Introduction — Cerebral palsy always has been the cause of dilemma since century for parents along with treating specialist regarding unpredictable outcome. With advancement in physiotherapy technique and new concept of OSSCS, the roll of an pediatric orthopedic surgeon and rehabilitation specialist has increase many fold in overall management of child with cerebral palsy .Treatment of cerebral palsy require team approach so that all these special children with cerebral palsy should not be suffer in inexperienced person. And to get best result, we should emphasized that therapist should be well trained in the management of developmental therapy otherwise child can deteriorate with improper physiotherapy.
Definition – The term cerebral palsy is taken from Latin term “Damaged Brain’ and also known as static encephalopathy. Cerebral Palsy is defined as disorder of movement and posture caused by a non progressive defect in immature brain by any insult from prenatal period to 2.5 years of post natal period.
Incidence —Incidence is 0.6-5.9 per thousand live births. Cerebral palsy is 27 times more common in children of low birth wt baby.
Etiology : Any insult of brain from prenatal period to 2.5 year postnatal period can cause cerebral palsy. Prenatal causes–intrauterine infection, toxemia and toxic drugs, multiple pregnancy, placental insufficiency. Perinatal causes– Prematurity, low birth Weight, neonatal asphyxia, kernicterus, septicemia, and respiratory distress syndrome, obstructed labour and Post natal causes– head injury and infection .
Clinical classification : 1) spastic – commonest (70-80%) 2. Dyskinesia : a) Athetosis b) Chorea c) Ballismus d) Tremor e) Dystonia 3) Atonia 4) Ataxia 5) Mixed
Topographical classification : Cerebral palsy can involve single extremity to all four extremities depending upon extant of brain damage. Pattern of involvement are 1) Monoplegia 2) Hemiplegia 3) Diplegia 4) Triplegia 5) Quadriparesis 6) Paraplegia 7) Double plegia
Pathophysiology of cerebral palsy: The immature partially damaged brain attempts to heal itself but falls short and the results are a fixed anatomical deficit. Peripheral manifestation depends upon the magnitude, extent and location of insult to brain. In spastic cerebral palsy Velocity dependent increase in tonic stretch reflex occurs because of a loss of inhibition in the basic neurological circuit of reflex arch normally under many modulatory influences (pyramidal tract). Unrelieved spasticity leads to fixed contracture, torsional deformity of bone and joints and dislocation during period of growth (Cosgrove & Graham 1994). Athetoid cerebral palsy is resultant of injury to extra pyramidal systems and ataxic variety is due to cerebellar damage.
Whole Problem seen in child with Cerebral Palsy-
Developmental milestone : delayed gross motor, fine motor etc…
Mobility: poor postural control, in coordination, poor balance, involuntary movement etc…
Cognition : Attention, concentration, memory etc.
Self care: Dependent/ partial dependent in basic ADL (feeding, dressing etc…)
Social: communication, social behavior (verbal& non-verbal)
Academic: Maintaining posture, hand function etc
Motor Disabilities :-
• Hyper-tonicity – spasticity, rigidity and athetosis (involuntary movement) along with its consequences like contracture, torsional deformity and joint dislocation
• Paralysis (weakness) of the propulsive and antigravity muscles
• Abnormal movements and postures and Persistence of primitive reflexes
• Difficulty in coordinated and alternative movements
• Difficulty in keeping the body in antigravity postures
Sensory Disability— loss of cortical sensation likes two point discrimination, stereognosis fine tactile sensation and joint position sense.
Associated Handicap: Associated problem define ultimate outcome in management of cerebral palsy . 1. Speech problem – 82%, 2. Mental Retardation – 19%,3. Deafness-15%, 4. Visual defect-34%, 5. Perceptual problem-14%, 6.Convulsive disorders (25%)
Other associated problem
• Mental retardation 8. Dental defects 9. Chest congestion 10. Sleeping disorder 11.Poor immunity 12.Growth retardation 13. social and emotional problems 14. Spinal defects 15. Bladder and bowel problems 16. Feeding problems 17. Constipation 18.Obesity 19. Malnourishment 20. learning disability
Diagnosis: Diagnosis of cerebral palsy is based mainly on detail history and clinical examination.
• MRI and CT scan are advisable in some cases to rule out other problem.EEG in case of epilepsy and genetic and metabolic tests are carried out in the case of family of affected sibling with progressive deterioration .
- Familial spastic Para paresis 2.Intracranial lesion 3.Metabolic disorder 4.Neurodegenerative disorder- primary and metabolic 5. Autism
Cerebral palsy is a clinical diagnosis made by an awareness of risk factors, regular developmental screening of all high risk babies and thorough neurological examination and achievement of mile stone in all position of baby. Cerebral palsy can suspected on following written feature.
• Premature birth, Difficult delivery, Asphyxia, Septicemia , Jaundice
• Delayed motor mile stones like poor head control, inability to sit and stand
• Asymmetry in functional use of extremities.
• Difficulty in feeding and drooping of saliva
• Abnormally increase or decrease in tone.
• Involuntary movement.
• Abnormal persistence of primitive reflexes.
Associated problems like mental retardation & speech problem, hearing loss, squint and seizure
Worry of Family Member:
• Overall functional delay, Abnormal performance , No change in motor behavior even after vigorous physiotherapy ,Worsening of condition with increase in age & Wt
Aims & Principles of Management :
• No permanent cure for cerebral palsy as Brain damage can not be repaired.
• Aim of treatment is to increase the patient’s assets as much as possible & minimize his deficit.
• With proper management we can diminish the functional impairment up to great extant in most of the children
• Regardless of their mental capacity, almost all patients can be taught something about self-care, mobility and communication, .
• Treatment should focus on child’s ability, not disability and method should be evolved to enhance utilization of his ability..
• It has been seen that with improvement in their physical condition, child also improve a lot in their cognition and their personality
• Management of child with Cerebral palsy needs multidisciplinary approach.
• Concept of integrated approach is to use all available proven modality of therapy and intervention modality in a combination to provide good therapy along with treatment of spasticity and contracture at earliest to prevent permanent consequences like bony torsion, dislocation and decompensated changes in joint.
Priorities of the Child with Cerebral Palsy:
- Communication 2. Activities of daily living 3. Mobility in the community 4. Ambulation
Poor prognostic value: 1. Moderate to sever mental retardation 2. Abnormal behavioral pattern 3. Athetotic and hypotonic cerebral palsy 4. Quadriplegic with sever contracture in early age 5. Absent neck holding after 20 month age 6. Absent Sitting after 4 year of age 7. Walking capability after 8 year 8. Persistent Moro, neck righting reflexes 9. Strong extensor thrust on vertical suspension 10. Absent parachute reaction after 11 month
Criteria for Treatment Modality — 1. Age 2. Developmental mile stones 3. Degree of contracture and deformity 4. Sensory and propioceptive problems 5. Degree of spasticity
Modality of treatment in cerebral palsy—
Primary modality of treatment—
• Physiotherapy – sensory intervention, neuro-developmental therapy, stretching and strength training exercise, gait training and balancing exercise. Therapist should well trained in developmental therapy programme. Now days strength training exercise are being more emphasized.
• Hydrotherapy (aquatic therapy)– Exercise in water appeals to children with CP because of the unique quality of buoyancy of water that reduces joint loading and impact, and decreases the negative influences of poor balance and poor postural control.
• Hypnotherapy (horse riding)– Therapeutic riding can facilitate cognitive and sensor motor development in childhood, help develop a sense of responsibility, self-confidence and fair play in adolescence and provide life-long recreation and sport. It can do all this while stimulating the good posture, balance and flexibility needed for functional independence off the horse
• Early intervention–Treatment of child with cerebral palsy start from ICU itself. It has been shown that with early intervention most of the children can lead to normal life (>60). Sensory integration, range of motion exercise and positioning of infant has a great role in early intervention. We should be causes in high risk children.
Braces, Night Splint and Mobility Aid:
• BRACES (AFO, Gaiter, Spinal frame) – helps in balancing ex. and gait training
• NIGHT SPLINT- keeps muscle in maximum stretched position.
• MOBILITY AID (Walker, Rolater, Tripod etc) – helps in mobilization
All treatment modalities should always be combined with vigorous therapy programme ( stretching and strengthening exercise along with balancing and gait training) and braces.
Intervention modality —
Repeated Corrective Plaster Application — it helps in correction of Static Muscular Contracture. It is indicated in Mild to moderate contracture and useful only in foot, ankle and knee problem. Plaster application after botulinum toxin injection enhances effect of spasticity reduction. But it is not indicated in cases with very sever contracture, dislocation and bony deformity. And it is very cumbersome and some time it lead to Incomplete correction.
Anti spastic treatment—
• Orally- Baclofen and Tizanidine. it causes drowsiness and generalized muscle weakness so .only short term use is advisable
• Intrathecal Baclofen- indicated mainly in generalized and quadriplegic CP. But complication rate are very high and very costly.
• Nerve block by Phenol and alcohol – it can cause sensory loss, disasthesia and some time irreversible muscle fibrosis and contracture
• Botulinum Toxin – Botulinum toxin is a powerful toxin which has been misused for biological warfare in the past. Its effect last for only 3 to 4 month but the duration of response of can be prolonged to up to some extent by use of serial cast, day night splint & good physiotherapy Mechanism of action: It acts pre-synoptically by blocking the release of the neuro-transmitter acetyl-choline at the NM junction. It does not kill neurons but causes temporary and ultimate reversible blocked of cholinergic transmission.
Role of Botulinum Toxin— Effective in only Spastic CP and it Facilitate better Physiotherapy
& nursing care. Agonist Muscles can be strengthen in better way and This toxin exerts its effect beyond the injection site in the form of relief of sustained abnormal posture.
Side Effects of Botulinum Toxin— Very-2 rare. Transient weakness, Swelling, bruising and calf pain, Skin rashes, Flue like syndrome. Asthenia, Urinary Incontinence.
Problem —– Due to short term effect it was being used repeatedly every six month but most of our children families are not able to afford this costly treatment .so we have stop using it repeatedly and now we are using only in certain children of 2-4 year age group with sever spasticity with the purpose to facilitate better physiotherapy and to post pone OSSCS till age of 6 year
Surgical intervention —
A, Selective posterior Rhizotomy– Selective Dorsal Rhizotomy (SDR) is a surgical procedure in which some of the sensory nerve fibers coming from the muscles to the spinal cord are cut. Its effects are permanent. Some time it can cause disabling and permanent weakness in limbs.
B, Neurectomy- now this surgery is not being done. it causes permanent weakness and fibrosis of muscles.
C, Selective Neurectomy— difficult to do precisely and can causes disasthesia and weakness of muscle. Eq. obturator and popliteal selective Neurectomy.
Orthopedic surgical intervention
a, Routine Orthopedic surgery— Orthopedic surgery is typically recommended when fixed deformities result in stalled motor progress, pain, Orthotic intolerance, or difficulties with care. Orthopedic surgery primarily involves fractional lengthening and tenotomy, muscle transfers, joint reconstruction, bone fusions, or bone realignment. Improper planning can lead to walking child into non-walker.
Problem arises from routine orthopedic surgery —
• Some time ambulatory patient became nonambulatory d/t loss of antigravity action
• Reverse deformity develop (genu recurvatum and weakness of tendoachilis)
• May require repeated surgery
• Not able to correct spasticity, athetosis ,torsional deformity and Lever arm disfunction
• Not based on concept of functional approach
• Not helpful in severely affected patient
This entire problem can be tackle by OSSCS and lever arm restoration surgery in a better ways (functional orthopedic surgery)
• Orthopedic Selective Spasticity Control Surgery —
• Earlier thought was that Result of surgery in cerebral palsy is unpredictable, some feel better and some worse following surgery. Now with advance technique and well planned surgery, child always became better.
• Contracture and bony deformities are almost inevitable in a growing child with spastic diplegia and need surgical intervention in the form of OSSCS at proper time to prevent joint de-compensation and over-lengthening of tendon.
• OSSCS+ Multi Level Lever Arm Restoration (bony correction) treat a wide range of problems in motor activities and activities of daily living and provide new path for functional improvement and for active life styles in most patients with cerebral palsy.
• OSSCS is an orthopedic procedure, designed to control or reduces all kinds of hypertonicity such as spasticity, rigidity, and athetosis in cerebral palsy. This surgical technique is based on concept of bi-articular spastic muscle. Long Biarticular muscle has more propensity of spasticity that weaken antigravity and voluntary activity of short mono articular muscle. Selective spasticity control may allow many patient with CP to use motor control in more effectively and functionally .
• Useful in all type of cerebral palsy including Athetoid, spastic cerebral palsy and Dystonia. Remarkable result in all grade of cerebral palsy irrespective of their severity
• Successful surgery give all round acceleration of other function like learning, speech, behavior along with motor function recovery
• Surgery should not be delayed to long, otherwise progressive deformity and co- spasticity of muscles will lead to de-compensated changes in joint and bone and makes gaits laborious, energy consuming and inefficient.
• Early surgery shortens the period of therapy even for years. OSSCS on lower limb is being performed in age group of 4-6 year and upper limb between 6-8 year ages. Although it can be done at any age group with proper indication.
• In upper extremity it help to improve the ability to turn over, to crawl and to use crutch
• It Can also be carried out in severely paralyzed to facilitate voluntary movement that are depressed by spasticity
• It help in acquiring in rolling, crawling, sitting, kneeling, standing and independent gait.
• Can correct spasticity in whole body, muscle imbalance, athetosis, Dystonia, contracture and bony deformities. / lever arm dysfunction
• There will be No loss of antigravity activity, No loss of sensation and stereognosis and No increase in deformity is going to happen.
• Orthopaedic selective spasticity control surgery is quite a reliable and promising procedure for patients, parents, physiotherapists and occupational therapists and even for school teachers.
• Well performed surgery on properly selected patient give good result provided the treatment after surgery is carefully managed
• Now surgery is being considered an important incident in total management of patient withcerebral palsy.
- Simultaneous correction of lever arm dysfunction– correction of lever arm dysfunction like tibial torsion, Anteversion of femoral neck and subluxation of femoral head should be treated simultaneously so that muscle forces start working in balance manner.
- Single stage multilevel corrective surgery (SEMLS) — now days all deformity in body is being corrected by OSSCS and multilevel lever arm bony corrective surgery in a single stage and under one anesthesia to save child from repeated surgery (I.e. BIRTHDAY SYNDROME ).