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Year : 2021  |  Volume : 1  |  Issue : 4  |  Page : 269

Congenital midline cervical cleft – A rare neck anomaly

1 Department of Pediatrics, Army College of Medical Sciences, New Delhi, India
2 Department of Pediatrics, Maulana Azad Medical College, New Delhi, India

Date of Submission19-Sep-2021
Date of Decision11-Nov-2021
Date of Acceptance30-Oct-2021
Date of Web Publication29-Nov-2021

Correspondence Address:
Shipra Agrwal
Department of Paediatrics, Army College of Medical Sciences, Delhi Cantonment, New Delhi - 110 010
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ipcares.ipcares_281_21

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How to cite this article:
Agrwal S, Dabas A. Congenital midline cervical cleft – A rare neck anomaly. Indian Pediatr Case Rep 2021;1:269

How to cite this URL:
Agrwal S, Dabas A. Congenital midline cervical cleft – A rare neck anomaly. Indian Pediatr Case Rep [serial online] 2021 [cited 2022 Jan 20];1:269. Available from: http://www.ipcares.org/text.asp?2021/1/4/269/331376

A 3-month-old, boy presented with a painless neck lesion noted since birth. There were no associated redness, discharge, and difficulties in breathing or breastfeeding. The antenatal, natal, and postnatal periods had been uneventful. Examination revealed stable vital parameters and normal anthropometry. A midline linear cervical cleft, 30 mm in length and 7 mm in width, extended from the submental to the suprasternal area, with atrophic skin at the base. There was a 3 mm × 3 mm nipple-like projection at the cephalic end and a dry sinus tract at the caudal end directed toward the manubrium [Figure 1]. No other midline defects in the lower lip, tongue, oral cavity, jaw, or chest were identified. The ears were normal. Neck movement was not restricted. Systemic examination was normal. A clinical diagnosis of congenital midline cervical cleft (CMCC) was made based on the clinical phenotype. Ultrasonography of the neck confirmed a depth of 2 mm of the tract that had a blind ending. No other anomalies were detected. Abdominal ultrasonography and echocardiography were normal. The parents were counseled, and the child was kept under pediatric surgery follow-up for surgical correction.
Figure 1: Image showing the frontal view of the congenital midline cervical cleft. A midline cervical skin defect extends from the submental to suprasternal area with atrophic pale skin at base (*). A nipple-like projection is seen at the cranial end (white arrow) and a sinus tract is present at the caudal end (black arrow)

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CMCC is rare, constituting about 2% of neck anomalies. It is hypothesized to result from impaired midline fusion of the first and second branchial arches during embryogenesis. The three components differ histologically: the skin tag usually demonstrates normal skin; the cleft is lined by atrophic keratinized stratified squamous epitheliumwithout any skin appendages, and the sinus tract is lined by pseudostratified squamous epithelium with seromucinous glands that often discharge. CMCC can be isolated; associated with clefts in the lower lip, tongue, mandible, sternum, thyroglossal duct cyst, bronchogenic cyst, ectopia cordis, and other intracardiac anomalies, or a component of Brachiootorenal syndrome. Neck contracture may result due to scarring and formation of a fibrous cord during healing. Treatment is surgical excision during infancy with closure of the defect by Z plasty to prevent contractures.[1],[2]

Clinicians should be aware of the four differential diagnoses. Thyroglossal cyst is the most common, a remnant of the thyroglossal duct that normally obliterates by the 5th week of gestation. It appears as a fluid-filled swelling that moves with deglutition and protrusion of the tongue. This pathognomic feature is due to its relation with the hyoid bone but is difficult to appreciate in an infant. Occasionally, it may intermittently discharge the sinus. The tract of the thyroglossal duct anomaly is cranially directed, in contrast to a CMCC which is directed caudally.[1],[3] Branchial sinuses and fistulae are the remnants of embryonic branchial cleft and pouches, usually of the third and fourth branchial arches. These present as cystic swellings or fistulae in the lower neck along the anterior border of the sternocleidomastoid muscle. They may have internal connections with the pharynx or pyriform sinus.[4] Dermoid cysts are superficial, subcutaneous masses or cysts that do not move with deglutition.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patients' parents have given their consent for patients' images and other clinical information to be reported in the journal. The patients' parents understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Crippa BL, Bedeschi MF, Cantarella G, et al. Congenital midline cervical cleft: Clinical approach to a congenital anterior neck defect. Congenit Anom (Kyoto) 2015;55:112-5.  Back to cited text no. 1
Villanueva-Meyer J, Glastonbury C, Marcovici P. Congenital midline cervical cleft. J Radiol Case Rep 2015;9:7-11.  Back to cited text no. 2
Narayana Moorthy S, Arcot R. Thyroglossal duct cyst-more than just an embryological remnant. Indian J Surg 2011;73:28-31.  Back to cited text no. 3
Acierno SP, Waldhausen JH. Congenital cervical cysts, sinuses and fistulae. Otolaryngol Clin North Am 2007;40:161-76.  Back to cited text no. 4


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