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.

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.

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.

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.

Congenital abnormalities; Branchial region; Bilateral; Fistula; Surgery; Endoscope assistance