Integrated approach in the management of cerebral palsy:
Posted by on Wednesday, 18th March 2009
Introduction-- Whenever, a child is diagnosed as cerebral palsy, the idea which comes to the our mind that he is suffering from permanent brain damage and will be permanently disable for life. This Child will not be able to start their normal routine activities in time; besides that this child may be suffering from other associated problem like difficulty in hearing, vision, fits, dribbling of saliva and mental retardation. Until now, it was believed that in the absence of effective treatment these children can not do their daily activity on their own for whole life and they have to dependent on others and it was also thought that their life expectancy will be short. Most of time parents used to wander from pillar to post in the hope of best advice and treatment. We always in dilemma regarding unpredictable out come. But truth is for away from this fact. These children can survive up-to the age of normal population with good quality of life if they have given good treatment. Recently, due to new concept of OSSCS and availability of fully trained developmental therapist, great success has been achieved in most of these children. All new technique and concept have been included in the integrated approach. Integrated approach involves primary modality like sensory integration, neuro-developmental therapy, strength training exercises, braces, and intervention modality like botulinum toxin and OSSCS surgical technique. With this holistic approach most of our children can be given fruitful life and they can be integrated in main stream of society. Treatment of cerebral palsy requires team approach so that all these special children with cerebral palsy should not be suffering in inexperienced person. Definition - The term cerebral palsy is taken from Latin term “Damaged Brain’ and also known as static encephalopathy. Cerebral Palsy is defined as group of 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 <1.5 Kg. as compared to 2.5 Kg. It is commonest cause of severe physical disability in childhood and constitutes largest group of pediatric patient with neuromuscular disease. With the availability of good neonatal intensive care unit, increasing number of pre term and low birth weight baby are being saved, the number of children with cerebral palsy is increasing day by day.
Etiology: Exact etiology in most of the cases with cerebral palsy is not clear. Cerebral palsy can occur due to variety of causes. Any insult of brain from prenatal period to 2.5 year postnatal period can cause cerebral palsy.
Prenatal risk factors -- intrauterine infection, toxemia and toxic drugs, multiple pregnancy, placental insufficiency.
Perinatal risk factors -- Prematurity, low birth Weight, neonatal asphyxia, kernicterus, septicemia, and respiratory distress syndrome, obstructed labor , infant on ventilator for more than 4 week
Post natal risk factor-- head injury and infection.
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. Damage to brain is one time event so condition does not change but its effect may change with time. Aging has negative effect on joint due to abnormal posture and rigidity.
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.
Clinical presentation— Every child with cerebral palsy is unique in presentation. Presentation of Cerebral palsy can be very wide from sever global dysfunction of mental and physical ability to isolated slight disturbances in gait, cognition, growth, or sensation .
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 behaviour (verbal & non verbal)
Academic: Maintaining posture, hand function etc
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 problems are
7. 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
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
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 is required in child with history of epilepsy. Genetic and metabolic tests are carried out in the case of family of affected sibling with progressive deterioration.
Early Identification— we can identify children with cerebral palsy in an early stage with the help of regular screening in all high risk babies.
Cerebral palsy can suspected on following feature. History of:- 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 & seizure
Aims & Principles of Management: We don’t have any permanent cure of 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.
Integrated approach--- Concept of integrated approach is to use all available proven modality of therapy and intervention modality in a combination to prevent permanent consequences like bony torsion, dislocation and decompensated changes in joint and if it happen then it should be treated early so that child can be given a good chance of recovery early without loosing important time of life. It requires multidisciplinary approach.
Prognosis-- Approximately 85% of partially involved children have the potential to become independent ambulators. Life expectancy is normal in most diplegic and hemiplegic children, who receive adequate medical care and have strong family support. The survival rate of severely affected quadriplegics is dependent upon associated disability and care given to child.
Good Prognostic Value—1. Mild mental retardation to good IQ 2. Spastic variety 3. Diplegic, and hemiplegic 4. Good family support 5. Early identification and early intervention from 3 month to 6month. 6. Good neck holding and spinal balance.
Poor prognostic value --- 1. Moderate to sever mental retardation 2. Abnormal behavioral pattern 3. Athetotic and mixed cerebral palsy 4. Quadriplegic with sever contracture in early age 5. Absent neck holding after 4 year age 6. Absent Sitting and standing capability even with support after 6 year of age
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--- We should emphasized that therapist should be well trained in the management of developmental therapy otherwise child can deteriorate with improper physiotherapy. Parents should be fully trained in home based therapy Programme by therapist incharge of child so that they can carry out therapy at home.
• Physiotherapy – sensory integration, neuro-developmental therapy, stretching and strength training exercise, gait training and balancing exercise.
• 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 (>80%). 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: The goals of bracing are to increase function, prevent deformity, keep the joint in the functional position, stabilize the trunk and extremities and facilitate selective motor control. Now only light weight braces made up of polypropylene is being used. Traditional metal and leather caliper has no place in management of cerebral palsy
• BRACES (AFO, Gaiter, Spinal frame) - helps in balancing ex. and gait training
• NIGHT SPLINT- keeps muscle in maximum stretched position.
• MOBILITY AID (Walker, Relaters, Tripod etc) - helps in mobilization
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 leads to incomplete correction.
Anti spastic treatment-- Baclofen & Tizanidine has been used as oral antispastic treatment. But it causes drowsiness and generalized muscle weakness so only short term use is advisable. Intrathecal Baclofen is indicated mainly in generalized and quadriplegic CP. But complication rate are very high and very costly. Local Nerve block by Phenol and alcohol can be done but 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 can be prolonged up to some extent by use of serial cast, day night splint & good physiotherapy. 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. It is Effective in only Spastic CP and it Facilitate better Physiotherapy & nursing care. Agonist Muscles can be strengthen in better way This toxin exerts its effect beyond the injection site in the form of relief of sustained abnormal posture. Side Effects are Very-2 rare. Transient weakness, Swelling, bruising and calf pain, Skin rashes, Flue like syndrome. Asthenias, Urinary Incontinence are the some minor complication. Due to short term effect, it is being used repeatedly every six month. It is not very effective in elder children with contracture so we have stop using in elder children and we use only in children of 2 to 5 year age group with sever spasticity and with the purpose to facilitate better physiotherapy and to post pone OSSCS till age of 5 year
Neurosurgical intervention--
1. 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.
2. Neurectomy- Now this surgery is not being done. It causes permanent weakness and fibrosis of muscles.
Orthopedic surgical intervention:
1. Routine Orthopedic surgery-- Orthopedic surgery is typically recommended when fixed deformities results in stalled motor progress, pain, Orthotic intolerance & 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— With routine orthopedic surgery, some time ambulatory patient became non-ambulatory and Reverse deformity may develop ( Eq, genu recurvatum and weakness of tendoachilis). Surgery is being considered in staged manner so child requires repeated surgery. We are not able to correct spasticity, athetosis, torsional deformity and Lever arm dysfunction by this surgery. Routinely this surgery is being done in later phase of childhood life at 9-12 year age, when torsional deformity and joint disintegration has been already settled.
This entire problem can be tackle by OSSCS and lever arm restoration surgery in a better ways (functional orthopedic surgery) so we have stop doing routine orthopedic surgery in children with cerebral palsy.
RECENT ADVANCEMENT
1. Orthopedic Selective Spasticity Control Surgery—
• 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 multi-articular spastic muscle. Long multi-articular muscle has more propensity of spasticity that weakens antigravity and voluntary activity of short mono articular muscle. Hypertonicity of the multi-articular muscles causes abnormal hypertonic posture.
• When the multi-articular muscles are lengthened or sectioned selectively, hypertonicity is reduced & the mono-articular muscles are preserved and facilitated. Selective spasticity control may allow many patients with CP to use motor control more effectively and functionally.
• Earlier thought was that, result of surgery in cerebral palsy is unpredictable, some feel better and some worse following surgery. Now with the 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. Now surgery is being considered an important incident in total management of patient with cerebral palsy.
• OSSCS+ Multi Level Lever Arm Restoration (bony correction) treats 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.
• Well performed surgery on properly selected patient give good result provided the treatment after surgery is carefully managed. Successful surgery give all round acceleration of other function like learning, speech, behavior along with motor function recovery .
• There will be No loss of antigravity activity, No loss of sensation and stereognosis and No increase in deformity is going to happen.
• 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 helps to improve the ability to turn over, to crawl and to use crutches.
• It helps in acquiring rolling, crawling, sitting, kneeling, standing and independent gait.
• Orthopedic selective spasticity control surgery is quite a reliable and promising procedure for patients, parents, physiotherapists and occupational therapists and even for school teachers.
2. Simultaneous correction of lever arm dysfunction – Disruption in the moment generation of a muscle joint complex due to an ineffective lever arm moment despite normal muscle force results in Functional weakness and decrease in power generation. Correction of lever arm dysfunction like tibial torsion, anteversion of femoral neck and subluxation of femoral head can be treated simultaneously so that muscle forces start working in balance manner. Due to complexity of problem in these children, there is only few indication of multi level lever arm restoration in children with cerebral palsy. First we should try OSSCS (soft tissue surgery) to make non ambulatory child into ambulatory capability. Indication are Subluxation (> 40%) and dislocation of hip joint, Moderate to sever tibial torsion, Plano valgus feet not correctible by soft tissue surgery m and child with ambulatory capacity want to improve their gait pattern.
3. Single stage multilevel corrective surgery (SEMLS) -- Now days all deformity in body is being corrected by multilevel OSSCS in a single setting anesthesia to save child from repeated surgery (I.e. BIRTHDAY SYNDROME).
Conclusion-- In this new era of latest concept, most of our children can be given a fruitful life and even they can be intergraded in main stream of society. Early intervention always gives good functional outcome. Botulinum toxin and OSSCS surgical technique has became a boon for these children. Holistic approach to Management requires multidisciplinary team, in which role of fully trained and dedicated developmental therapist and family member can’t be ignored. Otherwise result of any intervention will be fruitless. There should be proper coordination between therapist and pediatric orthopedic surgeon.
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