Bi Jing, Yu Bo, Chen Xiaowei, Fu Yong, Xu Bin, Zhang Yang
Department of ENT and Head & Neck Surgery, The Children's Hospital Zhejiang University School of Medicine, Binsheng Road 3333, Hangzhou, 310051, Zhejiang, China.
Zhejiang Provincial Key Lab of Genetic and Developmental Disorder, Hangzhou, Zhejiang, China.
Eur Arch Otorhinolaryngol. 2024 Jul 24. doi: 10.1007/s00405-024-08840-5.
This study aimed to summarise the clinical features and management strategies concerning auditory canal duplication anomalies in children with congenital first branchial cleft anomalies (CFBCAs), and to provide guidance for precise treatment.
We retrospectively analysed 84 children with CFBCAs who had complete data, diagnosed between December 2018 and February 2024.
All the lesions identified were located around the external auditory canal or near the mandibular angle, manifested as pinhead-sized perforations in 10 cases, painless masses in 18 cases, recurrent swelling and pain with purulent discharge in 52 cases, and otorrhea in 4 cases. Otoscopy examinations revealed external auditory canal swelling in seven children, fistulas within the auditory canal in four children, and a myringa web in three children. Fifty-six children had a preoperative history of infection. Using Work's classification system, Work I and II in 70 (87.5%) and 14 (12.5%) children, respectively. Intraoperatively, 80 (95.2%) children had auditory canal duplication anomalies at the base of the lesion, closely associated with the cartilage of the inferior wall of external auditory canal(EAC), We then classified auditory canal duplication anomalies into three types: Type A (duplication anomalies of epithelial tissue structure between the skin of the EAC and the cartilage of the inferior wall, n = 16 children), Type B (duplication anomalies of the epithelial and/or skin tissue structure, sharing a wall with the cartilage of the inferior wall, n = 40), and Type C (duplication anomalies of the skin and cartilage tissue structure, connected to the cartilage of the inferior wall of EAC, n = 24). Sixty-eight children had lesions superficial to the facial nerve, 12 had lesions deep to the facial nerve, and four had lesions between branches. There were two cases of transient postoperative facial paralysis, three cases of CFBCA recurrence, and two cases of transient auditory canal stenosis.
Auditory canal duplication anomalies are an important feature of first branchial cleft anomalies in children. Precise staging and accurate identification of the base of the lesion facilitate complete removal, thereby increasing the cure rate.
本研究旨在总结先天性第一鳃裂畸形(CFBCAs)患儿外耳道重复畸形的临床特征及治疗策略,为精准治疗提供指导。
回顾性分析2018年12月至2024年2月期间诊断明确且资料完整的84例CFBCAs患儿。
所有病变均位于外耳道周围或下颌角附近,表现为10例针头大小的穿孔、18例无痛性肿块、52例反复肿胀疼痛伴脓性分泌物及4例耳漏。耳镜检查发现7例患儿外耳道肿胀,4例患儿外耳道内瘘管形成,3例患儿鼓膜有蹼。56例患儿有术前感染史。采用Work分类系统,Work I型和II型分别见于70例(87.5%)和14例(12.5%)患儿。术中,80例(95.2%)患儿在病变基底处存在外耳道重复畸形,与外耳道下壁软骨紧密相连。随后将外耳道重复畸形分为三种类型:A型(外耳道皮肤与下壁软骨之间上皮组织结构重复畸形,n = 16例患儿)、B型(上皮和/或皮肤组织结构重复畸形,与下壁软骨共用一壁,n = 40例)和C型(皮肤和软骨组织结构重复畸形,与外耳道下壁软骨相连,n = 24例)。68例患儿病变位于面神经浅面,12例患儿病变位于面神经深面,4例患儿病变位于面神经分支之间。术后有2例出现短暂性面瘫,3例CFBCA复发,2例出现短暂性外耳道狭窄。
外耳道重复畸形是儿童第一鳃裂畸形的重要特征。精确分期和准确识别病变基底有助于完整切除,从而提高治愈率。