Joint surgery for EDS linked to high rates of patient satisfaction
Greater chance of surgical success with established EDS diagnosis: Study
While historically avoided by people with Ehlers-Danlos syndrome (EDS), joint surgery is generally associated with high rates of patient satisfaction and its chance of success is greater when there is an established EDS diagnosis, a study in France has found.
Rate of reoperations among the 69 people with non-vascular EDS ranged from 35.7% for the ankle to 60% for the knee. Vascular EDS is considered the most severe type of the disease, marked also by problems in the blood vessels and in which surgery is generally avoided.
“Surgical procedures may be beneficial in patients with non-vascular EDS and should be proposed with the aim of reducing pain and improving quality of life … after medical therapies have failed, including orthoses, compression garments, and physical therapy,” researchers wrote. Orthoses are external devices designed to fit the body part that’s showing problems and to assist in rehabilitation.
The study, “Functional benefit of joint surgery in patients with non-vascular Ehlers-Danlos syndrome: results of a retrospective study,” was published in the Orphanet Journal of Rare Diseases.
EDS comprises a group of genetic diseases affecting connective tissues, which provide structure to body tissues and organs. Disease symptoms include joint hypermobility, or abnormally mobile joints, and soft and fragile skin that is easily damaged.
Joint hypermobility can lead to dislocations, sprains, chronic joint pain
Joint hypermobility can cause joint instability and muscular problems that subsequently result in dislocations, sprains, muscle and ligament tears, and chronic joint pain.
“There is no cure for EDS and current approaches to treatment include physical therapy to improve joint stability, orthoses or compression garments, pain relief, appropriate treatments for other symptoms and psychological counselling,” the researchers wrote. “Surgical treatment may be an option in specific patients after failure of medical treatments, particularly procedures for joint instability or nerve decompression,” but “to date, there has been no consensus on the optimal surgical management of patients with EDS.”
With this in mind, a team of researchers in France retrospectively analyzed joint surgical procedures and their functional results in adults with non-vascular EDS who were followed at a single French hospital.
From a total of 1,368 patients diagnosed with EDS, 236 (17.3%) underwent a joint surgery, and 69 of them had non-vascular EDS and available follow-up data, and therefore were included in the analysis.
The mean age of the patients at the start of the study was 40.7 years, most of them were women (87%), and the majority were diagnosed with hypermobile EDS (79.7%), the most common type of the disease.
Among all patients, a total of 127 primary surgeries were performed for one or more joints at a mean age of 25.6 years. Most surgeries were performed in the knee (39.4%), followed by the ankle (22%), shoulder (17.3%), wrist (14.2%), and elbow (7.1%).
A total of 89 supplementary surgeries were needed, most commonly in the knee (44.9%).
About one-fifth (20.8%) of all surgeries were performed through arthroscopy, a procedure where the surgeon inserts a narrow tube with a fiber-optic camera to visualize and repair damaged tissues.
Confirmed or possible EDS diagnosis in 33.9% of patients before first surgery
At first surgery, the operation was informed by a confirmed or possible EDS diagnosis in 33.9% of the patients.
“A preoperative diagnosis of EDS was known for only 22% of patients undergoing surgery on the knee or elbow whereas this figure was 59% for patients undergoing shoulder surgery,” the team wrote.
For instance, 50 knees were treated with surgery, mainly due to instability of the patella, one of the bones that come together at the knee joint, and additional surgery was needed for 30 of them.
In most cases, surgery was performed under general anesthesia, although adding spinal anesthesia helped reduce pain. After surgery, “40% of patients reported instability of the knee,” the team wrote.
Surgery was performed in 28 ankles, mainly due to ankle instability (50%), and 10 of these ankles needed more than one intervention. General anesthesia was found to be more effective to ease pain for surgery in these patients.
Despite ankle surgery, nearly two-thirds of these patients “continued to need a mobility aid and among these, six patients walked with one or two crutches and eight always wore an orthosis,” the researchers wrote.
While there was a high rate of shoulder dislocations (72.7%), reintervention rates were generally low for all types of surgeries, ranging from 35.7% for the ankle to 60% for the knee. At least 70% of patients were satisfied with their surgery, except for those who had surgery performed on their dominant shoulder (41.7%).
“Our results show that surgery for joint instability has a greater chance of success when it is carried out in patients where the diagnosis of EDS is known [before surgery],” the researchers wrote. “Although surgery has historically been avoided in patients with EDS and the rates of surgery remain low,” these findings suggest this approach may help non-vascular EDS patients in terms of pain and quality of life.
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