Distraction Osteogenesis of Nasal Bone Including Piriform Aperture Rim for

Syndromic Craniosynostosis

Department of Plastic and Reconstructive Surgery, Yokohama City University Medical Center, 1Department of Plastic and Reconstructive Surgery, Kawasaki Medical School, 2Department of Oral and Maxillofacial Surgery, Yokohama City University Medical Center3Department of Orthodontics and Dentofacial Orthopedics, Tokushima University Graduate 　 School of Oral Sciences4Section of Maxillofacial Orthognathics, Department of Maxillofacial Reconstruction and 　　 Function, Graduate School, Tokyo Medical and Dental University

Katsuyuki Torikai, Nagi Nishikori, Hiroki Naganishi,Mayumi Yamamoto 1, Takeshi Kijima, Ikuo Yonemitsu 2, Kazuo Shimazaki 2, Keiichiro Watanabe 3, Eiji Tanaka 3, Keiji Moriyama 4, Susumu Omura 2

Objective of the study• Syndromic craniosynositosis has various

characteristic physical findings.• We are going to report a case with syndromic

craniosynostosis featuring severe midfacial hypoplasia without exophthalmos, who has mild occlusal discrepancy due to dental compensation and characteristic external nose deformity.

Case ； A 15-year-old maleBorn with syndromic craniosynostosis .At age 1 ； Cranioplasty for early release of the coronal suture areas with advancement of the frontal bone. At 5; Another cranioplasty for enlargement of total occipital area .At 15; Referred to our hospital for orthognathic surgery.

Materials and Methods:

15 y.o.

Photos of the 1st visit to our hospital

Preoperative Cephalometric Radiogram and Profilogram

SNASNBANBConvextyL1 to mandibularpl.Interincisalangle

64.770.6- 5.9-10.0

83.8

113.7

Preoperative 3D CT

Operation Planning ；modified Le Fort II – Le Fort I

Distraction osteogenesis of nasal bone including piriform aperture rim was planned.

Intraoperative photos

In order to get total external nose distraction, distracted bone contained antero-inferior piriform edge with nasal floor bone included.

Distraction Osteogenesis (DO)（ RED system ）

DO was initiated on post operative day(POD)6 with a rate of 1mm/day until POD28. Total distraction distance was about 20 mm.

Postoperative Cephalometric Radiograms

In order to remove RED device earlier,distracted bone was fixed by penetrating Kirschner wire (arrow) from lateral side of zygomatic bone to the other side.

Photos after completion of DO

Kirchner wire was removed easily 5 months after completion of DO. Nasal deformity remained and we performed nasal repair twice.

Nasal augmentationusing costal cartilage

16y 8m 17y 5m

Nasal repair including bilateral alar reduction

Pre Op. 15 y.o. Post Op. 17 y.o.

Results

Results and conclusions 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. Also, we could improve his feature by distracting nasal area forward.

Furthermore, we could improve his quality of living by removing RED device earlier using Kirschner wire for consolidaton.

Significance of the findings

It is suggested that this method, ‘ modified Le Fort II minus Le Fort I DO -including piriform aperture rim’ , might be effective in the treatment of syndromic craniosynostosis featuring severe midfacial hypoplasia without exophthalmos, who has mild occlusal discrepancy and severe nasal deformity.