Rare, inherited disorder, characterized by trismus and
pseudocamptodactyly. Patients cannot open completely their mouth.
Almost 3-year-old child with left hemifacial hypertrophy. No visual
or otologic impairment was noted.
Trismus Pseudocamptodactyly Syndrome; Camptodactyly
Limited Jaw Excursion; Hecht-Beals-Wilson Syndrome; Dutch-Kentucky Syndrome.
Genetic disorder first described in 1969 by Frederik
Hecht, an American pediatrician, and Rodney Kenneth Beals, an American
Rare syndrome; possible same familial origin for all
cases has been evocated.
Autosomal dominant; more common in females.
Clinically evocated in patients presenting with an
inability to open their mouth completely, combined with abnormally short
muscle-tendon units in the fingers, causing the fingers to curve or bend
with wrist dorsiflexion.
Features involve mouth (trismus), muscle, and bones (short stature, symphalangy, muscle anomalies,
restricted joint mobility, pseudocamptodactyly). Ptosis, prognathism,
dislocated hip and foot anomalies (metatarsus adductus, down-turning or
hammer toes, talipes equinovarus), and heart malformations (mitral valve
prolapse, aortic root dilatation) may occur. Feeding difficulties are
An anesthetic consultation is highly
recommended. Evaluate the airway for potential difficulty during
direct laryngoscopy and tracheal intubation
(clinical, radiographs) and orthopedic function (clinical).
Direct laryngoscopy and tracheal
intubation can be difficult because of trismus and may require adapted
anesthetic techniques such as fiberoptic intubation. Careful
intraoperative positioning is needed because of skeletal malformations but
can be difficult to realize.
Avoid muscle relaxant until airway
is secured and lung ventilation confirmed. Trismus is not related to a
structural muscle anomaly, and the administration of succinylcholine and
volatile anesthetic agents does not have to be avoided. Prophylactic
antibiotics in case of cardiopathy may be indicated.
Hecht F, Beals RK: Inability to open the mouth fully: An autosomal
dominant phenotype with facultative camptodactyly and short stature.
Preliminary note. Birth Defects Orig Artic Ser 5:96, 1969.
Mabry CC, Barnett IS, Hutcheson MW, et al: Trismus pseudocamptodactyly
syndrome; Dutch-Kentucky syndrome. J Pediatr
Vaghadia H, Blackstock D: Anaesthetic implications of the trismus
pseudocamptodactyly (Dutch-Kentucky or Hecht Beals) syndrome. Can J Anaesth