Congenital midline cervical cleft presenting as neck contracture

Abstract

Congenital midline cervical cleft is a rare congenital anomaly involving the neck. This developmental anomaly presents at birth with a ventral midline defect of the neck extending for a variable distance from the mandible to the manubrium with a superior nipple-like skin projection and a subcutaneous fibrous cord. Other associated clinical features include thyroglossal duct cysts, cleft lip/mandible/sternum, mandibular spurs, and or bronchogenic cysts. Initially, the cleft is covered by an exudative thin desquamating epithelium which toughens and dries during the following weeks, creating scarring. Caucasian females are more commonly affected than other races. Most of the earlier described cases were in initial years of life. Available literature highlights the possible long-term sequelae but only a few reports have described such a case and associated problems. Through this case report, we describe a delayed and neglected case that presented with neck contracture and protruded chin. Here, we have tried to show the long-term sequelae when the lesion is allowed to heal secondarily during earlier life. In delayed presentation, confusion in diagnosis may occur due to lack of typical features. Most of these lesions have healed with scarring leading to contractures and mandibular abnormalities.