内镜辅助的乙状窦前-迷路后锁孔手术入路的解剖学研究

目的:研究内窥镜辅助下乙状窦前-迷路后锁孔手术入路的相关解剖,为临床应用提供参考。方法:取15具30侧经福尔马林固定成人头颅标本,模拟乙状窦前迷路后手术,内窥镜下观察颅内相关解剖,测量入路相关数据。结果:岩上窦-乙状窦交点至Meckel’s囊、Dorelle’s管、内耳门、舌咽神经、舌下神经、椎动脉的距离分别为(38.41±2.49)mm(31.18～45.14mm),(48.65±2.94)mm(44.78～56.78mm),(27.32±2.55)mm(23.76～33.00mm),(31.16±3.20)mm(22.38～36.90mm),(43.71±2.54)mm(39.64～49.46mm),(48.10±2.90)mm(42.20～54.56mm)。内窥镜经小脑外侧间隙可顺利到达桥小脑角和脑干腹侧前方,清晰显示颅神经及其附近走行的血管。结论:内窥镜在桥小脑角的应用可弥补手术显微镜的不足,对显微外科手术起到重要的辅助作用。     

乙状窦前迷路后入路中后骨半规管定位与保护的应用解剖

目的:为乙状窦前入路中安全磨出半规管提供解剖学资料。方法:经10%福尔马林溶液固定的成人尸头标本15个共30侧。磨除Trautman三角的骨质,暴露乙状窦、岩上窦、岩上窦-乙状窦交点、上骨半规管及面神经垂直段、颈静脉球,测量后骨半规管与毗邻结构的距离。结果:后骨半规管与硬化型的乳突对后骨半规管的安全磨出影响较大。鼓室窦存在率100%。后骨半规管的定位:骨质的改变;乙状窦前含乳突小房的松质骨中出现致密的象牙骨质提示接近后骨半规管;颅后窝硬膜皱褶自乙状窦向深层汇集点,提示接近后骨半规管;鼓室窦暴露后,通过外骨半规管定位后骨半规管。结论:鼓室窦可以定位外骨半规管,继而定位后骨半规管;明确毗邻的结构关系可以为安全磨出后骨半规管提供保证。     

乙状窦前入路迷路后区应用解剖学研究

目的：为临床乙状窦前入路迷路后区域手术操作提供解剖学资料。方法：经10％甲醛溶液固定成人尸头标本15具共30侧。在外耳道后上棘、乳突上嵴、乳突尖之间磨除骨质，暴露乙状窦、岩上窦、岩上窦-乙状窦交点、颅中窝硬膜、乙状窦前方的颅后窝硬膜。磨出后骨半规管、外骨半规管、上骨半规管及面神经垂直段、颈静脉球。测量相关数据。结果：乙状窦前入路迷路后区域的骨质可分为三层：表面骨皮质，乳突蜂房骨质，覆盖深部结构的坚硬骨质。外耳道后上棘至后骨半规管的最近距离为（17．88±1．27）mm；面神经垂直段在外半规管下方、后半规管的前方走向二腹肌嵴前缘的内侧，其长度为（7．58～14．02）mm；外耳道后上棘．乙状窦最短距离为（13．84±2．74）mm。结论：此区域骨质分层明显的解剖学特点及测量的数据可以指导手术操作，避免损伤重要结构，为乙状窦前入路迷路后锁孔入路提供了重要的解剖学资料。     

内淋巴囊的应用解剖学

本文观察了60侧(左36,右24)正常成人的内淋巴囊,位于颞骨岩部后面的内淋巴囊压迹处,大小约9.2×10.0mm;另外还注意了内淋巴囊与周围结构(特别是乙状)的毗邻关系,从而提出手术入路的新设想,供临床应用作参考。     

经颞骨岩部乙状窦前入路处理岩斜区病变的应用解剖

目的 :研究经颞骨岩部乙状窦前入路进行岩斜区直接手术的方法 ,寻找出该入路的标志点和颞骨岩部的磨除方法 ,并观察手术的暴露范围和优缺点。方法 :用 15例血管经彩色乳胶灌注的成人尸头标本 ,显微镜下 (× 6～ 2 5 )解剖经颞骨岩部乙状窦前入路。结果 :岩乙状窦交叉点、内淋巴囊裂是磨除颞骨岩部后面的重要标志 ,用内淋巴囊裂可以初步定位前庭小管、总脚和后半规管。此入路能够暴露从鞍背到延髓中上部的区域 ,以暴露中岩斜区最佳。离断内淋巴囊 ,可扩大骨磨除的面积 ,增加手术的暴露。结论 :以岩乙状窦交叉点、内淋巴囊裂为标志为安全磨除颞骨岩部后面提供了保证。经颞骨岩部乙状窦前入路具有手术距离短、视野暴露充分的特点 ,特别适用于骑跨颅中、后窝而以颅后窝为主的岩斜区病变     

内淋巴囊的显微外科解剖

目的 :为后颅窝手术保护听力提供解剖学基础。方法 :2 0例甲醛固定后的成人头颅 (4 0侧 ) ,游标卡尺和量规测量内淋巴囊壁与表面标志的距离。结果 :内淋巴囊与内听道口后缘的平均距离为 1 1 6± 0 .1 4cm ,与XI神经后缘的距离 1 3 8± 0 .1 7cm ,岩嵴与乙状窦前缘相交点的平均距离为 1 67± 0 .3 5cm ,与岩嵴平均距离为 0 1 6± 0 .2 8cm。结论 :本文结果有助于后颅窝手术定位内淋巴囊 ,保留听力     

枕下乙状窦后入路和远外侧入路的比较研究

目的为临床选择合适的手术入路提供解剖依据。方法应用成人头部湿标本10例（20侧），动、静脉系统分别灌注混有红、蓝色染料的乳胶；分别模拟枕下乙状窦后入路和远外侧入路的手术操作。逐层解剖、拍照、测量。结果两种入路涉及的重要解剖结构有：Ⅴ～Ⅻ对脑神经、内耳道、枕髁、颈静脉结节、颈静脉突。结论应根据病变的特点选用合适的手术入路。     

大鼠内淋巴囊的解剖学与组织学观察

目的 研究大鼠内淋巴囊的解剖学与组织学特征。方法 选用健康成年SD大鼠，处死后取双侧颞骨，先观察颞骨内侧面的解剖结构、内淋巴囊的位置及毗邻关系，然后将标本固定、脱钙、石蜡包埋切片，HE染色，观察内淋巴囊的形态和组织结构；再用抗大鼠IgG、CD3单克隆抗体，应用免疫组织化学技术，观察免疫活性细胞在内淋巴囊组织的定位分布。结果 大鼠内淋巴囊的上皮类型以单层立方上皮和扁平上皮为主；内淋巴囊上皮层、上皮下及囊周组织有淋巴细胞，CD3、IgG免疫反应阳性淋巴细胞主要存在于内淋巴囊的上皮层。结论 大鼠内淋巴囊具有与人和豚鼠内淋巴囊基本相似的形态结构；组织中存在CD3、IgG免疫活性细胞，提示其在内耳免疫应答过程中可能发挥重要作用。     

基于解剖学研究的枕下乙状窦后入路精准开颅技术探讨

目的探索应用颅骨表面解剖标志指导枕下乙状窦后入路安全、精准、快速开颅技术。方法从2016年4月至2019年6月选取15具颅骨标本和8具尸头标本,定位颅骨标本解剖标志点:二腹肌沟顶点、乳突尖、星点及关键点(横窦-乙状窦移形处相对应颅外标志点),研究颅骨解剖标志点间的关系,制定开颅技术,并将该开颅技术应用于28例患者,通过评估相关指标,评价此开颅技术的可行性。结果(1)解剖研究及尸头验证:关键点与二腹肌沟顶点距离左侧为(16.79±3.50) mm、右侧为(14.82±2.96) mm,关键点与星点距离左侧为(19.53±3.84) mm、右侧为(22.59±4.08) mm,关键点与乳突尖的距离左侧为(33.98±3.87) mm、右侧为(32.78±3.29) mm,关键点与星点的距离左、右侧间差异有统计学意义(P=0.020)。将开颅技术应用于8例尸头标本,均未出现静脉窦损伤。(2)临床应用:28例患者进行开颅技术验证,23例横窦乙状窦暴露充分,无钻孔所致静脉窦破损,平均开颅时间为(23.1±2.2) min,骨瓣1.8 cm×2.0cm,骨窗直径2.0～2.5 cm,术中骨瓣复位。...     

Innate immune defense in the inner ear – mucines are expressed by the human endolymphatic sac

The human endolymphatic sac has been shown recently to have immunological capacities and has thus been proposed as the main entity protecting the inner ear from pathogen invasion, equivalent to mucosa‐associated lymphoid tissue (MALT). Although the sac expresses molecules of the innate immune system, the potential expression of members of the important mucin family has not been detailed. Thus, this paper explores endolymphatic sac expression of a number of mucins and mucin precursors. Twelve fresh tissue samples from the human endolymphatic sac were obtained during translabyrinthine surgery. The expression of Mucin 1, 2, 5B/AC and 16, as well as the core structure elements (mucin precursors) T‐antigen, Tn‐antigen and Sialyl‐Tn‐antigen was investigated by immunohistochemistry. The endolymphatic sac epithelium expressed MUC1 (both apically towards the endolymphatic sac (ES) lumen and basally towards the capillary network), MUC 16 and Tn‐antigen. There was no labeling after incubation with antibodies against T‐antigen, sialyl‐Tn‐antigen, MUC2 and MUC5B/AC. We conclude that the human endolymphatic sac epithelium expresses a number of mucin molecules, which supports the hypothesis of the sac as the primary immunological tissue structure of the inner ear, equivalent to MALT in other organs. The mucins may also play a role in the formation and continuous homeostasis of the inner ear fluids, as well as the pathogenesis of Meniere's disease.     

Cerebrospinal fluid cytology of an endolymphatic sac tumor

Endolymphatic sac tumor (ELST) is a rare neoplasm which is seldom evaluated by cytopathology. We report the clinicopathologic course and cytologic cerebrospinal fluid (CSF) findings in a 58‐year‐old patient with brainstem lesions who originally presented with vertigo but progressed to having left 7th, 8th, 9th, and 10th cranial nerve palsies, right‐sided weakness, and occipital headaches. Cytospin of the CSF revealed large epithelioid cells similar to cells seen in a surgical resection of a brain mass three months previously. Review of the surgical specimen revealed a well‐differentiated glandular and papillary neoplasm, most consistent with an endolymphatic sac tumor. Diagn. Cytopathol. 2015;43:339–342. © 2014 Wiley Periodicals, Inc.     

内淋巴囊肿瘤2例临床病理特征及误诊原因分析

目的探讨内淋巴囊肿瘤(endolymphatic sac tumor,ELST)的临床及病理形态特点,诊断及鉴别诊断要点,提高对其认识。方法收集2例分别被误诊为脉络丛乳头状瘤及生乳头状汗腺瘤的ELST的临床资料,重新切片行HE染色及免疫组化标记。结果 2例均为男性,以进展性耳聋,外耳道流血或流脓为主要症状。影像学提示颞骨岩部囊实性占位并周围骨质破坏。病理形态特征:肿瘤呈弥漫囊性乳头状结构,乳头表面被覆单层立方或高柱状上皮细胞,轴心为纤维血管组织;部分区域乳头间纤维间质中见腺体结构并囊性扩张,腺腔内可见均质红染的胶样物质。间质纤维组织增生、玻璃样变,见含铁血黄素沉积及胆固醇结晶。免疫组化结果示2例病变肿瘤细胞均表达CK、EMA、CK7、CK19、NSE、CD56和vimentin;其中1例S-100阳性;而GFAP、TTF-1、CEA、TG、Galectin-3、CD10、Syn及CgA均阴性,Ki-67增殖指数低。结论 ELST为罕见的低度恶性肿瘤,临床及病理学特征均易与其他肿瘤混淆。病理组织学形态及免疫组化标记是诊断及鉴别诊断的依据,同时需结合患者特殊发病部位。     

Clinicopathological features of low-grade malignant endolymphatic sac tumors

Background

Low-grade malignant endolymphatic sac tumor (ELST) is a rare neoplasm, occurring in the inner ear and invading the temporal bone. This study aims to investigate the clinicopathological features of low-grade malignant ELSTs.

桥脑小脑角区内淋巴囊肿瘤五例临床病理分析及鉴别诊断

目的 探讨内淋巴囊肿瘤(ELST)的临床病理学特征、免疫表型及其诊断与鉴别诊断。方法 分析5例ELST的临床和病理形态学特点,以EnVision二步法用波形蛋白、角蛋白(AE1/AE3)、CK8/18、CK5/6、上皮细胞膜抗原(EMA)、胶质纤维酸性蛋白(GFAP)、S-100蛋白、突触素、癌胚抗原(CEA)、甲状腺转录因子-1（TTF-1）、血管内皮生长因子(VEGF)、D2-40、Calponin、Calretinin、Ki-67等抗体进行免疫组织化学标记,取2例尸检正常内淋巴囊及8例桥脑小脑角区脉络丛乳头状瘤做对比观察。结果 ELST患者发病年龄23 ～ 35岁,男性2例,女性3例。首发症状有耳鸣、耳痛、听力下降、耳出血及流液,2例伴有头痛。病史半年至10年,其中3例有复发。影像学检查肿物位于桥脑小脑角区,伴有岩骨骨质广泛破坏。组织学改变：肿瘤呈乳头状及腺样结构,乳头被覆瘤细胞单层排列,瘤细胞界限清楚,胞质嗜酸或透明,胞核异型性不大,居中或偏向于腔面,可见扩张的腺样结构,其内有嗜酸性的胶样物质,纤维性间质中血管丰富,小血管紧贴于上皮下,5例均有硬膜或骨质内浸润。免疫组织化学标记：5例ELST的AE1/AE3、CK8/18、CK5/6及VEGF阳性,4例EMA阳性,3例Calponin局灶阳性,2例S-100蛋白局灶阳性,2例波形蛋白阳性,1例GFAP局灶阳性,1例突触素局灶弱阳性,5例CEA、TTF-1、D2-40、Calretinin均为阴性,5例CD34血管阳性,5例Ki-67阳性指数均＜1％。8例脉络丛乳头状瘤的突触素均为阳性,7例S-100蛋白阳性,2例GFAP阳性,1例D2-40阳性,4例AE1/AE3阳性,5例CK8/18阳性,8例EMA、CK5/6及Calponin均为阴性。结论 ELST很少见,其易侵袭骨质,有复发倾向,WHO中枢神经系统肿瘤分类为低度恶性,其最应与桥脑小脑角区的脉络丛乳头状瘤进行鉴别。不同的临床影像特点、组织形态及免疫组织化学染色可以鉴别。     

Endolymphatic sac tumours

This review article surveys clinical and pathological literature on endolymphatic sac tumours (ELST) and summarizes characteristics that describe the entity.
ELST are rare neuroectodermal neoplasms in the petrous bone, originating from inner ear structures. They can be encountered sporadically or in von Hippel–Lindau disease. The most prominent symptom is sensorineural deafness. Historically, nomenclature of invasive adenoid tumours in the petrous bone has been divergent, the term papillary adenocarcinoma used most frequently. Histologically, they have a follicular or papillary and adenoid pattern that can be easily confused with various other neoplastic conditions including metastatic carcinoma. It remains to be verified whether similar tumours (papillary adenocarcinomas) can originate from the middle ear. Middle ear adenomas have a similar appearance but probably originate from neural crest cells in the middle ear. ELST can express a variety of epitopes (including cytokeratin and neuro-ectodermal markers) which can be detected immunohistochemically.
In cases of von Hippel–Lindau disease the cerebello-pontine angle should be included in routine radiological examinations to detect ELST before the tumours lead to deafness. In apparently sporadic cases of ELST, genetic testing for von Hippel–Lindau disease should be considered. Correct distinction of ELST from metastatic carcinoma prevents futile searches for unknown primary tumours.     

The effect of anti-diuretic hormone on the endolymphatic sac of the inner ear

 The anti-diuretic hormone vasopressin (AVP) regulates water excretion from the kidney by increasing the water permeability of the collecting duct. AVP binds to V₂-receptors and induces the translocation of aquaporin-2 water channels (AQP-2) into the apical plasma membrane of principal cells. By this mechanism AVP controls water reabsorption in the kidney. The effects of AVP on the endolymphatic sac (ES) of the inner ear, which is thought to mediate reabsorption of endolymph, were investigated. Both the V₂-receptor and the AQP-2 water channel were found to be expressed in the ES epithelium. In the ES AVP binds to receptors most probably of the V₂-subtype. Application of AVP to organotypically cultured ES inhibits membrane turnover in ribosomal-rich cells of the ES epithelia, which is thought to mediate translocation of AQP-2 into the surface membrane. This suggests that AVP has contrasting effects in the inner ear and kidney, which may be physiologically useful for maintaining endolymphatic pressure during severe hypovolemia. Animal experiments show that AVP causes endolymphatic hydrops after systemic application to guinea-pigs, which suggests a causal role for the increased AVP levels found in humans suffering from Ménière’s disease. Received: 3 July 1998 / Accepted: 13 July 1998