From the Guidelines
Ehlers-Danlos Syndrome Type III is associated with a moderate bleeding risk, and patients should take precautions to minimize this risk, including informing healthcare providers about their condition and using hemostatic agents during surgical procedures. The bleeding risk in Ehlers-Danlos Syndrome Type III is less severe than in other types, such as vascular EDS, but can still lead to significant complications, including easy bruising, prolonged bleeding after injury, heavy menstrual periods, and nosebleeds 1. To manage this bleeding risk, patients should:
- Inform all healthcare providers about their condition before procedures
- Apply firm pressure for longer than usual (10-15 minutes) for minor bleeding
- Consider using hemostatic agents like gelfoam or surgicel during surgical procedures
- Use medications like tranexamic acid for heavy menstrual bleeding or before dental procedures, and desmopressin (DDAVP) for significant bleeding episodes
- Avoid blood-thinning medications like aspirin and NSAIDs when possible The bleeding tendency in EDS Type III occurs because the connective tissue abnormalities affect blood vessel walls and platelet function, making them more fragile and less effective at forming stable clots 1. While the provided evidence primarily discusses the vascular form of Ehlers-Danlos syndrome, the principles of managing bleeding risk can still be applied to Type III, with a focus on minimizing complications and improving quality of life.
From the Research
Ehlers-Danlos Syndrome Type III and Bleeding Risk
- Ehlers-Danlos syndrome (EDS) is associated with easy bruising and bleeding complications in the majority of patients 2.
- Patients with EDS type III have a bleeding tendency manifested by ecchymoses and haematomas, although thrombotic events are rare in this entity 3.
- A study found that nearly half of patients with EDS have an abnormal bleeding severity score, which appears to be attributable to platelet function abnormalities in 90% of cases 4.
- The risk of bleeding in EDS patients may be contributed by abnormalities in primary hemostasis, including platelet function abnormalities and von Willebrand factor antigen 4, 5.
- Endovascular procedures in patients with EDS can be safely performed with a low rate of dissections and access site complications, but certain indications still remain to be determined 6.
- The bleeding symptoms in EDS patients have been attributed to the fragility of skin and blood vessels caused by "defective collagen wickerwork" of the reticular layer of the skin, as well as abnormalities in subendothelial collagen and its interaction with platelets and von Willebrand factor 5.
Bleeding Risk Factors
- Platelet function abnormalities are a significant risk factor for bleeding in EDS patients, with the risk of bleeding increasing progressively with the number of platelet function abnormalities 4.
- Abnormalities in subendothelial collagen may alter its interaction with platelets and von Willebrand factor, contributing to the risk of bleeding in EDS patients 5.
- Co-existing joint hypermobility may exacerbate bleeding symptoms in patients with mild bleeding disorders 5.
- Mast cell disorders, which are associated with hypermobile-EDS, may also contribute to bleeding symptoms in EDS patients 5.