[MIM 158 300]

(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