From the Guidelines
The treatment for Vascular Ehlers-Danlos Syndrome (vEDS) should focus on preventing life-threatening vascular complications, with a multidisciplinary approach to manage symptoms and improve quality of life. According to the 2024 ESC guidelines for the management of peripheral arterial and aortic diseases 1, vEDS is a rare and severe form of Ehlers-Danlos syndrome, with a high risk of vascular complications such as dissections, aneurysms, arterial ruptures, and arteriovenous fistulas.
Key aspects of management include:
- Early identification and thorough family inquiry to identify individuals at risk
- Regular monitoring for vascular complications, with imaging studies as needed
- Avoidance of activities that may increase the risk of vascular complications, such as contact sports or heavy lifting
- Management of hypertension and other cardiovascular risk factors to reduce the risk of vascular complications
- Consideration of prophylactic measures, such as beta-blockers or other medications, to reduce the risk of vascular complications, although the evidence for these measures is limited 1
It is essential to note that the prognosis for vEDS depends on the type of COL3A1 variant, with null variants showing a better outcome 1. The rate of recurrence of organic complications in patients with vEDS is high, with 1.6 events per 5-year period, and life expectancy is reduced to an average of 51 years 1. Therefore, a comprehensive and multidisciplinary approach to management is crucial to improve quality of life and reduce the risk of complications.
From the Research
Treatment Options for Ehlers-Danlos Syndrome (EDS)
- The treatment of EDS is multifaceted and often requires a combination of different modalities, including physical therapy, occupational therapy, and pain management 2.
- A study found that occupational therapy and bracing were the most effective options for EDS patients, with 70% of patients reporting improvement 2.
- Complimentary and alternative therapies, such as traditional Chinese therapies, herbal medications, and marijuana, are also commonly used by EDS patients to manage chronic pain 3.
- Opioids should be used cautiously in EDS patients due to the risk of opioid use disorder and overdose, and personalized plans for opioid tapering may be necessary for those on long-term opioid therapy 4.
Pain Management in EDS
- Chronic pain in EDS involves both nociceptive and neuropathic pain, necessitating a comprehensive pain management approach 4.
- Non-opioid medications, physical therapy, and psychological support are essential components of pain management in EDS patients 4.
- A study found that patient-initiated complementary therapy use in EDS patients is widespread, with 56% of participants reporting the use of non-prescribed therapies 3.
Wound Healing and Management in EDS
- EDS patients may experience delayed wound healing due to the fragile nature of their skin and connective tissue 5, 6.
- A case report described the successful treatment of an EDS patient with extensive burn injuries using a multi-stage approach involving debridement, allogeneic acellular dermal matrix grafting, and skin grafting using the MEEK technique 5.
- Advanced equipment, such as laser speckle contrast analysis, can be used to assess wound healing progress in EDS patients 5.
Challenges in EDS Management
- EDS is a heterogeneous group of disorders, and management can be challenging due to the varying degrees of manifestation in different organ systems 6.
- No specific medical or genetic therapies are available for any type of EDS, and management consists of a care team responsible for surveillance of major and organ-specific complications 6.