Beals Hecht syndrome

Synonym(s)

Beals' arachnodactyly; Beals' syndrome; Hecht-Beals syndrome; congenital contrac-tural arachnodactyly syndrome

Definition

Heritable disorder of connective tissue, present from birth, combining features of Marfan's syndrome with arthrogryposis

Pathogenesis

Unknown; autosomal dominant inheritance

Clinical manifestation

Multiple, congenital, joint contractures; arachnodactyly; dolichostenomelia; kyphoscoliosis; changes of the ear muscle, producing crumpled-appearing ears

Differential diagnosis

Marfan's syndrome; Stickler's syndrome

Therapy

None

References

Jones JL, Lane JE, Logan JJ, Vanegas ME (2002) Beals-Hecht syndrome. Southern Medical Journal 95(7):753-755

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