Anesthetic Management of a Patient With Ehlers-Danlos Syndrome

Anesth Prog. 2016 Winter;63(4):204-207. doi: 10.2344/16-00003.1.

Abstract

We describe the case of a 37-year-old woman who had been diagnosed with Ehlers-Danlos syndrome (EDS) 4 years earlier and was scheduled to undergo removal of synovial chondromatosis in the temporomandibular joint. EDS is a heritable connective tissue disorder and has 6 types. In this case, the patient was classified into EDS hypermobility type. The major clinical feature of this type is joint hypermobility. The patient had sprain or subluxation of the elbows and ankles and dislocation of the knees. Anticipated problems during general anesthesia would be affected by the disease type. For this patient, extra attention was directed to positional injury-induced neuropathy and articular luxation, cutaneous injuries, injuries related to intubation and ventilation, and postoperative pain. Anesthesia was induced with propofol, remifentanil, and rocuronium and maintained with oxygen-air-desflurane, propofol, remifentanil, fentanyl, and rocuronium. In this case, the patient was safely managed without adverse events.

Keywords: Ehlers-Danlos syndrome; Hypermobility type; Joint hypermobility and dislocation.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Anesthesia / methods*
  • Chondromatosis, Synovial / surgery*
  • Ehlers-Danlos Syndrome / complications*
  • Female
  • Humans
  • Temporomandibular Joint / surgery*