Anesthetic Considerations In a Patient With History of Ehlers-Danlos Syndrome and Spontaneous TMJ Subluxation

Background: Ehlers-Danlos syndrome (EDS) encompasses a group of hereditary connective tissue disorders that can vary from mildly loose joints and arthritis to life threatening complications such as aortic dissection (1). It... [ view full abstract ]

Background: Ehlers-Danlos syndrome (EDS) encompasses a group of hereditary connective tissue disorders that can vary from mildly loose joints and arthritis to life threatening complications such as aortic dissection (1). It is caused by a genetic defect that interferes with the structure, production or processing of collagen or proteins that interact with collagen.

Case Description: Our patient is a 21 year old female with a history of EDS diagnosed at the age of 19 and spontaneous temporomandibular joint (TMJ) subluxation who presented to our neurosurgery department for consultation with complaints of intermittent and then more consistent, clear fluid drainage from both nostrils accompanied with tinnitus and infrequent headaches over the past 6 months. She was then worked up for a possibility of cerebrospinal fluid leak and rhinorrhea. Of note, her nasal fluid was positive for glucose and beta-2 transferrin protein. It was decided by the neurosurgery department along with otolaryngologist to proceed with endoscopic transnasal repair of the CSF leak. Upon pre-operative examination, patient had a Mallampati score of 1 with 3 finger breadths submental distance and adequate range of motion. Furthermore, it was noted that the patient had limited mouth opening (~2.5 cms) and tenderness over bilateral TMJs. Given these findings, we decided to proceed with fiberoptic intubation for intraoperative airway management.

Discussion: Based on a review conducted by Weismann et al on recommendations for anesthesia and pre-operative management of patient’s with EDS, difficult airway management may occur in many different forms in this patient population (2). These difficulties may stem from temporomandibular dysfunction, premature spondylosis or occipitalatlantoaxial instability (3). Care must be taken to manage the airway of these patients with extra attention given the high risk of joint dislocation and related postoperative neurological complications. Furthermore, given the minimal neck manipulation and mouth opening needed for fiberoptic intubation, this mode of management should always be considered when difficulties are anticipated in the care of patients with EDS.

References:

• Callewaert B, Malfait F, Loeys B, De Paepe A: Ehlers-danlos syndromes and marfan syndrome. Best Pract Res Clin Rheumatol. 2008, 22: 165-189. 10.1016/j.berh.2007.12.005.
• Wiesmann, Thomas, Marco Castori, Fransiska Malfait, and Hinnerk Wulf. "Recommendations for Anesthesia and Perioperative Management in Patients with Ehlers-Danlos Syndrome(s)." Orphanet Journal of Rare Diseases 9.1 (2014)
• Halko GJ, Cobb R, Abeles M: Patients with type IV ehlers-danlos syndrome may be predisposed to atlantoaxial subluxation. J Rheumatol. 1995, 22: 2152-2155.