Cleft lip and/or palate is one of the most common congenital anomalies affecting the craniofacial structures.1,2 These anomalies are considered a relevant public health issue by the World Health Organization3 due to its effects on facial aesthetics, function and psychosocial challenges for the child as well as for the social and financial burden for the family.1 The incidence of cleft lip and palate is not uniform and varies according to different ethnicities. The global average is approximately one in every 700 newborns.1,4 Increased rates have been reported for Native American and Asian populations at 1 in 500 births. European-derived populations present intermediate prevalence rates at 1 in 1000 and the lowest rates were reported for African populations at 1 in 2500 births.4,5
The treatment for this condition is multidisciplinary and includes different specialties of Medicine and Dentistry during the entire growth phase of the patient. The American Cleft Palate and Craniofacial Association recommends that individuals with craniofacial anomalies, including cleft lip and/or palate, should be evaluated and treated by an interdisciplinary team of specialists representing different disciplines such as: pediatric medicine, plastic surgery, pediatric dentistry, orthodontics, audiology, radiology/medical imaging, genetics/genetic counseling, neurology and neurosurgery, nursing, ophthalmology, oral and maxillofacial surgery, otolaryngology, psychology, social work, and speech-language pathology.6
The principal objective of the multidisciplinary dental treatment of cleft lip and/or palate individuals is to provide facial and dental aesthetics, functional occlusion, improved oral health environment, and speech within a normal range. Furthermore, facial/dental rehabilitation is associated with the development of improved self-esteem and inclusion in to society.7 Thus from the practitioner’s perspective, it is always essential to emphasize to the families that while the treatment process is lengthy and involved, interventions will be made at the ideal stages in order to provide the best outcome in the shortest amount of time.2
The craniofacial orthodontist has a critical role during the care of individuals with cleft lip and/or palate. They are involved with these patients from birth to adulthood providing the following treatments: infant pre-surgical orthopedics, orthodontic preparation for the alveolar bone graft (ABG), early phase of orthodontic treatment after ABG, and comprehensive orthodontic treatment associated with or without orthognathic surgery.7,8 The primary objective of this article is to provide the general dental practitioner an overview of the orthodontic management of individuals born with cleft lip and palate (CLP).
Clinical characteristics presented in CLP
Orofacial clefts are a collection of conditions that includes: clefting of the upper lip, the maxillary alveolus (dental arch), and the hard or soft palate, in various combinations. The anatomic combinations include:
• cleft lip
• cleft lip and alveolus
• cleft lip, alveolus, and palate
• cleft lip and palate (with an intact alveolus)
Clefting of the lip and/or alveolus can occur either unilaterally (one-side) or bilaterally (both sides). The morphological alterations caused by the cleft are related to its severity and classification. A newborn with a unilateral CLP will present extraorally with the nasal alar cartilage on the side of the cleft displaced and flattened, and the tip of the nose deviated toward to the non-cleft side (Fig. 1A). Intraorally there will be separation of the palatal shelves to various degrees, and the palatal segment on the side of the cleft is often tilted medially and superiorly. There is also a direct communication between the oral and nasal cavities on the affected side of the palate. On the other hand, a newborn with bilateral CLP may present with a symmetrical or asymmetrical defect. In a child with complete bilateral CLP, the pre-maxilla is positioned anteriorly and is detached from maxilla10 (Fig. 1B). The median portion of the lip is isolated in the midline and remains attached to the premaxilla and to the columella. The premaxilla typically protrudes considerably forward of the facial profile and the nasal chambers are in direct communication with oral cavity. Intraorally, the palatal processes are divided, and while the nasal septum remains attached to base of skull, it is mobile where it supports the premaxilla and the columella.
Unilateral cleft lip and palate; B. bilateral cleft lip and palate.
The initial plastic surgeries, typically completed before the first year of age, reconstruct the morphology of the lip and palate improving aesthetics and function. However, these procedures have a marked and progressive impact on maxillary growth (Fig. 2). The tension of the reconstructed lip, associated with the scar tissue caused by the cheiloplasty (lip repair) and the palatoplasty (palate repair), have a restrictive effect on maxillary sagittal growth11,12. Therefore, CLP individuals, operated during childhood, frequently present with a Class III skeletal pattern, a concave profile, the lower lip ahead of the upper lip and an anterior crossbite8,13 (Fig. 2). Transverse maxillary deficiency is likely a consequence of the absence of a midpalatal suture and of the iatrogenic effect of the palatoplasty.8,13 Thus, posterior crossbites are commonly observed in individuals with complete palatal clefts8 (Fig. 3).
Extraoral photographs of a female with a history of unilateral cleft lip and palate during growth at ages 8, 15 and 17. Observe that the Class III skeletal pattern is present at different ages.
Intraoral photograph of the malocclusion of the same patient at 17 years of age. Observe among the different dental compensations, the presence of an anterior crossbite.
In this image each row represents a different CLP patient. Note the various anomalies in teeth position, the presence of supernumerary teeth, and posterior and/or anterior crossbites.
Despite the early soft tissue closure of the clefted lip and palate, the alveolar and palatal bone defects of cleft individuals remain, leading to the malposition of teeth adjacent to the cleft8. Frequently observed in CLP individuals are dental anomalies (number, shape, position) and compensatory positioning of the teeth.8,14,15,16 The agenesis of the maxillary lateral incisor, on the affected side, is the most frequent dental anomaly, followed by the presence of a supernumerary lateral incisor located distal to the cleft.12,17,18 Regarding the compensatory positioning of the teeth, the central incisor adjacent to the cleft typically presents with distal crown angulation and mesio/distal rotation, while the lesser segment of the cleft arch presents with a Class II dental relation even in the presence of a skeletal Class III relation. Another recurrent compensatory characteristic in the permanent dentition of CLP individuals is excessive lingual tip of crowns of mandibular premolars and molars as the dentition of the mandibular arch attempts to adapt to the maxillary atresia (Fig. 3).
At the Hospital for Sick Children (HSC) in Toronto, Canada, tracking and evaluation of CLP patient development, is completed through the acquisition of complete orthodontic records (dental casts, photographs: extraoral and intraoral, and radiographs: lateral and frontal cephalometrics, panoramic, occlusal and periapical of the cleft area) at different stages of growth: 1. At six years old, 2. prior to maxillary expansion in preparation to the ABG surgery, 3. Prior to an early phase of orthodontic treatment after ABG and 3. Prior to comprehensive orthodontic treatment with or without orthognathic surgery.
Treatment protocol of CLP patients from birth to early adulthood
1. Naso-alveolar molding therapy
At HSC, the initial appointment in the Orthodontic Clinic occurs after a referral from the Plastic Surgery Department and the Cleft program coordinator within the first or second week after the child is born. During this appointment a detailed medical history of the patient and family is aquired, alongside a complete extraoral and intraoral evaluation of the newborn to classify the type/extension of the cleft and assess for the presence of cleft related natal or neonatal teeth. If natal/neonatal teeth are present, the family is advised of the need for their extraction due to the risk of aspiration, on account of their superficial implantation and their excessive mobility.19
At HSC, naso-alveolar molding (NAM) is provided as routine treatment for unilateral and bilateral CLP. NAM is a non-invasive technique to reshape the gums, lip and nostrils with an acrylic plate before cleft lip and palate surgery. Pre-surgery molding may decrease the difficulty and improve the success of the corrective surgeries, because it makes the cleft less severe.
Once the decision to proceed with the NAM therapy is made, the treatment approach is reviewed with the parents and after obtaining legal consent a maxillary impression is taken with polyvinylsiloxane impression material.20 The NAM appliance is subsequently fabricated with hard dental acrylic on a cast created from the initial impression and is delivered within one week of the impression. Initial fit adjustments to the appliances are completed during the insertion appointment, and specific instructions regarding the use and care with the appliance are provided to the parents. Further modifications to the NAM appliance are made weekly or biweekly in order to guide the alveolar segments into a continually improved position. Once the size of the cleft(s) are reduced and there is a good maxillary arch form achieved, a nasal stent is added to the acrylic plate in order to improve the patient’s nasal form and symmetry20,21,22 (Fig. 4). Corrective surgery is schedule after the NAM is complete with the cheiloplasty commonly done between three to six months of age while the palatoplasty is commonly done between 12-18 months of age.23
Observe, from left to right, the sequence of treatment with NAM of a patient born with a complete bilateral CLP and the subsequent post cheiloplasty result. Significant extraoral and intraoral improvements can be seen.
Even though there is an absence of strong scientific evidence regarding NAM efficacy, its long-term effects and the burden of care for the patient and family, this procedure is used at HSC due to its positive results and clinical preference of the cleft team. From the craniofacial team’s perspective, the use of NAM allows the plastic surgeon to perform a tension-free cheiloplasty, thereby providing a more predictable surgical outcome and a positive impact on the aesthetic of the immediate post-surgical result.20
2. Orthodontic interventions
a. Prior to alveolar bone graft (ABG) surgery
As previously mentioned, individuals with CLP typically present with transverse maxillary deficiency and posterior crossbites. The primary objective of orthodontic treatment during this mixed dentition phase is to prepare the maxillary segments for receiving the ABG, thus maxillary expansion is typically required. Different type of appliances can be used to achieve this goal and the type of appliance used is directly dependent on the maxillary arch form, clinical presentation of the crossbite and on the orthodontist’s preference and personal experience. The most common appliances used at HSC are the fan-shaped and Hyrax maxillary expanders (Fig. 5) with a slow pattern of activation. A quad Helix can also be used to achieve the same objectives.2,8
5A. Occlusal photograph of the maxilla in a Bilateral CLP patient. Note the degree of initial maxillary arch constriction seen. In this case two type of maxillary expanders were used in order to improve arch form and correct the bilateral posterior crossbites. B. fan-shaped expander: this appliance promotes differential expansion of the anterior and posterior areas of the arch. C. Hyrax expander: this device promotes symmetric expansion of the anterior and posterior areas of the arch.
The timing for completing the ABG surgery is critical. This surgical procedure is commonly performed approximately at 8–10 years of age, when the permanent canine in the cleft area presents half to two thirds of root formation and before it erupts into the cleft defect2,8,24. Using the dental age as a parameter to initiate treatment is more important than the chronological age since delayed dental development is usually observed in individuals with clefts.8
The maxillary expander promotes an expansion through widening the cleft. Therefore, there is a lateral positioning of the alveolar segments.25,26 The maxillary expansion is considered completed once the posterior crossbite(s) are corrected and the maxillary arch is preferably over-expanded.2 The plastic surgery department is subsequently notified in order to coordinate a pre-surgical assessment appointment and to enable preparation for the extraction of any deciduous and/or supernumerary (unerupted or erupted) teeth in the cleft area six to eight weeks prior the planned surgical procedure. In the CLP individuals the expansion is not followed by bone formation, as is seen with non-CLP individuals, due to the absence of a medial palatine suture. For this reason, the device must be maintained as a retainer until the ABG surgical date at which time the appliance is removed and a complete set of pre-surgical radiographs is acquired (Fig. 6A).
6A. Panoramic, occlusal and periapical radiographs taken immediately before the alveolar bone graft surgery.
B. Occlusal and periapical radiographs taken 6 months after the alveolar bone graft surgery. The panoramic radiograph
was taken 18 months after the surgery.
The ABG provides several potential advantages to the patient such as: closure of the oro-nasal fistula, repair of the alveolar cleft, bony support and mucosal coverage to the adjacent teeth, bony matrix to support the teeth erupting in the cleft site, stabilization of maxillary segments, and adequate volume of bone for future implant rehabilitation. Also, a successful bone fill at the alveolar cleft may allow for the permanent canine on the affected side to erupt mesially and commonly can be substituted for the missing lateral incisor 2.8.
The patient is clinically evaluated at the Orthodontic Department 6 weeks after the ABG surgery in a coordinated appointment with the Plastic Surgery Department to determine the initial outcome and healing of the ABG site. However, it will be only six months after the bone graft surgery that an occlusal and periapical radiographs will be taken in order to evaluate the definitive surgical results achieved (Fig. 6B). During this stage, the orthodontist will be evaluating the quality of the ABG, the development and pattern of eruption of the dentition, specially of the cuspid in the grafted area.
b. Orthodontics after alveolar bone graft surgery
Once the ABG Surgery is complete, typically no comprehensive orthodontic treatment is indicated as skeletal growth in the CLP patient population continues to be dysplastic and typically definitive corrective treatment is only initiated once the patient approached skeletal maturity. However, in the interim, an early stage of orthodontic treatment may be initiated in specific situations such as: the presence of impacted teeth, severe crowding affecting the periodontal health or if the malocclusion is affecting the patient’s self-esteem. Nevertheless, the patient and family need to be advised that a second stage of fixed orthodontic treatment with or without surgery will be necessary in the future.
As discussed previously, CLP individuals typically present with maxillary deficiency and a Class III malocclusion. In general, there are two alternatives of treatment for individuals with a skeletal pattern and Class III malocclusion: 1. Compensatory Orthodontic Treatment; 2. Orthodontic treatment in preparation for orthognathic surgery.
The orthodontist will evaluate all the clinical and radiographic records of the patient and according to parameters such as: sagittal skeletal relation, maxillary transverse relation, crowding, positioning of the maxillary canine in the cleft area, midline deviation; the craniofacial orthodontist will delineate a personalized treatment plan for the CLP patient.
Comprehensive orthodontic treatment for CLP individuals that do not present with significant skeletal alterations, that have mild/moderate midfacial deficiency, and that have no facial aesthetic concerns, can be initiated at an earlier age. However, careful assessment and monitoring of the growth patterns is essential. The existing alternatives to compensate a Class III malocclusion may include but are not limited to: proclination of the maxillary incisors and retroinclination of mandibular incisors within acceptable limits; orthopaedic traction of the maxilla, extraction of mandibular first premolars in combination with the extraction of the maxillary first premolar on the non-cleft side and canine substitution for the missing lateral incisor on the cleft side and orthodontic finishing with a class I molar relation.2
Comprehensive orthodontic treatment for cases in which the skeletal pattern is classified as moderate (affecting facial aesthetics) or severe should be postponed to the end of the facial growth or at skeletal maturity. In these situations, the skeletal discrepancy will only be corrected with a combined orthodontic and orthognathic surgical treatment approach. Surgically, this may involve an isolated maxillary advancement, mandibular set back, maxillary expansion, a combination of procedures, a repeated ABG and/or distraction osteogenesis procedures (Fig. 7). The pre-surgical orthodontic treatment aims to remove the dental compensations, provide ideal leveling and aligning of the teeth and position the dentition in good relation with the alveolar bone and the jaws. Post-surgical orthodontics aims to finish and detail the occlusion after jaw surgery and prepare the occlusion for possible future prosthetic replacement of missing teeth2,8 (Fig. 7).
Female with Unilateral CLP with significant maxillary hypoplasia and a Class III malocclusion A. Extraoral profile image prior to orthognathic surgery; B. Extraoral profile image post orthognathic surgery involving maxillary advancement.
C. Intraoral images demonstrating initial CLP malocclusion with classic compensatory characteristics: anterior and posterior crossbites, increased lower curve of Spee and deep overbite. D. Intraoral images post orthognathic surgery. This CLP patient was treated with bilateral canine substitution for the missing maxillary lateral incisors.
c. Finishing and retention
The finalization and retention of orthodontic treatment for CLP patients should follow the same principles for non-cleft individuals. Adequate overjet and overbite, intercuspation, coordinated dental arches, root parallelism, mutually protected occlusion should be achieved8. Once the orthodontic treatment is concluded the retention period includes at least: bonded fixed wire retainers and a maxillary Hawley retainer or Essix retainer. Protocols can vary according to the pre-treatment occlusion, final treatment results, and experience and preference of the treating orthodontist.
Consideration regarding missing maxillary lateral incisors
In CLP and non-cleft individuals, the main alternatives for the rehabilitation of missing maxillary lateral incisors include using the canine as a substitute for the missing lateral incisor (Fig. 7) or opening/maintaining the space for an implant placement. The orthodontist and prosthodontist should discuss which treatment alternative is better indicated for each patient considering: the position in which the maxillary canine erupted, the tooth-size discrepancy, and sagittal relationship between the dental arches.
The main advantages of orthodontic space closure are: avoiding the use of dental prosthesis and implants,27,28 preventing complications as gingival level changes in the longterm,29,30 the maintenance of the alveolar graft at the cleft area, providing improved aesthetic outcomes when compared to cases treated with dental implants or prosthetics,31,32 improved stability of the finished result and early completion of the entire treatment.2
The rehabilitation of CLP individuals represents a challenge to the multidisciplinary team. The main goal of the treatment is to provide adequate function and aesthetics of the nose, lips and teeth, improving
patient’s quality of life and self-esteem. The craniofacial orthodontist participates actively in the treatment of individuals with CLP from birth with NAM therapy, then subsequently in preparation for the ABG surgery, to possible early stages of corrective orthodontic treatment and finally to definitive comprehensive orthodontic treatment with or without orthognathic surgery. Orthodontic diagnosis and treatment planning in CLP patients during the mixed and permanent dentition follow the same principles as used in non-cleft individuals. However, special attention should be made in the CLP population regarding specific times of intervention, understanding of CLP patterns and characteristics and coordination of care with other disciplines.
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About the Author
Dr. Aura Sofia C. Manfio is a Specialist in Orthodontics graduated from the Pontifical Catholic University of Rio Grande do Sul (PUCRS, Brazil), received a Master’s Degree in Craniofacial Anomalies from the University of Sao Paulo (USP, Brazil) and is currently a Clinical Fellow in the Orthodontic Department at the Hospital for Sick Children in Toronto. Contact information: email@example.com.
Dr. Austin Chen BSc, DDS, MSc (Ortho), FRCD(C) is a Staff Orthodontist at the Hospital for Sick Children, Associate Professor at the University of Toronto, Exam Coordinator for the Royal College of Dentists of Canada and is in private practice in Vaughan, Ontario. He can be reached at firstname.lastname@example.org.
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