Branchial Cleft Cysts Sinuses and Fistulae

Branchial apparatus anomalies are lateral cervical lesions that result from congenital developmental defects arising from the primitive branchial arches, clefts and pouches.

The branchial apparatus appears around the 4th week of gestation and gives rise to multiple structures or derivatives of the ears, face, oral cavity and neck. These structures are described in more detail in other sources [126]. Anatomically, the branchial apparatus consists of a paired series of six arches, five internal pouches and five external clefts or grooves. The external grooves are of ectodermal origin and are called branchial clefts. The internal pouches are of endodermal origin and are known as pharyngeal pouches; they are separated by their branchial plates [126]. Each branchial arch is supplied by an artery and a nerve and develops into well-defined muscles, bone and cartilage. Thus, all three germ-cell layers contribute to formation of the branchial apparatus. The arches are numbered 1-6, from cranial to caudal, and the clefts and pouches 1-5. The corresponding cleft and pouch lie immediately caudal to their numerical arch, that is, the first cleft and pouch lie between the first and second arches, the second cleft and pouch lie between the second and third arches, and so on.

A number of theories exist to explain the genesis of branchial cleft anomalies. Regauer and associates have proposed that the cysts arise from the endodermally derived second branchial pouch [95]. An alternative explanation is that the cysts develop from cystic epithelial inclusions in lymph nodes that are either of salivary gland origin or from displaced epithelium from the palatine tonsil [43]. Golledge and Ellis recently reviewed the various theories on the histogenesis of branchial cleft cysts [43].

Papers dealing with anomalies of the branchial apparatus do not always distinguish between the terms sinus and fistula and often use them interchangeably as synonyms. A sinus is a tract that has only one opening, either cutaneous or mucosal. A fistula is a tract that has two openings, one cutaneous and one mucosal. A cyst may occur independently or in association with a sinus or fistula.

Most anomalies of the branchial apparatus of concern to the surgical pathologist present clinically as a cyst, fistula, sinus or skin tag. Fistulae, sinuses and skin tags occur in younger patients than cysts do [20]. Bran-

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