儿童先天性中耳胆脂瘤的临床治疗

Clinical treatment of congenital middle ear cholesteatoma in children

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:(1)14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%)。(3)A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)。(4)A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)。(5)14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。

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：（1）The 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.（2）Hearing 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%）.（3）There 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）.（4）The 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）.（5）The 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.