Introduction
Cerebral Palsy
Cerebral palsy is a physical disability that results from damage to the brain during development that negatively impacts the functional development of children14. Characteristics of cerebral palsy include an inability to control the body’s motor functioning with a potential to affect speech, learning, environmental exploration, and the skills required for independent living. In addition, these patients have delays in reaching milestones for gross motor development with problems moving such as weakness, spasticity, and impaired selective motor control27. Children born with the disability have varying levels of impairment, and while it usually develops by age three, it is non-progressive. The incidence of cerebral palsy is approximately 2 in every 1000 births19, and between 25 percent and 75 percent of the patients manifest the symptoms of painful hips3.
Hip Dysplagia
Over the course of time, most of children with cerebral palsy will develop secondary problems referred to as “progressive musculoskeletal pathology”7. The health issues include contractures of muscle-tendon units such as those that cross two joints; the gastrocnemius muscle, the iliopsoas in the abdomen and thigh, and hamstrings are examples. Bony torsional deformities affect many children, especially external tibial torsion and femoral neck anteversion2. Subluxation causes deformity of the femoral head16. In addition to pain, joints may become unstable, there is pain walking or even sitting, and commonly hip displacement occurs8. With subluxation and hip dislocation, quadriplegia is often seen in patients with cerebral palsy6, 13, 20; incidence ranges from 2.6 percent to 75 percent3, 20, 18.
Hip Arthroplasty
Traditional nonsurgical therapies such as medication, bracing, and exercise are not effective for cerebral palsy patients with hip pain22. The use of pelvic or femoral osteotomies or soft-tissue releases may be preventative therapies33. Further dislocation may be prevented with early surgical reduction28. The goal of treatment in hip location is to create a mobile hip for pain-free sitting upright and easy hygienic care. Unfortunately, once the femoral head has become deformed, surgical reduction may not prevent pain and osteoarthritis22. In the past, excisional arthroplasty was performed, removing the involved joint4. However, short limbs and poor functioned was a result; for patients without the ability to ambulate, this complication did not detract from a satisfactory result5.
An alternative surgical intervention for hip dislocation in cerebral palsy patients was to replace the joint using hip resurfacing21. However, hip replacement for cerebral palsy patients is high risk with influences of fractures secondary to abnormal bony anatomy and spasticity involvement16. The use of single-event multilevel surgery (SEMLS) is a possible surgical process for high risk patients with secondary musculoskeletal problems where between 4 and 20 different orthopedic procedures are performed during one operative session31. This allows the patient and family to endure only one hospital stay and one period of rehabilitation. Two surgical teams work together to use implants, perform techniques, and employ bone-graft materials.
The procedure of resection arthroplasty is a suggested treatment for cerebral palsy patient with a painful hip dislocation secondary to spastic quadriplegia1. The surgery is primarily used for nonambulatory patients, but if an inadequate amount of bone is removed, the patient may experience pain for up to 14 months after the operation34. Other reconstructive alternative for hp arthrosis in cerebral palsy patients are arthrodesis, prosthetic replacement, valgus osteotomy, and Chiari osteotomy22. Arthorodosis, fusing two adjacent bones to prevent mobilization, is generally used in pediatric cerebral palsy patients demonstrating unilateral hip disease and is not an option if there is bilateral hip involvement or an abnormal lumbosacral junction is present20.
Surgical Outcomes
There have been several recent reviews of surgical practices and rehabilitative outcomes for cerebral palsy patients undergoing treatment for hip dislocation. Thomason and Graham (2013) discussed the first randomized controlled trial of SEMLS was performed at the Melbourne Royal Children’s Hospital31. The trial reported conducting evaluation of gait using the Gillette Gain Index and gross motor function using the GMFM-66; the researcher concluded there was a 57 percent improvement in gait and 4.9 percent improved gross motor function. It was particularly noted there was an outlined rehabilitative program post-operatively. While the children’s function were improved, orthopedic surgery still results in less gross motor function, loss of independence, and weakness.
In a study of 539,372 primary his replacements performed on English cerebral palsy patients between April 1, 2003 and December 31, 2012, King et al. (2013) reported a high degree of patient satisfaction and a low risk of mortality with total hip replacements; however, the rates for revisions were twice as high as for patients who did not have cerebral palsy. The researchers state this is the largest number of patients and data used to 2013; Raphael et al. (2009) work with 59 cases and only these two studies under a comparison group.
Rehabilitative Therapy
Pre-Planning Stage
Most cerebral palsy patients who undergo any of the surgical interventions for hip problems are already under the care of a health care professional if not a rehabilitative therapist32. Therefore, the first step in the rehabilitation following surgery is preoperative planning. The optimum case management uses a multidisciplinary approach with a care team to promote maximum function and independence while decreasing the possibility of the progressive musculoskeletal deformities seen in cerebral palsy patients9. Some pediatric patients will have experienced interventions to promote management of spasticity such as intrathecal baclofen or dorsal rhizotomy17; botulinum neurotoxin type A injections are also common, combined with casting, orthoses, and enrollment in a physiotherapy program within the community. Preoperative planning for surgery begins when gross motor functions and gait begin to plateau, the injections show less effect, and fixed contractures become more apparent. This generally takes place between the ages of 5 and 827. Preoperative planning provides a far better situation for the patient and family than if surgery is required for a crisis such as a foot deformity or hip dislocation. The best time for SEMLS if between 6 and 10 years of age2; if the corrective surgery is performed prior to the age of 6, repeat operations may be required. It is preferred for pediatric patients to have concluded the long period of rehabilitation prior to entering middle school. If the patient is a teen or young adult, the surgeries are generally performed on one side at a time and the rehabilitation may be more strenuous. Adolescent and adult patients are more susceptible to depression and anxiety and regressive functioning than pediatric patients.
In the preparation for surgery, it is important for the rehabilitation therapist to establish an exact baseline for functioning which includes determination of whether the movement disorder is dystonic, spastic, or a combination29. Also, an appropriate diagnosis must be determined since expected outcomes are more predicable for some diagnoses than others32. In addition, all stakeholders should agree to the patient’s level on the Gross Motor Function Classification System and the Functional Mobility Scale10. Goals for surgical and rehabilitation outcomes consider the sagittal gait pattern and the orthotic prescription, which may vary between patients with true jump gait and those with crouch gait24.
It should be kept in mind that following surgery, the patient will need equipment, new orthoses, and assistive devices to being the rehabilitation process2, 32. The team’s occupational therapist should inspect the home for any additional equipment to assist in the activities of daily living regardless of the age of the patient. Patients wearing the hip spica cast are allowed to bear their own weight between two and four days after surgery22. Following removal of the cast and based on individual ability, range-of-motion in the hips and knees, standing, and walking are started32. The hip flexor muscles are often overlooked as “long sitting” takes place; however, the optimum length for the hamstring is promoted, but the psoas major muscle is held in a shortened position. For this reason, it is vital to have a period of time in the prone position each day to stretch the iliopsoas across the front of the pelvis. In addition, early motion is required to prevent adhesions that may develop into scar tissue. If the patient has had a rectus transfer, the knee immobilizer must be removed to allow at least 30 degrees flexion by the end of the first week, 60 degrees at the end of the second week, and 90 degrees at the end of the third week. Prior to discharged five days after surgery, the physiotherapist should have initiated weight-bearing, encouraged either independent or transfer with assistance, and safe wheelchair mobility. Community therapists require communication regarding the surgical procedures performed, the status of rehabilitation, and the proposed home program.
Initial Postoperative Period: 3 weeks after surgery
Once the patient has been discharged, he will recover from his procedure at home and start his rehabilitation program32. Full weight-bearing may be delayed for a short period of time if the procedures included bony surgery. Femoral osteotomies should allow for full weight-bearing within a few days following surgery, but tibial osteotomies may require waiting one or two weeks. The patient, family, and caregivers must be informed of the status of weight-bearing for the patient at all times. Postponing weight-bearing unnecessarily may show rehabilitation or even lead to a reduction in function. It is important that full weight-bearing be achieved no later than three weeks post-operatively.
Second Postoperative Period: 4-5 weeks after surgery
Rehabilitation concerning maintaining muscle length, regaining prior strength, keeping alignment of the lower limbs, and encouraging weight-bearing, standing, and walking takes place in the period four-to-five weeks postoperatively32. The level of assistance needed by the patient in long sitting and prone lying depends on the patient’s age, level of cognition, and the baseline gross motor functioning present prior to surgery. Steady progress may be seen with one-hour therapeutic sessions three to five times weekly. If there are concerns by the family about coping with the home program or if weight-bearing proves too challenging, placing the patient back into inpatient rehabilitation may be necessary. The possibility of this should be introduced at pre- planning activities so the patient and family do not feel the surgery has been unsuccessful.
The Postoperative Visit Six Weeks Post-Operatively
During the six-week visit six weeks after surgery, a video gait analysis may be performed in the gait laboratory11. Deformities at the ankle and foot level may be inhibiting extension of the hip and knee joints25. If the patient suffers from severe crouch gait secondary to spastic diplegia, there may be overloading of the patellofemoral joint with deterioration in gait, function loss, and pain25, 30. Additional multilevel orthopedic surgery may be indicated25.
Third Postoperative Period: 7-12 weeks after surgery
At this point post-operatively, plaster casts are removed and if an ankle-foot othosis (AFO) is used, fitting is required32. Alterations in the rehabilitative program may include recreational swimming activities and formal hydrotherapy. If an AFO is in place, it may be removed for water therapy. Large gains may be seen in this period of rehabilitation and the frequency of therapy session may be adjusted to progress and the needs of the patient and family; one hour session between three to five times weekly are suggested. It is not unrealistic to expect restoration of the ability to walk. If an AFO requires adjusting, this should not be allowed to interfere with progression. Again, if there are concerns regarding gait or other problems, inpatient rehabilitation may be necessary for a week or two.
At this point, the patient may progress from the use of parallel bars to forearm crutches or a posterior walker32. If the patient is strong enough to use crutches, but prefers the insufficient ground reaction force of the walker, a flexed knee gait may return. Therefore, the patient should be encouraged to ambulate in the most effective manner possible. Documentation during this stage of rehabilitation should include the Functional Movement Systems screening tool to determine where the patient is in terms of using assistive devices over the distances of 5, 50, and 500 meters10, 11, 12.
Fourth Postoperative Period: 13-24 weeks after surgery
Within four to six months postoperatively, function, independence, and gait pattern should be improved due to new biomechanical alignment32. Assessment of gait in the gait laboratory using video graphics array (VGA) will show sagittal alignment, transverse plane alignment, knee coupling, and proper fit of the orthoses. Rehabilitation session may be reduced to two to four times weekly. Dependence on wheelchairs and walkers is discouraged as appropriate, and most patients will return to normal activities.
Fifth Postoperative Period: 6-12 weeks after surgery
It is important during this period to continue to focus on independence and muscle strengthening32. Many patients will have become more independent and physiotherapy sessions may be reduced and replaced with family recreational activities. Adolescents may enjoy activities in a gym with different types of standard exercise equipment to develop muscles in the hip extensors, ankle plantar flexors, and quadriceps.
Sixth Postoperative Period: 1 year after surgery
Most patients qualify for a full Investigator Global Assessment (IGA) twelve months postoperatively32. A current physical examination and completion of the Gross Motor Function Classification System and Functional Movement Systems allows comparison with the preoperative baseline IGA to determine the outcome of the surgery and subsequent rehabilitation8. Problems may be identified and recommendations concerning physiotherapy, use of injections, or possible further surgery may be proposed. While gait pattern improvement is usually seen, there are seldom changes in gross motor function. These improvements are generally observed in the second postoperative year.
Conclusion
The rehabilitation of a patient diagnosed with cerebral palsy following surgical correction of hip problems required an in-depth knowledge of cerebral palsy and associated physical challenges with movement based on joint dysfunction. There are a number of surgeries available to correct the joint problems in these patients, many of which are performed on children. The assessment of the patient’s needs and options should include all members of the care team, the family, and the patient if it is age appropriate. The goals of rehabilitation are dependent on the individual patient and informed therapists maintain strict adherence to methods and communication with the care team for physician preference and evaluation. As research continues to advance pharmaceutical treatment, surgical interventions, and rehabilitation techniques, the future of cerebral palsy patients suffering from hip dysplagia is becoming brighter.
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