Abstract
Basal cell nevus syndrome (BCNS) is a rare genetic disorder that can be caused by mutation of multiple genes, including PTCH1, PTCH2, and SUFU, in an autosomal dominant manner. The symptoms include some craniofacial features such as keratocystic odontogenic tumors (KCOTs), macrocephaly, and cleft lip and/or palate. Although comprehensive orthodontic treatment is frequently required for some of these craniofacial deformities, there are few reports that show the outcomes of comprehensive orthodontic treatment. Here, we report a case of BCNS with multiple KCOTs, macrocephaly, skeletal class III malocclusion, asymmetric dental arch, and mandibular crowding, which was successfully treated with comprehensive orthodontic treatment.
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