Objective: To summarize the experience of the diagnosis, treatment and effects of the cases with coexistence of first branchial cleft anomaly(FBCA) and microtia with congenital aural atresia or stenosis(external auditory canal stenosis, EACS). Method: This was a retrospective study. The clinical data of 5 patients with microtia and EACS in Beijing Tongren Hospital of Capital Medical University from October 2015 to March 2018 were collected, including 3 males and 2 females, aged from 5 to 28 years. The clinical characteristics, imaging findings, treatment methods and effects of 5 patients were analyzed. Result: The 5 cases were all coexistence of EACS and FBCA, three of who associated with cholesteatoma of external auditory canal. CT showed external auditory canal stenosis with soft tissue shadow, sometimes gas or bone septum found inside, filling in the external auditory canal, combined with canal bone destruction irregularly. All patients underwent surgical resection of FBCA, 3 patients accompanied by cholesteatoma resection and canalplasty. The postoperative follow-up ranged from 10 to 39 months, and no recurrence of infection was observed. Conclusions: EACS and FBCA both result from maldevelopment of the first branchial cleft. These two malformations, FBCA and EACS with or without cholesteatoma, can occur simultaneously, in which situation CT shows external auditory canal stenosis with soft tissue shadow inside. These patients underwent surgical resection of FBCA combined with cholesteatoma resection with good result.
目的： 探索先天性外中耳畸形合并第一鳃裂瘘的临床病例特点。 方法： 回顾性分析首都医科大学附属北京同仁医院2015年10月至2018年3月收治的5例先天性外中耳畸形合并第一鳃裂瘘患者的临床资料，其中男3例，女2例，年龄5~28岁。分析5例患者的临床特点、影像学表现、治疗方法及效果。 结果： 5例患者均为先天性外中耳畸形外耳道狭窄合并第一鳃裂瘘，其中3例伴外耳道胆脂瘤，CT示外耳道狭窄，外耳道内软组织影充填，有时其内可见气体影或骨性分隔，外耳道骨质伴不规则破坏。5例患者均行第一鳃裂瘘切除术，合并胆脂瘤的同时切除胆脂瘤，并行外耳道成形术，术后随访10~39个月，未见感染及复发。 结论： 先天性外耳道狭窄伴或不伴胆脂瘤均可与第一鳃裂瘘同时存在，影像学可见外耳道狭窄，外耳道内软组织影，完整切除第一腮裂瘘管及胆脂瘤并外耳道成形术，效果良好。.
Keywords: Branchial Region; Cholesteatoma; Congenital abnormalities; Ear canal; Ear deformities; Embryonic development; Fistula.