Document 150187

Physical Therapy Treatment Approaches For a
Child with Morquio Syndrome: A Case Report
Audrey Pittman
Pittman, Angela Parks,
Parks PT,
PT Rick Wilson PT,
PT PhD
Department of Physical Therapy, Medical College of Georgia, Augusta Georgia
DISEASE PROCESS
Individuals with Morquio have a life expectancy of approximately 30 years. The majority of
the deaths are attributed to respiratory failure. Respiratory infection and poor
musculoskeletal development, along with a low exercise tolerance lead to a diminished
quality of life and poor patient prognosis once chronic respiratory infections set in.
PURPOSE
YEARLY PROGRESS 2008‐2009
100
The purpose of this study is to demonstrate the effect of School based physical therapy and
hippotherapy techniques in a patient with a diagnosis of Morquio Syndrome.
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80
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TREATMENT
This study focuses on the time when the subject was in the school system, from ages 4-7.
Treatment involved school based physical, occupational and speech therapy, School based
physical therapy took place 1-2 days per week, in the classroom and was aimed at disabilities that
prevented him from taking part in classroom activities. Treatment included functional
strengthening, gait, balance, coordination, postural, fine motor and ADL training. The
interventions were designed to incorporate play to encourage participation.
However, because he was a reluctant participant in these regular PT sessions, weekly
hippotherapy treatments were initiated to achieve goals set by his physical therapist and to
address parental concerns. Equine facilitated interventions included riding with his trunk
unsupported both facing forward and backward, reaching activities, putting together large jigsaw
puzzles, catching & throwing a lightweight ball in a hoop.
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50
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10
0
STRENGTH‐ Rel. Indep. STRENGTH‐Ambulate w/ COORDINATION‐ Catch a POSTURE‐ Floor Sit w/ POSTURE – Sit Upright f/ FINE MOTOR‐ Snip 5” FINE MOTOR‐ Trace f/ School Day
Post. Walker
Playground Ball
Imp. Pos.
10 Min.
along Lines
Curves & Zig‐zags
Additional Growth
Additional Growth
Initial % Function
Initial % Function
YEARLY GOALS
RELEVANCE
Morquio is a rare syndrome, but treatable mainly through physical therapy intervention.
Morquio results in severe debilitating physical anomalies to young children, so achieving
adequate levels of physical therapy intervention is often a problem. Since death from
Morquio Syndrome often is caused by respiratory failure, strengthening the thoracic
musculature
l t
iis off vital
it l iimportance.
t
C
Coupling
li carefully
f ll controlled
t ll d hippotherapy
hi
th
with
ith existing
i ti
clinical physical therapy provided additional motivating factors for the young patient, and
positive results were noted. Additional studies should be completed to verify effects of
hippotherapy coupled with clinical PT as viable treatments for Morquio Syndrome.
RESULTS
YEARLY PROGRESS 2006 2007
YEARLY PROGRESS 2006‐2007
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PERCENT FUNCTION
Morquio syndrome is one of six mucopolysaccharidosis disorders that causes abnormal
connective tissue collagen and matrix2. Skeletal changes may be present on X-ray’s while
the individual is still an infant and there are prenatal tests to determine if a fetus has
Morquio Syndrome3. The most prevalent physical signs of Morquio are: growth retardation,
skeletal dysplasia, short trunk dwarfism, joint laxity, and chest deformities4. The associated
bone deformities become progressively worse over time due to a decrease in epiphyseal
growth caused by abnormal bone ossification 1. Skeletal deformities include odontoid
dysplasia,
lower costal flaring, kyphosis, scoliosis, unformed carpal and metatarsal bones, flattened
vertebrae, wide disk spaces, kyphosis, large elbows with limited supination and pronation in
the forearms, genu valgum, and hip dislocation. Spinal cord compression is often caused by
kyphosis and in some cases the thickened soft tissues of the ligaments in the cervical
vertebra have been found to be the cause of spinal cord compression3.
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PERCENT FUNCTION
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LIMITATIONS
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PATIENT HISTORY
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A 7 year old boy was diagnosed with Morquio Syndrome at 9 months of age. The patient
has asthma, acid reflux, seasonal allergies, and an overall low energy level, in addition to
M
Morquio
i symptoms
t
off atlo-axial
tl
i l iinstability,
t bilit widely
id l spaced
d tteeth,
th short
h t stature,
t t
jjoint
i t llaxity,
it b
bellll
shaped chest, bilateral hip dysplasia, increased kyphosis of the thoracic spine, and
decreased strength. Physically, the patient is very small of stature and at the age of 6 he
was 2’8” tall.
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0
STRENGTH & ENDURANCE‐
COORDINATION‐ Put COORDINATION‐ Feed BALANCE‐ Transfer to FINE MOTOR‐ Snip w/ FINE MOTOR‐ Draw FINE MOTOR‐ Snap & POSTURE‐ Sit in Chair Maneuver w/ Walker
on Shirt
Self & Drink From Cup
Toilet
Scissors
Circ. & Straignt Lines
Zip Pants
& Transition
He began physical therapy at the age of 11 months for bilateral hip dysplasia. He did not
begin walking until 15 months of age and at the age of 2 he suffered a fall, which resulted in
a cervical myelopathy. He has since undergone several surgeries including spinal fusion
and decompression in at the age of 3 and spinal fixation at the age of 4. These have
required lengthy recuperation before returning to school, therefore he was been retained.
Initial % Function
ACKNOWLEDGEMENTS
YEARLY PROGRESS 2007‐2008
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I would like to thank the subject, his parents, Janet Carswell, Angela Park PT, and Rick
Wilson PT, PHD for their assistance with this case report.
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PERCENT FUNCTION
Cognitive delays are not a clinical feature of Morquio Syndrome, however the subject of this
case study presents with delays in all five assessed areas of childhood development.
Approximate developmental scores at 6 years of age are as follows: Cognitive- 3.5-4 years;
Speech & Language- 5.5 years; Adaptive- 2.5-3 years; Social-Emotional- 5.5 years; with the
most significant delay in Gross & Fine Motor Skills-3 years.
Additional Growth
YEARLY GOALS
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REFERENCES
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20
At the age of 4 he was unable to: ambulate, sit independently, feed himself, control a pencil,
catch a ball or dress himself. By the age of 7 he was able to ambulate with a posterior
rolling walker, sit on a level surface independently, eat and drink independently, write his
name with a large grip pencil, and throw and catch a ball while sitting. He has the potential
to continue to gain functional skills with continued physical therapy.
The limitations of this study are: 1) it is a single case report, 2) there is little research that has
been done concerning physical therapy treatment of this syndrome, and 3) the outcome
measures in this study were all based on his achievement of goals, as the subject suffered a
di l set-back
t b k and
d had
h d tto be
b absent
b
t from
f
school
h l the
th final
fi l 3 months
th off the
th school
h l year, so
medical
specific re-evaluations were not able to be performed, 4) there is no way to separate the
benefits received in school based physical therapy from the hippotherapy treatments.
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STRENGTH‐ Improve Overall Strength
COORDINATION‐
Transfer on and off Adaptive Toilet
BALANCE‐ Improve Overall Balance
POSTURE‐ Sit “Criss‐
Cross” or Long‐sit on Floor.
YEARLY GOALS
FINE MOTOR‐ Snip 5” Along Lines
FINE MOTOR‐Trace FINE MOTOR‐ Use Curved & Zig‐Zag Lines
Markers or Paint on Additional Growth50+% of Drawing
Initial % Function
1) Beighton, P. and J. Craig (1973). "Atlanto-axial subluxation in the Morquio syndrome. Report of a case." Journal of Bone & Joint Surgery British Volume 55(3): 478-481.
2) Fisher, R. C., R. L. Horner, et al. (1974). "The hand in mucopolysaccharide disorders." Clinical Orthopaedics & Related Research(104): 19119
3) Gulati, M. S. and M. A. Agin (1996). "Morquio syndrome: a rehabilitation perspective." Journal of Spinal Cord Medicine 19(1): 12-16.
4) Savci, S., M. Ozturk, et al. (2006). "Inspiratory muscle training in Morquio's syndrome: a case study." Pediatric Pulmonology 41(12): 12501253.