The first case of CMCC was recorded in 1848 by Luschka, while Bailey documented the first description of this abnormality in 1924 [1,2]. Occasionally it associated with other developmental defects including bifid mandible and micrognathia, clefting of the sternum and possible loss of other midline structures, such as portions of hyoid bone.

Embryologically impaired fusion of brachial arches is considered most accepted theory. CMCCs are noticed at birth as a defect at the ventral aspect of neck, with skin tag superiorly and inferior margin formed by a short sinus. No antenatal problems are generally identified and Caucasian women seem to be the most frequently affected.

Nine-month-old male child was brought by parents with a defect in the midline of neck.  Antenatal history including scans was normal.  Baby born by full term normal vaginal delivery. There was no family history of congenital defects or consanguinity. Developmental milestones were appropriate for age. General condition of child was good. There was no evidence of any facial deformity. Systemic examination was normal.

On local examination, the lesion was located on ventral aspect of in the midline. It was extending from hyoid bone to the suprasternal notch. At cephalic end, it had nipple-like skin tag and at caudal end, there was a longitudinal pink colored mucosal surface with fibrotic tissue and sinus (Figure1). The lesion did not move on protrusion of the tongue or on deglutition. There was no contracture of the neck. Examination of face, oral cavity neck was normal. Inspectory findings were confirmed on palpation. The lesion was not fixed to underlying structures. Sinus was confirmed by small lacrimal probe, it was coursing towards suprasternal notch, for around 0.5-1 cm. Our clinical diagnosis of CMCC was confirmed on ultrasonography of neck, which showed a blind ending 1cm long sinus in the subcutaneous plane. The thyroid gland was normal and there was no regional lymph node enlargement. X-ray neck with chest was normal.

Child was planned for surgery. Under general anaesthesia patient was positioned supine, with extension of neck by putting small bolster beneath the shoulders. An elliptical incision was made 1-2 mm from the margins of the lesion. It was deepened down to the supraplatysmal plane. The sinus at the caudal end of the lesion was probed and followed caudally until it ended. It was found to be approximately 1 cm long. Skin tag, sinus tract and fibrous tissue were excised completely (Figure 2). Hemostasis was confirmed. Skin defect was closed by double z-plasties using nonabsorbable, monofilament suture 6-0 in a simple interrupted manner (figure 3). Sterile compression dressing was applied.

Post-operative course was uneventful. There was no evidence of suture line wound infection. Sutures were removed on post-operative day 10 under general anaesthesia. Histological examination showed pseudostratified columner epithelium.

Patient was on regular follow-up after surgery. At 6 month follow-up, there is excellent healing of the z-platy wound. Wide range of neck movements was noted. There is no evidence neck contracture.

Congenital midline cervical cleft constitutes 2% of all congenital cervical malformations [4]. Usually, MCC is associated with a spectrum of midline anomalies related to the brachial arches, including a median cleft of the lower lip and mandible, hypoplasia, or absence of other midline neck structures. Multiple theories have been postulated; the most accepted being lack of fusion of first and second brachial arch. A male to female ratio of 1:2 is reported, with sporadic presentation. The age of presentation ranges from birth to 23 years [5]

It may be associated with other congenital abnormalities including, ectopic bronchogenic cyst, thyroglossal duct cyst [6], cleft sternum, tongue, mandible, and lower lip. The prevalence of CMCC with other neck malformations like thyroglossal cyst and brachial cleft sinuses is 1.7%.

CMCC is diagnosed by clinical examination. Diagnostic characteristic is a nipple like protruding lesion in the midline of anterior region of the neck, between the chin and suprasternal notch. This lesion extends caudally as a longitudinal pink mucosa with sinus [7].

Treatment of CMCC involves surgical resection and proper reconstruction. Since primary closure leads to an unsightly scar and restriction of neck movements after verticle closure, reconstruction of the defect needs surgical maneuvers other than primary closure. Z-plasty is one of the surgical procedures used to close the defect. Single, double or multiple Z-plasty is the surgical method of choice for CMCC. The Z-plasty technique involves creating two opposing triangular transposition flaps that are rotated synchronously to close a central defect by redistribution and rearrangement of tissue [9]. The Z-plasty is symmetrically designed so that the lateral limbs are equal in length to the tissue defect (central limb) and that the angles between the lateral limbs and the central limb are 60°.

If left untreated, there are chances of neck contracture and functional compromise or secondary anatomical disarrangement in the form of micrognathia or bony spur (exostosis) of the mandible or sternum.

Many authors suggest early excision of lesion mainly before 2 years of age. Early repair prevents contracture and cosmetic deformities.  Ercocen et al suggested that early intervention avoids the disfiguring appearance of malformation. Derbez et al reported five cases of CMCC. All were treated in early life at age ranging from 1 month to 2 years. Given the rarity of CMCC, we decided to report this case. Our findings are consistent with the literature. Our patient has recovered uneventfully after successful surgical reconstruction by double Z-plasty and has a fine, cosmetic scar and full-range of neck movements.

CMCC is an unusual congenital defect that may be encountered by the pediatrician, pediatric surgeon or other health care professionals caring for children. Early surgical excision and reconstruction of the entire defect with multiple Z-plasty reconstruction yields good cosmetic results while improving neck mobility and reducing the risk for long-term cervical tethering and mandibular defects.

Conflicts of Interest

There are no conflicts of interest.

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