Bernstein Robert M
Pediatric Orthopaedic Surgery, Cedars-Sinai Medical Center, Los Angeles, CA, USA.
J Am Acad Orthop Surg. 2002 Nov-Dec;10(6):417-24. doi: 10.5435/00124635-200211000-00006.
Arthrogryposis (multiple congenital joint contractures) is an uncommon problem. Because there are many causes, correct diagnosis is important to predict the natural history and determine appropriate treatment. Inconsistent terminology has caused confusion about both diagnosis and treatment. Amyoplasia, the most common type of arthrogryposis, is characterized by quadrimelic involvement and replacement of skeletal muscle by dense fibrous tissue and fat. Early physical therapy and splinting may improve contractures, but surgical intervention is often necessary. Aggressive soft-tissue releases in addition to casting may improve joint position. In more severe contractures, osseous surgery also may be needed. Deformity recurrence is common, particularly in skeletally immature patients.
先天性多发性关节挛缩症是一种罕见的病症。由于病因众多，准确诊断对于预测疾病自然史和确定恰当治疗方法至关重要。术语不一致导致了诊断和治疗方面的混乱。典型性多发性关节挛缩症是最常见的类型，其特征为四肢受累，骨骼肌被致密纤维组织和脂肪替代。早期物理治疗和夹板固定可能改善挛缩情况，但手术干预往往是必要的。除了石膏固定外，积极的软组织松解术可能改善关节位置。对于更严重的挛缩，可能还需要进行骨手术。畸形复发很常见，尤其是在骨骼未成熟的患者中。
