8) Distal Arthrogryposis
(DA) syndromes are often hereditary and joint involvement is predominantly in the hands and feet.
McMillin MJ, Beck AE, Chong JX, et al Mutations in PIEZO2 cause Gordon syndrome, Marden-Walker syndrome, and distal arthrogryposis type 5.
Mutations in TNNT3 cause multiple congenital contractures: A second locus for distal arthrogryposis type 2B.
Distal arthrogryposis is a group of syndromes characterized by congenital contractures of the hands and feet.
Freeman-Sheldon syndrome is essentially a severe form of distal arthrogryposis, specifically distal arthrogryposis type 2A.
Key Words: Freeman-Sheldon syndrome, distal arthrogryposis type 2A, spinal surgery, anaesthesia
FSS is part of the nosologic group of pathologies currently known as distal arthrogryposis (2).
A revised and extended classification of distal arthrogryposis.
Report-Mutations in PIEZO2 cause Gordon Syndrome, Marden-Walker Syndrome, and Distal Arthrogryposis