- Email: [email protected]
Collaborative care and the modern craniofacial treatment team
Author’s Accepted Manuscript Collaborative Care and the Modern Craniofacial Treatment Team Travis L. Gibson, Pradip R. Shetye
www.elsevier.com/locate/ysodo
PII: DOI: Reference:
S1073-8746(17)30022-1 http://dx.doi.org/10.1053/j.sodo.2017.05.001 YSODO499
To appear in: Seminars in Orthodontics Cite this article as: Travis L. Gibson and Pradip R. Shetye, Collaborative Care and the Modern Craniofacial Treatment Team, Seminars in Orthodontics, http://dx.doi.org/10.1053/j.sodo.2017.05.001 This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting galley proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
Collaborative Care and the Modern Craniofacial Treatment Team Gibson, TL; Shetye, PR
Travis L. Gibson, DMD, MSc, FRCDC(C) Craniofacial Orthodontic Fellow Hansjörg Wyss Department of Plastic Surgery, NYU Langone Medical Center, New York City
Pradip R. Shetye, DDS, MDS Assistant Professor of Plastic Surgery (Craniofacial Orthodontics) Director, Craniofacial Orthodontics and Craniofacial Orthodontic Fellowship Program Hansjörg Wyss Department of Plastic Surgery, NYU Langone Medical Center, New York City
Email: [email protected]
From: Hansjörg Wyss Department of Plastic Surgery, NYU Langone Medical Center, New York City Mailing address:Hansjörg Wyss Department of Plastic Surgery 307 East 33rd Street Lower Level New York, New York 10016 Tel: 212.263.5206
Abstract Cleft lip and palate is a complex craniofacial anomaly typically requiring treatment from a range of specialists to produce excellent outcomes. Due to the challenges of coordinating this extensive range of specialists, treatment is best provided by a centralized cleft lip and palate treatment team. This article outlines the members of a modern treatment team, their contributions and responsibilities in patient care, and the benefits to both patient and practitioner when treatment is provided by an experienced multidisciplinary team.
Introduction With a combined prevalence of 17 per 10,000 live births, orofacial clefting affects approximately 7000 children born in the United States annually. The most common presentation – cleft lip with or without cleft palate – has a national prevalence of 10.63 per 10,000 live births, followed by isolated cleft palate with a prevalence of 6.35 per 10,000 live births1. When combined, cleft lip and palate (all presentations) is the one of the most common birth anomalies reported, surpassing even Down Syndrome in prevalence1.
For affected children fortunate enough to be born in an area with access to treatment for cleft lip and palate, they and their families can expect two decades or more of treatment involving multiple disciplines of medicine and the allied health professions, spanning from the early post-natal period to young adulthood. Over
the past century, increased recognition of the complexity of such treatment has lead to the development of team-based interdisciplinary care2, in which the involved specialists work together at a centralized location. At present, the American Cleft Palate Association recommends that all patients with craniofacial anomalies, including cleft lip and palate, be managed by interdisciplinary teams of specialists3. The purpose of this article is to provide an overview of the members of a modern craniofacial treatment team, the important contributions of each discipline in providing the best possible care to patients, and the benefits offered by a collaborate interdisciplinary relationship.
Disciplines of the Craniofacial Team As outlined in the Parameters for the Evaluation and Treatment published by the American Cleft Palate-Craniofacial Association3, recommended specialist disciplines involved in an interdisciplinary treatment team include anesthesiology, audiology, radiology/medical imaging, genetics/genetic counseling, neurology & neurosurgery, nursing, ophthalmology, oral and maxillofacial/plastic surgery, orthodontics, otolaryngology, pediatric medicine, pediatric dentistry, psychology, social work, and speech-language pathology. At present, teams require participation of speechlanguage pathology, orthodontics, and surgical specialties to be recognized as an approved treatment team by the ACPA (http://www.acpacpf.org/team_care/standards). Furthermore, demonstrable access to other disciplines outlined above is also required, as each discipline from this extensive
collaboration provides specific expertise and contributions (Table 1) to the care of individuals with cleft lip and palate, many of which are briefly outlined below.
Pediatric Medicine Children born with cleft lip and palate present with significantly complex treatment needs. In addition to the cleft anomaly itself, cleft lip and palate has been associated with a range of other medical conditions and malformations, including those involving the central nervous system (anencephaly, hydrocephaly), vertebral column heart (atrial and ventricular septal defects), limbs (limb deficiencies, polyand syndactyly, club foot), and other craniofacial structures4,5. As a results of this complex presentation and the range of systems which may be involved, initial evaluation of patients by a pediatrician is required, and should occur shortly after birth and before hospital discharge6. Subsequently, a pediatrician is generally the most appropriate primary care physician for affected children3. Responsibilities of the pediatrician include regular physical examination, identification of parental concerns, and education of parents on child-specific needs3. Due to an increased risk of growth failure or delayed development, frequent monitoring is important, and may be performed in conjunction with a nurse practitioner as discussed below. Practical considerations such as geographic location will determine if the affected child is best followed by a cleft and craniofacial team pediatrician, or by a pediatrician in their own community. If a community pediatrician is the child’s primary care physician, close coordination with the overseeing cleft and craniofacial team is of critical importance.
Surgery in Cleft Lip/Palate Repair Surgical interventions for cleft lip/palate repair vary widely by treatment centre7 and individual patient presentation. Surgical interventions within the first year may include lip adhesion, lip closure (chelioplasty), primary rhinoplasty, and gingivoperiosteoplasty, as well as combination operations which combine two or more of the above procedures7. Palate closure (palatoplasty) frequently occurs by 18 months of age3, but may occur earlier, or be combined with some of the earlier surgical interventions outlined above8. In addition to the astounding variation in surgical treatment protocols reported, the surgical specialty responsible for these interventions varies, and may include plastic surgery, oral and maxillofacial surgery, pediatric surgery, head and neck surgery, and otolaryngology6,7.
Surgical involvement during childhood and adolescence may include revisions and repairs to previous surgeries, bone grafting, and surgical interventions for the treatment of velopharyngeal dysfunction6. Severe skeletal discrepancies associated with functional limitations, airway compromise, or psychological distress may be treated during this period by distraction osteogenesis9-11. It should be noted that whenever appropriate, correction of skeletal discrepancies is best delayed until growth completion to prevent growth-associated relapse of the surgical correction12. Similarly, definitive rhinoplasty and nasal septal surgeries should be delayed until growth completion except in cases of significant airway compromise or nasal deformity3. Thus, the involvement of a surgical specialist will frequently be
required throughout the treatment of individuals with cleft lip and palate, from shortly after birth into young adulthood.
Anesthesiology Involvement of team anesthesiologists is intimately related to the surgical interventions outlined above. Due to the young age of patients at the time of surgical intervention, as well as additional complicating factors such as the increased likelihood of airway obstructions13,14 and cardiac anomalies4,5 the presence of an anesthesiologist specifically experienced in pediatric anesthesiology is recommended3.
Orthodontics Orthodontic treatment for individuals born with cleft lip and palate may begin as early as a few weeks of age. Treatments provided at this age are better referred to as “orthopedic” as there are no teeth erupted, with treatment instead focusing on the movement of bony segments and surrounding soft tissues prior to lip repair. Numerous pre-surgical infant orthopedic methods and appliances have been reported over the past 4 to 5 decades, including lip taping, infant orthopedics with intraoral plates, and most recently nasoalveolar molding15. At present, these treatments are offered by approximately 1/3 of cleft lip and palate treatment centers in the United States16.
Prior to alveolar bone grafting between 6 and 10 years of age, orthodontic treatment is initiated to expand the alveolar arch and align teeth away from the cleft site – this provides surgical access and corrects the transverse maxillary constriction which develops secondary to surgical repair17. Orthodontic assessment of dental development is of use in determining the timing of alveolar bond grafting, as surgical intervention prior to canine eruption may increase graft success18,19. Reverse-pull headgear may also be prescribed during this treatment phase, though the long-term benefits of maxillary protraction in this population remain unclear2022
and research is ongoing. In the teenage years, conventional complete orthodontic
treatment may provide a functional and aesthetic occlusion. For patients with relatively minor maxillary hypoplasia, only orthodontic treatment is required, whereas for patients presenting with more significant maxillary hypoplasia, orthognathic surgery may be indicated23. As noted above, orthognathic surgery is generally delayed until growth completion to prevent post-surgical growth from causing relapse of the surgical correction12.
Dentistry In addition to routine dental care, individuals with cleft lip and palate require management of an increased prevalence of dental anomalies, including missing and supernumerary teeth, microdontia, ectopic eruption and impaction, enamel hypoplasia, delayed dental maturation, and periodontal compromise3,24. Given the complexity of such presentations, a pediatric dentist is often the specialist of choice for management during childhood, and can be of benefit in promoting good dental
habits and oral hygiene early in life, as well as in acclimatizing patients to examination of the oral cavity. Involvement of a periodontist is of benefit for the maintenance and augmentation of supporting tissues as required, with the goal of minimize tooth loss in patients who may already be missing teeth. For those patients who require tooth replacement, a prosthodontist is often the practitioner of choice. A prosthodontist may also be involved in the fabrication of removable appliances for fistula obturation or the treatment of velopharyngeal dysfunction in children3.
Audiology Children with craniofacial anomalies are at an increased risk for hearing disorders, due to both congenital abnormalities of auditory structures, as well susceptibility to recurrent otitis media secondary to dysfunction of soft palate muscles which normally promote opening of the pharyngotympanic tube and drainage of the middle ear3. As a result, audiologic testing should occur shortly after birth, with follow-up examinations through adolescence3. For patients diagnosed with hearing loss, amplification or bone conduction devices should be considered, and require regular monitoring by an audiologist to ensure correct functioning.
Genetics, Dysmorphology, and Genetic Counseling Cleft lip and palate may occur in isolation secondary to multifactorial environmental and polygenetic risk factors, or as part of a recognized syndrome, frequently with single gene inheritance8,25. Genetic diagnosis and determination of cleft etiology is
critical for providing parents counseling regarding recurrence risk in subsequent pregnancies3, and in determining the likelihood and prognosis of associated challenges such as developmental delay or deficits. These team members can also provide counseling for adolescents and young adults who were born with cleft lip and palate, with respect to family planning and hereditary risks.
Speech and Language Pathology The importance of speech and language pathology in the care of children born with cleft lip/palate was recognized early, with this discipline included in treatment as early as 19322. Children with cleft lip/palate have a greatly increased risk of speech and language disorders. Contributory factors are extensive, including both disruption of articulatory components (ex: dental and skeletal malocclusion, palatal clefts/fistulae) and resonance (impaired velopharyngeal closure)26. Additionally, variations in phonation or voice (hoarsness, habitual unusual volume or pitch) are more commonly seen in this population, and an increased prevalence of hearing impairment may also contribute to language difficulties. Accurate identification of the cause of speech errors is critical for providing the appropriate corrective intervention, which can range from speech therapy to surgery. Additionally, some surgical interventions such as maxillary advancement may have adverse effects on speech27, and the assessment of such risks is an important component of informed consent.
Psychology and Social Services Individuals born with cleft lip/palate, as well as their families and caregivers, present with unique psychological needs related to adaptation to social response, treatment burden, and other challenges. Individuals with cleft lip and palate have been reported to suffer decreased self-confidence28,29, depression30, and decreased oral health-related quality of life31. Parents and caregivers may also suffer significant psychological distress32. Involvement of mental health specialists on a treatment team is critical for both identifying concerns, and for accessing the appropriate assistance and services to promote mental well-being in patients and their families.
Nursing As outlined above, the treatment of individuals with cleft lip/palate involves the coordinated involvement of multiple specialties over many years. For many treatment teams, the responsibility for coordinating this treatment is assumed by a nursing practitioner acting team coordinator3. This practitioner is often the pointperson for patients and their families: both initiating contact and providing continuity of care through repeated follow-up. Their frequent involvement and contact with patients and their families makes team nurses ideally situated to monitor growth and development, and to provide education and reinforcement on a wide range of topics such as feeding, anticipated treatment, and wound care3. Team nurses may also act as advocates for patients and their families, guiding them in obtaining appropriate services.
Advantages of an Interdisciplinary Team A key aspect of the interdisciplinary cleft treatment team is the integration and close working relationship of the specialists involved. Integrated case management through collaborative and often simultaneous assessment and planning with team members from multiple disciplines ensures efficient use of patient and caregiver time and resources. Even if a particular specialty is not present, through the use of centralized multi-disciplinary records, practitioners have ready access to information which would otherwise require referral and potentially significant waiting periods to obtain. Furthermore, the presence of multiple specialties during patient assessment and treatment planning meetings may further benefit the patient in coordinating the sequencing and timing of inter-dependent treatments such as orthodontic treatment and alveolar bone grafting. Similarly, input from multiple disciplines may help balance the benefits and risks of interventions; for example, the potentially detrimental effect of orthognathic surgery on speech resonance. Centralizing treatment for all patients in a large region at a treatment center is also of benefit in ensuring that the treating professionals maintain adequate experience and involvement over time. Despite being a relatively common birth anomaly, cleft lip and palate is rare enough that a single practitioner outside of a treatment center could only expect to encounter a few affected patients in their career. An extensive group of studies conducted in the United Kingdom comparing outcomes before and after centralization of cleft and craniofacial care support these benefits, with improvements noted in length of post-surgical hospital stay33, facial
appearance34, dentoalveolar relationships34, patient self-confidence35, and a range of speech outcomes36 after centralization of care. For these reasons, the ACPA recommends that all patients born with cleft lip and palate or other craniofacial anomalies receive care from a treatment team6.
Conclusion Providing care for children born with cleft lip and palate is a complex, challenging, and ultimately rewarding undertaking requiring the input of an extensive team of dedicated healthcare professionals. Through participation in an interdisciplinary team, practitioners help ensure the provision of quality care suited to the individual needs of their patients.
Table 1: Key contributions and responsibilities of team members by discipline Audiology
- neonatal hearing tests - follow-up examinations through adolescence - maintenance of hearing assistance devices
Genetics, Dysmorphology, and Genetic Counseling
- genetic diagnosis, determination of cleft etiology - recurrence risk and prognosis counseling
Nursing
- frequently act as team coordinator
- patient education and support - monitoring of growth and development
Orthodontics
- pre-surgical orthopedics, nasoalveolar molding - interceptive treatment including expansion and maxillary protraction - assessment of dental development for informing surgical timing - definitive orthodontic treatment +/- orthognathic surgery
Otolaryngology
- monitoring for development of middle ear disease - myringotomy, tympanotomy tubes - surgical intervention for velopharyngeal dysfunction
Pediatric Medicine
- initial systemic examination - monitoring of growth and development - treatment and re-assessment of associated medical conditions
Psychology and Social Work
- evaluation and treatment of emotional, learning, developmental, and adjustment disorders
- consideration provided for both well-being of patient and family members
Speech and Language Pathology
- evaluation of articulation, resonance, phonation/voice, language development - speech therapy and/or identification of other treatments indicated for the improvement of speech anomalies
Surgery (Plastic, Oral and Maxillofacial, Pediatrics) AND Anesthesiology
- surgical interventions including, but not limited to: - lip adhesion - primary repair surgeries including chelioplasty, nasoplasty, gingivoperiosteoplasty, palatoplasty - alveolar bone grafting - surgical intervention for velopharyngeal dysfunction - orthognathic surgery
Works Cited 1.
Parker SE, Mai CT, Canfield MA, et al. Updated national birth prevalence estimates for selected birth defects in the United States, 2004-2006. Kirby RS, Collins JS, eds. Birth Defect Res A. 2010;88(12):1008-1016. doi:10.1002/bdra.20735.
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Vig KWL, Mercado AM. Overview of orthodontic care for children with cleft lip and palate, 1915-2015. Am J Orthod Dentofacial Orthop. 2015;148(4):543-556.
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American Cleft Palate-Craniofacial Association. Parameters for Evaluation and Treatment of Patients with Cleft Lip/Palate or Other Craniofacial Anomalies. Cleft Palate Craniofac J. 1993;30(Suppl 1):1-34.
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Stoll C, Alembik Y, Dott B, Roth MP. Associated malformations in cases with oral clefts. The Cleft Palate-Craniofacial Journal. 2000;37(1):41-47.
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Berkowitz S, ed. Core Curriculum for Cleft Lip/Palate and Other Craniofacial Anomalies. Berlin/Heidelberg: Springer-Verlag; 2006:285-300.
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Shaw WC, Semb G, Nelson P, et al. The Eurocleft project 1996-2000: overview. Journal of Cranio-Maxillofacial Surgery. 2001;29(3):131–40–discussion141–2.
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Mossey PA, Little J, Munger RG, Dixon MJ, Shaw WC. Cleft lip and palate. Lancet. 2009;374(9703):1773-1785.
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Rhee ST, Buchman SR. Pediatric mandibular distraction osteogenesis: the present and the future. Journal of Craniofacial Surgery. 2003;14(5):803-808.
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McCarthy JG, Stelnicki EJ, Grayson BH. Distraction osteogenesis of the mandible: a ten-year experience. Seminars in Orthodontics. 1999;5(1):3-8.
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Grayson BH, Santiago PE. Treatment planning and biomechanics of distraction osteogenesis from an orthodontic perspective. Seminars in Orthodontics. 1999;5(1):9-24.
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Wolford LM, Stevao ELL. Correction of jaw deformities in patients with cleft lip and palate. Proc (Bayl Univ Med Cent). 2002;15(3):250-254.
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Somerville N, Fenlon S. Anaesthesia for cleft lip and palate surgery. Contin Educ Anaesth Crit Care Pain. 2005;5(3):76-79.
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Van Boven MJ, Pendeville PE, Veyckemans F, et al. Neonatal Cleft Lip Repair: The Anesthesiologist's Point of View. The Cleft Palate-Craniofacial Journal. 1993;30(6):574-578..
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Grayson BH, Garfinkle JS. Early cleft management: The case for nasoalveolar molding. American Journal of Orthodontics and Dentofacial Orthopedics. 2014;145(2):134-142.
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Sischo L, Chan JW, Stein M, Smith C, van Aalst J, Broder HL. Nasoalveolar molding: prevalence of cleft centers offering NAM and who seeks it. Cleft Palate Craniofac J. 2012;49(3):270-275.
17.
Berkowitz S. Cleft Lip and Palate: Diagnosis and Management. 2013.
18.
Enemark H, Sindet-Pedersen S, Bundgaard M. Long-Term Results After Secondary Bone-Grafting of Alveolar Clefts. J Oral Maxillofac Surg. 1987;45(11):913-918.
19.
Freihofer H, Borstlap WA, Kuijpers-Jagtman AM, et al. Timing and Transplant
Materials for Closure of Alveolar Clefts - a Clinical Comparison of 296 Cases. J Maxillofac Surg. 1993;21(4):143-148. 20.
Meazzini MC, Capello AV, Ventrini F, et al. Long-term follow-up of UCLP patients: surgical and orthodontic burden of care during growth and final orthognathic surgery need. Cleft Palate Craniofac J. 2015;52(6):688-697.
21.
Tome W, Yashiro K, Kogo M, Yamashiro T. Cephalometric Outcomes of Maxillary Expansion and Protraction in Patients With Unilateral Cleft Lip and Palate After Two Types of Palatoplasty. Cleft Palate Craniofac J. September 2015.
22.
So LL. Effects of reverse headgear treatment on sagittal correction in girls born with unilateral complete cleft lip and cleft palate--skeletal and dental changes. American Journal of Orthodontics and Dentofacial Orthopedics. 1996;109(2):140-147.
23.
Daskalogiannakis J, Mehta M. The need for orthognathic surgery in patients with repaired complete unilateral and complete bilateral cleft lip and palate. The Cleft Palate-Craniofacial Journal. 2009;46(5):498-502. doi:10.1597/08176.1.
24.
Akcam MO, Evirgen S, Uslu O, Memikoğlu UT. Dental anomalies in individuals with cleft lip and/or palate. The European Journal of Orthodontics. 2010;32(2):207-213.
25.
Dixon MJ, Marazita ML, Beaty TH, Murray JC. Cleft lip and palate: understanding genetic and environmental influences. Nat Rev Genet. 2011;12(3):167-178.
26.
Kuehn DP, Moller KT. The state of the art: Speech and language issues in the cleft palate population. The Cleft Palate-Craniofacial Journal. 2000;37(4):348348.
27.
Taha M, Elsheikh YM. Velopharyngeal changes after maxillary distraction in cleft patients using a rigid external distraction device: A retrospective study. Angle Orthod. March 2016:011216–33.1–7.
28.
Turner SR, Thomas P, Dowell T, Rumsey N, Sandy JR. Psychological outcomes amongst cleft patients and their families. British Journal of Plastic Surgery. 1997;50(1):1-9.
29.
Broder H, Strauss RP. Self-concept of early primary school age children with visible or invisible defects. Cleft Palate J. 1989;26(2):114–7.
30.
Hunt O, Burden D, Hepper P, Stevenson M, Johnston C. Self-reports of psychosocial functioning among children and young adults with cleft lip and palate. Cleft Palate Craniofac J. 2006;43(5):598-605.
31.
Wehby GL, Cassell CH. The impact of orofacial clefts on quality of life and healthcare use and costs. Oral Dis. 2010;16(1):3-10.
32.
Hasanzadeh N, Khoda MO, Jahanbin A, Vatankhah M. Coping strategies and psychological distress among mothers of patients with nonsyndromic cleft lip and palate and the family impact of this disorder. J Craniofac Surg. 2014;25(2):441-445.
33.
Fitzsimons KJ, Mukarram S, Copley LP, Deacon SA, van der Meulen JH. Centralisation of services for children with cleft lip or palate in England: a study of hospital episode statistics. BMC Health Serv Res. 2012;12(1):148.
34.
Al-Ghatam R, Jones TEM, Ireland AJ, et al. Structural outcomes in the Cleft Care UK study. Part 2: dento-facial outcomes. Orthodontics & Craniofacial Research. 2015;18 Suppl 2:14-24.
35.
Waylen A, Ness AR, Wills AK, Persson M, Rumsey N, Sandy JR. Cleft Care UK study. Part 5: child psychosocial outcomes and satisfaction with cleft services. Orthodontics & Craniofacial Research. 2015;18:47-55.
36.
Sell D, Mildinhall S, Albery L, Wills AK, Sandy JR, Ness AR. The Cleft Care UK study. Part 4: perceptual speech outcomes. Orthodontics & Craniofacial Research. 2015;18 Suppl 2:36-46.
www.elsevier.com/locate/ysodo
PII: DOI: Reference:
S1073-8746(17)30022-1 http://dx.doi.org/10.1053/j.sodo.2017.05.001 YSODO499
To appear in: Seminars in Orthodontics Cite this article as: Travis L. Gibson and Pradip R. Shetye, Collaborative Care and the Modern Craniofacial Treatment Team, Seminars in Orthodontics, http://dx.doi.org/10.1053/j.sodo.2017.05.001 This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting galley proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
Collaborative Care and the Modern Craniofacial Treatment Team Gibson, TL; Shetye, PR
Travis L. Gibson, DMD, MSc, FRCDC(C) Craniofacial Orthodontic Fellow Hansjörg Wyss Department of Plastic Surgery, NYU Langone Medical Center, New York City
Pradip R. Shetye, DDS, MDS Assistant Professor of Plastic Surgery (Craniofacial Orthodontics) Director, Craniofacial Orthodontics and Craniofacial Orthodontic Fellowship Program Hansjörg Wyss Department of Plastic Surgery, NYU Langone Medical Center, New York City
Email: [email protected]
From: Hansjörg Wyss Department of Plastic Surgery, NYU Langone Medical Center, New York City Mailing address:Hansjörg Wyss Department of Plastic Surgery 307 East 33rd Street Lower Level New York, New York 10016 Tel: 212.263.5206
Abstract Cleft lip and palate is a complex craniofacial anomaly typically requiring treatment from a range of specialists to produce excellent outcomes. Due to the challenges of coordinating this extensive range of specialists, treatment is best provided by a centralized cleft lip and palate treatment team. This article outlines the members of a modern treatment team, their contributions and responsibilities in patient care, and the benefits to both patient and practitioner when treatment is provided by an experienced multidisciplinary team.
Introduction With a combined prevalence of 17 per 10,000 live births, orofacial clefting affects approximately 7000 children born in the United States annually. The most common presentation – cleft lip with or without cleft palate – has a national prevalence of 10.63 per 10,000 live births, followed by isolated cleft palate with a prevalence of 6.35 per 10,000 live births1. When combined, cleft lip and palate (all presentations) is the one of the most common birth anomalies reported, surpassing even Down Syndrome in prevalence1.
For affected children fortunate enough to be born in an area with access to treatment for cleft lip and palate, they and their families can expect two decades or more of treatment involving multiple disciplines of medicine and the allied health professions, spanning from the early post-natal period to young adulthood. Over
the past century, increased recognition of the complexity of such treatment has lead to the development of team-based interdisciplinary care2, in which the involved specialists work together at a centralized location. At present, the American Cleft Palate Association recommends that all patients with craniofacial anomalies, including cleft lip and palate, be managed by interdisciplinary teams of specialists3. The purpose of this article is to provide an overview of the members of a modern craniofacial treatment team, the important contributions of each discipline in providing the best possible care to patients, and the benefits offered by a collaborate interdisciplinary relationship.
Disciplines of the Craniofacial Team As outlined in the Parameters for the Evaluation and Treatment published by the American Cleft Palate-Craniofacial Association3, recommended specialist disciplines involved in an interdisciplinary treatment team include anesthesiology, audiology, radiology/medical imaging, genetics/genetic counseling, neurology & neurosurgery, nursing, ophthalmology, oral and maxillofacial/plastic surgery, orthodontics, otolaryngology, pediatric medicine, pediatric dentistry, psychology, social work, and speech-language pathology. At present, teams require participation of speechlanguage pathology, orthodontics, and surgical specialties to be recognized as an approved treatment team by the ACPA (http://www.acpacpf.org/team_care/standards). Furthermore, demonstrable access to other disciplines outlined above is also required, as each discipline from this extensive
collaboration provides specific expertise and contributions (Table 1) to the care of individuals with cleft lip and palate, many of which are briefly outlined below.
Pediatric Medicine Children born with cleft lip and palate present with significantly complex treatment needs. In addition to the cleft anomaly itself, cleft lip and palate has been associated with a range of other medical conditions and malformations, including those involving the central nervous system (anencephaly, hydrocephaly), vertebral column heart (atrial and ventricular septal defects), limbs (limb deficiencies, polyand syndactyly, club foot), and other craniofacial structures4,5. As a results of this complex presentation and the range of systems which may be involved, initial evaluation of patients by a pediatrician is required, and should occur shortly after birth and before hospital discharge6. Subsequently, a pediatrician is generally the most appropriate primary care physician for affected children3. Responsibilities of the pediatrician include regular physical examination, identification of parental concerns, and education of parents on child-specific needs3. Due to an increased risk of growth failure or delayed development, frequent monitoring is important, and may be performed in conjunction with a nurse practitioner as discussed below. Practical considerations such as geographic location will determine if the affected child is best followed by a cleft and craniofacial team pediatrician, or by a pediatrician in their own community. If a community pediatrician is the child’s primary care physician, close coordination with the overseeing cleft and craniofacial team is of critical importance.
Surgery in Cleft Lip/Palate Repair Surgical interventions for cleft lip/palate repair vary widely by treatment centre7 and individual patient presentation. Surgical interventions within the first year may include lip adhesion, lip closure (chelioplasty), primary rhinoplasty, and gingivoperiosteoplasty, as well as combination operations which combine two or more of the above procedures7. Palate closure (palatoplasty) frequently occurs by 18 months of age3, but may occur earlier, or be combined with some of the earlier surgical interventions outlined above8. In addition to the astounding variation in surgical treatment protocols reported, the surgical specialty responsible for these interventions varies, and may include plastic surgery, oral and maxillofacial surgery, pediatric surgery, head and neck surgery, and otolaryngology6,7.
Surgical involvement during childhood and adolescence may include revisions and repairs to previous surgeries, bone grafting, and surgical interventions for the treatment of velopharyngeal dysfunction6. Severe skeletal discrepancies associated with functional limitations, airway compromise, or psychological distress may be treated during this period by distraction osteogenesis9-11. It should be noted that whenever appropriate, correction of skeletal discrepancies is best delayed until growth completion to prevent growth-associated relapse of the surgical correction12. Similarly, definitive rhinoplasty and nasal septal surgeries should be delayed until growth completion except in cases of significant airway compromise or nasal deformity3. Thus, the involvement of a surgical specialist will frequently be
required throughout the treatment of individuals with cleft lip and palate, from shortly after birth into young adulthood.
Anesthesiology Involvement of team anesthesiologists is intimately related to the surgical interventions outlined above. Due to the young age of patients at the time of surgical intervention, as well as additional complicating factors such as the increased likelihood of airway obstructions13,14 and cardiac anomalies4,5 the presence of an anesthesiologist specifically experienced in pediatric anesthesiology is recommended3.
Orthodontics Orthodontic treatment for individuals born with cleft lip and palate may begin as early as a few weeks of age. Treatments provided at this age are better referred to as “orthopedic” as there are no teeth erupted, with treatment instead focusing on the movement of bony segments and surrounding soft tissues prior to lip repair. Numerous pre-surgical infant orthopedic methods and appliances have been reported over the past 4 to 5 decades, including lip taping, infant orthopedics with intraoral plates, and most recently nasoalveolar molding15. At present, these treatments are offered by approximately 1/3 of cleft lip and palate treatment centers in the United States16.
Prior to alveolar bone grafting between 6 and 10 years of age, orthodontic treatment is initiated to expand the alveolar arch and align teeth away from the cleft site – this provides surgical access and corrects the transverse maxillary constriction which develops secondary to surgical repair17. Orthodontic assessment of dental development is of use in determining the timing of alveolar bond grafting, as surgical intervention prior to canine eruption may increase graft success18,19. Reverse-pull headgear may also be prescribed during this treatment phase, though the long-term benefits of maxillary protraction in this population remain unclear2022
and research is ongoing. In the teenage years, conventional complete orthodontic
treatment may provide a functional and aesthetic occlusion. For patients with relatively minor maxillary hypoplasia, only orthodontic treatment is required, whereas for patients presenting with more significant maxillary hypoplasia, orthognathic surgery may be indicated23. As noted above, orthognathic surgery is generally delayed until growth completion to prevent post-surgical growth from causing relapse of the surgical correction12.
Dentistry In addition to routine dental care, individuals with cleft lip and palate require management of an increased prevalence of dental anomalies, including missing and supernumerary teeth, microdontia, ectopic eruption and impaction, enamel hypoplasia, delayed dental maturation, and periodontal compromise3,24. Given the complexity of such presentations, a pediatric dentist is often the specialist of choice for management during childhood, and can be of benefit in promoting good dental
habits and oral hygiene early in life, as well as in acclimatizing patients to examination of the oral cavity. Involvement of a periodontist is of benefit for the maintenance and augmentation of supporting tissues as required, with the goal of minimize tooth loss in patients who may already be missing teeth. For those patients who require tooth replacement, a prosthodontist is often the practitioner of choice. A prosthodontist may also be involved in the fabrication of removable appliances for fistula obturation or the treatment of velopharyngeal dysfunction in children3.
Audiology Children with craniofacial anomalies are at an increased risk for hearing disorders, due to both congenital abnormalities of auditory structures, as well susceptibility to recurrent otitis media secondary to dysfunction of soft palate muscles which normally promote opening of the pharyngotympanic tube and drainage of the middle ear3. As a result, audiologic testing should occur shortly after birth, with follow-up examinations through adolescence3. For patients diagnosed with hearing loss, amplification or bone conduction devices should be considered, and require regular monitoring by an audiologist to ensure correct functioning.
Genetics, Dysmorphology, and Genetic Counseling Cleft lip and palate may occur in isolation secondary to multifactorial environmental and polygenetic risk factors, or as part of a recognized syndrome, frequently with single gene inheritance8,25. Genetic diagnosis and determination of cleft etiology is
critical for providing parents counseling regarding recurrence risk in subsequent pregnancies3, and in determining the likelihood and prognosis of associated challenges such as developmental delay or deficits. These team members can also provide counseling for adolescents and young adults who were born with cleft lip and palate, with respect to family planning and hereditary risks.
Speech and Language Pathology The importance of speech and language pathology in the care of children born with cleft lip/palate was recognized early, with this discipline included in treatment as early as 19322. Children with cleft lip/palate have a greatly increased risk of speech and language disorders. Contributory factors are extensive, including both disruption of articulatory components (ex: dental and skeletal malocclusion, palatal clefts/fistulae) and resonance (impaired velopharyngeal closure)26. Additionally, variations in phonation or voice (hoarsness, habitual unusual volume or pitch) are more commonly seen in this population, and an increased prevalence of hearing impairment may also contribute to language difficulties. Accurate identification of the cause of speech errors is critical for providing the appropriate corrective intervention, which can range from speech therapy to surgery. Additionally, some surgical interventions such as maxillary advancement may have adverse effects on speech27, and the assessment of such risks is an important component of informed consent.
Psychology and Social Services Individuals born with cleft lip/palate, as well as their families and caregivers, present with unique psychological needs related to adaptation to social response, treatment burden, and other challenges. Individuals with cleft lip and palate have been reported to suffer decreased self-confidence28,29, depression30, and decreased oral health-related quality of life31. Parents and caregivers may also suffer significant psychological distress32. Involvement of mental health specialists on a treatment team is critical for both identifying concerns, and for accessing the appropriate assistance and services to promote mental well-being in patients and their families.
Nursing As outlined above, the treatment of individuals with cleft lip/palate involves the coordinated involvement of multiple specialties over many years. For many treatment teams, the responsibility for coordinating this treatment is assumed by a nursing practitioner acting team coordinator3. This practitioner is often the pointperson for patients and their families: both initiating contact and providing continuity of care through repeated follow-up. Their frequent involvement and contact with patients and their families makes team nurses ideally situated to monitor growth and development, and to provide education and reinforcement on a wide range of topics such as feeding, anticipated treatment, and wound care3. Team nurses may also act as advocates for patients and their families, guiding them in obtaining appropriate services.
Advantages of an Interdisciplinary Team A key aspect of the interdisciplinary cleft treatment team is the integration and close working relationship of the specialists involved. Integrated case management through collaborative and often simultaneous assessment and planning with team members from multiple disciplines ensures efficient use of patient and caregiver time and resources. Even if a particular specialty is not present, through the use of centralized multi-disciplinary records, practitioners have ready access to information which would otherwise require referral and potentially significant waiting periods to obtain. Furthermore, the presence of multiple specialties during patient assessment and treatment planning meetings may further benefit the patient in coordinating the sequencing and timing of inter-dependent treatments such as orthodontic treatment and alveolar bone grafting. Similarly, input from multiple disciplines may help balance the benefits and risks of interventions; for example, the potentially detrimental effect of orthognathic surgery on speech resonance. Centralizing treatment for all patients in a large region at a treatment center is also of benefit in ensuring that the treating professionals maintain adequate experience and involvement over time. Despite being a relatively common birth anomaly, cleft lip and palate is rare enough that a single practitioner outside of a treatment center could only expect to encounter a few affected patients in their career. An extensive group of studies conducted in the United Kingdom comparing outcomes before and after centralization of cleft and craniofacial care support these benefits, with improvements noted in length of post-surgical hospital stay33, facial
appearance34, dentoalveolar relationships34, patient self-confidence35, and a range of speech outcomes36 after centralization of care. For these reasons, the ACPA recommends that all patients born with cleft lip and palate or other craniofacial anomalies receive care from a treatment team6.
Conclusion Providing care for children born with cleft lip and palate is a complex, challenging, and ultimately rewarding undertaking requiring the input of an extensive team of dedicated healthcare professionals. Through participation in an interdisciplinary team, practitioners help ensure the provision of quality care suited to the individual needs of their patients.
Table 1: Key contributions and responsibilities of team members by discipline Audiology
- neonatal hearing tests - follow-up examinations through adolescence - maintenance of hearing assistance devices
Genetics, Dysmorphology, and Genetic Counseling
- genetic diagnosis, determination of cleft etiology - recurrence risk and prognosis counseling
Nursing
- frequently act as team coordinator
- patient education and support - monitoring of growth and development
Orthodontics
- pre-surgical orthopedics, nasoalveolar molding - interceptive treatment including expansion and maxillary protraction - assessment of dental development for informing surgical timing - definitive orthodontic treatment +/- orthognathic surgery
Otolaryngology
- monitoring for development of middle ear disease - myringotomy, tympanotomy tubes - surgical intervention for velopharyngeal dysfunction
Pediatric Medicine
- initial systemic examination - monitoring of growth and development - treatment and re-assessment of associated medical conditions
Psychology and Social Work
- evaluation and treatment of emotional, learning, developmental, and adjustment disorders
- consideration provided for both well-being of patient and family members
Speech and Language Pathology
- evaluation of articulation, resonance, phonation/voice, language development - speech therapy and/or identification of other treatments indicated for the improvement of speech anomalies
Surgery (Plastic, Oral and Maxillofacial, Pediatrics) AND Anesthesiology
- surgical interventions including, but not limited to: - lip adhesion - primary repair surgeries including chelioplasty, nasoplasty, gingivoperiosteoplasty, palatoplasty - alveolar bone grafting - surgical intervention for velopharyngeal dysfunction - orthognathic surgery
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