小切口内镜辅助双侧先天性第二鳃裂瘘切除及一家系报告

Mini-incision with endoscope-assisted surgery for bilateral congenital second branchial cleft fistula and a pedigree report

目的探讨小切口内镜辅助下双侧先天性第二鳃裂瘘切除的可行性,并对一家系进行报告。方法回顾性分析2007年4月至2018年12月间就诊于福建省立医院的5例双侧先天性第二鳃裂瘘患者临床资料,男2例,女3例,年龄3~31岁,总结小切口内镜辅助下切除瘘管的手术方法及经验,并对其中1个第二鳃裂瘘家系进行报告。结果5例患者中例1~4在全身麻醉下双侧均采用单一小切口内镜辅助下钛夹高位夹闭并切除瘘管的方法进行治疗,术后无明显并发症发生,随访40~164个月,均未见瘘管复发,恢复良好;例5放弃手术切除,随访24个月,出现急性感染再发作1次。例2和例4来自同一个家系,该家系四代31人中有7例出现第二鳃裂瘘,其中双侧4例,右侧3例,系谱分析符合常染色体显性遗传特征。对其家系成员进行相关检查,未发现有耳聋、耳前赘、外中耳畸形及肾脏畸形。结论双侧先天性第二鳃裂瘘罕见,手术切除为首选治疗方式,小切口内镜辅助下钛夹高位夹闭并切除瘘管的方式术野清晰,美容效果理想,疗效满意。

Objective To investigate the feasibility and efficacy of mini-incision with endoscope-assisted resection for bilateral congenital second branchial cleft fistula,and to report on a rare pedigree.Methods The clinical data of 5 patients with bilateral congenital second branchial fistula admitted in Fujian Provincial Hospital from April 2007 to December 2018 were retrospectively reviewed,including 2 males and 3 females,aged from 3 to 31 years old.The surgical strateges and clinical experience of single mini-incision with endoscope-assisted fistulectomy were summarized,and a rare pedigree was reported.Results In five patients,Case 1 to Case 4 were treated with bilateral endoscopic-assisted fistula high ligation with titanium clips and removal through a single small incision under general anesthesia.No obvious complications occurred after the operation.The patients were followed up for 40-164 months with no fistula recurrence.Case 5 gave up surgical resection and was followed up for 24 months with acute infection attack once.Case 2 and Case 4 came from the same family.In this family,7 out of 31 members of four generations had second branchial cleft fistulas,of which 4 were bilateral and 3 were right.Pedigree analysis was consistent with autosomal dominant inheritance.No deafness,preauricular tag,external and middle ear deformity and kidney malformation were found in the family members.Conclusions Bilateral congenital second branchial cleft fistula is rare.Surgical resection is the preferred treatment.Mini-incision with endoscopic-assisted fistula high ligation with titanium clip and resection has clear operative field,ideal cosmetic effect and definite curative effect.