Freeman-Sheldon syndrome

BACKGROUND: Many Freeman-Sheldon syndrome patients suffer from extensive microstomia resulting in possible inhibition of dental and skeletal development as well as difficulties in eating, speech and dental hygiene. Oral commissure contraction treatments vary from patient education to complicated surgical and/or prosthetic treatments, but recurrence is often described. This article reports on a combined surgical and non-surgical approach in order to increase maximum mouth opening and maintain the result of the surgery.

TECHNIQUE: The patient underwent bilateral commissuroplasty using 2 rhomboid flaps for each side. After two steps of intraoral and perioral tissue impression taking, a master cast was poured and a retractor was fabricated from thermoplastic material. The patient was encouraged to wear the retractor the entire day and night except at meal times.

RESULTS: After surgery maximum mouth opening was increased from 20 mm to 37 mm and remained the same six months after the surgery. Psychosocial factors were improved and the patient was satisfied with the results of her treatment.

CONCLUSION: The use of bilateral commissuroplasty in combination with this customized dynamic oral commissure retractor may be applicable in other syndromic patients with a small oral commissure and also in patients with circumoral burns.