von Recklinghausen病にともなった下顎後退症の1例

抄録

von Recklinghausen's disease is characterized by cafe-au-lait spots and multipleneurofibromas as major findings and eye lesions, bone lesions, and endocrine abnormalities as associated findings. A 23-year-old male with von Rechlinghausen's disease associated with mandibular retrognathia was reported. He visited our clinic due to malocclusion and retrognathia.
In the abdomen and the back, multiple small tumors and café-au-lait spots were observed. Severe deep bite (overbite: 7mm; overjet: 13mm) and narrowing of the maxillary and mandibular dental arches were noted. The interincisal distance was 41 mm, and the maximum ranges of the mandibular movements were 10 mm anteriorly, 9.5 mm to the right, and 12.0mm to the left. Microglossia showing geographical fissured tongue was observed.
Intraoral radiographic examination revealed congenital dental missing of 21|2 and impaction of but no other findings in the jaw characteristic of this disease. He was treated by anterior movement of the mandible (12 mm) via bilateral sagittal split ramus osteotomy of the mandible and overcorrection in consideration of possible relapse after operation (overbite: 1 mm; overjet: 1 mm). Subsequently, occlusion was restored by prosthetic treatment. The final measurement values 6months after operation were as follows: overbite: 4.5 mm; overjet: 5 mm; interincisal distance: 41 mm; amount of jaw movements: 7 mm anteriorly, 10 mm on theright side, and 10 mm on the left side. After that, skeletal relapse was not noted. However, atrophic changes and underdevelopment of the mandible appear to be involved in the development of retrognathism in this case.