Benign Hyper mobility Joint

Syndrome
 Benign Hyper mobility Syndrome

A connective tissue disorder with hyper mobility in

which musculoskeletal symptoms occur in the
absence of systemic rheumatologic disease.
Hyper mobility and pain in multiple joints
Nonprogressive, noninflammatory connective
tissue disorder
Benign hyper mobility joint syndrome (BHJS) is a
common source of joint or muscle complaints that
often cause concern for parents, children and school
personnel.
Benign hyper mobility describes looseness of joints
that may be associated with daytime pain, nighttime
awakening, or discomfort after exercise.
Patients generally report multiple complaints of pain
over a prolonged period.
 Signs and Symptoms

 Children or young adults with hyper mobility usually have

joint pain or mild swelling during the late afternoon, at night
or after an exercise or activity.
 The pain is more common in the lower extremities, such as
the calf or thigh muscles.
 The pain most often involves large joints such as the knees or
elbows, but can involve any joint.
 Swelling is not usually present, but when found is due to
normal, activity-related trauma to the joints.
 Swelling can cause an increase in fluid within the joint cavity.
 Signs of inflammation, such as joint redness and heat, are
usually absent.
 Fluid may come and go within hours.
 Signs and Symptoms

hyper mobility
Joint pain
Multiple joints
Increase with physical activity or repetitive activity
Pain later in the day
Morning stiffness is uncommon
 Signs and Symptoms

Myalgia
Muscle cramps
Nonarticular limb pain
History of dislocation
Signs of typical connective tissue disorder
Family history
Other neuromusculoskeletal signs
 Signs of typical connective tissue disorder

Scoliosis
Pesplanus
Genovalgum
Lordosis
Patellar subluxation or dislocation
Marfanoid habitus
Varicose veins
Thin skin
 Who is affected by BHJS?

The frequency of BHJS varies with sex, age and

ethnic background.
Girls tend to have more mobility (looseness) of the
joints than boys of the same age.
 Younger children tend to have more pain
complaints.
 Teenagers may have fewer symptoms because their
muscles and joints tend to become tighter and
stronger as they become older, and because they
better understand the relationship between
increased activity and discomfort.
Diagnosis
 Family history

Double-jointedness
Recurrent dislocations
Easy bruising
Ligament or tendon rupture
Congenital hip dysplasia
Temporomandibular joint dysfunction
 neuromusculoskeletal signs
(acute or traumatic)

Sprains
Meniscus tears
Dislocations
Traumatic arthritis
Bruising
fractures
 neuromusculoskeletal signs
(chronic or nontraumatic)

Soft tissue rheumatism

Chondromalacia
Back pain
Scoliosis
Fibromylgia
TMJ dysfunction
Nerve compression disorders
Osteoarthritis
Delayed motor development
Congenital hip dislocation
 Physical Examination

Hypermobility
Pain in response to manipulation
Mild effusion
Tenderness
Redness
Fever
warmth
 Five specific mobility tests

for diagnosing BHJS,

The wrist and thumb can be moved downward so the
thumb touches the forearm.
The fingers (especially the 5th finger) can be extended
back so they are parallel to the upper arm.
When standing, the knees are abnormally bowed
backward when viewed from the side.
When fully extended, the arms bend further than normal
(beyond straight).
When bending at the waist, with the knees straight, the
child or young adult can put his or her palms flat on the
floor.
 Beighton score
Joint Finding Points
left little (fifth) finger passive dorsiflexion beyond 90° 1
right little (fifth) finger passive dorsiflexion beyond 90° 1
left thumb passive dorsiflexion to the flexor 1
aspect of the forearm
right thumb passive dorsiflexion to the flexor 1
aspect of the forearm
left elbow hyperextends beyonds 10° 1
right elbow hyperextends beyonds 10° 1
left knee hyperextends beyonds 10° 1
right knee hyperextends beyonds 10° 1
forward flexion of trunk palms and hands can rest flat on the 1
with knees full extended floor
Total Score 9
 (Brighton criteria)

 Major criteria

• Beighton score of equal or greater than 4

• Arthralgia for longer than 3 months in 4 or more
joints
 (Brighton criteria)
 Minor criteria
• Beighton score of 1, 2, or 3
• Arthralgia(>3-months) in 1 or 3 joints or back
pain(>3-months duration) or spondylosis,
spondylolysis, spondylolisthesis.
• Dislocation or subluxation in more than one joint,
or in one joint on more than one occasion.
• 3 or more soft tissue lesions
 (Brighton criteria)
• Marfanoid habitus >1.03 ratio or upper segment
less than lower segment <0.89 ratio
• Skin
• Varicose veins, hernia, uterine or rectal prolapse
• Ocular signs
• Mitral valve prolapse
 Requirement for diagnosis

 Any one of the following

2 major criteria
1 major + 2 minor criteria
4 minor criteria
2 minor criteria and first degree relative in family
history.
 Treatment

is specialized for each child or young adult, based on

his or her overall health, medical history, severity of
pain or discomfort, and presence of other symptoms.
Physical Therapy. Basic strengthening and guided
exercise programs can help the child to reduce
flexibility and increase muscle strength to help
prevent future injury.
 Joint protection techniques

Practicing joint protection techniques can help the child to

avoid over-stretching the joints at risk:
Avoid sitting cross-legged with both knees bent (Indian-
style)
Bend the knees slightly when standing
Wear shoes with good arch supports
Decrease those unusual joint movements that
hypermobile children often use to entertain their friends
Avoid "popping" or "cracking" fingers or neck, or other
joints.
 Conditioning activities

conditioning activities such as swimming, walking

and skating are helpful to improve joint strength.
Inactivity should be avoided.
In addition, being overweight worsens one’s
problem.
Splints, braces, or taping may be recommended to
protect affected joints during activities.
 Proprioceptive exercises

Proprioceptive (balancing) exercises may be helpful

in reducing pain.
 Medications

Since the underlying problem with hyper mobility is

joint looseness, rather than inflammation, most
medications provide only limited relief.
Taking aspirin or an over-the-counter nonsteroidal
pain reliever, such as naproxen or ibuprofen, can
reduce post-activity joint discomfort
 Lifestyle modification

Activities that induce symptoms

Vigorous activities
Repetitive activities
overtraining
Activities that focused on joint flexibility
 Joint protection

Improve proprioception
Improve proprioception may reduce strain on the
ligaments
Supportive splints
Appropriate foot wear
 Proper body mechanics

Achieve joint mechanics by

Improve strength
Improve balance
Improve coordination
Improve proprioception
 Ehlers-danlos syndrome(EDS)
 Group of connective tissue disorders that have
Gross joint laxity
Purple papyraceous scars
Skin hyper elasticity
Skin fragility
Easy bruising
Inherited autosomal dominant disease
Defect in collagen
 Ehlers-Danlos syndrome

similar to Marfan syndrome

genetic defect of collagen fibrils - several types - both
autosomal dominant and recessive
Hyper extensibility of skin, hyper mobility of joints -
joint dislocations,
rupture of large vessels, colon, cornea
 Marfan syndrome
Family history ocular and cardiac features
Autosomal dominant disorder
Tall and thin body habitus
Generalized joint laxity
Elongated fingers
 myopia
Lense dislocation
 Marfan syndrome

French pediatrician Marfan - 1896 - young girl with

typical habitus
abnormal protein fibrillin - secreted by fibroblasts,
impairment of collagenous and elastic tissue -
decreased firmness of connective tissue
principal clinical manifestations - 3 systems
 1. skeleton

slender, elongated habitus

long legs, arms and fingers (arachnodactyly)
high, arched palate
Hyper extensibility of joints
spinal deformities, pectus excavatum, pigeon breast -
 2. Ocular changes
dislocation or subluxation of the lens (weakness
of suspensory ligaments)

3. cardiovascular system
• fragmentation of elastic fibers in tunica
media - aorta
• aortic dissection - rupture (35-45% of
pts.)
• incompetence (dilatation) - aortic valve
• tricuspidal and/or mitral valve - floppy
valve