儿童中耳先天性胆脂瘤

中耳先天性胆脂瘤最常见于儿童,发病与胚胎时期鼓室内表皮样结构的残留或鼓环抑制功能障碍等因素有关。临床上以渐进性传音性聋及完整鼓膜后白色团块为特征,无鼓膜穿孔及耳手术史。根据病变情况,选择适当手术,可望彻底清除病变,保存患耳听力。     

中耳先天性胆脂瘤二例

中耳先天性胆脂瘤二例赵全义，刘晓梅，纳玉萍有关中耳先天性胆脂瘤的文献国内外均不多见。对此病的诊断及治疗方法尚存在较大争议，本文就收治的２例先天性胆脂瘤进行了讨论。１病例例１女，５岁半，右耳后肿胀２０余天就诊，既往无耳流脓史；右耳后肿胀、稍红，鼓膜完整...     

中耳先天性胆脂瘤1例

<正>中耳先天性胆脂瘤是少见疾病,常因病变隐匿而导致临床上延误诊断和治疗,随着影像学技术的提高和显微镜检查的普及,发现报道的先天性胆脂瘤病例逐渐增多。先天性胆脂瘤早期可无任何表现,逐渐出现传导性聋、耳痛,听力进行性下降,严重者可出现面瘫和神经性聋,甚至出现颅内并发症[1],由于无流脓、鼓膜穿孔等耳部症状,单侧的听力下降容易被忽略,诊断不易做出。现将解放军405医院耳鼻喉科2014年3月收治的先天性胆脂     

先天性中耳和岩尖胆脂瘤一例

我科于2005年收治罕见先天性中耳、岩尖胆脂瘤1例，报道如下。     

中耳先天性胆脂瘤二例

颞骨先天性胆脂瘤是罕见的具有破坏性的耳部疾病．病变隐蔽，早期无症状，出现症状时已有骨质破坏和耳部功能性和器质性障碍，甚至严重颅内外并发症，常发生于颅内，发生于颞骨者少见，我们曾收治2例，报道如下。     

中耳乳突先天性胆脂瘤五例分析

先天性胆脂瘤在临床上并不少见 ,它可原发于颞骨的各个部位 ,包括岩尖、膝状节区域、中耳及乳突区和颞骨鳞部、外耳道。本文报道近 10年来收治的 5例发生在中耳乳突的先天性胆脂瘤。1　临床资料1.1　病例资料　病例 1,9岁 ,右耳后反复红肿、流脓 4月余 ,听力下降不明显。检查 :右耳后皮肤红肿 ,局部破溃 ,范围约1ｃｍ× 1.5ｃｍ ,乳突区压痛 ,外耳道正常 ,鼓膜完整 ,但浑浊、增厚、暗红。病例 2 ,男性 ,9岁 ,右耳后反复红肿、脓疡半年 ,脓液恶臭伴淡血水 ,听力下降。检查 :右耳后皮肤有一 7ｃｍ× 5ｃｍ红肿包块 ,压痛明显 ,中央破溃 ,有脓…     

儿童中耳先天性胆脂瘤的诊断和预后

目的:探讨儿童中耳先天性胆脂瘤的临床症状、位置、影像学特征、治疗及预后.方法:回顾性分析9例中耳先天性胆脂瘤患儿的临床资料.结果:患儿均行手术治疗,5例有听小骨破坏,术后复发4例,复发率为44.4%.平均随访7.4年,术前平均听阈为28 dB,术后平均听阈为26 dB.结论:中耳先天性胆脂瘤临床少见,发病隐匿,单侧传导性聋为主要表现.术前诊断可根据Levenson诊断标准和CT检查结果.其预后和复发率与病变范围、手术方式、是否2次手术有关.     

先天性中耳胆脂瘤1例

先天性胆脂瘤又称表皮样囊肿或珍珠瘤,是一种源于皮肤外胚层的先天性肿瘤,为胚胎早期神经沟封闭时,皮肤外胚层的剩件残留发展而成.按其部位,可分为颅外型和颅内型.颅外型的可以发生在中耳和乳突,因其发病隐匿,易被误诊,延误治疗.目前国内外有关先天性中耳胆脂瘤的文献不多见,本文就收治的1例先天性中耳胆脂瘤报告如下.     

先天性中耳胆脂瘤的临床诊断与治疗进展

先天性胆脂瘤起病隐匿,能进行性生长破坏周围正常结构,早期易被临床医师忽视,错失最佳的诊疗时间,影响治疗效果。本文通过回顾性分析近年来相关的研究文献,帮助临床医师早期正确诊断中耳先天性胆脂瘤,提高治疗效果。     

儿童双侧先天性中耳胆脂瘤的临床分析

目的 探讨儿童双侧先天性中耳胆脂瘤(bilateral congenital cholesteatoma, BCC)的临床特点及手术方式。方法 回顾性分析5例(10耳)BCC患儿的临床资料,分析其手术方式及疗效。结果 BCC手术清除胆脂瘤,均先行病变严重侧,术后3～6月再行对侧手术,术前0.5、1、2、4 kHz气导平均听阈为45.1±13.3 dB HL,骨导平均听阈为9.6±5.7 dB HL。其中Ⅰ期1耳,Ⅱ期1耳,Ⅲ期3耳,这5耳经外耳道耳内镜下手术;Ⅳ期5耳,其中2耳行完壁式乳突切开+鼓室成形术,3耳行开放式乳突切开+鼓室成形术。术后随访2年,术后听力均较术前提高;术后除1耳(10%)复发,重新行开放式乳突切开+鼓室成形术外,其余患儿均未出现并发症。术后患儿气导平均听阈18.3±4.5 dB HL,骨导平均听阈6.2±4.1 dB HL,手术前后差异有统计学意义(P<0.01)。结论 手术治疗是BCC的唯一治疗方式,手术可提高患儿听力;Ⅰ、Ⅱ、Ⅲ期胆脂瘤病变较局限,未累及乳突,可优先考虑经外耳道耳内镜下手术;而Ⅳ期病变较广泛,累及乳突,建议显微镜下行乳突切开+鼓室成形术...     

耳内镜下儿童中耳胆脂瘤的处理及疗效分析

目的 探讨耳内镜下经耳道径路切除儿童中耳胆脂瘤的手术适应证及临床疗效,为该类疾病的处理提供临床参考.方法 回顾性分析2017年9月-2020年10月上海交通大学医学院耳科学研究所耳内镜下经耳道径路处理的20例中耳胆脂瘤患儿临床资料,患者年龄3～14岁,平均年龄为(7±0.5)岁.根据术前影像学评估,病变范围均在中耳范围...     

中耳胆脂瘤的遗传学研究进展

中耳胆脂瘤是一类位于鼓室和/或乳突内的团块,由角化的鳞状上皮细胞、上皮下的结缔组织以及角化碎片构成,伴/不伴炎症反应。临床表现主要为耳流脓和听力下降,当侵袭周围组织结构时则可产生眩晕、周围性面瘫、颅内感染等一系列颅内外并发症,甚至危及生命。手术是目前唯一有效的治疗方式。其病因及发病机制仍未完全清楚。随着全外显子测序等新一代测序技术的发展,中耳胆脂瘤的遗传学研究取得了一些突破性进展。近年来,关于胆脂瘤的家族聚集性、基因突变及其与综合征的关系的相关研究越来越多,本文将对上述内容进行综述,从而为中耳胆脂瘤的非手术治疗、药物研发提供参考。     

肿瘤坏死因子受体1在中耳胆脂瘤上皮中的表达

目的 研究肿瘤坏死因子受体1（TNFR1）在中耳继发性胆脂瘤上皮的表达，探讨其在胆脂瘤型中耳炎的发病机制中的作用。方法 分别采用免疫组化法和Western blot技术检测TNFR1在36例中耳胆脂瘤组织及20例正常外耳道皮肤组织中的表达情况。结果 TNFR1在36例胆脂瘤上皮各层细胞中均有强表达，定位于胞膜和胞质，而外耳道皮肤表达较弱甚至没有表达，TNFR1在胆脂瘤上皮中的蛋白表达水平较外耳道皮肤显著增高，差异有统计学意义（P〈0．01）。结论 胆脂瘤上皮中TNFR1表达较外耳道皮肤显著增高，肿瘤坏死因子可能通过与TNFR1相结合而导致胆脂瘤上皮细胞的过度增殖及凋亡。     

上皮细胞增殖和凋亡在中耳胆脂瘤发病学的意义(附33例报告)

目的 :通过探讨胆脂瘤上皮 (CE)、胆脂瘤患者外耳道上皮 (CAMS)和正常健康者外耳道上皮(NAMS)的增殖和凋亡及 p5 3基因与上皮细胞增生和凋亡的关系 ,阐明增殖和凋亡异常在胆脂瘤发病中的作用。方法 :采用免疫组织化学和原位末端标记技术检测细胞增殖标记物 PCNA、Ki- 6 7和 p5 3蛋白在 33例 CE、2 5例CAMS和 10例 NAMS细胞的表达及细胞凋亡。结果 :在上述标本中均有 PCNA表达及凋亡细胞存在 ,但不同类型上皮阳性细胞数量、染色强度及分布不同。 CE存在过度增殖和凋亡 ,CAMS亦存在过度增殖。 p5 3表达与PCNA呈正相关 ,与细胞凋亡呈负相关。结论 :CE具有过度增殖和凋亡的特性 ,增殖与凋亡的紊乱与胆脂瘤形成密切相关。     

Efficacy of diffusion-weighted magnetic resonance imaging in the diagnosis of middle ear cholesteatoma

Objective

This study evaluated the usefulness of diffusion-weighted magnetic resonance imaging (DWI) in the diagnosis of middle ear cholesteatoma.

Methods

We performed DWI on 73 patients suspected of having middle ear cholesteatoma, including 21 revision cases. Magnetic resonance imaging was performed with 1.5 T units using diffusion-weighted spin-echo-type echo planar imaging (DWI).

Results

Of 73 subjects, 59 had cholesteatoma that consisted of 41 primary acquired cholesteatoma, 13 had residual and/or recurrent cholesteatoma, four had congenital cholesteatoma, and one had iatrogenic cholesteatoma. Positive DWI findings were observed in 42 subjects and negative findings in 31 subjects. The sensitivity, specificity, and positive and negative predictive values of DWI for cholesteatoma were 69.4%, 92.8%, 97.5%, and 41.9%, respectively. In the case of 34 patients who were positive for cholesteatoma on both otoscopic and CT examinations, 33 were diagnosed with cholesteatoma. Of the remaining 39 subjects with one or both negative results for cholesteatoma, the sensitivity, specificity, positive predictive value, and negative predictive value of DWI were 57.6%, 92.3%, 93.7%, and 52.1%, respectively. Cholesteatoma mass diameters were less than 5 mm in 10 out of 18 subjects with both cholesteatoma and negative DWI findings. Of the 21 subjects who received revision surgery, the sensitivity, specificity, and positive and negative predictive values of DWI for residual or recurrent acquired cholesteatoma were 71.4%, 100%, 100%, and 63.6%, respectively.

Conclusions

Since DWI clearly showed high specificity and positive predictive value, it is useful for diagnosing middle ear cholesteatoma, including postoperative recurrent cholesteatoma of 5 mm diameter or larger. DWI could sufficiently detect cholesteatoma with one or both negative results on otoscopic and CT examinations, but it was difficult to detect cholesteatoma of less than 5 mm diameter using DWI owing to the tiny mass and small volume of debris.     

儿童中耳胆脂瘤诊治进展

儿童中耳胆脂瘤在患病率、发病机制与临床表现等方面都具有人群的特殊性.其起病隐匿,早期诊治成为临床难点.随着对儿童中耳胆脂瘤认识的提高和普及,临床中对此疾病的关注度明显提高.临床分期的应用及手术治疗的精准性趋于完善,伴有腭裂、特纳综合征、唐氏综合症等特殊人群的中耳胆脂瘤患儿的远期预后管理也得到了重视.     

congenital cholesteatoma of the middle ear-a report of 10 cases

Objectives To study clinical, imaging features and treatment outcomes of congenital cholesteatoma of middle ear (CCME). Methods This is a retrospective review of 10 CCME cases selected from 952 cholesteatoma cases treated between January 1995 and December 2005 at the Department of Otolaryngology-Head and Neck Surgery, Chinese PLA General Hospital. The main outcome measures were the site of origin, clinical features, surgical findings, imaging characteristics and hearing results. Results The mean age of the 10 patients was 16 years(ranged from 10 to 24 years), with 6 being older than 18 years. There were 7 males and 3 females. The average delay to diagnosis was longer than 2 years. The mean preoperative PTA was 55 dB HL, with a mean ABG of 45 dB. Typical cholesteatomas were seen behind the tympanic membrane in the superoposterior quadrant on otoscopy only in 2 patients. High resolution CT was completed in all patients. Most of the patients(8/10) were diagnosed with otosclerosis or ossicular abnormality before operation. All patients underwent a one-stage tympanoplasty following transmeatal explorative tympanotomy and complete cholesteatoma removal, except one, who underwent a CWU mastoidectomy due to extensive cholesteatoma involvement. The choleasteatoma lesion was confined to the superoposterior mesotympanum in all patients. The mean postoperative PTA was 20 dB HL. All patients were followed-up for at least 1.5 years postoperatively. Revision procedures were performed in 2 patients for hearing deterioration. No residual or recurrence of cholesteatoma was found. Conclusion CCME is a rare disease that often gets delayed diagnosis. Residual lesions and the prognosis mainly depend on the extent of the lesion.