Faculty Bibliography

BACKGROUND: Maxillary position in patients with syndromic craniosynostosis after midface distraction has been shown to be stable 1 year postoperatively. The purpose of this study is to assess midfacial position in the growing child with craniosynostosis 5 years after Le Fort III advancement with a rigid external device (RED). METHODS: Seventeen consecutive patients were identified to have the diagnosis of syndromic craniosynostosis and who underwent midface advancement. There were 10 males and 7 females, 7 patients had Crouzon syndrome, 5 had Apert syndrome, and 5 had Pfeiffer syndrome. A standard subcranial Le Fort III osteotomy was performed. Cephalometric analysis was performed to assess the position of the maxilla. RESULTS: After device removal, orbitale advanced 13.67 mm along the x axis and downward 1.70 mm along the y axis. A Point advanced 15.97 mm along the x axis and downward 1.14 mm along the y axis. At 1 year post-distraction, both orbitale and A point had advanced an additional 0.47 mm and 0.24 mm along the x axis and downward 0.58 mm and 1.78 mm along the y axis respectively. At 5 years post-distraction, orbitale moved posterior 0.58 mm and A point advanced an additional 2.08 mm along the x axis. Orbitale and A point descended 3.23 mm and 5.2 mm along the y axis respectively. CONCLUSIONS: After Le Fort III advancement with distraction, the maxillary position remains stable and continues to advance minimally along the x axis and demonstrates more growth along the y axis over the long term.

BACKGROUND: The aim of this study is to identify cephalometric measurements associated with clinical severity in patients with Treacher Collins Syndrome (TCS). METHODS: A retrospective single-institutional review of patients with TCS was conducted. Pre-operative cephalograms and computed tomography scans (n=30) were evaluated. 50 cephalometric measurements were compared to age-specific normative data using ANOVA. These cephalometric measurements and the patient's Pruzansky classification were correlated to clinical severity using Spearman analysis. Clinical severity was defined as: severe (required tracheostomy), moderate (obstructive sleep apnea, oral cleft, or gastrostomy-tube), or mild (absence of listed co-morbidities). Cephalometric measurements with a strong correlation (rs>0.60) were identified as predictors of clinical severity. RESULTS: Cephalograms of the study population contained thirty measurements that were found to be significantly different from normative data (p<0.01). These measurements were largely related to maxillary/mandibular projection, maxillary/mandibular plane angle, mandibular morphology, facial height, facial convexity and mandible/throat position. Ten of these 30 statistically significant measurements in addition to Pruzansky classification were found to be strongly correlated (rs>.60) to clinical severity. These measurements include: Mandibular projection/position [Sella-Nasion-Pogonion (SN-Pg) rs=-0.64; Hyoid-Menton (Hy-Me) rs=-0.62]; Posterior facial height [Posterior Facial Height/Anterior Facial Height (PFH/AFH) rs=0.60; Condyle-Gonion (Co-Go) rs=-0.66]; Maxillary/mandibular plane angle [Sella-Nasion-Mandibular Plane (SN-MP) rs=0.62; Frankfort Horizontal-Mandibular Plane (FH-MP) rs=0.61; Sella-Nasion-Palatal Plane (SN-PP) rs=0.69; Sella-Nasion-Symphysis (SN-Symph) rs=-0.69; Pruzansky classification rs=0.82. CONCLUSIONS: Specific cephalometric measurements of increased mandibular retrognathia, decreased posterior facial height, more obtuse maxillary/mandibular plane angle and more obtuse symphasis notch angle are strongly correlated to increased clinical severity in patients with Treacher Collins syndrome.

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.

Presurgical infant orthopedics has been employed since 1950 as an adjunctive neonatal therapy for the correction of cleft lip and palate. Most of these therapies did not address deformity of the nasal cartilage in unilateral and bilateral cleft lip and palate as well as the deficiency of the columella tissue in infants with bilateral cleft. The NasolAveolar molding (NAM) technique, a new approach to presurgical infant orthopedics, developed by Grayson reduces the severity of the initial cleft alveolar and nasal deformity. This enables the surgeon and the patient to enjoy the benefits associated with repair of a cleft deformity that is minimal in severity. This paper will discuss the appliance design, clinical management, and biomechanical principles of nasolaveolar molding therapy. Long-term studies on NAM therapy indicate better lip and nasal form, reduced oronasal fistula and labial deformities, and 60% reduction in the need for secondary alveolar bone grafting. No effect on growth of midface in sagittal and vertical plane has been recorded up to the age of 18 years. With proper training and clinical skills NAM has demonstrated tremendous benefit to the cleft patients as well as to the surgeon performing the repair. (C) 2017 Elsevier Inc. All rights reserved.

Maxillary hypoplasia is a common finding in patients with cleft lip and palate (CLP). This study evaluated the skeletal and dentoalveolar changes in patients with CLP treated with an occlusally bonded maxillary protraction headgear (PHG) appliance. A total of 267 consecutive patients (1995-2012) treated with a PHG appliance were reviewed. In all, 40 patients with CLP (mean age 7.70 years) met the inclusion criteria. Mean treatment duration was 7.5 months with a mean force of 405 g per side. X- and Y-axis displacement for 38 lateral cephalometric landmarks was recorded at pretreatment (T-0), following removal of PHG (T-1), and at 1.5 years follow-up (T-2). From T-0 to T-1, A-point advanced by +2.48 mm (p &lt; 0.01), UIE advanced by +4.91 mm (p &lt; 0.01) and B-point moved posteriorly by -2.03 mm (p &lt; 0.01) and inferiorly by -3.86 mm (p &lt; 0.01). During the same time interval, the ANB angle changed from 0.08 to 3.77 (p &lt; 0.01). At 1.5 year follow-up, A-point moved posteriorly by -0.28 mm (p &gt; 0.05), B-point moved anteriorly by 3.69 mm (p &lt; 0.01) and the ANB angle decreased to 0.51 (p &lt; 0.01). A PHG appliance with a mean 810 g of force resulted in 54.60% skeletal and 45.40% dentoalveolar advancement. At 1.5 years (T2), the maxillary position was stable with minimal anterior growth; however, the mandible showed significant anterior growth contributing to reduction of the ANB angle. (C) 2017 Elsevier Inc. All rights reserved.

Background/Purpose: The aim of this study is to perform a cephalometric evaluation of the craniofacial skeleton of patients with TCS. Methods/Description: Retrospective single institution review of all patients (N= 104) with TCS and a preoperative cephalogram was conducted (30 patients). Patients were divided into three groups based on their ages: infancy (mean 0.62 yr; range:0.01-2.2 yrs) adolescence (mean 7.91 yr, range:5.18-11.26 yrs) and post adolescent-young adulthood (mean 17.04 yr; range:15.49-21.36 yrs). Right and left sides were evaluated separately if asymmetry was noted to be present (44 sides). The cephalometric variables were compared to Bolton and Moyers norms and also to each other using ANOVA and student's t-test. Results: All maxillary and mandibular measurements were significantly different from normative values with the exception of SNA and upper gonial angle (Na-Go-Me). SNB, SNPg angles were severely decreased and Pg (Pg-NB) was significantly retruded (p<0.001). Gonial angle (Ar-Go-Me) was significantly wider than normal as lower gonial angle (Ar-Go-Na) and antegonial angles were significantly increased (p<0.001) in all three age groups. There was no difference among the groups in terms of increased antegonial angles. All vertical plane angles (SN-MP, SN-GoGN, FH-MP, SN-PP, PP-MP) were increased significantly as well (p<0.001). Correspondingly, the ratio between lower anterior face height and total face height was significantly higher, while posterior face height to anterior face height was significantly decreased (p<0.001). More than half of the patients (N= 17/30) possessed a parasagittal symphyseal notch at the anterior surface of the chin. The depth and width of this notch were increased from infancy to adolescence (p<0.01). Accordingly, symphysis inclination (SN-Symp.) increased significantly over time (p<0.01). The maxillary posterior region showed decreased height (p<0.01). Our findings suggest that the maxillo-mandibular deformity demonstrates what we have termed a 'parasagittal orbito-maxillo-zygomatic cleft' which is aligned along the path of maximum mandibular atresia (diminished or missing coronoid, condylar processes and rami. Conclusions: When comparing cephalometric values in patients with TCS to Bolton and Moyers, all structures showed varying degrees of deformation or dislocation with the exception of maxillary sagittal position. These changes were most prevalent in the posterior maxillae, mandible, symphysis and antegonial area of the mandible. Certain skeletal changes did not show variance from infancy to adulthood, such as maxilla-mandibular angle and Wits value, however changes of the symphysis region became more severe over time. Further, soft tissue facial convexity increased severely in all growth periods

Background/Purpose: Distraction osteogenesis is indicated in patients with syndromic craniosynostosis with severe midface hypoplasia and proptosis. Pre-adolescent intervention is indicated when eye closure is limited, to prevent long-term ocular damage. The long-term stability of proptosis correction by Le Fort III distraction in this population has not been previously reported. Methods/Description: A retrospective review was conducted to identify patients with syndromic craniosynostosis treated by Le Fort III distraction prior to age 10, with cephalometric films available at least 3 years after treatment. 15 patients were identified (9 male, 6 female; age 4.9+/-1.5 years) with diagnoses of Crouzon(7), Apert(6), and Pfeiffer(2) syndromes. Lateral cephalometric radiographs at pre-surgery(T1), immediate post-distraction(T2), short-term post-distrac-tion(T3), and long-term(T4) were traced manually to identify the most inferior point on the orbital rim (orbitale), and the most anterior point on the globe. Tracings were superimposed on sella-ethmoid using the best-fit of cranial base details. Changes were measured using an x,y coordinate system with sella as the origin, and 7degree below the T1 sella-nasion line as the horizontal plane. Proptosis severity was defined as the horizontal distance between globe and orbitale. All measurements were corrected to a magnification factor of 0%. Changes in landmark location and proptosis severity were assessed by paired t-tests. Results: Orbitale advanced 10.2+/-3.8mm (p<0.001) horizontally from T1 to T2, with a superior movement of 2.1+/-3.5mm (p 0.02). The globe advanced 3.0+/-3.2mm (p 0.03) with no significant change in vertical position (p 0.539), resulting in a significant decrease in proptosis (-7.265.1mm, p<0.001). From T2 to T3 (1.160.7 years), orbitale remained stable horizontally (p 0.595), with minor inferior movement (-1.661.8mm, p 0.05). Globe position was stable horizontally (p 0.363) and vertically (p 0.436), as was proptosis correction (p 0.721). At long-term follow-up (T2 to T4, 4.87+/-0.93 years), orbitale remained stable horizontally (p 0.522) with no significant vertical change compared to T3 (p 0.113). Globe position was stable vertically (p 0.350); however, significant anterior globe movement occurred (+2.2+/-2.6mm, p 0.003) with an associated increase in proptosis (+1.9+/-2.7mm, p 0.015). Despite this, proptosis remained decreased by 5.3mm (95% CI 2.9mm, 7.6mm) at T4 compared to T1. Conclusions: Bony advancement of the orbital rim by Le Fort III distraction osteogenesis in pre-adolescent patients was stable 5 years post-distraction. Initial advancement of the orbit was associated with minor advancement of the globe, though considerable individual variation was noted. While proptosis was improved by treatment at all time-points, phenotypic relapse of 2mm was observed 5 years post-treatment, reinforcing the need for over-correction. At 5 year follow-up, 2.9 to 7.6 mm of proptosis correction remained

OBJECTIVE: Our study goal was to evaluate the rates of breast milk feeding among patients with oral clefts at a large North American Craniofacial Center. METHODS: Parents of patients with oral clefts born from 2000 to 2012 and treated at our center were interviewed regarding cleft diagnosis, counseling received for feeding, and feeding habits. RESULTS: Data were obtained from parents of 110 patients with oral clefts. Eighty-four percent of parents received counseling for feeding a child with a cleft. Sixty-seven percent of patients received breast milk for some period of time with a mean duration of 5.3 months (range 0.25 to 18 months). When used, breast milk constituted the majority of the diet with a mean percentage of 75%. Breast milk feeding rates increased successively over the 13-year study period. The most common method of providing breast milk was the Haberman feeder at 75% with other specialty cleft bottles composing an additional 11%. Parents who received counseling were more likely to give breast milk to their infant (P = .02). Duration of NasoAlveolar Molding prior to cleft lip repair did not affect breast milk feeding length (P = .72). Relative to patients with cleft lip and palate, patients with isolated cleft lip had a breast milk feeding odds ratio of 1.71. CONCLUSION: We present breast milk feeding in the North American cleft population. Although still lower than the noncleft population, breast milk feeding with regards to initiation rate, length of time, and proportion of total diet is significantly higher than previously reported.