Congenital midline cervical cleft.

We present a case of congenital midline cervical cleft (CMCC), a sporadic lesion with a predilection for white females. It characteristically occurs in the midline ventral neck and consists of a cephalad skin tag, a mucosal surface, and a caudal sinus. There is an associated submucosal fibrous cord and interwoven bundles of skeletal muscle. This is occasionally tethered to the mandible and/or sternum causing neck contractures. CMCC is usually isolated, but rare cases have been associated with midline clefts of the tongue, lower lip, mandible, and sternum. The embryogenesis is unclear but arguments are given here that CMCC represents ectopic first branchial arch derivatives, including the lower lip and/or tongue.