(5) Pediatric Conditions Ppt-1

PEDIATRIC
CONDITIONS
Prepared by:
Kristine O. Magno, PTRP
Faculty, Department of Physical Therapy
College of Allied Medical Professions
Angeles University Foundation

NEUROMUSCULAR
CONDITIONS

CEREBRAL
PALSY

CEREBRAL PALSY
A disorder of movement, muscle tone or
posture that is caused by an insult to an
immature developing brain
NON-PROGRESSIVE

CEREBRAL PALSY
A disorder of movement, muscle tone or
posture that is caused by an insult to an
immature developing brain
NON-PROGRESSIVE
Leading cause of childhood disability
Prenatal, Perinatal, Postnatal

CEREBRAL PALSY
Intraventricular Hemorrhage
Bleeding into the ventricular system
MC in premature infants or those of low BW

Four grades:
Grade I – Isolated to the germinal matrix
Grade II – IVH c (N) ventricular size
Grade III – IVH c ventricular dilatation
Grade IV – IVH + parenchymal hemorrhage

CEREBRAL PALSY
Subarachnoid Hemorrhage (Hunt & Hess)

I

Asymptomatic
Mild headache
Slight Nuchal Rigidity

II

Mod-Sev Headache
(+) Nuchal Rigidity
(+) CN Palsy

III

Dizziness, Confusion
(+) Focal Deficits

IV

Stupor
(+) Hemiparesis

V

Coma
(+) Decerebrate Rigidity

CEREBRAL PALSY
Subarachnoid Hemorrhage (Hunt & Hess)

I

Asymptomatic
Mild headache
Slight Nuchal Rigidity

II

Mod-Sev Headache
(+) Nuchal Rigidity
(+) CN Palsy

III

Dizziness, Confusion
(+) Focal Deficits

IV

Stupor
(+) Hemiparesis

V

Coma
(+) Decerebrate Rigidity

CEREBRAL PALSY
Prenatal and Neonatal Factors
Hereditary
Maternal Infections
Toxic or Teratogenic Agents
Multiple Births
Abdominal Trauma
Prematurity
Birth weight
Rh Incompatibility

CEREBRAL PALSY
Maternal Infections
Syphilis
Toxoplasmosis
Other Infections – HIV & other STDs, Hepatitis, etc
Rubella
CMV
Herpes Simplex II

CEREBRAL PALSY
Perinatal Factors
Asphyxia
 Cephalo-pelvic disproportion
 Cord coil
 Placenta Previa

CEREBRAL PALSY
Postnatal Factors
Trauma
Infection
Circulatory

CEREBRAL PALSY
Levine Criteria for CP Diagnosis
Posturing
Oropharyngeal Problems
Strabismus
Tone Abnormalities
Evolutional Development
Reflex

CEREBRAL PALSY
Hypoxic-Ischemic Encephalopathy
Types depending on injured areas

CEREBRAL PALSY
Neuropathology

Term

Description

Involved Areas

Parasagittal
Cerebral Injury

Term

(B) Cortical and
adjacent subcortical
white matter
necrosis

Superior Medial and
Posterior Cerebral
Convexities

CPSQ

Adjacent to the
external angles of
the lateral ventricles
affecting the
semiovale and optic
and acoustic
radiations; corona
radiatia

CPSD
CPSQ

Periventricular
Leukomalacia

Preterm (B) White matter
necrosis

Presentation

CEREBRAL PALSY
Neuropathology

Focal and
Multifocal
Necrosis

Term

Description

Involved Areas

Infarction within a
vascular distribution

Presentation

CPSQ
Seizures

MC affected: MCA
Status
Marmoratus

Selective
Neuronal
Necrosis

Rarely occurs in
isolation

MC variety of injuty
observed in hypoxicischemic
encephalopathy

Basal Ganglia
Thalamus

Somemr’s sector
Subculum of
Hippocampus
LGB
Thalamus
Oxygen deprivation
Basal Ganglia
and Excitotoxic amino CN V and VII
acids
Dorasla Vagal Nuclei

Choreoatheto
sis
Athetosis
MR
Seizures

CEREBRAL PALSY
Neurologic Classification
Pyramidal
Extrapyramidal
Mixed Types

CEREBRAL PALSY
Spastic Cerebral Palsy (~75%)
Manifestations
Hypereflexia
Clonus
Extensor Babinski
Persistent primitive reflexes
Overflow of reflexes

CEREBRAL PALSY
Spastic Cerebral Palsy (~75%)
 Topographic distribution:
 Monoplegia
 Diplegia – all 4s, UE>LE; aka Little’s Dse
-Prematurity, IVH, PVL
 Hemiplegia – 1UE +1LE, UE>LE
- early hand preference
-highest prognosis for ambulation
 Quadriplegia – all 4as, UE=LE
-Normal birth weight
-Perinatal and Asphyxia, PVL
 Triplegia – 3 limbs

CEREBRAL PALSY
Dyskinetic Type
Extrapyramidal Type
Pattern of UE: UE>LE
Sleep: No extraneous movements
(N) to slightly DTRs
Persistent Moro and ATNR
Adolescence: Dystonia

CEREBRAL PALSY
Dyskinetic Type
Athetosis
Slow, writhing, involuntary movements (distal
extremities)
Intensity may increase with emotions and
purposeful activities
Highly associated with Rh incompatibilities
TRIAD of KERNICTERUS
 Sensorineural hearing loss
 Athetosis
 Parinaud’s Sydnrome

CEREBRAL PALSY
Dyskinetic Type
Chorea
Abrupt, irregular, jerky movements occurring in
head, neck, and extremities
Proximal > distal

CEREBRAL PALSY
Dyskinetic Type
Choreoathetosis
Large amplitude, involuntary movements

CEREBRAL PALSY
Dyskinetic Type
Abnormal twisting and posturing
Slow, rhythmic movements with tone changes
generally found in trunk and extremities
Sustained contraction  AbN postures

CEREBRAL PALSY
Dyskinetic Type
Ataxic
Unsteadiness with uncoordinated movements
Associated conditions:
 Nystagmus
 Asymetria
 Wide-based gait

CEREBRAL PALSY
Gait Patterns
Spastic Diplegia
Scissoring Gait pattern
Hip Flex + ADD
Knee Flex + Valgum
Ankle Equinus

CEREBRAL PALSY
Gait Patterns
Hemiplegia
Posturing
Hip hiking and circumduction
Weak hip flex + Ankle DF
Supinated foot in stance phase

CEREBRAL PALSY
Gait Patterns
Crouch
Tight Hip Flex
Tight Hamstrings
Weak Quadriceps
Excessive DF

HYDROCEPHALUS

HYDROCEPHALUS
Increase in head circumference
Signs:
Inc head circumference
“Cracked pot” sound
Lid retraction
Impaired upward gaze

HYDROCEPHALUS
Monro-Kellie Doctrine

HYDROCEPHALUS
Monro-Kellie Doctrine
► The skull is an inelastic, completely closed
container
► Pressure is distributed evenly throughout
the intracranial cavity
► The sum of the intracranial volumes of
blood, brain, CSF, and other components is
constant
► An increase in any one of these must be
offset by an equal decrease in another, or
else pressure will rise

HYDROCEPHALUS
Communicating Hydrocephalus
Inability to reabsorb CSF by arachnoid
granulations
Meningeal Scarring
 Meningitis
 IVH

HYDROCEPHALUS
Noncommunicating
(+)obstruction
Congenital malformations
Mass Lesions

HYDROCEPHALUS
Hydrocephalus Ex Vacuo
Triad:
Dementia
Incontinence
Ataxia

HYDROCEPHALUS
Cushing’s Triad
Increased ICP

HYDROCEPHALUS
Cushing’s Triad
Increased ICP
Bradycardia
Widening Pulse Pressure
Irregular respiration

ARNOLD-CHIARI
MALFORMATION

ARNOLD-CHIARI
Cerebellar elongation and protrusion into the
foramen magnum

ARNOLD-CHIARI
Type I
Malformations in the base of the skull and the
upper cervical spine
Protrusion of >3mm
Hydromyelia
Syringomyelia
syrinx

ARNOLD-CHIARI
Type II
Spina Bifida
Brainstem and Cerebellum protrusion
Classic Arnold-Chiari Malformation

ARNOLD-CHIARI
Type III
Herniation of whole cerebellum
Spina bifida
Occipital encephalocele

ARNOLD-CHIARI
Type IV
Cerebellar hypoplasia

Without connection to other malformations

SPINA BIFIDA

SPINA BIFIDA
 Neural Tube defect
 2 nd MC disability in childhood
 Risk factors:






Low socioeconomic class
midspring conception
Maternity obesity
Anticonvulsant drugs
Heat exposure

SPINA BIFIDA
Prenatal Dx: (+) Alpha Fetoprotein
(+) Acetylcholinesterase Enzyme
(+) CSF in Amniotic Fluid
Signs:
 Paralysis
 Sensory Defecits
 Hyperactive DTRs and Spinal reflexes
 Increased Tone

COCKTAIL PARTY SYNDROME

SPINA BIFIDA
Two Types:
Spina Bifida Occulta
Spina Bifida Aperta/Cystica

SPINA BIFIDA
SPINA BIFIDA OCCULTA
Affected structure: Vertebrae
(-) Herniation
(-) Arnold-Chiari Malformation
Warning Signs:
 Fawn’s tail/ Fawn’s Beard
 Café Au Lait Spots / Pigmented Nevus
 Angioma
 Dermal Sinus
 Dimple

SPINA BIFIDA
SPINA BIFIDA APERTA
 Contents of spinal canal herniated
 Meningocele
 Meningeal cyst filled with CSF
 (-)Hydrocephalus

 Myelomeningocele
 Sac contains dysplactic neural tissue
 With leakage of CSF
 Motor Paralysis
 Sensory deficits
 Bowel/Bladder Impairments

SPINA BIFIDA
Myelomenigocele
Associated with Hydrocephalus
Arnold-Chiari Malformation
MC Level : Lumbosacral
Lumbar

SPINA BIFIDA
Thoracic Lesion
 Kyphoscoliosis / Kyphosis
 SUPINE: Hip in Abduction, ER
Foot in equinus
 SITTING: Hip Flexion, Knee Flexion
Foot in equinus

SPINA BIFIDA
L1 – L3 segment
 HIP – Flex, ADDuction
 Ankle in equinus
 Complication: Early Paralytic Hip Dislocation
L4 – L5 Segment
 Complication: Late Paralytic Hip Dislocation
 Coxa Valga, Acetabular Dysplasia
 L4: Hip Flex ADDuction Knee Ext Ankle
Calcaneovarus
 L5: Calcaneus deformity

SPINA BIFIDA
Sacral Lesion
Clawing of Toes
Pes Cavus

SPINA BIFIDA
Associated CNS Malformations
 Tethered Cord Syndrome – 2 nd MC cause of
neurodecline
 Diastematomyelia
 *Diplomyelia

 Syringomyelia
 *Hydromyelia

 Arnold Chiari II Malformation – MC defect in NTD
 Hydrocephalus
 Malformation of Forebrain

DYSTROPHIES

Dystrophin
“…a protein complex that connects the
cytoskeleton of a muscle fiber to the surrounding
extracellular matrix through the cell membrane.”

DUCHENNE MUSCULAR
DYSTROPHY
MC Myopathy
Early feature: Difficulty negotiating stairs
Tendency to fall
Progressive difficulty in getting up from the
floor
(+)Gower’s Sign

DUCHENNE MUSCULAR
DYSTROPHY
 Early signs - starts at age 3 to 5 years
- Pelvic girdle weakness (waddling gait, frequent
falls, difficulty climbing stairs, awkward running)
- Pseudohypertrophy of calf muscles
- (+) Gowers’ sign
 Later signs - 10 to 14 yrs old
- relentless progression & involvement of shoulder girdle ms
- Scoliosis & thoracic deformity
- Inability to ambulate
- at 20 to 25 yrs old - respiratory failure

DUCHENNE MUSCULAR
DYSTROPHY
Earliest weakness: Neck Flexors
Pattern of weakness: ProximalDistal

Wheelchair Age: 7-13 y/o

DUCHENNE MUSCULAR
DYSTROPHY
Respiratory Impairment
Heart Abnormalities
MR? Mildly Impaired

BECKER MUSCULAR
DYSTROPHY
Milder form of DMD
Partial deletion of the gene coding for
dystrophin
 Dytrophin is partially present , muscle
membrane is semi – functional

BECKER MUSCULAR
DYSTROPHY
Onset of muscle weakness at slower rate
Ambulatory up to adult life
Cardiac abnormality may be seen
Mental retardation is rare

BECKER MUSCULAR
DYSTROPHY
Ambulation beyond 16 y/o
Wheelchair Age: 20 y/o

GENETIC CONDITIONS

CRI-DU-CHAT
Chromosome 5
5p minus Syndrome

CRI-DU-CHAT





Lejeune’s Syndrome
characteristic cat-like cry
problems with the larynx and nervous system
Other presentations:











feeding problems because of difficulty swallowing and sucking;
low birth weight and poor growth;
severe cognitive, speech, and motor delays;
behavioral problems
unusual facial features which may change over time;
excessive drooling;
small head and jaw;
wide eyes; hypertelorism
Cardiac defects
Cleft lip/palate

PRADER-WILLI SYNDROME
Paternal Chromosome 15
Hypotonis
Hypopigmentation
Hyperphagia
Obesity

ANGELMAN SYNDROME
Maternal Chromosome 15
MR
Prone to unprovoked periods
of laughter
Strabismus
Hydrophobia

PATAU SYNDROME
Trisomy 13
MR
Congenital Heart Defect
Deafness
Cleft lip/palate
Eye Defects

EDWARD SYNDROME
Trisomy 18
MR
Congenital Heart Defects
Malformed ears
Clenched hands
Syndactyly
Skeletal malformations

DOWN SYNDROME
Trisomy 21

DOWN SYNDROME
Trisomy 21
Chromosomal abnormality caused by breakage
and translocation of piece of chromosome
onto normal chromosome
Milder form with some normal cells
interspersed with abnormal cells, called mosaic
type

DOWN SYNDROME
Facial features:
Upslanted palpebrae
Epicanthal folds
Small low set ears
Short neck
Prominent tongue
Flattened occiput
Hypotonia

Associated conditions
Congenital Heart Dse
GI Malformations
Hypothyroidism
Cataracts
Hearing Loss

TURNER’S SYNDROME
45 XO
Found in women
Classic features:
Webbed neck c low
hairline
Wide chest with widely
spaced nipples
Coarctation of aorta

KLINEFELTER’S SYNDROME
47 XXY

Pediatric Adaptive Equipment

PEDIATRIC ADAPTIVE EQUIPMENT
Positioning Equipment
Equipment for Therapeutic Exercise

POSITIONING EQUIPMENT
Maintain skeletal alignment
Prevent or reduce development of
contractures and deformities
Facilitate functional abilities

POSITIONING EQUIPMENT

POSITIONING EQUIPMENT

POSITIONING EQUIPMENT

EQUIPMENT FOR THERAPEUTIC
EXERCISE
Balls of different sizes to promote
strengthening, balance, coordination, and
make motor learning fun
Wedges to facilitate or increase muscle
contraction needed depending on position of
wedge
Bolsters combine characteristics of ball and
wedge

EQUIPMENT FOR THERAPEUTIC
EXERCISE

EQUIPMENT FOR THERAPEUTIC
EXERCISE
7

EQUIPMENT FOR THERAPEUTIC
EXERCISE
Swings to promote sensory integration
Scooter boards for prone stability/ mobility
work
Others: toys, modified tricycles, music pets,
and family members

EQUIPMENT FOR THERAPEUTIC
EXERCISE
7

Orthosis
Mobility Aids

FAMILY
Family –single most important constant and
environmental factor
PT must collaborate with child and family
Family-centered approach
Parents of children with developmental
disabilities often suffer from chronic sorrow
due to the loss of typical potential of their
child

EARLY INTERVENTION
Provider comprehensive, multidisciplinary EIP
For infants and children from birth to 3 years
Multidisciplinary assessment
Family is a member of the team

EARLY INTERVENTION
According to research…

EARLY INTERVENTION
According to research…
24 children (8 months to 5 years) with
developmental delay due to organic causes (e.g.
congenital microcephaly, DS, autism, prematurity
of the brain, congenital hypothyroidism,
metabolic disorders)
Assessed using Griffith’s mental development
scales: gross motor, personal-social, eye-hand
coordination, performance

EARLY INTERVENTION
According to research (Results)

EARLY INTERVENTION
According to research (Results)

EARLY INTERVENTION
According to research (Conclusion)
A well-structured EI program that works with
parents and children together will enhance the
social and cognitive abilities of children with
disabilities of definite origin, especially in the
first year of its implementation

PROGNOSIS

PROGNOSIS FOR FUNCTION:
DOWN SYNDROME
7

PROGNOSIS FOR FUNCTION:
DOWN SYNDROME
 121 subjects; 65 boys & 56 girls; 1.7 to 72 months
 (mean +SD, 28.9+20.7 mos)

Improvement in GMFM scores with age
largest change during infancy
smaller increases as they get older
require more time to learn movements as
movement complexity increases

PROGNOSIS FOR FUNCTION:
DOWN SYNDROME
7

PROGNOSIS FOR FUNCTION:
DOWN SYNDROME
7

PROGNOSIS FOR FUNCTION:
CEREBRAL PALSY
7

PROGNOSIS FOR AMBULATION:
CEREBRAL PALSY
 Retrospe ctive cohort study
 5,366 children; mean age at entry 2.7 years; 56% males
 Mean follow up period was 5.8 years

8.5% achieved full ambulation (walk well alone
for 20 feet without assistive devices)
4.3% walk unsteadily alone at least 10 feet
without assistive devices
18.4% walk with support or assistive devices

PROGNOSIS FOR AMBULATION :
CEREBRAL PALSY

PROGNOSIS FOR AMBULATION :
CEREBRAL PALSY
Probability of walking at 7 years, Stratified by
motor milestones at 2 years of age

Probability of walking at 7 years, Stratified by motor milestones at 2 years of age

PROGNOSIS FOR FUNCTION:
CEREBRAL PALSY
7

PROGNOSIS FOR FUNCTION:
CEREBRAL PALSY





Longitudinal cohort study, followed up serially up to 4 years
657 children; 1 to 13 years at onset of study
Classified according to GMFCS
Used the GMFM-66 to measure outcomes

The estimated limit of development decreased
as severity of impairment increased
Children with lower motor development
potential reach her limit more quickly than
those with higher potentials

PROGNOSIS FOR FUNCTION:
CEREBRAL PALSY
7

PROGNOSIS FOR FUNCTION:
CEREBRAL PALSY

"Only where children gather is there any real
chance of fun.”
-Mignon McLaughlin