Midline Congenital Cervical Cleft Mimicking Linear Scleroderma

Abstract

Midline congenital cervical cleft is an extremely uncommon anomaly of the neck. Fewer than 100 cases have been reported. It is usually described as a cervical scar-like skin defect. We present a case of midline cervical cleft mimicking linear morphea and treated with topical steroids for 2 years. This is an unusual presentation of this entity that must be treated with surgical excision to confirm the diagnosis histopathologically.

Midline Cervical Cleft: Review of an Uncommon Entity

“…However, the constellation of clinical and pathologic findings can distinguish between the two entities. The erythematous, linear, and atrophic skin defect of MCC can mimic other skin disorders such as linear scleroderma, but linear scleroderma lacks the other salient characteristics of MCC [ 111 ].…”

“…Those patients in whom the cord is apparent even without neck extension have difficulty extending their necks. When the fibrous cord extends to the level of the mandible, a bony spur is often seen on the anterior, inferior surface of the bone secondary to the traction placed on the mandible by this tethering cord which may be severe enough to produce an open bite deformity [ 11 , 19 , 91 , 102 , 111 ].…”

“…There was almost a direct correlation between the patient's age and the length of the defect ( Figure 1 ). Whereas some previous publications recommended early excision only in those patients in whom the fibrous cord was prominent and severe producing inability to extend the neck or remodeling of the mandible [ 10 , 11 ], early excision is recommended to prevent an increased scar length as well as the problems associated with a tethering midline cord [ 106 , 111 ]. We treated patient surgically at the time of presentation for those over the age of one year and waited until age one for those who presented very early in life.…”

Congenital Midline Cervical Cleft: A very rare clinical entity

“…We like others recommend early surgical excision because of the disfiguring appearance of the neck defect which is clearly apparent. Add to this the fact that delayed treatment will transform the defect into a cervical cord behaving like a cicatrix with tethering and limitation of the extension movement of the neck [5,17,18]. Treatment consists of complete surgical excision of the midline neck cleft and closure of the defect by Z-plasty technique.…”