Clinical Scenario

A 15‐year‐old patient is referred by the Ear, Nose and Throat (ENT) specialist for an orthodontic assessment. There is concern that the patient’s palatal morphology may be responsible for his chronic nasal respiratory distress (Figure 1.3.1).

Structured Learning

1. What factors are considered important in assessing the risk of managing this patient?
   • Social
     • Transport arrangements to attend dental appointments
     • Excessive protectiveness by his guardians (the grandparents have already lost their daughter to the disease)
   • Medical
     • Myotonic dystrophy type 1‐associated myotonia and multiorgan damage; muscle weakness, sleep disorders
     • Diagnosis confirmed at birth is generally associated with a poor prognosis
     • Risk resulting from cardiomyopathy and respiratory impairment
     • Position in the dental chair compromised by the kyphoscoliosis
       

       

       

   • Dental
     • Risk of aspiration during the dental procedure
     • Unsupervised oral hygiene habits
     • Follow‐up difficulties due to social situation

2. The patient is taking prednisone at a dose of 20 mg/day. What are the implications of this when performing an invasive dental procedure?
   • Delayed healing
     

     

   • Increased risk of significant infection
   • Adrenal crisis may be triggered by surgical interventions due to chronic hypothalamic–pituitary–adrenal axis suppression – a corticosteroid supplementation protocol is required to prevent this (see Chapter 12.1)
3. What precautions should be undertaken in view of the patient’s history of hypertrophic cardiomyopathy?
   • Limit the administration of anaesthetics containing adrenaline and avoid intravascular injections
   • Some cardiologists may disagree with international consensus guidelines, and hence recommend that these patients should receive antibiotic prophylaxis to prevent bacterial endocarditis if an invasive dental procedure is planned
   • A number of drugs, such as atenolol, can cause orthostatic hypotension
4. The patient’s grandparents are insistent that an orthodontic appliance should be fitted as they feel that the palate is becoming more arched. What requirements should the patient satisfy before proceeding with orthodontic treatment?
   • The oral hygiene should be extremely good
   • The dentition should be stabilised, including restoration of #36 and #46
   • The patient should be able to accept and tolerate both the intraoral and extraoral appliances
   • Close liaison with the medical team is required in order to evaluate the risk/benefit of proceeding, taking into account the progression of the muscular dystrophy
5. What type of appliances are recommended?
   • In general, functional orthodontic devices are not recommended because the biological muscle forces are usually impaired
   • Devices that may be prescribed for this patient include a palatal expander, multibracket appliances (for tooth alignment), a transpalatal bar with a stimulator/lingual pearl and/or a tongue guard and extraoral appliances such as a vertical traction chinguard
   • However, due to the age of this patient, they may not be effective
6. What is the prognosis of the orthodontic treatment?
   • The prognosis for the open bite and vertical mandibular growth is poor
   • The defect cannot be corrected in many patients, and the recurrence rate is high
7. Why are prevention and periodic follow‐up especially important for this patient?
   • The patient will have increasing difficulty performing mechanical oral hygiene techniques
   • Physiological mouth cleaning will worsen with time
   • The bacterial load of the oral cavity can promote respiratory infections
   • Managing the patient in the dental clinic will become increasingly complex

General Dental Considerations