Anesthetic management of a patient with musculocontractural Ehlers-Danlos syndrome undergoing scoliosis surgery

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Abstract

Background: Musculocontractural Ehlers-Danlos syndrome is a new and rare subtype of Ehlers-Danlos syndrome in which anesthetic considerations for airway and respiratory management, prevention of skin injuries and joint dislocations, and hemostatic management for severe perioperative bleeding are required. Case presentation: A 19-year-old woman with musculocontractural Ehlers-Danlos syndrome was scheduled to undergo posterior spinal fusion from the 4th thoracic to the 4th lumbar vertebrae under general anesthesia. Her trachea was easily intubated with a videolaryngoscope despite a small mouth and micrognathia. Pressure-controlled ventilation with limited peak inspiratory pressure was performed for prevention of pneumothorax. Skin damage and joint luxation were prevented by using a low rebounding mattress, terpolymer-based barrier film, and careful patient positioning. Blood transfusion was effectively performed on the basis of point-of-care viscoelastic hemostatic assay monitoring. She had an uneventful postoperative course without any complications. Conclusions: We safely managed a patient with musculocontractural Ehlers-Danlos syndrome undergoing scoliosis surgery.

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Wakabayashi, R., Tanaka, S., Tsuchiyama, K., Yamamoto, K., Maruyama, Y., Numata, K., & Kawamata, M. (2020). Anesthetic management of a patient with musculocontractural Ehlers-Danlos syndrome undergoing scoliosis surgery. JA Clinical Reports, 6(1). https://doi.org/10.1186/s40981-020-00352-5

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