Journal d'otologie et de rhinologie

Recurrent Laryngeal Dislocation in Ehlers-Danlos Syndrome

Andrew F Goodall, EH Qureshi and MA Siddiq

Recurrent Laryngeal Dislocation in Ehlers-Danlos Syndrome

Introduction: Ehlers Danlos Syndrome (EDS) is an inherited connective tissue disorder affecting collagen synthesis. Common symptoms are skin laxity, joint hypermobility and easy bruising. Patients may suffer recurrent skeletal joint dislocations that are frequently managed conservatively but sometimes require surgery. Patients with Ehlers Danlos Syndrome may present with a range of ENT disorders including dysphonia, difficulty swallowing, and globus sensation. We report an unusual case of recurrent laryngeal dislocation in EDS.

Methods: Case report and literature review.

Results: A 43 year-old female patient with hypermobility-type Ehlers-Danlos syndrome (EDS) and recurrent skeletal joint dislocations was referred to ENT clinic in April 2013 with episodes of recurrent and spontaneous dislocation of the larynx towards the left, which caused brief airway obstruction and respiratory distress before spontaneously resolving. Examination revealed a highly mobile laryngeal framework but nasolaryngoscopic examination was normal. All episodes have been self-limiting and treatment focused on helping the patient deal with the stress of an episode, as well as self-laryngeal manipulation to more rapidly relocate the larynx to the midline.

Conclusion: We report a case of recurrent and spontaneous lateral dislocation of the larynx in a patient with hypermobility-type EDS. It is proposed that the tissue laxity attributed to EDS allows the larynx to rotate spirally causing reversible airway obstruction. This is the first reported instance in the scientific literature. Keywords: Ehlers Danlos Syndrome; Larynx; Laryngeal dislocation; Laryngeal manipulation; Humans