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They can occur as cysts, sinuses, and fistulas found in the head and neck related to incomplete obliteration of the first, second, third, or fourth (extremely rare) branchial clefts during early fetal development. Cysts have mucosal or epithelial lining but no external openings. Sinuses may communicate either externally with the skin or internally with the pharynx. Fistulae, however, connect to both.
A complete fistula or sinus presents with intermittent drainage of a mucoid fluid on the neck. A cyst typically presents later with a mass (sterile or infected). Complete excision is the goal because of the risks of infection, enlargement, or malignancy. Elective excision is recommended beyond 3–6 months of age. An infected branchial cleft remnant is preferably treated with systemic antibiotics and aspiration; resection is performed after infection resolution.
Second branchial cleft anomalies are by far the most common. The typical presentation is a small draining cutaneous pit along the anterior border of the lower sternocleidomastoid muscle. The sinus tract travels through the carotid bifurcation, over the glossopharyngeal and hypoglossal nerves, and enters the tonsillar fossa of the pharynx.
Branchial Cleft | Internal Opening | Exterior Opening | Frequency |
---|---|---|---|
First | External auditory canal | Angle of the jaw | 8% |
Second | Tonsillar fossa | Anterior border of SCM | 90% |
Third | Piriform sinus | Suprasternal notch | <1% |
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