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Anesthetic Management for Ehlers-Danlos Syndrome, Hypermobility Type Complicated by Local Anesthetic Allergy: A Case Report

Alexandra E. Cesare, Lorenzo C. Rafer, Conrad S. Myler, Kristin B. Brennan

(Department of Anesthesiology and Perioperative Medicine, Penn State Health Milton S. Hershey Medical Center, Hershey, PA, USA)

Am J Case Rep 2019; 20:39-42

DOI: 10.12659/AJCR.912799

BACKGROUND: Ehlers-Danlos syndrome, hypermobility type is characterized by increased extensibility, permeability, and fragility of the affected cartilaginous tissues, including the trachea, larynx, and skin. Anesthetic considerations for patients with this syndrome include intubation difficulties secondary to the collapse of fibro-elastic tissues in the trachea and a reported resistance to local anesthetics.
CASE REPORT: Our patient was a 22-year-old G4P0030 woman with a history of morbid obesity, seizures, Barrett’s esophagus, hypermobility being evaluated for Ehlers-Danlos syndrome, and anaphylaxis to an unknown local anesthetic who was scheduled for cesarean delivery. She refused allergy testing. After rapid-sequence induction of general anesthesia, video laryngoscopy facilitated endotracheal intubation. Delivery and recovery were uneventful for the mother and child.
CONCLUSIONS: No guidelines for neuraxial or general anesthesia exist for patients with Ehlers-Danlos syndrome, hypermobility type. Increased rates of cervical spine instability and local anesthetic resistance have been reported in this population and should be considered when developing the anesthetic plan.

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