小骨窗前纵裂入路显微手术切除巨大颅咽管瘤(17例报告)

目的评价小骨窗前纵裂入路在颅咽管瘤手术中的应用价值及并发症。方法经小骨窗前纵裂入路切除巨大颅咽管瘤17例,对手术技术及结果进行总结。结果本组全切除15例、近全切除2例,无手术死亡病例。由于术中直视下处理肿瘤在垂体漏斗部位的粘连,可较好保护垂体柄、下丘脑结构及局部穿支血管,13例患者超过2年的随访未见肿瘤复发及再生长。结论经小骨窗前纵裂入路可直视下处理肿瘤鞍内、鞍上及三脑室前部分,是鞍上生长位置较高的大型颅咽管瘤安全有效的手术入路之一。     

Treatment of craniopharyngiomas—the stereotactic approach in a ten to twenty-three years' perspective

Summary Fourty-two consecutive patients with craniopharyngioma were treated by stereotactic approach, i.e. preferentially stereotactic puncture and installation of colloid isotope into cystic tumours and external stereotactic single dose irradiation to solid tumour parts. In a minority of cases, such treatment was less suitable, and surgical removal and/or radiotherapy was used. There was no peroperative mortality. A long-term follow up (observation time 10–23 years) of the 31 patients alive indicated that they were socially well adapted with a high rate of fulltime work and a low rate of intercurrent disease. In spite of substitution therapy for pituitary insufficiency in most cases, the patients were subjectively seldom disturbed by their disease.Our results support a change in the choice of therapy for craniopharyngioma patients, from open neurosurgery to the less invasive stereotactic techniques.     

颅咽管瘤全切除术及术后并发症的防治

目的探讨颅咽管瘤全切除术的手术方法和术后并发症的防治措施。方法24例颅咽管瘤都位于鞍区。术前准备主要包括糖皮质激素的替代疗法和癫痫的预防。手术均采取翼点入路，采用显微技术从鞍区各个手术间隙分块切除肿瘤。术后处理主要包括严密观测病人的意识、尿量和血清电解质，及时防治尿崩症、电解质紊乱等并发症，预防癫痫和糖皮质激素替代治疗。结果22例病人显微镜下肿瘤全切，2例肿瘤有少部分残余，术后无明显的神经功能障碍。16例发生了尿崩症，12例发生了电解质紊乱，6例发生了体温失衡。经过处理后，电解质紊乱和体温失衡完全纠正，13例尿崩症痊愈，3例得到了缓解。结论根据肿瘤的扩展范围以选用最合适的手术入路，以及熟悉鞍区的各个手术间隙解剖并在术中充分利用之是完成颅咽管瘤全切除术的关键。术前采用糖皮质激素的替代疗法，术后严密监测尿量和血电解质，积极处理尿崩症、电解质紊乱等并发症，可以有效降低手术死亡率。     

MVD及VEGF在颅咽管瘤中的表达及其与预后的关系

背景与目的：颅叫管瘤为难治性肿瘤，其预后不良，复发机制尚不清楚。本研究检测颅Ⅱ阏管瘤组织中微血管密度（microvascular density，MVD）及血管内皮生长因子vascular endothelial growth factor，VEGF表达．分析其与肿瘤预后等生物学行为的关系。方法：采取前瞻性队列研究方案，经纳入、排除标准筛选卅颅咽管釉质上皮型肿瘤32例、鳞形乳头瘤型肿瘤31例，平均随访843个月，收集原发及复发患者的肿瘤组织标本，应用单克隆抗体CD34、VEGF抗体检测肿瘤组织MVD及VEGF蛋白表达，计算机辅助成像系统对其作出定量分析，分析其与肿瘤复发等生物学行为的关系。结果：VEGF蛋白、MVD计数在颅咽管瘤队列复发组、非复发组间不具有显著性差异（P〉0．05），而在颅咽管瘤不同病理类型间差异则具有显著性（P〈0．05）。结论：VEGF蛋白、MVD计数与肿瘤复发无关，不能预测颅咽管瘤复发的危险性，但可为了解肿瘤的同有侵袭性行为提供参考。     

Aggressive resection of craniopharyngioma achieved by drilling the tuberculum sellae/planum sphenoidale using a frontal basal interhemispheric approach: A review of 55 cases

ObjectiveThe frontal basal interhemispheric approach (FBIA) is preferable for resection of craniopharyngioma (CP), achieving desirable total resection rates in early reports of lesions located in the suprasellar region to the third ventricle. For tumours that have created a larger obstruction of the tuberculum sellae and planum sphenoidale, aggressive resection in the intrasellar region and medial wall of the cavernous sinus is not feasible compared to improving tumour visualization by drilling the tuberculum sellae and planum sphenoidale. In a report of drilling the sellar tuberculum and sphenoid planum, drilling allowed the direct visualization of tumours invading the intrasellar region and medial wall of the cavernous sinus. Reconstructing the opening of the sellar-sphenoid cavity is achieved by microsuturing a piece of the pericranium/dura around the dural edge of the defective dura of the open sphenoid sinus and sellar cavity to prevent cerebrospinal fluid (CSF) leakage.Patients and methodsThe FBIA with drilling of the tuberculum sellae and planum sphenoidale was performed to remove the tumours that invaded the intrasellar region and cavernous sinus in 55 patients from January 2014 to October 2019 at our institution. The pre- and postoperative pituitary hormone levels and vision were evaluated as effective standards after surgery and compared using paired t-tests. The different rates of CSF leakage between the packing and microsuture groups were compared by χ² test, p < 0.05.ResultsIn all patients with a mean 37-month follow-up (range, 3–2 months), 43 (78.2%) patients returned to their normal life or school independently, 7 (12.7%) patients were able to perform normal activities with minor complaints or effort, and 4 (7.3%) patients could care for themselves or only required occasional assistance. One (1.8%) death occurred, attributed to CSF leak-related meningitis at 5 months after surgery. Postoperative CSF leakage occurred in eight (19.0%) of 42 patients with packed bone wax or pieces of muscle to the sphenoid sinus. Of 13 patients with a piece of the periosteum/dura microsutured around the defective dura of the sellar region and open sphenoid sinus, one (7.7%) of 13 patients experienced CSF leakage in the perioperative period. With statistical analysis, there was a potential risk for postoperative CSF leakage in the bone wax and muscle piece in the open sphenoid sinus, whereas microsuture manoeuvres were effective for avoiding the risk of postoperative CSF leakage (χ² = 8.865, p < 0.005). The microsutures closed the open sphenoid sinus such that it was water-tight. Postoperative visual acuity and the visual field were not affected by the increased intrasellar exposure or the open sphenoid sinus achieved by drilling the tuberculum sellae and planum sphenoidale.ConclusionTuberculum sellae/planum sphenoidale drilling via FBIA is feasible to enhance the direct visualization of CP resection, which expands the intrasellar region with a direct resection of recurrent tumours in the sellar cavity and adhering to the medial wall of the cavernous sinus. The potential risk of a CSF leakage seemed to be mitigated when using water-tight microsutures on a piece of the pericranium/dura around the edge of the defective dura in the sellar region and the open sphenoid sinus cavity.     

Endonasal endoscopic resection of suprasellar craniopharyngioma: A retrospective single-center case series

IntroductionEndoscopic endonasal approach (EEA) has recently been proposed as an option for resection of primary and recurrent suprasellar craniopharyngioma. However, surgical outcome has not yet been fully evaluated, especially in regards to recurrent cases.MethodsWe analysed our institution (Sir Charles Gairdner University Hospital, Perth, Australia) case-series retrospectively. There were 16 patients operated through an endonasal endoscopic approach from February 2014 to February 2019 for suprasellar craniopharyngiomas. There were 14 primary, and two recurrent lesions. Extent of resection, complications, visual and endocrinological outcomes are presented.ResultsMean age of the patients was 42.9 ± 19.3 years old, with 56% female. The most common clinical symptoms were headaches (9 patients, 56%) and bi-temporal hemianopsia (9 patients, 56%), followed by unilateral optic neuropathy (5 cases, 31%), memory loss (1 case, 6%), hydrocephalus (1 case, 6%), delayed growth and puberty (1 case, 6%), and secondary amenorrhoea (1 case, 6%). Only two cases (12%) initially presented with normal visual function. Gross total resection (GTR) was achieved in 10/16 patients (62.5%), with subtotal resection (STR) in the remainder. Visual symptoms improved in 13/16 patients (81%) and remained unchanged in 3/16 patients (19%). Most common complications included new endocrinological deficit in nine patients (56%), mostly diabetes insipidus, and cerebrospinal fluid leak requiring a new intervention in three patients (19%). There was one mortality case (complicated meningitis, stroke and vasospasm). Mean follow-up time was 22.05 ± 14 months and three patients (19%) had a recurrence of the disease during this period and were referred for radiation therapy.ConclusionEndonasal endoscopic approach is a safe and effective surgical option for both primary and recurrent suprasellar craniopharyngiomas.     

Transsphenoidal surgery

Transsphenoidal surgery has evolved much over nearly 100 years. Initially operations were performed often without any input from endocrinology colleagues, and without preoperative imaging, operative magnification and illumination. Advances in the understanding of the biology of pituitary tumours, close co-operation between endocrinologists, surgeons and oncologists, and huge advances in imaging and surgical techniques have led to the evolution of the current transsphenoidal operation to the pituitary fossa to the point where a ‘cure’ is often possible with low complication rates. The indications, contraindications of transsphenoidal surgery will be discussed, together with nature of the surgical approach and how it can be applied to particular pituitary tumours and suprasellar lesions.     

Hypothalamic obesity in children

Hypothalamic obesity is an intractable form of obesity syndrome that was initially described in patients with hypothalamic tumours and surgical damage. However, this definition is now expanded to include obesity developing after a variety of insults, including intracranial infections, infiltrations, trauma, vascular problems and hydrocephalus, in addition to acquired or congenital functional defects in central energy homeostasis in children with the so‐called common obesity. The pathogenetic mechanisms underlying hypothalamic obesity are complex and multifactorial. Weight gain results from damage to the ventromedial hypothalamus, which leads, variously, to hyperphagia, a low‐resting metabolic rate; autonomic imbalance; growth hormone‐, gonadotropins and thyroid‐stimulating hormone deficiency; hypomobility; and insomnia. Hypothalamic obesity did not receive enough attention, as evidenced by rarity of studies in this group of patients. A satellite symposium was held during the European Congress of Obesity in May 2011, in Istanbul, Turkey, to discuss recent developments and concepts regarding pathophysiology and management of hypothalamic obesity in children. An international group of leading researchers presented certain aspects of the problem. This paper summarizes the highlights of this symposium. Understanding the central role of the hypothalamus in the regulation of feeding and energy metabolism will help us gain insights into the pathogenesis and management of common obesity.     

前纵裂入路显微手术切除鞍上脑室内外型颅咽管瘤

目的 探讨前纵裂入路在鞍上脑室内外型颅咽管瘤显微手术切除中的应用,并比较与其他手术入路的优缺点.方法 回顾性分析22例采用前纵裂入路显微手术切除鞍上脑室内外型大型颅咽管瘤患者,分析手术技巧及术后效果.结果 肿瘤全切20例,次全切除2例,无手术死亡病例.术后发生电解质紊乱15例,尿量增多17例,2-7周后好转,术后1周内视力好转15例.随访6-60个月,肿瘤复发2例,均为12个月内复发.结论 前纵裂入路可直视下处理肿瘤在垂体漏斗部位的粘连,是鞍上脑室内外型颅咽管瘤较好的手术入路.