Atypical Hemifacial Microsomia with disrupted development of ramus

Abstract

Atypical Hemifacial Microsomia with disrupted development of ramus(Case Report)Eyüp DeğirmencioğluIstanbul University Faculty of Dentistry Department of Orthodontics, Istanbul, TurkeyAIM: The aim of this report is to describe an unusual case of hemifacial microsomia with its characteristic clinical and radiographic findings with an effort towards improving the knowledge and an attempt to differentiate it from other similar syndromes.CASE: The male patient with a calendar age of 12 years and a skeletal age of 12 years and 6 months (prepeak) was referred to our clinic. Based on the chief complaint and the clinical features a provisional diagnosis of hemifacial microsomia of left side was made. There were no similarities among family members. As a result of consultation with medical genetics, the diagnosis was confirmed. Asymmetry was clear 2 mm upper dental midline deviation to the right, 6.6 mm lower dental midline deviation to the left, 12.40 mm menton deviation to the left. Unilateral cross bite was not observed. Posterior segment between the teeth 36 and 43 deviation was to the lingual, anterior segment deviation was to the mesial. There was an occlusal cant. Radiographic evaluation revealed that normal head of the condyle and glenoid fossa with ramus agenesis. There was an orbital distortion eye and epibullar dermoid in his right eye. He had no ear deformity.CONCLUSIONS: This case draws attention to hemifacial microsomia with atypical findings. Hemifacial microsomy with ramus agenesis is the first case report published in the literature so far.

Keywords: Hemifacial microsomia, Hypoplasic condyle, Ramus agenesis