Rehabilitation and Orthopedic Interventions in Children with Cerebral Palsy
from School Health Reporter, Fall 2005
Cerebral palsy (CP) is a non-progressive injury to the developing brain. The injury affects a child’s motor skills. The incidence of CP remains steady at one per 1,000 despite the advances in technology over the last 20 years.
The etiology of CP is varied and includes prenatal or perinatal infections, intrauterine stroke or prematurity. Fifty percent of all children with CP were born before 36 weeks gestation. The majority of children with cerebral palsy have spastic-type CP; fewer children have dystonic/dyskenetic or hypotonic types. The involved extremities also help to define the type of CP. Hemiplegic CP affects one side of the body; diplegic CP primarily affects both legs; and quadriplegic CP affects all four extremities. Children with CP can have many associated problems, including feeding issues, visual difficulties, seizures, gastroesophageal reflux, and impairments in gross motor and fine motor skills and speech and language. The focus of this update is to review rehabilitation and orthopedic interventions used to improve the quality of life of a child with CP.
Children with CP receive specialty care related to their disability in a CP clinic, found in most tertiary-care pediatric hospitals. A multidisciplinary team including pediatric rehabilitation physicians (physiatrists) and orthopedic surgeons see these children. This interdisciplinary clinic approach provides non-surgical and surgical options for children and their families.
Spasticity Management
Spasticity is defined as velocity-dependent tone, which means that when an examiner provides a quick stretch to a muscle, resistance is felt. Spasticity can impact a child’s motor skills and also can lead to development of joint contractures. Because of the functional and structural impacts that spasticity can have on a child with CP, a coordinated effort between medical and surgical specialists is critical. The management of spasticity must begin with an assessment of how the increased tone may be interfering with function, range of motion, comfort, and daily care activities such as dressing and hygiene. The ultimate goal of treatment always should be improving function. But the reality is that in a child with severe impairments, the sole goal of treatment may be to make it easier for caregivers to bathe, dress and perform perineal care. Once the child and family goals are identified, it is important to determine the tone pattern. Some children have generalized tone, which affects all of the extremities. On the other hand, a specific muscle or group of muscles may be negatively impacting function. For example, a child with hemiplegic CP, affecting only one side of the body, may have spasticity in their gastrocnemius muscle (calf muscle), which causes a toe-walking pattern on the side that is affected.
Therapeutic Interventions
The first line of treatment in managing spasticity is an aggressive stretching program that will serve to maintain range of motion. A daily program in the home will provide a more effective treatment than weekly stretching done in a therapist’s office. It is critical for the physical or occupational therapist to provide instruction and guidance to caregivers to ensure that the program is being done safely and effectively. Use of equipment such as a stander also may provide a static stretch to a child with spasticity. When spasticity is interfering with range of motion, the therapist may consider doing serial casting to an extremity. The process of serial casting involves placing a cast on an extremity for approximately one week. After the initial cast is removed, the limb is stretched again, and a second or even third cast may be applied to gain more range of motion. Casting will temporarily decrease the underlying spasticity. When a cast is placed on the lower extremity, a child is allowed to walk and participate in normal activities at home and in school. It is unusual for the child to complain of pain in the casted limb. However, if there are complaints, it may be necessary to have the cast removed to examine the skin for pressure sores.
Injections
The use of local injections with either botulinum toxin (Botox or Myobloc) or phenol is an effective way to decrease tone in specific muscle groups. When a child, parent or therapist can point to a specific muscle as interfering with function such as reaching for objects, injections are an excellent treatment option. In addition to improving function, injections can be used to improve range of motion or maintain hygiene. For example, a child with severe finger flexor tone causing skin breakdown on the palm of the hand may benefit from botulinum toxin injections to the finger flexor mass in the forearm.
Botulinumtoxin is derived from the toxin produced by the bacteria clostridiumbotulinum. This medication is effective for approximately three to four months. Phenol is a medication that is injected at the nerve level and causes denervation of the nerve. The nerve is identified by using a hand-held nerve stimulator that causes a muscle contraction when the needle is on the nerve. The duration of action of phenol is typically four to six months. Frequently a combination of botulinum toxin and phenol is used inone visit as it allows the physician to target more muscle groups. Phenol is painful when injected, so the injections are typically performed in the operating room under general anesthesia. When necessary, additional procedures also may be performed, such as dental work, ophthalmologic or orthopedic surgeries, to limit a child’s exposure to anesthesia. If injections are the sole procedure, the child can be expected to return to school the next day without discomfort. Caution against overstretching the muscles for the first few days after injections should be observed at home and at school. Most children can resume normal activities, but active stretching of the child’s injected extremity by a parent or therapist should be avoided.
Medications
An effective treatment for generalized spasticity is the use of oral medications such as baclofen and diazepam. Both medications act centrally to provide generalized tone relief, but the side effects of both medications can limit their success. Before the reduction of tone is observed in the spastic limbs, truncal hypotonia, sedation, confusion, or increased drooling may be noted. Given the relative short half-life of both medications, children may need to receive a dose of medication during their school days.
The use of intrathecal (the space surrounding the spinal cord/nerves) baclofen delivered by an implanted medicine pump is an effective way to deliver baclofen without the side effects described above. A pump the size of a hockey puck is implanted under the skin of the abdomen. The pump is attached to a catheter that is tunneled around to the child’s back; the catheter is inserted into the the cal space in the thoracic region of the back. A continuous infusion of baclofen is delivered directly into the cerebral spinal fluid at very low doses, causing marked decrease in spasticity. Because small doses are administered, the side-effect profile is more acceptable to children and their families. Complications related to the implantations such as infection are a risk. Other risks are dislodgment of the catheter or pump malfunction, which can lead to acute withdrawal of baclofen. Symptoms of acute baclofen withdrawal include a marked increase in spasticity, irritability, and itching without a rash. The family should have access to oral baclofen in the event of acute withdrawal.
Surgical Interventions
Despite aggressive range of motion and judicious use of oral medications or injections, children with cerebral palsy can develop musculoskeletal deformities including joint contractures or spine deformities. These boney abnormalities can affect function including gait, postural stability, comfort, hygiene and cosmesis. Surgical interventions can correct the deformities, but goals and expectations of surgery need to be identified prior to proceeding. For example, a child with plantarflexion contractures (ankles pointing downward) who is ambulatory may benefit from a surgical muscle/tendon lengthening to improve gait, while a child who is in a wheelchair may benefit from the same surgery to allow him or her to assist with stand-pivot transfers. A child with hip adductor contractures (limited ability to open legs up) may benefit from a surgical lengthening with the goal of improving the caretaker’s ability to diaper the child with greater ease. Casting post-operatively varies by the type of surgery performed. When the cast is placed below the knee, the child is permitted to ambulate once pain is well-controlled. Surgical lengthenings, if done without any boney surgery, is an outpatient procedure; the child can return to school as soon as two or three days post-operatively. Surgical interventions of the upper extremities also can be performed to improve range of motion, hygiene and function.
Non-ambulatory children with CP have a high incidence of hip dysplasia, which may result in hip subluxation and dislocation. Muscle imbalance and increased tone lead to hip flexion and adduction, putting abnormal stresses on the femoral neck and acetabulum. Children followed in a CP clinic will have serial pelvis films to monitor the status of the hips. There are no restrictions in activity including weightbearing when there is evidence of dysplasia/subluxation. A subluxation with a progression of greater than 50 percent indicates the need for surgery. A varus derotational osteotomy (VDRO) is performed by an orthopedic surgeon with an approximate five-day hospital stay. Children are casted in a full spica cast from their nipple line to their toes for six to eight weeks. Return to school in the spica cast is appropriate once the child’s pain is under control. A rental reclining wheelchair is prescribed for the child to accommodate the large cast and hip abducted (frog-legged) position required for post-operative immobilization.
Spine abnormalities in children with CP can lead to difficulty with sitting posture and tolerance. School personnel are frequently the first to identify progressive scoliosis because of the decreased sitting tolerance in the classroom. Spine X-rays are obtained to document the degree of the curve; the child is then followed closely to assess the speed at which the curve is progressing. General treatment recommendations include bracing between 20 and 50 degrees and a spine fusion when the curve progresses beyond 50 degrees. The amount of time a child is in the brace each day varies between clinicians. Care should be taken when the child is in the brace all day during school that the skin beneath the brace is not developing pressure sores. Children who undergo spine fusions are out of school for several weeks, and upon their return may have reduced endurance and poor sitting tolerance. Home-based schooling may need to be arranged for a short-term basis in some situations.
Therapy
Children with CP participate in school and/or community-based therapy programs including treatment with physical, occupational and speech-language therapists. The frequency of visits depends on the age of the child and the goals being addressed. Following injections or a surgical procedure, the frequency of therapy may be two to three times a week, while an older child whose goals are to maintain range of motion may require monthly visits by a therapist to insure that caregivers are comfortable with the home exercise program. The role of school-based physical therapists may include gait training, assisting classroom teachers or para-professionals to facilitate an appropriate standing program, or to instruct them in performing appropriate transfers in or out of wheelchairs. The role of the occupational therapist in the school setting may be to address fine motor control as it impacts tasks such as handwriting.
The use of a gait and movement analysis laboratory has allowed physical therapists, rehabilitation physicians and orthopedic surgeons to objectively assess a child’s gait and make non-surgical and surgical recommendations for improvement of function and efficiency of gait. Recommendations may include a change in orthotics, the need for injections or oral medications to manage spasticity, and/or surgical interventions such as tendon lengthenings, tendon transfers or osteotomies to improve alignment of the lower extremities. Post-operative care including casting regimens, weight-bearing precautions and therapy needs is dependent on the interventions performed.
Conclusion
Given the complexity of children with CP, an interdisciplinary approach to care is critical, with input from children, families, therapists and physicians to accomplish the goals of improving range of motion, function, and ultimately quality of life. School personnel are important in helping to identify specific goals that may improve participation and success in the school setting. Both non-surgical and surgical rehabilitation and orthopedic interventions are expected in the life cycle of a child with cerebral palsy.
