颅咽管瘤术后应用甘露醇对水电解质紊乱的影响

目的比较颅咽管瘤术后应用甘露醇对尿崩和电解质紊乱的影响.方法将84例经手术治疗的成人颅咽管瘤病人分为甘露醇组与非甘露醇组,对术后尿量和血钠进行统计学分析.结果颅咽管瘤术后,两组尿量和血钠在统计学上无显著性差异.结论颅咽管瘤术后应用甘露醇并不是影响尿崩和电解质紊乱发生的重要因素,不应该成为治疗的禁忌.     

颅咽管瘤侵袭第三脑室的方式对手术切除的影响

目的 探讨大型颅咽管瘤与第三脑室的关系在肿瘤切除手术中的意义. 方法 南方医科大学南方医院神经外科白1997年1月至2003年1月共采用手术治疗大型颅咽管瘤患者72例.根据肿瘤的影像学表现及手术所见对其进行分类,每一类肿瘤根据大小及其与第三腩室底的关系分为3级,根据术中判断和术后CT和(或)MPd增强扫描结果确定颅咽管瘤手术切除程度,分析肿瘤分级与手术切除程度的关系. 结果颅咽管瘤总体上可以分为第三脑室内型(本组7例)和第三脑室累及型(本组65例)两大类;手术切除程度在不同分级肿瘤间总体分布位置不同,差异有统计学意义(P<0.05). 结论 明确大型颅咽管瘤与第三脑室底及下丘腩结构间的关系对提高大型颅咽管瘤手术疗效有重要意义.     

颅咽管瘤切除术后水钠代谢紊乱机制研究(附102例分析)

目的探讨颅咽管瘤术后水钠代谢紊乱的原因和最佳处理方式.方法对102例经胼胝体切开穹窿间入路切除巨大颅咽管瘤的病人,记录术后尿量、血电解质、抗利尿激素(ADH)、醛固酮(ALD)、皮质醇水平,比较术后激素水平变化与水、钠代谢紊乱的关系.结果本组均出现水、钠代谢紊乱,术后2周完全恢复52例,4周基本恢复33例,6周恢复12例,需长时间人工调整电解质水平5例.术后ADH、ALD和皮质醇的不足是导致术后水钠代谢紊乱的主要原因.结论颅咽管瘤切除术后水、钠代谢紊乱与手术损伤下丘脑有关,紊乱类型与ADH、ALD和醛固酮的缺乏情况有关;及时给予相应激素及对症治疗,可获满意疗效.     

颅咽管瘤组织炎症与术后尿崩关系的探讨

目的研究颅咽管瘤组织炎症与术后尿崩程度的关系。方法对30例经手术治疗的颅咽管瘤病例进行回顾性分析,并采用免疫组化SP法检测毗邻下丘脑的颅咽管瘤组织中白细胞共同抗原CD45,以标记炎性细胞。根据CD45标记的炎性细胞数量将肿瘤炎症分为4级,术后尿崩按发生程度分为轻度尿崩、中度尿崩及重度尿崩,并对它们之间的关系进行分析。结果CD45标记的炎症反应在毗邻第三脑室底部的颅咽管瘤组织中广泛存在,炎症Ⅰ级(0.0%,0/5)和Ⅱ级(0.0%,0/8)患者重度尿崩发生率明显低于(P<0.05)Ⅲ级(25.0%,3/12)和Ⅳ级者(60.0%,3/5)。结论颅咽管瘤组织炎症与术后尿崩程度有关,分析颅咽管瘤组织炎症有助于预测术后尿崩发生的严重程度。     

颅咽管瘤切除术后水钠代谢紊乱机制研究

目的探讨颅咽管瘤术后水钠代谢紊乱的原因和最佳处理方式。方法对102例经胼胝体切开穹窿间入路切除巨大颅咽管瘤的病人，记录术后尿量、血电解质、抗利尿激素（ADH）、醛固酮（ALD）、皮质醇水平，比较术后激素水平变化与水、钠代谢紊乱的关系。结果本组均出现水、钠代谢紊乱，术后2周完全恢复52例，4周基本恢复33例，6周恢复12例，需长时间人工调整电解质水平5例。术后ADH、ALD和皮质醇的不足是导致术后水钠代谢紊乱的主要原因。结论颅咽管瘤切除术后水、钠代谢紊乱与手术损伤下丘脑有关，紊乱类型与ADH、ALD和醛固酮的缺乏情况有关；及时给予相应激素及对症治疗，可获满意疗效。     

鞍区肿瘤术后水钠失衡的临床因素分析

目的探讨影响鞍区肿瘤水钠失衡的临床因素。方法对55例鞍区肿瘤病例进行回顾性分析。结果35例出现一过性多尿,20例出现脑性盐耗综合症无永久性尿崩恶性肿瘤患者水钠失衡发生率为,。100%(7/7),颅咽管瘤37.5%(6/16),垂体瘤27.3%(6/22),脑膜瘤10%(1/10)。结论鞍区肿瘤术后易发生水钠失衡,术后发生率同术前是否有水钠失衡,肿瘤是否侵入第三脑室手术入路的选择及肿瘤切除程度无明显相关同肿瘤的性质及术中是否损伤下丘脑垂体柄有关。     

基于QST分型的颅咽管瘤与第三脑室底脑膜层次的关系

目的探索基于QST分型的、累及第三脑室底的颅咽管瘤与第三脑室底脑膜层次的关系及临床意义。方法回顾性分析2018年1月至2019年10月南方医科大学南方医院神经外科在神经内镜下行肿瘤全切除术治疗累及第三脑室底的原发性颅咽管瘤患者的临床资料(共17例,其中Q型6例,S型3例,T型8例),所有患者术中均留取肿瘤组织标本。正常鞍区标本来源于同期该院行人工引产或自然流产的胎儿(8例)。对以上标本进行苏木素-伊红(HE)和免疫荧光双标染色,应用波形蛋白抗体标记硬脑膜,Ⅰ型胶原蛋白抗体标记蛛网膜,胶质纤维酸性蛋白抗体和层粘连蛋白抗体标记软脑膜,以CK18抗体标记腺垂体,以CK5/6抗体标记颅咽管瘤。观察胎儿脑组织标本的脑膜染色情况以及不同QST分型的颅咽管瘤组织与第三脑室底脑膜的层次关系。结果8例胎儿标本均成功标记硬脑膜、蛛网膜、软脑膜。颅咽管瘤标本HE染色及免疫荧光双标染色结果显示,所有Q型肿瘤(6/6)与第三脑室底之间存在硬脑膜(鞍膈);所有S型肿瘤(3/3)与第三脑室底之间存在蛛网膜和软脑膜;T型肿瘤与第三脑室底之间存在3种病理学形态关系,分别命名为卯榫样、地幔样及护城河样关系,所有T型肿瘤(8/8)与第三脑室底之间有软脑膜分隔,但在肿瘤起源点处,软脑膜可逐渐消失。当肿瘤极度挤压第三脑室空间时,第三脑室室管膜仍可保持完整。结论不同QST分型的颅咽管瘤均可累及第三脑室底,且与第三脑室底之间存在不同的脑膜层次;这些脑膜层次是手术安全切除累及第三脑室底的颅咽管瘤的天然屏障。     

儿童第三脑室室内型颅咽管瘤的显微手术治疗及术后并发症的防治

目的 探讨儿童第三脑室室内型颅咽管瘤显微手术治疗方法及术后并发症的防治方法. 方法 自2005年7月至2011年5月云南省第一人民医院神经外科共收治儿童第三脑室室内型颅咽管瘤患者18例,均采用胼胝体-透明隔-穹窿间入路进行手术切除,现回顾性分析该18例患儿临床资料、治疗效果及术后并发症的防治过程. 结果 肿瘤全切13例,近全切除3例,大部分切除2例.患者症状和体征均得到不同程度的改善.术后主要并发症为:尿崩(15/18)、血电解质紊乱(12/18)、中枢性高热(5/18)和癫痫(4/18),经积极防治后均得到控制,总体治疗效果良好. 结论 经胼胝体-透明隔-穹窿间入路是切除儿童第三脑室室内型颅咽管瘤的理想路径,积极防治术后并发症对改善患者预后意义重大.     

颅咽管瘤切除术后常见并发症的处理

目的 探讨颅咽管瘤切除术后常见并发症的特点和处理。方法 对我科80例颅咽管瘤切除患者的临床资料进行回顾性分析。结果 术后出现尿崩72例，出院时39例尿量恢复正常，仍有尿崩者33例；钠代谢紊乱58例，其中低钠血症31例，高钠血症14例，高钠、低钠交替13例，出院时血钠正常者51例，仍有钠代谢紊乱者7例；高热34例，出院时体温均正常；意识障碍20例，1例因长期昏迷死于循环功能衰竭，出院时11例意识清楚，8例仍有意识障碍；癫痫5例。1例死于癫痫持续状态，余4例得到良好控制。结论 处理尿崩时要合理使用抗利尿制剂。引起低钠的原因有脑性盐耗综合征和抗利尿激素异常分泌综合征，治疗前者应补液、补盐。后者应适当限水、利尿。高钠血症要限钠、补液并给予抗利尿制剂。发生高热应及时降温。出现意识障碍，除做CT检查确定有无颅内血肿和脑积水外。还应从电解质和激素用药方面查找原因。癫痫是颅咽管瘤术后极危险的并发症，要及时控制。避免出现癫痫持续状态。     

儿童颅咽管瘤术后血钠紊乱与癫痫相关性的研究

目的研究儿童颅咽管瘤术后血钠紊乱与癫痫的关系及治疗。方法将107例儿童颅咽管瘤术后血钠状态分为4组。分别统计各组术后癫痫的发生率，用χ^2检验来分析术后癫痫与各术后血钠状态组的关系。结果在总体上，血钠紊乱组与血钠正常组的癫痫发生率有统计学意义（P=0．042）。各组之间比较：交替性血钠异常组与血钠正常组之间有统计学意义（P=0．013）；单纯低钠组与血钠正常组之间有统计学意义（P=0．03）。其他各组之间不具有统计学意义。12次术后癫痫中有8次伴有血钠的波动，8次发生在血钠水平较低时。结论儿童颅咽管瘤术后血钠紊乱者癫痫可能性大，其中以交替性血钠异常者最易发生癫痫。儿童颅咽管瘤术后血钠偏低时是癫痫的易发期。血钠突然波动，尤其是血钠下降更容易引发癫痫发作。     

儿童第三脑室颅咽管瘤术后并发症的防治策略

目的 分析经胼胝体-穹窿间入路显微手术切除儿童第三脑室颅咽管瘤术后并发症并探讨其正确的处理措施.方法 回顾性分析26例经胼胝体-穹窿间入路切除儿童第三脑室颅咽管瘤术后出现的并发症及其处理方法.结果 全组患者术后均出现了不同程度的各种并发症,其中尿崩症24例,高钠高氯血症22例,低钠血症18例,高热12例,脑窜感染3例,癫痢大发作2例.临床痊愈出院16例,好转出院10例,未出现死亡病例.结论 术后严密监测血电解质及尿量、甲状腺功能及血清皮质醇;手术前后补充糖皮质激素、术后补充甲状腺素,积极处理尿崩症和电解质紊乱、高热、脑室感染等并发症,具有与手术本身同样的重要性.     

大型颅咽管瘤的显微手术治疗

目的:结合颅底手术入路提高鞍上、三脑室、脚间池大型颅咽管瘤的手术全切除率,提高疗效。方法:采用不同颅底手术入路显微手术切除37例鞍上、三脑室、脚间池大型颅咽管瘤。结果:肿瘤全切除35例,次全切除2例。术中解剖保留垂体柄31例,所有患者术后伴有不同程度一过性尿崩、动眼神经麻痹10例。全部病例平均随访3年,复发1例。结论:对位于鞍上、三脑室和脚间池的大型颅咽管瘤应根据肿瘤的不同生长方向采用相应的手术入路,充分暴露鞍区各脑池、视神经、前循环及其穿通支和三脑室底部等结构,对脑组织和脑神经牵拉小,术后并发症少且易控制,可取得较好的手术治疗效果。     

Comparison of hypothalamopituitary axis dysfunction of intrasellar and third ventricular craniopharyngiomas in children

In this study, we attempted to determine if different locations of a tumor influence the hypothalamopituitary axis function and outcomes with childhood craniopharyngiomas. The preoperative, postoperative, and long-term follow-up endocrinological disturbances of 66 children with a craniopharyngioma were retrospectively studied. The patients were divided into two subgroups according to the location of the tumor (intrasellar and third ventricle floor). The mean age at onset was 8.02 (range, 1.42-17.58)years. These patients were followed-up for a median duration of 7.2 (range, 2-22)years. Vision problems as the first symptom were more common in Group One (with intrasellar tumors) compared to Group Two (55.6% vs 15.4%; p=0.001; Fisher's exact test). Increased intracranial pressure was the most common initial symptom in patients in Group Two (51.3%) and the second most common symptom in Group One (37%). The majority of patients in both Group One and Group Two required some forms of pituitary hormone supplements (96% vs 84%). At the last follow-up, more patients with intrasellar craniopharyngiomas needed cortisone supplements (79.2% in Group One vs 45.9% in Group Two; p=0.016; Fisher's exact test); however, children with third ventricle floor tumors had more prevalent weight gain (4.2% in Group One vs 27.0% in Group Two; p=0.038; Fisher's exact test). There were different initial presentations and endocrinological outcomes between children with intrasellar and third ventricle floor craniopharyngiomas. The intrasellar tumors had greater pituitary hormone disturbance. However, at the long-term follow-up, children with third ventricle floor tumors had a greater prevalence of being overweight and obese, which was associated with hypothalamic dysfunction.     

Prolonged cerebral salt wasting following craniopharyngioma surgery and posterior reversible encephalopathy syndrome: a case report]

A 9-year-old boy was admitted to our hospital with daytime urinary incontinence for the past one year. MRI showed craniopharyngioma occupying the third ventricle. The tumor was excised by interhemispheric approach. Because hyponatremia and polyuria with high renal loss of sodium were observed on postoperative day 3, hydrocortisone and DDAVP were replaced. On postoperative day 24, successive general convulsions and hyponatremia recurred, and MRI FLAIR imaging showed marked brain edema in the bilateral parieto-occipital lobes. This finding disappeared late in the course of treatment, and the case was diagnosed as posterior reversible encephalopathy syndrome. The pathophysiology of cerebral salt wasting and posterior reversible encephalopathy syndrome in a craniopharyngioma patient are also discussed in the article.     

Premio Sixto Obrador 2013. Modelo topográfico de 3 ejes para el tratamiento quirúrgico de los craneofaringiomas. Parte II: Evidencias anatómicas y neurorradiológicas que definen el modelo de clasificación de 3 ejes y su utilidad para predecir el riesgo quirúrgico individual

Introduction and objectivesThis study evaluates the pathological and magnetic resonance imaging evidence to define the precise topographical relationships of craniopharyngiomas and to classify these lesions according to the risks of hypothalamic injury associated with their removal.Material and methodsAn extensive, systematic analysis of the topographical classification models used in the surgical series of craniopharyngiomas reported in the literature (n = 145 series, 4,588 craniopharyngiomas) was performed. Topographical relationships of well-described operated craniopharyngiomas (n = 224 cases) and of non-operated cases reported in autopsies (n = 201 cases) were also analysed. Finally, preoperative and postoperative magnetic resonance imaging studies displayed in craniopharyngiomas reports (n = 130) were compared to develop a triple-axis model for the topographical classification of these lesions with qualitative information regarding the associated risk of hypothalamic injury.ResultsThe 2 major variables with prognostic value to define the topography of a craniopharyngioma are its position relative to the sellar diaphragm and its degree of invasion of the third ventricle floor. A multivariate diagnostic model including 5 variables –patient age, presence of hydrocephalus and/or psychiatric symptoms, the relative position of the hypothalamus and the mammillary body angle– makes it possible to differentiate suprasellar craniopharyngiomas displacing the third ventricle upwards (pseudointraventricular craniopharyngiomas) from either strictly intraventricular craniopharyngiomas or lesions developing primarily within the third ventricle floor (infundibulo-tuberal or not strictly intraventricular craniopharyngiomas).ConclusionsA triple-axis topographical model for craniopharyngiomas that includes the degree of hypothalamus invasion is useful in planning the surgical approach and degree of resection. Infundibulo-tuberal craniopharyngiomas represent 42% of all cases. These lesions typically show tight, circumferential adhesion to the third ventricle floor, with their removal being associated with a 50% risk of hypothalamic injury. The endoscopically-assisted extended transsphenoidal approach provides a proper view to assess the degree and extension of craniopharyngioma adherence to the hypothalamus.     

Growth and weight of children with craniopharyngiomas based on the tumour location and growth pattern

This study aimed to characterise the weight and growth of children with craniopharyngiomas and to analyse the role of the tumour location and growth pattern in the development of obesity and growth retardation in these children. We retrospectively analysed the records of 109 consecutive children with primary craniopharyngiomas. The patients were divided into two subgroups according to the location of the tumour: intrasellar (Group A); and the floor of the third ventricle (Group B). Height and body mass index were measured at standardised ages and at time points before, after, and at the time of diagnosis. Endocrinological and hypothalamic measurements before and after surgery were compared. Reduced growth rates occurred in early infancy and persisted until diagnosis in Group A, but were only present from age 5–6 in Group B. Therefore, reduced growth rates occur early in the history of intrasellar tumours, whereas rapid postoperative weight gain invariably occurs in patients with third ventricle tumours, which is a significant predictive factor for severe long term obesity in patients with childhood craniopharyngiomas.     

Hyponatremia in central partial diabetes insipidus due to postoperative hypothalamic tumor

We report the case of a 4-year-old boy with a postoperative hypothalamic tumor, who exhibited unusual water and electrolyte disturbance. This developed as a late manifestation during the course of central diabetes insipidus (DI), which started when the patient was 2 years old. Clinically, hyponatremia and DI appeared alternatlely within 1 day. The hyponatremia (lowest value Na⁺ 115 mmol/l) was associated with afebrile convulsions. Assessment of fluid status revealed that the patient had a reduced capacity for arginine vasopressin (AVP) secretion (partial DI), which was not physiologically regulated and which was not concomitantly sufficient to produce maximally concentrated urine and allow the production of maximally diluted urine. This defective osmoreceptor function in association with the previously existing reduced capacity for AVP release seemed to be responsible for the fluid disturbance in the patient. The administration of nasal 1-desamino-8d-arginine vasopressin (DDAVP) only when urine output was increased, instead of regular administration at a fixed time, prevented both worsening of hyponatremia and development of DI.     

Stiletto stabbing: Penetrating injury to the hypothalamus with hyperacute diabetes insipidus

Diabetes insipidus (DI) is a well documented complication observed after traumatic head injuries. We report a case of hyperacute onset DI in a 19-year-old male who sustained a hypothalamic-pituitary injury when he was stabbed in the head with a 30-cm long thin-bladed knife. At CT, our patient showed significant hemorrhagic contusions of the lower hypothalamus. He developed polydipsia, polyuria, and mild hypernatremia in the Emergency Department. Diagnostic digital subtraction angiography showed a hypervascular congestive pituitary gland with prominent draining veins. On the third day his hypernatremia became severe (183 mEq/L). He was managed with parenteral fluids and a regimen of intranasal DDAVP (1-desamino 8-d-arginine vasopressin), leading to improved plasmatic sodium levels, urine output, and urinary specific gravity. In patients presenting with hyperacute posttraumatic DI, emergency room physicians and neurosurgeons should rule out direct injury to the hypothalamus and/or the posterior lobe of the pituitary, and initiate early pharmacological treatment.