Congenital midline cervical cleft and web

Congenital Midline Cervical Cleft: Case Report and Review of the English Language Literature

“…However, there is still the risk of undesirable cosmetic results, as the scar is noticeable and somewhat irregular in appearance. In addition, there is a tendency for hypertrophic scarring in the oblique limbs of the Z‐plasty (5,11). Because our patient was lost to follow‐up, we unfortunately cannot comment as to the potential value of linear midline closure versus Z‐plasty closure, nor can we say whether early intervention in this particular case was beneficial.…”

“…The embryologic mechanism that accounts for CMCC is not firmly established; however, most investigators believe that it involves a failure of the branchial arches to fuse in the midline (2,5,8,11,13,16,18,21,23–26,28,30). During normal embryologic development, the branchial arches begin to merge in a cephalad to caudal fashion during the third and fourth weeks of gestation.…”

“…In addition to its central features, CMCC may be associated with other anomalies of the head and neck, including clefts of the lower lip, mandible, chin, and tongue (2,4,5,(10)(11)(12)20,27), an underdeveloped mandible (5,8), and hypoplasia/absence of supporting structures of the neck, such as the hyoid bone (2,22,27). It has occurred in the presence of, and may even be related to, both bronchogenic cysts (13,16,21,28) and thyroglossal duct abnormalities (1,3,6,15).…”

“…Congenital midline cervical cleft (CMCC) is a rare developmental abnormality of the ventral neck. Fewer than 100 cases have been reported overall with fewer than 50 published in the English language literature (1–28)(Table 1). None have been published in the dermatology literature.…”

Quiz Case 1

“…[1][2][3][4][5] Other associated defects include a cleft lower lip and mandible 7 and an underdeveloped mandible. 8 A subcutaneous fibrous cord associated with the cleft is usually present and, over time, may act as a tether to limit extension of the neck. 9,10 Developmentally, midline cervical clefts represent failure of the branchial arches to fuse in the midline.…”