A Case of an Adult Patient with Freeman-Sheldon Syndrome

<p>Freeman–Sheldon syndrome (FSS) is characterized by whistling face, alinasal hypoplasia, and ulnar drift deformity. It induces various symptoms in the orofacial area. In particular, microstomia always appears, causing difficulties with food ingestion and dental treatment, articulation disorder, and poor oral hygiene；such symptoms are closely associated with the field of dentistry. In this study, we report a patient with FSS who consulted our hospital for the first time in adulthood, and underwent dental treatment.</p><p>As physical findings, flexion contracture of the bilateral fingers and bilateral congenital clubfoot were noted. The facial and oral findings were mostly consistent with common findings of FSS. A small oral fissure was observed, with a mouth width of 32.4mm. Perioral tension and contracture were marked, and maximal mouth opening between the maxillary and mandibular central incisors was 21.5mm. Concerning food ingestion, the patient was satisfied with the present situation, and there was no articulation disorder. In addition, plaque adhesion, dental calculus deposition, and slight gingival swelling were observed at the neck of teeth in both jaws. On the occlusal surfaces of the maxillary and mandibular molars, caries class I to II was noted. There was no intellectual disability. The patient cooperated with dental treatment, but extension of the lips was unfavorable. When trying to widen the buccal mucosa, tension was strong, and the mouth was closed, making treatment difficult. However, treatment was continued, and perioral tension was slightly reduced 2 years after the initial consultation. There was a 1-mm increase in mouth opening.</p><p>Continuous dental treatment for patients with FSS may reduce perioral tension, leading to an improvement in microstomia-related trismus. To establish and maintain a favorable oral environment and function, early, positive, continuous dental intervention may be important.</p>