Pterygium colli medianum and midline cervical cleft: Midline anomalies in the sense of a developmental field defect

Abstract

We report on 2 patients (and review a third) with a vertical midline neck web which extends from the lower symphysis of the mandible to a variable extent down to the jugular notch. This uncommon congenital anomaly, previously reported primarily in the (plastic) surgery literature, is interpreted as the mildest form and most dorsal extension of anterior midline cleft defects which include the supraumbilical raphé and cleft sternum with or without congenital hemangiomata. By analogy with the former, and the fact that in a boy with a severe cervical midline web there was a cleft of the upper sternum, we interpret this neck-web as a developmental field defect. It is a sporadic anomaly but may be associated with other midline malformations including congenital heart defect(s). Thus, finding such a web at birth should prompt a careful search for other anomalies.

Congenital midline cervical cleft: A practical approach to Z-plasty closure

“…Failure to recognize the stigmata of this abnormality and delay of surgical management will lead to cicatrical contracture and potentially mandibular hypoplasia [10]. No general consensus exists as to an appropriate age for elective surgical resection of CMCC.…”

A case of untreated midline cervical cleft associated with congenital heart defect

“…The purpose of early surgery is to avoid cicatrical contractures and mandibular deformities such as exostosis-like protuberances, both of which are more likely to occur as the child gets older. [4,7,8] Many authors suggest that a proper time for the surgery in children is before the second birthday, with earlier repair being indicated in more severe cases. Delayed surgical treatment of such congenital anomaly can lead to recurrence of neck contracture as described in our case report.…”

“…It can be associated with cleft of lower lip or cleft tongue and rarely with a bronchogenic cyst or with respiratory epithelium, hemangiomata. [4] Associated congenital heart defects have been reported occasionally, although most of these cases have been associated with cleft sternum [4] or ectopia with intracardiac anomalies. [5,6] …”

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

“…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.…”