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Anaesthesia for a patient with central alveolar hypoventilation syndrome (Ondine’s curse)

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Abstract

The perioperative anaesthetic management of an adult patient with central alveolar hypoventilation syndrome (CAHS), On-dine’s Curse, is described for anterior resection of a carcinoma of the bowel. This rare syndrome results in alveolar hypoventilation, hypercarbia, hypoxaemia with secondary polycythaemia, pulmonary artery hypertension, and cor pulmonale. Epidural morphine was used for postoperative analgesia in an attempt to improve postoperative respiratory function. However, post-operative mechanical ventilation was required until recovery of the respiratory drive, which was ablated by anaesthetic drugs, epidural morphine and high inspired oxygen concentrations. The pathophysiology and treatment of this syndrome are reviewed.

Résumé

La conduite anesthésique périopératoire d’un patient adulte atteint du syndrome d’hypoventilation alvéolaire d’origine centrale (CAHS), est décrite lors d’une résection antérieure d’un carcinone. Ce syndrome rare consiste en une hypoventilation alvéolaire, hypercarbie, hypoxémie et secondairement une polycythémie, hypertension pulmonaire et cœur pulmonaire. La morphine en injection épidurale a été utilisée pour l’analgésie postopératoire dans une tentative d’améliorer la function respiratoire postopératoire. Cependant, une ventilation mécanique postopératoire fut requise jusqu’au recouvrement du stimulus respiratoire qui était aboli par les agents anesthésiques, la morphine épidurale et les concentrations élevées d’oxygène inspirée. La pathophysiologie et les traitements de ce syndrome sont revus.

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Wiesel, S., Fox, G.S. Anaesthesia for a patient with central alveolar hypoventilation syndrome (Ondine’s curse). Can J Anesth 37, 122–126 (1990). https://doi.org/10.1007/BF03007492

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