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Anaesthetic managements for conjoined twins with complex cardiac anomalies

Abstract

Purpose

To describe the perioperative assessment and anaesthetic management for surgical separation of three sets of conjoined twins with complex cardiac anomalies threatened with arterial desaturation and haemodynamic instability.

Clinical features

Three sets of conjoined twins, one omphaloischiopagus, one omphalopagus, and one thoraco-omphalopagus, were considered for separation during the perinatal or infantile period. Preoperative functional evaluation including continuous pulse oximetry, capnography, and cardiac electrophysiological studies were considered to be as important as anatomical evaluation of the cardiac anomalies and cross-circulation by angiography in assessing the feasibility of surgical separation. Ipsilateral infusion of prostaglandin E1 and phenylephrine were applied to the cyanotic and healthy twins respectively, to restore arterial oxygenation intraoperatively and to avoid profound hypoxaemia.

Conclusion

Surgical separation and anaesthesia should be well planned and rehearsed before clinical deterioration of the weaker twin. Aggressive pharmacological intervention and understanding of the cross-circulation pathophysiology is necessary to manage critical situations during surgical separation and in the postoperative period.

Résumé

Objectif

Décrire l’évaluation périopératoire et la gestion anesthésique de la séparation chirurgicale de trois couples de jumeaux siamois porteurs d’anomalies cardiaques complexes susceptibles de désaturation artérielle et d’instabilité hémodynamique.

Éléments cliniques

Trois couples de jumeaux siamois, dont un omphaloischiopage, un omphalopage et un thoraco-omphalopage étaient programmés pour une séparation pendant la période périnatale ou infantile. L’évaluation fonctionnelle préopératoire dont l’oxygmétrie de pouls continue, la capnographie, et les épreuves électrophysiologiques cardiaques étaient considérées comme aussi importantes que l’évaluation anatomique des anomalies cardiaques et l’étude de la circulation croisée par angiographie pour décider de la faisabilité d’une séparation chirurgicale. Une perfusion homolatérale de protaglandine E1 et de phényléphrine ont été administrées respectivement au jumeau cyanotique et au jumeau sain pour restaurer l’oxygénation artérielle pendant l’intervention et pour éviter l’hypoxémie profonde.

Conclusion

La séparation chirurgicale et l’anesthésie doivent être bien planifiées et répétées avant la détérioration du jumeau faible. Une intervention pharmacologique agressive et une connaissance de la physiopathologie de la circulation croisée sont essentielles pour la gestion des situations critiques pendant l’intervention pour séparation ainsi qu’à la période postopératoire.

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Correspondence to T. -L. Chen.

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Chen, T.-., Lin, C.-., Lai, H.-. et al. Anaesthetic managements for conjoined twins with complex cardiac anomalies. Can J Anaesth 43, 1161 (1996). https://doi.org/10.1007/BF03011846

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Key words

  • Anaesthesia: paediatric
  • Congenital Anomaly: cardiac
  • Twins: conjoined