2002 年 51 巻 3 号 p. 625-628
We report a case of congential contractual arachnodactyly (CCA) with extentional limitation of the knee joint. CCA is characterized by multiple contractures, arachnodactyly, dolichostenomelia, scoliosis, abnormality of the external ears, and autosomal dominant transmission.
A 15-year-old female had been suffering from extentional limitation of the left knee (20 degrees) since age 8. When she was 12, physical examination showed scoliosis of 20 degrees, but scoliosis continued to remain at 20 degrees. She experienced no pain while resting, walking, and in the full flexion position of the left knee. Plain radiograph, MRI and arthroscopy did not indicate abnormal changes of the left knee, but plain radiograph showed elongation of the proximal phalanges. She also had extentional limitation of the fingers in each hand. She was diagnosed with CCA because of multiple contractures, arachnodactyly, dolichostenomelia, and scoliosis.