Abstract
The trismus pseudocamptodactyly syndrome is a relatively rare, autosomal dominant condition first described in 1969. Affected patients classically present with two main features: limited excursion of the mandible and flexion deformity of the fingers that occurs with wrist extension (pseudocamptodactyly). Foot deformities and a shorter-than-normal stature may also be present. The underlying abnormality is short muscle tendon units, which prevent normal growth and development. We reviewed the anaesthetic experience in three paediatric patients with the trismus pseudocamptodactyly syndrome and the pertinent clinical findings in three other members of the same family, spanning three generations. Limited mandible excursion was present in all six cases, but was not obvious preoperatively in the patients because of its subtle presentation. All three cases were successfully managed using mask anaesthesia with spontaneous ventilation, avoiding muscle relaxants. Attempts to visualize the larynx under anaesthesia were unsuccessful in two cases. Blind nasotracheal intubation was successful in one patient. Postoperatively, there were no problems with the airway.
Résumé
Le syndrome de trismus pseudocamptodactylie est relativement rare, autosomal dominant décrit initialement en 1969. Les patients atteints présentent classiquement des caractéristiques majeures: une excursion limitée de la machoire inférieure et une déformité en flexion des doigts accompagnée ďune extension du poignet (pseudocamptodactylie). Des déformités du pied et une stature plus courte que la normale peuvent aussi être présentes. Ľanomalie de base serait des tendons musculaires courts qui empêchent la croissance normale et le développement. On a revu ľexpérience anesthésique chez trois patients pêdiatriques atteints de ce syndrome ainsi que dans trois autres membres de la même famille, pour trois générations. La mobilisation limitée de la mandibule était présente chez tous les six cas et n’était pas évidente en période préopératoire à cause de sa présentation subtile. Une conduite anesthésique en masque à ventilation spontanée évitant les relaxants musculairesfut utilisée pour tous les trois cas. Des tentatives de visualiser le larynx sous anesthésie ne furent pas possibles dans deux cas. Une intubation nasotrachéale aveugle fut réussie chez un patient. En période postopératoire aucun problème des voies aériennes ne fut noté.
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Vaghadia, H., Blackstock, D. Anaesthetic implications of the trismus pseudocamptodactyly (Dutch-Kentucky or Hecht Beals) syndrome. Can J Anaesth 35, 80–85 (1988). https://doi.org/10.1007/BF03010551
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DOI: https://doi.org/10.1007/BF03010551