Complex Needs, CTEV, Scoliosis Flashcards
What is the Scottish Government definition of complex needs?
A child with multiple and complex disabilities has at least 2 different types of severe or profound impairment such that no one professional, agency or discipline has a monopoly in the assessment and management
Give some examples of complex needs?
- Cerebral Palsy
- Spina Bifida
- Muscular Dystrophy
- Arthrogyrposis Multiplex Congenita
- Neurofibromatosis
- Syndromes (Down’s, Turner’s)
What is Arthrogryposis Multiplex Congenita?
AMC, or simply arthrogryposis, describes congenital joint contractures in two or more areas of the body.
Children born with one or more joint contractures have abnormal fibrosis of the muscle tissue causing muscle shortening, and therefore are unable to perform passive extension and flexion in the affected joint or joints.
Describe basically the 3 types of Arthrogryposis Multiplex Congenita
Amyoplasia
-Characterized by severe joint contractures and muscle weakness.
Distal arthrogryposis
-Mainly involves the hands and feet.
Types of arthrogryposis with a primary neurological or muscle disease belong to the syndromic group.
Describe the 3 types of Neurofibromatosis
Neurofibromatosis type I,
-In which the nerve tissue grows tumors (neurofibromas) that may be benign and may cause serious damage by compressing nerves and other tissues.
Neurofibromatosis type II,
-In which bilateral acoustic neuromas (tumors of the vestibulocochlear nerve or cranial nerve 8 (CN VIII) also known as schwannoma) develop, often leading to hearing loss.
Schwannomatosis,
-In which painful schwannomas develop on spinal and peripheral nerves.
What is Neurofibromatosis?
Neurofibromatosis (NF) refers to several genetically inherited conditions that are clinically and genetically different and carry a high possibility of tumor formation
A child or young person (
Severe impairment in at least 4 categories together with enteral/ parenteral feeding
OR
Severe impairment in at least 2 categories and ventilation/ CPAP
AND
Impairments are sustained for more than 6 months and ongoing
What are the categories for complex exceptional needs?
Learning and mental functions Communication Motor skills Self care Hearing Vision
What is cerebral palsy?
A permanent and non-progressive motor disorder due to brain damage before birth or during the first 2 years of life
How common is cerebral palsy?
2 per 1000 live births
What can some of the prenatal causes of cerebral palsy be?
Placental insufficiency Toxaemia Smoking Alcohol Drugs
Infection such as toxoplasmosis, rubella, CMV and herpes type II (TORCH)
What can some perinatal causes of cerebral palsy be?
Prematurity (most common) Anoxic injuries Infections Kernicterus Erythroblastosis fetalis
What is Kernicterus?
Kernicterus is a bilirubin-induced brain dysfunction.
Hyperbilirubinemia may cause bilirubin to accumulate in the gray matter of the central nervous system, potentially causing irreversible neurological damage.
Depending on the level of exposure, the effects range from clinically unnoticeable to severe brain damage and even death. Newborns are especially vulnerable to hyperbilirubinemia-induced neurological damage and therefore must be carefully monitored for alterations in their serum bilirubin levels.
What is erythroblastosis fetalis?
Hemolytic disease of the newborn, is an alloimmune condition that develops in a fetus, when the IgG produced by the mother pass through the placenta.
Among these antibodies are some which attack the red blood cells in the fetus’ circulation, causing hemolysis. The fetus can develop reticulocytosis and anemia.
This fetal disease ranges from mild to very severe, and fetal death from heart failure (hydrops fetalis) can occur.
When the disease is moderate or severe, many erythroblasts (immature red blood cells) are present in the fetal blood, and so these forms of the disease can be called erythroblastosis fetalis (or erythroblastosis foetalis).
What can some postnatal cerebral palsy causes?
Infection (CMV, rubella)
Head trauma
What are the physiologic classifications of cerebral palsy?
Spastic (pyramidal system, motor cortex)
Athetoid (extrapyramidal system, basal ganglia)
Ataxia (cerebellum and brainstem)
Rigid (basal ganglia and motor cortex)
Hemiballistic
Mixed (combination of spasticity and athetosis)
What are the 4 anatomical classifications of cerebral palsy?
Monoplegia (one limb involved)
Hemiplegia (one side of the body)
Diplegia (lower limbs)
Quadriplegia or total body involvement
WHat percentage of cerebral palsy patients have some form of learning disability?
50%
Persistence of how many primative reflexes usually means the child will be non-ambulatory?
Give some examples of these reflexes
2 or more primative reflexes
Moro startle reflex Parachute reflex Tonic neck reflex Neck righting reflex Extensor thrust
Describe the character and deformity in dynamic contractures
Increased muscle tone and hyperreflexia
No fixed deformity of joints
Deformity can be overcome
Describe the character and deformity in fixed muscle contractures
Persistent spasticity and contracture
Shortened muscle tendon units
Deformity cannot be overcome
What are the orthopaedic priorities in CP?
Spine
-Tendency to get scoliosis
Hip
- Dislocating
- May be difficulty getting to perianal area
Feet
-Varus and valgus
Torsional problems
KEEP THE PATIENT AS ACTIVE AS POSSIBLE AS LONG AS POSSIBLE
What 5 prerequisites of normal gait should you look for?
Stability in stance
-Stable or unstable?
Clearance in swing
-Dragging feet?
Preposition of foot
-Are they placing their foot or does it just dangle below their leg?
Adequate step length
Energy conservation
-Bouncing around inefficiently?
How can you analyse gait (in CP)?
5 marks
By observation
By video
By 3D instrumented analysis
+/- EMG, energy expenditure
What is kinematics (in medicine)
What is the unit used?
How many orthogonal planes are used?
“How the body moves through space”
Unit = meters
3 orthogonal planes
Describe the actions of concentric and eccentric muscles in gait
Concentric
- Shortening
- Power generation
- Acceleration
Eccentric
- Controlled lengthening
- Power absorption
- Deceleration
Is the rectus femoris muscle concentric or eccentric at hip and knee joints?
Concentric at hip flexion
Eccentric at knee limiting knee flexion
Hips are normal at birth in cerebral palsy but displacement is likely in how many patients?
What does hip displacement depend on?
1/3
GMF kinetcs and forces on hip
What leads to better long term outcomes for hip problems in cerebral palsy?
Early surgical intervention
One study reduced dislocation from 10% to 0%
Describe clinical assessment of cerebral palsy
UNRELIABLE
Sitting, lying and prone if possible
-Look at spine without lower body interfering
Hip ROM- pain
Hamstring tightness
Pelvic obliquity
Spine in coronal and sagittal planes
Record and reassess later
Describe th 5 GMFCS levels
Level I
-Walks without limitations
Level II
-Walks with limitations
Level III
-Walks using a hand held mobility device
Level IV
-Self Mobility with limitations; may use powered mobility
Level V
-Transported in a Manual Wheelchair
The higher the GMFCS the higher the risk of what?
Dislocation
Pretty much a linear increase in risk
What interventions can you use for posture management?
Physiotherapy
Seating
What interventions can you use for spasticity management?
General
- Baclofen
- Diazepam
Specific
- Botulinum toxin
- Surgery
What interventions can you use for deformity management?
Soft tissue release
- Adductors
- Hamstrings
Bony realignment
- Varus derotation osteotomy
- Pelvic osteotomy
What are the pros and cons of surgery for cerebral palsy?
Pro
- Reduced risk dislocation
- reduced risk pain
- Better seating
Con:
- Not all would have gone on to dislocate
- BIG surgery
What is the medical term for club feet?
Congenital Talipes Equinovarus
What is the epidemiology of congenital talipes equinovarus?
Most common congenital deformity
-1 to 2 in 1000 live births
3 Male: 1 Female
50% bilateral
Risk for 2nd child 1 in 35
Give some aetiologies of congenital talipes equinovarus
Pressure theories
- Oligohydramnios
- Abnomal fetal position
- Unstretched uterus
Placental insufficiency Constriction bands Toxin Temperature Infective pathogen (entovirus) Drugs EM radiation Chromosomal abnormality Sex linked Single dominant Single recessive Polygenic
MULTIFACTORIAL
How many cases of congenital talipes equinovarus can be identified prenataly by ultrasound?
60%
What percentage of babies who recieve a prenatal diagnosis of congenital talipes equinovarus have defects in other systems?
Give some examples
50%
Hip dysplasia(?) Down's and larsen's syndrome Arthrogryposis Neuro-muscular disorders Others
What are the treatment options for congenital talipes equinovarus
Strapping
-Positional talipes only
Series casting
Dennis browne boots
Surgery (VERY VERY RARE)
- Postero-medial release
- Ilizarov frame
Who was the master of club foot treatment?
Professor Ignacio Ponseti
Describe the 4 anatomical features of club foot
Cavus
Adductus (midfoot)
Varus (hindfoot)
Equinus (hindfoot)
How many casts are usually required to fix congenital talipes equinovarus?
Average of 5 casts
works 95% of time
What small procedure can be used to correct equinus?
Percutaneous tenotomy of achilles tendon
90% will need this
What is the definition of scoliosis?
When is it clinically significant?
Any deviation in coronal plane is a scoliosis
Clinical significance > 10 degree deviation
Describe structural and non structural scoliosis
Structural
- Abnormal rotation of the vertebrae and is an intrinsic spinal problem.
- It has a propensity to progress
Non-structural
- Due to extrinsic cause: a leg length discrepancy, a hip problem etc.
- Resolves when causal factor is addressed
You can classify scoliosis by aetiology
Give some examples
Congenital deformity Idiopathic Neuromuscular Traumatic Infection Others - neurofibromatosis, neoplasm etc
How can you classify scoliosis by age?
3 marks
Infantile (10y)
Descibe the 4 geographical classifications of scoliosis
Thoracic
Lumbar
Thoracolumbar
Double
What are the risk factors for progression of scoliosis?
Female, premenarchal
Is scoliosis painful?
NO
What are the 4 broad aetiologies of back pain
Mechanical
Developmental
Neoplastic
Inflammatory
What investigations can you do for scoliosis?
AP Erect whole spine +/- lateral
MRI:
- Cerebellar tonsils
- Syrinx
- Tethering
- Vertebral anomalies
Bone scan- pain
Severe curves have less favourable outcomes
Give some examples
Cardiorespiratory compromise
Pain from rib/pelvic abutment
Seating issues
Surgical challenge
What is the management of scoliosis?
Bracing
Surgery
- Anterior
- Posterior
- Combined
What are the complications of scoliosis surgery?
4 marks
Nerve root damage
Cord traction injury
Vascular injury
Problems of growth
- Growing rods
- Changing rods
- Crackshaft pnenomenon
