Dutch-Kentucky, syndrome
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(Hecht syndrome, Hecht-Beals syndrome, trismus-pseudocamptodactyly syndrome, Netherland Kentucky syndrome, distal type 7 arthrogryposis)
Very rare. Autosomal dominant transmission of a mutation of the MYH8 gene (17p13.1). Small mouth opening and deformation of the extremities (pseudocamptodactyly by shortness of the muscles of the hand). Sometimes mitral valve prolapse.
Warning (1): there is often a confusion with the Hecht-Beals or Beals syndrome (see this term) which is a marfanoid syndrome.
Warning (2): can be confused with the Freeman-Sheldon syndrome (see this term).
Anesthetic implications:
Difficult intubation.
References :
- Browder FH, Lew D, Shahbazian TS.
Anesthetic management of a patient with Dutch-Kentucky syndrome.
Anaesthesiology 1986; 65: 218-9.
- Vaghadia H, Blackstock D.
Anesthetic implications of the trismus pseudocamptodactyly syndrome.
Can J Anaesth 1988; 35: 80-5
- Lano CF, Wekhaven J.
Airway management in a patient with Hecht’s syndrome.
South Med J 1997 ; 901241-3
- Seavello J, Hammer GB.
Tracheal intubation in a child with trismus pseudocamptodactyly (Hecht) syndrome.
J Clin Anesth 1999; 11: 254-6.
Updated: November 2019