Abstract
Objectives Congenital midline cervical cleft (CMCC) is a very uncommon congenital anomaly of the midline anterior neck, and although it has very pathognomonic features (including nipple-like protuberance), it could be mistaken for other congenital neck lesions, such as thyroglossal duct cyst and branchial apparatus anomalies. Thus, it represents a challenging diagnosis. In this 21-patient series, we discuss the clinical features of CMCC, its pathophysiology characteristics, and its modalities management. Material and Methods We conducted a retrospective chart review of children presenting with CMCC at our institution, between January 1998 and January 2016. Results Twenty-one patients were identified with CMCC. Ages ranged between 1 day and 14 years. The length of the lesion varied from 0.5 to 5 cm, and the size of the skin tag varied from 0.2 to 1.5cm. No other significant associated anomalies were found. Surgery was the mainstay treatment, and no recurrence was found. W-plasty was used in most patients to close the defect. Conclusion With a little more than 200 published cases, our series represents the largest series worldwide. The lesion is usually isolated, and no further investigation is required. Surgery is the mainstay of treatment, with complete excision being usually curative. It should be treated at an early age to prevent complications. In our experience, W-plasty was a good alternative to the most commonly used Z-plasty, in skin closure, with respect to both aesthetic and functional results.
Highlights
Congenital midline cervical cleft (CMCC) is a very uncommon congenital anomaly of the anterior neck that has very characteristic features: (1) a nipple-like protuberance, (2) a skin defect in the middle with palpable subcutaneous fibrous cord, and (3) a blind sinus at the end
Congenital midline cervical cleft is a rare malformation of the midline anterior neck
We operated on 380 patients with a thyroglossal duct cyst, and only 21 patients had a CMCC over the same period of the study (18 years)
Summary
Congenital midline cervical cleft (CMCC) is a very uncommon congenital anomaly of the anterior neck that has very characteristic features: (1) a nipple-like protuberance (skin tag), (2) a skin defect in the middle with palpable subcutaneous fibrous cord, and (3) a blind sinus at the end. First described by Luschka in 1848 [1] and first reported in the English literature by Bailey in 1924 [2], it represents a variant (caudal extension) of cleft number 30 in Tessier’s classification of craniofacial clefts [3]. The branchial arches grow medially in a cephalad to caudal direction with the first arch closing initially, followed by the others subsequently. Many theories have been proposed, including failure of fusion of the 1st and 2nd branchial arches in the midline during the third and fourth weeks of gestational age [4]. The standard procedure for CMCC closure is Z-plasty. There is some controversy over what procedure is best for CMCC. There have been a few case reports about
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