RT Journal Article SR Electronic T1 Congenital contractural arachnodactyly (Beals syndrome). JF Journal of Medical Genetics JO J Med Genet FD BMJ Publishing Group Ltd SP 640 OP 643 DO 10.1136/jmg.31.8.640 VO 31 IS 8 A1 Viljoen, D YR 1994 UL http://jmg.bmj.com/content/31/8/640.abstract AB Congenital contractural arachnodactyly (CCA) is an autosomal dominant disorder akin to, but usually less severe than, Marfan syndrome. The clinical features are marfanoid habitus, arachnodactyly, crumpled ears, camptodactyly of the fingers and adducted thumbs, mild contractures of the elbows, knees, and hips, and mild muscle hypoplasia especially of the calf muscles. Many patients have kyphoscoliosis and mitral valve prolapse and, very occasionally, aortic root dilatation and ectopia lentis have been described. Linkage to a gene coding for fibrillin on chromosome 5q23-31 has been shown in several kindreds. The prognosis for a normal lifespan is good and improvement in joint contractures is usual.