先天性第一鳃裂畸形的诊治和分类

Management and classification of first branchial cleft anomalies

目的:对第一鳃裂畸形的走行进行总结,并讨论其诊治和分类。方法:回顾性总结24例第一鳃裂畸形患者的病例资料。对第一鳃裂畸形的走行和相应的诊治方法进行分析,并根据Olsen分类法和Work分类法对患者进行分类。结果:根据Olsen分类法,该组患者可分为囊肿型(4例)、窦道型(13例)和瘘管型(7例)。内瘘口位于外耳道16例,而2例瘘管走行则平行于外耳道及咽鼓管,内瘘口位于鼻咽部的咽鼓管口附近;病变与腮腺关系密切且需解剖面神经14例。术后出现暂时性下颌缘支力弱2例;腮瘘1例,局部加压包扎2周后治愈;外耳道狭窄1例,于术后3个月行外耳道成形术。24例标本都含有鳞状上皮,其中6例有皮肤附属器,14例有软骨。2例通向鼻咽部的瘘管病理检查显示其外侧部分被覆鳞状上皮,内侧部分被覆纤毛柱状上皮。根据Work分类法,9例为一型,13例为二型,2例特殊患者不能用Work分类法进行分类。术后随访12～152个月,平均83个月,无一例复发。结论:第一鳃裂畸形走行多样。对疑似患者均应检查外耳道有无内瘘口,并行CT等检查以了解病变范围,以免切除不彻底。对于外耳道无内瘘口的患者,应行CT瘘管造影等检查以明确瘘管走行,选择适当的手术方式。2例特殊病例应属于一种新类型的病变。

Objective：We aimed to identify the different courses of first branchial cleft anomalies and to discuss the management and classification of these anomalies. Method：Twenty four patients with first branchial cleft anom alies were reviewed. The courses of first branchial cleft anomalies and their corresponding managements were ana- lyzed. Each case was classified according to Olsen＇s criteria and Work＇s criteria. Result： According to Olsen＇s crite ria, 3 types of first branchial cleft anomalies are identified： cysts（n=4）, sinuses（n=13）,and fistulas（n=7）. The internal opening was in the external auditory meatus in 16 cases. Two fistulas were parallel to the external audilo ry canal and the Eustachian tube, with the internal openings on the Eustachian tube. Fourteen cases had close rela- lions to the parotid gland and dissection of the facial nerve had to be done in the operation. Temporary weakness of the mandibular branch of facial nerve occurred in 2 cases. Salivary fistula of the parotid gland occurred in one pa lient, which was managed by pressure dressing for two weeks. Canal stenosis occurred in one patient, who under went canalplasty after three months. The presence of squamous epithelium was reported in all cases, adnexal skin structures in 6 cases, and cartilage in 14 cases. The specimens of the fistula which extended to the nasopharynx were reported as tracts lined with squamous epithelium （the external part） and ciliated columnar epithelium （the internal part）. According to Work＇s criteria, 9 cases were classified as Type I lesions, 13 cases were classified as Type II lesions, and two special cases could not be classified. The average follow-up was 83 months （ranging from 12 to 152 months）. No recurrence was found. Conclusion： First branchial cleft anomalies have high variability in the courses. If a patient is suspected to have first branchial anomalies, the external auditory canal must be examined for tbe internal opening. CT should be done to understand the exlension of the lesion. For cases without internal openings in the external auditory canal, CT fistulography should be done to demonstrate the courses, followed by corresponding treatment. Two special cases might be classified as a new type of lesions.