Pre and Post Surgical Protocol in Cerebral Palsy Children

There is no special investigation required for SEMLS by OSSCS concept. Simple blood test and x-ray required in some cases. Total duration of surgery is depending upon many factor but 3-4 hour in diplegic and 5-6 hours in quadriplegic. OSSCS Surgery in lower limb is being performed in epidural anesthesia and upper limb in brachial block.

We try that child is not completely unconscious but in small child medicines only given to take them in sleep. Till now no adverse effect is seen. After taken out from Operation Theater water should be given after two hours and food can be given in evening. After surgery no special precaution is to be taken. Child can turnover and sit on bed also. In this surgery all stiffness and Spasticity can be control by only one procedure and muscle balancing power becomes good.

Normally, physiotherapy started after two weeks of surgery. In starting, exercises done only to decrease pain, increase the range of joints and decrease the stiffness of muscles. Then after 1 month of surgery, strength training exercises and exercises for activities of daily living will start. These strengthening and daily living exercises run for almost six months to one year. After this protocol, exercises will be continued till the adolescent phase and child became independent as much as possible. Child should be under close observation. By close observation, child can be secured by the adverse effect of any further problem.

Child feels easy in walking and balancing. Result of surgery depends on many things like therapy, child’s ability to understand and the main parent’s hard work.  In last five years it is never seen that there is no effect of surgery, ever child who has undergone surgery has shown response depending upon their pre operative functional disability.

Operation in these child done after complete investigation and child is operated only who has need and capability too. In this only physical specially deformities and Plasticity is to be corrected and function recovery depends upon therapy and the child’s ability.

Electrical Stimulation for Cerebral Palsy

Electrical stimulation have given some positive results in the muscles of cerebral palsy children. Electrical stimulation is a type of therapy that provides the muscles with a low dosing of electric current. Electrical stimulation for cerebral palsy is used to decrease the effects of stiffness and plasticity in the muscles of the children. The effects of electrical stimulation are not long lasting and need to be given over a fairly long period of time.

The therapy is often used in combination with other types of treatments such as physical therapy. When the muscles are less stiff, physical therapy helps with movement and relieves associated symptoms. Electrical stimulation helps in reducing these symptoms. This combination therapy is effective in treating contractures in cerebral palsy patients.

Electrical stimulation alone may be effective in helping smaller body parts, such as the fingers and toes. Exercise and physical therapy are often combined with electrical stimulation to produce results in larger body parts, like the legs or hips. Some of the benefits of electrical stimulation for use in cerebral palsy are a marked reduction in muscle plasticity. Electrodes can be placed either over the muscle or near the offending body part. Skin electrodes send slight, almost unperceivable electronic impulses to the muscle tissue. Often other methods of reducing plasticity in cerebral palsy patients result in a weakened or damaged muscle, but electrical stimulation does not harm the muscle.

Electrical stimulation(ES) has also been successfully combined with positioning and voluntary effort, when possible, to correct contractures and maintain range of motion. It is critical to the success of ROM protocols to use a comfortable ES protocol one or more times each day. When the body segment to be moved is relatively small (fingers, wrist or ankle), the muscle pull created by ES alone may accomplish the goal. When the body segments are larger (knee or hip) ES may assist the patient in exercising to the end of their range. The therapist should combine ES with voluntary effort. It is important to remember that the maximum benefit of ES for spasticity may not be realized until ES has been used for 1-2 hours each day for 1-3 months.

If ES is discontinued, spasticity usually can be expected to return. For this reason, many patients elect to continue to use ES throughout their life. In addition to the modulation of interfering spasticity, ES can be incorporated into a variety of therapeutic strategies to enchance voluntary movement and function.

These activities can be carried into functional tasks such as using the hands, standing, shifting weight from one leg to the other, and walking. All electrical stimulation therapy should be done under the supervision or recommendation of a physiotherapist or any medical practitioner.

Diagnosis of Cerebral Palsy - Understanding Cerebral Palsy

Initial signs for parents to notice, that their infant is not developing normally. Infants with cerebral palsy perform movements slowly, take longer time to roll over, sit, crawl, or walk. When an infant develops comparatively slowly then it is called developmental delay.

Some of the skills that normal infants should have: Holding own head up when lying flat in a bed at 3 months, Sitting and rolling over by 6 months, Walking by 12-18 months, Speaking simple sentences by 24 months.

Earlier most children with cerebral palsy were diagnosed by the time they are two years old. But if a child’s Symptoms was mild; it would be hard for a doctor to make a true diagnosis before the child is four or five years old. With better understanding of patho-mechanism and presentation, now we can make suspicion as high risk baby at the time of birth and definite diagnosis at the age of three month so we can give much better response in these children by early intervention. 

Doctors diagnose cerebral palsy by obtaining a complete medical history of development and examining the child, paying special attention to the child’s movement pattern and associated medical problem like epilepsy, vision, hearing, speech problem& recurrent chest infection. In addition to checking for the most common symptoms

such as slow development, abnormal muscle tone, and unusual posture -- a doctor also has to make sure the child doesn’t have something else that could cause similar symptoms.

Some children have hypotonia, which means that their muscles are too relaxed. In this case, the Childs muscle may appear very flexible. Sometimes a child can have hypotonia that later become hypertonia two to 24 months after birth. Other children have hypertonia which makes their muscles seems stiff. Child can also have fluctuating pattern of tone known as dyskinetic pattern of movement disorder. Children may also have unusual posture or favouring one side of their body.

What’s most important to the doctor is making sure that the child's condition is not getting worse. Although cerebral palsy symptoms may change over time, children with cerebral palsy do not usually lose function at rapid pace. That means, if a child does seem to be losing motor skills, the problem is probably not cerebral palsy. It more likely a genetic or muscle disease, a metabolism disorder, or tumours in the nervous system. A complete medical history, special medical tests, and, in some cases, repeated check-ups can help confirm whether or not the child has cerebral palsy for certain. Usually the diagnosis of cerebral palsy has been made based on the basis of detail medical history and physical examination. Lab investigation is not required in most of the cases. But in few cases with doubtful cases and suspected brain lesion few investigation may required.

With recent advancements in technology and its reach, some time Doctors conduct brain scans (like MRI scans), that allow doctors to look into the brain, can find problems that may be able to be treated.  If it is cerebral palsy, an MRI scan can also show a doctor the location and type of injury to the brain. This test uses a computer, a magnetic field, and radio waves to create a picture of the brain's tissues and structures. Doctors prefer MRI imaging because it offers better detail and does not involve radiation. Other methods may include Cranial ultrasound. 

This test is used for high-risk premature infants because it is the least intrusive of the imaging techniques. However, it is not as effective as the two methods described below at seeing small changes in “white matter” – which is the type of brain tissue that is affected in cerebral palsy.

Computed tomography (CT) scan. This technique creates images that show brain injury. On rare occasions, metabolic disorders can be mistaken as cerebral palsy and some children will require additional tests to rule them out.

• Xray- xray of pelvis and spine are required to see hip and spinal problem

• Blood investigation are required to see metabolic causes and other associated medical problem.

• Genetic analysis is being advised in certain cases to see some genetic cause of neurological deficit.

To confirm a diagnosis of cerebral palsy, a doctor may send a child to other doctors who have specialized knowledge and training or to specialty clinics where these doctors work with a team of health professionals who specialize in working with children with cerebral palsy and other developmental delays. These doctors  Might be child neurologists, developmental paediatricians, ophthalmologists (eye doctors), or otologists (ENT doctors). Additional observations by these specialists can help the doctors make a more accurate diagnosis and begin to develop a specific plan for treatment.

Communication Problems and Dysarthria

Dysarthria refers to speech problems. The child has difficulty producing sound and articulating words. Dysarthria occurs in 40% of patients. The causes are respiratory difficulties due to respiratory muscle involvement, phonation difficulties due to laryngeal involvement, and articulation difficulty due to oromotor dysfunction. Spasticity or athetosis of the muscles of the tongue, mouth and larynx cause dysarthria. It is important that every child is provided with an alternative means of communication as early as possible to avoid further disability.

Oromotor dysfunction

The child has difficulty sucking, swallowing, and chewing. Drooling , dysarthria and inability to eat result in failure to thrive, delayed growth and nutrition, poor hygiene and impaired socialization.

Teeth problems

Dentin : Primary or hyperbilirubinemia

Malocclusion : Spasticity

Tooth : Decay Feeding swallowing problems

Gingival : Hyperplasia Antiepileptic drug use

Gastrointestinal problems and nutrition

There is a general deficiency of growth and development. Children with dyskinesia and spastic quadriplegia fail to thrive. This is related to inadequate intake of food, recurrent vomiting with aspiration secondary to gastroesophageal reflux and pseudobulbar palsy. Difficulties in swallowing (dysphagia), hyperactive gag reflex, spasticity or loss of fine motor control impair feeding. Gastroesophageal reflux and impaired swallowing cause aspiration pneumonia. Many children with CP have high basal metabolic rates. Increase in basal metabolic rate coupled with feeding difficulties cause malnutrition. Malnutrition may be severe enough to affect brain growth and myelination in the first 3 years of life. There is immune system suppression and increased risk of infection.

Respiratory problems

Aspiration in small quantities leads to pneumonia in children who have difficulty swallowing. Premature babies have bronchopulmonary dysplasia. This leads to frequent upper respiratory tract infections. Respiratory muscle spasticity contributes to the pulmonary problems. These problems can be reduced by various chest physiotherapy.

Bladder and bowel Dysfunction

Loss of coordination of bowel and bladder sphincters results in constipation and/or incontinence. Enuresis, frequency, urgency, urinary tract infections and incontinence are common problems. The causes are poor cognition, decreased mobility, poor communication and neurogenic dysfunction. Urodynamic assessment has demonstrated bladder hyper-reflexia, detrusor sphincter dyssynergia, hypertonic bladders with incomplete leakage and periodic relaxation of the distal sphincter during filling.

Constipation is a common but overlooked phenomenon. It causes distress in the child, increases spasticity and results in poor appetite. It is a result of many factors, including poor diet and decreased mobility. Establishing a routine for bowel training and encouraging upright posture help reduce constipation.

Urinary problems may include enuresis, frequency, urgency, urinary tract infections, incontinence. Causes of urinary problems can be poor cognition, decreased mobility, decreased communication skills, Neurogenic dysfunction.

Psychosocial problems

A diagnosis of CP is extremely stressful for the family and the child when he grows up. This causes various reactions ranging from denial to anger, guilt and depression. Coping with the emotional burden of disability is easier if the family has strong relationships, financial security, and supportive members of the community. The child and the family need to find ways to connect to each other. A healthy relationship between the mother and the child forms the basis of future happiness. Prevention or appropriate treatment of associated problems improves the quality of life of the child and the family.

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NeuroDevelopmental Therapy in Cerebral Palsy

NDT is a technique to facilitate the movements which are not present in the Cerebral Palsy children.

Facilitation is a process in which therapist hand gives a direction to the child’s body how to move. In this technique therapist holds the complete control of child’s body weight and the movement. Therapist gives a direction to the body to shifts its COG (Center of Gravity). As the COG shifted to some direction the control over the balance in the child is lost. Therapist should teach the child how to gain control again, by shifting weight to another side. This whole procedure is the part of NDT.

Equipments used in the NDT are Ball, Bolster, or Table/Bench.

Equipments are to challenge the body weight, to help accommodation the structural deformities of the child. These facilitation techniques are used improve the postural control of the child by challenging the posture.

BOLSTER/ROOSTER: is devices which is used to for prone, sitting and sit to stand facilitating techniques. It should be firm, provides a mobile surface that is easy to control because it rolls predictably front to back when child lies prone or sit on it.

BALL: is the most challenging device for the facilitation techniques. It is not firm because it can move in any direction and the base of support is very less.
There are two type of cerebral palsy according to the tone, Hypertonic and Hypotonic. Facilitation is given according to the tone of child. Hypertonic child is given facilitation techniques to decrease its tone and to improve its posture. Hypotonic is given different facilitation to increase the tone and posture.
 

First of all we should check the child’s stage and milestones before taking in the NDT.

Neck holding is the first step which should come in the child if it is absent then just facilitate neck extensors to extend the neck. On bolster put child in prone then ask him to hold the neck in extension by facilitating trapezius muscle or taking the shoulders in retraction. On ball put child in creep/crawl positions then the same facilitation done. Facilitation on the spine can also be done to make spine in extension and neck in hold position.

Quadruped position is the next position attends by the child. In this weight bearing on all four limbs is essential. So the power on all four limb must be good. This posture is attended on the ball. We can make it more challenging by rolling the ball so that the COG of the child’s body shifts and the opposite muscles work to prevent a fall.

Pelvic control facilitation techniques can be done on the ball and bolster both. On the bolster we can make child sit and the roll the bolster to shift COG and facilitate muscles to prevent any fall. Like this we can make child sit on the ball and can move ball too to shift the base of support and facilitate muscles. On ball different positions can be attended like cross sitting, kneeling, sitting by legs open wide on ball, sitting and hands are extended and sitting with hand in overhead position. Rankly decrease base of support and increase pelvic control. In all these positions we can facilitate pelvic muscles by rolling ball, shifting base to either side, ask child to reach an object to either side, make base less supportive by tilting ball on opposite side. All these make the muscles work and repetition of this strengthen the muscles.

The main purpose of the NDT is to improve balance with less support. Facilitation technique is more effective then passive movements. Resistive exercises are difficult in cerebral palsy child because they are not so cooperative and not following commands. So the most effective technique is NDT in the cerebral palsy.

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Classifications of Cerebral Palsy

Cerebral Palsy is an umbrella term that covers a spectrum of different problems including motor control disturbance, epilepsy, vision, speech, hearing and recurrent chest infection. Once the diagnosis has been made on the basis of medical history and physical examination, child physical disability is classified for ease of communication among health professionals as well as for suggesting proper prognosis and treatment. The classification system is based on the physiology of the motor dysfunction, the number of limbs involved and the functional status of the child.

Physiological Grouping - Depending upon type of muscular control loss.

Spastic Cerebral palsy plasticity is defined as a velocity-dependent increased muscle tone, determined by passively flexing and extending muscle groups across a joint. It is the commonest type of cerebral palsy and has much better prognosis than other varieties.

Dyskinesia - Dyskinesia is defined as abnormal motor movements that become obvious when the patient initiates a movement. When the patient is totally relaxed, usually in the supine position, a full range of motion and decreased muscle tone may be found.

 Dyskinetic patients are divided into two subgroups.

The hyperkinetic or choreo-athetoid children show purposeless, often massive involuntary movements with motor overflow, that is, the initiation of a movement of one extremity leads to movement of other muscle groups. b, The dystonic group manifest abnormal shifts of general muscle tone induced by movement. Typically, these children assume and retain abnormal and distorted postures in a stereotyped pattern.

Both types of dyskinesia may occur in the same patient. Simply stated, spasticity you feel; dystonia you see. Ataxias -Patients with ataxia have a disturbance of the coordination of voluntary movements due to muscle dys-synergia. These patients may be hypotonic during the first two or three years of life. They commonly walk with a wide-based gait and have a mild intentional tremor (dysmetria). Mixed Group-- Patients in this category commonly have mild Spasticity, dystonia, and / or athetoid movements. Ataxia may also be a component of the motor dysfunction in patients placed in this group.

Hypotonic – Decrease tone in whole body. This type of cerebral palsy usually converts in athetoid or mixed cerebral palsy as time passes.

Anatomical Grouping depending upon no. and extend of limbs affected

Diplegia-  Diplegia refers to involvement predominantly of the legs. Quadriplegic refers to dysfunction of all four extremities; in some children one upper extremity might be less involved; the term triplegia then would be substituted.

Hemiplegia - Hemiplegia refers to individuals with unilateral motor dysfunction; and in most children the upper extremity is more severely involved than the lower. Finally, an unusual situation may occur, where the upper extremities are much more involved than the lowers; the term Double Hemiplegia is applied to this group of patients.

Functional Classifications of Cerebral palsy Depending upon the degree of functional independence.

• Clumsy Child. Uses no resistive devices (such as crutches), Can walk indoors and outdoors and climb stairs , Can perform usual activities such as running and jumping, Only has decreased speed, balance and coordination.

• Walks Independently. Limited in outdoor activities, Has the ability to walk indoors and outdoors and climb stairs with support of railing, has difficulty with uneven surfaces, inclines or in crowds, has minimal ability to run or jump.

• Uses Assistive Mobility Devices Walks with assistive mobility devices indoors and outdoors on level surfaces May be able to climb stairs using a railing, may propel a manual wheelchair (with assistance needed for long distances or uneven surfaces).

• Severely Limited, Self-mobility severely limited even with assistive devices, uses wheelchairs most of the time and may propel their own power wheelchair.

• No Self-Mobility.  They have physical impairments that restrict voluntary control of movement.

The ability to maintain head and neck position against gravity is impaired in all areas of motor function, children cannot sit or stand on their own even with equipment, cannot do independent mobility; though may be able to use a powered Wheel chair.

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What are The Botulinum Toxin in Cerebral Palsy

Clinical use of botulinum was recognized by Bergen et al. in 1949 by identifying the effect of botulinum toxin on neuromuscular transmission. Once it was thought to be potent biological toxin to human body, now its potential is being utilized as therapeutic agent for different clinical problems specially spasticity & cosmetic purpose. In all variety of botulinum toxin, type A is used for the clinical application. This is being utilized in Mx of cerebral palsy since 20 years.

This toxin is used as intramuscular injection at most condensed site of neuromuscular junction of affected muscle. This toxin block acetylcholine release from neuromuscular junction & causes local temporary chemo-denervation and effect last for 4-6 month. Even after disappearance of drugs, some modulation of nerve transmission has been seen, so effect persist beyond the time limit and dystonic posture also get corrected due to slight diffusion of drugs in systemic manner.  It is being commonly used for spastic & dystonic cerebral palsy Treatment.

Cerebral Palsy Treatment

Dose & safety margin depends upon brand of botulinum toxin, number of muscle and wt of child. Total maximum dose is about 10-12 unit per Kg of body Wt. it is being used in all affected spastic muscles and usually at multiple site (3-4 in each muscle). 

Effect of botulinum toxin can be prolonged with post injection plaster, proper patient & muscle selection, injection under anesthesia and post injection braces and good therapeutic protocol. Plaster is given for only 7-10 days then patient is advised braces and therapy.

It is very effective when child have dynamic spasticity between the age group of 2-5 years and not so useful when child develop contracture and deformity. Botox injection is advisable when Spasticity is interfering in therapy programme and muscle excursion become too less.

Usually botulinum toxin injection are used in all affected muscle in a single setting and it is better to give it under anesthesia, because it require multiple injection puncture and is painful and it also save the toxin from wastage. Most commonly botulinum toxin is used in Gastrocnemius, Hamstring, Rectus Femoris, Adductor, Pronator Teres, and Flexor Digitorum.

Injection can be repeated every three monthly but if proper postoperative therapeutic protocol has been taken into consideration it may not be so frequent. At Samvedna, we rarely require repetition of toxin due to good therapeutic protocol and use of braces. It reduces pain, facilitate therapy, better cooperation of child, better functional and motor gain.

By the use of toxin, OSSCS Surgery also can be delayed till the maturity of gait. Antibodies to toxin have been one of the possible problems with repeated injection that can interfere in next injection of toxin.  Otherwise it is well tolerated. Side effects are very -2 rare.