ICD-11 classes
15 Diseases of the musculoskeletal system or connective...
LD28 — Syndromes with connective tissue involvement as a major...
LD28.0 — Marfan syndrome or Marfan-related disorders
LD28.00 — Congenital contractural arachnodactyly
ICD-11 LD28.00 — Congenital contractural arachnodactyly
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.
The diagnosis includes nothing.
The diagnosis excludes nothing.
It has no clarifying diagnoses.
The diagnosis is coded elsewhere: