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Nonprofit

Organization
U.S. Postage
P A I D
Twin Cities, MN
VOLUME 22, NUMBER 1 2013 200 University Ave. E. Permit No. 5388
St. Paul, MN 55101
651-291-2848
www.gillettechildrens.org
CHANGE SERVICE
REQUESTED VOLUME 22, NUMBER 1 2013

Deborah Quanbeck, M.D.

Deborah Quanbeck, M.D., is a A Pediatric Perspective focuses on


KEY INSIGHTS
Joint Hypermobility: Normal
specialized topics in pediatrics, orthopedics,
board-certified pediatric ortho- neurology, neurosurgery and rehabilitation
pedic surgeon. She has extensive medicine.

Variation or Cause for Concern?


experience in treating children ■ Typically, clinicians discover joint
To subscribe or unsubscribe from hypermobility when a child is being
with acute pediatric orthopedic A Pediatric Perspective, please send an
conditions and a wide variety of email to Publications@gillettechildrens.com. evaluated for something else, such as
musculoskeletal disorders. By Deborah Quanbeck, M.D., Kristine Greer, D.P.T., and Katie Wilkins, D.P.T. joint pain or fatigue, flat feet or bunions,
Editor-in-Chief – Steven Koop, M.D. and hip dislocations or subluxations.
Editor – Ellen Shriner
She received her medical degree
Designers – Becky Wright, Kim Goodness
from the University of Minnesota Medical School. Following her Photographers – Anna Bittner, ■ The Beighton scale for assessing joint
orthopedic surgery residency at the University of Minnesota, Paul DeMarchi Joint hypermobility is a condition in which hypermobility is considered the gold
she completed a fellowship in pediatric orthopedic surgery at a person’s joints can be flexed beyond the standard for diagnosis, because it is
Copyright 2013. Gillette Children’s Specialty
Gillette Children’s Specialty Healthcare and Shriners Hospitals Healthcare. All rights reserved. normal range of motion. It affects approx- quick, it is easy to use, and it has high
for Children. She is a member of the Pediatric Orthopaedic imately 10 to 15 percent of children.1 The intra-rater reliability.
Society of North America and the American Academy of Ortho-
diagnosis often appears straightforward—
paedic Surgeons. She has published journal articles and made ■ Nonmusculoskeletal signs and symp-
numerous professional presentations. excessive laxity in finger (Fig. 1), elbow, hip
toms, such as skin hyperelasticity,
and knee joints—and often it is that simple.
To make a referral, call 651-325-2200 or excessive bleeding or bruising,
855-325-2200 (toll-free). If there are no other associated symptoms, prominent veins, or heart murmurs,
joint hypermobility is usually considered Fig. 1 - A classic example of finger joint may point to more serious conditions
Katie Wilkins, D.P.T
benign. hypermobility such as Ehlers-Danlos syndrome.
Katie Wilkins earned a doctorate NEWS & NOTES
Although joint hypermobility alone may not be cause for concern, the condition can ■ Treatment for joint hypermobility
in physical therapy from the
University of Minnesota. She be accompanied by joint pain and more frequent injuries (e.g., dislocations, sprains). depends on the extent of the condi-
Gillette’s Maple Grove Clinic Expands Services tion. Physical therapy may suffice for
gained her first year of experience It also can contribute to arthritis. In some cases, the symptoms signal a more serious
in sports medicine and ortho- Our Maple Grove Clinic has expanded our offering of pediatric specialty services. Patients some patients, while others with more
Visit www.gillettechildrens.org/ living in the northwestern metropolitan area now have convenient access to these specialists: condition, such as Ehlers-Danlos syndrome.
pedics at the Sports Medicine involved conditions should be referred
MedicalStaffBios to learn more about Orthopedics – Deborah Quanbeck, M.D., Angela Drummond, P.N.P.
and Wellness Center in River Gillette’s specialists.
to a pediatric orthopedic surgeon or
Falls, Wis.  She works at Gillette Pediatric rehabilitation medicine – Supreet Deshpande, M.D., Angela Sinner, D.O. When examining a child or adolescent who is unusually flexible or more inclined to pediatric rehabilitation specialist.
Children’s Specialty Healthcare’s Clinical Education Plastic surgery – Paul Kim, M.D. have sprains than most children, joint hypermobility may be the cause, and further
Minnetonka Clinic.  Visit the Resource Center on our website Craniofacial care – Martha McGrory, P.N.P. evaluation is recommended.
to find videos and professional present-
Neurotrauma care – Leslie Larson, P.N.P., Amanda Seeley, P.N.P.
ations. www.gillettechildrens.org
Sleep medicine – Laurel Wills, M.D.
About Joint Hypermobility Inside
Additionally, the Maple Grove Clinic provides comprehensive rehabilitation therapies and
Kristine Greer, D.P.T. assistive technology services. Joint hypermobility varies with age, gender (it is more common in females than ■ Beighton scale for assessing the
Back Issues of males) and race. Often, it is familial. The condition is much more common in infants extent of joint hypermobility, P. 2
Kristine Greer earned a doctor- A Pediatric Perspective and young children than in adults (people tend to become less flexible as they grow
ate in physical therapy from the www.gillettechildrens.org/ ■ Physical Therapy Can Treat and
older), and it occurs in a high percentage of Asian children. Randomly ascertained
University of Minnesota. She pediatricperspective Prevent Joint Hypermobility
data on school-age children found that 50 percent of Chinese-Asian subjects had
began working with adult and Symptoms, P. 3 (inside flap)
pediatric acute care patients at joint hypermobility 2 as opposed to 7 percent of English-Caucasians 3. The average
Mercy Hospital in Coon Rapids, age of onset of symptoms is 6.2 years, but the average age at diagnosis is 9.0 years,
Minn. Shortly thereafter, Kristine indicating a two to three year delay in diagnosis.4
transitioned into full-time
pediatrics at Gillette Children’s
Specialty Healthcare. She has worked at Gillette’s Minnetonka and
Maple Grove clinics.     

w w w. g i l l e t t e c h i l d r e n s . o r g
Diagnosing Joint Hypermobility Kyphoscoliosis, arthrochalsia and dermatosparaxis are rare Physical Therapy Can Address Joint Hypermobility Symptoms
Typically, clinicians discover joint hypermobility when a ■ Beighton Scale types of EDS; classic, hypermobile and vascular EDS are
child is evaluated for something else, such as: considered to be the major types. While classic and hyper- Joint hypermobility syndrome may have these clinical
• Joint pain and/or fatigue mobile EDS primarily affect the skin and musculoskeletal implications:
• Deformities such as flat feet, bunions (See Fig. 2.), clubfeet, Passive dorsiflexion of the system, vascular EDS is of particular concern because of the
Pain – Patients might experience intermittent or activity- Postural Re-education and Joint Protection Principles
hip dislocation or subluxation, and developmental metacarpophalangeal joint potential for arterial, bowel or uterine rupture. Consequently,
related pain, especially among adolescents who are We educate patients about neutral sitting postures, avoid-
dysplasia of the hip (DDH); as many as half of children beyond 90 degrees any patient who has joint hypermobility requires a more
involved in sports and other activities. ing knee and elbow hyperextension while standing or in
with DDH have signs of hypermobility 1 point for each hand thorough assessment to establish or rule out EDS.
Increased fatigue quadruped positions, lifting mechanics, and the proper
• Joint effusions
Fibromyalgia and complex regional pain syndrome way to carry a backpack.
• Injuries such as recurrent sprains or dislocations
Balance – Patients who have excessive joint hyper-
• Developmental delay Passive apposition of the thumb Managing Joint Hypermobility
• Clumsiness mobility often have difficulty with balance and may Education on Stretching
to the flexor aspect of the When a serious underlying genetic diagnosis such as Ehlers- have decreased awareness of a joint’s position during We recommend that patients avoid stretching hyper-
• Gait abnormalities forearm Danlos syndrome is suspected, refer the patient for genetic
movement (impaired proprioception). mobile joints to relieve pain. Instead, we focus on
1 point for each thumb testing, and potentially for assessment of bleeding disorders,
Abnormal autonomic responses – Patients might have strengthening exercises to improve joint stability,
mitral valve prolapse or aortic dilation. For concerns about
Fig. 2 - Bunions postural orthostatic tachycardia syndrome, orthostatic which will eventually relieve patients’ symptoms.
rheumatic conditions, refer the patient to a pediatric rheum-
and flat feet in a atologist. hypotension or orthostatic intolerance.
nine-year-old boy Orthopedic concerns and musculoskeletal injuries – Footwear and Bracing
Hyperextension of the elbows For less involved cases, in which symptoms are primarily Patients might experience: Excessive ankle joint laxity and pes planus are more
beyond 10 degrees musculoskeletal, refer the patient to a pediatric orthopedic Feet – Pes planus common in patients who have joint hypermobility.
1 point for each elbow surgeon, a pediatric rehabilitation medicine specialist or a Ankles – Ankle sprains We may recommend orthoses, footwear, ankle stabilizing
physical therapist—depending on the extent of impairment. Knees – Patellofemoral pain and patellar instability braces and patellar stabilizing braces.
At Gillette, we take an interdisciplinary approach to care and (subluxation/ dislocation)
draw on the appropriate specialists to address each patient’s Hips – Possible hip subluxation (rare to have actual hip Gross Motor Skills
At Gillette Children’s Specialty Healthcare, we use the
Beighton scale to screen for joint hypermobility. The scale chief complaints. For example, a patient who has hip dysplasia dislocation); frequent hip subluxations can result in labral Children who have joint hypermobility syndrome
Hyperextension of the knees
assesses only a few joints and is based on four passive will be assessed and may require orthopedic surgery. However, tears. may have difficulties with coordination or gross
beyond 10 degrees
maneuvers and one active maneuver. Instead of focusing 1 point for each knee another less involved patient may be referred for physical Shoulders – Multidirectional shoulder instability, motor skills, and physical therapists will evaluate them
on the degree of hypermobility at a particular joint, the tool therapy to address pain, strengthening, proprioception, shoulder impingement for delays in gross motor development.
helps clinicians establish how widespread the hypermobility balance and delays in gross motor skills. Spine – Spondylolisthesis, scoliosis, degenerative disc
is in that individual. A score of five or more defines general- disease, thinning of discs, stenosis and herniated discs Lifelong Physical Activity
ized joint hypermobility. Delays in gross motor skills Maintaining an active lifestyle with strengthening
Conclusion exercises can help provide stability to a joint and reduce
Not only is the tool quick and easy to use, but it also Depending on a patient’s needs, our physical therapists painful symptoms. Physical therapists can provide
provides an objective measure based on a nine-point Forward flexion of the trunk with Patients who present with symptoms of joint hypermobility
should have a thorough assessment to determine the extent will develop a plan that incorporates the following instruction on joint protection principles that can help
scale, and it has high intra-rater reliability. As a result, knees fully extended so that the
of the condition and establish whether joint hypermobility is principles and includes some or all of these treatments. minimize joint overuse during physical activity. 
the Beighton scale is considered the gold standard for joint palms of the hands rest flat on
hypermobility assessment.5 the floor benign or symptomatic of a serious disorder such as EDS.
1 point Strengthening Exercises
Once the extent of joint hypermobility is established, Patients who have joint hypermobility inherently have
clinicians should assess nonmusculoskeletal factors,
1
http://www.medicinenet.com/hypermobility_syndrome/article.htm low muscular stiffness and joint instability. Gillette’s
including: 2
Cheng J and Chan P. Joint laxity in children. J Pediatr Orthop. 1991, 11:752-756. physical therapists focus on strengthening proximal
• Skin hyperelasticity, which results from defective collagen Evidence of the nonmusculoskeletal signs and symptoms 3
Carter C and Wilkinson J. Persistent joint laxity and congenital dislocation
muscle groups (scapular stabilizers and trunk/core
molecules in the skin mentioned at left is worrisome, because it points to more of the hip. J Bone Joint Surg Br. 1964, 40:40-45. muscles) to help improve joint stability. Trunk and core
• Doughy or fragile skin, which tears easily or shows serious conditions such as Ehlers-Danlos syndrome, 4
Adib, N., K. Davies, R. Grahame, and KJ Woo. “Joint hypermobility syndrome stabilization exercises also help children learn to keep
excessive scarring in childhood. A not so benign multisystem disorder.” Rheumatology (Oxford)
osteogenesis imperfecta, Marfan syndrome and Larsen 44 (2005): 744-50. their joints in the neutral and protected range.
• Excessive bleeding or bruising syndrome. 5
Byers, P. H., M. Duvic, M. Atkinson, M. Robinow, L.T. Smith, S.M. Krane, M.T.
• Physical education difficulties Greally, M. Ludman, R. Matalon, S. Pauker, D. Quanbeck, U. Schwarze. “Ehlers- Proprioception and Balance Training
• Hernias Danlos Syndrome Type VIIA and VIIB Results from Splice-Junction Mutations
About Ehlers-Danlos Syndrome or Genomic Deletions that Involves Exon 6 in the COL1A1 and COL1A2 Genes of Closed chain exercises enhance proprioceptive feedback
• Gingival recession Type 1 Collagen” Abstract. American Journal of Medicine Genetics (1997)
and assist with control of the joint. Open chain multi-
• Prominent veins, hemorrhoids, or early-onset varicose Joint hypermobility is one of the hallmarks of Ehlers-Danlos Oct 3; 72(1):94-105
syndrome (EDS), an inherited connective tissue disorder. 6 directional exercises may be added in later stages of
veins Beighton, Peter, Anne De Paepe, Beat Steinmann, and Petros Tsipouras. “Ehlers-
Hyperelastic skin and fragile tissue are also associated with Danlos syndrome nosology.” Ehlers-Danlos National Foundation. 30 Jan. 2008 rehabilitation, if the joints are stable. Static and dynamic
• Blue sclera <http://www.ednf.org/index.>. Reprinted from the American Journal of Medical
• Heart murmurs EDS. According to the Villefranche nosology, the syndrome is Genetics in the August 1998 issue of Loose Connections. balance training helps improve joint stability during
• Family history classified into six main types: classic, hypermobile, vascular, functional and recreational activities.
kyphoscoliosis, arthrochalsia and dermatosparaxis.6

2 3
Diagnosing Joint Hypermobility Kyphoscoliosis, arthrochalsia and dermatosparaxis are rare Physical Therapy Can Address Joint Hypermobility Symptoms
Typically, clinicians discover joint hypermobility when a ■ Beighton Scale types of EDS; classic, hypermobile and vascular EDS are
child is evaluated for something else, such as: considered to be the major types. While classic and hyper- Joint hypermobility syndrome may have these clinical
• Joint pain and/or fatigue mobile EDS primarily affect the skin and musculoskeletal implications:
• Deformities such as flat feet, bunions (See Fig. 2.), clubfeet, Passive dorsiflexion of the system, vascular EDS is of particular concern because of the
Pain – Patients might experience intermittent or activity- Postural Re-education and Joint Protection Principles
hip dislocation or subluxation, and developmental metacarpophalangeal joint potential for arterial, bowel or uterine rupture. Consequently,
related pain, especially among adolescents who are We educate patients about neutral sitting postures, avoid-
dysplasia of the hip (DDH); as many as half of children beyond 90 degrees any patient who has joint hypermobility requires a more
involved in sports and other activities. ing knee and elbow hyperextension while standing or in
with DDH have signs of hypermobility 1 point for each hand thorough assessment to establish or rule out EDS.
Increased fatigue quadruped positions, lifting mechanics, and the proper
• Joint effusions
Fibromyalgia and complex regional pain syndrome way to carry a backpack.
• Injuries such as recurrent sprains or dislocations
Balance – Patients who have excessive joint hyper-
• Developmental delay Passive apposition of the thumb Managing Joint Hypermobility
• Clumsiness mobility often have difficulty with balance and may Education on Stretching
to the flexor aspect of the When a serious underlying genetic diagnosis such as Ehlers- have decreased awareness of a joint’s position during We recommend that patients avoid stretching hyper-
• Gait abnormalities forearm Danlos syndrome is suspected, refer the patient for genetic
movement (impaired proprioception). mobile joints to relieve pain. Instead, we focus on
1 point for each thumb testing, and potentially for assessment of bleeding disorders,
Abnormal autonomic responses – Patients might have strengthening exercises to improve joint stability,
mitral valve prolapse or aortic dilation. For concerns about
Fig. 2 - Bunions postural orthostatic tachycardia syndrome, orthostatic which will eventually relieve patients’ symptoms.
rheumatic conditions, refer the patient to a pediatric rheum-
and flat feet in a atologist. hypotension or orthostatic intolerance.
nine-year-old boy Orthopedic concerns and musculoskeletal injuries – Footwear and Bracing
Hyperextension of the elbows For less involved cases, in which symptoms are primarily Patients might experience: Excessive ankle joint laxity and pes planus are more
beyond 10 degrees musculoskeletal, refer the patient to a pediatric orthopedic Feet – Pes planus common in patients who have joint hypermobility.
1 point for each elbow surgeon, a pediatric rehabilitation medicine specialist or a Ankles – Ankle sprains We may recommend orthoses, footwear, ankle stabilizing
physical therapist—depending on the extent of impairment. Knees – Patellofemoral pain and patellar instability braces and patellar stabilizing braces.
At Gillette, we take an interdisciplinary approach to care and (subluxation/ dislocation)
draw on the appropriate specialists to address each patient’s Hips – Possible hip subluxation (rare to have actual hip Gross Motor Skills
At Gillette Children’s Specialty Healthcare, we use the
Beighton scale to screen for joint hypermobility. The scale chief complaints. For example, a patient who has hip dysplasia dislocation); frequent hip subluxations can result in labral Children who have joint hypermobility syndrome
Hyperextension of the knees
assesses only a few joints and is based on four passive will be assessed and may require orthopedic surgery. However, tears. may have difficulties with coordination or gross
beyond 10 degrees
maneuvers and one active maneuver. Instead of focusing 1 point for each knee another less involved patient may be referred for physical Shoulders – Multidirectional shoulder instability, motor skills, and physical therapists will evaluate them
on the degree of hypermobility at a particular joint, the tool therapy to address pain, strengthening, proprioception, shoulder impingement for delays in gross motor development.
helps clinicians establish how widespread the hypermobility balance and delays in gross motor skills. Spine – Spondylolisthesis, scoliosis, degenerative disc
is in that individual. A score of five or more defines general- disease, thinning of discs, stenosis and herniated discs Lifelong Physical Activity
ized joint hypermobility. Delays in gross motor skills Maintaining an active lifestyle with strengthening
Conclusion exercises can help provide stability to a joint and reduce
Not only is the tool quick and easy to use, but it also Depending on a patient’s needs, our physical therapists painful symptoms. Physical therapists can provide
provides an objective measure based on a nine-point Forward flexion of the trunk with Patients who present with symptoms of joint hypermobility
should have a thorough assessment to determine the extent will develop a plan that incorporates the following instruction on joint protection principles that can help
scale, and it has high intra-rater reliability. As a result, knees fully extended so that the
of the condition and establish whether joint hypermobility is principles and includes some or all of these treatments. minimize joint overuse during physical activity. 
the Beighton scale is considered the gold standard for joint palms of the hands rest flat on
hypermobility assessment.5 the floor benign or symptomatic of a serious disorder such as EDS.
1 point Strengthening Exercises
Once the extent of joint hypermobility is established, Patients who have joint hypermobility inherently have
clinicians should assess nonmusculoskeletal factors,
1
http://www.medicinenet.com/hypermobility_syndrome/article.htm low muscular stiffness and joint instability. Gillette’s
including: 2
Cheng J and Chan P. Joint laxity in children. J Pediatr Orthop. 1991, 11:752-756. physical therapists focus on strengthening proximal
• Skin hyperelasticity, which results from defective collagen Evidence of the nonmusculoskeletal signs and symptoms 3
Carter C and Wilkinson J. Persistent joint laxity and congenital dislocation
muscle groups (scapular stabilizers and trunk/core
molecules in the skin mentioned at left is worrisome, because it points to more of the hip. J Bone Joint Surg Br. 1964, 40:40-45. muscles) to help improve joint stability. Trunk and core
• Doughy or fragile skin, which tears easily or shows serious conditions such as Ehlers-Danlos syndrome, 4
Adib, N., K. Davies, R. Grahame, and KJ Woo. “Joint hypermobility syndrome stabilization exercises also help children learn to keep
excessive scarring in childhood. A not so benign multisystem disorder.” Rheumatology (Oxford)
osteogenesis imperfecta, Marfan syndrome and Larsen 44 (2005): 744-50. their joints in the neutral and protected range.
• Excessive bleeding or bruising syndrome. 5
Byers, P. H., M. Duvic, M. Atkinson, M. Robinow, L.T. Smith, S.M. Krane, M.T.
• Physical education difficulties Greally, M. Ludman, R. Matalon, S. Pauker, D. Quanbeck, U. Schwarze. “Ehlers- Proprioception and Balance Training
• Hernias Danlos Syndrome Type VIIA and VIIB Results from Splice-Junction Mutations
About Ehlers-Danlos Syndrome or Genomic Deletions that Involves Exon 6 in the COL1A1 and COL1A2 Genes of Closed chain exercises enhance proprioceptive feedback
• Gingival recession Type 1 Collagen” Abstract. American Journal of Medicine Genetics (1997)
and assist with control of the joint. Open chain multi-
• Prominent veins, hemorrhoids, or early-onset varicose Joint hypermobility is one of the hallmarks of Ehlers-Danlos Oct 3; 72(1):94-105
syndrome (EDS), an inherited connective tissue disorder. 6 directional exercises may be added in later stages of
veins Beighton, Peter, Anne De Paepe, Beat Steinmann, and Petros Tsipouras. “Ehlers-
Hyperelastic skin and fragile tissue are also associated with Danlos syndrome nosology.” Ehlers-Danlos National Foundation. 30 Jan. 2008 rehabilitation, if the joints are stable. Static and dynamic
• Blue sclera <http://www.ednf.org/index.>. Reprinted from the American Journal of Medical
• Heart murmurs EDS. According to the Villefranche nosology, the syndrome is Genetics in the August 1998 issue of Loose Connections. balance training helps improve joint stability during
• Family history classified into six main types: classic, hypermobile, vascular, functional and recreational activities.
kyphoscoliosis, arthrochalsia and dermatosparaxis.6

2 3
Diagnosing Joint Hypermobility Kyphoscoliosis, arthrochalsia and dermatosparaxis are rare Physical Therapy Can Address Joint Hypermobility Symptoms
Typically, clinicians discover joint hypermobility when a ■ Beighton Scale types of EDS; classic, hypermobile and vascular EDS are
child is evaluated for something else, such as: considered to be the major types. While classic and hyper- Joint hypermobility syndrome may have these clinical
• Joint pain and/or fatigue mobile EDS primarily affect the skin and musculoskeletal implications:
• Deformities such as flat feet, bunions (See Fig. 2.), clubfeet, Passive dorsiflexion of the system, vascular EDS is of particular concern because of the
Pain – Patients might experience intermittent or activity- Postural Re-education and Joint Protection Principles
hip dislocation or subluxation, and developmental metacarpophalangeal joint potential for arterial, bowel or uterine rupture. Consequently,
related pain, especially among adolescents who are We educate patients about neutral sitting postures, avoid-
dysplasia of the hip (DDH); as many as half of children beyond 90 degrees any patient who has joint hypermobility requires a more
involved in sports and other activities. ing knee and elbow hyperextension while standing or in
with DDH have signs of hypermobility 1 point for each hand thorough assessment to establish or rule out EDS.
Increased fatigue quadruped positions, lifting mechanics, and the proper
• Joint effusions
Fibromyalgia and complex regional pain syndrome way to carry a backpack.
• Injuries such as recurrent sprains or dislocations
Balance – Patients who have excessive joint hyper-
• Developmental delay Passive apposition of the thumb Managing Joint Hypermobility
• Clumsiness mobility often have difficulty with balance and may Education on Stretching
to the flexor aspect of the When a serious underlying genetic diagnosis such as Ehlers- have decreased awareness of a joint’s position during We recommend that patients avoid stretching hyper-
• Gait abnormalities forearm Danlos syndrome is suspected, refer the patient for genetic
movement (impaired proprioception). mobile joints to relieve pain. Instead, we focus on
1 point for each thumb testing, and potentially for assessment of bleeding disorders,
Abnormal autonomic responses – Patients might have strengthening exercises to improve joint stability,
mitral valve prolapse or aortic dilation. For concerns about
Fig. 2 - Bunions postural orthostatic tachycardia syndrome, orthostatic which will eventually relieve patients’ symptoms.
rheumatic conditions, refer the patient to a pediatric rheum-
and flat feet in a atologist. hypotension or orthostatic intolerance.
nine-year-old boy Orthopedic concerns and musculoskeletal injuries – Footwear and Bracing
Hyperextension of the elbows For less involved cases, in which symptoms are primarily Patients might experience: Excessive ankle joint laxity and pes planus are more
beyond 10 degrees musculoskeletal, refer the patient to a pediatric orthopedic Feet – Pes planus common in patients who have joint hypermobility.
1 point for each elbow surgeon, a pediatric rehabilitation medicine specialist or a Ankles – Ankle sprains We may recommend orthoses, footwear, ankle stabilizing
physical therapist—depending on the extent of impairment. Knees – Patellofemoral pain and patellar instability braces and patellar stabilizing braces.
At Gillette, we take an interdisciplinary approach to care and (subluxation/ dislocation)
draw on the appropriate specialists to address each patient’s Hips – Possible hip subluxation (rare to have actual hip Gross Motor Skills
At Gillette Children’s Specialty Healthcare, we use the
Beighton scale to screen for joint hypermobility. The scale chief complaints. For example, a patient who has hip dysplasia dislocation); frequent hip subluxations can result in labral Children who have joint hypermobility syndrome
Hyperextension of the knees
assesses only a few joints and is based on four passive will be assessed and may require orthopedic surgery. However, tears. may have difficulties with coordination or gross
beyond 10 degrees
maneuvers and one active maneuver. Instead of focusing 1 point for each knee another less involved patient may be referred for physical Shoulders – Multidirectional shoulder instability, motor skills, and physical therapists will evaluate them
on the degree of hypermobility at a particular joint, the tool therapy to address pain, strengthening, proprioception, shoulder impingement for delays in gross motor development.
helps clinicians establish how widespread the hypermobility balance and delays in gross motor skills. Spine – Spondylolisthesis, scoliosis, degenerative disc
is in that individual. A score of five or more defines general- disease, thinning of discs, stenosis and herniated discs Lifelong Physical Activity
ized joint hypermobility. Delays in gross motor skills Maintaining an active lifestyle with strengthening
Conclusion exercises can help provide stability to a joint and reduce
Not only is the tool quick and easy to use, but it also Depending on a patient’s needs, our physical therapists painful symptoms. Physical therapists can provide
provides an objective measure based on a nine-point Forward flexion of the trunk with Patients who present with symptoms of joint hypermobility
should have a thorough assessment to determine the extent will develop a plan that incorporates the following instruction on joint protection principles that can help
scale, and it has high intra-rater reliability. As a result, knees fully extended so that the
of the condition and establish whether joint hypermobility is principles and includes some or all of these treatments. minimize joint overuse during physical activity. 
the Beighton scale is considered the gold standard for joint palms of the hands rest flat on
hypermobility assessment.5 the floor benign or symptomatic of a serious disorder such as EDS.
1 point Strengthening Exercises
Once the extent of joint hypermobility is established, Patients who have joint hypermobility inherently have
clinicians should assess nonmusculoskeletal factors,
1
http://www.medicinenet.com/hypermobility_syndrome/article.htm low muscular stiffness and joint instability. Gillette’s
including: 2
Cheng J and Chan P. Joint laxity in children. J Pediatr Orthop. 1991, 11:752-756. physical therapists focus on strengthening proximal
• Skin hyperelasticity, which results from defective collagen Evidence of the nonmusculoskeletal signs and symptoms 3
Carter C and Wilkinson J. Persistent joint laxity and congenital dislocation
muscle groups (scapular stabilizers and trunk/core
molecules in the skin mentioned at left is worrisome, because it points to more of the hip. J Bone Joint Surg Br. 1964, 40:40-45. muscles) to help improve joint stability. Trunk and core
• Doughy or fragile skin, which tears easily or shows serious conditions such as Ehlers-Danlos syndrome, 4
Adib, N., K. Davies, R. Grahame, and KJ Woo. “Joint hypermobility syndrome stabilization exercises also help children learn to keep
excessive scarring in childhood. A not so benign multisystem disorder.” Rheumatology (Oxford)
osteogenesis imperfecta, Marfan syndrome and Larsen 44 (2005): 744-50. their joints in the neutral and protected range.
• Excessive bleeding or bruising syndrome. 5
Byers, P. H., M. Duvic, M. Atkinson, M. Robinow, L.T. Smith, S.M. Krane, M.T.
• Physical education difficulties Greally, M. Ludman, R. Matalon, S. Pauker, D. Quanbeck, U. Schwarze. “Ehlers- Proprioception and Balance Training
• Hernias Danlos Syndrome Type VIIA and VIIB Results from Splice-Junction Mutations
About Ehlers-Danlos Syndrome or Genomic Deletions that Involves Exon 6 in the COL1A1 and COL1A2 Genes of Closed chain exercises enhance proprioceptive feedback
• Gingival recession Type 1 Collagen” Abstract. American Journal of Medicine Genetics (1997)
and assist with control of the joint. Open chain multi-
• Prominent veins, hemorrhoids, or early-onset varicose Joint hypermobility is one of the hallmarks of Ehlers-Danlos Oct 3; 72(1):94-105
syndrome (EDS), an inherited connective tissue disorder. 6 directional exercises may be added in later stages of
veins Beighton, Peter, Anne De Paepe, Beat Steinmann, and Petros Tsipouras. “Ehlers-
Hyperelastic skin and fragile tissue are also associated with Danlos syndrome nosology.” Ehlers-Danlos National Foundation. 30 Jan. 2008 rehabilitation, if the joints are stable. Static and dynamic
• Blue sclera <http://www.ednf.org/index.>. Reprinted from the American Journal of Medical
• Heart murmurs EDS. According to the Villefranche nosology, the syndrome is Genetics in the August 1998 issue of Loose Connections. balance training helps improve joint stability during
• Family history classified into six main types: classic, hypermobile, vascular, functional and recreational activities.
kyphoscoliosis, arthrochalsia and dermatosparaxis.6

2 3
Nonprofit
Organization
U.S. Postage
P A I D
Twin Cities, MN
VOLUME 22, NUMBER 1 2013 200 University Ave. E. Permit No. 5388
St. Paul, MN 55101
651-291-2848
www.gillettechildrens.org
CHANGE SERVICE
REQUESTED VOLUME 22, NUMBER 1 2013

Deborah Quanbeck, M.D.

Deborah Quanbeck, M.D., is a A Pediatric Perspective focuses on


KEY INSIGHTS
Joint Hypermobility: Normal
specialized topics in pediatrics, orthopedics,
board-certified pediatric ortho- neurology, neurosurgery and rehabilitation
pedic surgeon. She has extensive medicine.

Variation or Cause for Concern?


experience in treating children ■ Typically, clinicians discover joint
To subscribe or unsubscribe from hypermobility when a child is being
with acute pediatric orthopedic A Pediatric Perspective, please send an
conditions and a wide variety of email to Publications@gillettechildrens.com. evaluated for something else, such as
musculoskeletal disorders. By Deborah Quanbeck, M.D., Kristine Greer, D.P.T., and Katie Wilkins, D.P.T. joint pain or fatigue, flat feet or bunions,
Editor-in-Chief – Steven Koop, M.D. and hip dislocations or subluxations.
Editor – Ellen Shriner
She received her medical degree
Designers – Becky Wright, Kim Goodness
from the University of Minnesota Medical School. Following her Photographers – Anna Bittner, ■ The Beighton scale for assessing joint
orthopedic surgery residency at the University of Minnesota, Paul DeMarchi Joint hypermobility is a condition in which hypermobility is considered the gold
she completed a fellowship in pediatric orthopedic surgery at a person’s joints can be flexed beyond the standard for diagnosis, because it is
Copyright 2013. Gillette Children’s Specialty
Gillette Children’s Specialty Healthcare and Shriners Hospitals Healthcare. All rights reserved. normal range of motion. It affects approx- quick, it is easy to use, and it has high
for Children. She is a member of the Pediatric Orthopaedic imately 10 to 15 percent of children.1 The intra-rater reliability.
Society of North America and the American Academy of Ortho-
diagnosis often appears straightforward—
paedic Surgeons. She has published journal articles and made ■ Nonmusculoskeletal signs and symp-
numerous professional presentations. excessive laxity in finger (Fig. 1), elbow, hip
toms, such as skin hyperelasticity,
and knee joints—and often it is that simple.
To make a referral, call 651-325-2200 or excessive bleeding or bruising,
855-325-2200 (toll-free). If there are no other associated symptoms, prominent veins, or heart murmurs,
joint hypermobility is usually considered Fig. 1 - A classic example of finger joint may point to more serious conditions
Katie Wilkins, D.P.T
benign. hypermobility such as Ehlers-Danlos syndrome.
Katie Wilkins earned a doctorate NEWS & NOTES
Although joint hypermobility alone may not be cause for concern, the condition can ■ Treatment for joint hypermobility
in physical therapy from the
University of Minnesota. She be accompanied by joint pain and more frequent injuries (e.g., dislocations, sprains). depends on the extent of the condi-
Gillette’s Maple Grove Clinic Expands Services tion. Physical therapy may suffice for
gained her first year of experience It also can contribute to arthritis. In some cases, the symptoms signal a more serious
in sports medicine and ortho- Our Maple Grove Clinic has expanded our offering of pediatric specialty services. Patients some patients, while others with more
Visit www.gillettechildrens.org/ living in the northwestern metropolitan area now have convenient access to these specialists: condition, such as Ehlers-Danlos syndrome.
pedics at the Sports Medicine involved conditions should be referred
MedicalStaffBios to learn more about Orthopedics – Deborah Quanbeck, M.D., Angela Drummond, P.N.P.
and Wellness Center in River Gillette’s specialists.
to a pediatric orthopedic surgeon or
Falls, Wis.  She works at Gillette Pediatric rehabilitation medicine – Supreet Deshpande, M.D., Angela Sinner, D.O. When examining a child or adolescent who is unusually flexible or more inclined to pediatric rehabilitation specialist.
Children’s Specialty Healthcare’s Clinical Education Plastic surgery – Paul Kim, M.D. have sprains than most children, joint hypermobility may be the cause, and further
Minnetonka Clinic.  Visit the Resource Center on our website Craniofacial care – Martha McGrory, P.N.P. evaluation is recommended.
to find videos and professional present-
Neurotrauma care – Leslie Larson, P.N.P., Amanda Seeley, P.N.P.
ations. www.gillettechildrens.org
Sleep medicine – Laurel Wills, M.D.
About Joint Hypermobility Inside
Additionally, the Maple Grove Clinic provides comprehensive rehabilitation therapies and
Kristine Greer, D.P.T. assistive technology services. Joint hypermobility varies with age, gender (it is more common in females than ■ Beighton scale for assessing the
Back Issues of males) and race. Often, it is familial. The condition is much more common in infants extent of joint hypermobility, P. 2
Kristine Greer earned a doctor- A Pediatric Perspective and young children than in adults (people tend to become less flexible as they grow
ate in physical therapy from the www.gillettechildrens.org/ ■ Physical Therapy Can Treat and
older), and it occurs in a high percentage of Asian children. Randomly ascertained
University of Minnesota. She pediatricperspective Prevent Joint Hypermobility
data on school-age children found that 50 percent of Chinese-Asian subjects had
began working with adult and Symptoms, P. 3 (inside flap)
pediatric acute care patients at joint hypermobility 2 as opposed to 7 percent of English-Caucasians 3. The average
Mercy Hospital in Coon Rapids, age of onset of symptoms is 6.2 years, but the average age at diagnosis is 9.0 years,
Minn. Shortly thereafter, Kristine indicating a two to three year delay in diagnosis.4
transitioned into full-time
pediatrics at Gillette Children’s
Specialty Healthcare. She has worked at Gillette’s Minnetonka and
Maple Grove clinics.     

w w w. g i l l e t t e c h i l d r e n s . o r g
Nonprofit
Organization
U.S. Postage
P A I D
Twin Cities, MN
VOLUME 22, NUMBER 1 2013 200 University Ave. E. Permit No. 5388
St. Paul, MN 55101
651-291-2848
www.gillettechildrens.org
CHANGE SERVICE
REQUESTED VOLUME 22, NUMBER 1 2013

Deborah Quanbeck, M.D.

Deborah Quanbeck, M.D., is a A Pediatric Perspective focuses on


KEY INSIGHTS
Joint Hypermobility: Normal
specialized topics in pediatrics, orthopedics,
board-certified pediatric ortho- neurology, neurosurgery and rehabilitation
pedic surgeon. She has extensive medicine.

Variation or Cause for Concern?


experience in treating children ■ Typically, clinicians discover joint
To subscribe or unsubscribe from hypermobility when a child is being
with acute pediatric orthopedic A Pediatric Perspective, please send an
conditions and a wide variety of email to Publications@gillettechildrens.com. evaluated for something else, such as
musculoskeletal disorders. By Deborah Quanbeck, M.D., Kristine Greer, D.P.T., and Katie Wilkins, D.P.T. joint pain or fatigue, flat feet or bunions,
Editor-in-Chief – Steven Koop, M.D. and hip dislocations or subluxations.
Editor – Ellen Shriner
She received her medical degree
Designers – Becky Wright, Kim Goodness
from the University of Minnesota Medical School. Following her Photographers – Anna Bittner, ■ The Beighton scale for assessing joint
orthopedic surgery residency at the University of Minnesota, Paul DeMarchi Joint hypermobility is a condition in which hypermobility is considered the gold
she completed a fellowship in pediatric orthopedic surgery at a person’s joints can be flexed beyond the standard for diagnosis, because it is
Copyright 2013. Gillette Children’s Specialty
Gillette Children’s Specialty Healthcare and Shriners Hospitals Healthcare. All rights reserved. normal range of motion. It affects approx- quick, it is easy to use, and it has high
for Children. She is a member of the Pediatric Orthopaedic imately 10 to 15 percent of children.1 The intra-rater reliability.
Society of North America and the American Academy of Ortho-
diagnosis often appears straightforward—
paedic Surgeons. She has published journal articles and made ■ Nonmusculoskeletal signs and symp-
numerous professional presentations. excessive laxity in finger (Fig. 1), elbow, hip
toms, such as skin hyperelasticity,
and knee joints—and often it is that simple.
To make a referral, call 651-325-2200 or excessive bleeding or bruising,
855-325-2200 (toll-free). If there are no other associated symptoms, prominent veins, or heart murmurs,
joint hypermobility is usually considered Fig. 1 - A classic example of finger joint may point to more serious conditions
Katie Wilkins, D.P.T
benign. hypermobility such as Ehlers-Danlos syndrome.
Katie Wilkins earned a doctorate NEWS & NOTES
Although joint hypermobility alone may not be cause for concern, the condition can ■ Treatment for joint hypermobility
in physical therapy from the
University of Minnesota. She be accompanied by joint pain and more frequent injuries (e.g., dislocations, sprains). depends on the extent of the condi-
Gillette’s Maple Grove Clinic Expands Services tion. Physical therapy may suffice for
gained her first year of experience It also can contribute to arthritis. In some cases, the symptoms signal a more serious
in sports medicine and ortho- Our Maple Grove Clinic has expanded our offering of pediatric specialty services. Patients some patients, while others with more
Visit www.gillettechildrens.org/ living in the northwestern metropolitan area now have convenient access to these specialists: condition, such as Ehlers-Danlos syndrome.
pedics at the Sports Medicine involved conditions should be referred
MedicalStaffBios to learn more about Orthopedics – Deborah Quanbeck, M.D., Angela Drummond, P.N.P.
and Wellness Center in River Gillette’s specialists.
to a pediatric orthopedic surgeon or
Falls, Wis.  She works at Gillette Pediatric rehabilitation medicine – Supreet Deshpande, M.D., Angela Sinner, D.O. When examining a child or adolescent who is unusually flexible or more inclined to pediatric rehabilitation specialist.
Children’s Specialty Healthcare’s Clinical Education Plastic surgery – Paul Kim, M.D. have sprains than most children, joint hypermobility may be the cause, and further
Minnetonka Clinic.  Visit the Resource Center on our website Craniofacial care – Martha McGrory, P.N.P. evaluation is recommended.
to find videos and professional present-
Neurotrauma care – Leslie Larson, P.N.P., Amanda Seeley, P.N.P.
ations. www.gillettechildrens.org
Sleep medicine – Laurel Wills, M.D.
About Joint Hypermobility Inside
Additionally, the Maple Grove Clinic provides comprehensive rehabilitation therapies and
Kristine Greer, D.P.T. assistive technology services. Joint hypermobility varies with age, gender (it is more common in females than ■ Beighton scale for assessing the
Back Issues of males) and race. Often, it is familial. The condition is much more common in infants extent of joint hypermobility, P. 2
Kristine Greer earned a doctor- A Pediatric Perspective and young children than in adults (people tend to become less flexible as they grow
ate in physical therapy from the www.gillettechildrens.org/ ■ Physical Therapy Can Treat and
older), and it occurs in a high percentage of Asian children. Randomly ascertained
University of Minnesota. She pediatricperspective Prevent Joint Hypermobility
data on school-age children found that 50 percent of Chinese-Asian subjects had
began working with adult and Symptoms, P. 3 (inside flap)
pediatric acute care patients at joint hypermobility 2 as opposed to 7 percent of English-Caucasians 3. The average
Mercy Hospital in Coon Rapids, age of onset of symptoms is 6.2 years, but the average age at diagnosis is 9.0 years,
Minn. Shortly thereafter, Kristine indicating a two to three year delay in diagnosis.4
transitioned into full-time
pediatrics at Gillette Children’s
Specialty Healthcare. She has worked at Gillette’s Minnetonka and
Maple Grove clinics.     

w w w. g i l l e t t e c h i l d r e n s . o r g

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