hEDS and knee pain in the adult patient

Ross Hauser, MD

This article will focus on the adult patient with a diagnosis of hEDS (Hypermobile Ehlers-Danlos Syndrome and Hypermobility Spectrum Disorders). Many of these people have suffered from among many joint ailments, a predomination of knee pain, and a history of past knee surgeries.

Please see our companion article Ehlers-Danlos Syndrome and knee replacement complications for more information post knee replacement.

One of the great frustrations in this patient group of older patients is that much of the research and treatment guidelines center on adolescents or considerably younger patients. We will often get an email from an hEDS patient that begins with: “I am not an adolescent. . . ” Yet for many of these people, their problems did begin at this age, and, in the decades since they have been subjected to medical care that did not understand them or what type of treatment would be most beneficial in their particular situation.

Discussion points of this article:

• The stories of some hEDS patients seeking a different treatment.
• The problems of walking creating muscle loss and knee pain in the Hypermobile Ehlers-Danlos Syndrome patient.
• Surgical options explored: The Characteristics of hEDS patients with knee pain that may warrant a surgical recommendation.
• Knee instability after knee replacement.
• Our research Prolotherapy and Ehlers-Danlos Syndrome (hEDS) and Joint Hypermobility Syndrome (Hypermobility Spectrum Disorders (HSD)).

The stories of some hEDS patients seeking a different treatment.

All hEDS patients have their unique story, but many common characteristics. Here are some examples of emails we get:

The dislocating knee cap

I have always been very flexible. This helped me a lot when I participated in youth sports. As I started entering my teen years I started to have terrible knee pain. My parents took me from specialist to specialist, orthopedist to orthopedist, and after many exams, consultations, and long “wait and see,” periods, I finally had knee surgery for what was determined to be my primary problem patellar chondromalacia.

Over the years this did not help with my knee pain, my knees got worse. Not only did I start dislocating my patella, but the constant over-compensation of my knee problems lead to dislocations of my hips. I have tried many treatments, therapies, I actually dislocated myself in physical therapy. My search for treatments continues.

A dozen surgeries 

I am sixty years old. My knee problems have gone on for decades, I have had numerous ligament reconstruction surgeries and now add to that numerous failed total knee replacements, when you add in my latest procedures. My knees are in constant pain. Even with the knee replacements, they want to give out and feel unstable. My doctors want to spend more time on tests and less time on treatments. I can see it already, they are lining me up for a surgery that will make it an even dozen procedures in my lifetime. I need some options.

My meniscus

I cannot even count how many times I have dislocated my patella. I also had four surgeries to remove or repair parts of my meniscus in both knees. This all started when I was a teenager and now I am seventy. Yes, I have had medical treatments on my knees for almost sixty years. I am sometimes amazed that I have only had a handful of surgeries, especially when my doctors are always recommending more. I think not having so many surgeries has helped me stay active and I want to stay that way. I am looking for non-surgical options to save what is left of my meniscus. I know when the meniscus is gone, I have to get a knee replacement on both knees.

Recommendation to double knee replacement

My knees are now bent inward significantly. To say I have “knocked knees” would be an accurate assessment. This is causing me a lot of problems with knee pain, degenerative arthritis, knee instability, and patella malalignment. I can’t stand for any length of time without excruciating pain. When I try to straighten my knee to provide some comfort or “pop” my patella back into place, it hurts all the more. Double knee replacement has been recommended with the understanding that it may not help as much as I and my doctors want it to.

Surgery can help many people

For many people, some in similar situations to our examples above, surgery can be very beneficial and significantly increase the quality of life these people are missing. The people who did or will do well with surgery and not typically the people that we see. We see that people who are still looking for answers well after surgical procedures and other treatments have not offered significant results or even the smallest quality-of-life benefit.

The problems of walking creating muscle loss and knee pain in the Hypermobile Ehlers-Danlos Syndrome patient

A November 2020 study in the journal Arthritis Care and Research (1) examined the relationship between joint and ligament laxity and muscle strength during walking.

• “The objectives of this study were to compare muscle activation, joint angles, and spatiotemporal parameters during gait, and to compare isometric strength between participants with EDS (hypermobility and classical subtypes) and healthy adults.”
  • Quick explanatory point: Spatiotemporal parameters during gait are measurements of the length of the walking stride, the distance between the front and back of the heel strikes, the side or lateral distances between the midlines of the left and right foot.
• Results: The EDS group had delayed vastus lateralis (A powerful thigh muscle and part of the quadriceps femoris that helps move the knee forward) and medialis activation (the medialis helps move the knee and stabilize the knee cap during movement), higher rectus femoris (a muscle of the quadriceps group that assists in the knee and hip flexion – the movement that helps your heel move upwards towards your buttocks) and tensor fascia latae activation (tensor fascia latae is a muscle that is specialized for walking and providing pelvic stability while walking), prolonged gluteus medius activation (the gluteus medius helps stabilize the pelvis when walking) and lower medial gastrocnemius activation (A calf muscle).
  • While Joint angles were similar between the EDS and healthy groups. The EDS group had slower gait speeds, shorter stride lengths, and a greater percentage of time instance (the time between the first contact with the ground and last contact with the ground during one stride). The EDS group had weaker hip and ankle muscles.

The researchers of this study concluded: “Alterations in muscle activation and spatiotemporal parameters during gait in patients with EDS may be a result of impaired proprioception and balance and muscle weakness. ”

Comment: What is being stressed is that during walking motion there is instability. Something is impacting and creating a delayed muscle activation response and the muscles are becoming weaker. We are going to explore this below with a discussion of the supportive ligaments of the pelvis, hips and knees, and a discussion of treatments.

What are we seeing in this image?

Forces across the pelvis with walking: With every step of walking one leg is supporting the weight of the body while the other leg is propelled forward. On the supporting limb side, the force of the weight of the body is transmitted through the sacrum and the sacroiliac joint and is counted by a ground force in a muscle reaction force that elevates the hip on this side while the other side is pulled down by the weight of the hanging limb. This leads to a shearing force in the pubic symphysis which tends to raise the pubic bone on the side of the supporting limb and lower the opposite pubic bone. In patients with pelvic stability, this can cause significant problems with gait.

Surgical options explored: The Characteristics of hEDS patients with knee pain that may warrant a surgical recommendation:

This is a current surgical review of knee abnormalities and surgical guidelines presented by doctors at the University of Southern California (USC)  Epstein Family Center for Sports Medicine, Keck Medicine of USC. It appeared in the journal Clinics in Orthopedic Surgery. (2) Presented below is a summary highlight of this research.

Characteristics of patients with knee pain that may warrant a surgical recommendation:

• In a study of 300 hEDS patients,
  • Patellar instability is found in in 57%, chronic pain in 85% of the patients. These numbers were similar to earlier research which suggested 81.5% of patients with hEDS reported knee pain and 40% reported patellar dislocations.
• Patients with EDS were found to have patella maltracking with a relatively large lateral tilt.
• A previous study found that patients with ACL injury were 4.4 times more likely to have joint hypermobility and a more common association was made between joint hypermobility and female gender, where ACL injury is more common.
• Patellar tendon ruptures can occur frequently in patients with EDS, with most ruptures occurring at the inferior pole of the patella. It is hypothesized that this is a result of tendon weakness in this category of patients and is a primary concern for orthopedic surgeons.

Knee Surgeries:

Patellar stabilization procedures

• Medial patellofemoral ligament (MPFL) reconstruction is a widely accepted procedure to treat recurrent patellar instability in young patients. Little has been reported on the outcomes of this procedure in whether it will help patients with hEDS. A recent study found no difference in clinical outcomes following MPFL reconstruction in patients with joint hypermobility syndrome versus without joint hypermobility syndrome. Therefore, attention must be paid when managing patients with patellofemoral joint instability and hEDS/joint hypermobility syndrome due to the risk of inferior surgical outcomes.

Patellar tendon repair-reconstruction

• Rupture of the patellar tendon might occur in patients with hEDS. These patients often present with the classic signs and symptoms of patellar tendon rupture (knee pain, swelling, and inability to extend the knee joint), but they often report a low-energy mechanism of injury or absence of trauma.
• There is a lack of studies comparing the efficacy of the commonly used patellar tendon repair or reconstruction techniques in patients with versus without connective tissue abnormalities, which would be helpful to determine whether new techniques must be considered for this special patient population.

Reconstruction of the cruciate ligament of the knee

No evidence exists regarding the graft choice and outcomes for cruciate ligament (anterior or posterior) reconstructions in patients with hEDS or other connective tissue disorders. No studies have explored the outcomes of the above procedures in larger groups of patients with EDS or other connective tissue disorders, and therefore, future investigation in this field is warranted.

In this research, surgeons try to help make clear the realistic expectations for patients who undergo surgery. Again for many people, surgery may be the only option and may help them with quality of life.

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 the 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 an 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 from 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. The treatment is explained below.

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

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 and, most importantly, in getting people back to a happy and active lifestyle.

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:

• 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.
• 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 is the treatment of knee instability in patients with and without hEDS

In this video, Ross Hauser, MD discusses Ehlers-Danlos Syndrome and ligament laxity. Explanatory notes and a summary is provided below the video.

The main points from the video as it relates to ligament laxity in EDS 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.

Prolotherapy for Knee Pain

• Prolotherapy can be very helpful in patients with knee instability or hypermobility caused by damaged knee ligaments and tendons. Knee instability is a cause of knee osteoarthritis and degenerative wear and tear.
• In this video, Ross Hauser, MD is seen demonstrating intra-articular (inside the knee) as well as injections surrounding the outside of the knee.
• In addition to knee osteoarthritis, Prolotherapy injections can help patients with problems that will eventually lead to degenerative knee disease.

In this image of a patient receiving Prolotherapy injections into their knee, the dots on the skin represent those areas of the knee that should receive an injection. In this particular patient, there are 50 dots on their knee. Each dot would represent where each injection would be given. 

• Prolotherapy knee cartilage regeneration: Standard Clinical X-ray Studies Document Cartilage Regeneration in Five Degenerated Knees
  • This paper was published in the Journal of Prolotherapy.
  • Hauser R, Cukla J. Standard Clinical X-ray Studies Document Cartilage Regeneration in Five Degenerated Knees After Prolotherapy. Journal of Prolotherapy. 2009;1(1):22-28. [Google Scholar]
• Outcomes of Prolotherapy in Chondromalacia Patella Patients: Improvements in Pain Level and Function
  • This paper was published in the journal Clinical Medicine Insights. Arthritis and Musculoskeletal Disorders.
  • Hauser RA, Sprague IS. Outcomes of prolotherapy in chondromalacia patella patients: improvements in pain level and function. Clin Med Insights Arthritis Musculoskelet Disord. 2014 Feb 17;7:13-20. DOI: 10.4137/CMAMD.S13098. PMID: 24596471; PMCID: PMC3937178. [Google Scholar] [Pubmed]
• Bone Marrow Prolotherapy for Degenerative Joint Disease
  • This paper was published in the journal Clinical Medicine Insights. Arthritis and Musculoskeletal Disorders.
  • Hauser RA, Orlofsky A. Regenerative injection therapy with whole bone marrow aspirate for degenerative joint disease: a case series. Clin Med Insights Arthritis Musculoskelet Disord. 2013 Sep 4;6:65-72. DOI: 10.4137/CMAMD.S10951. PMID: 24046512; PMCID: PMC3771705.  [Google Scholar] [Pubmed]
• A Retrospective Study on Dextrose Prolotherapy for Unresolved Knee Pain at an Outpatient Charity Clinic in Rural Illinois
  • This paper was published in the Journal of Prolotherapy.
  • Hauser RA, Hauser MA. A retrospective study on dextrose Prolotherapy for unresolved knee pain at an outpatient charity clinic in rural Illinois. Journal of Prolotherapy. 2009;1(1):11-21. [Google Scholar]

Recent Prolotherapy knee research

A May 2020 study in the Annals of Family Medicine (3) made this simple statement at the conclusion of the research findings:

“Intra-articular dextrose prolotherapy injections reduced pain, improved function and quality of life in patients with knee osteoarthritis compared with blinded saline injections. The procedure is straightforward and safe; the adherence and satisfaction were high.”

A study from May 2020 published in The Journal of Alternative and Complementary Medicine (4) made these observations: “These findings suggest that dextrose prolotherapy is effective at reducing pain and improving the functional status and quality of life in patients with knee osteoarthritis.

Questions about our treatments?

If you have questions about your knee pain and how we may be able to help you, please contact us and get help and information from our Caring Medical staff.

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References

1 Robbins SM, Cossette‐Levasseur M, Kikuchi K, Sarjeant J, Shiu YG, Azar C, Hazel EM. Neuromuscular activation differences during gait in patients with Ehlers‐Danlos syndrome and healthy adults. Arthritis care & research. 2020 Nov;72(11):1653-62. [Google Scholar]
2 Homere A, Bolia IK, Juhan T, Weber AE, Hatch GF. Surgical Management of Shoulder and Knee Instability in Patients with Ehlers-Danlos Syndrome: Joint Hypermobility Syndrome. Clinics in Orthopedic Surgery. 2020 Sep;12(3):279. [Google Scholar]
3 Shan Sit RW, Keung Wu RW, Rabago D, et al. A Randomized Controlled Trial. Ann Fam Med. 2020;18(3):235‐242. doi:10.1370/afm.2520 [Google Scholar]
4 Sert AT, Sen EI, Esmaeilzadeh S, Ozcan E.J Altern Complement Med. 2020;26(5):409‐417. doi:10.1089/acm.2019.0335 [Google Scholar]

This article was updated March 19, 2021

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