Duchenne influencedorofacial muscles(facial and oral muscles) and affects orofacial function (chewing, swallowing, etc.). There is a high prevalence ofMalocclusion(the wrong ratio between the teeth of the upper and lower arches when they approach with the jaw closed). Greater expansion of the lower arch compared to the upper arch can result in open bites (upper and lower teeth not touching as the teeth/jaws close) and crossbites (upper and lower teeth separating). incorrectly and ineffectively contacted). This leads to a significantly weak bite force. With age, weakness of the orofacial muscles can impair orofacial function and lead to difficulty chewing and swallowing.
changes in structure
The orofacial skeleton is the structure of the face and mouth, including the position of the teeth and the shape of the dental arches. Habits such as thumb-sucking and mouth-opening breathing, as well as the strength and function of the orofacial muscles play a role in the development (shape) of the orofacial skeleton.
changes in the tongue
In Duchenne, the tongue can enlarge with age while tongue strength and function decrease. As the tongue grows and weakens, it can have trouble maintaining the movement and pressure needed to chew, swallow, and speak. An enlarged tongue can also contribute to a worsening of the misaligned teeth, change the appearance of the face, and cause more difficulty chewing. Keeping the tongue in and avoiding "mouth breathing" can help minimize this change.
changes in function
As we age, the tongue muscle, the muscles in and around the mouth, and the muscles used to swallow become progressively weaker, making eating slower and more difficult.
Changes in chewing and swallowing
If you are diagnosed with misaligned teeth, you may have difficulty chewing your food completely, making it difficult to swallow. As a result of inefficient chewing combined with weakened swallowing muscles, there can be a build-up of unswallowed "waste" in the throat. This accumulation causes a feeling of choking or food getting stuck in the throat. These debris, which settle in the throat, can be aspirated into the lungs, increasing the risk of aspiration pneumonia. Minimizing solid foods during meals (weight loss foods or after meals with at least 3 drinks of water) can help reduce the amount of waste remaining in the throat.
The progressive weakening of the chewing muscles can be slowed down by movement. Stimulating exercise like chewing sugar-free gum can help keep the muscles needed to chew stronger.
procedures for swallowing
called difficulty swallowingDysphagia. More information on how to deal with dysphagia can be found herehere.
Poor dental hygiene can lead to tooth decay and gum disease. Dental hygiene can be a problem when the jaw is difficult to open and the tongue difficult to move. Abnormal dental conditions often include high levels of tooth decay, heavy plaque, especially around the lower teeth, unhealthy gums, and bad teeth.
Children should start taking care of their teeth at an early age. A healthy diet, daily oral hygiene (brushing and dental floss), fluoride prophylaxis, the correct use of sealants and a visit to the dentist every 6 months are particularly important.
In people of Duchenne age, impaired function of the upper extremities makes oral hygiene difficult. Parents/carers should be shown how to effectively brush another person's teeth. This may involve how to move the tongue away to access all of the surfaces of the teeth. The areas between the tongue and cheeks must also be included (if the muscles are weaker, there is less “natural cleaning” in this area). For this reason, it is also important to rinse your mouth out with water after each meal to remove any residue that has not been swallowed.
Given the oropharyngeal changes associated with this diagnosis, no one should initiate orthodontic treatment without a thorough knowledge of Duchenne. Talk to your GP or neuromuscular team for orthodontic recommendations.
Even preventative wisdom teeth removal may not be suitable for everyone. This procedure should be evaluated from an individual benefit-risk perspective, including the risk of anesthesia, aspiration, and osteonecrosis (severe bone disease) of the jaw caused by the extraction of molars if patients also take itBisphosphonate.
When a person with Duchenne undergoesgeneral anesthetic, they run the risk of suffering from a number of serious problems.
Recommendations for dental procedures
Dental hygiene and proper care are extremely important and will help reduce the incidence of oral and respiratory infections, particularly pneumonia. Dental procedures can and should generally be performed with minimal or no anesthesia to provide the patient with maximum physical and emotional comfort. Local anesthetics (eg, novocaine, lidocaine) or inhaled nitrous oxide ("laughing gas") are generally safe to use in people with Duchenne, regardless of their lung function or ability to walk. People with Duchenne who are unable to walk and/or have abnormal lung function should be discouraged from using oxygen.
The usage ofinhaled anesthetics(e.g. halothane, isoflurane, seroflurane) can cause serious complications. A complication isRhabdomyolysis, which is the massive breakdown of skeletal muscle tissue that can ultimately damage the kidneys. Another is hyperkalemia, which is when too much potassium is released into the bloodstream, which can lead to cardiac arrest ("heart attack").
laughing gas(“Laughing gas”) used by an attentive dentist in in-office dental procedures is accepted and safe practice, even when inhaled. Nitrous oxide is an inhaled anesthetic commonly used in dentistry, emergency and outpatient settings. The benefits of nitrous oxide are:
- Impressive security profile
- Provides excellent minimal to moderate sedation for anxious patients.
- It is quickly and easily absorbed into the bloodstream and brain and is easily eliminated from the body.
After nitrous oxide, patients are usually given oxygen for 1 to 2 minutes to "wash" the gas out of the airways. Oxygen is supplied in an "open system" (mixed with ambient air) so the oxygen concentration is not 100%. Using an "oxygen scrub" is also a safe and appropriate practice for people with Duchenne.
In many dental procedures, local anesthetics are often administered by injection. Commonly used anesthetics are novocaine or lidocaine. Local anesthetics are considered safe for use in Duchenne.
Many parents express concerns about the use of oxygen. The use of oxygen in an ambulatory patient with normal lung function poses minimal threat. The use of oxygen itself is a problem when it is intended to treat hypoventilation in a non-ambulatory patient with reduced lung function.
Duchenne patients who havepulmonary dysfunction(abnormal breathing) you should consider dental care that requires general anesthesia in a hospital or surgery center staffed by an anesthetist and equipped to monitor intraoperative respiratory function and manage potential respiratory and cardiac emergencies.
Non-ambulatory Duchenne patients have weaker respiratory muscles. Therefore, as the disease progresses, it becomes difficult to cough and breathe deeply. To a certain extent, shallow breathing can supply the body with sufficient oxygen and sufficiently remove carbon dioxide. This delicate balance of oxygen and carbon dioxide allows breathing to continue. When supplemental or supplemental oxygen is administered, this delicate balance is disrupted. The respiratory center can get the wrong impression that the body has enough oxygen and the drive to breathe is decreasing. Without effective breathing, carbon dioxide can build up to dangerous levels (calledHyperkapnie).
oxygenshould never be givenwithout constantly monitoring the level of carbon dioxide CO2 in the exhaled air (the "end-tidal CO")2’) by el CO2blood level. A normal CO at the end of the tide2is between 30-45 mmHg. UN CO2(greater than 45 mmHg) indicates that CO2it is not eliminated from the body. Non-invasive ventilation (Bi-PAP via a mouthpiece or nasal cannula) supports the mechanical process of breathing, oxygenation and CO removal2.
Facts about dental procedures to remember
- Dental procedures can and should generally be performed with the least possible anesthesia while providing maximum physical and emotional comfort to the patient.
- Local anesthetics, nitrous oxide, and an "oxygen flush" are safe for most Duchenne patients, particularly ambulatory patients with normal lung function (normal breathing).
- Duchenne patients with pulmonary dysfunction (abnormal breathing) should consider dental care requiring general anesthesia in a hospital or surgical center staffed by an anesthetist and equipped to monitor intraoperative respiratory function and manage potential respiratory and cardiac emergencies is.
- Becker DE, Rosenberg M, "Laughing gas and the inhalation anesthetics", Anesth Prog, 2008, invierno, 55(4): 124-131.
- „Respiratory Care of the Duchenne Muscular Dystrophy Patient“, American Thoracic Society Paper, Am J Respir Crit Care Med, 2004, 170: 456-465.
- Birnkrant D, Panitch HB, Benditt JO, Boitano LJ, Carter ER, Cwik VA, Finder JD, Iannaccone ST, Jacobson LE, Kohn GL, Motoyama EK, Moxley RT, Schroth MK, Sharma GD, and Sussman MD, "American College of Chest Physician consensus statement on the respiratory and associated management of patients with Duchenne muscular dystrophy undergoing anesthesia or sedation”, Chest, 2007, 132:1977-1986.
Pulmonology (Dr Jonathan Finder, Dr Hemant Sawnani and Dr Richard Shell), Dentistry (Dr Elizabeth Vroom) and Anesthesia (Dr Norbert Weidner)