Abstract
Anaesthesia for the repair of a large occipito cervical encephalomyelocele in a neonate with Kippel-Feil syndrome is described. The fusion of the cervical spines, a short neck, low posterior hair line and Sprengel’s deformity, which were present in this patient, collectively indicated Klippel-Feil syndrome. In addition to the usual stigmata of the syndrome, this patient had a large encephalomyelocele and persistant patent ductus arteriosus complicated by congestive heart failure. Patients with this syndrome are vulnerable to cervical spinal cord injury and are at high risk for neurological injury not only during laryngoscopy and intubation but thereafter. Implications of Kippel-Feil syndrome for the anaesthetist are reviewed and discussed.
Résumé
L’anesthésie pour la répartation d’une encéphalomyocèle occipito cervicale chez un nouveau-né atteint d’un syndrome Klippel-Feil est décrite. La fusion de la vertèbre cervicale, un cou court, une insertion postérieure basse de la ligne du cuir chevelu et une déformité de Sprengel’s, présent chez ce patient signes le syndrome de Klippel-Feil. En plus des signes habituelles du syndrome ce patient avait un large encéphalomyocèle ainsi qu’un canal artériel persistant compliqué d’une insuffisance cardiaque. Les patients atteints de ce syndrome sont succeptibles de subir des lésions de la colonne cervicale, des lésions neurologiques non seulement lors de la laryngoscopie et de l’intubation mais aussi lors de la procédure. Les implications du syndrome de Klippel-Feil pour les anesthésistes sont discutées.
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Naguib, M., Farag, H. & Ibrahim, A.E.W. Anaesthetic considerations in Klippel-Feil syndrome. Can Anaesth Soc J 33, 66–70 (1986). https://doi.org/10.1007/BF03010911
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DOI: https://doi.org/10.1007/BF03010911