Fissura congênita cervical da linha média

Abstract

Rodriguez AHR et al. Congenital midline cervical cleft Fissura congênita cervical da linha média ABSTRACT Congenital midline cervical cleft is a deformity known by several names. Although its em bryologic origin has not been clearly established, the most widely accepted etiology is impaired midline fusion of the distal branchial arches. The main characteristic of this malformation is a protruding lesion at the midline of the anterior neck, between the chin and the suprasternal notch. Complete surgical resection of the lesion is recommended, with repair of the defect using W-plasty or Z-plasty. This article reports the case of a 38-year-old patient with congenital midline cervical cleft. A simple Z-plasty was carried out after excision of the lesion, and a satisfactory aesthetic and functional result was achieved. Congenital midline cervical cleft is a rare abnormality; however, early diagnosis and appropriate surgical treatment are necessary to avoid changes in the development of the face and limitation of neck extension.

Congenital midline cervical cleft: a case report

“…A delay in this could result in ectodermal cells with underlying mesodermal cells being deposited in the ventral aspect of the neck. These would continue to differentiate and form skeletal muscle (tongue derivative), salivary glandular tissue, and a mucosal surface, thus resulting in the MCC defect [3, 8]. This second theory explains the absence of hair follicles or sweat or sebaceous glands.…”

“…Usually, a mucus-secreting opening of the fistulous tract can be found in the most caudal portion of the cleft. Palpation reveals a subcutaneous fibrous cord that extends to the entire cleft or part of it, from the submentonian region to the suprasternal notch [3]. The sinus should be probed and the blind end visualized by radiography.…”

“…Salivary gland tissue and pseudostratified ciliated columnar epithelium are observed in the fistulous tract and in the cyst. In the area of the nipple-like protuberance, there is normal skin, but cartilage and muscle can also be found [3, 10].…”

“…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].…”

“…MCC is diagnosed by clinical examination, and the main characteristic is a protruding lesion at the midline of the anterior region of the neck, between the chin and the suprasternal notch [3]. The cardinal diagnostic features are: (a) fistula opening is located caudally, (b) intermittent serous fluid discharge in the early neonatal period, (c) nipple-like appearance of the cleft in the superior aspect, and (d) widened scar and minimal neck contracture in later life [6].…”