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

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.

Cervical Spinal Surgery of a Patient with Hemophilia and Ehlers-Danlos Syndrome: Anesthetic Considerations

“…Factors that require anesthesiologist's attention in patients with EDS are: fragile and poorly healing skin, excessive bleeding, predisposition to spontaneous pneumothorax, especially with high ventilatory pressures and excessive tidal volumes, easy joint dislocation, cardiac mitral valve prolapse, presence of coronary and cerebral aneurysms, spontaneous dissections or ruptures of major vessels, small mouth and microretrognathia, mucosal damage and bleeding as a result of endotracheal intubation [7,12].…”