Faculty Bibliography

Background/Purpose: LeFort III distraction osteogenesis is commonly recommended for children with syndromic craniosynostosis to reduce exorbitism, improve airway function, and decrease dysmorphism. This purpose of this study is to report on the long-term clinical outcomes of patients with syndromic craniosynostosis patients who have undergone early primary subcranial LeFort III distraction osteogenesis and who have been followed longitudinally through skeletal maturity. Methods/Description: Retrospective review of all patients who underwent LeFort III distraction osteogenesis between the ages of 3 and 11 years and were followed throughout development with longitudinal dental, medical, radiographic, and photographic evaluations conducted through skeletal maturity and beyond. Inclusion criteria entailed having preoperative medical photographs and cephalometric studies at 6 months and 1, 5, and 10 years postoperatively after the primary LeFort III distraction osteogenesis as well as cephalometric documentation 6 months and 1 year after the secondary midface advancement after skeletal maturity.
Result(s): Seventeen patients fulfilled inclusion criteria, with a mean age of 5.7 years at the time of initial LeFort III distraction. The mean advancement of point A was 14.9 mm anteriorly and 2.7 mm inferiorly along the x- and y-axis, respectively. Orbitale moved 10.5 mm anteriorly and 2.2 mm inferiorly along the x- and y-axis, respectively. At 10 years postoperatively, point A moved 3.4 mm anterior along the xaxis and 4.7 mm inferiorly along the y-axis, while orbitale moved 0.4 mm posteriorly and 3 mm inferiorly along the x- and y-axis, respectively. At the time of skeletal maturity, there was a return of occlusal disharmony from normal mandibular growth and a return of proptosis owing to remodeling of orbitale inferiorly, and the lateral orbital rim posteriorly, while the globe continued to grow in the anterior vector. All but 1 study patient underwent or is scheduled to undergo a secondary midface advancement at the LeFort III and LeFort I level after skeletal maturity was attained.
Conclusion(s): The data demonstrate that patients who undergo early LeFort III distraction osteogenesis before the age of mixed dentition will still most likely need a secondary midface advancement after skeletal maturity is reached given that there is a small degree of anterior growth at the level of the maxilla and no anterior growth at orbitale over time

Background/Purpose: Early LeFort III (LFIII) surgery or LFIII distraction involve osteotomies and disjunction in the region of the maxillary tuberosity in proximity to the maxillary posterior tooth buds. The purpose of this study was to determine the effect of early LFIII advancement and/or distraction on survival of the maxillary posterior permanent dentition. Methods/Description: A retrospective review of patients with syndromic craniosynostosis treated by early LFIII surgery and distraction was conducted. Of 225 syndromic craniosynostosis patients enrolled between 1973 and 2006, a total of 50 patients satisfied the inclusion criteria: 1) surgical intervention prior to age 8 years; 2) two panoramic radiographs, one prior to surgery and one in adolescence; 3) no apparent abnormalities in the position of permanent tooth buds. Of the 50 patients, 25 underwent LFIII surgery and 25 underwent midface distraction (M = 21, F = 29, average age at time of surgery = 5 +/- 1.1 years with diagnoses of Crouzon (20), Apert (17) and Pfeiffer (13), syndromes). Panoramic radiographs presurgically (T1) and postsurgically (T2) were inspected by a trained observer. The tooth buds were classified as being present (P), displaced (D), impacted (I), ankylosed (ANK), extracted (E), or absent (A). SPSS software was used to carry out chi-squared analysis and Fisher exact test.
Result(s): In the LFIII surgery group, 94% of maxillary second molars (D = 16%, I = 8%, E = 6%, A = 64%) and 28% of maxillary first molars (D = 18%, I = 4%, ANK = 2%, E = 2%, A = 2%) experienced a disturbance in eruption. Of the displaced second molars, 75% were located in the maxillary sinus and 25% in the maxillary tuberosity. Of the displaced first molars, 78% were located in the maxillary sinus and 22% in the maxillary tuberosity. In the distraction group, 80% of maxillary second molars (D = 38%, ANK = 4%, E = 14%, A = 24%) and 18% of maxillary first molars (D = 10%, I = 2%, E = 2%, A = 4%) experienced a disturbance in eruption. Of the displaced second molars, 37% were located in the maxillary sinus and 63% in the maxillary tuberosity. Of the displaced first molars, 100% were located in the maxillary tuberosity. Traditional LFIII osteotomy was significantly more likely to result in an adverse event for maxillary second molars compared to distraction (chi² = 4.33, P = .037).
Conclusion(s): The eruption of maxillary second molars had a high incidence of disruption following early LFIII intervention, with traditional LFIII surgery having greater negative consequences for the maxillary second molars compared to distraction. The maxillary first molars show significantly less disruption during early LFIII intervention with no significant differences noted between surgical procedures. Furthermore, a common disruption seen postsurgically is the displacement of the maxillary second molar tooth buds into the maxillary sinus, leading to the question if presurgical planning should include extraction/enucleation of the second molar tooth buds to avoid this sequela

INTRODUCTION/BACKGROUND:Treacher Collins syndrome (TC) and Pierre Robin sequence (RS) are associated with hypoplastic mandible, glossoptosis, and consequent airway obstruction. Although TC and RS are often grouped together, airway outcomes of bilateral mandibular distraction osteogenesis (MDO) have not been specifically studied in TC. The purpose of this study is to report on the clinical outcomes of MDO in the TC patient population. MATERIALS AND METHODS/METHODS:A twenty-year single-institution retrospective review of all patients with TC who underwent bilateral MDO was performed. Twenty-four patients were identified after exclusion due to different diagnoses or insufficient medical records. Data on comorbidities, airway status, MDO operations, and complications were collected. Data were compared with published clinical outcomes in RS and data for 13 RS patients from our institution. RESULTS:Surgical success, defined as prevention of imminent tracheostomy or successful decannulation within 1 year after primary distraction, was observed in 21% of TC patients and 65% of RS patients (P = 0.01). Repeat distraction was necessary for 11 TC patients (46%) and 1 RS patient. Complications were divided into minor, moderate, and major based on need for invasive management. Overall, 67% of TC patients had complications, 20% of which were major. CONCLUSIONS:Clinical outcomes to airway function after MDO are significantly inferior in patients with TC compared with patients with RS. Repeat MDO and longer course to decannulation are more prevalent in patients affected by TC.

Le Fort III distraction osteogenesis may be indicated in the treatment of syndromic craniosynostosis with severe midface retrusion and proptosis. This study assesses the stability of proptosis correction over 10-years.A retrospective review identified 15 patients with syndromic craniosynostosis treated by Le Fort III distraction prior to age 10 (9 males, 6 females; age 4.9 ± 1.5 years). Untreated, non-craniosynostotic age- and gender-matched controls were obtained from historical growth records. Lateral cephalometric tracings at pre-surgery (T1), immediate (T2), 1 year (T3), 5 years (T4), and 10 years (T5) (n = 11) post-distraction were superimposed using the best-fit of cranial base. Proptosis severity was defined as the horizontal distance between the Ant. Globe cephalometric point and orbital rim landmarks Orbitale and Lat. Orbit.The orbital rim advanced 10.54 ± 3.78 mm (P < 0.001) at Orbitale and 9.73 ± 4.54 mm (P > 0.001) at Lat. Orbit from T1 to T2; Ant. Globe advanced 3.13 ± 3.02 mm (p 0.001). Proptosis decreased 7.41 ± 5.29 mm (P < .001) from Orbitale and 6.60 ± 6.50 mm (p 0.002) from Lat. Orbit. Comparison to controls demonstrated phenotypic correction. In craniosynostotic patients from T2 to T5, the bony orbital rim demonstrated non-significant remodeling posteriorly and inferiorly. Anterior Globe moved 3.79 ± 1.47 mm anteriorly (P < .001), which did not differ significantly from controls. Proptosis increased by 4.18 ± 2.94 mm in craniosynostotic patients from T2 to T5.Le Fort III distraction was stable, with no significant anteroposterior relapse of the maxilla or bony orbit. Phenotypic relapse of proptosis to pre-treatment levels occurred through deficient growth of the midface, surface resorption at the orbital rim, and preservation of normal forward movement of Ant. Globe.

OBJECTIVE:To quantify 3-dimensional (3D) nasal changes in infants with unilateral cleft lip with or without cleft palate (UCL±P) treated by nasoalveolar molding (NAM) and cheilorhinoplasty and compare to noncleft controls. DESIGN/METHODS:Retrospective case series of infants treated with NAM and primary cheilorhinoplasty between September, 2012 and July, 2016. Infants were included if they had digital stereophotogrammetric records at initial presentation (T1), completion of NAM (T2), and following primary cheilorhinoplasty (T3). Images were oriented in 3dMD Vultus software, and 16 nasolabial points identified. PATIENTS/METHODS:Twenty consecutively treated infants with UCL±P. INTERVENTIONS/METHODS:Nasoalveolar molding and primary cheilorhinoplasty. MAIN OUTCOME MEASURES/METHODS:Anthropometric measures of nasal symmetry and morphology were compared in the treatment group between time points using paired Student t tests. Postsurgical nasal morphology was compared to noncleft controls. RESULTS:Nasal tip protrusion increased, and at T3 was 2.64 mm greater than noncleft controls. Nasal base width decreased on the cleft side by 4.01 mm after NAM and by 6.73 mm after cheilorhinoplasty. Columellar length of the noncleft to cleft side decreased from 2:1 to 1:1 following NAM. Significant improvements in subnasale, columella, and nasal tip deviations from midsagittal plane were observed. Treatment improved symmetry of the alar morphology angle and the nasal base-columella angle between cleft and noncleft sides. CONCLUSIONS:Three-dimensional analysis of UCL±P patients demonstrated significant improvements in nasal projection, columella length, nasal symmetry, and nasal width. Compared to noncleft controls, nasal form was generally corrected, with overcorrection of nasal tip projection, columella angle, and outer nasal widths.

OBJECTIVES/OBJECTIVE:To compare 3-dimensional nasal symmetry in patients with UCLP who had either rotation advancement alone or nasoalveolar molding (NAM) followed by rotation advancement in conjunction with primary nasal repair. DESIGN/METHODS:Pilot retrospective cohort study. MATERIALS AND METHODS/METHODS:Nasal casts of 23 patients with UCLP from 2 institutions were analyzed; 12 in the rotation advancement only group (Iowa) and 11 in the NAM, rotation advancement with primary nasal repair group (New York). Casts from patients aged 6 to 18 years were scanned using the 3Shape scanner and 3-dimensional analysis of nasal symmetry performed using 3dMD Vultus software, Version 2507, 3dMD, Atlanta, GA. Cleft and noncleft side columellar height, nasal dome height, alar base width, and nasal projection were linearly measured. Inter- and intragroup analyses were performed using t tests and paired t tests as appropriate. RESULTS:; P = .02). Intergroup analysis performed on the most sensitive linear measure, alar base width, revealed significantly less asymmetry on average in group 2 than in group 1 ( P = .013). CONCLUSION/CONCLUSIONS:This study suggests the NAM followed by rotation advancement in conjunction with primary nasal repair approach may result in less nasal asymmetry compared to rotation advancement alone.

OBJECTIVE:To determine the effects of nasoalveolar molding (NAM) on nasal airway architecture. DESIGN/METHODS:Retrospective case-control study of patients with unilateral cleft lip treated with NAM vs without NAM. SETTING/METHODS:Tertiary referral center specializing in cleft and craniofacial care. Patients, Participants, and Interventions: Thirty-six patients with complete unilateral cleft lip and alveolus: 19 with NAM therapy and 17 without NAM therapy. MAIN OUTCOME MEASURES/METHODS:Cone beam computed tomography (CBCT) scans were compared in multiple coronal sections and were evaluated for linear and angular septal deviation, inferior turbinate hypertrophy, and linear and 2-dimensional airway area. RESULTS:There were no significant differences in linear or angular septal deviation, inferior turbinate area, linear stenosis, or airway area between NAM- and non-NAM-treated patients. CONCLUSIONS:NAM effectively molds the external nasal cartilage and structures but may have limited effects on internal nasal structures.

The orthodontic treatment of adolescents with cleft lip and palate is complex and highly individualized. For such patients, there is a great need for thorough and comprehensive diagnosis as well as attention to multi-disciplinary aspects of orthodontic care. A framework for categorizing patients with varying forms and degrees cleft lip and palate into three levels of skeletal discrepancy from least to most severe is presented, and the specific treatment objectives of phase II orthodontic treatment for each of the three categories is then outlined. Moreover, due to specific challenges of a cleft-related dentition, the various aspects of the management of missing teeth are reviewed. Finally, the importance and most pertinent methods of retention are emphasized. (C) 2017 Elsevier Inc. All rights reserved.