Table 2 Anaesthestic preparations for patients with EDS

Patient history

Check for the specific EDS subtype

Perform thorough bleeding anamnesis

Patient history with focus on EDS complications

Check for airway difficulties

Inform patient

Inform the patient about relevant Issues of the underlying disorder with regard to planned anaesthesia

Discuss specific anaesthetic risks for general anaesthesia; discuss regional anaesthesia options with regard to the underlying subtype

Discuss with surgeon

Discuss the severity of the respective EDS in each specific patient

Has your local centre the appropriate facilities and experience for the planned operation AND the underlying EDS?

Strategies for bleeding therapy in vascular EDS subtypes

Avoidance of tourniquets, adequate patient positioning

Avoid ambulatory surgery

Early mobilization strategy

Organize

Cross match blood products, preparation of autotransfusion devices

Difficult airway management (performed by an expert with adequate devices)

Prolonged postoperative care facilities

Inform preoperatively

Inform all team members including PACU and ward nurses in advance for optimal patient care

Be aware

Typical EDS-related emergency-like situations such as difficult airway status, bleeding risks and organ rupture in specific subtypes

Bleeding prophylaxis

Use desmopressin in patients with positive bleeding history

Inform postoperatively

Inform the patient of any anaesthetic difficulties. Fill out a detailed anaesthesia problem card or medical report to anticipate difficulties in the future.