Incidence of maxillofacial involvement in arthrogryposis multiplex congenita.

PURPOSE: This study determined the incidence of maxillofacial involvement in patients diagnosed with arthrogryposis multiplex congenita (AMC).

PATIENTS AND

METHODS

Twenty-three patients were evaluated by the pediatric physical medicine and rehabilitation, orthopedic surgery, and pediatric oral and maxillofacial surgery departments. Any patient in whom the diagnosis of AMC was in doubt was excluded from the study. All patients with limited mandibular function underwent computed tomography (CT) examination of their temporomandibular joints (TMJ). The results of physical therapy were followed.

RESULTS

Five of the 23 patients diagnosed with AMC were found to have maxillofacial involvement, eg, presence of cleft palate, Robin-like sequence, high-arched palate, open-bite deformity, facial muscle weakness, esophageal dysfunction, and limited mandibular opening. No TMJ abnormalities were found by CT scan. Physical therapy was used for treatment of the limited opening, but relapse occurred quicky after therapy was discontinued.

CONCLUSION

The incidence of maxillofacial findings is similar to that of most other reports. Treatment involves surgical correction of abnormal anatomy when possible (ie, cleft repair), symptomatic management (ie, esophageal dysfunction), and physical therapy.