Distraction Osteogenesis of Nasal Bone Including Piriform Aperture for Syndromic Craniosynostosis

<Backgound>We are going to report a case with syndromic craniosynostosis featuring severe midfacial hypoplasia. A 15-year-old male was referred to our hospital for orthognathic surgery. He was born with craniosynostosis and underwent cranioplasty for early release of the coronal suture areas (with advancement of the frontal bone )at age 1, and cranioplasty for enlargement of total occipital area at 5. He displayed midfacial hypoplasia but discrepancy of occlusion was not remarkable because of dental compensation. Exophthalmos was not observed, either.<Material and Methods>We planned (Le Fort II minus Le Fort I) osteotomy, as we should call it. This osteotomy included nasal bone, lateral side of lacrimal canal, edge of piriform aperture including nasal floor of maxilla. Distraction Osteogenesis(DO)using RED device was performed. <Results>DO was initiated on post operative day(POD)6 with a rate of 1mm/day until POD28. Total distraction distance was about 20mm. In order to remove RED device earlier, distracted bone was fixed by penetrating Kirschner wire from lateral side of zygomatic bone to the other side. At the age of 17, we performed nasal repair using costal cartilage. Shown in (Fig.1)are his configuration and dental occlusion before operation (age 15)and (Fig.2) are those after operation (age　17)<Discussion>Syndromic craniosynositosis has various characteristic physical findings. Our case displayed severe midfacial hypoplasia, but exophthalmos or severe occlusional discrepancy was not observed. We performed nasal and ethmoidal osteotomy with a small incision on root of nose without using coronal incision. And we could improve his feature by distracting nasal area forward. It is suggested that this method might be effective in the treatment of syndromic craniosynostosis featuring severe midfacial hypoplasia without exophthalmos.