Clinical Paper
Craniofacial Anomalies
Facial morphology and growth following surgery for congenital midline cervical cleft patients

https://doi.org/10.1016/j.ijom.2017.12.006Get rights and content

Abstract

Congenital midline cervical cleft (CMCC) is a rare condition that consists of a cutaneous midline neck lesion with a sinus extending inferiorly towards the sternum. A fibrous band that extends superiorly to the mandible is a consistent feature of the condition. Restriction of growth of the mandible, possibly due to incomplete removal of the band, is the most significant long-term problem. It remains unclear whether early removal of the fibrous band might allow catch-up growth of the mandible. This study utilized non-invasive three-dimensional photographs to objectively evaluate the facial growth of six CMCC patients. The growth of these CMCC patients was compared to the average growth of age- and sex-matched controls from a database of three-dimensional facial photographs of clinically normal subjects. After surgical removal of the fibrous cord, CMCC patients experience growth in the chin at the same rate as in the normal population; no evidence was found for catch-up growth. As a result, individuals with CMCC are likely to require further surgical intervention to correct the residual retrognathia on completion of facial growth. Early excision of the lesion including aggressive resection of the fibrous band is still recommended, as this should optimize the early growth of the mandible in infancy.

Section snippets

Case series

All cases of CMCC treated at the Royal Children’s Hospital in Melbourne, Australia over a 10-year period were reviewed. The diagnosis was made clinically based on the presence of classic features of CMCC: a superior nipple-like skin tag and a reddened strip of atrophic skin occurring at any level between the chin and the sternal notch, with a sinus tract at the caudal end. A typical clinical case is shown in Fig. 1.

Image acquisition

Children were routinely photographed prior to surgery and at follow-up

Results

Six patients with a midline cervical cleft underwent surgical treatment between 2006 and 2016. None of the patients had any other associated major congenital anomalies. A clinical photograph was used to illustrate the ‘tethering effect’ of the lesion to the mandible (Fig. 2). In all cases, the patient underwent attempted complete excision of all of the pathological tissue, including the attenuated skin and the fibrous bands beneath the surface. All surgical procedures were performed by one

Discussion

Congenital midline cervical cleft (CMCC) is a rare congenital anomaly1, 16, 17. Most clinicians are likely to see only one or two cases in a lifetime, and so empirical clinical trial data are difficult to collect. Therefore, treatment planning must be based on limited empirical evidence. In this study, dense morphometric techniques were used to assess facial growth following the surgical removal of the pathological band connecting the neck structures to the mandible. It was found that anterior

Funding

None.

Competing interests

None.

Ethical approval

Approval for the study was obtained from the Human Research Ethics Committee of the Royal Children’s Hospital (36114A).

Patient consent

Written patient consent was obtained to publish the clinical photographs.

Acknowledgements

The authors would like to thank all of the patients and their parents who participated in this study.

References (18)

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