抄録
von Recklinghausen's disease (neurofibromatosis type I) is a chromosomal dominant hereditary disease, characterized by café-au-lait spots of the skin and multiple neurofibroma. Importantly, the neurofibroma arising in the oral and maxillofacial region is known to result in a jaw deformity. We report a case of facial asymmetry which may have been due to the neurofibroma of von Recklinghausen's disease and the repeated surgical intervention for it. The patient was a 20-year-old male. He had repeatedly undergone surgical extirpation of a tumor in the left parotid gland region since he was 3 years old. In the present findings, his face was asymmetric and his dental arch was narrow because of the mandibular deviation and the soft tissue mass in the left mandibular region. Radiographic examinations revealed a bone defect at the mandibular angle and atrophy of the left ramus. To improve these problems, distraction osteogenesis to expand the dental arches, and two-jaw surgery and iliac bone graft to restore the skeletal asymmetry were performed. Upon follow-up one year later, skeletal relapse was not noted.
