Combined cutaneous findings with segmental odontomaxillary dysplasia: Review of the literature and proposal of a new clinical classification

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Authors: Othman M Yassin, Farouk B Rihani

Published Date November 2008 , Volume 2008:1

Othman M Yassin¹, Farouk B Rihani²

¹Consultant in pediatric dentistry, Chairman of Pediatric Dentistry Specialty at King Hussien Medical Center, Royal Medical Services, Amman, Jordan; ²Specialist in pediatric dentistry, Prince Rashid Bin AL-Hassan Hospital, Royal Medical Services, Irbid, Jordan

Abstract: Segmental odontomaxillary dysplasia is characterized by variability of its clinical and radiological features, and may mimic other fibro-osseous lesions. We describe the case of a segmental odontomaxillary dysplasia in a 12-year-old male comprising of dermal, gingival, bony, and dental abnormalities. He presented with left midfacial diffuse hyperkeratotic erythematous lesion, ipsilateral hypopigmentation of upper lip with indistinct vermilion border, left-sided facial swelling, unilateral maxillary enlargement and ipsilateral failure of eruption of permanent teeth. Radiographic imaging showed abnormal bony trabeculation, impacted and missing teeth. Histopathologic findings showed characteristic features of segmental odontomaxillary dysplasia. We herein report a case of this rare unusual anomaly, review the literature, and propose a new clinical classification based on the limited number of previously reported cases in an attempt to categorize the clinical variants of the condition which might be helpful when treatment options are to be considered. Clinicians should be aware of its presence when encountering patients presenting with a facial cutaneous lesion especially when it is associated with facial asymmetry and unresponsive to treatment.

Keywords: hemimaxillofacial dysplasia, segmental odontomaxillary dysplasia, facial asymmetry, maxillofacial developmental anomaly