Role of the Craniofacial Orthodontist on the Craniofacial and Cleft Lip and Palate Team

Role of the Craniofacial Orthodontist on the Craniofacial and Cleft Lip and Palate Team

Role of the Craniofacial Orthodontist on the Craniofacial and Cleft Lip and Palate Team Pedro E. Santiago, DMD, and Barry H. Grayson, DDS Patients bor...

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Role of the Craniofacial Orthodontist on the Craniofacial and Cleft Lip and Palate Team Pedro E. Santiago, DMD, and Barry H. Grayson, DDS Patients born with a craniofacial deformity and their families experience significant psychosocial effect as they deal with physical appearance that has been esthetically and functionally compromised. The deformity usually involves skeletal and soft-tissue elements, which often affect facial symmetry and esthetics. As the dentition is directly related to the jaw structures, a wide variety of malocclusions may result. As patients with craniofacial anomalies present with multiple dental and medical conditions, an interdisciplinary team approach is highly recommended to accurately diagnose and to properly customize a treatment plan. Craniofacial Orthodontics is the area of orthodontics that treats patients with congenital and acquired deformities of the integument and its underlying musculoskeletal system within the craniofacial area and associated structures. As part of the craniofacial and cleft teams, the craniofacial orthodontist is involved in data collection, clinical examination, diagnosis, treatment planning, and orthopedic or orthodontic treatment of the craniofacial disorder. The craniofacial orthodontist has been shown to play an intrinsic role in the care of patients with craniofacial anomalies and cleft lip and palate. (Semin Orthod 2009;15:225-243.) © 2009 Elsevier Inc. All rights reserved.

he family’s initial visit takes place after identification of a craniofacial disorder, often on a prenatal ultrasound (Fig. 1). If prenatal diagnosis was not performed, the initial visit with the interdisciplinary cleft palate or craniofacial team occurs during the first postnatal days. Parent counseling is initiated by the cleft palate or craniofacial team and a parent support group. The family is provided with information regarding the etiology and the clinical management of the condition

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From the Department of Orthodontics, School of Dentistry, The University of North Carolina at Chapel Hill, Chapel Hill, NC, Department of Surgery, Division of Plastic Surgery, Duke University Medical Center, Durham, NC, Center for Craniofacial Disorders, School of Dental Medicine, University of Puerto Rico, San Juan, Puerto Rico, Institute of Reconstructive Plastic Surgery, NYU School of Medicine, New York University Langone Medical Center, New York, NY, and Department of Orthodontics, New York University College of Dentistry, New York, NY. Address correspondence to Pedro E. Santiago, DMD, Department of Orthodontics, School of Dentistry, 201 Brauer Hall, Campus Box 7450, Chapel Hill, NC 27599-7450; Phone: (919) 966 4428; Fax: (919) 843 8864; E-mail: [email protected] © 2009 Elsevier Inc. All rights reserved. 1073-8746/09/1504-0$30.00/0 doi:10.1053/j.sodo.2009.07.004

from infancy through adulthood. Audiovisual aids are used to facilitate their understanding of the proposed treatment and to lower their anxiety levels through education and reviewing clinical photographs of pre- and post-treatment outcomes. This orientation provides the parents with information and an incentive that enhances their ability to commit to the treatment program. The parents’ enthusiastic involvement with the treatment is essential to its success. The craniofacial orthodontist is actively involved in the life of a patient born with a craniofacial deformity and/or cleft lip and palate from birth through skeletal maturity. This may include infant presurgical orthopedics, early mixed dentition treatment, dentofacial orthopedics and orthodontics, preparation for alveolar bone graft procedures, adolescent/adult orthodontics, preprosthetic orthodontics, and pre- and postsurgical orthodontics.

Presurgical Nasoalveolar Molding for Patients with Cleft Lip and Palate In an infant, the presence of a cleft is observable shortly after birth. At the end of the initial ap-

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Figure 1. (A) Three-dimensional ultrasound of an infant with right unilateral cleft. (B) Photo of the same infant after birth. (Color version of figure is available online.)

pointment, clinical examination is performed and photographs, intraoral and nasal impressions, and 3-dimensional (3D) stereophotogrammetry images (Fig. 2), if possible, are obtained to document and evaluate the deformity and establish a treatment plan.

The initial extraoral examination is performed by members of the cleft palate team. We observe the deformity and record various measurements that describe and quantitate the cleft lip, nose, and alveolar process. In the past, some surgeons have requested molding of the alveolar ridges to close the alveolar gap and to reduce the protrusive position of the alveolar processes. It is believed that the improved alignment of the underlying alveolar anatomy would enhance the surgeon’s ability to achieve a refined primary lip repair. Although this is true, some orthodontists have also reported that presurgical infant orthopedics (alveolar molding) improved feeding, speech, and occlusion.1,2 It has been shown that alveolar molding alone does not deliver most of these benefits. In recent studies, Prahl et al3 found a small but significant improvement in speech development. They also concluded that infant orthopedics (passive plate) has no effect on early esthetic outcome and that it does not have a positive or negative influence on arch form.4 It is important to note that this study did not include a population of children that had nasoalveolar molding. In 1987, Ross5 reported that “pre-surgical orthopaedics in the neonatal period has no apparent long-term effect on facial growth in height and depth.” Ross5 presents an important observation that alveolar molding does not have a negative impact on growth of the midface. Although the major national and international studies on presurgical infant orthopedics (alveolar molding plate) have found no significant positive or negative impact on speech, nutrition, occlusion, or growth of the midface, these findings cannot be extrapolated or applied to nasoalveolar molding. The introduction of nasal stents to the conventional alveolar molding plate is considered a paradigm shift in cleft care, as it addresses correction of the severe and stigmatizing nasal deformity. For the first time, the dentist has an opportunity, during the first 3-4 months of life, to significantly improve the projection of the nasal tip and to achieve correction of the alar cartilage distortion and asymmetry.6-12 In the case of a bilateral cleft lip and palate, the clinician can use the nasal stents to gradually lengthen the deficient columella.6-9,13 The columella created in this manner grows normally and results in improved nasal tip projection. This is important because conventional surgical correction of the deficient columella re-

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Figure 2. (A-C) Three-dimensional stereophotogrammetric images of an infant with a unilateral cleft lip and palate. (Color version of figure is available online.)

sults in scar tissue that diminishes growth of the columella and nasal tip projection and leads to the typical appearance of the broad and flat cleft nose. This results in the need for one or more nasal surgical revisions and the associated expenditure and risks. When the authors combine presurgical infant orthopedic molding of the nose and alveolar processes, it is called nasoalveolar molding.6-14 As it has been shown that presurgical alveolar

molding has no impact on orthodontic or surgical outcome,3 arch form,4 and maxillary growth,5 it can be used to approximate the cleft alveolar segments. As the alveolar segments come together, the cleft lip segments more easily approximate one another at rest. Under these circumstances, the surgical closure heals under less tension, resulting in a fine scar and improved alignment of the vermillion border.8,13 In addition, closure of the

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Figure 3. (A) Pre-alveolar molding study model of alveolar ridges in an infant with left unilateral cleft lip and palate. Note the asymmetry and displacement of the greater alveolar segment. (B) Molding plate adhered to the palatal shelves and alveolar processes. (C) Post-alveolar molding study model of alveolar ridges showing improved arch symmetry and reduction in the size of the alveolar gap. (Color version of figure is available online.)

alveolar gap, through molding, reduces the nasal deformity to a degree that enables the initiation of more precise nasal molding. It is important to recognize the distinct differences between the conventional presurgical infant orthopedics (or molding plate therapy) and nasoalveolar molding. In the former case, the direct benefit is the approximation of the cleft alveolar segments to facilitate primary surgery of the lip. In contrast, nasoalveolar molding has been shown to be a major advance for the surgical repair of the lip and nose, particularly in restoration of the deficient columella in the bilateral cleft lip and palate. Although these benefits have been demonstrated in multiple clinical publications,6-14 there is no doubt that a need

for long-term and perhaps federally supported clinical trials exist. In the unilateral cleft lip, alveolus, and palate, the preferred presurgical infant orthopedic treatment plan is to mold the greater alveolar segment toward the mid-sagittal plane, in the direction of the lesser alveolar cleft segment (Fig. 3). Correction of the alveolar deformity will improve bony symmetry and support of the softtissue nasal base. This often facilitates a more symmetric surgical correction of the lip and nose. By reducing the size of the cleft lip gap in repose, the surgical scar will heal under less tension and should be less visible (Fig. 4). Severity of the nasal deformity is often correlated with the magnitude of the lip and alveolar

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Figure 4. (A) Patient with a left unilateral cleft lip and palate before alveolar molding. (B) Patient after alveolar molding. Note the lips are in close proximity when at rest, facilitating primary lip surgical repair. The quality of the scar is usually best when the healing occurs under minimal tension. (C) Patient 12 months after primary lip repair. Note the fine alignment of the vermillion borders and the nearly absent scar. (Color version of figure is available online.)

cleft gaps. The lower lateral alar cartilage on the cleft side is often concave where it ought to be convex. The nostril apex is low on the cleft side, and the alar rim is stretched in the horizontal plane (Fig. 5). To acquire a precise anatomical maxillary alveolar model, an impression tray is selected from a series of preformed acrylic trays. Polyvinylsiloxane impression material is used for the initial impression. The impression is taken in a hospital setting with a surgeon as an integral part of the impression team to manage the limited risk of airway obstruction. The impression should include the height of the alveolar ridge up to the vestibular folds showing the buccal and labial freni. It should be poured in a dense and durable material, such as dental gypsum (gypsum) or dental stone to fabricate the permanent cast. The molding plate and attached retention buttons are constructed using one of various materials and techniques. The most common material is a methylmethacrylate (self-cured acrylic). Retention

button(s) deliver force to the molding plate and the alveolar processes from a system of elastic bands and cheek tapes. In addition, it serves to retain the molding plate to the palate and alveolar processes. Activation of the molding plate occurs through selective addition and reduction of hard and soft acrylic to the external and internal walls of the appliance. These adjustments are performed in small increments of approximately 0.5-1.0 mm to maintain appliance retention without causing breakdown and ulceration of the soft tissues (Fig. 6). Ideally, the infant’s response to treatment should be observed and the appliance activated weekly. General treatment objectives for the unilateral patient involve approximation of alveolar and lip segments and correction of the distorted nasal cartilages to facilitate the primary lip, alveolar, and nasal surgical repair. In the patient with bilateral clefts, the goal is to retract and align the protrusive premaxilla and prolabium while elongating the deficient

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Figure 5. (A) Patient born with a right unilateral cleft lip and palate and its associated nasal deformity. (B) Nasal deformity associated with unilateral cleft lip and palate: (a) concave lower lateral alar cartilage, (b) depressed nasal tip, (c) dropped nostril apex, (d) columella base deviated to the noncleft side, (e) deficient columella length on the cleft side, (f) wide alveolar gap, and (g) large interlabial gap. (Color version of figure is available online.)

columella. As in the unilateral case, this will also facilitate primary lip, alveolar, and nasal surgical repair. In the patient with a unilateral cleft, a nasal stent is added to the molding plate appliance when the alveolar cleft width has been reduced to ⬍ 5 mm. The nasal stent consists of a wire armature (0.036⬙ or 0.040⬙) that projects from the labial flange of the molding plate and terminates in an intranasal portion. The intranasal portion of this wire is covered with hard and soft methylmethacrylate. Gradual weekly modifications of the stent are made to mold the lower

lateral alar cartilage, to stretch the nasal lining, and to achieve projection of the nasal dome before the primary surgical repair (Fig. 7). In the patient with bilateral clefts, the protruding premaxilla (Fig. 8A) is very gradually retracted into the space between the alveolar segments by modifications of the molding plate and application of gentle elastic forces. Bilateral nasal stents (Fig. 8B) are inserted into the nasal apertures to achieve nasal tip projection, Columella elongation, correction of the lower lateral alar cartilage deformity, and increase in the surface area of the nasal mucosa. The differential forces exerted by the nasal stents, surgical tapes, and a horizontal band placed across the columella, provide for these changes (Figs. 8C-8E). After the presurgical orthopedic treatment has been completed, the primary lip or nose surgery is performed with or without a gingivoperiosteoplasty (GPP). It has been reported that when a GPP is performed in the primary surgery with the alveolar segments in close proximity, there is a 60% reduction in the need for a secondary bone graft.15 It has been shown16,17 that GPP has no effect on the position of the skeletal midface (ANS-PNS in relation to cranial base SN) up to the age of 12 years16 and 18 years.17 This surgery is followed by a palate repair at 12-15 months of age. From this surgical event and until the child is 5-6 years old, special attention is focused on speech development, oral hygiene, pediatric dentistry, and skeletal growth guidance.

Early Mixed Dentition Treatment and Dentofacial Orthopedics The craniofacial orthodontist evaluates the patient and records any significant dental, facial, and skeletal findings in annual follow-up visits with the cleft palate team. Primary areas of interest and concern are the presence of anterior and/or posterior skeletal and dental cross-bites, malocclusion, supernumerary teeth, and oronasal fistulae. A functional lateral shift of the mandible caused by premature occlusal contacts during a prolonged period of active facial growth may lead to asymmetrical maxillary or mandibular development. This could affect facial esthetics and psychosocial development if facial symmetry, balance, and proportion are severely affected. Anterior dental cross-bites could lead to

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Inadequate growth of the premaxilla may present as an anterior dental cross-bite with normal occlusion in the buccal segments. In such cases, a 2- or 3-way bonded palatal expander is indicated, to protract the premaxilla and, if needed, to widen the posterior segments (Fig. 10). If both anterior and posterior skeletal crossbites are present, maxillary deficiency is most likely manifested in all 3 dimensions: transverse, sagittal, and vertical (Fig. 11A). This scenario often includes a Class III dental occlusion, a midface skeletal deficiency, and a straight or concave facial profile. In such cases, either a Figure 6. Diagram showing the alveolar spaces inside the molding plate before treatment (top left). Arrows indicate the sites at which hard acrylic is removed (top right). The resulting space allows for growth and molding of the alveolar processes. Striped area indicates the location for addition of soft acrylic liner. A soft acrylic liner applies a gentle force to the alveolar ridge, molding it in the desired direction. Gradual hard and soft acrylic modifications result in approximation of the alveolar ridges (bottom left). Eventually, the acrylic is removed between the greater and lesser alveolar segments, permitting contact of the facing alveolar walls (bottom right). Note that the outer wall of the molding plate has been gradually modified to follow the changes occurring on the interior surfaces. (Color version of figure is available online.)

anterior posturing of the mandible, with resulting impact on the temporomandibular joint and mandibular growth. A small reduction in maxillary dental arch width is often accommodated by a mandibular lateral shift, resulting in a posterior dental cross-bite. Premature dental contacts and excessive occlusal wear can result from malocclusions associated with skeletal and dental cross-bites. Posterior cross-bites are usually corrected by the use of palatal expanders. The most common expanders use screws, that is, Hyrax (Fig. 9A) or a Quad Helix (Fig. 9B). If a cross-bite is limited to the anterior buccal segment, ie, canines and the deciduous molar(s), then a “fan expander” might be indicated both in unilateral (Figs. 9C and 9D) and bilateral (9F) cases. This appliance rotates around a posterior hinge, resulting in differential amount of expansion, more anterior than posterior (Figs. 9E and 9G). The selection of the expansion appliance is based on the characteristics of the clinical problem as well as on the orthodontist’s personal preference and experience.

Figure 7. (A) Nasoalveolar molding (NAM) appliance showing intraoral molding plate, retention button, and nasal stent attached to the labial flange. (B) Patient with molding plate that is retained by a system of elastics and tapes, engaging the retention button. Note the symmetry of the nasal domes and alar rims. (Color version of figure is available online.)

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Figure 8. (A) Patient with bilateral cleft lip and palate showing protrusion and eversion of the premaxilla and prolabium. Note the nearly absent columella. (B) A bilateral NAM appliance showing 2 intranasal stents and the horizontal columella band. (C) Patient showing bilateral NAM appliance. Note the 2 intranasal stents, the horizontal columella band, and the elastic traction system in place. Note also the vertical tape on the prolabium that places gentle but constant force on the prolabium and columella soft tissues. This force, in conjunction with the gradual increase in the height of the columella band and the forward projection of the nasal stents, is responsible for elongation of the deficient columella. (D) After the retraction of the premaxilla and prolabium. Note the increase in columella length. (E) The same patient after primary surgical repair. Note the scar-free columella and nasal tip projection. (Color version of figure is available online.)

Hyrax or bonded expander with hooks for a protraction face mask is indicated (Fig. 11B). The patient is instructed to activate the expander and to wear the face mask for at least 14 hours per day. The goal is to achieve transverse expansion while overcorrecting the skeletal anteroposterior (A-P) deficiency and to attain an overjet of approximately 2.0 mm to compensate for anticipated maxillary growth deficiency (Fig. 11C).

Preparation for Alveolar Bone Graft Procedures Most unilateral and bilateral alveolar clefts are associated with deficient bone, soft tissue, and missing or abnormally formed (peg, scoliosed) teeth. The most commonly missing tooth is the maxillary lateral incisor, although a central inci-

sor or canine could be absent or deformed as well. When a complete alveolar cleft is present, a secondary bone graft procedure is recommended to facilitate the eruption of the teeth (central, lateral, or canine) found along the margin of the alveolar cleft. If this procedure occurs before the eruption of the adjacent permanent tooth, then there will be adequate bone for its support and acceptable alveolar crest height in the area. The bone graft is usually harvested from the cancellous or marrow portion of the iliac crest. Orthodontic and orthopedic preparation for the secondary alveolar bone graft procedure usually involves arch width coordination by means of a maxillary expander, fixed orthodontic appliances, or both. If oronasal fistulae are present, the amount of expansion is limited by

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Figure 9. (A) Modified Hyrax appliance for transverse maxillary expansion. (B) Quad Helix expansion appliance. Through selected activation of the loops, rotation of first permanent molars, and transverse and anterior expansion can be achieved. (C) Fan expander in closed position. (D) Appliance bonded to occlusal surface of the maxillary dentition. (E) Fan expander opened half way through the activation period. The appliance will expand the anterior part of the palate considerably more than the posterior. (F) Patient with a bilateral cleft lip and palate showing an anterior collapse of the lateral alveolar segments causing a maxillary constriction. (G) A fan expander was used to expand the anterior part of the palate, as the posterior transverse dimension was adequate. Notice the amount of correction achieved and the increased intercanine width. (Color version of figure is available online.)

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Figure 10. Three-way expander bonded to the occlusal surface of molars. Notice the appliance in contact with the lingual surface of maxillary incisors. Upon activation, molding of anterior teeth and alveolar ridge is achieved. (Color version of figure is available online.)

the surgeon’s ability to close such fistulae. Consultation with the surgeon is indicated during the active phase of palatal expansion in order for the surgeon to evaluate the increase in size of the oronasal fistulae. After the proper maxillary arch width and form are achieved, a surgical splint is fabricated and inserted before the completion of the surgical procedure (Fig. 12). The splint should maintain the previously expanded arch width and occlusion, prevent food from affecting and contaminating the surgical site, and remind the patient to take appropriate care of the affected area while eating and of performing oral hygiene.

Adolescent/Adult Orthodontics At the outset of phase II (complete adult dentition) orthodontics, the patient, family, surgeon, and orthodontist must meet and discuss the treatment alternatives, which may include orthodontics alone, or orthodontics in conjunction with orthognathic surgery. At present, the objective is to assess the patient’s skeletal pattern and to determine the best way to meet that patient’s esthetic and functional needs. A decision should be made as to whether the objectives can be achieved by compensation of the dentition or by decompensating and aligning the dentition to their respective skeletal bases. In the mildest skeletal deformities, a normal functional occlusion can be achieved with minimal compromise and good facial esthetics. In mild-to-moderate skeletal deformities, a normal

functional occlusion can be achieved with more significant dental compensations and moderate-topoor facial esthetics. In these cases, a combined surgical/orthodontic treatment plan is recommended. However, some patients might decline this recommendation for psychological, financial, or health reasons. The most severe cases require an orthodontic/surgical approach that cannot be compromised by simple orthodontic compensation of the dentition. Many, if not most, of these patients present with severely crowded teeth, significantly deformed, supernumerary, small, or missing teeth. Therefore, the orthodontic treatment plan should be formulated in consultation with a prosthodontist. The prosthodontist will help to determine the location of future implants, veneers, and possibly bridges. The preservation or creation of space for these prosthetic restorations should be part of the orthodontic treatment plan from the beginning (Fig. 13A). Whenever possible, the prosthodontist is asked to restore the full anatomical size of dwarf or peg teeth before or during treatment with fixed orthodontic appliances. This helps to preserve the appropriate amount of space within the dental arch for the final restoration of full size and natural-looking dentition (Fig. 13B).

Presurgical Orthodontics Most craniofacial disorders present a complex 3D skeletal, soft-tissue, and dental deformity. To provide an accurate diagnosis and treatment plan that could address and resolve the encountered clinical abnormalities, the orthodontist must gather precise, standardized diagnostic records. These include, but are not limited to, a thorough clinical examination (including the dynamics of the functional relationship between teeth and lips), clinical photographs (conventional and 3D if available), articulated dental casts, cephalograms (lateral and posteroanterior), and panoramic x-rays. Where there is ambiguity regarding 3D skeletal/dental relationships that are important to the diagnosis and treatment plan, 3D computed tomography or 3D cone beam images are highly recommended. Complete records are usually taken at the patient’s initial visit with the craniofacial or cleft team. If orthodontics is recommended as part of the patient’s surgical/orthodontic treatment plan,

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Figure 11. (A) Patient showing a Class III skeletal and dental relationship previous to protraction mask therapy. (B) Patient with protraction mask, showing elastics entering the oral cavity and attaching to hooks on the bonded maxillary acrylic appliance. (C) Intraoral photograph during active protraction mask therapy showing the bonded maxillary expander appliance with hooks for mask elastics. Note the amount of maxillary advancement and overjet achieved. (Color version of figure is available online.)

follow-up records are taken to assess progress in preparation for surgery. Postsurgical records should also be taken to evaluate the orthodontic and surgical outcomes. These records are reviewed by the surgeon and orthodontist, with comparisons made to the surgical prediction, splint construction mounted models, and the original treatment plan. After the initial records are completed, they are evaluated and discussed by the Cleft or Craniofacial team members, and a treatment plan is formu-

lated to address the patient’s functional, esthetic, and psychosocial needs. When a combined orthodontic and surgical treatment is recommended, the orthodontist and surgeon should discuss and agree upon a carefully designed and customized integrated treatment plan. This will include common understanding and agreement on the clinical and radiographic findings, treatment objectives, limitations, and risks. When dealing with an actively growing patient, a combined plan might involve one or more orthodontic and surgical inter-

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jet and overbite relationships from canine to canine with acceptable coordination of midlines. To achieve these esthetic and functional goals, it may be necessary to restore the size and shape of the individual incisors or to open a space that is large enough to accommodate an implant or prosthesis (ie, missing lateral incisor). The decision pertaining to space distribution and prosthetic replacement or reconstruction is made with the prosthodontist during the development of the initial orthodontic treatment plan. Sometimes temporary restorations of tooth shape and size are performed before orthodontic brackets are placed on the teeth. This avoids having to maintain space with coils around peg or malformed teeth throughout the course of orthodontic treatment. The objective of this orthodontic surgical or prosthodontic plan is to strive for a functional occlusion with good lip and incisor relationship, appropriate amounts of gingival show at smile, and balance in the various com-

Figure 12. (A) Alveolar bone graft splint. (B) Intraoral view of patient after alveolar bone graft surgery and placement of splint. (Color version of figure is available online.)

ventions at different stages of the child’s development. If one definitive surgical procedure is the treatment of choice, orthodontic and surgical overcorrection might be indicated to anticipate the changes expected from residual craniofacial growth. The presurgical orthodontic treatment objectives should achieve a functional and esthetic occlusion that is consistent with the correction of both skeletal and soft-tissue components of the face. This is usually accomplished by the removal of existing dental compensations and by positioning teeth in an ideal relationship to basal bone. Dental extractions might be required to reduce crowding and to improve alignment of teeth over the alveolar crest. It is also critical to achieve coordination of transverse and anteroposterior maxillary and mandibular arch form. In addition, we attempt to establish Class I over-

Figure 13. (A) Patient showing a small lateral incisor in which adequate space was created to allow a full final restoration of the anatomy. (B) Restored small lateral incisor. (Color version of figure is available online.)

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Figure 14. (A) Mandibular distraction stabilization appliance. The mandibular appliance consists of a lower lingual arch attached to 4 bands with soldered hooks on the lingual and brackets on the labial/buccal surfaces. (B) The maxillary appliance is often an expander or palatal arch attached to 4 bands with soldered hooks on the palatal and brackets on the labial/buccal surfaces. Intermaxillary elastics are used to optimize the position of the mandible throughout the activation phase of distraction osteogenesis. (C and D) Use of cross-tongue elastics. The vector of elastic force results in a couple with the vertical ramus distraction vector. The net effect of these balancing forces maintains the dental midline under the skeletal mid-sagittal plane, while the ramus lengthens vertically and a posterior open bite occurs on the distracted side of the mandible. (Color version of figure is available online.)

ponents of the craniofacial skeleton. These presurgical orthodontic and prosthodontic corrections are assessed by repeated orthodontic study models. The models are hand-articulated into the predicted postsurgical occlusal relationship and studied to determine the necessary presurgical orthodontic and/or prosthetic adjustments. After the planned occlusion is achieved, the patient is then a candidate for surgery.

Appliances A multitude of orthopedic and orthodontic appliances may be used in the pre- and post-

surgical orthodontic treatment stages. These include conventional fixed orthodontic appliances, palatal expansion appliances (including surgically assisted rapid palatal expansion [SARPE]), intra-alveolar distraction appliances, temporary anchorage devices, and plates used for temporary anchorage. During mandibular distraction, the distraction-stabilization appliance described by Hanson and Melugin18 is often used in conjunction with orthodontic intermaxillary elastics (Figs. 14A and 14B). This appliance is one of several that enable the craniofacial orthodontist and surgeon to mold the regenerate and

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Figure 15. (A) Predistraction patient with hemifacial microsomia. Note the oral commissure cant and the short mandibular ramus on the right side. (B) Patient after unilateral mandibular distraction using the distraction stabilization appliance (Figs. 14A and 14B) and cross-tongue elastics (Fig. 14D), showing improved facial symmetry. (C) Intraoral photograph showing the open bite on the side of vertical ramus distraction (right side). (Color version of figure is available online.)

control the direction of distraction (Figs. 14C and 14D). The goal in unilateral mandibular distraction is to place the mandibular body and symphysis into a more normal relationship with the skeletal midsagittal plane and to achieve symmetry of ramus height, the position of gonions (left and right), and a leveled mandibular occlusal plane (Figs. 15A and 15B). In the growing child, overcorrection of each of these relationships is attempted in anticipation of losing some of the corrections due to subsequent asymmetrical growths. In this treatment protocol, it is common to create a posterior open bite on the side on which the ramus is lengthened in the vertical plane (Fig. 15C). It is important to note that the open bite corresponds directly with the development of

the regenerate found in the ramus. As the regenerate is exposed to the compressive forces applied by the muscles of mastication, it is vulnerable to resorption and loss. Therefore, a bite block is placed at the end of the activation phase that completely fills the open bite on the distracted side, providing for bilateral balanced occlusion and protection of the regenerate from the compressive forces of the muscles of mastication (Figs. 16A-16F). The impression for the construction of this bite plate is performed before the placement of distraction devices, as the patient is often unable to tolerate intraoral impressions at the end of the distraction activation phase. Instead, a silicone bite record is made documenting the open bite at the end of the activation phase. This silicone bite is used to

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Figure 16. (A) Intraoral photograph of another patient showing the open bite on the side of vertical ramus distraction (left side). (B) Intraoral view of bite block, providing bilateral balanced occlusion, and protection of the newly formed regenerate. (C and D) The bite block is gradually adjusted to allow for descent of the dentoalveolar structures until teeth reach the mandibular occlusal plane. Note the use of attachments on the teeth and splint that are engaged by elastics to accelerate the downward growth of teeth and alveolar process. (E and F) Intraoral photograph before (E) and after (F) closure of the open bite on the side of vertical ramus distraction. (Color version of figure is available online.)

mount the predistraction study models for fabrication of the bite plate. When performing midface distraction osteogenesis using a rigid external distractor, control of the position of the midface is achieved by using an intraoral tooth-borne appliance that connects directly to the distraction device (Figs. 17A-17G). Control of the dentition and skeletal midface is achieved by altering the vectors that are applied to the appliance through adjustment made in the activation components of the rigid

external distractor device. The craniofacial orthodontist and surgeon establish esthetic, skeletal, dental, and functional goals. These goals are based on shared observations, review of the clinical examination, the chief complaint, and records obtained from collaborating clinicians (ie, otolaryngologist, speech therapist, pediatrician, internist, cardiologist, etc). These records usually include cephalograms, CT scans, cone beam scans, and 2D/3D medical photographs. With the analysis of these records and a global perspective of the

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Figure 17. (A) Intraoral appliance of the rigid external distractor (RED) device consisting of an occlusal splint fabricated on a mesh metal plate and coupling device. (B and C) Extraoral RED device with the intraoral splint attached to the head frame. (D) Patient with Crouzon syndrome before the midface distraction procedure. (E and F) Show the RED device attached to the intraoral appliance during the activation phase. This intraoral appliance can be used to achieve controlled skeletal midface distraction. (G) The patient after distraction. Note the improved facial convexity because of controlled Le Fort III midface advancement. (Color version of figure is available online.)

patient’s problem, an integrated interdisciplinary treatment plan has been formulated. The presurgical orthodontic component of this treatment plan is initiated. After the presurgical dental relationships are achieved, prediction tracings are performed and mock surgery on newly collected records: articulated study models, photographs, radiographs, and sometimes stereolithographic models. The predicted surgical outcome is discussed first with the surgeon and finally with the patient and the patient’s family. After all agree with the proposed treatment plan, surgical splint construction is begun on the articulated dental study models. If two-jaw surgery is indicated, intermediate and final splints will be constructed. In some Craniofacial/Cleft Palate teams, the trained craniofacial orthodontist fabricates these splints and assists the surgeon by inserting and tying in the splint during surgery.

Postsurgical Orthodontics Shortly after surgery, when the patient is able to tolerate postsurgical records, cephalograms are performed to determine whether the surgical treatment plan has been achieved. The surgeon and orthodontist discuss these findings and the surgeon makes a final determination regarding this outcome. Intermaxillary elastics may be used to facilitate or guide the mandibular dentition into the splint bite. However, after midface surgery, the patient may temporarily have difficulty breathing through the nose due to edema and swelling. After the patient is able to breathe through the nose (approximately 5 days from surgery), intermaxillary elastics will be initiated. In the case of distraction osteogenesis, the craniofacial orthodontist might apply intermaxillary elastics during the activation phase to modify

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Figure 17. (continued)

the vector of distraction and to achieve a more precise occlusal outcome. If the objective of mandibular distraction is to achieve vertical ramus lengthening (resulting in posterior open bite), the orthodontist will insert a bite block to fill the posterior open bite at the end of the distraction activation phase. This bite block can also be adjusted to progressively erupt the maxillary dentoalveolar process downward toward the newly positioned mandibular occlusal plane.19 Oral functional physical therapy is often necessary after orthognathic surgery, temporomandibular joint ankylosis release and mandibular

distraction to re-establish normal range of motion and masticatory function. There is a wide range of commercially available devices designed to increase the range of motion and to provide strength training for the muscles of mastication (ie, bite opening devices). In addition, some clinicians use therapies that employ heat/ cold and electrical stimulation among others. In patients with hypotonic orbicularis oris and lip incompetency, a program to enhance lip muscle function may be initiated. In patients who present with an anterior skeletal open bite in conjunction with a prominent tongue thrust, therapy to correct the habit (swallowing exercises) may be im-

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plemented before and/or after surgery. If this habit persists, it could potentially cause dental and/or skeletal relapse. Changes in the mature facial skeleton become progressively more stable during the period of 12 months after surgery. In the year after surgery, retaining the surgically repositioned components of the craniofacial skeleton and refining dental occlusion are focused upon. This is especially true for patients who have had large skeletal movements or when advancing the midface of a patient with a repaired and scarred cleft palate. Therefore, intermaxillary elastics and face mask therapy are used to balance the forces of relapse as needed. After the orthodontic and postsurgical treatment objectives have been met, fixed appliances are removed and orthodontic retainers placed. As in conventional orthodontic therapy, retention appliances are designed to prevent relapse of the original condition. In craniofacial orthodontics, often patients with severe dental and skeletal deformities are treated. Therefore, increased attention is given to retention appliance design and long-term stability. Patients with a cleft palate deformity, who required maxillary expansion during treatment, must be retained with a device, such as the Hawley appliance that provides support to the palatal shelves and dentition. In view of the potential for relapse in the transverse and sagittal dimensions, lifetime retention is highly recommended.

Craniofacial and Special Needs Orthodontics as a Formal Postresidency Fellowship Training Program of Dentistry and Orthodontics Children born with a craniofacial disorder often present with a multitude of medical and dental problems. Consequently, it is advantageous to treat these individuals as part of an interdisciplinary team program, which includes medical, dental, psychological, genetics, speech, audiology, and social service professionals. It is desirable that the specialists in this team have subspecialty training, if available, in craniofacial anomalies and cleft lip and palate. Specialized postresidency fellowship training in Craniofacial and Special Needs Orthodontics has recently been recognized by the American Dental Association and the American Association of Orthodontists.

Patients born with craniofacial disorders are often treated by the craniofacial orthodontist from infancy to adulthood. The earliest form of treatment may be presurgical infant orthopedics. This might be followed by dentofacial orthopedics or collaboration with the surgeon in early interventions. Many of these children will need some form of orthodontic/ orthopedic therapy during primary and mixed dentition (ie, palatal expanders, face mask therapy, preparation for bone graft). Finally, presurgical and preprosthetic orthodontics are initiated around the period of facial/skeletal maturation, in preparation for the definitive craniofacial surgical reconstruction and prosthetics. By the time the postsurgical orthodontic therapy is completed, the craniofacial orthodontist may have formed a very special 20-year experience with the patient and the patient’s family. This relationship, combined with the important clinical contribution to their lives, results in great personal and professional satisfaction.

References 1. MacNeil CK: Orthodontic procedures in the treatment of congenital cleft. Dent Rec 70:126, 1950 2. Gnoinski WM: Infant orthopedics and later orthodontic monitoring for unilateral cleft lip and palate patients in Zurich, in Bardach J, Morris HL (eds): Multidisciplinary Management of Cleft Lip and Palate. Philadelphia, WB Saunders, 1990, pp 578-585 3. Prahl C, Prahl-Anderson B, van’t Hof MA, et al: Infant orthopedics and facial appearance: a randomized clinical trial (DutchCleft). Cleft Palate Craniofac J 43:659, 2006 4. Prahl C, Kuijpers-Jagtman AM, van’t Hof MA, et al: A randomized prospective clinical trial into the effect of infant orthopaedics on maxillary arch dimensions in unilateral cleft lip and palate (Dutchcleft). Eur J Oral Sci 109:297-305, 2001 5. Ross B: Treatment variables affecting facial growth in unilateral cleft lip and palate. Part 2: presurgical orthopedics. Cleft Palate J 24 1:24, 1987 6. Grayson BH, Santiago PE: Presurgical orthopedics for cleft lip and palate, in Aston S, Beasley R, Thorne CH (eds): Grabb and Smith’s Plastic Surgery, (5th ed). Philadelphia, Lippincott-Rave, 1997, pp 237-244 7. Grayson BH, Santiago PE, Brecht LE, et al: Pre-surgical Naso-alveolar molding in patients with cleft lip and palate. Cleft Palate Craniofac J 36:486-498, 1999 8. Grayson BH, Cutting C: Presurgical nasoalveolar orthopedic molding in primary correction of the nose, lip and alveolus of infants born with unilateral and bilateral clefts. Cleft Palate Craniofac J 38:193, 2001

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9. Grayson BH, Maull D: Presurgical nasoalveolar molding for infants born with clefts of the lip, alveolus and palate. Clin Plast Surg 31:149-158, 2004 10. Maul DJ, Grayson BH, Cutting CB, et al: Long-term effects of nasoalveolar molding on three-dimensional nasal shape in unilateral clefts. Cleft Palate Craniofac J 36:391-397, 1999 11. Singh GD, Levy-Bercowski D, Santiago PE: Three-dimensional nasal changes following nasoalveolar molding in patients with unilateral cleft lip and palate: geometric morphometrics. Cleft Palate Craniofac J 42:403-409, 2005 12. Singh GD, Levy-Bercowski D, Yanez MA, et al: Three-dimensional facial morphology following surgical repair of unilateral cleft lip and palate in patients after nasoalveolar molding. Orthod Craniofac Res 10:161-166, 2007 13. Cutting CB, Grayson BH, Brecht LE, et al: Presurgical columellar elongation and primary retrograde nasal reconstruction in one-stage bilateral cleft lip and nose repair. Plast Reconstr Surg 101:3-10, 1999 14. Lee C, Garfinkle J, Warren S, et al: Nasoalveolar molding

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improves appearance of children with bilateral cleft lip and cleft palate. Plast Reconstr Surg 122:1131-1137, 2008 Santiago PE, Grayson BH, Cutting CB, et al: Reduced need for alveolar bone grafting by presurgical orthopedics and primary gingivoperiosteoplasty. Cleft Palate Craniofac J 35:77, 1998 Lee CT, Grayson BH, Cutting CB, et al: Prepubertal midface growth in unilateral cleft lip and palate following alveolar molding and gingivoperiosteoplasty. Cleft Palate Craniofac J 41:375-380, 2004 Garfinkle J, Grayson BH, Brecht LE, et al: Long-term effects on midface growth of gingivoperiosteoplasty with presurgical infant orthopedics in unilateral cleft lip and palate. Prog Am Cleft Pal Craniofac Assoc 63:100, 2006 Hanson PR, Melugin MB: Orthodontic management of the patient undergoing mandibular distraction osteogenesis. Semin Orthod 5:25-34, 1999 Grayson BH, Santiago PE: Treatment planning and biomechanics of distraction osteogenesis from an orthodontic Perspective. Semin Orthod 5:9-24, 1999