儿童先天性中耳胆脂瘤的临床治疗

郝津生; 陈敏; 刘冰; 杨扬; 刘薇; 张杰

doi:10.13201/j.issn.1001-1781.2018.14.014

儿童先天性中耳胆脂瘤的临床治疗

- 国家儿童医学中心首都医科大学附属北京儿童医院儿科研究所耳鼻咽喉头颈外科重点实验室首都医科大学附属北京儿童医院耳鼻咽喉头颈外科(北京, 100045)
基金项目:
北京市医管局临床医学发展专项资助项目（No：XM201409）

详细信息

通讯作者: 张杰,E-mail:stzhangj@263.net

中图分类号: R764.2

收稿日期: 2018-04-08

Clinical treatment of congenital middle ear cholesteatoma in children

- Children's National Medical Center, Department of Otolaryngology, Head and Neck Surgery, Beijing Children's Hospital, Capital Medical University, Beijing Key Laboratory for Pediatric Diseases of Otolaryngology, Head and Neck Surgery, Beijing Pediatric Research Institute, Beijing Children's Hospital, Capital Medical University, Beijing, 100045, China

More Information

Corresponding author: ZHANG Jie, E-mail: stzhangj@263.net

Received Date: 08 April 2018

摘要

摘要: 目的：总结先天性中耳胆脂瘤患儿的临床特点及治疗，提供早期诊断方法并探讨规范诊治方案。方法：收集2009-2015年在北京儿童医院耳鼻咽喉头颈外科收治的94例中耳胆脂瘤患儿的临床资料，进行回顾性分析。根据Levenson标准，94例患儿中14例诊断为先天性中耳胆脂瘤。根据病程时间（≤ 3个月或>3个月），分成A、B 2组。对主诉、发现方式、听力学情况、颞骨CT及手术治疗等进行记录分析。结果：①14例先天性中耳胆脂瘤患儿年龄是3.33~10.17岁，中位年龄为7.20岁。②听力下降（13/14，92.86%））是最常见的主诉。发现方式有：听力筛查加颞骨CT （11/14，78.57%），鼓膜切开术中发现（2/14，14.28%）以及耳镜检查（1/14，7.14%）。③A组6例，B组8例，根据Potsic's分级标准，2组间的差异有统计学意义（P=0.043）。④A和B 2组术前气骨导差分别为（38.10±7.43） dB和（42.09 ±9.96） dB，差异无统计学意义（P=0.427）。⑤14例患儿手术前后气骨导差分别为（36.26±5.56） dB和（21.70±3.80） dB，差异有统计学意义（P=0.004）。完壁式乳突切除术加鼓室成形术（11/14，78.57%）是最常用的手术方法。结论：病程越短，中耳胆脂瘤分级越低，提示早期发现的重要性。但先天性胆脂瘤隐匿性较强，病程即使在3个月内，胆脂瘤同样会对患儿听力及中耳结构造成较为严重的损害。早期筛查方案可推荐听力筛查辅助颞骨CT检查，以利早期干预治疗。
- 胆脂瘤,先天性 /
- 听力下降 /
- 诊断 /
- 治疗 /
- 儿童
Abstract: Objective: To summarize the clinical characteristics and clinical treatment of congenital cholesteatoma (CC) of the middle ear in children, provide early diagnosis methods and explore standardized diagnosis and treatment plan.Method: A retrospective chart review of 94 patients with a diagnosis of middle ear cholesteatoma, in Beijing Children's Hospital, between 2009 and 2015 was carried on. 14 patients with CC were identified using the criteria proposed by Levenson, and were divided into two groups according to the course of disease. The course of disease in group A was less than 3 months, and group B was more than 3 months. The main complains, diagnostic methods and Potsic's stage of temporal bone CT findings were recorded.Result: ①The age of 14 cases of congenital cholesteatoma of the middle ear ranged from 3.33 to 10.17 years, with the median age of 7.20 years. ②Hearing loss (13/14, 92.86%) was the most common complain. Finding methods included hearing screening and CT scan (11/14, 78.57%), tympanotomy (2/14, 14.28%) and otoscopic examination (1/14, 7.14%). ③There were 6 people in group A and 8 in group B. According to Potsic's grading standard, the difference between the two groups was statistically significant (P=0.043). ④The preoperative Air-Bone conduction threshold Gap (ABG) in A and B two groups were (38.10±7.43) dB and (42.09±9.96) dB, respectively, and there was no significant difference in analysis (P=0.427). ⑤The difference between pre-ABG and post-ABG[(36.26±5.56)dB and (21.70±3.80)dB, P=0.004] was significant. Canal wall up mastoidectomy was the preferred procedure and 11/14 (78.57%) patients had this surgery done.Conclusion: The shorter the course of disease, the lower the stage of cholesteatoma of the middle ear indicates the importance of early detection. But congenital cholesteatoma is more occult, and even within 3 months, cholesteatoma can cause severe damage to the hearing and middle ear structure. Early screening programs can recommend hearing screening and CT scan to facilitate early intervention.
- cholesteatoma,congenital /
- hearing loss /
- diagnosis /
- therapy /
- children

HTML全文

参考文献(23)

[1]	LIN V, DANIEL S, JAMES A, et al.Bilateral cholesteatomas:the hospital for sick children experience[J].J Otolaryngol, 2004, 33:145-150.
[2]	LEVENSON M J, MICHAELS L, PARISIER S C.Congenital cholesteatomas of the middle ear in children:origin and management[J].Otolaryngol Clin North Am, 1989, 22:941-954.
[3]	POTSIC W P, SAMADI D S, MARSH R R, et al.Astaging system for congenital cholesteatoma[J].Arch Otolaryngol Head Neck Surg, 2002, 128:1009-1012.
[4]	HIDAKA H, YAMAGUCHI T, MIYAZAKI H, et al.Congenital cholesteatoma is predominantly found in the posterior-superior quadrant in the Asian population:systematic review and meta-analysis, including our clinical experience[J].Otol Neurotol, 2013, 34:630-638.
[5]	TAKAGI T, GYO K, HAKUBA N, et al.Clinical features, presenting symptoms, and surgical results of congenital cholesteatoma based on Potsic's staging system[J].Acta Otolaryngol, 2014, 134:462-467.
[6]	KAZAHAYA K, POTSIC W P.Congenital cholesteatoma[J].Curr Opin Otolaryngol Head Neck Surg, 2004, 129:398-403.
[7]	LIM H W, YOON T H, KANG W S.Congenital cholesteatoma:Clinical features and growth patterns[J].Am J Otolaryngol, 2012, 33:538-542.
[8]	WALKER D, SHINNERS M J.Congenital cholesteatoma[J].Pediatr Ann, 2016, 45:e167-170.
[9]	PEDRUZZI B, MION M, COMACCHIO F.Congenital intra tympanic cholesteatoma in an adult patient:a case report and review of the literature[J].J Int Adv Otol, 2016, 12:119-124.
[10]	POTSIC W P, KORMAN S B, SAMADI D S, et al.Congenital cholesteatoma:20 years' experience at The Children's Hospital of Philadelphia[J].Otolaryngol Head Neck Surg, 2002, 126:409-414.
[11]	KAYHAN F T, MUTLU C, SCHACHERN P A, et al.Significance of epidermoid formations in the middle ear in fetuses and children[J].Arch Otolaryngol Head Neck Surg, 1997, 123:1293-1297.
[12]	NELSON M, ROGER G, KOLTAI P J, et al.Congenital cholesteatoma:classification, management, and outcome[J].Arch Otolaryngol Head Neck Surg, 2002, 128:810-814.
[13]	EL-BITAR M A, CHOI S S, EMAMIAN S A, et al.Congenital middle ear cholesteatoma:need for early recognition-role of computed tomography scan[J].Int J Pediatr Otorhinolaryngol, 2003, 67:231-235.
[14]	KOJIMA H, TANAKA Y, SHIWA M, et al.Congenital cholesteatoma clinical features and surgical results[J].Am J Otolaryngol, 2006, 27:299-305.
[15]	INOKUCHI G, OKUNO T, HATA Y, et al.Congenital cholesteatoma:posterior lesions and the staging system[J].Annal Otol Rhinol Laryngol, 2010, 119:490-494.
[16]	PRECIADO D A.Biology of cholesteatoma:special considerations in pediatric patients[J].Int J Pediatr Otorhinolaryngol, 2012, 76:319-321.
[17]	CORRALES C E, BLEVINS N H.Imaging for evaluation of cholesteatoma:current concepts and future directions[J].Curr Opin Otolaryngol Head Neck Surg, 2013, 21:461-467.
[18]	ALZOUBI F Q, ODAT H A, AL-BALAS H A, et al.The role of preoperative CT scan in patients with chronic otitis media[J].Eur Arch Otorhinolaryngol, 2009, 266:807-809.
[19]	VON KALLE T, AMRHEIN P, KOITSCHEV A.Non-echoplanar diffusion-weighted MRI in children and adolescents with cholesteatoma:reliability and pitfalls in comparison to middle ear surgery[J].Pediatr Radiol, 2015, 45:1031-1038.
[20]	GRIMES E R, ISAACSON G.The mechanical reduction of early acquired cholesteatomas in children:indications and limitations[J].Ear Nose Throat J, 2006, 85:252-256.
[21]	PRASAD S C, LA MELIA C, MEDINA M, et al.Long-term surgical and functional outcomes of the intact canal wall technique formiddle ear cholesteatoma in the pediatric population[J].Acta OtorhinolaryngolItal, 2014, 34:354-361.
[22]	STAPLETON A L, EGLOFF A M, YELLON R F.Congenital cholesteatoma.Predictors for residual disease and hearing outcomes[J].Arch Otolaryngol Head Neck Surg, 2012, 138:280-285.
[23]	KOBAYASHI T, GYO K, KOMORI M, et al.Efficacy and safety of transcanal endoscopic ear surgery for congenital cholesteatomas:a preliminary report[J].Otol Neurotol, 2015, 36:1644-1650.