Clinical features and imbalance of reparative regeneration in craniovertebrofacial structures in patients with genetically determined fibrodysplasia ossificans progressiva

OBJECTIVE

To analyze clinical manifestations of fibrodysplasia ossificans progressiva (FOP) with detailed skeletotopy of ossifications.

MATERIAL AND METHODS

We followed-up 21 patients with FOP of both sexes aged 4-51 years divided into three groups according to ratio of dentition. We identified consequences characterized primarily by ossifications in craniovertebrofacial structures. Clinical and laboratory parameters caused or resulted from dysfunction of regenerative processes were analyzed. We considered data of CT, cone-beam CT, clinical examination, photofixation and palpation.

RESULTS

We established the pattern of disease onset with peak body growth. The last one was recorded at the stage of ankylosis and ossifications formation. Focusing on manifestations of FOP in craniovertebrofacial structures, we revealed the following data for the first time: dental evagination of the crown of the frontal teeth in 19%, dental malformation in 33.3%, carious lesions of hard tissues of the teeth in 33.3%, orthodontic disorders of position of teeth in dental arches in 66.6% of cases (including 38% with skeletal malocclusion). Long-term progressive restriction of mouth opening in 81% of cases was accompanied by complete loss of function in 19% of patients due to ankylosis with ossifications in articulating surfaces of craniovertebrofacial structures.

CONCLUSION

Probably, the presented general data and a detailed analysis of the local manifestations of the disease in craniovertebrofacial structures at different age periods will contribute to a rational approach to care based on the preliminary results presented in diagnostic tests.