Chen Wei, Xu Mengrou, Wang Qingyu, Xu Rong, Chen Jiarui, Xu Hongming, Li Xiaoyan
Department of Otolaryngology-Head and Neck Surgery, Shanghai Children's Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, Luding Road, No. 355, PuTuo District, Shanghai, 200062, China.
Department of Pathology, Shanghai Children's Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, Shanghai, 200062, China.
Eur Arch Otorhinolaryngol. 2023 Jan;280(1):425-433. doi: 10.1007/s00405-022-07607-0. Epub 2022 Aug 30.
To investigate the clinical features and surgical outcomes of pediatric congenital first branchial cleft anomalies (CFBCAs).
We conducted a retrospective analysis of 100 children who were referred to Shanghai Children's Hospital from March 2014 to March 2022 for the treatment of CFBCAs.
This study included 100 patients (33 males, 67 females) with an average age of 4.0 ± 2.7 years. 64 cases were type I FBCAs and 36 were type II. The main clinical manifestations included having a skin pit or discharge from it (62%), painless masses (5%), mucopurulent otorrhea (8%) and recurrent swelling with pain (90%) in the Pochet's triangle area. 92% had infection histories, 84% had incision and drainage histories, and 18% had surgical histories. 6 cases of tympanic membranous attachment were found by auricular endoscopy. Ultrasonography (US) was 55.6% (30/54) accurate and enhanced CT was 75% (75/100) accurate in diagnosing CFBCAs. We dissected the facial nerve (FN) in 46% cases. Lesions ended in the external auditory canal (EAC) wall in 86 cases. 69 exhibited close relationship with the parotid. The patients were followed up 0.25-8.2 years. 11 had postoperative temporary facial paralysis and all improved within 6 months. 3 had recurrence and they were secondarily successfully retreated. No EAC stenosis were found.
CFBCAs often presented with repeated swelling and purulence in Pochet's triangle. CT, US and auricular endoscopy can assist in diagnosis and planning the surgical strategy. Complete excision in non-infection stage as soon as possible is the first choice for the treatment of CFBCAs.
探讨小儿先天性第一鳃裂畸形（CFBCAs）的临床特征及手术疗效。
对2014年3月至2022年3月因CFBCAs转诊至上海儿童医学中心的100例患儿进行回顾性分析。
本研究纳入100例患者（男33例，女67例），平均年龄4.0±2.7岁。I型FBCAs 64例，II型36例。主要临床表现为有皮肤瘘口或有分泌物（62%）、无痛性肿块（5%）、黏液脓性耳漏（8%）以及Pochet三角区反复肿胀伴疼痛（90%）。92%有感染史，84%有切开引流史，18%有手术史。耳内镜检查发现6例鼓膜附着异常。超声（US）诊断CFBCAs的准确率为55.6%（30/54），增强CT的准确率为75%（75/100）。46%的病例术中解剖了面神经（FN）。86例病变止于外耳道（EAC）壁。69例与腮腺关系密切。患者随访0.25 - 8.2年。11例术后出现暂时性面瘫，均在6个月内恢复。3例复发，二次手术成功治愈。未发现EAC狭窄。
CFBCAs常表现为Pochet三角区反复肿胀和化脓。CT、US和耳内镜有助于诊断并制定手术策略。尽早在非感染期完整切除是CFBCAs治疗的首选。
