MBHD: Course 3 Lecture notes

3.1 lecture “Pediatric rehabilitation: introduction”

Periventricular leukomalacia:
 A form of white-matter brain injury, characterized by necrosis of white matter
near the lateral ventricles
 Motor control problems or developmental delays:
o Spasticity (usually mostly the legs)
o Cerebral visual impairment
o Epilepsy
o Impairments in hearing, attention and behaviour

Basal ganglia damage:

 Due to perinatal asphyxia
 Dyskinesia
 Cognitive functions relatively good

Interventions for improving walking capacity CP:

 Physical therapy
 Crutches
 Orthoses
 Serial casting
 Botulinum toxin
 Oral baclofen
 ITB: Intrathecal baclofen
 SDR
Interventions over time:

3.1 lecture “Obstetric Brachial Plexus: neurosurgical aspects”

Obstetric brachial plexus lesion:
 Incidence: 0,5-3 in 1000 births
 Spontaneous recovery 75-90%
 Severe lesions >10%:
o Palsy (shoulder, hand, arm)
o Secondary consequences (joints, capsula, bony deformity)

Associated with birth lesions:

Gilbert & Tassin muscle grading system in babies:

 M0 = no muscle contraction
 M1 = contraction without movement
 M2 = slight to complete movement, without gravity
 M3 = complete movement against gravity
Neuroma = growth or tumor of nerve tissue (swelling of a nerve)

Treatment OBPL = multidisciplinary

3.1: “OBPL: rehabilitation”

Narakas level OBPL:
1. C5 & C6
2. Idem + C7
3. Idem + C8
4. Idem + Th1

Body functions/deformities:
 Paresis
 Sensory problems
 Reflexes
 Pain
o Atrofia
o Contractures/deformities
o Growth

Diagnosis:
 History of traumatic delivery
 Shoulderdystocia (problems with the delivery on the level of the shoulder)
 High birth weight

DD:
 Fracture
 Cerebral palsy
 Arthrogryposis
 Neuromuscular disorder

If there is an avulsion between nerves  no recovery

 70-80% complete – good recovery
 10% operation
3.1 Practical “casus”
Unilateral CP:
 Monoplegia
 Hemiplegia

Bilateral CP:
 Diplegia
 Triplegia
 Quadriplegia

CP motor types:

Clinical reasoning on walking capacity in CP:

3.2 lecture “The Growing Spine”
Skeletal growth determinations:
 Height, weight, BMI
 Dental development
 Age at menarche
 Sexual development (Tanner scale)
 Bone age

Biometric measurements: Height

 Standing height
 Sitting height
 Subischial limb length
o Standing height – sitting height
 Arm span

Standing height is necessary but is not sufficient to assess growth

Sitting height is the most reliable parameter to monitor trunk growth

Biometric measurements: Weight

 BMI between 20-40
 If weight >10% above normal, a scoliosis brace is no longer effective
 Menarche if > 40 kg

Chronology:
Estimation of skeletal age:
 Hand
 Olecranon
 Pelvis
o Risser 1, 2, 3, 4 and 5
o Tri-radiate cartilage closes
o Greater trochanter fusion

Pubertal growth peak:

Risser sign:
Tri-radiate cartilage:
 On the ascending side of pubertal growth
 12 y bone age (women) and 14 y bone age (men)
o Women  13 cm growth remains
o Men  14 cm growth remains

Olecranon method (Dimeglio):

Clinical relevance growth:

 Thorax growth  lung volume
 Trunk growth  scoliosis
 Lower limb growth  leg discrepancy

Scoliosis:
 Uneven growth of the trunk
 Thoracic insufficiency syndrome
 Crankshaft phenomenon
o A asymmetric growth of the trunk due to fixation that leads to rotation of
the trunk (often an increase in gibbus)
Lecture 3.2 “Clubfoot”
Voetstand:
 Fysiologisch
o Soepel
o Asymptomatisch
 Pathologisch
o Rigide
o Symptomatisch

Kenmerken klompvoet:
 Diepe, enkelvoudige huidplooien
Pes equinovarus (klompvoet):
 1 : 1000
 70% mannelijk
 50% bilateral
o 80% idiopathisch
o 20% gerelateerd aan:
 arthrogryposis
 Myelomeningocele (open rug)
 Diastrofe dysplasia

Deformiteit klompvoet (CAVE):

 Cavus van de mid- voorvoet
 Adductie van de mid- voorvoet
 Varus van de achtervoet
 Equinus (spits) van de achtervoet

Behandeling = Ponseti serial casting

 Achillestenotomie
 Boots and bar  3 maanden continu, tot 4 jaar nachturen

Klompvoet recidief  peestransfer (pees van m. tibialis anterior verplaatsen van medial
naar lateral van de voet)

Pes Planovalgus (platvoet)

PPV kan rigide of soepel zijn

Congenitale Verticale talus (talus verticalis):

 Rocket bottom feet
 Operatie vrijwel altijd noodzakelijk

Pes Calcaneovalgus (hakvoetje):

 Corrigeert binnen 3 maanden vanzelf tenzij onderdeel
van gegeneraliseerde afwijking
 Veel frequenter dan klompvoeten
 Door intra-uteriene malpositie
 Zonder actieve behandeling: uitstekende prognose

(Ante)pes adductus:
 Synonym: C, komma, banana voetje
 1:100
 Fysiologisch
 Verdwijnt vanzelf
Lecture 3.2 “Congenital Hip Dysplacia”

Heupdysplasie:
 Onderontwikkeling acetabulum
 Klachten op latere leeftijd
 Vroege artrose
 Meest voorkomende aangeboren afwijking aan
de onderste extremiteit

Vroege herkenning = snelle behandeling

 Veelal conservatief uitstekend resultaat
Late herkenning = deformeerde heup
 Sterk mankind looppatroon
 Vervroegde coxartrose

Diagnostiek afwijkingen:
 Abductiebeperking
 Beenlengteverschil (Galeazzi sign)
 Ortholani test

Graf klassen CHD:

Behandelingen:
 Conservatieve therapie (Pavlik)
o Dynamische spreidvoorziening (ca. 3 maanden)
o Echografische controle
o >90% succesvol
 Gesloten repositie
 Open repositie
 Salterostomie
 Pemberton bekkenosteotomie

3.2 lecture “SCFE and Perthes”

Morbus Legg-Calvé-Perthes:
 Idiopathic osteonecrosis of the epiphysis of the femoral head in children
 1 : 1200 children
 M:F = 4:1
 Higher incidence: Asians, Eskimos and Europeans
 Lower incidence: native people of Australia and Amerika

Pathoanatomy:
 Disruption vascularisation femoral head epiphysis
 Avascular necrosis and revascularisation followed
 Deformity because of weakness
 Femoralhead cartilage, epiphysis and physis are abnormal in histologic view
 Unorganized cartilage with hypercellularity and fibril forming
LCP diagnosis by exclusion:
 Other causes for AVN:
o Septic arthritis
o Sickle cell disease
o Cortico steroids

Evaluation:

Physical examination:
 Different walking pattern
 Diminished abduction and internal rotation
 Not common flexion contracture
 Sometimes mild leg length discrepancy

Diagnostiek en classificatie in de dia’s

SCFE = Slipped Capital Femoral Epiphysis

 The neck is slipped, not the head

SCFE in de dia’s
3.3 Lecture “Exercise testing in children with disabilities”

Cardiopulmonary Exercise Test (CPET):

 Golden standard for assessment of exercise (in) tolerance
 Goal is to apply a load on the patient, through incremental steps, till the
maximum and determine the maximal oxygen uptake
 On treadmill or bicycle ergometer
 Reach maximum load in 8-12 minutes

Energy systems:
1. The anaerobic creatine phosfate
a. < 30 sec
2. The anaerobic glycolysis system
a. 1-2 minutes
b. Anaerobic capacity
3. The aerobic (oxidative) system
a. > 2 minutes
b. Aerobic capacity

Main outcomes and interpretation CPET:

 VO2 peak (ml/kg/min)
o No plateau in children
 VO2 en VCO2 Criteria maximal effort in children:
 RER (VCO2/VO2)  HR > 180 BPM
 Maximal heart rate  RER > 1.0
 Maximal workload  Subjective signs of exhaustion

Children with CP: Aerobic fitness

Walking economy test:
 6-min walk test on an oval walkway
 Comfortable walking speed
 Distance and time measured > walking speed
 Measurements:
o VO2  Joules ( 1 liter = 21 KJ)
o Energy consumption (J/kg/min)
o Normalized for walking speed (J/kg/m)  energy cost

Anaerobic performance: Sprint performance test: (Wingate test)

 Cycling as fast as possible against fixed load
 Mean power (W) over 20 s  sprint power
 Sprint power is estimation for anaerobic fitness
o Children with CP have a lower sprint power

Clinical implementation:

Exercise testing  to find out what the cause of the complaints is (such as fatigue or
reduced walking speed)
 Determine whether energy expenditure is increased and/or fitness is reduced

Summary exercise tests:

3.3 Lecture” Dyskinetic Cerebral Palsy”

Dyskinesia = involuntary, uncontrolled, recurring, occasionally stereotyped movements

with variable muscle tone

Motor disorders in cerebral palsy:

Why is dyskinesia in CP important?
Dyskinesia can…
 Be painful
 Result in high energy expenditure and malnutrition
 Impede arm/hand function
 Interfere with positioning for sitting and sleeping
 Interfere with sleep
 Hamper care-giver
o Dyskinesia is often confused with spasticity, the treatment is different
though

Dystonia:
 Involuntary movements or intermittent muscle contractions
that cause abnormal movements such as twisting an repetitive
movements, and/or abnormal posture

Choreo-athetosis:
 Ongoing, constantly changing, involuntary movements

The dyskinetic type of cerebral palsy contains the highest GMFC scores.

A lesion is the basal ganglia causes the problems in dyskinetic CP:

 This resion is responsible for facilitating voluntary movements and inhibiting
competitive movements

Causes:
 Acute hypoxic-ischemia
o Also neonatal convulsions and jitteriness
 Hyper bilirubinemia
o Also hearing problems
 Intoxication
o Cyanide, methanol, alcohol, copper
 Metabolic disease
o Such as Glutaric aciduria
Treatment options:
 Oral medication
o Baclofen
o Benzodiazepines
 Botulinum toxin
o For patients with focal dystonia
o Temporary solution
 Intrathecal baclofen
o Baclofen pump
 Deep Brain Stimulation (DBS)

Intrathecal baclofen (ITB):

When?
 Intractable spasticity/dystonia
 When other treatment options do not work enough
 Patient is able and motivated
Effect of ITB:
 Reduces dystonia
 Improves upper limb function, comfort and satisfaction
 Reduces pain
 Reduces individual problems of daily life, individual care
 Improves quality of life

Take home message:

 Do not underestimate patients: Dyskinetic patients have better auditory
comprehension, visual and verbal memory. They do have problems with
communication
 Dystonia has a significant influence on ADL
 Aetiology lies in the basal ganglia
 Treatment options:
o Pharmacological
o Botulinum toxin
o ITB
o DBS
3.3 Lecture “Multidisciplinary consultation of a child: diagnostics
and treatment”

International Classification of Functioning, Disability and health (ICF):

Definition of Cerebral Palsy, CP describes:

 A group of permanent disorders
 Of the development of movement and posture, causing activity limitations
 That are attributed to non-progressive disturbances that occurred in the
developing fetal or infant brain
 The motor disorders are accompanied by disturbances of sensation, perception,
cognition, communication, behaviour, by epilepsy and by secondary
musculoskeletal problems

Treatment goals in CP:

 Improving activity and participation
 Prevention of secondary problems
o Normality is not a goal
o Needs of child and parent

GMFCS:
Interventions for improving walking capacity in CP:
 Physical therapy
 Walking aids
 AFO
 Serial casting
 Oral baclofen
 Botulinum toxin
 Selective Dorsal Rhizotomy (RDS)
 Intrathecal baclofen (IBS)
 Orthopedic surgery

Motor problems in spastic CP:

 Muscle activation disturbances
o Excess symptoms: spasticity, co-activation
o Deficit symptoms: weakness, lack of selective motor control
 Changes in biomechanical properties:
o Muscle stiffness
o Muscle shortening
o Contractures
o Bony deformity
 Problems during growth

Bi-articular muscle changes are more common than mono-articular

Short muscles are also very weak

Rehabilitation treatment after orthopaedic surgery:

 Takes long  up to a year
 Strength training
 Functional training
 Orthoses often still necessary

Take home:
 Cerebral palsy: Orthopedic surgery and SEMLS

Orthopaedic surgery for:

 Contractures
 Deformities
 Luxations

Possibilities for orthopaedic surgery:

 Lengthening of muscles
 Shortening of muscles
 Transfer of muscles
 Growth control of bones in children
 Fuse joints
 Joint replacement (THP)

Straightening of the legs:

 To walk as efficient as possible
 Always start conservative or combination
 Look at the back, hip, femur, knee, tibia, foot and ankle

Hip surgery in CP:

 Adductor psoas release
 Open reposition
 Pelvic osteotomy
 Proximal femur

Detailed surgeries are in the slides.

3.3 Practical fetal movements

First movement = sideways bending of head and or rump

 Always move sideways
 Slowly
 With low amplitude
 About every two minutes
General movement:
 Activity in arms, legs, trunk and head
 No fixed order
 Variation in speed, length, amplitude and participating body parts
 Fluency in movement
 In- and decreasing activity

3.4 Lecture “Spina Bifida”

Neural tube defect:

 A congenital abnormality of the neural tube, that is formed from the ectoderm in
the first 4 weeks of pregnancy

Neurulation = the folding process in embryos that includes the transformation of the
neural plate into the neural tube
 The neural tube later forms the spinal cord, brain and meninges

Spina bifida is often noticed in at the 20 weeks ultrasound

Spina bifida:
 Means “split spine”
 Neural plate fails to close completely  defect or absence of the vertebral arches
 Usually in the lower back

3 types:
 Myelomeningocele
o Most severe
o Spinal cord + meninges protrude out of an opening in the bony vertebrae,
held together by only skin
o Or it’s covered by no skin at all  open spina bifida  damage and risk of
infection
o Symptoms:
 Loss of sensation
 Paralysis
 Bladder and bowel movement problems
 Leg and foot deformities

 Meningocele:
o Only the meninges slip into the gaps between the deformed vertebrae (not
the spinal cords)
o Least common form
o Spinal cord is not damaged  no severe symptoms
  Spina Bifida Occulta
o Most common and most mild form
o Occulta = hidden (prenatal tests often don’t detect it)
o Spinal cord and tissue don’t protrude the vertebrae
o Usually no symptoms, often found accidentally
o Most people only have hair, a dimple or a birthmark at the location of the
lesion

Cause:
 Mostly unknown
 Risk factors:
o Folate (vit B9) deficiency
o Obesity
o Poorly controlled diabetes
o Medications that interfere with folate metabolism

Diagnosis myelomeningocele:
 Prenatally
o Increased level AFP in mothers serum
 Ultrasound

Treatment:
 Prenatal surgery
o To close the myelomeningocele
o Can be dangerous
 Postnatal surgery:
o Days within birth
o To minimize risk of meningitis
 Additional intervention
o Catherization
o Wheelchair or crutches (in case of paralysis of low muscle tone)
Other risk factors:
 Trisomy 18 and 13
 Earlier pregnancy with neural tube defect
 Low vitamin B12
 Hyperthermia
 Diarrhea

Prognose tav lopen:

 Het niveau van cèle is bepalend
 L5 (enkel-dorsoflexie)
o Loopt buitenshuis en is niet afhankelijk voor transfers, mogelijke orthose
en goede voetzorg
 L3-L4 (Knie extensie)
o Loopt korte afstanden met orthese, rolstoel voor lange afstanden
 T6-L2 (rompstabiliteit en heupflexie)
o Niet functioneel lopen, rolstoelgebonden
 Loopfunctie gaat vaak achteruit als kind ouder wordt

Intelligence and cognition:

 Most children don’t have problems
 Average IQ ca. 90
 PIQ<VIQ
 Worse prognosis if:
o Associated developmental problems of the brain
o Draininfections

Continence:
 Sacral roots S3-S5
 Neurological bladder and bowel movement problems in almost all Spina Bifida
Operta patients

Mortaliteit en morbiditeit:
 Overlijden bij:
o Draindysfunctie
o Draininfectie
o Nierfalen
 Meerderheid wordt normal volwasse, 30% blijft hulpbehoevend
Prenatale chirurgie:
 Inclusie criteria moeder voor prenatal repair in België:
o Eenling zwangerschap
o Meningomyelocele tussen Th1-S1
o Amenorroeduur 19-25 weken
o Normal karyotype
 Exclusiecriteria:
o Ernsitge kyfose
o BMI > 35
o Contraindicatie chirurgie moeder
o Eerdere hysterotomie
o Verhoogde kans op premature bevalling

3.4 Lecture “Spina Bifida: rehabilitation aspects”

Goals rehabilitation:
 Prevent secondary complications/deformity
 Improve and support activities and development in all domains

Levels of functioning:
 Mobility: ambulation, transfers, wheelchair
 Self care
o Dressing, bathing, toilet
o Continence bowel and bladder
 Cognition
 Communication
 Social/emotional functioning
 School/day care

Levels of mobility in SB according to Hoffer:

1. Community walker: ambulating outdoors with or without braces but using
wheelchair for longer distances
2. Household walker: using braces or crutches indoors but wheelchair outdoors
3. Exercise walker: walking only in therapeutic settings
4. Non-walker: wheelchair-dependent
 Significant correlation between the Hoffer criteria, the level of lesion, the
walking distance and ability to stand up right
Clinical signs and course:

Associated malformations – spinal cord:

 Tethered Cord:
o Abnormal attachment of the distal end
 Can be the cause of neurological decline in myelomeningocele
 Signs:
o Spasticity
o Decline in strength
o Worsening scoliosis
  Syringomyelia:
o Tubular cavitation filled with spinal fluid
o Seen in up to 40% of myelomeningocele
o Symptoms:
 Decreased function
 Scoliosis above the level of the lesion

Associated malformations – brain

 Arnold-Chiari 2 malformation (congenital)
o Present in 80-90%
 20% symptomatic
o Caudal displacement or herniation of the brainstem
o Often associated with hydrocephalus
o Most severe symptom  respiratory compromise
o Dysphagia (Swallow problems), ocular dysfunction can also occur

Feet:
 Almost 90% of patients have abnormalities
 Conservative casting and splinting vs. surgery
 Goal is to develop a plantigrade foot and prevent soft tissue contractures

**Types of rehabilitation equipment in the slides

Treatment neurogenic bladder:

 Storage dysfunction  anticholinergics
 Emptying dysfunction  intermittent cathererization
 Hyperactive external urethral sphincter  baclofen, botulinum toxin, surgery
 Mitrofanoff procedure:
o Appendix used to create a catherizable conduit between bladder and
umbilicus

**Neurogenic bowel, osteoporosis and obesity covered in the slides

Learning disability:
 Hydrocephalus is a major risk factor
 Level of lesion may be a factor
 Poor executive functioning noted
 Poor nonverbal skills
 Superficial level of verbal skills leads to overestimation of verbal abilities
o “Cocktail party effect”
Psychological concerns:

Conclusion:
3.4 Lecture “Cerebral palsy: gait patterns and orthotic
management”

Orthotics can be useful in CP patients with a GMFC score I-III

 Ankle foot orthoses (AFO) most common

Why an AFO:
 To correct and/or prevent deformity
 To provide a base support
 To facilitate training in motor skills
 To improve the efficiency of gait

Treatment decision:
 Physical examination:
o Range of motion
o Muscle tone
o Functional strength
 Degree of orthopaedic deformation/muscle shortening are modifiers for
therapeutic measures, clinical assessments are essential information

Pes planovalgus (platvoet):

 Valgus of the calcaneus
 Talonavicular subluxation
 Forefoot abduction

Pes varus adductus:

 Varus of the calcaneus
 Forefoot adduction
 (Komma voetje)
Classification of gait patterns in CP:

Gait types according to Rodda:

AFO type:
 Dorsal or ventral shell
 Rigid or hinged
 Footplate stiff or flexible

Type of AFO in relation to gait type:

Hyperextended knee  dorsal AFO

Flexed knee  ventral AFO

** Examples on the choice of orthotics in the slides

Rigid AFO’s:
 Counteract knee flexion Improves gait efficiency
 Improve knee extension moment

 Obstruct ankle ROM Impedes gait efficiency

 Impede push-off power
Take home message:

3.4 Lecture “Orthotics in CP en Spina Bifida”

Goals of orthotic treatment:

 Correction of gait
 Correction of foot deformity

Orthotic design to correct gait:

 Ground reaction force in midstance (on the knee)
 External moment: what would happen if only gravity applied?
 Orthosis design:
o What should happen to the knee
o Rigid or flexible footplate?
Goals and disadvantages of AFO’s:

Orthotic devices for patients with Spina Bifida

Lesion level and orthosis:

Reciprocating Gait Orthosis (RGO):
 Forces reciprocal movement: flexion of one hip leads to extension in the other hip
(old RGO)
 Construction needs to be very strong and rigid
 Problem when children become heavier
 New RGO  Salera orthosis
o No longer a reciprocal construction
o Active hip extension is necessary for this orthosis
o Does make rotations possible  3D hip movement
 Internal and external rotation
 Abduction an adduction
 Flexion and extension

Summary:

3.4 Lecture “Communication in children with severe CP”

Parents lead the interaction:

 Decide on the topic
 Start conversations
 Ask questions about immediate environment
 Yes/no questions
Children respond:
 Reply to parents’ initiations
 Yes/no answers, limited information
 Rarely ask questions

Speech in children with CP:

 51%  speech disorder
 37%  dysarthria

Classification systems:

Overzicht GMFCS, MACS en CFCS:

CFCS:
 Based on important aspects of functional communication
 The performance of sender and receiver roles
 The pace of the communication
 The type of communication partner

CFCS identification chart:

GMFCS I-III may have impairments with communication but are intelligible
GMFCS IV-V are often non-speaking
Diagnostics in non-speaking children with CP:

AAC systems:
 Augmentative and Alternative Communication
 AAC may lead to development of natural speech or
spoken language comprehension
 Selection of appropriate AAC depends on:
o Learning an developmental priorities
o Language and comprehension skills
o Current and future communications needs

Test components C-BiLLT:

 Details in the slides

Utility C-BiLLT:
 Knowledge of discrepancy between expressive and receptive communication
skills of the child
 Clinical intervention program
 Appliance for a suitable AAC device

** Voor- en nadelen C-BiLLT in de dia’s

3.4 Lecture “Transition to adulthood”

Transitions in medical care:
 90% of children with disabilities live beyond 20 years of age
 30% have at least one chronic illness or disability
 Adolescent patients experience more isolation, suicide and depression
 Focus needs to move beyond chronic disease  sexuality, substance abuse,
lifestyle issues

Skills for Growing Up tool (SGU):