Physical Therapy for Patients with Multiple Hereditary Exostoses

Elena McKeogh Spearing, MA,PT,DPT,PCS

Physical Therapy Manager

Children’s Hospital of Philadelphia
INTRODUCTION:
What is Physical Therapy?
Physical therapy is a profession that specializes in the diagnosis and management of movement dysfunction
with the goal of restoring, enhancing, maintaining and promoting not only optimal physical function but optimal
wellness, fitness and quality of life as it relates to movement and health. (1)  

Physical Therapy can only be performed by a licensed Physical Therapist.  Physical Therapists possess
specialized training at the post-graduate level and have a license to practice Physical Therapy. Many people
who have suffered an injury, disease or disability can benefit from Physical Therapy intervention.   People who
want to prevent illness and disability can benefit from Physical Therapy as well. (1)

What does a Physical Therapist do?
A Physical Therapist performs an examination of many systems in the body.  These are the cardiovascular
system, the neuromuscular system, the musculoskeletal and the integumentary system. The physical therapist
looks specifically at how much a joint can move (
range of motion) and how strong the muscles of the body
are, including the heart.  They look at what activities are hindered by pain or loss of motion or strength.
Physical Therapists also examine balance, coordination and walking abilities.

After a complete assessment of the information and an interview with the patient, the Physical Therapist
develops a plan of care to address any issues that are present.   Based on a patient’s personal goals, the
Physical Therapist will develop a plan of care with specific interventions to help the patient to achieve those
goals. Physical Therapists strive to provide patient and family centered care, which recognizes the importance
of the patient and their family in the decision making process.

Physical Therapy interventions can include stretching, strengthening, postural and aerobic exercises,
functional activities and activities of daily living training.  Physical Therapists also educate patients on the
importance of wellness and injury prevention.

Physical Therapists work as a team with other health care professionals including physicians, nurses, social
workers, occupational therapists, speech therapists, recreational therapists, psychologists, and nutritionists.

PHYSICAL THERAPY FOR PATIENTS WITH MHE
How can Physical Therapy help patients with MHE?
For patients with Multiple Hereditary Exostoses, Physical Therapy is very important.  The physical therapist
works together with the orthopedic surgeon to determine the best course of treatment for exostoses.  

Pre-surgery:
As described throughout this book, exostoses can be present in any bone of the body.  Depending on the
location and amount of pain and disability, the orthopedic surgeon may or may not recommend surgery.  Prior
to surgery, the focus of Physical Therapy is to prevent or slow the loss of range of motion and function that
can be caused by exostoses.  Conservative treatment of exostoses may include physical modalities for pain
relief.  Although there is no evidence that exostoses growth can be prevented or slowed with Physical Therapy,
the disability associated with the exostoses can sometimes be managed effectively with therapeutic
interventions.  Flexibility and strengthening exercises have been shown to decrease progressive disability in
patients with other musculoskeletal disorders like fibromyalgia and rheumatoid arthritis (1, 2, 3).

Another focus of physical therapy, before and after surgery is required, may be to accommodate some of the
deformities that occur as a result of the exostoses.  These could include shoe lifts to make lengths of the legs
equal, splints to protect joints and cushions to make certain positions more comfortable.   Equipment can also
be provided to make activities of daily living easier and less painful. These include long handled utensils,
brushes, reachers and grippers.   Problems with mobility can be addressed with walking aides like canes and
crutches.  

Additionally, it has been shown that cardiovascular exercise can decrease pain and improve overall well being
in patients with musculoskeletal impairments (4, 5, 6).

A supervised exercise program that includes aerobic exercise and strength training may also help to decrease
the pain and stiffness associated with MHE.  While there are many benefits to exercise, anything that causes
increased pain in the area of exostoses should be discontinued and reported to the medical professional.

When the decision to have surgery is made, the patient’s individual needs after surgery should be anticipated.  
Often, patients can be seen for a pre-operative Physical Therapy visit. During this visit, patients can learn how
to use some of the equipment that they may need to use after the surgery.  Practicing these new skills, like
walking with crutches, or moving around with a cast or fixator, can make the patient less apprehensive about
the rehabilitation process that will take place after the surgery.  

This pre-surgery visit is also beneficial to problem solving obstacles to post-surgery rehabilitation.  For
example, many patients with painful exostoses under their arms, may not be able to use traditional axillary
crutches to maintain decreased weight bearing on their legs after surgery.  In this case, forearm crutches or a
walker may be more appropriate for the patient.  Additionally, a patient who does not demonstrate sufficient
endurance may need a wheelchair to use for going outside of the house after surgery.

Similarly, the patient who may have decreased weight-bearing abilities after surgery will need to practice new
approaches to everyday activities. These might include going up and down stairs, getting into and out of a car,
using the toilet, bathing and going to school or work. The patient and the therapist can simulate these activities
and problem solve together, before the surgery, so that they are prepared with successful strategies after the
surgery is performed.  

Post-surgery:
If it is determined that surgery is indicated to remove painful exostoses and increase a patient’s function,
physical therapy is important following surgery.  Depending on the surgery, there may be a period of
rehabilitation and the potential for  a temporary decrease in function due to pain and muscle weakness.  The
focus of Physical Therapy after surgery is to minimize the pain and maximize the patient’s movement potential
around the area that the exostoses were removed.

Some patients with MHE may only require a brief hospital stay after removal of exostoses; others may require a
rehab stay where more frequent and intense therapy is required.  This depends on the location of the surgery,
the extent of the surgery, the amount of function that is lost by the exostoses and the patient’s prior level of
functioning. During rehabilitation, therapy occurs daily and includes specifically stretching and strengthening of
the muscles around the area of surgery. If pain and function were limited prior to the surgery, there may be
some soft tissue limitations that are present after the surgery that will require special attention.

Based on the evaluation and orthopedic recommendations, weight bearing will be monitored and progressed
as directed. In procedures, which include limb lengthening, physical therapy will also address the joints that
surround the
fixator to prevent further contractures.

Once surgery incisions are healed, a heated pool may be a good environment for therapy. The water’s
property of buoyancy can decrease the pain that may occur with weight bearing.  Aquatic therapy can also
provide an environment where muscles can be strengthened in a fun way with swimming.

Once the patient’s goals are achieved and intense physical therapy is not required, transition planning will
occur and recommendations for the home, work or school and community will be provided. The development of
a home exercise program will maintain the gains that have been achieved through surgery and therapy and
prevent secondary complications that are due to pain and immobility.

Is Physical Therapy painful?
Some activities that are performed in Physical Therapy can be uncomfortable because it is hard work.  There
are things that a therapist can do for their patient to make therapy and exercise more comfortable.  Some
examples of this are relaxation techniques such as deep breathing and imagery.  There are also modalities like
heat and ice, which can ease the discomfort caused by exostoses or surgery.  Music therapy has been shown
to be effective at decreasing pain in patients with other types of chronic pain (7).

Are there other diagnoses that are associated with MHE and/ or MHE surgery?
There are some neurological disorders that can been associated with MHE. These include peripheral
neuropathy and Complex Regional Pain Syndrome. (9,10,11) Secondary complications from these can also be
addressed with Physical Therapy.  

In
peripheral neuropathy, the nerves that control the muscles are damaged due to being compressed by
exostoses. This leads to a loss of nerve conduction to the muscle and resulting weakness in that muscle. It can
affect the motor part of the nerve or the sensory part of the nerve. Impairment can range from slight to
complete. Because the nerves are not central to the nervous system, they can regenerate once the
compression is relieved surgically.  This is, however, a slow process. Physical Therapy can address this with
strengthening exercises and bracing, while the nerves to the muscles are healing. (9,10)  

Complex Regional Pain Syndrome, type I, (also known as reflex neurovascular or sympathetic dystrophy or
reflex sympathetic dystrophy (“RSD”) is a common condition characterized by extreme limb pain associated
with autonomic dysfunction.  This condition is associated with mild trauma to an extremity, as in the case of a
painful exostoses or surgical removal of exostoses.  

In this situation, there can be temperature, hypersensitivity, and trophic changes to the effected extremity.  
Treatment of this disorder in adults ranges from medications, surgical sympathetic nervous system blocks and
psychotherapy.  In children, studies show the symptoms of this condition can be controlled with physical and
occupational therapy.  

Treatment for this includes de-sensitization techniques where various textures are applied to the affected area
for prolonged periods of time. This usually begins with very light touching with cotton and progresses to
different textures, such as cloth and brushes. Weight bearing activities are also essential to retraining the
sensory system. Progressing weight bearing to the patient’s tolerance is an important part of treatment.  If the
patient’s surgical procedure initially prohibits weight bearing, other activities will have to be improvised in the
interim. Exercise is also an integral part of treatment for complex regional pain syndrome and has been shown
to be an effective treatment for this chronic pain disorder without the use of medications. (11)

Is it safe for patients with MHE to participate in sports and recreation?
Fitness and well-being is important for everyone, including the patient with MHE.  Every opportunity for
continuing exercise in a supervised manner should be encouraged. It may be advisable for schools to provide
parents with a detailed physical education curriculum that can be reviewed by the child’s orthopedist.  Sports
and physical education can often be safe to participate in with a doctor’s approval as long as the patient is
being monitored by the orthopedic surgeon and physical therapist. Non-contact sports like swimming, cycling,
dancing, tai chi, yoga and "Pilates" can be safe and fun forms of exercise for some patients with MHE.  
However, it is important to remember that MHE affects patients differently and specific sports and activities may
not be appropriate choices for people whose mobility is blocked by pelvic, hip and leg exostoses.  In addition, if
a child with MHE experiences pain while participating in sports or PE activities, the child should not be pushed
to continue that activity and medical advice should be sought.

Some people with MHE tire very easily when doing physical activity.   In addition to blocking certain
movements, exostoses put significant pressure on vital structures, causing fatigue. Some children are unable
to participate in their school’s physical education class. In these cases, there are federal laws under the
Americans with Disabilities Act (ADA) and Individuals with Disabilities Education Act (IDEA), which entitle
these patients to adaptations and accommodations and prohibit discrimination based on physical disability.  
Physical Therapists can work with schools to adapt physical education classes or provide alternative activities
in order to meet the curriculum’s health and physical education requirement.  Some families have reported that
certain school districts have tried to impose an alternative PE curriculum consisting of a series of written
reports on PE-related topics every week.  When working with patients suffering from chronic fatigue it is
important to remember that this type of extra work, particularly when the child is affected by hand and wrist
exostoses, exacerbates fatigue and worsens, rather than remedies, the situation.   In some cases where
fatigue is severe and participating in PE or complying with standard alternatives are not an option, physical
therapy exercises (whether performed at a physical therapy center or as part of a home program), can be
used to meet State physical therapy requirements.

Physical Fitness is an individualized concept. There are many types of activities that can be beneficial even if a
person with MHE cannot participate in competitive or recreational sports due to fatigue or mobility impairments.
Options for fitness include adapted wheelchair sports, seated aerobics and dance, and water aerobics.  
Physical Therapists can work with patients to determine their optimal activity level and options for fitness and
well-being. It is important to remember that the main goal is to have the child function as much as possible in a
normal school setting, and that for some students walking to and from classes, sitting through classes, and
keeping up with their studies constitutes physical activity.

CONCLUSION:
An overview of Physical Therapy has been provided; however, each patient is individual and not all Physical
Therapy interventions are indicated for all patients.  All Physical Therapy should be patient and family
centered in its approach. This is very important with MHE, due to the hereditary nature.  Exercise and fitness
can be a family event and something that they can share to benefit each other.

References:

  1. Guide to Physical Therapy Practice. 2nd edition. Physical Therapy: 2001; 81:9 744.
  2. Anthony KK, Schanberg LE.  Juvenile Primary Fibromyalgia Syndrome. Curr Rheumatol Report.2001
    Apr;3(2):165-71.
  3. Klepper SE  Effects Of An Eight-Week Physical Conditioning Program On Disease Signs And Symptoms
    In Children With Chronic Arthritis. Arthritis Care Res. 1999 Feb;12 (1): 52-62.
  4. Han A, Robinson V, Judd M, Taixiang W, Wells G, Tugwell P. Tai Chi For Treating Rheumatoid Arthritis.
    Cochrane Database Syst rev. 2004; (3): CD004849H
  5. Frost, JA Klaber Moffett, JS Mose, JCT Fairbank, Randomised Controlled Trial For Evaluation Of Fitness
    Programme For Chronic Low Back Pain. BMJ 1995; 310: 151-154 (21 January)
  6. Varju C, Kutas R, Petho E, Czirjak L. Role Of Physiotherapy In The Rehabilitation Of Patients With
    Idiopathic Inflammatory Myopathies. Orv Hetil. 2004 Jan 4; 145 (1) 25-30.
  7. Stanton-Hicks M, Baron R, Boas R, Gordh T, Harden N, Hendler N, Koltenzenburg M, Raj P, Wilder R.
    Complex Regional Pain Syndromes: Guidelines For Therapy. Clin J Pain. 1998 Jun; 14 (2): 155-66.
  8. Standley JM, Hanser SB. Music Therapy Research and Applications in Pediatric Oncology Treatment. J
    Pediatric Oncol Nurs. 1995 Jan; 12 (1):3-8.
  9. Paik NJ, Han TR, Lim SJ. Multiple peripheral nerve compressions related to malignantly transformed
    hereditary multiple exostoses.Muscle Nerve. 2000 Aug;23(8):1290-4.
  10. Levin KH, Wilbourn AJ, Jones HR Jr. Childhood peroneal neuropathy from bone tumors. Pediatr Neurol.
    1991 Jul-Aug;7(4):308-9.
  11. Sherry D, Wallace C, Kelley C, Kidder M  and Sapp.Short and Long term Outcomes of Children with
    Complex Regional Pain Syndrome type I Treated with Exercise Therapy.  J Clinical Pain. 1999 15 (3):
    218-223.
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