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Special Dentistry Issues
for Children with Developmental Disabilities

Children with special health care needs and/or developmental disabilities often present a substantial challenge to the dental practitioner. The dentist may frequently encounter difficulties providing treatment to this population due to physiological abnormalities, which predispose the child to poor oral and dental health, and/or communication/behavioral issues which may make the patient's compliance with dental procedures difficult to obtain. Additionally, many dentists, including many pediatric dentists, have not had adequate preservice training to prepare them for the specific issues often encountered when administering dental treatment to children with special needs and/or cognitive/behavioral concerns. Adequate reimbursement may also present a problem due to Medicare regulations. Often dental practitioners avoid accepting such individuals as patients altogether.

Advances in medicine in the last few decades have resulted in many more individuals with disabilities living a "normal" lifespan. Children who would not have survived in the past are now living in the community, going to school, and becoming employed. Progressive disability rights legislation has also increased standards of care, improved attitudes, and deinstitutionalized most individuals with disabilities. Unfortunately, children with special needs are still among the most neglected groups when it comes to receiving necessary dental care. According to an article published recently in the Journal of Dental Education, one organization attempting to provide a database of dental providers willing to accept patients with developmental disabilities found that 26 states had no providers at all who wished to be listed.

There are several medical issues/conditions associated with developmental disabilities which may result in difficulty for the practitioner attempting to provide dental care. Some of the most common are discussed below.

Musculoskeletal Abnormalities

Alterations in muscle tone may be associated with some disabilities such as Down syndrome or cerebral palsy. In Down syndrome, decreased muscle tone (hypotonia) is typically present. This decrease in muscle tone alters orofacial development. The open bite frequently seen in individuals with Down syndrome, as well as problems with mastication, may be attributed to decreased orofacial muscle tone. An oral appliance may be used occasionally in hopes of stimulating development of the oral morphology. Conversely, individuals with cerebral palsy typically experience hypertonia/spasticity of the muscles. This increased muscle tone may be associated with significant oral motor dysfunction. Additionally, neurological abnormalities in cerebral palsy may result in the presence of tonic bite reflex and/or tongue thrust, which may make provision of oral interventions very challenging indeed. Alterations in orofacial skeletal morphology, such as midface hypoplasia, overbite, deep or narrow palate, cleft lip or palate, macroglossia, small oral cavity, microdontia, and/or oligodontia may be present in some children with special needs. The presence of Down syndrome may result in the frontal and maxillary sinuses being reduced in size, or altogether absent.

Problems with mobility may be present in children with some types of developmental disabilities, such as muscular dystrophy and cerebral palsy. It may not be feasible in some instances to move the child into the dental chair; thus the practitioner must exercise reasonable flexibility when attempting to deliver care to such individuals.

Alterations in Dentition

Children with developmental disabilities frequently demonstrate abnormalities in dentition. Delays may occur, such as in Down syndrome, in both the time and sequence of eruption of both primary and secondary teeth. There may also be an abnormal number of teeth, and/or the teeth themselves may be malformed. Enlargement of the pulp of the tooth with surface thinning (taurodontism) may be present in some genetic disorders, including Down syndrome and Klinefelter's syndrome, and results in "blocky" shaped teeth. Some conditions may result in severe crowding of teeth, necessitating selective extraction. These changes in dentition frequently make optimal dental hygiene difficult to maintain.

Decay/Peridontal Disease

Persons with developmental disabilities are often at risk for dental decay and/or periodontal disease. This risk may be due to a variety of factors, particularly the physical and/or cognitive ability of the individual to perform adequate oral hygiene. A lowered host immune response may be experienced by those with certain types of disability, such as Down syndrome, and may further contribute to the development of periodontal disease. Occlusal sealants, fluoride treatments, and/or chlorhexedine mouth rinses may be indicated as preventative measures. Children with cerebral palsy frequently experience chronic gastroesophageal reflux, which may result in significant erosion of tooth enamel without intervention. They may also experience gingival hyperplasia (overgrowth of the gums) as a side effect of some medications. Poor oral health may place children at risk for chronic pulmonary infections, as well as endocarditis in those with cardiac defects. Individuals at risk should be seen more frequently for dental care than the normal population.

Bruxism

Bruxism refers to grinding of the teeth. This may occur in the daytime, as a type of self-stimulatory behavior, or at night, during sleeping hours. Chronic bruxism may result in severe wear of the teeth. There is no singular cause of bruxism. Factors such as severe anxiety, malocclusion of dentition, temporomandibular joint dysfunction, poor nervous control, or self-stimulation may be involved. Bruxism is common among individuals with Down syndrome and cerebral palsy.

Ligamentous Laxity (Atlantoaxial Instability)

Children with Down syndrome occasionally experience laxity in the ligaments surrounding the first and second vertebrae (C-1 and C-2, or "atlas and axis"). This laxity may result in instability of this joint which may place the child at risk for incurring cervical cord trauma. Children with this condition typically are cautioned against activities (e.g., contact sports, diving, trampoline exercise) which may increase the likelihood of such injury occurring. The dental practitioner should be aware of the possibility of this condition existing among Down syndrome patients and take necessary precautions when positioning the head, such as providing adequate support and alignment.

Drooling (Sialorrhea)

Drooling is a physical disorder which frequently has social implications for individuals with disabilities such as cerebral palsy and Down syndrome. Any disability which results in malocclusion of dentition, oral-motor dysfunction, hypotonicity, and/or dysphagia may result in excessive drooling. This may be due to an inability to close the lips with these conditions and/ or inability to swallow excess salivary secretions. Drooling may make daily oral care difficult and often results in oral infections and bad mouth odor, as well as macerated skin surrounding the lips. Drooling is very negatively perceived socially and thus may negatively affect the child's self-esteem.

Oral-Motor Dysfunction

Oral motor dysfunction refers to problems encountered involving the functional control of the muscles of mastication (chewing, bolus formation), swallowing, and speaking. Hypotonicity, present in some developmental disabilities such as Down syndrome, or hypertonicity, often demonstrated in children with cerebral palsy, may each result in abnormal oral motor function. Additionally, some children may experience primitive reflexes, such as tonic bite and tongue thrust, which make provision of oral care challenging. In children with severe symptoms, sedated dentistry should be considered after all other options are exhausted.

Involuntary Body Movements

Children with cerebral palsy, as well as some other types of neuromotor syndromes, often experience uncontrolled body movements, or even may experience seizure activity. It is important for the dental practitioner to understand the nature of involuntary movements when present. Attempts to thwart such movements may actually increase the patient's involuntary response. When involuntary movements are a problem, the provider may simply have to observe their pattern, in order to "anticipate" them - then proceed to blend his or her own movements with the patient's in order to provide care.

Primitive reflexes may be present as well in children with cerebral palsy and may present significant difficulty when providing oral care. Such reflexes include the asymmetric tonic neck reflex, tonic labyrinthine reflex, and the startle reflex. Tonic bite reflex and/or tongue thrust may also be present. Each of these reflexes is important for the dental practitioner to understand - as they each may easily be elicited through the course of "typical" dental care. Those providing such care must take precautions to prevent reflex stimulation as much as possible. Maintaining a calm, quiet, working environment which is free of unnecessary distractions is helpful, as is using a slow, gentle but firm approach.

Dental practitioners should be alert to the possibility of a seizure occurring when working with children having neuromotor disabilities such as cerebral palsy. The dentist should be prepared to manage a seizure should it occur, being careful to protect the teeth and oral cavity from injury. DO NOT attempt to place anything between the teeth during seizure activity. Care should be taken to manage airway patency and prevent aspiration.

Communication Difficulties

Children with developmental disabilities affecting the oral-motor complex, auditory system, and/or cognitive development may experience difficulties communicating effectively. It is important for healthcare providers to realize that even severe speech pathology may not be indicative of cognitive ability, as disorders such as Down syndrome and cerebral palsy may result in articulation difficulties which are often purely mechanical in origin. Receptive language skills typically far exceed expressive ability in such individuals. Involuntary muscle movements of the mouth and tongue in individuals with cerebral palsy (such as tongue thrust) may make speech sounds unintelligible to the dental practitioner. Additionally, some children may experience hearing deficits or deafness which also may make communication difficult.

It is no surprise that children with Down syndrome often experience difficulty with communication, articulation, and linguistic development. Both auditory and oral structural anomalies contribute to this phenomenon. Hearing loss due to inner ear involvement is common, with 60-80% of children with Down syndrome affected. It is impossible for the child to correctly reproduce sounds which are heard poorly - or not at all. Articulation is commonly altered in persons with Down syndrome and is related to structural alterations of the tongue, palate, and facial muscles. Defects in dentition may also be present, such as malocclusion of teeth and/or missing teeth, which further contribute to articulation difficulties.

It is important for the dentist to evaluate both the communication level and techniques for each child in their care. Children with speech pathology may employ various methods of communication, such as formal or informal sign language, alphabet boards, picture/symbol boards, or augmentative communication devices. The dentist should strive to build rapport with children experiencing communication difficulties by taking the time to understand their specific communication needs, rather than simply communicating through a third party such as a parent.

Cognitive Disability

Children with developmental disabilities possess a wide range of intellectual and adaptive functioning. Some individuals may have a disability which in no way affects cognition. Alternatively, another type of disability may result in cognitive deficits, which may range from very mild to severe. It is important for all healthcare providers to understand that even severe speech pathology may not be indicative of cognitive ability. Many structural abnormalities of the oral-motor complex may result in poor speech production. These particularly may be experienced by individuals with disabilities such as cerebral palsy and Down syndrome.

When working with all children, it may be useful to employ demonstration of procedures beforehand. This may be a particularly important tool for those patients who experience cognitive deficits. Although the parent may be able to offer helpful advice about the patient's level of understanding, so that communication may be adjusted accordingly, it is important to converse directly with the patient as well. The dental provider may need to speak slowly, using concrete terms. Additionally, the dentist should be as consistent as possible in the delivery of dental care for such individuals, using the same staff and operatory whenever possible. Familiar surroundings will help to build trust and are likely to improve patient cooperation.

References

Above materials summarized in part from:

Clinical guideline on management of persons with special health care needs. Adopted 2004 by the Council on Clinical Affairs, American Academy of Pediatric Dentistry. Reference Manual, 2004-2005.

Oral Health Care for People with Special Needs: Guidelines for Comprehensive Care. Retrieved September 27, 2005 from http://www.nidcr.nih.gov/HealthInformation/DiseasesAndConditions/
DevelopmentalDisabilitiesAndOralHealth/ContinuingEducation.htm

Practical Oral Care for People with Developmental Disabilities, pp 1-7, posted on the National Institute of Dental and Craniofacial Research informational website @ http://www.nidcr.nih.gov/HealthInformation/DiseasesAndConditions/
DevelopmentalDisabilitiesAndOralHealth/ContinuingEducation.htm
on February 8th, 2005

Additional References

Casamassimo, P.S., Seale, N.S., & Ruehs, K. (2004). General dentists' perceptions of educational and treatment issues affecting access to care for children with special healthcare needs. Journal of Dental Education, 68 (1), 23-28.

Chapman, R.S. (1997). Language development. In S.M. Pueschel & M. Sustrova (Eds.), Adolescents with Down syndrome: Toward a more fulfilling life (chap.10). Baltimore, MD: Paul Brookes Publishing.

Dykens, E.M., Hodapp, R.M., & Finucane, B.M. (2000). Genetics and Mental Retardation Syndromes (pp. 67-71). Baltimore, MD: Paul Brookes Publishing.

Fenton, S.J., Hood, H., Holder, M., May, P.B., & Mouradian, W.E. (2003). The American Academy of Developmental Medicine and Dentistry: Eliminating health disparities for individuals with mental retardation and other developmental disabilities. Journal of Dental Education, 67 (12), 1337-1344.