Congenital midline cervical cleft (CMCC) is a rare defect of the midline neck which is often misdiagnosed. The aim of this study is to show the efficacy and safety of a novel surgical technique for CMCC to get excellent aesthetic and functional results. We report a series of four cases, two female and two male patients, affected by midline cervical cleft who underwent surgical excision and primary reconstruction of the neck defect through a double opposing Z-plasty, at the Pediatric Plastic and Maxillo-Facial Surgery Unit of a third-level referral hospital in Rome. No complications nor recurrences were noted; cosmetic results were satisfactory in all cases with progressive scar improvement. Early and complete surgical excision is essential to ensure good aesthetical results. The double opposing Z-plasty closure allowed us to obtain an excellent functional outcome and reduce cervical contracture.

Congenital midline cervical cleft (CMCC) is a rare developmental anomaly of the anterior neck, often misdiagnosed due to its similarity to other cervical malformations. It results from impaired midline fusion of the branchial arches, leading to a linear skin defect with a fibrotic cord and, in some cases, a sinus tract. Left untreated, CMCC can cause progressive contracture, restricted neck mobility, and aesthetic deformities. This review examines the embryological basis, clinical presentation, histopathological characteristics, differential diagnosis, and surgical management of CMCC, with a focus on Z-plasty as the preferred reconstructive technique. Z-plasty effectively lengthens the scar, prevents recurrent contracture, and restores normal neck contour. In addition, we present a case of a 3-day-old female neonate with CMCC, successfully treated with Z-plasty reconstruction, reinforcing the importance of early intervention. Emerging genetic research suggests a potential hereditary component in CMCC, warranting further investigation into its molecular underpinnings. Advances in regenerative medicine and surgical innovation may improve treatment outcomes, offering new possibilities for personalized management of congenital cervical anomalies.

Congenital midline cervical cleft (CMCC) is an extraordinarily rare midline lesion that is present at birth. The lesion can cause micrognathia and cervical contracture via underlying traction on the mandible. It is essential to recognize the condition and excise the lesion within 1 year of diagnosis to mitigate long term cosmetic and functional problems. We report a 10-month-old male infant with CMCC diagnosed immediately after birth. Ultrasound with color doppler imaging confirmed the anatomic characteristics of the lesion. The lesion was surgically excised and closed in layers, with no postoperative complications and good functional and cosmetic results. The pathognomonic presentation, etiology, differential diagnosis, and optimal surgical approach are also discussed.

Congenital midline cervical cleft is a rare anomaly classified as a malformation of the branchial arches and represents less than 2% of congenital cervical malformations. Its clinical presentation involves cervical midline deformities: cephalic nodular lesion, linear groove with atrophic surface, and/or caudal sinus. Other midline alterations of variable complexity may also be present. Early treatment allows for avoiding long-term complications. Based on our experience in four clinical cases, a performed literature search on the topic in the last twenty years, and subsequent discussion of the employed surgical approaches, we included 150 reported cases in our review. Correct diagnosis and early treatment with complete removal of the fibrous midline band is paramount to avoid patient complaints until adolescence or adulthood.

本文报道河南省儿童医院收治的1例利用改良“Z”形皮瓣转移修复先天性颈部正中裂的病例。患儿为1岁6个月男童，因“自幼发现颈正中部皮肤缺损伴瘘管”来我院治疗，入院后完善颈部彩超、颈部MRI并结合体格检查结果，诊断为先天性颈部正中裂，于全麻下行电子支气管镜下喉气管探查术+颈部正中裂切除术+改良“Z”形皮瓣转移修复术。术后随访10个月，患儿恢复好，颈部无挛缩、运动无受限，瘢痕恢复中。.