儿童外耳道胆脂瘤的临床分型与手术

目的总结儿童外耳道胆脂瘤的手术疗效。方法[HTK〗根据36例儿童外耳道胆脂瘤的临床特征及胆脂瘤对外耳道破坏的程度进行临床分型;Ⅰ型为外耳道肉芽型,耳道骨质无破坏;Ⅱ型为外耳道狭窄骨质破坏型;Ⅲ型外耳道胆脂瘤已侵蚀上鼓室或乳突形成中耳胆脂瘤。结果36耳随访半年以上33耳,30耳外耳道皮肤完整,2耳术后8周耳道出现瘢痕性狭窄,经治疗耳道上皮愈合无再狭窄。1耳行改良乳突根治术多年仍有间歇性耳溢,3耳失访。结论儿童外耳道胆脂瘤的突出表现是外耳道有肉芽。显微镜下手术有助于识别病变破坏的轮廓及与周围结构的关系,恰当磨宽骨性耳道口部,磨光耳道骨质破坏区的骨壁,耳道皮肤缺损区移植皮片,可防止后期耳道瘢痕性狭窄及胆脂瘤复发。耳道填塞碘仿纱条不得少于4周,每周更换1次,直到耳道上皮愈合为止。Ⅲ型外耳道胆脂瘤应行改良乳突根治术。由于儿童外耳道胆脂瘤更具侵蚀性,手术的难度和危险性更大,应予高度重视。     

先天性耳畸形的手术治疗(附24例报告)

目的探讨治疗先天性耳畸形的不同手术方式及疗效，评估同期行耳廓成形及听力重建术的可行性。方法回顾性分析1985～2003年本科收治的24例(24耳)先天性耳畸形的患者，分别为单纯外耳道成形术6耳，外耳道-鼓室成形术10耳，同期行耳廓耳道成形及鼓室成形术8耳。结果行听力重建术的18耳，术后1个月纯音测听言语频率平均气骨导差缩小10～30dB；外耳道成形24耳中，5耳耳道狭窄；耳廓再造8耳均成活，近期耳轮、对耳轮出现，但远期效果较差，耳廓有不同程度的缩小变形。结论外耳道成形及听力重建术是治疗先天性耳畸形有效的手段，而同期行耳廓成形是可行的；以扩张后的薄皮瓣行耳廓成形效果较好，耳后带蒂皮瓣修复外耳道能有效防止外耳道再闭锁。     

适形骨片后壁重建技术在外耳道胆脂瘤术中应用探讨

目的 探讨自体乳突皮质骨制备适形骨片重建外耳道后壁治疗Ⅲ期外耳道胆脂瘤的手术效果。方法收集33例乳突气化良好的Ⅲ期外耳道胆脂瘤病例,行部分乳突切开+自体皮质骨外耳道后壁重建术,自体乳突皮质骨雕刻成适形骨片重建外耳道后壁,并以筋膜（骨膜）瓣覆盖完成外耳道成形。结果 33耳手术均顺利完成,共31耳一次手术实现干耳,随访无胆脂瘤复发、无重建外耳道后壁塌陷及狭窄闭锁;其中1例患者术后鼓膜穿孔,二次手术后鼓膜完整、愈合良好;1例患者术后重建的外耳道后壁出现窦道与乳突腔相通而导致间断耳漏,二次手术后干耳并恢复了外耳道的正常生理结构,共32耳干耳。另有1耳外耳道胆脂瘤复发,同时伴耳漏及耳道狭窄。结论部分乳突切开+自体皮质骨适形骨片外耳道后壁重建手术治疗Ⅲ期外耳道胆脂瘤,以筋膜（骨膜）瓣予以覆盖完成外耳道成形,可有效清除病变同时恢复接近正常外耳道的结构,可以进一步应用及深入研究。     

自体皮质骨在III型外耳道胆脂瘤手术中的应用

目的　探讨自体皮质骨重建骨性外耳道后壁在手术治疗Ⅲ型外耳道胆脂瘤（external auditory canal cholesteatoma,EACC）中的应用。方法　收集10例（11耳）临床诊断为Ⅲ型EACC患者,均一期行完壁式乳突切开+自体皮质骨外耳道后壁重建术+鼓室成形术治疗。结果　术后随访10例（11耳）患者,10耳恢复外耳道的正常生理结构; 1耳术后出现外耳道狭窄,给予膨胀海绵扩张3个月后恢复外耳道的正常生理结构。结论　手术治疗Ⅲ型EACC中应用自体皮质骨重建外耳道后壁并同期行完壁式乳突切开+鼓室成形术,能彻底有效清除胆脂瘤、恢复外耳道及中耳正常生理结构、术后复发率低、听力提高满意,值得进一步应用观察。     

耳内镜下外耳道“锁孔”技术处理累及乳突的中耳胆脂瘤的初步实践

目的本文旨在探索耳内镜手术中的“锁孔”技术在中耳胆脂瘤手术中的临床应用价值。方法2017年1月至2018年12月间收治的65名单侧中耳胆脂瘤患者,27例患者术前的颞骨CT提示低密度影及骨质破坏局限于上鼓室,鼓窦和乳突区域未见异常;38例患者术前的颞骨CT显示上鼓室内形成的低密度影像,存在骨质破坏,而鼓窦及乳突内也存在类似的低密度影像,难以确定病变是否累及乳突腔。术中耳内镜下经耳道“锁孔”技术早期探查上鼓室、鼓窦和后方的乳突腔内的空间,确定胆脂瘤范围,修正手术方案、优化手术策略。结果术前通过颞骨薄层CT显示的胆脂瘤仅局限于上鼓室的27例患者中,其中17例患者的影像学病变范围与“锁孔”技术探查结果吻合;另外10例病变累及鼓窦及乳突。术前38例患者的影像学显示上鼓室胆脂瘤可能累及鼓窦和乳突,术中通过“锁孔”技术验证,29例为上鼓室胆脂瘤侵及乳突;9例为堵塞形成的黏膜增厚及肉芽组织等非胆脂瘤病变。患者术后随访时间均超过了2年,随访方式为耳内镜及颞骨CT,8例患者的鼓窦及乳突区存在可疑软组织影,进行了便捷的内镜下“锁孔”的探查,鼓窦及乳突区未见胆脂瘤复发,软组织影为增厚黏膜及肉芽。结论在耳内镜下经外耳道径路,可结合持续灌流模式,在耳道后上壁快速开放直径4-6mm的骨窗,通过“锁孔”可以早期明确和判断中耳胆脂瘤累及乳突、鼓窦、上鼓室的病变范围,有利于修正手术方式,减少不必要的骨质磨除,更利于微创的实现和便于手术中耳道重建。     

耳后径路取带蒂皮瓣修复外耳道皮肤缺损18例

目的 探讨应用耳后带蒂皮瓣修复外耳道皮肤缺损扩大外耳道腔的疗效。方法 选取16例外耳道胆脂瘤患者及2例外耳道闭锁患者,均在全麻下行手术治疗。经耳后径路,术中见外耳道狭窄、外耳道峡部皮肤环状肉芽生长或闭锁,术中扩大外耳道骨管,于耳后切口处取带蒂皮瓣,向前转入耳道,覆盖于耳道后壁皮肤缺损处,扩大外耳道腔。结果 术后所有患者耳道皮瓣均存活,术后6个月,所有患者耳道形态恢复好,未发生狭窄或闭锁。结论 耳后径路取带蒂皮瓣易于取材,无需新切口,耳道植皮后,因瓣片有血运、不易坏死,减少了耳道再次狭窄或闭锁的发生。     

上鼓室胆脂瘤手术治疗

目的评估保留完整外耳道后上壁的上鼓室开放术治疗局限性胆脂瘤的临床疗效。方法38耳上鼓室胆脂瘤及内陷袋形成患者,耳内镜检查鼓膜松弛部内陷9耳,鼓膜松弛部穿孔27耳,外耳道胆脂瘤侵入上鼓室2耳。颞骨轴位和冠状位CT检查,病变局限在上鼓室区,天盖无明显破坏或下垂。虚拟耳镜显示听骨链完整13耳,听骨链变形12耳,听小骨不同程度破坏13耳。手术时在颞线下磨开外耳道后上嵴至颧弓后根骨壁,保留菲薄完整的外耳道后上壁和天盖,开放上鼓室经上而下处理胆脂瘤及鳞状上皮,完成听骨链成形术。结果术后外耳道完整,鼓膜松弛部穿孔或内陷袋行鼓膜修补术均一期愈合,术后平均听力较术前平均提高15～25dBHL,随访1.0～4.0年未见胆脂瘤复发。结论保留完整外耳道壁的上鼓室开放技术,在彻底清除上鼓室和听骨链病变的同时,完整保持外耳道和鼓室结构,临床疗效满意。     

骨性外耳道后壁重建鼓室成形术治疗胆脂瘤型中耳炎的疗效观察

目的：探讨处理乳突根治术后遗留的外耳道后壁缺损及开放的乳突腔的方法。方法：应用骨性外耳道后壁重建鼓室成形术治疗胆脂瘤型中耳炎58例（58耳）,清除病变的同时以颞骨皮质骨行骨性外耳道后壁重建并Ⅰ期行鼓室成形术。结果：随访1～3年,仅有1例胆脂瘤复发（复发率1．72％）。外耳道接近正常形态,保留含气乳突腔。纯音听阈提高30dBHL以上者（极效）5耳,提高20～29dBHL者（显效）11耳,提高10～19dBHL者（有效）30耳,总有效率为79．31％（46／58）,〈10dBHL者（基本无效）8耳,无听力提高者（无效）4耳。术后平均气导听阈明显减少。结论：乳突根治术后以颞骨皮质骨行骨性外耳道后壁重建并Ⅰ期行鼓室成形术,有助于修复乳突根治术后遗留的外耳道后壁缺损及开放的乳突腔,改善听力,降低胆脂瘤复发率,是一种较为实用的手术方法。     

先天性外耳道闭锁成形术后狭窄或再闭锁10例

目的探讨先天性外耳道闭锁成形术后狭窄或再闭锁的处理方法及效果。方法对10例（耳）先天性外耳道闭锁成形术后出现狭窄或闭锁的患者行再次手术,选取耳道后方作切口,切除狭窄或闭锁的瘢痕,扩大外耳道,术腔植皮,7耳同期鼓室成形。结果10耳中2耳外耳道出现再狭窄,经局部注射激素,纱条压迫后缓解,术后语频听力提高15dBHL者7耳,听力无改变3耳。结论先天性外耳道闭锁成形术后狭窄或再闭锁病例,再次手术仍是有效的方法,并可同期行鼓室重建。     

保留或重建部分外耳道后骨壁的乳突根治鼓室成形术

目的探讨乳突根治鼓室成形术中保留或重建部分外耳道后骨壁对提高胆脂瘤型中耳炎术后患者的干耳率及听力水平的意义.方法保留部分外耳道后壁24例、25耳,重建外耳道部分后壁6例、6耳,均行I期鼓室成形术.结果 术后随访11个月～4年,术后干耳并提高听力达实用听力(25dB以上)23耳,治愈率 82.1%;复发4耳,复发率12.9%.结论在乳突根治鼓室成形术中保留或重建部分外耳道后骨壁有利于增加中耳含气腔,提高听力;且有利于术后观察乳突腔病变, 避免胆脂瘤复发,提高干耳率.     

The formation of sinus in congenital stenosis of external auditory canal with cholesteatoma

CONCLUSIONS: In congenital stenosis of the external auditory canal (CSEAC) with cholesteatoma, the bony wall of the external auditory canal (EAC) is most commonly involved. This involvement will lead to bone erosion of the EAC and may subsequently lead to the formation of postaural or cervical sinuses. High-resolution computed tomography (HRCT) of temporal bone can show characteristic signs of soft tissue mass in EAC, with adjacent bone erosion. OBJECTIVE: To investigate the clinical features, differential diagnosis and management of CSEAC with cholesteatoma. PATIENTS AND METHODS: The clinical information for 10 cases of CSEAC with cholesteatoma was retrospectively reviewed. RESULTS: The patients' ages ranged from 4.75 to 22 years (average 12 years). The diameter of EACs was < 2 mm. All 10 ears had a history of postaural fistulae or sinuses. Bone erosion of EAC was distinctly shown in HRCT of all cases, as well as soft tissue masses, which led to enlargement of the bony canals. All patients underwent canaloplasty; eight ears received hearing reconstructions at the same time. Cholesteatoma in EACs was confirmed during the operations, accompanied by compression and destruction of the post-superior and/or inferior bony wall. Postoperative pathologic examinations proved the diagnosis of cholesteatoma, and excluded any tissue of bronchial cleft cyst or fistula. After a follow-up of 1-3 years, no recurrent cholesteatoma was found in any of the 10 cases. All reconstructed EACs were clean and smooth. The hearing levels in the eight ears that received hearing reconstructions increased 20-35 dBHL.     

Exostoses and Cavernous Venous Formation in the External Auditory Canal of the Hooded Seal as a Functional Physiological Organ

Exostoses of the external auditory canal (EAC) develop after protracted mechanical, chemical or thermal irritation in particular. This is a common disorder among aquatic sportsmen and has been considered unique to Man. We dissected and photodocumented the EACs of 5 newborn and 3 adult Hooded Seals (Cystophora cristata). Serial sections of the EACs were prepared for light microscopic evaluation after staining with haematoxylin-eosin or toluidine blue. All EACs exhibited a firm, broad-based, mountain peak-shaped exostosis on the floor of the meatus, lateral to the eardrum. In addition, the meatal skin of the bony EAC harboured large venous sinuses. The exostosis and venous sinuses of the seal EAC participate in the protection of the sensitive hearing apparatus, particularly the pars tensa portion of the drum, during diving.     

Exostoses and cavernous venous formation in the external auditory canal of the hooded seal as a functional physiological organ

Exostoses of the external auditory canal (EAC) develop after protracted mechanical, chemical or thermal irritation in particular. This is a common disorder among aquatic sportsmen and has been considered unique to Man. We dissected and photodocumented the EACs of 5 newborn and 3 adult Hooded Seals (Cystophora cristata). Serial sections of the EACs were prepared for light microscopic evaluation after staining with haematoxylin-eosin or toluidine blue. All EACs exhibited a firm, broad-based. mountain peak-shaped exostosis on the floor of the meatus, lateral to the eardrum. In addition, the meatal skin of the bony EAC harboured large venous sinuses. The exostosis and venous sinuses of the seal EAC participate in the protection of the sensitive hearing apparatus, particularly the pars tensa portion of the drum, during divine.     

外耳道胆脂瘤的临床特点及误诊分析

目的：分析外耳道胆脂瘤（EACC）的临床特点及误诊原因。方法：对1993～2003年收治的经临床和病理确诊的18例EACC患者,根据病变的范围和程度,采取不同的治疗方法,其治疗原则是彻底清除EACC和肉芽组织,促进创面愈合。结果：外耳道骨质破坏13例,外耳道深部为白色或黄色上皮角化物或耵聍样团块阻塞18例,外耳道口肉芽阻塞、皮肤红肿或外耳道狭窄15例,EACC侵犯乳突和上鼓室4例,鼓膜穿孔2例。随访1～6年,无复发。结论：EACC是以疼痛、间歇耳漏和骨质破坏为其特征的外耳道疾患,其治疗原则是彻底清除胆脂瘤囊袋、肉芽组织,促进创面愈合。     

Congenital external auditory canal stenosis and partial atretic plate

Objectives

To report outcomes of surgery for severe congenital external auditory canal (EAC) stenosis with or without partial atretic plate (PAP).

Design

Retrospective review.

Subjects

Thirteen patients (18 ears) had surgery for severe EAC stenosis with (n = 10, 56%) or without PAP (n = 8, 44%). Indications included severe stenosis with hearing loss, cerumen impactions, and/or canal cholesteatoma. Mean age = 7.8 years (range 0.4-19.9 years). Mean follow up = 5.2 years (range 0.4-10.0 years).

Setting

Tertiary care children's hospital.

Interventions

Nineteen endaural canaloplasties were performed in 17 ears (2 revisions). There was one post-auricular approach. Fifteen tympanoplasties were performed in 13 ears (2 revisions). PAP was reconstructed with drilling to enlarge the bony annulus and fascia grafting to enlarge the tympanic membrane in 10 (56%) ears.

Outcome measures

Patency of EACs; otologic findings; pure tone averages (PTA); complications.

Results

All (100%) EACs had improved patency (≥4 mm). Findings included canal cholesteatoma (2/18, 11%), ossicular fixation (4/18, 22%), stapes abnormality (2/18, 11%), and incudostapedial discontinuity from cholesteatoma (1/18, 6%). Mean preoperative PTA = 38.7 dB HL (range 60-20 dB HL). Mean post-operative PTA = 23.6 dB HL (range 50-6.7 dB HL). Audiologic results were significantly better for cases without PAP (p < .01) and without ossicular fixation (p < .01). There were seven minor and no major complications.

Conclusions

Endaural canaloplasty is safe and effective for providing patent EACs and hearing improvement for severe congenital EAC stenosis. However, since hearing outcomes were worse for cases with PAP and ossicular fixation, alternatives such as hearing aids or BAHA^® may be considered.     

外耳道胆脂瘤的临床特征及疗效评估

目的 探讨外耳道胆脂瘤(EACC)的临床特点,并评估其治疗效果。 方法 回顾性分析2015年1月至2016年12月47例(47耳)EACC的临床资料,所有患者术前接受听力学检查,耳内镜及颞骨CT检查。 结果 所有患者外耳道均可见黄白色鳞状或肉芽样物阻塞。耳闷胀感、耳痛及耳流脓是EACC常见症状。CT显示41耳有骨破坏。按Holt分期:47耳中,Ⅰ期6耳,Ⅱ期29耳,Ⅲ期12耳。对Ⅰ期6耳及Ⅱ期5例儿童患者行EACC和/或肉芽去除术;对24例Ⅱ期成人患者联合行胆脂瘤清除及外耳道成形术;12例Ⅲ期患者中,9例行乳突改良根治术和/或鼓室成型术,3例行乳突根治术。所有患者术后2周干耳,3个月内术腔完全上皮化。除3例行乳突根治术的患者术后听力无改善,其余44耳都有不同程度的提高。所有患者术后随访3~24个月,未见EACC复发者。 结论 EACC可被误诊,骨质破坏是其最重要的特征。颞骨CT有助于EACC的分期及制定治疗方案,应根据疾病分期、患者年龄及听力水平选择手术方法。彻底清除胆脂瘤及保持外耳道宽敞是治愈该疾病及预防复发的关键。     

肌骨膜瓣填塞乳突腔、耳甲腔成形并一期鼓室成形术

目的观察对慢性化脓性中耳乳突炎(胆脂瘤或骨疡型)患者行耳后肌骨膜瓣填塞乳突腔、耳甲腔成形、WullsteinⅢ型鼓室成形术的手术疗效.方法采用耳后切口对37例(37耳)慢性化脓性中耳乳突炎(胆脂瘤或骨疡型)病人施行耳后肌骨膜瓣填塞乳突腔及耳甲腔成形和Ⅲ型鼓室成形术.结果术后听力平均提高16dB,26耳平均听力达35.8dB,9耳平均听力达25dB.跟踪随访2-4年,术腔均上皮化好,外耳道无痂皮堆积.26例鼓膜形态完全正常,5例鼓膜疤痕内陷,3例鼓膜穿孔但干耳,干耳率91.9%(34/37);3耳仍有间断性流脓,经再次手术干耳,2例胆脂瘤复发,复发率5.4%(2/37).干耳时间在5-9周,平均6.5周.结论耳后肌骨膜瓣填塞乳突腔、耳甲腔成形并Wullstein Ⅲ型鼓室成形术能使术腔迅速上皮化、易干耳,术后听力提高.     

侵及鼓室、乳突的外耳道胆脂瘤诊断和治疗

目的探讨侵及鼓室、乳突的外耳道胆脂瘤的临床表现、影像特点及其手术治疗方法.方法回顾性分析我科1998～2003年收治的侵及鼓室、乳突的外耳道胆脂瘤14例患者的临床资料.结果14例患者病变均不同程度破坏外耳道四壁并向后扩展至乳突腔.其中4例鼓膜松弛部穿孔,胆脂瘤侵入鼓室,听骨链受压、内移,或不同程度破坏.3例面神经垂直段骨质破坏.1例乳突广泛破坏,硬脑膜裸露.1例先天畸形外耳道狭窄.根据病变的范围,8例行改良乳突根治术,5例行开放式乳突根治术,1例行外耳道扩大成形术.10例听骨链未受累的,术后听力恢复正常,1例听骨链受压变形、移位,术后仍达到正常听力.3例听骨链中断,行听骨链重建术,语言频率气导平均听阈提高15 dB～20 dB.14例患者随访18个月至5年未见复发.结论外耳道胆脂瘤的病因目前尚不十分清楚.侵及鼓室乳突者临床表现不典型,诊断有一定难度,术前常规高分辨颞骨CT扫描,有助于原发部位的判断及确定病变范围,以选择合适的手术方式.     

筋膜外植法鼓室成形术的手术体会

目的 探讨筋膜外植法在鼓室成形术中的应用及价值.方法 回顾分析筋膜外植法鼓室成形术63耳,随访观察患者的外耳道宽敞度、鼓膜形态及听力恢复情况.结果 本组病例包括中耳胆脂瘤25耳,慢性化脓性中耳炎38耳.手术方式分别为筋膜外植法鼓室成形术、筋膜外植法鼓窜成形术+完肇式乳突根治术和筋膜外植法鼓室成形术+开放式乳突根治术三种类型,中耳胆脂瘤和慢性化脓性中耳炎患者实施三种手术的数最分别为4、17、4耳和19、18、1耳.术后切口全部Ⅰ期愈合.随访0.5～3.5年,外耳道宽敞,鼓膜形态良好,听力提高或维持术前水平,未有听力下降者.按时随访者未发现有明显并发症.结论 筋膜外植法鼓室成形术具有操作流程规范、术野暴露充分、病变清除彻底等优点,在慢性中耳炎的外科治疗中具有积极意义.     

Development of canal cholesteatoma in a patient with prenatal isotretinoin exposure

Purpose

To describe the clinical and radiologic findings in a case of isotretinoin embryopathy-like syndrome and discuss management of hearing loss, congenital external auditory canal (EAC) stenosis, and EAC cholesteatoma.

Methods

Review of medical, audiological, and radiological records.

Results

An 8 year old female presented with bilateral moderate conductive hearing loss, bilateral microtia, left EAC stenosis, and right EAC atresia, secondary to prenatal isotretinoin exposure. Comorbidities included developmental delay, ventricular septal defect, hypotonia, and retinal maldevelopment. The left EAC was sharply upsloping with a 2 mm-diameter meatus. Computed tomography (CT) scan of the temporal bone demonstrated normal middle and inner ears bilaterally; serial CT scans over 6 years demonstrated progressive development of left canal cholesteatoma. Implantation of a right BAHA system was performed, followed by left canalplasty and excision of cholesteatoma with facial nerve monitoring. An endaural incision was utilized to avoid compromising future microtia repair. Postoperative left-sided hearing improved to mild low-frequency conductive hearing loss rising to normal at 2000 Hz and above.

Conclusions

Despite extensive precautions for its use, isotretinoin remains a cause of major birth defects, including sensorineural, conductive or mixed hearing loss. Congenital EAC stenosis is much less common than congenital atresia or acquired stenosis; optimal surgical approaches vary depending on hearing status and facial nerve anatomy. Close monitoring for development of canal cholesteatoma is necessary.