Laryngological presentations of Ehlers-Danlos syndrome: case series of nine patients from two London tertiary referral centers

1.This is a first-in-literature case series of 9 patients with Ehlers-Danlos syndrome (EDS) presenting with laryngological manifestations of their condition.

2.Laryngological and upper digestive tract complaints are relatively common and very varied in EDS, and in this series included dysphagia, dysphonia, laryngospasm, chronic throat pain and post-surgical scarring complicating airway stenosis.

3.Clinical findings in the larynx and pharynx are subtle and may not guide the diagnosis of the syndrome or its local manifestations.

4.Nasal and upper GI endoscopy, pH studies, laryngeal electromyography and radiological investigations were of limited diagnostic value. However esophageal manometry revealed weak to absent peristalsis in 1 patient.

5.To aid in the diagnosis of EDS, the laryngologist should be aware of some of its systemic manifestations. Management is usually non-surgical; but surgical treatment can be complicated by tendency to excessive scarring.

This article is protected by copyright. All rights reserved.

from #ENT via xlomafota13 on Inoreader http://ift.tt/2a926t6
via IFTTT