Long-term orthodontic and surgical treatment and stability of a patient with Beckwith-Wiedemann syndrome.
Beckwith-Wiedemann syndrome (BWS) is a congenital growth disorder. Children born with BWS develop enlarged organs, including the tongue, a large body, and other signs. A woman with BWS was treated and followed for 30 years. Treatment consisted of tongue reduction, orthopedic and orthodontic treatment, orthognathic surgery, and retention. The patient was first treated when she was 5 years old. Her original orthodontic problems included macroglossia, anterior open bite, anterior crossbite, and a skeletal Class III jaw relationship caused by significant mandibular protrusion. The jaw-base relationships did not improve in the early preadolescent period after phase 1 of orthodontic treatment with a vertical chincap. With the growth spurt accompanying puberty, she developed a severe skeletal Class III jaw relationship and a constricted maxillary arch. Surgically assisted rapid maxillary expansion was performed at 23 years of age to correct the severe discrepancy between the maxillary and mandibular dental arch widths. Then, at 26 years, a LeFort I osteotomy, a horseshoe osteotomy, a bilateral sagittal split ramus osteotomy, and genioplasty were performed after presurgical orthodontic treatment with extraction of the mandibular first molars. Both the facial profile and the occlusion were stable after 6 years of retention. This case report discusses the result of long-term observation of a patient with BWS who underwent tongue reduction, early orthodontic treatment, and surgical-orthodontic treatment.
Resident, Department of Maxillofacial Reconstruction and Function, Division of Maxillofacial/Neck Reconstruction, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan.
Rina
R
Hikita
Assistant professor, Department of Maxillofacial Reconstruction and Function, Division of Maxillofacial/Neck Reconstruction, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan.
Yukiho
Y
Kobayashi
Assistant professor, Department of Maxillofacial Reconstruction and Function, Division of Maxillofacial/Neck Reconstruction, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan.
Michiko
M
Tsuji
Junior associate professor, Department of Maxillofacial Reconstruction and Function, Division of Maxillofacial/Neck Reconstruction, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan. Electronic address: t-kawamoto.mort@tmd.ac.jp.
Tatsuo
T
Kawamoto
Professor and chair, Department of Maxillofacial Reconstruction and Function, Division of Maxillofacial/Neck Reconstruction, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan; professor, Hard Tissue Genome Research Center, Tokyo Medical and Dental University, Tokyo, Japan.
Keiji
K
Moriyama
D001506:Beckwith-Wiedemann Syndrome / Q000601:surgery / Q000628:therapy*; D002675:Child, Preschool; D005115:Extraoral Traction Appliances; D005260:Female; D005500:Follow-Up Studies; D063172:Genioplasty / Q000379:methods; D005927:Glossectomy / Q000379:methods; D006801:Humans; D008137:Longitudinal Studies; D008260:Macroglossia / Q000601:surgery; D008313:Malocclusion, Angle Class III / Q000601:surgery / Q000628:therapy; D008437:Maxilla / Q000002:abnormalities; D024343:Open Bite / Q000601:surgery / Q000628:therapy; D009971:Orthodontics, Corrective / Q000379:methods*; D056948:Orthognathic Surgical Procedures / Q000379:methods*; D019340:Osteotomy, Le Fort / Q000379:methods; D059229:Osteotomy, Sagittal Split Ramus / Q000379:methods; D010155:Palatal Expansion Technique; D010347:Patient Care Planning; D011378:Prognathism / Q000601:surgery / Q000628:therapy; D016896:Treatment Outcome