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. 90 80 70 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. 60 50 40 30 20 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 90 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. 80 70 PERCENT FUNCTION 60 LIMITATIONS 50 PATIENT HISTORY 40 30 20 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. 10 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 80 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. 70 60 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 50 REFERENCES 40 30 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. 10 0 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.
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