Can Ehlers-Danlos Syndrome (EDS) increase the risk of postoperative bleeding after a septoplasty procedure?

From the Guidelines

Yes, Ehlers-Danlos Syndrome (EDS) can increase the risk of postoperative bleeding after a septoplasty procedure due to its effects on collagen production, leading to fragile blood vessels and poor wound healing. EDS is a group of inherited connective tissue disorders that can lead to vascular fragility and platelet dysfunction, making patients more susceptible to bleeding during and after surgical procedures, including septoplasty 1. The underlying reason for this increased bleeding risk is that the collagen defects in EDS affect blood vessel integrity and clotting mechanisms, making it harder for the body to form stable clots and repair damaged vessels.

Key Considerations for Septoplasty in EDS Patients

• Informing the surgeon about the condition beforehand is crucial to take additional precautions such as having blood products available, using hemostatic agents during surgery, and planning for a potentially longer recovery period 1.
• Temporarily stopping medications that could increase bleeding risk, such as NSAIDs, for 1-2 weeks before surgery may be recommended 1.
• Post-operatively, patients may require more careful monitoring, longer nasal packing, and possibly additional follow-up appointments to ensure proper healing.
• The decision to intervene for aortic and branch vessel aneurysms and dissections in EDS patients involves a Multidisciplinary Aortic Team and shared decision-making, highlighting the complexity of managing vascular complications in these patients 1.

Management and Treatment

• Surgical repair in vascular EDS carries an increased risk due to vascular fragility and associated bleeding complications, emphasizing the need for meticulous technique to lessen vascular and tissue trauma 1.
• Medical therapy includes education, lifestyle modification, and avoidance of invasive procedures when possible, with some studies suggesting a benefit of celiprolol, a beta blocker with vasodilatory properties, in patients with vascular EDS 1.

Outcome and Quality of Life

• The increased risk of postoperative bleeding in EDS patients undergoing septoplasty can impact their quality of life, making it essential to weigh the benefits and risks of the procedure and to take necessary precautions to minimize complications 1.
• The collagen defects in EDS can lead to reduced life expectancy, with a median survival of only 48 years, primarily due to arterial ruptures, highlighting the importance of careful management and monitoring of these patients 1.

From the Research

Ehlers-Danlos Syndrome and Postoperative Bleeding Risk

• Ehlers-Danlos Syndrome (EDS) is a heterogeneous group of inherited connective tissue disorders that can affect various organs, including skin, bones, vessels, and others 2, 3, 4, 5.
• Patients with EDS are at a higher risk of increased bleeding, delayed wound healing, and other complications due to the fragility of their blood vessels and tissues 2, 6, 3, 4, 5.
• The risk of bleeding in EDS patients is associated with platelet function abnormalities, which can be detected using the Bleeding Assessment Tool of the ISTH and the bleeding severity score (BSS) 5.
• Studies have shown that EDS patients have a higher incidence of vascular lesions, aneurysm formation, and spontaneous rupture, making surgical management hazardous and often unrewarding 2.
• However, with careful patient selection, modification of surgical technique, and appropriate perioperative hemostatic management, surgery can be safe and effective in patients with EDS 6, 4.

Septoplasty Procedure and EDS

• There is no direct evidence in the provided studies that specifically addresses the risk of postoperative bleeding after a septoplasty procedure in patients with EDS.
• However, considering the increased risk of bleeding and vascular complications associated with EDS, it is likely that patients with EDS may be at a higher risk of postoperative bleeding after a septoplasty procedure 2, 6, 3, 4, 5.
• Surgeons should be aware of the potential risks and take necessary precautions, such as careful patient selection, modification of surgical technique, and appropriate perioperative hemostatic management, to minimize the risk of complications in patients with EDS undergoing septoplasty or other surgical procedures 2, 6, 4.