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Signicance of oral health in persons with Down syndrome: a literature review

M Hennequin* HDR MCU-PH, Special Needs Dentistry; D Faulks BDS, Senior House Ofcer, Special Needs Dentistry; J-L Veyrune DCD PH, Special Needs Dentistry; P Bourdiol CECSMO MCU-PH, Orthodontics, Facult de Chirurgie Dentaire, Universit dAuvergne; Clermont Ferrand, France. *Correspondence to rst author at Facult de Chirurgie Dentaire, Universit dAuvergne 11 Boulevard Charles de Gaulle, 63000 Clermont Ferrand, France. E-mail: martine.hennequin@u-clermont1.fr

Down syndrome (DS) is an autosomal chromosomal anomaly resulting from trisomy of all or a critical part of chromosome 21. It affects approximately 1 in 700 to 800 live births. Despite the development of prenatal diagnosis, the incidence of DS births is predicted to remain static or even to increase over the next decade, partly due to increased maternal age in Western societies13. Many of the medical and physiological characteristics of DS have direct consequences for the oral health of subjects affected and indirect consequences for the quality of life of persons with DS and their carers. This article aims to give an overview of the current literature concerning the orofacial problems confronting patients with DS, and to explain the different treatment modalities available. Anatomical considerations The different anatomical aspects of DS have been well described4 and form the basis of the orofacial problems experienced by this population. The principal skeletal craniofacial features include brachycephaly with a attened occiput and decreased length and attening of the cranial base59. The facial mid-third is underdeveloped, producing a hypoplastic maxilla with a high, short, and narrow palate10. The frontal and paranasal sinuses11 are hypoplastic and the ethmoid bone is retracted7. Mandibular prognathism is mild or marked relative to the maxilla8. Soft-tissue features include a ssured and protrusive tongue that often rests between the dental arches and high against the palate12. The tongue appears macroglossic due to the relatively small size of the oral cavity (relative macroglossia13). The tonsils and adenoids are enlarged14. Dental anomalies noted include structure (taurodontia, decreased root to crown ratio, decreased tooth size, altered crown shape)15, number (hypodontia or partial anodontia, frequently 7s and 8s), eruption (delayed or abnormal

sequence of eruption)16,17, and position (protrusion of the lower incisors)18. There is often a pseudo Class-III malocclusion7 or maxillary endognathism with an anterior open bite due to abnormal tongue position19. Facial appearance is altered with short palpebral ssures, hypertelorism, a wide nasal root, a narrow soft nose with a high nasal tip, a high upper lip, and wide short low ears8. The muscles of mastication and facial expression are hypotonic14,20 and there may be laxity of the temporomandibular joint ligaments21. These classically described features vary signicantly between individuals despite the widely recognized typical facies of DS. Mastication and swallowing

The structural features discussed can be directly related to the chromosomal abnormality22. The functional manifestations of these abnormalities are indirectly related to the underlying pathology. Normal development of oral structure and function is altered19,23, leading in turn to compromised development of suckling, swallowing, mastication, and speech. Without intervention, drooling is a common consequence14,24,25. The degree of difculty experienced by each patient is variable but is primarily due to hypotonicity of the muscles of mastication and facial expression, particularly of the tongue and lips13,20. Normal development Lingual development is a reflection of the overall muscle tone of the child and the bony development of the facial mid-third is dependent on normal lingual function2628. During in utero development, the oral structures develop around the reflex suckleswallow movement of the fetus26,29, an influence that continues throughout the early

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life of the child. In the neonate, the tongue fills the oral cavity and lies between the developing arches and the lips. During early swallowing, there is active placement of the tongue between the upper and lower incisors and against the hard and soft palate30. The lips and perioral musculature actively form the anterior seal and forwardbackward movement of the tongue results in a swallow. In the young child, therefore, a mild anterior open bite and a tendency to tongue protrusion or thrust form part of the normal evolution of an adult swallow31. The development and form of the maxilla is dependent on pressure applied by the muscles of the tongue. The close relation between oral structure and muscle tone (tongue function and lip tonicity) has been demonstrated by functional neuromuscular therapy27,32,33. In the adult and in children over the age of approximately 4 years, the tongue forms a seal against the teeth and alveolus and depresses centrally allowing the bolus to be projected towards the oesophagus (vertical movement). Lateral tongue movements commence with the development of the primary dentition at 6 to 12 months30 and increase to 4 years of age. Development in Down syndrome In DS lingual hypotonicity leads to the marked underdevelopment of the facial mid-third10. There is marked discrepancy between the alveolar arcades and thus the primary incisors erupt into a position without a comfortable resting occlusion. The child tries to avoid this discomfort by advancing the mandible. Mandibular protrusion is facilitated in a subject with DS due to the laxity of the temporomandibular joint ligaments. One or more new resting positions may become established, effectively trapping the maxilla behind the mandible. A clenching or grinding habit may develop in an attempt to nd a position of comfort and subconsciously

to eliminate occlusal interferences. In the absence of treatment, maxillary growth is thus further retarded. The underdeveloped maxilla, combined with an enlarged tonsillar volume, results in congestion of the upper airways and induces a tendency for mouth breathing3436 and may lead to sleep apnoea14,30,37. The mandible is lowered, the lips are parted and the tongue assumes an anterior position over the lower teeth to allow free passage of air38 (Fig. 1). Swallowing is compromised39 as an anterior seal and thus depression of the tongue is difcult to achieve making bolus transit inefcient. This is added to the preexistent problem of poor intrinsic tongue tonicity. Children with DS are therefore obliged to displace actively the bolus backward by a primitive forwardbackward movement of the tongue against the palate, and tongue thrusting is encouraged. The consequences of this abnormal function may be the development of a high narrow palate and an elongated tongue due to the preferential development of the longitudinal muscle bres. The tongue is inefcient in lateralizing food for chewing particularly in the molar region38,40. Mastication is impaired because of poor oral exploration and an inability to initiate, grade, or sustain oral patterns. Movement is arrhythmic with incoordination of the lips and mouth and excessive vertical opening30,41. This may lead to drooling and potential swallowing incoordination. In addition, the presence of an endognathic maxilla, an anterior open bite, and a reduced number of teeth may further decrease masticatory capacity. Muscular hypotonicity in the cervical region adds to the problem of swallowing as the neck is extended at rest29 (Fig. 2). Approximately 15% of children with DS have atlantoaxial instability and this should be assessed radiographically before treatment under general anaesthetic with endotracheal intubation42.

Control subject

Subject with Down syndrome

Figure 1: Comparison of the position of oral structures at rest in a control subject and a subject with Down syndrome. Note that the mandible is lowered, the lips are parted, and the tongue assumes an anterior position over the lower teeth to allow free passage of air.

Many studies address the different feeding problems of subjects with DS as reported by their carers4347. These include problems with breast- and bottle-feeding; slow development of the ability to manipulate food in the ngers and in the use of feeding utensils; the failure to progress through a normal sequence of food textures and the refusal of certain foods, particularly those of a hard texture; behavioural problems such as the refusal to swallow, the spitting out of food, or the retention of food or utensils in the mouth. Related difculties are identied on clinical examination: a poor or delayed suckling ability, problems with mastication, drooling, a tendency to allow the mouth to hang open at rest, a protruded tongue posture persisting beyond the age of 2 years, a delay in dental eruption, and the presence of a malocclusion48. Several longitudinal studies have shown that children with DS take longer to develop the motor coordination necessary for normal feeding44,45,47. Feeding difculties expressed by carers are often over and above those associated with the degree of intellectual delay47. Progress is further impeded if the child is particularly hypotonic, has a serious cardiac malformation, is markedly immature, or if the parents are not sufciently motivated to follow specic educational programmes45. Nutritional problems may equally be related to intercurrent illness and thereby the affected childparent relationship47.


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Swallowing Swallow function in children with DS has been assessed using videofluoroscopy in conjunction with foods of different texture49. Silent aspiration was shown to be a problem among this population with liquid and semiliquid food reaching the bronchi. On a daily basis any aspiration can be assumed to contribute to the incidence of pulmonary infection. Problems with swallowing have been described as being due to slow development of motor skills in the child45,50. However, such difficulties often persist into adulthood and may have grave consequences for general health. Mastication Masticatory function was examined in a group of adolescents with DS and a group of similar age without DS (controls) eating 10 foods of different texture40. It was clear that certain types of oral manipulation, such as biting into a whole apple, were very difcult, if not impossible, for the group with DS. This resulted in the refusal of certain foods, non-functional masticatory cycles, and the swallowing of large, poorly chewed morsels. Such behaviour led directly to belching (corresponding to the swallowing of air), coughing (corresponding to the aspiration of food), sighing, and in copious drinking between mouthfuls. Video recordings of 4- to 5-year-old children with DS eating a standardized meal show that tongue protrusion in this population is marked38,39. Food is held longer in the mouth than in control subjects, whether solid, semisolid, or pure. This is partly because there is a delay47 in the initiation of oral movement in subjects with DS and partly due to pauses within the masticatory cycle. This may be due to a reluctance to chew and more sucking of the food38. Abnormal chewing cycles may be induced by the need to nd a dental contact despite a marked malocclusion. This may involve a degree of mandibular protrusion which, combined with hyperlaxity of the temporomandibular joint ligaments 21, can result in chronic luxation of the joint. It is suggested that decreased masticatory efciency entails a loss of sensory input and may decrease the pleasurable aspects of eating. Patients who swallow their food whole may tend to overeat in order to compensate for the loss of gustatory input. It is possible that this may be one of the factors affecting the development of bulimia and obesity in subjects with DS.

discipline of frequent exercises or with appliance therapy, others cope very well. The best results have followed very early intervention (between 6 months and 4 years of age). Improvements have been noted in the tongue position at rest, the position and strength of the lips, mouth closure, suckling, swallowing, drooling, and feeding20,21,31,54. This may be explained by the concept of a critical period for the introduction of certain stimuli after which time a particular pattern of behaviour becomes increasingly difcult to learn30. Methods of behaviour modication include parental reinforcement of the desired behaviour11, and the use of tactile or audio cueing aids30,55. Orofacial discrepancy can be corrected during puberty by functional orthodontic therapy. These techniques are highly dependent on the full cooperation of the child and highlight the need for early familiarization of the child with DS to the dental environment56. Regular visits during childhood are important in order to be able to intervene as soon as the primary dentition is in place and/or as the denitive dentition becomes established. Glossectomy, tonsillectomy, and plastic surgery are widely practised5761 in the United States but serious evaluation has yet to be undertaken62. Some of these procedures raise serious ethical questions6365 and are now contraindicated, particularly glossectomy, as the clinical impression of macroglossia is largely due to tongue position rather than to a true hyperplasia. In one study, only ve patients of 400 were found to have true macroglossia13. Function is rarely

2 5

The different methods of therapy are highly contested. They include neuromuscular stimulation, behaviour modication, orthodontic and surgical intervention. Orofacial therapy by neuromuscular stimulation is of particular interest. These techniques were introduced approximately 20 years ago5153 and consist of physiotherapy of the oral structures and appliance therapy to stimulate the lips and tongue. The main goal is to increase muscle tonicity and therefore improve the development of oral morphology and function. This should establish a rest position of the tongue behind the incisors and correct the open mouth habit31 eliminating drooling and improving swallowing and mastication. Evaluation of these techniques is only possible by clinical observation and is therefore relatively subjective. The results are variable; while some children are unable to cope with the

Control subject

Subject with Down syndrome

Figure 2: Effect of dorsal and cervical muscle weakness on posture and on the position of oral structures. A lordosis (1) and the tendency towards atlantoaxial subluxation (2) incline the head backwards (3) such that the tongue blocks the oropharynx. In order to maintain a clear airway the tongue protrudes (4) and the mouth hangs open (5).

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improved following surgery66 but language development and psychological stability may be compromised14,67. Dental disease

Independent of the particular orofacial problems of DS, these patients are also naturally prone to the same degree of dental disease as the rest of the population. Unfortunately these problems are aggravated by systemic weakness such as immunological deciency. In one study, 24% of subjects with DS had suffered an ear or throat infection in the previous year68. There is a particularly high frequency of upper-respiratory-tract infection, ear infection, pneumonia, bronchitis14,49, and leukaemia69. The oral tissues have been particularly studied, as there appears to be increased colonization by certain pathological bacteria70 and differences in the specic and non-specic host mechanisms leading to more extensive gingival inammation71,72 than would be expected for the degree of plaque present73. Imbrication, poor motor control, and lack of self-cleansing by the weak tongue and lips leads to the accumulation of food debris in the mouth. Mouth breathing reduces the protective function of saliva on the tooth surfaces. The result is a predisposition to dental disease, which may be systemically serious if the patient is at risk of endocarditis69,74.

Periodontal disease Periodontal disease may be dened as a disease of the supporting tissues of the teeth involving early degeneration of bony support associated with chronic gingival inammation and localized areas of infection. Its basis in patients with DS is largely due to immunological deciency75 and its evolution to poor masticatory function, poor oral hygiene, and dental imbrication. Patients with DS (children71,73 and adults76,77) are prone to a particularly aggressive form of periodontal disease which is similar to the juvenile periodontitis seen in certain adolescents without DS78. This may be due to the bacterium Actinobacillus actinomycetemcomitans that is implicated in juvenile periodontitis. An increased frequency of this bacterium has been found in the subgingival plaque of patients with DS (35%) when compared with control subjects (5%)70. Periodontal problems were found in 94% of subjects aged 16 to 20 years79. The progression of this disease involves periods of acute infection, inammation, and pain which may result in behavioural changes including either the absolute refusal of food or the tendency to swallow food whole. The disease follows a chronic evolution marked by phases of acute inammation that leads eventually to the spontaneous loss of teeth. Caries Various studies have shown that caries incidence is reduced in children and young adults with DS (84% caries free80) in comparison with healthy subjects or controls with mental retardation8082, although differences may reduce in the permanent dentition83. An extensive search of the literature failed to reveal caries data for the adult population over 30 years of age. These ndings may be explained by the fact that many children with DS are under the supervision of a dietician in order to prevent their tendency to obesity, and therefore do not have a highly cariogenic diet44,69. They are also

prone to hypodontia and therefore comparisons of caries incidence with control populations may be skewed73. Nursing caries still presents a problem in this group. Nursing or bottle caries is a phenomenon due to the constant availability to the child of a bottle lled with milk, squash, or fruit juice. Rapid destruction of the developing teeth ensues due to the action of oral bacteria on the abundant supply of carbohydrate. The problem is aggravated as it concerns children under 4 years old who are too young to be treated in the normal dental setting. Although in general children with DS have a reduced caries incidence, behavioural difculties and/or feeding problems may result in the constant use of the bottle as parents try to compensate for other restrictions placed on their children47. Daily intake in adults with DS is less well controlled than in children due to the need to cater for large groups and due to the fact that the subject makes a more autonomous choice. Any food debris left in the mouth following a meal is an excellent substrate for cariogenic bacteria. Examination after meals reveals that food remnants persist longer in the mouth in subjects with DS than in control children. Food debris tends to collect due to neuromotor incapacity and reduced oral sensitivity that limits the self-cleansing action of the oral structures. The problem is aggravated by the mobility of periodontally involved teeth that allows food to be trapped interdentally. It follows that a bacterial ora develops more easily in adult subjects and this produces the acids responsible for dental demineralization. These carious lesions develop slowly from the age of 18 years approximately and can often only be detected on meticulous examination. They only start to cause symptoms from the age of 25 to 30 years.

The basic treatment approach should be the same for patients with DS as for the general population with a clear emphasis on prevention84,85. Modications need only occur when behavioural difculties prevent the patient from receiving the optimum treatment. It is clear from the literature that many patients with DS do not receive an equivalent level of dental healthcare as the general population86. In one study of 25 subjects it was shown that children with DS tended to receive less aid with brushing, less uoride supplementation, and fewer visits to the dentist under the age of 5 than control children87(60% versus 84%, 0% versus 24%,12% versus 40%, respectively). They are also weaned at a later stage than children without DS (56% versus 24% after 18 months) and are more likely to have prolonged access to the bottle than their peers. Most parents of children with DS felt that they would benet from preventive advice (72%)87. Even within institutions, oral healthcare has been shown to be the most frequently neglected aspect of overall care84. Risks associated with infection The risk to systemic health of a dental infection is increased in children with DS, particularly those with concurrent medical problems. The incidence of congenital cardiac disease is increased in this group8890 and reaches 30 to 40%. Thus, any dental infection (periodontal or periapical) carries with it a serious risk of endocarditis91,92. Of 41 patients with DS diagnosed with a prolapsed mitral valve, 15 (36%) were normal to auscultation74. It is of utmost importance that any dental intervention where there is risk of bleeding (e.g.


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extractions, scaling, root treatments) be systematically undertaken under appropriate antibiotic cover unless there is conclusive evidence of normal cardiac function following an ECG examination93. For those unable to cope with dental treatment, a full dental clearance may be indicated in the presence of severe periodontal disease and a cardiac abnormality. Behavioural problems Personality disorders may lead to a refusal of treatment on the part of the patient. The quality of the dentistpatient relationship is paramount, particularly for the very young or patients with DS. It is impossible to undertake any form of treatment without the establishment of a trusting relationship. These patients are capable of accepting a degree of discomfort provided that the interpersonal relationship is not betrayed. The patients entourage and the behaviour management skills of the dentist are the key factors in the childs acceptance of dental treatment. It is also essential that any intervention must be pain free. It may be difcult for patients with DS to express pain experienced and their discomfort may therefore be overlooked by the clinician. Refusal to cooperate with treatment and introversion may result. Local anaesthetic should be used systematically for any treatment where there is the slightest possibility of pain, including scaling and placement of orthodontic bands. Gag reex Patients with DS often have a marked gag reex which can be stimulated even in the anterior portion of the oral cavity. This is partly due to the anterior positioning of the tongue and to the lack of intraoral stimulation23 and partly due to anxiety. Any dental examination that passes the limit of the premolar region can cause a gag reex that may be accompanied by gastrooesophageal reux. This reex is naturally uncomfortable for the child and results in a refusal to cooperate with further treatment. This oral hypersensitivity may also be a cause of the aversion to certain textures of food49. The gag reex may often be reduced if the discomfort is quickly recognized, if the patientdentist relationship is good, and by the use of facial and intraoral massage. Balance Patients with DS are often wary of the dental chair as their reduced natural balance makes it difcult for them to adjust to the movement of the chair. The patient should always be forewarned of any adjustment to chair position. Other dental problems

state of chronic anxiety, dental malocclusion, temporomandibular joint dysfunction due to laxity of the supporting ligaments, and underdevelopment of nervous control, hence the increased frequency of bruxism in subjects with DS. In severe cases, bruxism may lead to gradual but premature tooth wear. The patient with DS should be on a regular review programme to allow problems associated with bruxism to be identied early. When correction of the underlying morphological problem is not undertaken or is not possible as a child then the placement of an equilibrated bite raising appliance may help to reposition the mandible and reduce grinding. Unfortunately, for those patients whose bruxism is due to an underlying lack of nervous control even appliance therapy is not successful.

Adults with DS may be more prone to falls than the average adult patient due to their lack of motor development50. Fracture or luxation of the anterior teeth is frequent and often involves loss of tooth vitality. In children, however, the relative difference in motor coordination is less marked95 and there appears to be little difference between dental trauma in DS and control child populations. Discussion and interaction between factors As demonstrated in Figure 3, the different dental pathologies and functional difculties suffered by patients with DS interact to increase the rate of evolution of these problems. The complexity of this table merits a full explanation. Chronic gingivitis becomes established early in the child due to mouth breathing, inadequate oral stimulation and lack of hygiene. It remains undetected as the primary dentition is shed but evolves throughout adolescence until serious periodontal disease becomes apparent at around 20 to 25 years of age. With the establishment of severe periodontal disease, tooth mobility becomes a problem and the periodontium is poorly maintained due to a lack of masticatory stimulation. Food trapping is favoured by tooth mobility and malposition and by reduced tongue activity. Many young adults learn to compensate for their reduced masticatory efciency by adopting a particular muscular posture and by the choice of soft foods that are often rich in fermentable carbohydrates. Food debris from such diets favours a cariogenic oral ora and eventually results in dental decay. Despite a reduced caries experience overall, early carious lesions are more likely to evolve in a patient with DS over time than in a control subject because of their cognitive difculties in dealing with dental treatment, their difculty in recognizing and expressing pain, and their reduced access to dental care. It is more difcult to obtain full cooperation for treatment from a mentally compromised patient than from a control subject9698. This is particularly true in the dental setting, which provokes particular anxiety in much of the general population. Thus the primary factor in the prevention of dental disease in the patient with DS is their capacity to develop a trusting relationship with the clinician, which in itself is dependent on the patients intellectual ability and psychosocial integration99,100, and on the behaviour-management skills of the clinician. It is for this reason that some authors feel that a specialist care service is essential for these patients101.

Bruxism can be dened as the stereotypical orofacial movements associated with diurnal or nocturnal grinding or clenching of teeth. This phenomenon may be present in those without DS as well as those with DS and in adults or children. It does not usually require treatment in healthy subjects except in the most severe cases as this behaviour is essentially non-pathological. There have been many theories as to the aetiology of this problem, amongst which the anxiety state of the patient, dental malocclusion, the autonomic nervous system responsible for oral posture, and the action of certain neurotransmitters94 have been implicated. Current thinking is that the aetiology is of multifactorial origin. Patients with DS are affected by all of the above factors a

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Reduced sensory discrimination has been described in the literature but is rarely severe. A study was undertaken to compare the pain threshold and ability to locate a cold stimulus between 20 young patients with DS and a control group of similar age102. The group with DS showed a higher pain threshold and marked difficulty in localization of the stimulus. Errors in discrimination may be due to retarded development of the psychomotor system leading to a distorted body image50,103. An inability to localize or express discomfort could explain the delay in seeking treatment that is frequently observed in these patients. The differentiation of dental pathology is often based on reported pain characteristics and therefore accurate diagnosis is difficult in subjects with DS. It follows that dental disease often remains undetected until it reaches such a stage that it is irreversible and presents a serious risk of infection. Treatment available also depends on the degree of cooperation of the patient. If cooperation is good then high-quality conservative dentistry is possible in the normal dental setting. If cooperation is impossible then treatment under sedation or general anaesthetic is the only solution. These conditions are frequently incompatible with complex restorative dentistry and extractions gure more heavily in treatment planning104, resulting in an overall loss of function and compromised aesthetics. Other barriers to care for this population are related to health-service access. Oral health-care services are not avail-

able to all due to nancial, geographical, or social barriers. These problems are amplied for the physically and mentally compromised105107. Specialist services may be rare and are dependent on the public health-service policies of each country or region. A study of health service access was undertaken in France where no specic special-care services exist. Parents reported having more difficulty finding both medical and dental care for their child with DS than for a sibling without DS108. Conclusion It has been shown that persons with DS are particularly prone to orofacial disorders. Systemic dysfunction in this population may predispose to oral disease and oral disease may in turn aggravate systemic disease. The particular orofacial problems of subjects with DS should be taken into account by the patients immediate entourage and by the medical team involved. Regular reviews, preferably by a specialist team, should be undertaken to identify and prevent both functional problems and disease processes. These patients should be seen at least every 6 months, but more regularly should specic problems arise. These regular reviews should have the following aims: early introduction to the dental environment in order to facilitate subsequent preventive intervention, diagnosis and, if possible, to allow appliance therapy; early neuromuscular stimulation of oral proprioception essential for full development of orofacial function in the infant; education of carers with regard to nutrition, taking into account

Immunological deciency

Orofacial problems associated with Down syndrome Dental anomalies Hypotonicity Skeletal Malposition Lingual maldevelopment Hypodontia Buccal Labial

Neurosensory underdevelopment

Psychomotor underdevelopment

Respiration Mastication Alteration of oral function Speech swallow

Weakened immune defences

Stagnation of food debris

Understimulated periodontium

Reduced sensory discrimination

Poor oral hygiene


Caries in the long term


Pain Infection

Compromised treatment

Figure 3: Interaction between orofacial and systemic manifestations of Down syndrome in the development of dental disease and compromised oral function. For full explanation, see text.


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masticatory function, food texture, caries prevention, and uoride administration; and institution of a personalized oralhygiene regime for the prevention of periodontal disease, taking into account the motor capacity of the patient and aiming for autonomy of care.
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