝶窦垂体探查术。     

Endoscopic transsphenoidal treatment of pituitary adenomas

BACKGROUND AND PURPOSE: The aim of this study is to present a new endoscopic transnasal transsphenoidal method of surgical treatment of pituitary adenomas and to evaluate the results and complications of the method. MATERIAL AND SURGICAL TECHNIQUE: From October 2001 to June 2003 in the Department of Neurosurgery of the Medical University of Silesia in Katowice 88 operations of pituitary adenomas were performed using the transnasal transsphenoidal endoscopic method. The group of patients consisted of 50 females and 38 males. The youngest patient was 11 years old and the oldest was 79 years old. Patients were operated on using the 4-mm diameter endoscope with 0- and 30-degree angled lenses, using a method of operation according to Jho and Carrau with own modifications. At the time of surgery the operation team included 2 neurosurgeons, an anesthesiologist and a laryngologist. RESULTS: In the group of 51 nonfunctioning adenomas, in 32 cases we obtained the total removal of the tumor, which amounts to 63%. Among 37 of hyperfunctioning adenomas there were 11 prolactinomas, 19 GH secreting adenomas and 7 ACTH secreting adenomas. In all cases of prolactinomas the tumor was removed totally and in the cases of GH secreting adenomas and ACTH secreting adenomas the total removal of the tumor was performed in 58% and 86% of the cases, respectively. One patient within our group died after the operation. It was the patient with a huge nonfunctioning macroadenoma, with hydrocephalus and preoperative disturbances of consciousness. The permanent diabetes insipidus occurred in 2 cases, which amounts to 2.3% of all operated patients. In this group we noticed the intraoperative CSF leakage in 20 cases but we did not observe the postoperative CSF leakage or any rhinological complications. CONCLUSIONS: The endoscopic transnasal transsphenoidal approach is an efficient method of surgical treatment of pituitary adenomas. The advantage of this method is low invasiveness and a small number of serious complications.     

垂体ACTH腺瘤经蝶术后复发的诊断与治疗

目的讨论复发性垂体促肾上腺皮质激素(ACTH)腺瘤的诊断和治疗.方法男性6例,女性12例.年龄8～43岁,平均25.7岁.平均病程29.5个月.均表现典型库欣综合征,诊断为垂体ACTH腺瘤行经蝶手术,术后病理为垂体腺瘤.术后皮质醇曾降至正常半年以上,再次出现库欣综合征,血和尿皮质醇增高,CT或MRI显示9例垂体腺瘤复发.12例采用再次经蝶手术,术中9例发现肿瘤,3例未发现肿瘤.6例未再行手术,其中4例行垂体放疗和肾上腺切除术,2例行肾上腺切除术.结果随诊1～12年,再次经蝶手术的治愈缓解率为58%(7/12).共有7例行垂体放疗加肾上腺切除术,4例行肾上腺切除术,术后皮质醇下降,有5例需用激素替代治疗.结论对于复发性垂体ACTH腺瘤首先应进行再次经蝶手术,如果术后皮质醇仍高,可行垂体放疗和/或肾上腺切除术.     

经蝶窦显微外科手术治疗541例库欣病

目的 探讨经蝶窦入路显微外科手术治疗库欣病的疗效。方法 总结北京协和医院神经外科1981年至2007年收治的541例库欣病的临床资料。结果CT和（或）MRI显示微腺瘤、大腺瘤、巨大腺瘤和未见异常的比例分别为66.2％( 358/541)、14.0％( 76/541)、0.6％( 3/541)和19.2％(104/541)。病理为垂体ACTH腺瘤、增生和既未见肿瘤又未见增生的比例分别为75.4％(408/541)、8.3％ (45/541)和16.3％ (88/541)。病理明确腺瘤、增生以及病理阴性者术后近期（半年内）治愈率分别是85.0％( 347/408)、24.4％ (11/45)和45.5％(40/88)。随访0.5 -25.0年（平均3.1年）,复发率13.3％(53/398),复发时间0.5 -16.5年（平均3.4年）。结论 经蝶窦选择性垂体腺瘤切除加瘤周垂体组织大部分切除是治疗库欣病安全有效的首选方法。对首次手术无效或复发性库欣病,可以再次经蝶窦手术或垂体放疗。     

垂体瘤经蝶入路术后常见问题的临床分析

目的:探讨垂体瘤经蝶入路术后常见问题产生的原因和处理的方法。方法:对89例经蝶入路垂体瘤患者的临床表现、术后常见问题处理等作回顾性分析。结果:34例(38．2％)术后出现暂时性尿崩;术后肿瘤残留有17例 (19．1％);16例(17．98％)术后垂体前叶功能不足;术后脑脊液漏3例(3．37％)。结论:手术中操作应轻柔,避免损伤垂体后叶和鞍隔;术后MRI早期增强扫描可发现肿瘤残留:内分泌激素水平监测是必须的,术后垂体功能低下者需激素替代治疗。     

ACTH pituitary adenomas: neurosurgical aspects

We report our experience with 19 cases of ACTH secreting pituitary adenomas. They were microadenomas in 50% of the cases, coming with the typical picture of the Cushing syndrome. The ACTH adenoma associated with other types of hormones tend to show visual alterations. The treatment is often surgical using the transsphenoidal approach. The results were satisfactory in most of the cases. For those in which surgical cure was not reached, radiotherapy was indicated.     

复发垂体ACTH腺瘤的诊断和再次经蝶手术治疗

目的探讨垂体ACTH腺瘤库欣病术后复发的诊断和再次经蝶手术疗效.方法352例ACTH垂体腺瘤病人经蝶手术后,皮质醇降至正常,其后12例症状复发,皮质醇增高,CT或MRI显示9例垂体腺瘤复发,均行再次经蝶手术.结果随诊1～12年,7例皮质醇降至正常,5例皮质醇仍高,其中3例行垂体放疗和肾上腺切除术,2例行肾上腺切除术.结论对于病理为复发性垂体腺瘤的库欣病首选再次经蝶手术,如果术后皮质醇仍高,可行垂体放疗和肾上腺切除术.     

单纯神经内镜下经单鼻腔-蝶窦入路手术治疗垂体腺瘤

目的 总结我科单纯神经内镜下经单鼻腔-蝶窦入路切除垂体腺瘤的手术经验,探讨该手术方式的优越性.方法 对经CT或MRI诊断为垂体腺瘤的42例患者(肿瘤直径<1cm11例,1～2 cm 11例,2～3cm 18例,>3cm 2例)采用经单鼻腔-蝶窦入路手术,术中不切除鼻中隔,直接打开蝶窦前壁,经鞍底切除垂体腺瘤.结果 全切除38例,次全切除4例.视力、视野均较术前有所恢复.6例术后出现一过性脑脊液漏,23例发生暂时性尿崩.术后18例内分泌检查恢复正常,4例随访激素水平下降.结论 单纯神经内镜下经单鼻腔蝶窦入路切除垂体腺瘤,具有手术创伤小、照明好、安全、提供360度全景的术野且并发症少.操作时间短,术后康复快等优点.该术式值得进一步推荐应用.     

垂体细胞增生所致库欣病术后复发的诊断和处理

目的讨论垂体细胞增生所致库欣病经蝶术后复发的诊断和处理。方法根据临床症状,内分泌学检查和蝶鞍区MRI检查,5例病人被诊断为垂体ACTH腺瘤而行经蝶手术,术后病理为垂体细胞增生。术后1例症状无改善,4例症状改善3～11个月后复发皮质醇增高为降低皮质醇例行垂体放疗和肾上腺切除术,。2,3例行肾上腺切除术。在肾上腺切除的例中例症状32再次复发复查,MRI发现垂体腺瘤并再次经蝶手术,术后病理为垂体腺瘤。结果随诊1～5年,行二次经蝶手术的2例病人皮质醇降至正常行垂体放疗和肾上腺切除术者因垂体功能低下用强的松替代治疗结论当垂体细胞增生病人术后症状复发影像学。检查发现垂体肿瘤时应再次经蝶手术没有发现垂体腺瘤时可行肾上腺切除和垂体放疗。     

Pediatric pituitary adenoma: a series of 42 patients.

Pituitary adenomas are uncommon in childhood. This report describes the presentation, endocrinological profile, management and outcome of 42 children with pituitary adenomas. The majority of the tumors were functioning adenomas (40/42, 95.2%). Only two patients (4.8%) had non-functioning tumors. Amongst the functioning tumors, there were 20 patients (47.6%) with prolactinomas, 11 patients (26.2%) with Cushing's disease and nine patients (21.4%) with growth hormone (GH)-secreting adenomas. The most common presenting features were endocrinopathy and visual disturbances. The majority of tumors were macroadenomas, with only eight patients (all with Cushing's disease) having a microadenoma. Transsphenoidal tumor decompression was performed in most cases (71.4%). Complications following transcranial surgery were more frequent when compared to transsphenoidal surgery. There was one death following surgery. Response to radiotherapy was satisfactory. Overall, 67.6% of patients achieved endocrinological remission. Of these, 89% of the children with GH-secreting tumors and 100% of the children with Cushing's disease achieved remission. We conclude that the transsphenoidal approach is effective and safe in surgery for pituitary adenomas in children and is the procedure of choice if there is no contraindication. The combination of surgery and radiotherapy, as well as medical therapy with bromocriptine, achieves good results in the management of these uncommon tumors.     

儿童和青春期垂体腺瘤的诊断和治疗

儿章和青春期垂体腺瘤多数指16或18岁以前经手术证实的病例。全面评估内分泌、影像学改变是诊断的关键、治疗的基础。儿童和青春期PRL腺瘤与成人PRL腺瘤在对溴隐停有效性方面相同。甲介型或气化不良的经窦并不是经蝶手术的禁忌症。关于Cushing’s病的治疗，首选经蝶手术，治愈率可达80％～98％．放射治疗应严格掌握适应症，尽可能减少延迟性的垂体功能低下的发生。术后可以发生各种垂体和下丘脑功能障碍，长期、正规的随访具有特别重要的意义。     

内镜下经鼻蝶入路鞍结节脑膜瘤切除术

目的总结内镜下经鼻蝶入路切除鞍结节脑膜瘤的方法、疗效、手术指征和并发症。方法回顾性分析6例鞍结节脑膜瘤的临床资料,均选择内镜下经鼻入路。结果 SimpsonⅠ级切除5例,SimpsonⅣ级切除1例。术后视力减退改善4例,加重1例;视野障碍改善1例,无变化4例;视力视野术后未查1例。术后并发脑脊液漏2例,暂时性垂体前叶功能减退1例,死亡1例。随访5例,时间6个月～3年,经MRI复查肿瘤均无复发。结论内镜下经鼻蝶入路是鞍结节脑膜瘤外科治疗方法之一,具有手术创伤小、手术时间短及可达SimpsonⅠ级切除的优点。但本方法并不适合所有鞍结节脑膜瘤,需有严格的手术指征。     

在导航引导下经鼻蝶切除垂体腺瘤治疗效果的研究

目的探讨在导航引导下经鼻蝶切除垂体腺瘤的治疗效果。方法回顾2004-2005 年导航引导与非导航引导下经鼻蝶切除垂体腺瘤患者57例,对所有病例分别统计术前年龄、肿瘤大小、手术麻醉时间、切除肿瘤时手术时间、术后住院天数、术后并发症,术后3个月复查MRI提示肿瘤全切程度,结果用SPSS 11．0软件进行统计,分析两组之间结果的差异。结果导航组与非导航组中患者年龄、肿瘤大小、麻醉时间、手术后住院天数、术后并发症方面无显著性差异,而手术时间及术后3 个月复查MRI提示肿瘤全切率有显著性差异。结论在导航引导下经鼻蝶切除垂体腺瘤提高了垂体腺瘤患者肿瘤的全切率,缩短了手术切除肿瘤的时间。     

儿童和青春期垂体腺瘤的经蝶手术治疗

目的探讨年龄小于17岁的儿童及青春期垂体腺瘤的诊断及其经蝶窦显微外科手术治疗。方法本组20例(垂体PRL腺瘤7例，ACTH腺瘤5例，GH腺瘤4例，无功能腺瘤4例)均行经蝶窦显微外科垂体腺瘤切除术。结果肿瘤全切除18例，部分切除2例；随访8个月～8年，治愈75％，缓解25％，复发5％；常见并发症为尿崩症(25％)和电解质紊乱(25％)。结论经蝶窦垂体腺瘤切除术是治疗儿童及青春期垂体腺瘤的安全有效的方法：     

显微镜下经鼻-蝶窦入路术中脑脊液鼻漏的处理

目的探讨显微镜下经鼻-蝶窦入路术中脑脊液鼻漏的处理方法。方法回顾性分析21例显微镜下经鼻-蝶窦入路术中发生脑脊液鼻漏的临床资料。鞍结节脑膜瘤1例,脊索瘤1例,垂体腺瘤19例。在切除肿瘤后均采用人工硬脑膜-明胶海绵-蛋白生物胶方法重建颅底硬膜,并辅以碘仿纱条填塞蝶窦腔。结果术后随访3个月~1年,无脑脊液鼻漏发生20例,发生可疑脑脊液鼻漏1例,保守治疗康复。结论即使手术导致颅底骨质、硬膜缺损明显,重建颅底硬膜辅以碘仿纱条填塞蝶窦腔,仍可有效预防术后脑脊液鼻漏的发生。     

额下经蝶入路显微手术切除鞍区肿瘤20例报告

目的　报告２０ 例鞍区肿瘤采用额下经蝶入路显微手术的体会, 确立一种手术方法,评价其利弊。方法　额部骨瓣开颅后, 先切除肿瘤的鞍上部分, 而后磨除鞍结节、蝶骨平台及蝶鞍前壁骨质, 显露并切除鞍内及蝶窦内肿瘤。结果　全切１８ 例, 近全切除２ 例。脑脊液鼻漏３ 例, 无颅内感染及死亡。结论　该入路适于一期手术全切除鞍内及鞍上的肿瘤, 尤其适于肿瘤侵入蝶窦内并且合并视交叉前置的患者, 值得重视和推广。     

大型、巨大型垂体腺瘤与垂体柄显微解剖关系的术中观察

目的在切除大或巨大型垂体腺瘤术中更好地保留垂体柄的解剖和功能.方法回顾性分析123例大型和巨大型垂体腺瘤的手术资料和影像学资料.在鞍结节至鞍背的平面上按时钟划分为6个区间,观察垂体柄在此6个区的分布.结果垂体柄没有位于第Ⅰ区间者,位于第Ⅱ区间者12例（9.8%）,第Ⅲ区间者46例（37.4%）,第Ⅳ区间者29例（23.6%）,第Ⅴ区间者31例（25.2%）,第Ⅵ区间者5例（4.1%）.结论大多的垂体柄位于垂体腺瘤的后方或侧后方,术中按其分布特点可更好地寻找垂体柄.     

Severe myopathy and psychosis in a patient with Cushing's disease macroadenoma

Cushing's disease is most commonly caused by a corticotrope adenoma of the pituitary. Between 50 and 70% of patients with spontaneous hypercortisolism have ACTH-producing pituitary adenomas. The tumors are usually microadenomas with approximately 20% of patients with the disease showing no evidence of tumor on CT-scans or MR imaging of their pituitary glands. In contrast to patients with ectopic ACTH production, plasma ACTH concentrations in patients with spontaneous disease are generally within the normal range. We describe here a patient with a pituitary macroadenoma that showed evidence of necrosis on MRI. The patient had an atypical clinical presentation with plasma ACTH levels considerably higher than that seen in patients with non-ectopic ACTH-secretory syndrome, markedly elevated urine free cortisol, lack of phenotypical signs of hypercortisolism such as wide purplish striae, and whose most prominent and distressing symptom was severe myopathy that resulted in the patient becoming bed-ridden. Psychosis was another striking feature in this patient who during his hospital course developed multiple opportunistic infections that contributed to his demise.