Ehlers-Danlos Syndrome and knee replacement complications

Ross A. Hauser, MD. 

There is not much by way of published research on the problems of knee replacement complications in Ehlers-Danlos Syndrome patients. Below I have listed and summarized the most recent research.

Patients with Ehlers-Danlos Syndrome can have successful knee replacement surgery. However, for some, recovery and complication from knee replacement surgery can also be very challenging. Those who do have successful knee replacement surgery may find that theirs is a short-lived success story. Those with a lesser advanced or milder form of hypermobile Ehlers-Danlos Syndrome hEDS may give good outcome reports. People with more advanced hEDS, noting dozens of knee subluxations or dislocations before the knee replacement, will report lesser success in many cases. The obvious goal of knee replacement in hEDS patients is less pain, less subluxation, more stability. In this article, we will explore knee replacement outcomes, who it may work for and who it may not, and alternatives to the knee replacement that may work for some patients.

Article summary:

• Sometimes a patient will finally be diagnosed with hEDS after they have a knee replacement that fails.
• Following a total knee replacement, concern exists that hypermobility will affect the outcome as the prosthesis relies on soft tissues for stability.
• Two knee replacements, three revision knee replacement surgeries, granuloma.
• “I have finally been diagnosed with Ehlers-Danlos Syndromes and I am just as confused as before.”
• The difficulty in Ehlers-Danlos Syndrome patients being understood is difficulty in getting treatments that can help prevent knee surgery after knee surgery.
• Knee replacement appears to be an effective option for knee arthritis and instability in Ehlers-Danlos Syndrome patients, although results and satisfaction are lower.
• Knee replacement can help hEDS patients who had knee fusion.
• The main points in regard that Ehlers-Danlos Syndrome as a relates to ligament laxity and Prolotherapy injections.
• Our research Prolotherapy and Ehlers-Danlos Syndrome (hEDS) and Joint Hypermobility Syndrome (Hypermobility Spectrum Disorders (HSD)).

Sometimes a patient will finally be diagnosed with hEDS after they have a knee replacement that fails

When people reach out to us, they will often report a long history of knee surgeries. Not just knee replacements, Some surgeries were to repair damaged ligaments, this would include multiple ACL reconstructions. Some surgeries were to fix the original knee replacement surgery. It is in patients like these, especially someone on the younger side, in their 40’s, a history of four, it took five, six, even 10 surgeries for a diagnosis of Ehlers-Danlos syndrome hypermobility type (hEDS) to be finally be suspected at some point during the long surgical history.

Following a total knee replacement, concern exists that hypermobility will affect the outcome as the prosthesis relies on soft tissues for stability.

Someone will get a knee replacement or replacements because, as they they will tell us, “they had no choice.” They had advanced arthritis, extreme knee instability, and they could no longer walk without a walker or cane. Many times these people will only be in the 40s or 50s. Some patients reported that they were fortunate to have hEDS knowledgeable surgeons who knew to spare as much soft tissue as possible to allow the patient a more successful physical therapy following surgery and the ability to keep their knee muscular support stronger.

A key issue, as we will discuss below is the soft tissue of the knee, particularly the ligaments. In February 2019 research from doctors at the Mayo Clinic and published in The Journal of Arthroplasty,(1) noted that following total knee arthroplasty (knee replacement), concern exists that hypermobility will affect the outcome as (the) prosthesis relies on soft tissues for stability.

In this research, 16 patients (20 knees) with Ehlers-Danlos Syndromes (EDS) who had total knee replacement was examined were followed.

Observational notes:

• EDS patients had significantly more surgical interventions prior to total knee replacement (compared to non-EDS patients) and were more likely to require constrained components. (More hardware or the use of bone to hold the surgical repair or hardware, prosthesis in place.)
  • Those of you who had the surgery will recognize constrained components as the hardware alternations that allow for a hinged prosthesis. This type of hardware is especially used in patients who have highly unstable knees with ligaments that will be unable to support aby other types of prostheses.
• Following the knee replacement, 4 patients in the EDS group and 9 patients in the non-EDS control group underwent reoperation.
• EDS patients did not appear to be at higher risk than control patients for secondary knee surgery.
• Although patients with EDS were more likely to require a constrained component, they are not at increased risk of revision or reoperation following total knee replacement in the intermediate-term.

In 2018, surgeons reporting in the BMJ Case Reports,(2) a publication of the British Medical Association, the case of a 53-year-old postmenopausal woman with hypermobile EDS and a history of a partial lateral meniscectomy and right-sided knee pain for over a year. She had lateral osteoarthritis and a valgus (knee bent inward) deformity of 10 degrees.

The Beighton criteria were determined to evaluate the grade of hypermobility and she scored 7 out of 9 and therefore she was classified as ‘generalized hypermobility’. The patient had no other clinical signs of (hEDS) except for the musculoskeletal hyperlaxity.

Two knee replacements, three revision knee replacement surgeries, granuloma (nflammation)

• The patient was initially treated with a varus unloader brace. In June 2011, (at age 44) she revisited the outpatient clinic because of persistent pain and was eventually listed for a total knee replacement.
• Initially, the patient was very satisfied with the knee replacement. So in May 2012, she decided to have her left knee replaced as well. (Patient was 45).
• In October 2012, she revisited the clinic because of instability of the left total knee replacement, which was initially treated by a stabilizing knee brace.
• Due to persistent instability, fluoroscopy-guided knee examination demonstrated significant medial and lateral instability. The decision was made to undergo a revision total knee replacement to a thicker implant. Postoperative satisfaction was again reported as good.
• In October 2014, she presented with the instability of the right total knee replacement. Because of the good result after revision of the left knee, she was listed for a similar procedure in the right knee (January 2015). The replacement was complicated by a granuloma. Granuloma is the immune system’s response to isolate a foreign body (such as a prosthetic device) from the rest of the body. This can cause significant inflammation.
• In June 2017, she came back to the doctor’s office with recurrent progressive instability of the right knee. The left knee was still stable. During the examination, the knee again proved unstable in both varus and valgus stress.
  • A revision to a rotating hinged total knee replacement was considered; however, due to the extensiveness of the operation, the high possibility of an infection, and the young age of the patient, it was agreed that another insert exchange would be attempted.
• The re-revision of the liner was performed in July 2017 and a 20 mm thicker liner was placed
• After 6 weeks, the patient again experienced increased stability; however, she did experience tenderness of the medial collateral ligament which was treated with a hinged knee brace which led to satisfactory function.
• At final follow-up after the last surgery, both knees were stable, on the left side with a 15 mm liner insert and on the right side liner 20 mm insert.

“I have finally been diagnosed with Ehlers-Danlos Syndromes and I am just as confused as before.”

One of the incredible things about Ehlers-Danlos Syndromes is the amount of confusion it can create in some patients and their doctors. A person will contact us having an idea that there are different classifications of Ehlers-Danlos Syndromes but unsure which one impacts them. Once in a while we will hear a patient say that they never got a classification because their primary doctor told them the classifications didn’t matter because there is nothing that can be done about it, there are no effective treatments.

The difficulty in Ehlers-Danlos Syndrome patients being understood is difficulty in getting treatments that can help prevent knee surgery after knee surgery.

When we receive emails from people looking for help, they typically start with a medical history of many years even decades. We get emails that go like this:

“The typical Hypermobile Ehlers-Danlos story.”

During my teenage years, I would fall down a lot and get hurt. I was told that I was just awkward and would grow out of it. When I did not grow out of it, awkwardness became clumsiness and that I should stop being a “drama queen,” people think I am faking it. When my knee pain became worse, the orthopedist I saw during my freshman year of college started to understand that I had something going on.

Later that year I had my first knee surgery. However, it was not for another 20 years, after many other health problems including digestive problems, gynecological problems, hip, neck, and shoulder instability with continued knee pain that I finally was diagnosed with hEDS. I am looking for a more permanent relief than physical therapy, chiropractic manipulations, tapes, and knee braces are providing.

My first knee replacement in my early 30’s

I started developing terrible knee pain when I was young. By the time I was in my early thirties I had to have a knee replacement. After the knee replacement, I continued to have pain issues. I was finally diagnosed with hEDS. I have been trying to manage my hypermobility with good nutrition and proper exercise but I still find myself needing medications. I can feel the obvious degenerative disease going on in all my joints.

Both knees replaced

I had both knees replaced over 10 years ago. The first few years everything was great, I was very happy with the knee replacements. Then a few years back I started to have a more intense type of pain in both knees. One knee started to hurt worse than the other, but they both hurt. It is very similar pain to the type of pain I had before the knee replacements. I am having issues with the instability that is making my walking clumsy and off balance. I have seen many doctors and they don’t know what to do. I had surgery to burn out the nerves in my right knee, with no success.

Knee replacement appears to be an effective option for knee arthritis and instability in Ehlers-Danlos Syndrome patients, although results and satisfaction are lower

The challenges of having a satisfactory knee replacement have been discussed in the medical literature for many years. In 2004 (3) doctors at the Department of Orthopedic Surgery at the Mayo Clinic, Rochester followed ten Ehlers-Danlos Syndrome patients with 12 primary knee replacements. Two patients having both knees replaced. It should be noted that the average age of these ten patients at the time of knee replacement was about 43 years old. The outcomes in these patients were observed for an average of 65 months after surgery.

The reasons these patients had the knee replacement surgery was because they had:

• Tibiofemoral or patellar instability (found in eight of the 12 replacement knees) and arthritis (found in four of the 12 replacement knees).
• The surgeons of this study concluded: (Total Knee Replacement) appears to be an effective option for knee arthritis and instability in EDS patients, although results and satisfaction are lower than that reported for conventional arthroplasty indications.”

Knee replacement can help hEDS patients who had knee fusion

Patients who have had knee fusion understand that they had this procedure because at the time knee replacement was not an option for them. In essence the thigh bone and shin bone are made into one long bone that does not bend or rotate. In 2013 doctors reported (4) on a 46-year-old female patient with Ehlers-Danlos Syndrome had undergone fusion of her right knee 25 years earlier. This markedly affected her quality of life. She underwent a two-stage conversion to a constrained rotating-hinge total knee arthroplasty. She regained a satisfying range of motion and she has a pain free, mobile and stable knee at 42 months follow-up. Conversion of knee fusion to total knee replacement is a demanding procedure that should only be performed by experienced knee surgeons, in selected cases and on highly motivated patients. Complication and revision rates are reportedly very high but global satisfaction is surprisingly good.

So what do we do for people like this? Prolotherapy Treatment for hEDS

In this video, Ross Hauser, MD discusses Ehlers-Danlos Syndrome and ligament laxity. Explanatory notes and a summary is provided below the video. Please see the companion article: Hypermobile Ehlers-Danlos Syndrome and Hypermobility Spectrum Disorders Injection Treatments.

The main points in regard that Ehlers-Danlos Syndrome as a relates to ligament laxity and Prolotherapy

• Ehlers-Danlos Syndrome pain can be caused by joint instability from ligament laxity
• So I look at Ehlers-Danlos Syndrome as a genetic progressive destructive ligament problem and base treatment on that.
• In 99% of EDS patients, they display normal genetics meaning that Ehlers-Danlos Syndrome is a problem with type 3 and type 5 collagen. This means that the type 1 collagen that gives strength to the ligaments is not the main factor in joint stability. If you have the genetic mutation of type 1 collagen that conditions called osteogenesis imperfecta.
• It is true that  5 to 9% of ligaments are made up of type 3 collagen which can be defective in Ehlers-Danlos Syndrome but what gives the strength to the ligaments that hold the joints together is the type 1 collagen and genetically Ehlers-Danlos Syndrome patients have normal genetics as it relates to type 1 collagen so that’s why we see these patients heal well with Prolotherapy

Alternative to surgery

• We know people with Ehlers-Danlos Syndrome may not respond well to surgery. So do, some don’t. We saw one patient who had 14 Orthopedic surgeries on one joint.
• The more loose-jointed a person is the more orthopedic surgery stabilization procedures will not work.

Our research Prolotherapy and Ehlers-Danlos Syndrome (hEDS) and Joint Hypermobility Syndrome (Hypermobility Spectrum Disorders (HSD)).

A recent paper published in the medical journal Disability and Rehabilitation (5) suggests that medical research seems to be stuck in offering good treatments for patients with hEDS related joint pain and degenerative joint disease.

Here are the summary learning points of this paper:

• Ehlers-Danlos Syndrome is a complex, multi-systemic condition that can be difficult to diagnose and poses challenges for healthcare practitioners who engage with Ehlers-Danlos Syndrome patients in holistic care.
• Improved healthcare provider knowledge of Ehlers-Danlos Syndrome is needed, and additional research on the co-occurring diagnoses with EDS may assist in comprehensive pain management for Ehlers-Danlos Syndrome patients.

The standard care treatments evaluated

“Participants with Ehlers-Danlos Syndrome reported the most helpful methods for managing acute pain were:

• opioids, see our article How opioids can increase chronic pain.
• surgical interventions,
• splints and braces,
• heat therapy,
• nerve blocks and
• physical therapy,

while chronic pain was treated most effectively with opioids, heat therapy, splints or braces, and surgical interventions.”

COMMENT:

• With new research of the hazards and side-effects of opioids and other painkiller medications can we really call a lifetime dependency on medications the MOST effective treatment? See our article How opioids can increase chronic pain.
• Surgeries may be risky due to very sensitive inner organs as well as an inclination to sprains and dislocations.
• Nerve blocks may be effective for those with multiple failed treatments or “no other way,” to alleviate their pain.

If we take splints and braces, heat therapy, and physical therapy treatments at face value we find a group of treatments that seek to stabilize a painful joint (hold it together with splints and braces), bring circulation to the joint, (heat therapy), stresses movement (physical therapy) to build up strength in the joint. These are all part of the therapeutic properties of Prolotherapy injections.

We believe Prolotherapy offers great hope to those with symptoms of hypermobility because it is designed to successfully treat the ligament and tendon laxity that accompanies Ehlers-Danlos Syndrome and Joint Hypermobility Syndrome, as well as strengthen the joints in the body.

Prolotherapy is

In this section, we will lay the foundation for the treatment of patients suffering from Ehlers-Danlos Syndromes, specifically Hypermobile Ehlers-Danlos Syndrome and Hypermobility Spectrum Disorders with Prolotherapy injections. The treatment is designed to stimulate connective tissue growth and rebuilding in damaged painful joints where there is collagen deficiency. Patient outcomes are described below.

Prolotherapy is an in-office injection treatment that research and medical studies have shown to be an effective, trustworthy, reliable alternative to surgical and non-effective conservative care treatments. In our opinion, based on extensive research and clinical results, Prolotherapy is superior to many other treatments in relieving the problems of chronic joint and spine pain.

Knee instability after knee replacement

In our office, we see many patients with continued knee instability after knee replacement.

In this video, Ross Hauser, MD explains the problems of post-knee replacement joint instability and how a treatment to repair damaged and weakened ligaments can tighten the knee. This treatment does not address problems of hardware malalignment. 

Summary of this video:

It is very common for us to see patients after knee replacement who have these clicking sounds coming from knee instability. This is not instability from hardware failure. The hardware may be perfectly placed in the knee. It is instability from the outer knee where the surviving ligaments are. I believe that this is why up to one-third of patients continue to have pain after knee replacement. Dr. Hauser performs an ultrasound scan of the patient’s knee. Small, gentle stress on the knee reveals hypermobility. This is the ligaments’ inability to hold the whole knee joint in place. Prolotherapy can be very successful in helping patients who had a knee replacement and still have knee pain. The treatment tightens the whole joint capsule.

Our research Prolotherapy and Ehlers-Danlos Syndrome (hEDS) and Joint Hypermobility Syndrome (Hypermobility Spectrum Disorders (HSD)).

In the Journal of Prolotherapy in 2013. The full research can be downloaded as a PDF file – Treatment of Joint Hypermobility Syndrome, Including Ehlers-Danlos Syndrome, with Hackett-Hemwall Prolotherapy.

Here are our bullet points:

• While traditional medical treatments including education and lifestyle advice, behavior modification, physiotherapy, taping and bracing, exercise prescription, functional rehabilitation, and pain medications offer some symptomatic control, they do little in regard to curbing the progressive debilitating nature of the diseases.
• The excessive joint mobility with its subsequent joint degeneration and multiple joint dislocations can then lead the individual to seek out surgical intervention, which has suboptimal results in the hypermobile patient population versus the normal population. As such, some patients with Ehlers-Danlos Syndrome (hEDS) and Joint Hypermobility Syndrome (HSD) are seeking alternative treatments for their pain, including Prolotherapy.
• Prolotherapy offers great hope for those with symptoms from generalized hypermobility because it is designed to successfully treat the ligament and tissue laxity that accompanies Ehlers-Danlos Syndrome and Joint Hypermobility Syndrome.
• Prolotherapy works by initiating a brief inflammatory response, which causes a reparative cascade to generate new collagen and extracellular matrix giving connective tissue strength and the ability to handle strain and force.
• Prolotherapy has a long history of success treating ligament injuries, including patients with joint hypermobility.
• Studies on Prolotherapy have shown that it eliminates chronic pain even in those patients who have been told by their medical doctor(s) that surgery was the only treatment option for their pain.

Prolotherapy does not work for every patient, some of the rationales for using Prolotherapy for patients with Ehlers-Danlos Syndrome and Joint Hypermobility Syndrome include that it has:

• a high safety record is comprehensive (all or most joints can be treated at each visit),
• is an outpatient procedure,
• is cost-effective (compared to surgery),
• pain relief is often quick,
• and it provides joint stabilization.

Perhaps its greatest asset is the fact that this one treatment modality can handle most of the painful musculoskeletal conditions that occur in individuals with Ehlers-Danlos Syndrome and Joint Hypermobility Syndrome.

If this article has helped you understand your complications of knee replacement and you have more questions, get help and information from our specialists

References

1 Tibbo ME, Wyles CC, Houdek MT, Wilke BK. Outcomes of Primary Total Knee Arthroplasty in Patients With Ehlers-Danlos Syndromes. The Journal of arthroplasty. 2019 Feb 1;34(2):315-8. [Google Scholar]
2 Farid A, Beekhuizen S, van der Lugt J, Rutgers M. Case Report: Knee joint instability after total knee replacement in a patient with Ehlers-Danlos syndrome: the role of insert changes as practical solution. BMJ Case Reports. 2018;2018. [Google Scholar]
3 Arthur K, Caldwell K, Forehand S, Davis K. Pain control methods in use and perceived effectiveness by patients with Ehlers-Danlos syndrome: a descriptive study. Disabil Rehabil. 2016 Jun;38(11):1063-74. [Google Scholar]
4 Cermak K, Baillon B, Tsepelidis D, Vancabeke M. Total knee arthroplasty after former knee fusion in a patient with Ehlers Danlos syndrome. Acta Orthop Belg. 2013 Jun 1;79(3):347-50. [Google Scholar]
5 Rose PS, Johnson CA, Hungerford DS, McFarland EG. Total knee arthroplasty in Ehlers-Danlos syndrome. The Journal of arthroplasty. 2004 Feb 1;19(2):190-6.  [Google Scholar]

This article was updated May 24, 2023

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