大臼歯の萌出遅延を伴ったvon Recklinghausen病の1例

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  • A Case of von Recklinghausen's Disease with Delayed Eruption of Molars.

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An 11-year-old female visited our facility complaining of delayed eruption of the first and second molars on the left maxilla. Diffuse swelling of the left cheek was found, and a cafe-au-lait spot was detected. Multiple café-au-lait spots were also observed on the right hand, abdomen, and axilla. An intraoral, bony swelling was found in the left maxillary molar lesion. Alkaline phosphatase levels were high, at 878 IU. She had not yet menstruated. Bony changes were not found on radiographic examination. MRI and CT scans, however, revealed areas of soft tissue density in the left maxilla and cheek, with slight enhancement. Her mother, grandfather, younger brother, and sister had previously been diagnosed as having von Recklinghausen's disease. They had no intraoral symptoms.<BR>She was diagnosed as having von Recklinghausen's disease with a diffuse plexiform neurofibroma, and was treated by resection of the tumors in the maxilla, and biopsy of the cheek lesion. The histopathological diagnoses were neurofibromas. Subsequently, resection of the cheek tumor was performed to prevent facial deformity. Subsequently, the left first molar erupted in 1 year, and good facial symmetry was maintained. However, careful observation continues to be required.

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