Ehlers-Danlos Syndrome Type IV - Anaesthetic Considerations

Ehlers-Danlos syndrome (EDS) is a rare disorder that occurs due to genetic defect in the collagen synthesis. The vascular subtype of EDS (type IV) is defined by characteristic facial features, translucent skin, easy bruising, and spontaneous arterial rupture and visceral perforation of such organs as the uterus and intestines, with possible life-threatening consequences. We report a case of a 15-year-old male patient with no past medical history undergoing emergency laparotomy after a spontaneous sigmoid colon perforation. Post-operatively and during the ICU stay complications developed and several revision operations and invasive procedures were necessary. A chest CT angiography revealed a right subclavian artery aneurysm, which was treated by an endovascular stent grafting. Taking into consideration the family history and clinical presentation EDS type IV was discussed as a possible cause of the patient's condition. DNA analysis confirmed the diagnosis. Due to the lack of evidence-based recommendations the anaesthetic management of these patients is still challenging.