Congenital Midline Cervical Cleft: A 7 Year Prospective Study

Abstract

Congenital cervical midline cleft (CCMC) is a rare disease of the head and neck region with only a few reported series. The patients attending the ENT or Plastic surgery OPD of a tertiary referral hospital of eastern India were screened for the presence of CCMC. All the patients found to have the developmental abnormality were included in the study and treated. Five patients were identified as having CCMC within the 7 year study duration. Of them three were female and two male. The youngest patient was a 2 day old baby and 25 years was the maximum age of presentation in the present series. All except the new born were treated by surgical excision and primary repair of the neck defect. In literature CCMC has been described almost exclusively in the Caucasian population. The present series documents the occurrence of this developmental abnormality in the Indian population. This is perhaps the first case series on CCMC to be presented from the Indian subcontinent.

Midline Cervical Cleft: An Anatomical Finding and a Proposal for a New Approach

“…Bergevin et al (1989) described the skin protuberance as a vertical outgrowth of the tongue muscle, since they found the fibrous cord was related to the fibrous median septum of the tongue base; salivary glands in the sinus tract and mucoid discharge contribute to this theory. Vascular anomalies causing ischemia, persistence of thyroglossal duct, and increased pericardial roof pressure are other embryological hypotheses (Genç et al, 2002;Saha et al, 2013).…”

“…Midline cervical cleft has a prevalence of 2/1 in females. The most affected race is Caucasian (Saha et al, 2013). Etiology is thought to be related to a lack of fusion of the first and second branchial arches at an early stage of development (Saha et al, 2013).…”

“…The most affected race is Caucasian (Saha et al, 2013). Etiology is thought to be related to a lack of fusion of the first and second branchial arches at an early stage of development (Saha et al, 2013). Abnormal migration of mesodermal cells between the branchial arches or a failure of the mesenchyme to penetrate the midline could be other explanation (Genç et al, 2002;Hernan et al, 2012).…”

Congenital Midline Cervical Cleft and W-Plasty: Our Experience

“…In their retrospective case series of 8 patients, Achard et al [17] found only one patient with an atrophic kidney. Some authors found that CMCC can be associated with other anomalies like thyroglossal duct cyst [21], bronchogenic cyst [22], or a midline cleft from mandible to sternum [23].…”

“…Although in the literature Z-plasty is the most commonly used way of incision closure in CMCC [17, 23, 24], we mostly used W-plasty (in 18 patients). In our experience, we found that it gave long-term satisfactory cosmetic and functional results.…”