Segmental odontomaxillary dysplasia (hemimaxillofacial dysplasia): case report.

Segmental odontomaxillary dysplasia (SOD) has been delineated from other bone and odontogenic dysplasias, and only recently has been described as a separate entity.' This condition was first introduced as hemimaxillofacial dysplasia (HD) by Miles et al. in 1987.' Reports have appeared directed primarily at oral and maxillofacial surgeons and oral pathologists, so the condition may be relatively unknown to pediatric dentists. SOD is characterized by unilateral maxillary enlargement, gingival hyperplasia, facial asymmetry, ipsilateral dental abnormalities, and an unusual radiographic bone pattern. Immature woven bone forming irregular patterns is seen histologically. To date 10 cases have been reported, and in one patient, ipsilateral facial hypertrichosis was recorded. With one exception, all patients have been children or adolescents. None of the cases demonstrated a hereditary pattern. The incidence is unknown. Pediatric dentists and orthodontists who have encountered these patients may have misinterpreted the condition as hereditary gingival fibromatosis because of the maxillary enlargement and thickened gingivae. The poor-quality bone encountered on biopsy may have suggested atypical fibrous dysplasia. This case is reported in the pediatric dental literature in an effort to encourage the reporting of additional cases and define the range of clinical manifestations of this unusual condition.