Congenital microtia with aural atresia or stenosis accompany with first branchial cleft anomaly: report of 5 cases

Abstract

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.