Steinberg B, Nelson V S, Feinberg S E, Calhoun C
Section of Oral and Maxillofacial Surgery, University of Michigan, Ann Arbor 48109-0018, USA.
J Oral Maxillofac Surg. 1996 Aug;54(8):956-9. doi: 10.1016/s0278-2391(96)90391-2.
This study determined the incidence of maxillofacial involvement in patients diagnosed with arthrogryposis multiplex congenita (AMC).
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.
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.
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.
本研究确定了先天性多发性关节挛缩症（AMC）患者颌面部受累的发生率。
23例患者接受了儿科物理医学与康复科、整形外科以及儿科口腔颌面外科的评估。任何诊断存疑的患者均被排除在研究之外。所有下颌功能受限的患者均接受了颞下颌关节（TMJ）的计算机断层扫描（CT）检查。随访物理治疗的结果。
在23例诊断为AMC的患者中，有5例存在颌面部受累，例如腭裂、罗宾序列征、高拱腭、开牙合畸形、面部肌肉无力、食管功能障碍以及下颌开口受限。CT扫描未发现TMJ异常。采用物理治疗来处理开口受限问题，但治疗中断后很快复发。
颌面部表现的发生率与大多数其他报告相似。治疗包括尽可能对异常解剖结构进行手术矫正（如腭裂修复）、对症处理（如食管功能障碍）以及物理治疗。
