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

JA Clinical Reports
Ryo WakabayashiMikito Kawamata

Abstract

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. 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. We safely managed a patient with musculocontractural Ehlers-Danlos syndrome undergoing scoliosis surgery.

References

Apr 24, 2003·European Spine Journal : Official Publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society·Sercan AkpinarFatih Dikici
Apr 19, 2008·Anesthesia and Analgesia·Michael T Ganter, Christoph K Hofer
Jul 24, 2014·Orphanet Journal of Rare Diseases·Thomas WiesmannHinnerk Wulf
Dec 10, 2015·Pediatrics International : Official Journal of the Japan Pediatric Society·Tomoki Kosho
Mar 18, 2017·American Journal of Medical Genetics. Part C, Seminars in Medical Genetics·Fransiska MalfaitBrad Tinkle
May 2, 2017·European Journal of Anaesthesiology·Sibylle A Kozek-LangeneckerKai Zacharowski

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