Segmental odontomaxillary dysplasia — a rare disorder

Abstract

Segmental odontomaxillary dysplasia (SOD) is a rare developmental disorder of the maxilla characterised by abnormal growth and maturation of bone, teeth and gingival of the affected segment. Due to the rarity of the disorder, the full range of clinical manifestations is unknown. Since there are concerns that cases may be subject to misdiagnosis, this report aims to increase awareness of the condition among paediatric dentists. A 7-year-old boy was initially referred regarding failure of eruption of maxillary primary molars. Characteristic clinical and radiographic findings led to the diagnosis of SOD. Interestingly, the patient presented with unilateral ectopic eyelashes, a finding previously unreported in association with this condition. Reassurance was provided regarding the benign nature of the condition. While no active intervention was needed at the time of diagnosis, close monitoring is essential. Future management may require surgical, orthodontic and prosthetic input. A period of four years elapsed between initial presentation of the condition and its definitive diagnosis. No significant increase in the unilateral maxillary swelling was noted. The patient will continue to be monitored on a regular basis. SOD is a rare cause of facial asymmetry. Prompt diagnosis can reassure both patient and healthcare professionals. Reporting of cases is encouraged to help determine the full range of clinical manifestations and establish a management protocol.