创伤性内淋巴积水（报告2例及文献复习）

内淋巴积水的病因机制可能涉及自身免疫、遗传、感染、创伤等。我们研究梅尼埃病60例,发现创伤性内淋巴积水2例。例1,33岁,女性,左耳乳突区受拳击伤后发作性眩晕,波动性耳鸣、耳聋、耳胀1年。临床检查提示左耳内淋巴积水。左鼓室内注射庆大霉素8mg眩晕治愈。例2,45岁,女性。左颞骨骨折后间发眩晕及耳鸣3年。临床特征指向内淋巴积水。创伤性内淋巴积水是一个疾病实体。颞骨创伤损害内淋巴系统,导致迟发性内淋巴积水。详细的病史、全面的耳神经学及放射学检查可确定诊断。     

内淋巴积水动物模型的制备

目的为梅尼埃病的实验研究提供可靠的动物模型.方法以豚鼠为实验对象,介绍应用枕后硬脑膜外径路阻塞内淋巴囊制备内淋巴积水动物模型的方法.结果术后4周可见耳蜗各回前庭膜不同程度向前庭阶膨出.结论枕后硬脑膜外径路阻塞内淋巴囊是一种能造成内淋巴积水的造模方法.     

内淋巴积水对豚鼠外周前庭频率感受功能的损伤

目的 探讨内淋巴积水对豚鼠外周前庭频率感受功能的损伤.方法 健康豚鼠40只,随机分为对照组、术后2周组（A组）、术后4周组（B组）、术后8周组（C组）,每组10只.术后组均行右侧内淋巴囊阻塞以建立内淋巴积水动物模型.于相应时间段用冰水试验和旋转试验诱发并记录眼震电图,然后处死相应组别动物,取右侧听泡进行切片并染色.结果 冰水试验时,A组有4只豚鼠（40％）引出眼震,但是眼震较微弱,持续时间较对照组短、眼震速度较慢.其余术后各组均未引出明显眼震.旋转试验时,对照组、A组、B组、C组左、右眼震慢相斜率无明显差异（P＞0.05）.染色结果显示,对照组耳蜗无膜迷路积水表现,而术后各组均出现不同程度膜迷路积水,前庭膜向前庭阶膨出.术后时间越长,积水程度越严重.结论 内淋巴囊阻塞可有效建立膜迷路积水的动物模型.内淋巴积水主要影响前庭器官的低频旋转感受功能.     

内淋巴积水模型豚鼠前庭及耳蜗功能的动态变化

目的观察内淋巴积水模型动物前庭及耳蜗功能的动态变化.方法20只健康豚鼠行右侧内淋巴囊切除及部分内淋巴管阻塞以建立内淋巴积水动物模型,系统观察手术前后豚鼠行为学、眼震电图(ENG),听觉脑干电反应(ABR),畸变产物耳声发射(DPOAE)的变化,最终以形态学方法确认内淋巴积水形成.结果术后未出现前庭功能紊乱的行为学征象,术后第25天、45天、75天时曾出现不对称性眼震反应.术后第15天术耳ABR反应阈值轻度升高,之后随时间的推移逐渐升高,90天之后阈值基本趋于稳定不再有较大的变化.术耳DPOAE反应幅值于术后15天开始下降,以后逐渐降低,90天之后大部分豚鼠DPOAE不能引出.病理切片示前庭膜明显膨隆,以第三、四回为重.结论内淋巴囊切除及部分内淋巴管阻塞方法建立的内淋巴积水模型动物,前庭及耳蜗功能呈现一个动态变化的过程,术后90天功能趋于稳定,可用于进一步的研究.     

别嘌呤醇对内淋巴积水模型豚鼠耳蜗的影响

目的建立豚鼠的内淋巴积水模型,并研究别嘌呤醇对豚鼠内淋巴积水耳蜗的影响.方法采用右侧枕后硬脑膜外进路微创内淋巴囊联合醛固酮腹腔注射制备豚鼠内淋巴积水模型,观察药物别嘌呤醇对豚鼠耳蜗形态和功能的影响.结果模型耳与正常耳比较耳蜗积水程度的差异有显著意义(P＜0.05)ABR反应阈的差异有显著意义(P＜0.05);治疗耳和预防耳比较耳蜗积水程度的差异有显著意义(P＜0.05)ABR反应阈的差异有显著意义(P＜0.05).结论内淋巴积水可以通过右侧枕后硬脑膜外进路微创内淋巴囊联合醛固酮腹腔注射来成功制备.别嘌呤醇在预防和治疗实验型内淋巴积水方面有较为显著的效果,同时可以降低听觉脑干电反应的阈值.(中国眼耳鼻喉科杂志,2006,684～85)     

高清磁共振内耳钆造影技术对膜迷路积水的成像及诊断价值

内耳膜迷路积水被认为与梅尼埃病、突发性耳聋等内耳疾病相关,临床实践中内耳膜迷路的清晰成像是诊断内淋巴积水存在与否的关键。本文结合国内外文献以及笔者团队研究成果,探讨高清磁共振内耳钆造影技术对膜迷路积水的成像及诊断价值。     

眼眶内恶性淋巴上皮病变一例

恶性淋巴上皮病变又称未分化癌伴淋巴样间质，最常发生于鼻咽部，其发病与EB病毒(Epstein-Barr virus)密切相关。发生于泪囊部位的恶性淋巴上皮病变仅见1例报告，该侧首先表现为颈部未分化癌淋巴结转移。笔者曾诊治1例首诊于眼科的原发于泪囊部位的恶性淋巴上皮病变，现报告如下。     

内淋巴囊外科手术应用解剖及其意义

目的研究内淋巴囊的形态、定位等外科手术应用解剖,为内淋巴囊手术提供解剖学基础.方法15例甲醛固定后的成人头颅(30耳),游标卡尺和量规测量内淋巴囊的长度和宽度,内淋巴囊中心、上、下缘与手术时各个标志的距离.结果内淋巴囊的平均长度和宽度分别为8.9±3.2 mm、9.1±2.8 mm,内淋巴囊中心、最高点、最低点至砧骨短脚、面神经垂直段最后缘、外耳道后壁内侧中点、后半规管最上和最下缘、外半规管最上缘的距离分别为15.5±3.2、11.1±2.6、13.3±3.2、16.2±3.8、7.5±2.5、2.1±0.9、14.1±2.5、12.2±2.7、15.2±2.3、14.6±2.4、2.3±0.8、1.8±0.7、17.2±1.8、12.2±2.4、14.3±2.5、17.5±1.6、12.5±2.4、5.2±1.4 mm.结论本文结果有助于乳突入路内淋巴囊减压或引流定位内淋巴囊,避免面瘫和听力损害等并发症的产生.     

儿童单眼眶内原发淋巴造血系统恶性肿瘤1例

患者　女　 7岁　因右眼球突出 5个月于 2 0 0 1年 8月 2 0日入院。 2 0 0 1年 3月无明显诱因发现右眼下睑肿 ,右眼球突出。既往无头面部炎症及外伤史。在当地医院行先锋霉素Ⅵ号及强的松治疗 ,有好转。 2 0 0 1年 5月右眼睑肿右眼球突出加重 ,转入我院治疗 ,无效。视力 :右 1     

Ectopic orbital meningioma: report of two cases and literature review

Ectopic orbital meningioma is a rare tumor usually affecting the medial orbit. We present two cases that occurred in a 56-year-old woman and a 28-year-old man. The tumors in both patients were subtotally excised via orbitotomy surgery and were located in the superior quadrant in one of our patients and in the temporal quadrant in the other. Following histopathologic diagnosis, external beam radiotherapy (EBRT) was administered to one patient and intensity modulated radiotherapy to the other. We identified 12 other well-documented cases of ectopic orbital meningioma previously reported. Ectopic meningioma should be considered in the differential diagnosis of medial as well as lateral and superior orbital tumors. The tumor is usually well circumscribed but can be ill defined in imaging studies. There are intralesional calcifications and sclerosis of adjacent bone in some cases. Ectopic orbital meningioma can recur after incomplete excision. Based on the efficacy of EBRT in optic nerve sheath meningioma, we used this treatment to decrease the risk of recurrence in our two patients and found no tumor recurrence at follow-ups of 24 and 74 months, but one patient had severe vision loss from radiation retinopathy.     

Orbital solitary fibrous tumor: report of two cases and literature review

BACKGROUND: Solitary fibrous tumor (SFT) is a rare, benign, and very uncommon lesion in the orbit. Because of its complex and variable clinical and histological appearance the SFT is often misdiagnosed. CASES: Two new cases of orbital SFT are reported, one in a man and the other in a woman, both unilateral and in the superomedial orbit. OBSERVATIONS: Clinical and tomographical evaluations were conducted and the lesions were excised. The histological evaluation showed the tumors were composed of spindle-shaped cells within collagen bundles and vascular channels. Immunohistochemical staining was positive for CD 34 and negative for S-100 protein. CONCLUSION: Immunohistochemical study is an important adjuvant in determining the SFT diagnosis. Long-term follow-up is necessary because of the possibility of SFT recurrence after excision.     

Recurrent Salzmann's nodular degeneration: report of two cases and review of literature

Recurrence of Salzmann's nodular degeneration following corneal transplantation is uncommon. The earlier reports of recurrence of Salzmann's degeneration were based on clinical evidence. We report two histopathologically proven cases of recurrence of Salzmann's nodular degeneration following keratoplasty; one recurring after lamellar keratoplasty and the other following penetrating keratoplasty. Two male patients (40 yrs and 24 yrs) presented to us with a clinical picture of Salzmann's nodular degeneration. One patient had undergone lamellar keratoplasty in both eyes for Salzmann's degeneration, 22 years ago. A repeat lamellar keratoplasty was performed in the right eye (7.5 mm/8 mm). In the other patient, penetrating keratoplasty was performed in the left eye, 6 years ago for the same. Lamellar keratoplasty was performed in the left eye (8 mm/8 mm). The histopathologic evaluation of the host button in both the cases, showed features very much suggestive of a recurrence of Salzmann's nodular degeneration.     

Congenital cystic eye: report of two cases and review of the literature.

A 13-month-old boy and a 2-week-old girl, who were considered to be anophthalmic and who later each developed a cystic lesion in the left orbit with protrusion of the lower eyelid, were studied. The fellow eye in case 1 was subsequently found to be microphthalmic with cyst and was normal in case 2. Histopathologic study of each case revealed a cyst lined externally by dense fibrous connective tissue to which skeletal muscle and adipose tissue were attached. The inner aspect of the cyst was lined by neuroglial tissue, possible immature retinal tissue, and cuboidal epithelium. No fully developed ocular structures or microphthalmos were identified. Fourteen cases of congenital cystic eye, including our cases, have been published in the English-language literature since 1964. We discuss and illustrate the findings in our cases and 10 others in which histopathologic findings were reported. Congenital cystic eye, microphthalmos with cyst, and microphthalmos with cystic teratoma should be suspected in patients with a small or unrecognizable eye and an orbital cystic mass that is detected by palpation or visualization.     

Bardet-Biedl syndrome: a review of Chinese literature and a report of two cases

Choroideremia (CHM), an X-linked recessive hereditary disease, is an intractable chorioretinal dystrophy. The rate of disease progression of CHM reportedly shows considerable variability. A number of mutations involving the gene that codes for Rab escort protein-1 (REP-1) have been detected in CHM patients. We have analyzed REP-1 gene mutations of Japanese CHM patients. The present study was designed to investigate the clinical variability and the genotype to phenotype relationship in 15 Japanese CHM patients referred to the Department of Ophthalmology of Juntendo University Hospital. The clinical investigation of visual acuity, visual field, color vision and refraction revealed inter-individual variability. Mutation analyses of the REP-1 gene revealed 10 types of mutations in 13 patients from 11 families, including an insertion, small deletions, nonsense mutations and an A to CC mutation. In 13 CHM patients with detectable REP-1 gene mutations, no relationship of genotype to phenotype was detected. At present, we consider the REP-1 genotype to be an unreliable prognostic factor for counseling of CHM patients. In two patients from one family, no mutations were detected in coding regions of the REP-1 gene. These patients may have intron mutations of the REP-1 gene, not detectable by the techniques employed in this study, or other causative genes. Both were observed to have somewhat slower disease progression than the other 13 patients. More advanced analyses are necessary to answer questions regarding the genotype-phenotype relationship in CHM patients.     

Solitary fibrous tumor of the orbit: report of two cases and review of literature

Solitary fibrous tumor (SFT) is a rare mesenchymal neoplasm that most frequently arises in the pleura and uncommonly involves the orbit. There are 54 cases of SFT of the orbit available in the literature. The authors report tow cases of an orbital SFT and discuss the clinical, radiological, pathological features and differential diagnosis. Long-term follow-up of patients is necessary because SFT may recur many years after operation. Complete surgical resection is the most important prognosis factor of this tumor.     

Multifocal intraocular malignant melanoma: report of two cases and review of the literature.

PURPOSE: To describe two eyes from two patients with multifocal primary intraocular melanoma. DESIGN: Two case reports. METHODS: The history and histologic findings in the enucleated eyes of two patients with multifocal intraocular melanoma are described in comparison to previously reported cases. MAIN OUTCOME MEASURES: Pathologic examination of enucleated eyes. RESULTS: One of the two eyes contained mixed cell type melanomas, and one eye contained spindle cell type melanomas. Examination of serial sections showed no continuity between the intraocular melanomas. There were no associated ocular or systemic conditions with the multifocal intraocular melanomas. CONCLUSIONS: Multifocal primary intraocular melanoma is rare. There is no known predisposing factor to this condition.     

Adenoid cystic carcinoma of the lacrimal gland: report of two cases and literature review

The adenoid cystic carcinoma of the lacrimal gland is a rare malignant orbital tumour, which has a generally poor prognosis. The therapy for adenoid cystic carcinoma of the lacrimal gland remains controversial. We present two cases of patients with adenoid cystic carcinoma of the lacrimal gland, who were treated with radical surgery in combination with radiotherapy. The therapeutic results are discussed with reference to relevant literature reports.