Congenital midline cervical cleft presenting as neck contracture

Congenital midline cervical cleft presenting as neck contracture Congenital midline cervical cleft is a rare congenital anomaly involving the neck. This developmental anomaly presents at birth with a ventral midline defect of the neck extending for a variable distance from the mandible to the manubrium with a superior nipple-like skin projection and a subcutaneous fibrous cord. Other associated clinical features include thyroglossal duct cysts, cleft lip/mandible/sternum, mandibular spurs, and or bronchogenic cysts. Initially, the cleft is covered by an exudative thin desquamating epithelium which toughens and dries during the following weeks, creating scarring. Caucasian females are more commonly affected than other races. Most of the earlier described cases were in initial years of life. Available literature highlights the possible long-term sequelae but only a few reports have described such a case and associated problems. Through this case report, we describe a delayed and neglected case that presented with neck contracture and protruded chin. Here, we have tried to show the long-term sequelae when the lesion is allowed to heal secondarily during earlier life. In delayed presentation, confusion in diagnosis may occur due to lack of typical features. Most of these lesions have healed with scarring leading to contractures and mandibular abnormalities. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png European Journal of Plastic Surgery Springer Journals

Congenital midline cervical cleft presenting as neck contracture

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Publisher
Springer Journals
Copyright
Copyright © 2009 by Springer-Verlag
Subject
Medicine & Public Health; Plastic Surgery
ISSN
0930-343X
eISSN
1435-0130
D.O.I.
10.1007/s00238-009-0342-7
Publisher site
See Article on Publisher Site

Abstract

Congenital midline cervical cleft is a rare congenital anomaly involving the neck. This developmental anomaly presents at birth with a ventral midline defect of the neck extending for a variable distance from the mandible to the manubrium with a superior nipple-like skin projection and a subcutaneous fibrous cord. Other associated clinical features include thyroglossal duct cysts, cleft lip/mandible/sternum, mandibular spurs, and or bronchogenic cysts. Initially, the cleft is covered by an exudative thin desquamating epithelium which toughens and dries during the following weeks, creating scarring. Caucasian females are more commonly affected than other races. Most of the earlier described cases were in initial years of life. Available literature highlights the possible long-term sequelae but only a few reports have described such a case and associated problems. Through this case report, we describe a delayed and neglected case that presented with neck contracture and protruded chin. Here, we have tried to show the long-term sequelae when the lesion is allowed to heal secondarily during earlier life. In delayed presentation, confusion in diagnosis may occur due to lack of typical features. Most of these lesions have healed with scarring leading to contractures and mandibular abnormalities.

Journal

European Journal of Plastic SurgerySpringer Journals

Published: Oct 1, 2009

References

  • Congenital mid-line cervical cleft: case report and review of literature
    Mlynarek, A; Hagr, A; Tewfik, TL; Nguyenc, VH
  • Congenital midline cervical cleft: a practical approach to Z-plasty closure
    Cochran, CS; Defatta, RJ; Brenski, AC

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