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Bilateral second branchial cleft fistulae in a boy with congenital heart disease.

A 13-year-old boy consulted for an intermittent, bilateral, mucus-like discharge from his neck (figure, A). This had occurred since his birth, especially when he was eating. According to his parents, the boy had a history of complex congenital heart disease and had undergone corrective surgery when he was a baby.

Physical examination revealed two cervical openings located at the anterior border of the bilateral sternocleidomastoid (SCM) muscles at the level of the hyoid bone. The openings had synchronous movement with the intermittent mucus-like discharge (see video at www. entjournal.com in the July 2017 issue *). Esophagography demonstrated contrast leakage in the ipsilateral cervical openings via tracts from the bilateral tonsillar fossae (figure, B). The tracts were also clearly seen during fistulography (figure, C and D). Based on these findings, a diagnosis of bilateral second branchial cleft fistulae was made. After we discussed the case with the patient and his family, they decided to tolerate this inconvenient situation and refused surgical correction.

Branchial anomalies comprise approximately 20% of pediatric congenital head and neck masses and are classified as first, second, third, and fourth branchial cysts, sinuses, and fistulae. All are distinct entities and are thought to result from a similar embryologic error: the incomplete obliteration of the branchial apparatus during embryogenesis. (1) The diagnosis of branchial anomalies depends on good history taking, physical examination, proper image studies, and a high index of suspicion and clinical awareness.

The branchial apparatus consists of six grooves (ectoderm), arches (mesoderm), and pouches (endoderm). When both a pouch and a groove fail to become obliterated, these may form a communication between the skin and mucosa, which is called a fistula. (1,2) A complete fistula with both an internal and an external opening is thought to be extremely rare, with only a few reported cases in literature. (3) Bilateral second branchial fistulae are even more rare and, if present, warrant a consult with a geneticist because of the possible association with branchio-oto-renal syndrome or other genetic disorders. (1,4)

The complete course of second branchial fistula begins near the anterior border of the SCM, tracks superiorly and laterally to the common carotid artery, passes medially between the external and internal carotid arteries, moves lateral and superior to the glossopharyngeal and hypoglossal nerves, and penetrates the middle pharyngeal constrictor muscle to open into the tonsillar fossa. (5)

Image studies such as fistulography, ultrasonography, computed tomography (CT), or magnetic nuclear resonance imaging may provide anatomic details and help to achieve an appropriate diagnosis. Preoperative ultrasound and CT scans demonstrate no advantage for the surgical procedure or for diagnosis, as imaging fails to completely visualize the entire tract. (4) With contrast fistulography, the tract of the fistula up to the internal opening at the tonsillar fossa can be traced. This is valuable for preoperative planning.

To date, the treatment of choice for such fistulae is complete surgical excision of the tract and any associated cyst, either through a traditional transverse cervical incision or via an endoscopic retroauricular approach. Infective exacerbations should always be treated before surgery.

References

(1.) Goff CJ, Allred C, Glade RS. Current management of congenital branchial deft cysts, sinuses, and fistulae. Curr Opin Otolaryngol Head Neck Surg 2012;20(6):533-9.

(2.) Schroeder JW Jr., Mohyuddin N, Maddalozzo J. Branchial anomalies in the pediatric population. Otolaryngol Head Neck Surg 2007;137(2):289-95.

(3.) Agaton-Bonilla FC, Gay-Escoda C. Diagnosis and treatment of branchial cleft cysts and fistulae. A retrospective study of 183 patients. Int J Oral Maxillofac Surg 1996;25(6):449-52.

(4.) Maddalozzo J, Rastatter JC, Dreyfuss HF, et al. The second branchial cleft fistula. Int J Pediatr Otorhinolaryngol 2012;76(7):1042-5.

(5.) Waldhausen JH. Branchial cleft and arch anomalies in children. Semin Pediatr Surg 2006;15(2):64-9.

Yi-Fang Lee, MD; En-Li Shiau, MD

* Video shows the synchronous movement of the bilateral cervical skin openings, with mucus-like discharge coming out intermittently.

From the Department of Otorhinolaryngology, Taipei Veterans General Hospital, Taipei, Taiwan (Dr. Lee); and the Department of Radiology, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan (Dr. Shiau).

Caption: Figure. A: Photograph shows the bilateral second branchial cleft fistulae, with the external openings over the bilateral neck (arrows). B: Esophagography shows contrast leaking into the ipsilateral cervical opening through a tractfrom the right tonsillar fossa (arrow). C and D: Fistulography clearly demonstrates the tracts (arrowheads) from the bilateral tonsillar fossa to the bilateral cervical skin openings.
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Title Annotation:PEDIATRIC OTOLARYNGOLOGY CLINIC
Author:Lee, Yi-Fang; Shiau, En-Li
Publication:Ear, Nose and Throat Journal
Article Type:Clinical report
Date:Jul 1, 2017
Words:732
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