儿童中耳胆脂瘤的临床特征及EAONO/JOS分期指导下的个体化治疗

Clinical characteristics of middle ear cholesteatoma in children and analysis of individualized treatment effects under the guidance of EAONO/JOS staging

摘要

目的 采用单中心回顾性队列研究方法，回顾性分析儿童中耳胆脂瘤的临床病例特点及手术疗效，验证欧洲耳神经外科学/日本耳科学会(European academy of otology and neurotology/Japanese otological society，EAONO/JOS)分期指导个体化手术的有效性并识别术后复发的独立危险因素。方法 纳入2005年1月1日至2024年12月31日收治的14岁以下儿童中耳胆脂瘤患者171例(177耳)，采用回顾性分析方法提取患者临床表现、影像学特征、手术方式及术后随访结果等核心临床特征，对不同类型胆脂瘤特征、不同分期手术疗效等分类指标行分析，利用多因素logistic回归分析方法探究儿童中耳胆脂瘤术后复发的重要影响因素。结果 儿童中耳胆脂瘤好发于男性，8~10岁为发病高峰，左右耳发病率无明显差异。主要临床表现为耳流脓(83.0%)及听力下降(71.9%)，耳道分泌物恶臭(36.3%)为重要鉴别特征。并发症以耳后骨膜下脓肿相对多见。鼓膜松弛部穿孔多见(51.4%)，板障型乳突为主(76.2%)。胆脂瘤易侵及上鼓室、鼓窦，其次为后鼓室及乳突，听骨链损伤以砧骨及砧镫关节同时受累最为常见。面神经鼓室段裸露比例较高(29.4%)。根据EAONO/JOS分期，Ⅱ期病变占比最高(89.3%)。术后完成随访150耳，随访时间0.5~14年，复发3耳(2.0%)。术后平均纯音听阈及气骨导差较术前明显改善(P均<0.001)。多因素分析显示，听骨链Ⅳ型损伤是术后复发的重要相关因素。结论 儿童中耳胆脂瘤具有独特的临床与病理特征。依据EAONO/JOS分期选择个体化手术方案(Ⅰ期以上鼓室-鼓窦开放为主，Ⅱ~Ⅳ期以开放式乳突根治为主)可获得良好疗效。术后定期随访对监测对侧耳、及时处理填塞物残留、耳道膜性闭锁及早期发现复发胆脂瘤具有重要意义。

Abstract

Objective A retrospective cohort study was conducted at a single centre with the objective of analysing the clinical characteristics and surgical outcomes of paediatric cholesteatoma of the middle ear. The objective of this study was twofold: firstly, to validate the effectiveness of the European academy of otology and neurotology/Japanese otological society (EAONO/JOS) staging in guiding individualised surgical approaches, and secondly, to identify independent risk factors for postoperative recurrence. Methods A total of 171 paediatric patients (177 ears) with middle ear cholesteatoma under 14 years of age, admitted from January 1, 2005 to December 31, 2024, were included in the study. A retrospective analysis was conducted to extract core clinical features such as patients' clinical manifestations, imaging characteristics, surgical methods, and postoperative follow-up results. Chi-square tests were used for the purpose of classification, with indicators including characteristics of different types of cholesteatoma and surgical efficacy at different stages. Multivariate logistic regression analysis was employed to explore the significant factors influencing postoperative recurrence of paediatric middle ear cholesteatoma. Results The prevalence of cholesteatoma was found to be higher in males, with a peak incidence observed between the ages of 8 and 10 years. No significant difference was observed between the left and right ears. The predominant clinical manifestations were otorrhea (83.0%) and hearing loss (71.9%), with foul-smelling ear canal secretions (36.3%) serving as a significant distinguishing feature. Complications included subperiosteal abscess behind the ear. Perforation of the pars flaccida (51.4%) and diploetic mastoid (76.2%) were frequently observed. Cholesteatoma has a propensity to invade the epitympanum and antrum, followed by the posterior tympanum and mastoid process. The most prevalent ossicular chain injury pertains to concurrent involvement of the incus and incudostapedial joint. In 29.4% of cases, the tympanic segment of the facial nerve was exposed. According to the EAONO/JOS staging system, the majority (89.3%) of cases were classified as stage Ⅱ disease. Postoperative follow-up was conducted in 150 cases, with a range of 0.5-14 years, and recurrence was observed in 3 cases (2.0%). The pure-tone average and air-bone gap demonstrated significant postoperative improvement (all P<0.001). Multivariate analysis indicates that type Ⅳ ossicular chain injury is a significant predictor of postoperative recurrence. Conclusion Paediatric middle ear cholesteatoma is characterised by distinct clinical and pathological features. A surgical approach based on EAONO/JOS staging (atticotomy/antrotomy for stage Ⅰ, open radical mastoidectomy for stages Ⅱ-Ⅳ) has been shown to yield favourable outcomes. It is imperative that patients undergo regular postoperative follow-ups in order to monitor the contralateral ear, manage packing material retention, address membranous atresia, and facilitate early detection of recurrent cholesteatoma.

关键词

儿童 / 胆脂瘤 / 中耳炎 / 耳科手术 / EAONO/JOS分期

Key words

Children / Cholesteatoma / Tympanitis / Ear surgery / EAONO/JOS staging

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基金资助

河北省医学科学研究课题计划(20241588)