Abstract
Purpose
The King-Denborough syndrome (KDS) is a rare disorder that is associated with myopathy, susceptibility to malignant hyperthermia (MH) as well as congenital skeletal and facial anomalies. We report the anesthetic management of a parturient with KDS.
Clinical features
We describe the management of a 24-yr-old primiparous woman with a diagnosis of KDS and a history of previous MH reaction (age two). Her KDS resulted in chronic respiratory failure. She had a permanent tracheostomy and required overnight ventilatory support for the previous two years. She had three admissions during her pregnancy, one for pneumonia and two for preterm labour. Labour was induced at 37 weeks. Her labour was managed in the operating room where a “clean” anesthesia machine was ready. Cooling aids and a MH emergency kit were immediately available. Intravenous access, an arterial line and a lumbar epidural catheter were inserted before induction of labour.
Ropivacaine 0.08% + fentanyl 2μg·mL−1 were used for patientcontrolled epidural analgesia. After 6.5 hr of labour the patient required ventilation. An outlet forceps was performed for delivery. Postpartum, she was ventilated overnight in the intensive care unit.
Conclusion
The use of epidural analgesia, close monitoring and collaboration between the various disciplines were important in achieving a safe and uneventful labour in this high-risk parturient.
Résumé
Objectif
Le syndrome de King-Denborough (SKD) est une affection rare, associée à une myopathie, une susceptibilité à l’hyperthermie maligne (HM) et à des anomalies squelettiques et faciales congénitales. Nous présentons la prise en charge anesthésique d’une parturiente atteinte du SKD.
Éléments cliniques
La patiente est une primipare de 24 ans atteinte du SKD et qui a présenté, à l’âge de deux ans, une réaction d’HM. Le SKD a entraîné une insuffisance respiratoire chronique. Porteuse d’une trachéotomie permanente, la patiente avait besoin d’une assistance ventilatoire pendant la nuit depuis deux ans. Elle avait été hospitalisée trois fois déjà pendant sa grossesse, une fois pour une pneumonie et deux fois pour un travail prématuré. Le travail a été induit à 37 semaines. Il a été pris en charge dans la salle d’opération où un appareil d’anesthésie non contaminé était prêt à utiliser. Du matériel de refroidissement et une trousse d’urgence pour l’HM étaient aussi disponibles. Un accès intraveineux a été pratiqué, et un cathéter intra-artériel et un cathéter péridural lombaire insérés avant l’induction du travail. La ropivacaïne à 0,08 % avec du fentanyl (2 μg·mL−1) ont servi à l’analgésie épidurale autocontrôlée. Après 6,5 h de travail, la patiente a eu besoin de ventilation. L’accouchement a été réalisé avec l’application de forceps. En post-partum, la patiente a été sous ventilation pendant la nuit à l’unité des soins intensifs.
Conclusion
L’utilisation d’analgésie épidurale, d’une surveillance étroite et de la collaboration multidisciplinaire ont assuré un travail obstétrical sûr et sans incident chez une parturiente à haut risque.
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Habib, A.S., Millar, S., Deballi, P. et al. Anesthetic management of a ventilator-dependent parturient with the King-Denborough syndrome. Can J Anesth 50, 589–592 (2003). https://doi.org/10.1007/BF03018646
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DOI: https://doi.org/10.1007/BF03018646