儿童先天性第一鳃裂畸形的临床分析

Clinical analysis of pediatric congenital first branchial cleft anomaly

目的总结儿童先天性第一鳃裂畸形(CFBCA)的临床特点。方法回顾分析2014年8月~2020年4月我科收治的82例CFBCA病例的临床资料。临床表现:Pochet三角区无痛性肿块22例、反复肿痛合并流脓55例、耳道溢液9例。术前有感染史55例。病灶位于耳周47例、下颌角附近35例。耳内镜检查:外耳道见内瘘口9例、鼓膜蹼5例。增强CT检查的诊断率为75.6%(62/82),超声的诊断率为54.9%(45/82)。结果瘘管位于面神经浅面65例、深面15例、分支间2例。WorkⅠ型42例、Ⅱ型40例;Olsen囊肿30例、窦道39例、瘘管13例。术后短暂性面瘫8例,复发2例。WorkⅠ型多为囊肿型,WorkⅡ型多为窦道/瘘管型(P<0.05)。Ⅰ型、囊肿型,瘘管多位于面神经浅面,术前多无感染史,病灶常位于耳郭周围;Ⅱ型、窦道/瘘管型,瘘管多位于面神经深面或分支间,术前多有感染史,病灶常位于下颌角附近,术后易复发,易发生一过性面瘫(P<0.05)。瘘管与面神经的解剖关系与年龄、性别、侧别等无关(P>0.05)。结论CFBCA常表现为Pochet三角区无痛性肿块、反复红肿合并流脓或耳道溢液等症状。耳内镜、CT、B超等辅助检查可提高诊断率,预判瘘管与周围解剖的关系,指导手术方式。炎症静止期尽早彻底手术是治疗首选。

Objective To summarize the clinical manifestations of congenital first branchial cleft anomaly(CFBCA).Methods The clinical data of 82 patients accepted from August 2014 to April 2020 in our hospital were retrospective analyzed.There were 22 cases of painless mass in pochet triangle,55 cases of repeated swelling and purulence,and 9 cases of ear canal effusion.Fifty-five cases had a history of infection before operation.The lesions were located around the ear in 47 cases and near the mandibular angle in 35 cases.Nine cases of internal fistula and 5 cases of tympanic membranous attachment were found by auricular endoscopy.The diagnostic rate by that of enhanced CT was 75.6%and was 54.9%by ultrasound.Results The fistulas were located on the superficial surface of facial nerve in 65 cases,deep in 15 cases and between branches in 2 cases.There were 42 cases of Work typeⅠand 40 cases of typeⅡ.There were 30 cases of Olsen cyst,39 cases of sinus and 13 cases of fistula.There were 8 cases of transient facial paralysis and 2 cases of recurrence.Statistical analysis showed that most of Work typeⅠwas cyst type and WorkⅡtype was sinus/fistula type(P<0.05).In typeⅠand cyst type,the fistulas were mostly located on the superficial surface of the facial nerve,and the lesions were mostly located around the auricle;in typeⅡ,sinus/fistula type,the fistula was mostly located in the deep surface or between the facial nerve branches,with a history of infection before operation,and the lesions were mostly located near the mandibular angle,which were easy to recur and had transient facial paralysis(P<0.05).The anatomic relationship between fistula and facial nerve was not related to age,gender or lateral sex(P>0.05).Conclusions CFBCA often presents with painless mass in Pochet triangle,repeated swelling,purulence or ear canal discharge.Auricular endoscopy,CT,ultrasound and other auxiliary examinations can improve the diagnostic rate,predict the relationship between fistula and surrounding anatomy,and guide the operation mode.Complete surgery as soon as possible is the first choice for the treatment of static inflammation.