Surgical management of midline cervical cleft

Introduction: Midline Cervical Cleft (MCC) is a midline cervical congenital anomaly that manifests as a vertical cutaneous/subcutaneous defect with abnormal dermal elements as well as an underlying fibrous cord that extends from the sternum to the mentum of the mandible, which can lead to "wry neck" and hypoplastic mandible. The goal of surgical correction of MCC is to provide adequate healthy tissue coverage, as well as restore contour of the anterior neck. The primary treatment modality for midline cervical cleft is surgical. We describe a technique involving complete excision of the fibrous cord, and use of double z-plasty flap in order to create a tension-free closure and restore contour to the anterior neck. Methods: Using a database search method, children with the clinical diagnosis of midline cervical cleft treated between 2006 and 2016 were identified at a pediatric tertiary care center. Chart review was completed to assess for age at surgery, follow up, results, and complications. Results: 12 patients were identified in the Lurie Children's Hospital (LCH) database. 8 patients underwent complete cord excision by the seniorauthor using the double z-plasty (DZ) technique for closure, with no recurrences. 4 patients underwent linear closure by another surgeon, had persistent contracture, and underwent revision using the DZ technique by the senior author, with no recurrence. Average age of surgery was 9.5 months. Most common post op complication was hypertrophic scar (3/12). Recurrence was only seen in the linear closure cases (4/12). Conclusions: Midline Cervical Cleft is a rare entity with less than 200 cases in the literature. We believe the double z-plasty closure and complete excision of the fibrous cord results in reproducible restoration of neck contour and prevents cord recurrence, and should be considered the standard method for surgical excision of MCC.