Congenital Midline Cervical Cleft: A Case Report with Review of Literature

Abstract

Aim: To highlight a rare case of a congenital midline cervical cleft (CMCC) in context with embryological theories/hypothesis, presentation, and management along with review of literature. Introduction: Congenital midline cervical cleft is a rare but interesting anterior neck anomaly with controversial theories/ hypothesis regarding its embryogenesis. Case report: We describe here a classical case of midline cervical cleft that presented at birth with a cephalocaudal orientation, extending from the level below the hyoid bone to the suprasternal notch with a length of 3 cm and width of 0.5 cm. At 6 months of age, the lesion was excised and closure was done by multiple Z-plasty, with satisfactory results. Discussion: Although the diagnosis is clinical, it is frequently misdiagnosed. The associated clinical features could include thyroglossal duct cysts, cleft lip/mandible/sternum, cervical contractures, mandibular spurs, microgenia, and/or bronchogenic cysts. If it is not treated at an early age, it can result in complications like webbing of the neck, dental malocclusion, and restricted neck movements. Conclusion: Earliest recognition of CMCC and proper intervention can provide better esthetic and functional prognosis. Clinical significance: A correct earlier recognition of the lesion and appropriate surgical management are key to avoid longterm complications.

Congenital midline cervical cleft: a case report

“…It is concluded that the skin protuberance could be a vertical outgrowth of tongue muscle, since the fibrous cord is related to the fibrous median septum of the tongue base; salivary glands in the sinus tract and mucoid discharge contribute to the theory. Usually, MCC is associated with a spectrum of midline anomalies related to the branchial arches, including a median cleft of the lower lip and mandible, hypoplasia, or absence of other midline neck structures [2, 4].…”

“…According to a literature search, by 2014 only 205 cases had been reported [1]. The first recorded case of MCC was in 1848 by Luschka, while Bailey documented the first description of this abnormality in 1924 [2, 3]. It is an entirely sporadic lesion that is only occasionally associated with other developmental defects including bifid mandible and microgenia, clefting of the sternum, and a possible loss of other midline structures, such as portions of the hyoid bone [4, 5].…”

“…It is not considered a true cleft because no skin gap exists. It represents a variant of the cleft category number 30 of the Tessier classification system of craniofacial defects [2, 6, 7].…”