Dyke-Davidoff-Masson 증후군 환자의 두개골 변형: 증례보고

Craniofacial Deformity in a Patient with Dyke-Davidoff-Masson Syndrome: A Case Report

Purpose: The Dyke-Davidoff-Masson syndrome is a rare disease entity that was first reported in 1993, and it is characterized by not only the cerebral hemiatrophy that is accompanied by the ipsilateral ventriculomegaly and ipsilateral compensatory osseous hypertrophy, but also the overgrowth of the paranasal sinuses. No studies have attempted to examine it from perspectives of the skull deformity and plastic surgery. Here, we report our case with a review of the literatures. Methods: A 45-year-old man with Dyke-Davidoff-Masson visited our medical institution with nasal bone fracture. Based on the previously taken brain MRI scans, we measured the degree of craniofacial deformity, and the horizontal distance, which is based on the margin of the skull, as well as the falx cerebri. Results: We made a comparison of the degree of craniofacial deformity. This showed that the mean horizontal distance on the axial view was shorter by approximately 28.46%, as compared with that of the left unaffected side. Conclusion: The Dyke-Davidoff-Masson is characterized by a concurrent presence of the atrophy of the cerebral hemisphere, with the cranial deformity. For the reconstruction of the bone and soft-tissue deformity with Dyke-Davidoff-Masson syndrome, it is needed to perform objective assessments.