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What's New in Cerebral Palsy

It is becoming more evident that difficult labors and post-natal asphyxia may be the result of defective infants rather than the cause of them.

THE CARE OF THE HANDICAPPED CHILD is one of the major medical and social issues facing our society today. Huge strides have been made in technology, treatment, and recognition of psycho social problems, leading to the ultimate goal of integrating these individuals as useful and productive members of society. This progress is well illustrated in our current approach to cerebral palsy.

Etiology
Cerebral palsy was first described by William John Little of England in 1843. He related this condition to asphyxia from birth problems. It has taken many years and intensive epidemiological studies to overcome the popular notion that the major cause relates to the birth process. A significant prospective study was carried out in this country in the 1960's and 1970's in which 50,000 individuals were followed from the onset of pregnancy to age seven. This study revealed that ten percent or less of cerebral palsy could be attributed to problems of oxygenation at birth. It is becoming more evident that difficult labors and post natal asphyxia may be the result of defective infants rather than the cause of them. More and more of these babies are found to have congenital malformations of the brain on CT scan.

Prematurity is now recognized as a major risk factor. Although the majority of premature infants develop normally, the risk of cerebral palsy is 27 times higher in infants who weigh less than three pounds at birth compared to those who weigh five pounds or more. The cerebral arterial system is very fragile in this group and transitory increases in blood pressure often cause hemorrhage into the region around the third ventricle. If this hemorrhage is extensive, it results in periventricular leucomalacia in the region of the internal capsular leg fibers causing spastic diplegia.

In some instances, it is possible to identify the etiology of C.P. such as in genetic syndromes, congenital malformations, and in utero infections. There is a high risk factor in premature infants and those with prolonged low Apgar scores. However, in the majority of cases, no specific etiology can be found. We can probably diminish the incidence of cerebral palsy by decreasing the occurrence of prematurity and perinatal asphyxia.

Incidence
The incidence of cerebral palsy in the Western World is approximately two per thousand live births. This has not diminished despite more wide spread use of fetal monitoring and the proliferation of technologically advanced neonatal intensive care units. Many high risk infants who formerly died are now surviving with cerebral palsy, and many who formerly survived with cerebral palsy are now emerging neurologically intact. In fact, the incidence of cerebral palsy is lower in Third World countries because fewer of their high risk infants survive.

Despite the failure of medical progress to diminish the incidence of cerebral palsy, there have been profound changes in the spectrum of this condition. With the elimination of Rh incompatibility, the occurrence of athetoid quadriplegia has greatly diminished. With improved neonatal care, there are fewer globally involved patients attributable to hypoxia. We are seeing more spastic diplegics secondary to prematurity. The pendulum appears to be swinging toward children with less severe and more treatable types of cerebral palsy.

Treatment
Communication, independence in self care activities, and mobility are considered by patients to be more critical to their function than ambulation. Because of the multiplicity of problems associated with C.P.. a multi disciplinary clinic is the optimal setting for treatment of this condition. The Newington Children's Hospital C.P. Clinic includes orthopaedics, neurology, pediatrics, rehabilitation services, orthotics, and social services with accessibility to speech and hearing, ophthalmology, psychology, and educational evaluations. It is comforting for C.P. patients and their parents to have all their needs addressed at one time and in one place by experienced and caring professionals.

Communication problems in C.P. are receiving great attention. Recent advances in computer technology have enabled many children to write or actually speak through machines with programmed responses, utilizing the most minimal physical abilities such as eye movements and breathing. This has opened new vistas to the cognitively adept patient who is locked into a severely handicapped body. We can predict that this group of individuals will be much more productive members of society.

Orthotics have been widely used to prevent deformity and enhance function. Light weight plastic materials improve cosmesis and are less burdensome to the patient. Very few above knee orthoses are used today. Knee joint extension is achieved by utilizing the plantar flexion knee extension couple of floor reaction orthoses. More articulated ankle AFO's are being prescribed to allow a closer approximation to normal gait. New technology produces customized seating devices for the severely handicapped which lessen the risk of skin irritation and allow maximal upper extremity function from the upright position.

The role of physical therapy is becoming more defined. There are some recent prospective studies showing no difference between groups receiving early P.T. and those with no P.T. Or early intervention programs. However, P.T. is very useful for parental guidance, addressing equipment needs, prevention of deformity, post operative rehabilitation, and maintenance of operative gains. Selective dorsal rhizotomy is the latest neurosurgical procedure being utilized in the treatment of a segment of the

C.P. population. Increased muscle tone results from a combination of over-stimulation of the anterior horn cell from the muscle spindle and diminished inhibitory control from the cerebral cortex. By resecting the sensory fibers within each lumbar and upper sacral dorsal root showing hyperactivity on intraoperative EMG exam and sparing those showing a normal response, muscle tone is diminished, but not lost. No loss of peripheral sensation has been reported and the early results have bee encouraging in the C.P. patient whose function is being hampered by overwhelming tone or spasticity. It is contra-indicated in patients with athetosis, rigidity, and underlying weakness. It will not correct contractures and angular deformities already present.

Medications play a very limited role in treatment except for seizure medications. Valium is quite useful in managing perioperative spasm. No agent has been shown to have long term tone reducing ability.

Center for Motion Analysis

The Center for Motion Analysis at Connecticut Children's Medical Center is world-renowned for its involvement with the study of cerebral palsy problems.

Gait analysis is the most exciting new modality to arrive on the scene in many years. In a state of the art gait analysis laboratory, such as the one at Connecticut Children's Medical Center, three dimensional kinematics, EMG's, and kinetic or force and moment analyses can be produced through appropriate computer software in a very short period of time. This allows the experienced gait analyst to assess the physical exam, video analysis, along with the above studies and formulate an appropriate, objective plan of treatment for the individual being evaluated. Post operative studies document the results of surgery and allow an objective assessment of various treatment methods. The gait laboratory at Connecticut Children's Medical Center is world-renowned for its involvement with the study of cerebral palsy problems.

Finally, orthopaedic surgery is still necessary to correct contractures, correct bony deformities, and re-balance abnormal muscle forces producing gait deviations. Operative procedures have evolved. Muscle releases often resulted in functional weakness or over correction, producing a deformity opposite to the one initially addressed. Hence, we now perform more muscle lengthening and split tendon transfers to achieve balance around a joint. Kinetic studies in the gait laboratory demonstrate the importance of the ilio-psoas and the gastrocnemius as power sources for the swing phase of gait. Thus we are more apt to spare these muscles or to perform very minimal lengthening. Osteotomies are secured with stable infernal fixation to avoid postoperative immobilization and functional regression. Hips are not allowed to dislocate and progressive scoliosis is surgically stabilized to maintain sitting ability and optimize function.

We have a better understanding of the interdependence of the joints of the lower extremity and the severe psycho social trauma of multiple hospital admissions. Most of the involved lower extremity muscles span two joints, i.e. gastrocnemius, hamstrings, rectus femoris, and psoas. Thus, operating upon one joint will produce an effect on the adjacent joints. Because of this, we now attempt to perform all our corrective surgery, bony and/or soft tissue, during one surgical episode, often utilizing two teams of surgeons for bilateral cases to shorten the operative time. We can balance all the joints of the lower extremity, avoid multiple hospital admissions, and allow for an uninterrupted period of rehabilitation when the entire limb can be treated definitively. This also eliminates multiple periods of functional regression that normally follow each surgical procedure.

The C.P. patient and his family are best served by a multidisciplinary clinic where all needs and problems, physical and emotional, can be addressed at one time by one group.

Summary
The etiology of cerebral palsy appears to be less related to hypoxia at birth than was once thought. The type of involvement has been shifting from the severe quadriplegics, such as seen with Rh incompatibility, to the more treatable spastic diplegics, often seen in those born prematurely. Despite improved perinatal care, the incidence of C.P. has not declined, but actually may be rising in the Western World.

The priorities of C.P. patients are more widely recognized and society is becoming more responsive to their needs. New light weight orthoses enhance functional capacity. Seating devices and computerized speech aides have become more sophisticated. Selective dorsal rhizotomy shows initial promise in helping to control overwhelming tone. Orthopaedic surgeons are recognizing the need to balance joints and eliminate multiple hospital admissions by performing all surgical procedures at one time. Gait analysis is of great value for preoperative planning and postoperative assessment. And, lastly, the C.P. patient and his family are best served by a multidisciplinary clinic where all needs and problems, physical and emotional, can be addressed at one time by one group. 



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