From the Guidelines
Spine surgery in patients with Ehlers-Danlos syndrome poses significant risks, including poor wound healing, tissue fragility, excessive bleeding, and increased risk of postoperative complications, and should be approached with caution. The connective tissue defects in Ehlers-Danlos syndrome make surgical tissues more fragile and prone to tearing, while also impairing the normal healing process, as noted in the guidelines for the diagnosis and management of patients with thoracic aortic disease 1. Key risks associated with spine surgery in these patients include:
- Poor wound healing due to tissue fragility and impaired collagen synthesis
- Increased risk of dural tears and cerebrospinal fluid leaks during surgery
- Excessive bleeding due to fragile blood vessels
- Joint hypermobility causing hardware failure or adjacent segment degeneration
- Increased risk of postoperative complications, such as infection or respiratory issues
- Anesthesia complications, including issues with intubation and medication responses
- More severe postoperative pain requiring specialized pain management approaches
Given these risks, a thorough preoperative evaluation and multidisciplinary approach are essential to minimize complications and ensure the best possible outcomes for patients with Ehlers-Danlos syndrome undergoing spine surgery. This may involve consideration of minimally invasive techniques, careful selection of surgical hardware, and close monitoring of the patient's condition during and after surgery, as well as specialized pain management and rehabilitation strategies 1. Conservative management should be exhausted before considering surgical intervention, and patients should be thoroughly informed of the potential risks and benefits of spine surgery in the context of their underlying condition.
From the Research
Risks of Spine Surgery in Patients with Ehlers-Danlos Syndrome
The risks of spine surgery in patients with Ehlers-Danlos syndrome are significant and varied. Some of the potential complications include:
- Vascular complications, such as major bleeding, torn external iliac artery, avulsed segmental arteries, and abdominal aorta damage 2
- Neuropathic pain attributed to the type of implant used 3
- Hemoperitoneum, which can be life-threatening 3
- Wound infections or disruptions 4
- Instability, pain, or discomfort from hardware 5
- Persistent pain or discomfort 5
- Continued subluxation or dislocation 5
Surgical Outcomes
Surgical outcomes for patients with Ehlers-Danlos syndrome are often worse than for the general population. Studies have shown that:
- Complications occur more frequently in patients with Ehlers-Danlos syndrome than in the average population 5
- Patients with Ehlers-Danlos syndrome may experience a higher rate of post-operative complications, such as bleeding, infection, or instability 4, 3, 2
- The fragility of the patient's connective tissue can increase the risk of complications during and after surgery 3, 2
Pre-Operative Planning
Due to the high risk of complications, it is essential to undertake careful pre-operative planning and to have a multidisciplinary team of clinicians involved in the patient's care. This includes: