0. Rehabilitation of Myelodysplasia
Moon Suk Bang, MD
Department of Rehabilitation Medicine,
Seoul National University College of Medicine

1. Prevention of secondary complications
One of the major goal in rehabilitation of myelodysplasia patients
Secondary acquired deficits can decrease this potential in all areas.
ex) Contractures, leading to decreased mobility, skin ulceration, and pain.
Obesity contributing to skin ulceration, to loss of mobility, and to social stress

2. Rehabilitation approach
Comprehensive care;
must begin when children with MMC are young
Individualized approach
Team approach;
physical therapy, occupational therapy, proper orthosis and assistive device prescription, special education and etc.
Goal-directed approach
should base on accurate evaluation; physical,exam, imaging, electrophysiologic study

3. Rehabilitation of Spinal Dysraphism
Bladder & bowel care
Prevention of contracture, deformity etc.
Mobility training including early standing
Orthosis & whelchair
Electrodiagnostic evaluation
Sexual function

5. Clinical sign and course -motor paralysis-
usually lower motor neuron type.
combination of flaccid and spastic type; 10~40%
two types of joint abnormalities
(1)  static, rigid deformities at birth
(2)  deformity developing over the years as a result of neurogenic muscle imbalance or improper positioning

6. Motor paralysis
early accurate neurologic evaluation including diagnostic imaging study and electrophysiologic study(prerequisite for prognostic evaluation)
electrophysiologic study; more informative
(NCS, needle EMG, SEP, BCRL)
both motor and sensory deficit may be different, incomplete, or asymmetric.

7. Clinical signs by spinal level -thoracic lesion-
intact upper extremity function
intercostals, abdominal, and back muscle weakness
kyphosis or kyphoscoliosis
often spastic lower extremity
partial hip external rotation, abduction, ankle plantar flexion from supine position
hip, knee flexion, and equinus foot deformities from sitting

8. Clinical signs by spinal level L1-L3 segments spared
hip flexion and adduction contracture,
early paralytic hip dislocation, pelvic obliquity,
scoliosis,
gravity related equines deformity

10. Clinical signs by spinal level L4-L5 segments spared
hip abductor and extensor weakness
usually calcaneovarus foot deformity

11. Clinical signs by spinal level Sacral lesion
pes cavus with high arch and clawing of the toes
major problem; bladder & bowel, sexual problems
functional community ambulatory; all children and adults

12. consequences of denervation
vasomotor dysfunction decreased skin temperature over the involved area
asymmetric lower extremity growth
neuropathic charcot joint
osteoporosis; prevention; weight bearing by passive standing

13. Common problems; skin problems
predisposing to skin injury because of decreased sensation to pressure, pain, trauma, or heat
 decubitus ulcer over areas of bony prominence or weight bearing
Contributing factors; neurogenic bladder or bowel-         
tendency of recurrence, infection, delayed healing

14. skin problems
neurotrophic factor; healing of chronic wound after release of tethered cord
acute or chronic osteomyelitis
education of children and parents;
touching with hand first, careful daily inspection, pressure relief
most common complaints among patients with orthoses

15. Influencing factor; obesity
short stature, short lower extremities as well as scoliosis and at times vertebral anomalies
heights; at or below the fifth percentile for age, weights; approximately the fiftieth percentile, weight-height ratio above the ninety-fifth percentile

16. Obesity
in children with L1–L3 lesions; obesity; critical factor in the loss of ambulation
secondary to decreased energy expenditure among non-ambulatory patients
hypothalamic-pituitary dysfunction may also play a role.

17. Rehabilitation Therapy
Physical therapy and occupational therapy;
one of the first educational processes for a parent of an infant with spina bifida

18. positioning and range of motion exercises
goals of positioning;
to promote development and to prevent contractures
goal of maintaining functional joint range of motion;
education pf parents from hospital, promotion of equilibrium responses and righting reactions.

19. Rehabilitation therapy
developmental stimulation therapy;
for the development of fine and gross motor skills, including coordination,
designed to assist with both general childhood activities of daily living as well as mobility.
mobility training including crawling or scooting;
depending on a child’s motor abilities, and progression to an upright mobility approach

20. Self-care
self care including activity of daily living (ADL) should be monitored and encouraged
delay in ADL ; occupational therapy evaluation
OT sessions include training the children as well as parents
OT; necessary in extensive lower extremity paralysis and thoracic lesion to learn technique of dressing and other ADLs adapted to the home environment and other functional situation

21. Orthotics in MMC Often without objective evidence of efficacy
Influenced by practice pattern of geographic area (early instruction for walking)

22. Principles of L/E orthotics
Prevent deformity
Support normal joint alignment and mechanics
Provide variable range of motion when appropriate
Facilitate function

23. Standing frame
Parapodium
Swievel walker; for C6 injured child

24. ambulatory function and orthosis T11-L3 deficit
Hip Guidance Orthosis(HGO),
Reciprocating Gait Orthosis(RGO) isocentric RGO,
advanced RGO with bowden cable in tube
Mooring medial linkage orthosis
with crutch or walkerette,
RGO; not recommended before 30 to 36 months

26. Prognosis of ambulation
HKAFO; higher energy consumption, but faster velocity in swing through gait than RGO, controversy about the energy efficiency
outcome; house hold or exercise level, one third of high lumbar lesions; some degree of community ambulatory
most important factor; strong motivation

27. Effects of walking
Subjects who walked as children
fewer fractures & pressure ulcer
more independent, better able to transfer
than wheelchair user from early in life

28. midlumbar lesion
hip-knee-ankle-orthosis (HKAFO), KAFO, or AFO with upper extremity assistive devices depending upon hip and knee instability
frequent reassessment and variation needed for many variations
functional household and limited ambulation; feasible

30. Low lumbar lesion
AFO including ground reaction force AFO or shoe modification,
Trendelenburg lurch, gastrocnemius limp, anteroposterior instability, foot deformity
functional community ambulatory; 33% in children and 95% between 15 and 31 years   

31. 5 key componets of ankle & foot support
Control of the subtalar joint
Control of the mid tarsaljoint
High(flexible) medial & lateral wall
Toe elecation; intrsic or extrinsic
Transmetatarsal arch

32. AFO Conventional metal type Plastic type since 70’s good for molding
medial or lateral wedge inserts

33. AFO Common benefits improving knee(reduced excessive knee flexion)
improving ankle(reduced excessive dorsiflexion)
Park et al(1997)
use of AFO decreased prolongation duration of muscle activity of knee extensor in stance phase(24 S level patients) – by gait analysis(surface EMG)

34. Biomechanics of AFO
Barefoot degree
90 degree rocker sole

35. Various AFOs

36. Various AFOs

37. Various AFOs

38. Ground reaction AFO

40. FO, SMO FO not to impinge on the medial or lateral malleoli
distal trim line; just proximal to the metatarsal heads
SMO
proximal trim line; 3 to 5cm above the malleoli

41. Walking aid; crutch gait training
2-3 years, prerequisite to learn
at least 4-5 years old for upper lumbar or low thoracic lesion
swing-through gait pattern;33% more efficient than four-point gait.
pelvis and hip kinematics, improved with crutch, closer to normal gait

42. Walking aid;walkerette
more stability and easier to handle than crutch
first choice in early stage of training to use upper extremity ambulatory aids
reverse or posterior walker preferred for encouraging better erect posture

43. Wheelchair use
may partial ambulators eventually give up walking for full time wheelchair use around adolescence
faster and more convenient for mobility
wheelchair training; beginning at age 2
special seating support for anesthetic skin, useful for sports activity

44. mobility training including crawling or scooting;
depending on a child’s motor abilities, and progression to an upright mobility approach

45. Factors determining ambulatory function
neurologic level and active muscle activity, commonly the quadriceps
other important factors
obesity, contractures or deformity(esp. hip), age, cognitive status, motivation
type of spina bifida and other factors
early predictors of walking; sitting balance and motor level
unfavorable factors; deformity of spine and obesity

46. Reason for stop walking
Neurologic deterioration
Spasticity
Knee & hip contracture
Low back pain
Lack of motivation
Serious major medical events

47. Spinal orthosis - goals of spine treatment -
maintenance of a balanced trunk and pelvis to help maintain a seating position prevention of skin problems
preservation of respiratory function and maximal trunk height

48. Treatment of scoliosis:
bracing; with a curve of 20 degrees and progression of 5 degrees per year or more
potential problems with bracing; compromised pulmonary function, skin problems,
and potential rib deformity
custom-molded thoracolumbosacral orthosis; most common, then Boston brace

49. Congenital kyphosis(gibbus)
Incidence; approximately 10% in patients with MMC
Problems caused by kyphosis
skin ulceration at the most prominent portion
respiratory difficulty due to decreased lung cavity size
compression of abdominal contents
seating problems
decreased urologic function
decreased ability to use the hands well secondary to requiring increased use of the hands for balance

50. Cogenital Kyphosis Bracing
impractical for long-term care because of skin ulceration
and compression of abdominal structures
Goals of surgery
skin protection, improved sitting posture, and increased
upper-extremity use, along with preservation of pulmonary
and abdominal cavity area.