颅咽管瘤术后并发尿崩症和下肢深静脉血栓一例并文献复习

颅咽管瘤是鞍区常见的先天性良性肿瘤,占颅内肿瘤的2%～5%[1],约55%的病例发生在20岁之前[2]。因肿瘤毗邻下丘脑、垂体等重要神经组织结构,其本身压迫、侵犯或手术损伤易导致患者出现内分泌功能障碍及水电解质平衡紊乱。中枢性尿崩症是颅咽管瘤术后常见的并发症,患病率可高达86.8%[3]。深静脉血栓(DVT)也是神经系统术后常见并发症之一[4],而国内尚无颅咽管瘤术后DVT与中枢性尿崩症相关     

颅咽管瘤切除术中垂体柄和下丘脑损伤的防治体会

目的：探讨颅咽管瘤手术中避免垂体柄和下丘脑损伤的方法。方法：对12例经手术治疗的颅咽管瘤患者进行回顾性分析。结果：9例患者翼点入路,2例患者胼胝体-穹隆间入路,1例经额底入路。术后并发硬膜外血肿死亡1例。无病例死于垂体柄和下丘脑损伤。结论：选择恰当的手术入路,显微外科严格沿肿瘤分离、辨认垂体柄和下丘脑结构以及保留穿通动脉是避免颅咽管瘤手术垂体柄和下丘脑损伤的关键。     

颅咽管瘤围手术期护理

颅咽管瘤是最常见的先天性颅内肿瘤 ,占先天性颅内肿瘤的 6 0 % ,颅咽管瘤虽为良性肿瘤 ,但肿瘤周围有下丘脑垂体柄、视神经等重要结构 ,故肿瘤切除术后并发症多且变化快 ,术后早期发现及防治并发症是降低病死率的重要环节 ,因此肿瘤切除术后的护理极为重要 ,现将护理体会总结如下。1　临床资料1.1　一般资料　我科自 1997年 6月～ 2 0 0 2年 6月共收治颅咽管瘤 2 6例 ,其中男性 15例 ,女性 11例 ,年龄最大 70岁 ,最小 14岁。1.2　临床表现1.2 .1　内分泌功能障碍　由于肿瘤侵犯垂体及下丘脑 ,病人常表现生长发育和性功能障碍 ,出现尿崩、肥…     

翼点入路显微手术切除鞍区颅咽管瘤15例

目的:探讨显微手术切除鞍区颅咽管瘤的有效方法及术后并发症的预防.方法:回顾性分析15例鞍区颅咽管瘤病人,均行翼点入路显微手术切除肿瘤.结果:8例达到肿瘤全切除,4例近全切,3例部分切除.术中垂体柄保留5例,术中断裂1例,术中未见垂体柄9例.结论:经翼点入路是切除鞍区颅咽管瘤的有效方法,利用显微外科技术,保护下丘脑结构和功能,对降低术后并发症起重要作用.     

颅咽管肿瘤的透射电镜观察

颅咽管瘤是胚胎期颅咽囊残留上皮发生的肿瘤,占颅内肿瘤的5%~8%,多位于蝶鞍内,有的位于蝶鞍外颅咽管各部位,肿瘤大小不一,呈实性或囊性,可压迫视神经、垂体或下丘脑,引起偏盲、内分泌障碍等症状[1]。近年来,我们对22例脑部肿瘤患者的肿瘤组织进行透射电镜观察,旨在探讨颅咽管瘤     

鞍区肿瘤术后观察及护理

鞍区肿瘤包括垂体肿瘤、颅咽管瘤、鞍结节脑膜瘤、异位松果体肿瘤 ,常需手术治疗。鞍区含有视神经、颈内动脉、丘脑下部等重要结构 ,术后可发生昏迷、高热、尿崩症、视力障碍等并发症 ,故术后病情观察及护理应视为手术成功的关键步骤之一。现结合我科 1995～ 1999年对 118例鞍区肿瘤患者的术后观察及护理总结报告如下。1　临床资料本组 118例 ,男 6 8例 ,女 5 0例 ,年龄 2 2～ 74岁 ,平均 48岁 ,垂体肿瘤 80例 ,颅咽管瘤 2 2例 ,鞍结节脑膜瘤 16例。术后发生中枢性高热 3例 ,尿崩症 12例 ,视力障碍、偏瘫 3例 ,血管痉挛性脑水肿 4例 ,其中 2…     

颅咽管瘤的MRI诊断

目的 探讨颅咽管瘤的MRI影像学表现,评价其诊断价值.方法 回顾性分析59例经手术病理证实的颅咽管瘤的MRI表现.结果 59例颅咽管瘤患者,40例(67.8%)病灶位于鞍上,16侧(27.1%)位于鞍内,3例(5.1%)位于鞍旁,视神经受压、变形、移位51例,垂体正常显示32倒,垂体受压变小15例,垂体未显示12例.肿瘤的信号在T2WI上多呈混杂高信号,而T1WI上可有高、等、低等多种表现.结论 MRI可清楚显示鞍区肿瘤与周围结构,其多信号变化反映了肿瘤囊液成分的不同,MRI是诊断颅咽管瘤的有效方法.     

颅咽管瘤显微手术中下丘脑功能损害的防治

颅咽管瘤是起源于Rathke囊残存物的一种良性肿瘤,一般发生在垂体柄、漏斗、乳头体、灰结节、垂体前叶及第三脑室前部．约占颅内肿瘤的2％～5％,占儿童脑肿瘤的10％。颅咽管瘤治疗的关键是在减少副损伤的前提下,尽可能全切肿瘤,其中下丘脑功能损害的防治是重要环节。本文系统回顾了2001年1月～2006年3月间我科收治的62例颅咽管瘤显微手术的情况,重点讨论了下丘脑功能损害的围手术期处理。     

儿童中枢性尿崩症影像表现与临床相关性探讨

目的：分析儿童中枢性尿崩症的脑部影像表现,并探讨临床相关性。方法：复习36例儿童中枢性尿崩症的临床资料,对其CT或（和）MR／检查的脑部影像表现及临床进行回顾性分析。结果：生殖细胞瘤6例。颅咽管瘤5例,下丘脑胶质瘤2例,垂体腺瘤2例,郎罕氏细胞组织增生症5例;28例行MRI检查病例中,除了2例具有正常垂体后叶T1加权像高信号外,26例垂体后叶T1加权像高信号均消失,其中垂体小、发育不良8例,垂体柄异常16例。结论：儿童中枢性尿崩症中继发性尿崩症比例较高,且病因各异,因此对临床确诊的儿童中枢性尿崩症非常有必要做CT或（和）MRI检查。     

下丘脑错构瘤的MRI影像表现

目的 分析总结下丘脑错构瘤的MRI影像特征.方法 回顾性分析经手术、病理证实的下丘脑错构瘤2例.结果 男、女各1例,2例均位于灰结节,呈类球形或椭球形等T1、等T2信号,信号均匀,边界清楚,注入造影剂后无强化.较小1例肿瘤最大径为10 mm×12 mm×13 mm,视交叉略受压,较大1例肿瘤大小为20 mm×18 mm×15 mm,局部略突入鞍内,视交叉明显受压移位.结论 如患者为儿童,临床上表现为性早熟和痴笑样癫痫,影像学上表现为灰结节或乳头体肿块,且有注入造影剂后无强化之典型MRI表现,应诊断为下丘脑错构瘤.本病与颅咽管瘤、垂体瘤、生殖细胞瘤等鞍区肿瘤鉴别不难.     

头颅核磁和肿瘤标志物在儿童颅内生殖细胞瘤诊治的价值

目的 评价头颅核磁共振显像 (MRI)和脑脊液及血清肿瘤标志物动态改变在儿童颅内生殖细胞瘤诊治的价值.方法 总结 2009年1月至2010年12月诊治的5例中枢性尿崩症儿童患者(女性3例、男性2例),全部患者在初诊和随诊时进行头颅MRI增强扫描,并检测垂体前叶激素和脑脊液及血清肿瘤标志物人绒毛膜促性腺激素(hCG)和甲胎蛋白水平.结果 3 例既往未经过检查和治疗,2 例起病时病因未明,就诊于我院时病情加重.起病年龄8岁至12岁1个月,起病至就诊时间1至78个月.全部患者均以多尿、多饮症状起病,除 1 例以外,其余患者均有生长迟滞,第二性征未发育;1 例患者在随访的 2 年内身高生长速度正常,已进入正常青春期发育,但是在颅内肿瘤显著增大后,5 例患者均有垂体前叶功能减退,血浆泌乳素水平升高.3 例分别在起病后18、24和 78 个月出现脑疝.3 例起病时头颅MRI均表现为垂体柄增粗,在随访18-22个月表现为下丘脑-垂体区巨大占位,2 例在起病后1和78个月首次就诊,MRI 示颅内巨大占位;全部患者均有T1加权像垂体后叶高信号的消失.5例患者脑脊液hCG均升高,其中4例血清hCG也相应升高,并且随着肿瘤的增大而升高,放疗后随肿瘤的缩小而下降.只有 1 例脑脊液和血清甲胎蛋白显著升高.结论 诊断为"特发性中枢性尿崩症"的患者必须进行密切的随访来鉴别病因,尤其是合并有垂体前叶激素缺乏时.初诊时MRI表现为正常或单纯垂体柄增粗者,在随访过程中应连续观察头颅 MRI 增强扫描的变化,以尽早诊断出潜在的下丘脑垂体柄病变.推荐在初诊时评价脑脊液 hCG 水平,因为hCG升高可能早于 MRI 阳性表现.     

Ocular manifestations of intracranial germinomas: three cases report and literature review

Intracranial germinomas are malignant neoplasms of gonadal origin, which have some features in terms of age, sex, and clinical manifestations. They mainly occur in children and adolescents. Patients with intracranial germinomas mainly manifest with hypothalamic pituitary axis dysfunction and/or compression syndromes. Visual disturbance is one of the most significant clinical presentations, which is mainly caused by tumor cell infiltration into the optic pathway. In this article, we present three cases of patient with intracranial germinoma to analyze the ocular manifestations. All the three patients presented with endocrine symptoms in the early stage and with visual disturbances (including decreased visual acuity and visual field defects) later. In general, germinoma is diagnosed by its characteristic radiological appearance, supported by tumor markers and/or stereotactic biopsy. However, decisive diagnoses were established when ocular manifestations were presented. A suspicion for germinoma should be considered, when young patients manifest visual disturbances accompanied by endocrine symptoms.

     

One-year-old male with accelerated growth and development

A 1-year-old male child with isosexual central (gonadotropin-dependent) precocious puberty caused by hypothalamic hamartoma is reported. Details of the diagnosis based solely on neuromaging characteristics, and satisfactory results of medical treatment with gonadotropin releasing hormone agonist analogues, are highlighted.     

One�\year�\old male with accelerated growth and development

A 1‐year‐old male child with isosexual central (gonadotropin‐dependent) precocious puberty caused by hypothalamic hamartoma is reported. Details of the diagnosis based solely on neuromaging characteristics, and satisfactory results of medical treatment with gonadotropin releasing hormone agonist analogues, are highlighted.     

雌性性早熟大鼠下丘脑､垂体褪黑素受体表达的变化

目的:通过研究雌性性早熟大鼠血浆褪黑素(melatonin,MT)水平,下丘脑?垂体MT受体MT1?MT2表达,探讨MT及MT受体在性发育启动和性早熟中的作用?方法:正常26日龄雌性SD大鼠40只,随机分为性早熟组?性早熟干预组?生理盐水对照组,后者又分为青春前期组和青春期组?使用N-甲基-DL-天冬氨酸(NMA)建立雌性性早熟大鼠模型,促性腺激素释放激素(GnRH)类似物(曲谱瑞林)干预性早熟,通过酶联免疫法检测各组大鼠夜间血浆MT促黄体生成素(LH)水平,实时定量PCR法检测下丘脑?垂体GnRH?MT1?MT2 mRNA表达?结果:各组大鼠夜间血浆MT水平无明显差异,性早熟组下丘脑及垂体MT1 mRNA表达水平均低于青春前期组,差异具有统计学意义,与正常发育的青春期组比较无明显差异?与性早熟组相比,性早熟干预组下丘脑?垂体MT1 mRNA表达均升高,差异有统计学意义?下丘脑?垂体MT2表达各组间无差异?结论:雌性性早熟大鼠下丘脑?垂体MT1表达减少,GnRH类似物可上调MT1的表达?MT受体在青春期发育中可能起抑制性作用,通过下丘脑?垂体的MT受体表达的减少,减弱对中枢的抑制作用,参与正常青春发育或性早熟过程?     

Cerebral Histiocytosis-X with Endocrine Symptoms

A neuropathologically verified case of histiocytosis-X in a 21-year-old man with endocrine symptoms is presented. The granulomas were confined to the central nervous system and occupied mainly the pituitary stalk and hypothalamus, thus giving us the opportunity to observe the endocrine symptoms caused by such localized lesions.

Diabetes insipidus was one cardinal endocrine symptom and is considered to be caused by a hypothalamic lesion in the supraoptic nuclei or the pathways to the posterior pituitary. In this case the anterior pituitary was not involved by granulomas, but the posterior lobe was atrophic.

Severe hypogonadism and probably also a growth hormone defect were present, probably resulting from pituitary insufficiency secondary to the hypothalamic lesions.

Hypothyroidism was another symptom but in this case was probably due to thyroiditis. Such a finding has previously been observed by two other authors and may therefore be pathologically connected to histiocytosis-X even though no granulomas were present in the thyroid gland.

Repeated X-ray investigations from 9 years of age showed that the development of the sella turcica ceased about the same time as the patient got signs of diabetes insipidus. Increase in sellar volume normally reflects pituitary growth. The lesions in the pituitary stalk or the hypothalamus must therefore have been present from the time when diabetes insipidus started.     

Suprasellar Germinoma - Occult Presentation with Hypothalamic Failure 18 Months Before Diagnosis

Hormonal evidence of widespread hypothalamic failure, including diabetes insipidus, hyper-prolactinaemia, growth failure, hypothyroidism, and adrenal failure, was present in a boy of 14 years. Investigation with computerised axial tomography (CT) and metrizamide encephalography failed to identify any anatomical abnormality at the time of presentation. However, 18 months later papilloedema was noted at which time a suprasellar mass and gross dilatation of the ventricular system were readily identified using CT and a germinoma was subsequently diagnosed and treated. In the light of this experience we recommend that when “idiopathic” hypothalamic failure is diagnosed, imaging procedures, including CT or nuclear magnetic resonance examinations, should be repeated on at least one occasion, after an interval of approximately 6 months.     

鞍区生殖细胞瘤合并妊娠病例报告并文献综述

目的分析妊娠合并鞍区生殖细胞瘤的关系,并探讨鞍区生殖细胞瘤的影像特征。方法对1例鞍区生殖细胞瘤孕妇患者行MRI检查误诊为垂体大腺瘤,结合病理及免疫组化诊断为鞍区生殖细胞瘤,总结鞍区生殖细胞瘤的影像诊断特点,并与鞍区其它肿瘤（垂体大腺瘤、脑膜瘤）进行对比研究,并对妊娠合并生殖细胞瘤相关文献回顾分析。结果本例鞍区生殖细胞瘤发生于孕37周的孕妇,MRI影像于垂体窝内见一等T1稍长T2软组织信号肿块,增强肿块明显不均匀强化,病灶突向鞍上池,双侧海绵窦受压,蝶鞍扩大。患者急诊行剖宫产术,3天后急诊全麻下行内镜经鼻蝶肿瘤切除术,标本行病理及免疫组化检查,诊断为鞍区生殖细胞瘤。结论妊娠合并鞍区生殖细胞瘤非常罕见,其影像表现容易误诊为垂体大腺瘤及脑膜瘤,需结合临床表现及影像学特点综合诊断。     

痴笑、性早熟与先天性下丘脑错构瘤

为探讨人下丘脑错构瘤的超微病理特点及其与临床症状、体征的关系 ,将 1 4例下丘脑错构瘤患者的手术切除新鲜活检组织 ,在光学显微镜及电子显微镜下检查。结果 :光镜下可见散在大小不等的神经细胞 ,部分病例胶质细胞增生。电镜检查可见神经细胞形态不一 ,胞核相对较大 ,胞质内细胞器丰富 ,可见少量致密核心颗粒 ;间质内见神经毡结构 ,细胞突起终末有突触形成并有大量兴奋性神经递质小泡。超微结构证实下丘脑错构瘤由神经上皮成分构成 ,可见发育不成熟的神经元并有胶质细胞增生。细胞突起内见神经递质小泡及致密核心的神经分泌颗粒 ,这些形态特点与患儿的性早熟及痴笑样癫痫有关。结果提示电镜是诊断下丘脑错构瘤的重要手段     

ISOSEXUAL PRECOCIOUS PUBERTY CLINICAL ALALYSIS OF 109 PATIENTS

We treated 109 girls with isosexual precocious puberty with true precocious puberty in 63 and pseudoprecocious in 46. The true and pseudopreco- cious puberties were classified etiologically and dif- ferentiated according to their clinical features. Re suIts of treatment showed that chlormadinone was effective in controlling menstruation and secondary sex characters in 43 patients with true precocious puberty except 2 who were more than 8 years old, and that this agent has no effects on physical de- vdlopment including the final height.