Soonchunhyang Med Sci.  2023 Dec;29(2):49-53. 10.15746/sms.23.011.

Anesthetic Management of Emergency Surgery for a Patient with Vascular Ehlers Danlos Syndrome (Type IV): A Case Report

• Woo UT
• Shin WJ
• Shim JH
• Jeon WJ
• Park HJ

• KMID: 2549909
• DOI: http://doi.org/10.15746/sms.23.011

Abstract

Ehlers-Danlos syndrome (EDS) is a very rare genetic disorder characterized by defects in the production of connective tissue. Among them, vascular EDS is the subtype known to have the worst prognosis due to fragile blood vessels. Accordingly, we would like to report a case report of a patient with vascular EDS with a review of the literature on possible massive transfusion and anesthesiological problems. Patients with vascular EDS have very weak blood vessels and tissues that are easily broken. So there is a high possibility of unexpected massive bleeding during emergency surgery in these patients. Therefore, the anesthesiologist should be fully prepared for the possibility of massive blood loss, keeping in mind the possibility of damage to large blood vessels. The central vein must be secured using ultrasound, sufficient blood and fluids must be prepared, and equipment capable of rapid administration must be perfectly prepared before the start of operation.

Keyword

Anesthesia; Ehlers-Danlos syndrome; General surgery; Hemoperitoneum; Case report