Thursday, September 19, 2013

Cerebral Palsy-Advanced Interventions

Cerebral palsy includes various kinds of disability, such as difficulty in coordinate and alternative movements, abnormal movements and postures, and difficulty in keeping the body in antigravity postures or upright posture. Hence, manifestations of cerebral palsy are not all the same. Since these motor impairments are due to damage of the central nervous system, extraordinary difficulties have been clinically presented in the control of hypertonicity, in facilitation of the paralyzed muscles, and in restoring balance. These motor disabilities are summarized into hypertonicity including Spasticity, rigidity, athetosis involuntary movement, paralysis (weakness) of the propulsive and antigravity muscles, and difficulty in maintaining equilibrium.

Recently, various types of Spasticity-control methods are being advocated. However scientific they are, there are many problems including grave disadvantages.

Physiotherapy and occupational therapy inhibits hypertonicity, and facilitates voluntary movement and equilibrium. These therapies seem to have played an important role in controlling these factors. However, hypertonicity and fixed deformities in cerebral palsy children more than 3 years old are too complex to treat. It is almost impossible to alleviate hypertonicity permanently with therapeutic exercises alone. It has been mentioned in the books that parents must aware that Spasticity of their children will be forever. Therapy alone cannot control Spasticity with long lasting effect.

Stereotaxic thalamotomy is a Neuro-surgical procedure, which was used to control rigidity and involuntary movements due to cerebral degeneration. The main indications of this procedure are to control tremors, dystonic movement, and rigidity. Hence the procedure has been applied to reduce involuntary movement and rigidity in cerebral palsy, but with varying responses. They cannot alleviate Spasticity, which is the predominant feature of cerebral palsy. Therefore, the indications for stereotaxic thalamotomy are definitely limited to patients, having involuntary movements and rigidity due to the damaged extra pyramidal tract.

Oral medication has been used for reduction of Spasticity. It is useful not only for relieving Spasticity but also is helpful for relieving pain especially in adult patients. Importance of oral medication should never be ignored. However, it is also true that this is not used without side effect. Disadvantages include decrease in mental vividness, and weakening of respiratory function with decrease in respiratory volume, depending on the dosage of oral medication. Hence, to provide and reserve vividness for CP patients, considerations on how to minimize the use of this oral medication would be needed, substituting oral medication with other methods.

Intrathecal Balcolen therapy is another type of medication therapy. It is reported that this therapy reduces Spasticity in various levels of patients including the totally involved quadriplegic patients, as well as ambulatory diplegic patients. Disadvantage of Intrathecal Baclofen therapy are a possibility of intradural infection and fibrosis, causing some adhesions around the spinal cord and roots, the need for repetitive replacement of the pump and tube, and need to refill the pump much often. Another disadvantage is the possibility of respiratory depression due to inhibition of the nerves to the intercostal muscles. Hence, to preserve a healthy life, breathing difficulty should be avoided, even if it is to a minimal extent.

Use of Botulinum toxin injection is also popular. This injection seems to be very effective in reducing Spasticity. However, the shortcoming of Botulinum injection is that its effect is not long lasting and therefore frequent injections are needed. Another rare possible shortcoming is that breathing difficulty will be inevitably caused, depending on the dosage of injection and it can become fatal and dangerous for patients if it is used in multiple sites in sufficient amounts.

Selective dorsal rhizotomy (SDR) is a neurosurgical approach and is gaining popularity. Many papers of the effect of SDR on reduction of Spasticity are presented. It seems certain that this approach is very effective in reducing Spasticity and for weakening muscle power, while increasing the range of motion in gait analysis. However, one of the major problems of SDR could be loss of antigravity stability, due to loss of activity of the antigravity muscles. There is no way of differentiating the nerve fibers to spastic muscles and the ones to antigravity muscles. In the literature, there are reports of increased range of motion, but reports of improvement in antigravity stability are few. Reports of postoperative deformities such as severe lordosis, gross increase in anterior tilt of the pelvis, severe pes valgus deformity and frequent worsening of dislocation of the hip suggest the loss of antigravity stability around the joints and worsening of motor function. Another serious drawback is that fibers of sensory nerves are sectioned unselectively in this surgery. SDR induce injury of sensory and sympathetic nerves. It is not documented that sensory nerves can be selectively differentiated from the afferent fibers at the rootlet level. This means that sensory nerves get sectioned unintentionally and that the damage depends on the extent of the section. More detailed scientific study would be needed, before they are advocated as an antispasticity therapy in patients with cerebral palsy.

Orthopedic surgery has been considered to have serious limitations in achieving functional improvements. It was used as an adjutant to assist of other approaches and to restore normal alignment, correcting contractures and deformities, and acquiring stability by arthrodesis. It has been considered that orthopaedic surgery cannot control Spasticity, acquire reciprocal movements, to facilitate antigravity muscles, and improve skills and voluntary movement of the hand. Recently, restoration of muscle balance has been tried, with use of muscle releases and tendon transfers. However, loss of antigravity activities after release of antigravity muscles has presented serious problems. The adductor tenotomy with or without anterior obturator neurectomy is one of them. They have been recommended, and have been indispensable procedures for correction of adduction deformity of the hip in some cases. However, careful observations revealed that adductor release including tenotomy of the adductor longus and brevis are likely to destroy antigravity activities of these adductors and induce deteriorating gait without stability. Most physiotherapist and occupational therapist seem to hesitate to recommending this surgery, because postoperatively the stability of the gait of the patient has been somewhat lost.
Iliopsoas recession or tenotomy is effective procedures planned to relieve Spasticity of the hip flexors and to correct flexion deformity of the hip. However, in literature recently, loss of power in hip flexion after total division of iliopsoas has been reported, and hence selective release of the psoas muscle (Concept of OSSCS) has been recommended. Achilles tendon lengthening and heel-cord advancement are popular procedures for correcting equinus deformity in cerebral palsy, and have been the recommended procedures. However, it is a question, if these procedures are evaluated objectively as reliable, by unbiased and introspective orthopedic surgeons. Serious loss in supporting activity of the foot has been reported in cases with spastic diplegia or spastic quadriplegia, after heel cord lengthening and after heel cord advancement. With disillusionment due to poor results after standard orthopedic surgery, many orthopedic surgeons are apt therefore to consider that physical mechanism of the cerebral palsy is completely different from that of normal human body. Many orthopedic surgeons have abandoned avoiding getting involved in treatment of cerebral palsy. Nevertheless, in spite of these desperate situations, many attempts have been pursued in the field of orthopedic surgery all over the world. Various progresses have been made and reported, and various improvements have been presented.

Our approach for control of Spasticity is OSSCS – Orthopedic Selective Spasticity Control Surgery. In the process to overcome these serious problems after the treatment for hip-adduction, hip-flexion deformity, and equinus deformities, it has been confirmed that the multiarticular muscle work differently from the monoarticular muscles in the human body, although they mostly co-exist in the same place. It was also noted the fact that the multiarticular muscles are more hyperactive and are more responsible to hypertonic posture. Contribution of the biarticular muscle to spastic condition in cerebral palsy is already mentioned. It has also been noticed the fact that the monoarticular muscles are closely related to the antigravity activity, which keeps body in an upright position. Thus, the consistency of the muscle activity and the motor functional characteristics of the hypertonicity of cerebral palsy have been clinically analyzed. Here, clinical approvals and applications have been made on the deformities in the cervical spine, thoraco-lumbar spine, shoulder, elbow, hand and fingers, and even for abnormal postural deformities, based on the clinical, anatomical, functional and biological analysis. On the basis of these consistent findings, the idea of orthopedic selective Spasticity-control surgery (OSSCS) was initiated and hereby confirmed that this idea can be applicable not only for control of Spasticity but also to control athetosis movement in any part of the body.
We now reach to the point of fundamental importance that the abnormal postural reflex is only a combination of individual Spasticity and athetosis in the neck, trunk, upper extremities and lower extremities. It becomes obvious that, by relieving Spasticity in these individual parts of the body at each level, these abnormal postural reflexes can be relieved and controlled. Thus, through careful analyses of muscle function and hyper tonicity in each joint, a working concept of orthopedic selective Spasticity-control surgery has been formulated, and confirmed at each clinical case study.
This treatment is also effective for reduction of severe postural abnormality, such as tonic labyrinth reflex and asymmetric tonic neck reflex. With simultaneous release of spastic or athetotic flexors and extensors in each joint, rigidity of the joint can now be lessened and therefore relaxation of the joint can be achieved. With relaxation of the each extremity, the individual movement of each extremity becomes easily feasible and therefore, alternation such as reciprocal movement and cross-patterned movement of the extremities is also facilitated. Here, we can now understand that poor coordination is caused due to difficulty in relaxation of both individual multiarticular flexors and extensors at each joint. Now, we can activate coordinate and alternate movement, while decreasing abnormal movements and postures. We can achieve more stable postures such as sitting and standing, and stable transfers such as crawling and gait by facilitating activity of the antigravity muscles. It has been well documented that orthopedic surgery cannot control Spasticity, but only weakens the muscle. It has been also mentioned that orthopedic surgery can only correct deformities and treat dislocation. However, situation is changing. If we can analyze Spasticity, local reflexes, abnormal reflexes in cerebral palsy in motor-function-wise, and can understand whole movement of cerebral palsy and non-paralyzed body in motor-function-wise, and in motor-developmental point of view and we will be able to control Spasticity effectively with the use of OSSCS. Orthopedic Spasticity control surgery provides us enormous possibilities of controlling Spasticity in the entire human body with cerebral palsy, without losing any useful activities, while facilitating mental and physical activities. By combining the Spasticity control surgery with conventional orthopedic surgery, such as open reduction, VDRO, pelvic osteotomy for dislocation of hip, arthrodhesis of wrist and ankle, spinal fusion, we can deal with wide range of problems in motor activities and activities of daily living with encouraging results while providing a new path for functional improvement and for active life styles in most patients with cerebral palsy. OSSCS can provide us with an opportunity to assist physiotherapist and occupational therapist by controlling hypertonicity in the whole body while making it easy to facilitate basic motor functions such as rolling, crawling, sitting, kneeling, standing and independent gait.
The fundamentals of OSSCS are clear and uncomplicated and are based on the physical and biological findings. OSSCS is based on the concept that muscles are classified into two types – monoarticular and multiarticular. Electromyographic studies proved that multiarticular muscles are spastic or hypertonic and are responsible for various deformities in cerebral palsy and the monoarticular muscles are normal and responsible for joint stability and erect posture in human being. Thus by selectively releasing these multiarticular muscles we can activate the monoarticular muscles to facilitate function and erect posture.
The most important aspect of OSSCS is postoperatively there is no decrease of motor function as monoarticular muscles are preserved. There is no loss of sensations as seen in many Spasticity reducing procedures. The technique is quite reliable and promising procedure for control of Spasticity.
Another important aspect of OSSCS is that its indications are wide and hypertonicity in the entire body can be relieved. All kinds of hypertonicity – Spasticity, rigospasticity and athetosis can de corrected.
The decision making and treatment techniques are demanding. In order to achieve successful results, orthopedic techniques should be skillfully conducted by trained and experienced orthopedic Surgeons trained in Pediatric Orthopaedics, since we are going to correct complex motor disorders which have not responded to other procedures. Which muscles should be released? Which end should be released proximal or distal? What kind of release should be done? Intramuscular lengthening? Sliding lengthening? Z lengthening? Tenotomy? How much and how long these lengthening should be done? What other conventional orthopedic surgeries should be combined? All these questions and problems have to be answered correctly and there is no scope for error.
When the Orthopedic surgeon applies these principles of OSSCS accurately in the management of cerebral palsy, he will be able to promise definite improvements for patients with cerebral palsy. Here, we would like to present our experience with OSSCS for the last 13 years and show how these approaches can be used in the treatment of cerebral palsy.