Freeman-Sheldon Syndrome: Dental and Orthodontic Implications

Methods: The manifestations of FSS in four members of a South African family of Xhosa stock have been documented.

Objectives: Orofacial manifestations are a major syndromic component and warrant early specialized orthodontic intervention.The objective of this article is to highlight the oral and dental management of these individuals.

Results: The most striking yet variable orofacial features of FSS include a stiff immobile flat mid-face and elongated philtrum with rounded cheeks and a small nose, together with dimpling of the chin. Microstomia is a consistent feature and when excessive, may substantially reduce the intercommissural distance.There is considerable variation in severity in affected persons, and between affected members of the same family.

Conclusions: Management in the FSS is essentially orthodontic and orthopaedic. The microstomia in our first patient led to difficulty in swallowing, speech impairment, deficient oral hygiene and dental caries. Stretching of the mouth by regular use of an expansion prosthesis has been recorded and this measure may have a place in the management of the affected mother and child.

Muscular rigidity following halothane anaesthesia has been recorded. In view of the myopathic element in FSS, malignant hyperthermia is also a potential hazard that warrants an appropriate anaesthetic approach.