Effect of partial glossectomy on the dentofacial development of patients with Beckwith-Wiedemann syndrome

Oral Abstract Session 4: Pathology and Medicine/Orthognathic Surgery/TMJ/Maxillofacial Reconstruction surgical advancements, without the use of bone grafts, in OSA patients. Materials and Methods: Twenty-three patients with polysomnogram diagnosed OSA comprised the study group. There were 20 males and 3 females with a mean age of 45 years (range: 27 to 59 years). Each patient underwent Le Fort I maxillary osteotomy (step design) without the use of bone grafting, stabilized with 4 bone plates. Mandibular advancement was accomplished using bilateral sagittal split ramus osteotomies stabilized with either bicortical screws or bone plates, and 3 weeks of maxillomandibular fixation. Preoperative, immediate postoperative, and long-term (⬎1 year) lateral cephalometric radiographs were analyzed. Specific maxillary (Apoint and PNS) and mandibular (B-point) landmarks were used to assess surgical movement and long-term stability. A 2-tailed paired t test and Pearson’s correlation coefficient were used to analyze movements and stability. Results: The mean surgical advancement of the maxilla was 9.68 ⫾ 2.50 mm. The mean surgical advancement of the mandible was 11.73 ⫾ 2.57 mm. The mean vertical maxillary impaction was 2.20 ⫾ 2.31 mm, and the mean vertical mandibular surgical movement was 3.16 ⫾ 3.39 mm. The mean relapse in the advancement of the maxilla was small: 0.52 ⫾ 1.13 mm; 87% of these patients had less than 2 mm of relapse. The mean relapse of vertical maxillary impaction was extremely small: 0.02 ⫾ 1.36 mm; 91% of these patients had less than 2 mm of long-term relapse in the vertical dimension. The mean relapse in the mandibular advancement was 0.57 ⫾ 2.28 mm; 65% of these patients demonstrated less than 2 mm of relapse. Conclusion: The findings of this study demonstrate that large maxillary and mandibular advancements in OSA patients, utilizing the techniques of bone plating or bicortical screws without the use of bone grafts, results in long-term skeletal stability. References Nimkarn Y, Miles PG, Waite PD: Maxillomandibular advancement surgery in obstructive sleep apnea syndrome patients: Long-term surgical stability. J Oral Maxillofac Surg 53:1414, 1995 Waite PD, Tejera TJ, Anucul B: The stability of maxillary advancement using LeFort I osteotomy with and without genial bone grafting. Int J Oral Maxillofac Surg 25:264, 1996 Funding Source: Vanderbilt University Oral and Maxillofacial Surgery Department.

Effect of Partial Glossectomy on the Dentofacial Development of Patients With Beckwith-Wiedemann Syndrome Jose Beltran, DMD, Children’s Hospital, Division of Plastic and Oral Surgery, 300 Longwood Avenue, Boston, MA 02115 (Padwa BL; Ferraro N; August M) AAOMS • 2003

Purpose: Macroglossia is the most common manifestation of Beckwith-Wiedemann syndrome. Previous studies have found the enlarged tongue leads to protrusion of dentoalveolar structures with resulting anterior open bite, obtuse gonial angle, and increased mandibular length. Early partial glossectomy has been advocated to minimize the dentoskeletal deformity. The purpose of this study was to compare the dentofacial morphology of patients who had partial glossectomy to those who did not to determine the effect of tongue reduction on orofacial development. Materials and Methods: Retrospective case series of 13 patients (9 males, 4 females; mean age 10 years, range 1 to 34 years) with Beckwith-Wiedemann syndrome. Records were reviewed and clinical findings documented based on photographs and reports of clinical examinations. When available lateral cephalograms were traced and a Steiner analysis performed, the findings between groups were compared. Results: All 13 patients had macroglossia: 7 had partial glossectomy done at a mean age of 16.8 months (range 3 to 36 months). Nine patients (of whom 5 had glossectomy) had speech disorders. On clinical examination 8 had anterior open bite, of these 6 had Class I and 2 had Class III molar relationship. Lateral cephalograms were available for 5 patients (3 postglossectomy). Based on Steiner analysis, 4 were orthognathic and 1 had maxillary hyperplasia. All 5 patients had an increased mandibular plane angle. Conclusions: Based on the clinical and cephalometric evaluation partial glossectomy does not appear to change the developing dentofacial morphology in Beckwith-Weidemann patients. This suggests that previously reported skeletal and dental anomalies may be the typical “facies” and that tongue reduction should not be offered as a means of changing orofacial development. References Menard RM, Delaire J, Schendel SA: Treatment of craniofacial complications of Beckwith-Wiedemann syndrome. Plast Reconstr Surg 96: 27, 1995 McManamny DS, Barnett JS: Macroglossia as a presentation of the Beckwith-Wiedemann syndrome. Plast Reconstr Surg 75:170, 1985

Endoscopic Condylectomy and Reconstruction W. Bradford Williams, Department of OMS, Mass General Hospital, 55 Fruit Street, WRN 1201, Boston, MA 02114 (Troulis MJ; Kaban LB) Purpose: The benefits of minimally invasive surgery have been well documented. The purpose of this paper is to present early results in a series of patients who had endoscopic mandibular condylectomy and reconstruction with costochondral grafts. Patients and Methods: This is a retrospective evalua63