Congenital fistula of the fourth branchial arch: Report of case with long-lasting misdiagnosis

Type II branchial cleft anomalies are the most frequent branchial anomaly (90%). Type I, III and IV branchial cleft anomalies are much rarer. We report the case of a young woman who underwent 12 operations before the correct diagnosis of a type IV branchial arch anomaly was made. Direct laryngoscopy and computed tomography after barium swallow confirmed the sinus tract. Branchial cysts, fistulas, and sinuses are anomalies of the branchial apparatus occurring after failure of a branchial cleft to involute. Branchial anomalies may be diagnosed at any age but present most commonly in infancy and childhood.1 Derivates from the fourth pharyngeal arch are the superior parathyroid glands, various muscles, the thyroid and epiglottic cartilage, and the vagal nerve.2 Fourth branchial anomalies begin in the apex of the piriform fossa, pierce the larynx near the cricothyroid ligament, and then pass between the superior and recurrent laryngeal nerves (Figure ​(Figure1).1). Left‐sided anomalies descend in the tracheoesophageal groove and loops around the aorta, ascending posteriorly to the common carotid before passing over the hypoglossal nerve. On the right side, the fistulas loop around the subclavian artery.3 Open in a separate window Figure 1 Anatomic course of type IV fistulas, SLN superior laryngeal nerve, CN cranial nerve, RLN recurrent laryngeal nerve, JV jugular vein