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


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.


CONCLUSIONS
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.
Steinberg
B
B
Section of Oral and Maxillofacial Surgery, University of Michigan, Ann Arbor 48109-0018, USA.
Nelson
V S
VS
Feinberg
S E
SE
Calhoun
C
C
D000293:Adolescent; D001176:Arthrogryposis / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D002972:Cleft Palate / Q000473:pathology / Q000601:surgery; D004935:Esophageal Diseases / Q000151:congenital; D005152:Facial Muscles / Q000473:pathology*; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D007571:Jaw Diseases / Q000151:congenital*; D008297:Male; D008310:Malocclusion / Q000473:pathology; D008336:Mandibular Diseases / Q000151:congenital / Q000000981:diagnostic imaging / Q000628:therapy; D008439:Maxillary Diseases / Q000473:pathology; D018908:Muscle Weakness / Q000151:congenital; D010159:Palate / Q000473:pathology; D026741:Physical Therapy Modalities; D010855:Pierre Robin Syndrome / Q000473:pathology; D013705:Temporomandibular Joint Disorders / Q000151:congenital / Q000000981:diagnostic imaging / Q000628:therapy; D014057:Tomography, X-Ray Computed