ICD·index Chapters ENESDE
LD28.00 category

Congenital contractural arachnodactyly

Definition

Congenital contractural arachnodactyly (CCA, Beals syndrome) is a connective tissue disorder characterised by multiple flexion contractures, arachnodactyly, severe kyphoscoliosis, abnormal pinnae and muscular hypoplasia. Although the clinical features can be similar to Marfan syndrome (MFS), multiple joint contractures (especially of the elbow, knee, and finger joints), and crumpled ears in the absence of significant aortic root dilatation are characteristic of Beals syndrome and rarely found in MFS.

Also indexed as

ArachnodactylyBeals syndromeCongenital contractural arachnodactylyDistal arthrogryposis type 9

Nearby in Marfan syndrome or Marfan-related disorders