THE AIM OF THE STUDY

Was to assess age-related changes in mouth opening and tongue mobility in children with various forms of epidermolysis bullosa (EB). Materials and methods. The study comprised 50 EB children (mean age 8.8±3.9 years). Oral slit width (the distance between mouth commissures at rest), mouth opening amplitude (the distance between incisive edges of the lower and upper incisors) were measured by orthodontic caliper. Tongue mobility was assessed using Bristol Tongue Assessment Tools (BTAT). The results were compared with 55 healthy age-matched controls (mean age 9.3±3.7 years).

RESULTS

Mean moth opening in EB group was 22.6±11.1 mm which is twice lower than in controls (44.3±7.2 mm). The amplitude was progressively increasing in EB patients in the mixed dentition period but then dropped drastically in the permanent dentition because of oral fibrosis. Tongue mobility was lower in EB group when compared to controls even in cases with anatomically appropriate frenulum fixation sites. In permanent dentition maximal tongue raising was 8 times lower than in controls. Microstomia and ankyloglossia were very specific for dystrophic EB patients while in EB simplex, junctional EB and Kindler syndrome the values were similar to healthy controls.

CONCLUSION

Microstomia, limited mouth opening and tongue mobility are typical oral features of dystrophic EB patients. Age-related dynamics shows progressive growth of the values in mixed dentition with significant lowering after so preventive measures for oral fibrosis are more feasible before permanent dentition phase.