Joint Hypermobility Syndrome

What is Joint Hypermobility Syndrome?

Joint range of motion varies greatly among individuals and depends on a large number of factors. Joint hypermobility syndrome (JHS) is a connective tissue disorder in which the joints of the body move beyond what is considered the normal range of motion in most individuals. Patients may complain of pain in the knees, elbows, hips, and fingers. Joint hypermobility (JHM) itself is a very common condition in the U.S., affecting 10-20% of people. It is more common in female children and adolescents. With the proper diagnosis and treatment, patients can modify their activities and prevent future injuries.

Causes/Pathophysiology

The cause of JHS is not well understood, however it does involve the collagen in our connective tissues and seems to run in families. It is important to rule out other connective tissue conditions, such as Ehler’s-Danlos (EDS) or Marfan’s, that may also present with joint hypermobility. There are no biological markers for JHS, which makes it different than these other classified connective tissue disorders.

Signs/Symptoms

JHS can affect multiple body systems. Because of this, patients can exhibit a wide range of symptoms. Some of these symptoms are listed below, however keep in mind that these may not include all of the symptoms a person can experience.

Musculoskeletal complaints:

  • Joint sprains
  • Ligament/tendon injuries
  • Recurrent joint dislocations – especially the thumb, shoulder, hip and ankle. Sometimes this occurs with the patella (kneecap) as well.
  • Poor balance due to poor proprioception

Skin:

  • Hyperextensible skin
  • Easy bruising
  • Stretch marks

Miscellaneous:

  • Chronic pain and fatigue
  • Mood disorders (depression, anxiety, phobias)
  • Chest pain, palpitations
  • Orthostatic symptoms
  • Eye abnormalities (e.g. drooping eyelids)

Diagnosis

The diagnosis of JHS is made using the Beighton 1998 criteria, including the use of the Beighton hypermobility score. It is important to recognize that not all patients with hypermobility have JHS:

  • Beighton score (1 point for each for a maximum of 9)
    • Passive dorsiflexion (towards the back of the hand) of the pinky finger by at least 90 degrees: 1 point per pinky
    • Oppose thumb to the volar (palm side) of the forearm: 1 point per thumb
    • Hyperextend the elbow by at least 10 degrees: 1 point per elbow
    • Hyperextend the knee by at least 10 degrees: 1 point per knee
    • Place the hands flat on the floor without bending the knees: 1 point
  • Beighton criteria (2 major criteria OR 1 major criteria + 2 minor criteria OR 4 minor criteria)
    • Major criteria
      • Beighton score of 4 or greater
      • Joint pain for more than 3 months in at least 4 joints
    • Minor criteria
      • Beighton score of 1, 2, or 3 if patient is older than 50
      • Joint or back pain for longer than 3 months in 1-3 joints; spondylosis, spondylolysis, or spondylolisthesis
      • Dislocation of more than 1 joint (or in 1 joint, more than once)
      • Soft tissue irritation (epicondylitis, tenosynovitis, bursitis) in at least 3 locations
      • Marfanoid body habitus
      • Abnormal skin (stretch marks, hyperextensible, thin skin)
      • Eye abnormalities (drooping eyelids)
      • Varicose veins, hernias, or uterine/rectal prolapse

Treatment

Treatment varies depending on a patient’s specific symptoms. A tailored treatment plan should include referrals for physical and/or occupational therapy as well as referrals to medical specialists as needed. Often times, patients do not require any treatment.

Musculoskeletal symptom treatment options:

  • Physical therapy can provide joint stability and strength, which aid in improved function and injury prevention.
  • Splints and orthotics can improve joint alignment which in turn may provide joint stability.
  • Chronic widespread pain can often be managed with analgesics, NSAIDs, antidepressants, and muscle relaxants. If medications provide no relief to patients, a referral to pain management may be indicated.

By Kristie Thatcher and Mo Mortazavi, MD

References:

Grahame, R., & Hakim, A. (2016, July 7). Joint Hypermobility Syndrome. Retrieved December 14, 2017, from https://www.uptodate.com/contents/joint-hypermobility-syndrome?search=hypermobility&source=search_result&selectedTitle=1~61

Kirk, J. A., Ansell, B. M., & Bywaters, E. G. (1967). The hypermobility syndrome. Musculoskeletal complaints associated with generalized joint hypermobility. Annals of the Rheumatic Diseases, 26(5), 419-425.

Pictures:
http://kidshealth.org/AetnaBetterHealthKentucky/en/parents/az-bjhs.html?WT.ac=ctg

Joint hypermobility managed with Orthoses


http://www.ezhealthmd.com/medical-condition/joint-hypermobility-syndromeehlers-danlos/

What is Joint Hypermobility Syndrome?

Joint range of motion varies greatly among individuals and depends on a large number of factors. Joint hypermobility syndrome (JHS) is a connective tissue disorder in which the joints of the body move beyond what is considered the normal range of motion in most individuals. Patients may complain of pain in the knees, elbows, hips, and fingers. Joint hypermobility (JHM) itself is a very common condition in the U.S., affecting 10-20% of people. It is more common in female children and adolescents. With the proper diagnosis and treatment, patients can modify their activities and prevent future injuries.

Causes/Pathophysiology

The cause of JHS is not well understood, however it does involve the collagen in our connective tissues and seems to run in families. It is important to rule out other connective tissue conditions, such as Ehler’s-Danlos (EDS) or Marfan’s, that may also present with joint hypermobility. There are no biological markers for JHS, which makes it different than these other classified connective tissue disorders.

Signs/Symptoms

JHS can affect multiple body systems. Because of this, patients can exhibit a wide range of symptoms. Some of these symptoms are listed below, however keep in mind that these may not include all of the symptoms a person can experience.

Musculoskeletal complaints:

  • Joint sprains
  • Ligament/tendon injuries
  • Recurrent joint dislocations – especially the thumb, shoulder, hip and ankle. Sometimes this occurs with the patella (kneecap) as well.
  • Poor balance due to poor proprioception

Skin:

  • Hyperextensible skin
  • Easy bruising
  • Stretch marks

Miscellaneous:

  • Chronic pain and fatigue
  • Mood disorders (depression, anxiety, phobias)
  • Chest pain, palpitations
  • Orthostatic symptoms
  • Eye abnormalities (e.g. drooping eyelids)

Diagnosis

The diagnosis of JHS is made using the Beighton 1998 criteria, including the use of the Beighton hypermobility score. It is important to recognize that not all patients with hypermobility have JHS:

  • Beighton score (1 point for each for a maximum of 9)
    • Passive dorsiflexion (towards the back of the hand) of the pinky finger by at least 90 degrees: 1 point per pinky
    • Oppose thumb to the volar (palm side) of the forearm: 1 point per thumb
    • Hyperextend the elbow by at least 10 degrees: 1 point per elbow
    • Hyperextend the knee by at least 10 degrees: 1 point per knee
    • Place the hands flat on the floor without bending the knees: 1 point
  • Beighton criteria (2 major criteria OR 1 major criteria + 2 minor criteria OR 4 minor criteria)
    • Major criteria
      • Beighton score of 4 or greater
      • Joint pain for more than 3 months in at least 4 joints
    • Minor criteria
      • Beighton score of 1, 2, or 3 if patient is older than 50
      • Joint or back pain for longer than 3 months in 1-3 joints; spondylosis, spondylolysis, or spondylolisthesis
      • Dislocation of more than 1 joint (or in 1 joint, more than once)
      • Soft tissue irritation (epicondylitis, tenosynovitis, bursitis) in at least 3 locations
      • Marfanoid body habitus
      • Abnormal skin (stretch marks, hyperextensible, thin skin)
      • Eye abnormalities (drooping eyelids)
      • Varicose veins, hernias, or uterine/rectal prolapse

Treatment

Treatment varies depending on a patient’s specific symptoms. A tailored treatment plan should include referrals for physical and/or occupational therapy as well as referrals to medical specialists as needed. Often times, patients do not require any treatment.

Musculoskeletal symptom treatment options:

  • Physical therapy can provide joint stability and strength, which aid in improved function and injury prevention.
  • Splints and orthotics can improve joint alignment which in turn may provide joint stability.
  • Chronic widespread pain can often be managed with analgesics, NSAIDs, antidepressants, and muscle relaxants. If medications provide no relief to patients, a referral to pain management may be indicated.

By Kristie Thatcher and Mo Mortazavi, MD

References:

Grahame, R., & Hakim, A. (2016, July 7). Joint Hypermobility Syndrome. Retrieved December 14, 2017, from https://www.uptodate.com/contents/joint-hypermobility-syndrome?search=hypermobility&source=search_result&selectedTitle=1~61

Kirk, J. A., Ansell, B. M., & Bywaters, E. G. (1967). The hypermobility syndrome. Musculoskeletal complaints associated with generalized joint hypermobility. Annals of the Rheumatic Diseases, 26(5), 419-425.

Pictures:
http://kidshealth.org/AetnaBetterHealthKentucky/en/parents/az-bjhs.html?WT.ac=ctg

Joint hypermobility managed with Orthoses


http://www.ezhealthmd.com/medical-condition/joint-hypermobility-syndromeehlers-danlos/

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