Dental and Orthodontic Management
Developed by Sylvia A. Frazier-Bowers D.D.S., Ph.D., School of Dentistry, Department of Orthodontics, University of North Carolina at Chapel Hill in cooperation with the NMF's Professional Advisory Board Marfan syndrome is a potentially life-threatening genetic disorder that affects the body’s connective tissue. It may affect the eyes, skeleton, blood vessels and heart. More than 200,000 Americans have Marfan syndrome or a related disorder.
Connective tissue is present throughout the entire skeleton, including the bones in the face and mouth. As a result of Marfan syndrome which affects a specific type of connective tissue, there may be changes in the jaw and palate that affect the teeth and their function.  (Ad as seen in The Journal of the American Dental Association)
Why is it important for dentists and orthodontists to be aware of Marfan syndrome?
Many people with Marfan syndrome do not know that they have the disorder, but they may be visiting a dentist or orthodontist for treatment of the specific dental aspects. In these cases, it is important for the dentist or orthodontist to combine their observation of the face and mouth with their knowledge of other outward physical signs of Marfan syndrome and, to refer a suspicious patient to appropriate specialists for further evaluation.
It is also important for dentists and orthodontists to be aware of these characteristics because they can play an important role in diagnosing a related Marfan disorder called Loeys-Dietz syndrome which was recently identified. If, when examining a patient a dentist or orthodontist notices a bifid uvula (the tissue hanging down in the middle of the throat that has two or more lobes instead of one), refer the patient to a cardiologist for evaluation for Loeys-Dietz syndrome (LDS). LDS has some overlapping characteristics with Marfan syndrome. However, patients with LDS tend to have aneurysms throughout the arterial tree, not just in the aorta, thus their disease management is different. Many patients originally diagnosed with Marfan syndrome have been, more recently, diagnosed with LDS. Since a bifid uvula is uncommon, dental professionals can help patients obtain a correct diagnosis and the proper treatment.
What is the cause of Marfan syndrome?
A single abnormal gene causes Marfan syndrome. Usually, this gene is inherited from a parent who is also affected. Approximately one-quarter of the cases occur as a result of a new mutation. In this case, neither parent would be affected.
What are the common dental and orthodontic characteristics of Marfan syndrome?
Although there is no evidence that people with Marfan syndrome may be more susceptible to dental cavities or periodontal disease than the general population, there are some changes to the bones in the face and mouth that are more prevalent. These are:
V-shaped or high-arched palate that results from a narrow maxilla, the major bone in the upper jaw. The reduced size of the maxilla may lead to considerable crowding of the teeth. The shape of the palate may also cause a posterior cross-bite – when the upper teeth align inside the lower teeth when an individual bites down.
Having a long, narrow face is characteristic of Marfan syndrome. Sometimes this interferes with "facial harmony" and the proper relationship between the upper and lower jaw and teeth.
Skeletal Class II malocclusion (a deviation from the normal alignment of the upper and lower jaw) is also common in Marfan syndrome. Although this has been inconsistently reported in the literature, it appears that the Class II malocclusion is due to ‘mandibular retrognathia,’ (where the lower jaw hasn’t grown far enough).
Based on limited studies, a skeletal Class II malocclusion is more common than a skeletal Class III malocclusion (generally due to a lower jaw that grows too far). While a Class III malocclusion has been reported, this is usually due to an abnormal tooth position versus an abnormal jaw position.
TMJ problems may also be evidenced. The temporo-mandibular joint (TMJ) is the hinge that links the jaw to the skull. A misshapen joint or laxity of the ligaments that hold this point in place can cause a variety of problems that are known as the "TMJ syndrome." In all likelihood, people with Marfan syndrome are more prone to TJM problems, but this has not been studied rigorously. As a result, they may experience locking of the jaw when it is opened widely, pain when chewing, “popping” or clicking of one or both joints when the mouth is opened, and a persistent ache that can progress to a sense of headache. A variety of approaches to treatment are available.
How are dental and orthodontic problems treated in people with Marfan syndrome?
The growth and development of the human face is a complex, continuous process that varies from person to person. For children with Marfan syndrome – as with other children – the facial characteristics tend to become more noticeable as the child grows.
The challenge presented by the upper jaw and narrow palate in people with Marfan syndrome is to make the teeth fit and ensure that the upper jaw has the correct relationship with the lower jaw. This is always a challenge for orthodontic treatment, whether or not the patients have a disorder such as Marfan syndrome.
There is little published research on the oral manifestations of Marfan syndrome. However, dentists and orthodontists who have treated people with the disorder agree that, for the most part, treatment of orthodontic problems is the same regardless of the presence of Marfan syndrome (see special considerations below). Stability (making sure that the teeth don’t move) and retention (maintaining the results of orthodontic treatment) is an issue for everyone and is monitored closely by orthodontists. Treatment for people with and without a connective tissue disorder is truly individualized.
Are there any special considerations in the treatment of people with Marfan syndrome?
People with known abnormalities of the heart valves (aortic stenosis, mitral stenosis, mitral regurgitation, etc.) and those who have undergone valve replacements and/or aortic graft surgery are at increased risk of developing endocarditis (a serious infection of the heart valves). Many people with Marfan syndrome fall into this category. Therefore, they must take precautions prior to any procedure that may introduce bacteria into the bloodstream. This includes routine dental work. Many dental procedures go below the gum line and provide an opportunity for bacteria to enter the blood stream. Extracting teeth, which is often done to make room for new teeth and help alignment warrants caution in people with Marfan syndrome because of the risk of infection. People with Marfan syndrome should advise their dentist if they have a heart problem so that the dentist can consult with the cardiologist about the need for antibiotics prior to beginning the dental work.
If an individual is getting dental implants – which go into the bone – antibiotic treatment ahead of time would be needed. Cosmetic bonding of the teeth is not invasive, but any time a high speed instrument is used by a dentist or hygienist, there is a chance that the gum tissue can get injured, which could lead to bacteria entering the blood stream.
Typically, when an individual requires a lot of dental work, it is stretched over a number of visits. However, due to the need for antiobiotics prior to dental treatment, people who require this may choose to have as much work as possible done in one day to reduce the frequency of pre-medication. In some cases, a dentist and the patient may opt to have the work done in the hospital while the patient is under sedation.
Routine visits to the orthodontist are not invasive, except for fitting and cementing bands on molar teeth (this procedure requires going below the gum line and, therefore, may provide an entry for bacteria). Therefore, people with Marfan syndrome should also make their condition known to the orthodontist so that he/she can plan for the appropriate pre-medication during the banding procedure (only one visit).
A person with Marfan syndrome who is scheduled for elective cardiac surgery should have a complete dental exam, cleaning and any major dental work done prior to the cardiac operation.
The American Heart Association guidelines for endocarditis prophylaxis (prevention of an infection of the heart and valves) should be followed for people at increased risk for this infection. Can people with a high-arched palate be fitted for dentures?
While it may be more difficult to take an impression (mold) of the individual’s teeth, there is no reason why people with Marfan syndrome who have a high arched palate cannot wear dentures. The challenge is obtaining the proper fit, which may require that the design of the denture be modified. However, this is true of anyone in the general population who has a high arched palate.
Why is preventive dental care so important, especially for people with Marfan syndrome?
Because of the risk of endocarditis, people with Marfan syndrome should aim to keep their teeth as healthy as possible before any problems arise in order to minimize the amount of dental work needed. Periodontal disease (gum infections) provides a breeding ground for harmful bacteria so keeping gums healthy by practicing good oral hygiene is essential. Regular dental care is a must!
When is dental work not recommended for people with Marfan syndrome?
Cosmetic dental and orthodontic procedures should only be undertaken after carefully considering the individual’s health and the need for the procedure. If there are other serious health concerns, a cardiologist or primary care physician should be consulted before any dental and orthodontic issues are addressed. Unless the dental or orthodontic problems impact the individual’s ability to speak or eat, they should not be considered a priority for someone with otherwise fragile health, as occurs in a minority of people with Marfan syndrome.
Most people with Marfan syndrome are excellent candidates for elective dental procedures, including those associated with the risk of introducing bacteria into the bloodstream. Given proper evaluation and management, the risk associated with these procedures is very low.
Does someone with Marfan syndrome need to visit a dentist or orthodontist with special expertise in treating people with the disorder?
Children and young teens with Marfan syndrome should first visit a pediatric dentist, who is trained to treat a variety of children, including those with various syndromes. Most general dentists would be comfortable treating people with other health concerns, but if they are not, they will usually refer you to the nearest University or Medical Center. If an individual has added concern about seeing a general dentist, he or she may visit a hospital-based dentist, who is accustomed to treating patients with special needs. For orthodontic treatment, a private practice orthodontist is fine because they are also trained to treat people with different conditions – both children and adults.
In many cities there are craniofacial teams, which consist of an orthodontist, pediatric dentist, oral surgeon, speech pathologist, audiologist and plastic surgeon. If the local orthodontist feels that a case is too difficult to be treated in his/her practice, a referral may be made to the nearest craniofacial team.
In any case, a person with Marfan syndrome or a related connective tissue disorder should advise the dentist or orthodontist about their condition and explain any cardiac problems that exist. It is especially important that the dentist and orthodontist know about any mitral valve abnormality, aortic regurgitation, artificial heart valves and heart surgeries. In most cases, they will also request a letter from the treating cardiologist regarding the status of the heart.
Key Points Daily dental care – brushing and flossing -- and regular visits to the dentist to prevent problems are important for everyone, but are essential for people with Marfan syndrome. Consult a cardiologist about the need for antibiotics before dental procedures. Consider if dental or orthodontic work is needed to improve function (eating or speaking) or cosmetics (to improve the appearance).It is important to balance the treatment of dental issues against risks to the heart and cardiovascular system. When dental or orthodontic work is needed, it is important to use antibiotics to lessen the potential risk of endocarditis.
Additional information on endocarditis prophylaxis.
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