Dr Child's Casebook: Molar Influence

As Marfan syndrome passes down the generations and arrives in the newest family member, it’s all about the known unknown. With the inevitable manifestation in at least two of the three major systems, and the individual variability between those affected, there is much to anticipate. But whether it be eyes or heart or skeleton, there is the probability that teeth may well display marfanoid features, namely overcrowding and a high-arched palate. Mindful of this, a mother with two young children sought advice on their long-term dental care.

Q: I am from a large family, many of whom are affected with Marfan syndrome. My father had Marfan syndrome and his five children were all affected. Three of my siblings have already had aortic root surgery. Both my two children, aged two and three respectively, are affected. I am concerned about their dental care.

A: They only need to be seen once a year at this age, provided that they do not eat a lot of sweets or drink fizzy drinks and brush their teeth every night. It is when they get older and have their second teeth that crowding and other problems can occur. The teeth are not soft, and so they do not have an increased risk of cavities.

As we say in our booklet the most common sight dentally speaking in Marfan syndrome is that of a high-arched palate. Some degree of malalignment of the teeth and open bite, tend to be associated with a high palate and in turn can create orthodontic complications. As such, dental planning from an early age is prerequisite to good aesthetic results. But with a slightly increased morbidity and mortality risk associated with general anaesthesia, owing to potential cardiovascular complications amongst those with MFS, a dentist intimately acquainted with the condition is essential.  If general anaesthesia is ever required, the anaesthetist should take into account the high palate, lack of neck extension, and narrow trachea (breathing tube). As with anyone, good oral hygiene is essential, along with regular routine dental check-ups. But in particular for those with MFS prevention of bacterial endocarditis (an infection of the lining of the heart chamber and valves) is all-important because of the high incidence of heart valve involvement and abnormalities of the great vessels.

Antibiotic prophylaxis for any invasive dental procedure such as scaling or tooth extraction should be considered and these guidelines are periodically updated in the British National Formulary and Dental Practitioners Formulary. However, these measures for antibiotic cover are no longer routine for all patients and advice should be sought from the medical team regarding this if there is any doubt prior to dental surgical intervention.


Dr Child's Casebook: Molar Influence
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