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Tessier number 7 facial cleft

Disease definition

A rare lateral facial cleft characterized by a temporo-zygomatic defect, usually with absence of the zygomatic arch and deformities of the mandibular ramus, condyle, and coronoid process. Associated soft tissue abnormalities include malformations of the ear and hypoplasia or absence of the temporal muscle. Preauricular hair may be absent or divided into two portions. Facial manifestations include macrostomia (with extension of the cleft to the corner of the mouth) and pre-auricular tags. Incomplete clefts may be found in the molar region and between the maxillary tuberosity and pterygoid process.

ORPHA:141276

Classification level: Disorder
  • Synonym(s):
    • Commissural facial cleft
    • Macrostomia
    • Transverse facial cleft
  • Prevalence: -
  • Inheritance: Autosomal dominant 
  • Age of onset: Antenatal, Neonatal
  • ICD-10: Q18.4
  • OMIM: 613545
  • UMLS: C0024433
  • MeSH: -
  • GARD: -
  • MedDRA: -
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