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Laryngological Presentations of Ehlers-Danlos Syndrome: Case Series of Nine Patients from Two London Tertiary Referral Centers
source: Clinical Otolaryngology
year: 2017
authors: Arulanandam S, Hakim AJ, Aziz Q, Sandhu G, Birchall MA
summary/abstract:Ehlers–Danlos syndrome (EDS) is a heterogeneous heritable disorder of connective tissue characterised by hypermobilityof the joints, skin hyperextensibility and tissue fragility. It does not have racial or gender predilection. The Villefranche classification divides it into six major forms: classic, hyper-mobility, vascular, kyphoscoliosis, arthrochalasia and der-matosparaxis, replacing the older nosology that described types I–XI. Most patients have a normal or near-normal life expectancy, with variable morbidity. However, the vascular type is associated with the serious complications of spontaneous arterial rupture and visceral perforation.
organization: Singapore General Hospital; The Hospital of St John and St Elizabeth; Queen Mary University of London; Charing Cross Hospital; Royal National Throat Nose and Ear HospitalDOI: 10.1111/coa.12708
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