80% of involved hands of patients with cerebral palsy demonstrate significant functional impairment; less than 20% are appropriate surgical candidates.
Multidisciplinary input improves appropriate delineation of realistic and appropriate surgical goals.
Patients with voluntary grasp and release, good sensibility, and cognitive capability are candidates for muscle/tendon transfers to balance muscle forces across selected joints.
Patients with dystonia, rigidity, poor sensibility, fixed contractures, and cognitive impairments have unpredictable results after muscle/tendon transfers. However, they may benefit from athrodeses and tendon lengthening or tenotomy.
When used in conjunction with extensor tendon rerouting and plication, fusion of thumb metacarpal phalangeal joint eliminates one motion segment, improves stability, enhances function, and improves appearance in a reliable manner.
Multiple procedures involving the hand, wrist, and forearm often are required.
Postoperative therapy is crucial. It should be instituted even while the patient is in a cast. Static and occasionally dynamic outriggers are valuable adjuncts. In addition to retraining muscles after transfers, strengthening is a crucial component of postoperative care in the child or adult with cerebral palsy.
With an incidence of 2.6 per 1000 live births, cerebral palsy is the most common cause of disability in childhood. Significant functional impairment exists in 80% of the upper limbs of children and adults with upper limb involvement (hemiparesis or quadriparesis). In addition to spasticity, patients may present with pain, muscle weakness, poor sensibility, and movement disorders. Consequently, patients often demonstrate poor self-esteem, difficulty with functional activities, and deformities that impair caregiving. Historically, less than 20% of affected limbs in pediatric patients with cerebral palsy benefit from surgical intervention. This chapter provides an overview of (1) operative options used to improve or to maintain upper extremity range of motion to prevent osseous deformity and to delay or to decrease joint contracture and (2) postoperative rehabilitation strategies.
History and Physical Examination
The history ideally involves a team approach and includes input from the patient, caregiver(s), therapists, teachers, social workers, and other health-care providers. However, for many encounters, all caregivers and health-care providers are not available, and the patient may be unable to convey historical information effectively. Use of standardized questionnaires, functional classification scales (e.g., House Scale ), and standardized testing regimens (e.g., Melbourne Assessment of Unilateral Limb Function ) facilitates decision making. A complete upper extremity examination includes an assessment of active and passive range of motion, functional capability, muscle tone, sensibility, and the effect of volitional activity on the extremity. The presence of deformities such as thumb-in-palm deformity, swan-neck deformity, boutonnière contractures, and pronation contractures and their impact on range of motion and function are assessed. Voluntary control of all motor groups is assessed and helps to distinguish deformity secondary to muscle spasticity from muscle and joint contractures. Movement disorders are noted and recorded. If present, the magnitude of involuntary movement is noted, and the effectiveness of upper limb motor control is assessed, quantified, and recorded. The effectiveness of grasp and release is determined with the wrist in flexion, extension, and dorsiflexion. If possible, the patient is observed during ambulation, standing, sitting, and recumbency to observe posturing and motion patterns. Serial evaluations are helpful to fully assess patients because factors such as the time of day, degree of anxiety, and level of fatigue can influence clinical findings.
Functional motor testing (such as the Jebsen’s Hand Function Test) may augment the evaluation of patients with mild to moderate upper extremity involvement; however, it is not practical for evaluating patients with severe involvement. Muscles being considered for transfer are assessed to determine phasic and nonphasic control. If there is a clinical concern, electromyographic evaluation is requested. Sensibility testing should include an evaluation of proprioception, stereognosis, and two-point discrimination. These tests are helpful in identifying patients who are suitable candidates for complex reconstruction procedures. Individuals with limited sensory capability are not good candidates for sophisticated surgical techniques.
Radiologic studies, electromyographic data, motion analysis, and diagnostic muscle blocks provide valuable additional diagnostic information. Plain radiographs, computed tomography, and magnetic resonance imaging may be used to evaluate joint and osseous deformities. Surface and needle electromyography help to provide qualitative and quantitative assessments of voluntary motor recruitment and the appropriateness of motor activations. A quantitative measure of a patient’s motor performance and functional capacity can be determined from three-dimensional upper extremity motion analysis. Motor blockade produced by injection of bupivacaine or botulinum A toxin into the muscles that have been identified for surgery may serve as a diagnostic tool to predict postoperative outcomes or as an adjunct to surgical treatment.
Selection of Surgical Candidates and Indications for Surgery
The patient, caregiver, therapist, and surgeon should formulate specific goals that they expect from upper extremity surgery. Often, involved parties cannot agree on which goals take priority. Such discrepancies must be reconciled before surgical intervention. Some of the most common goals include decreased pain, specific functional improvement, enhanced self-esteem, and facilitation of caregiver activities. To attain these goals, surgical interventions must be performed on several different joints ( Table 130-1 ). The presence of movement disorders (e.g., athetosis, chorea, choreoathetosis, dystonia) is a relative contraindication for tendon transfer surgery. These patients may benefit from arthrodesis. Furthermore, sophisticated tendon transfers should be undertaken with caution in patients who demonstrate poor or limited sensibility. The selection of an appropriate procedure(s) required to achieve both specific and global outcomes must be individualized for each patient. Once a child is identified as a candidate for surgical intervention, an appropriate surgical plan, including a postoperative regimen and rehabilitation program, is formulated.
|Shoulder||Joint stabilization||Fusion, capsular reconstructions|
|Increase external rotation||Lengthen PM/subscapularis; transfer LD and teres major|
|Increase internal rotation||Lengthen/release infraspinatus/teres minor|
|Increase extension||Lengthen or release biceps brachii, brachialis brachioradialis release (slide), flexor–pronator mass release, joint capsule release, humeral osteotomy, excise radial head|
|Forearm||Improve supination||Reroute, lengthen, or release PT; osteotomy radius ± ulna; release flexor–pronator muscle; excise radial head|
|Wrist||Stabilization||Fusion (tendon transfer; release; proximal row) carpectomy|
|Increase range of motion|
|Extension||Transfers of FCU, ECU, FCR, BR, FDS, FDP|
|Thumb||Stabilization||Volar plate arthroplasty, fusion MCP joint|
|Increase extension||Plication/transfer, EPL, APB, EPB; release/lengthen FPL|
|Improve power||Reinforce EPL, reinforce FPL|
|Improve abduction||Release adductor, abduction transfer, osteotomy metacarpal|
|Fingers||Flexion||FDS to FDP transfer; flexor/pronator release (slide), lengthen FDS, lengthen FDP|
|Swan neck||EDC FDS tenotomy; fusion PIP joint; tenodesis FDS|
Adduction and internal rotation shoulder deformities are common in children with cerebral palsy due to a dynamic imbalance of the internal rotators and/or fixed contractures of the pectoralis major, subscapularis, and/or joint capsule. Depending on the etiology, these deformities may be classified as dynamic, static, or combined dynamic-static. Surgery on the shoulder joint may be necessary due to dislocation, subluxation, or contractures. Deformity may occur in more than one plane. Moreover, joint incongruity with deformity of the humeral head, dysplasia of the glenoid, and/or arthritis of the glenohumeral articulation should be addressed. In dynamic deformities, the glenohumeral articulation is typically congruous and stable with satisfactory articular surfaces. Muscle lengthening and muscle–tendon transfer are treatment options for these patients with shoulder deformities. To manage shoulder internal rotation deformity, the pectoralis major and subscapularis may be released or lengthened by Z -plasty. In severe contractures, capsular release may also be required. Transfer of the latissimus dorsi and teres major muscles may augment external rotation power ( Fig. 130-1 ). Patients with significant adduction and internal rotation shoulder contractures may be aided by latissimus dorsi and teres major releases in conjunction with the procedures described previously. Alternatively, a proximal or distal osteotomy of the humerus may be used to improve either external rotation or internal rotation. In most cases, osteotomy is reserved for patients with arthritic or dysplastic/subluxed joints and for patients who have undergone unsuccessful tendon transfers. In patients without arthritis or significant humeral head or glenoid deformity, subluxation and dislocation may require open reduction, capsular release and/or capsulodesis, and/or humeral or glenoid osteotomy. Shoulder fusion is an option to treat refractory arthritic shoulder pain, but must be undertaken with caution due to the attendant difficulties created in caregiving and in activities of daily living.
Casting, shoulder orthoses, and therapy are usually required after shoulder procedures. For internal rotation contracture releases and transfers, either an orthosis or a modified shoulder spica cast is applied in the operating room. The orthosis/cast is worn full time for 3 to 4 weeks. Patients may then transition to an orthosis worn only at night for an additional 3 months. At 4 weeks, active and passive range-of-motion activities are initiated, followed by strengthening activities at 6 weeks.
Correction of an elbow flexion deformity is not required to improve functional activities of daily living unless the flexion deformity exceeds 30 degrees. Deformities are typically dynamic and static, with fixed contractures of the capsule as well as the biceps, brachialis, and brachioradialis muscles. The flexor–pronator mass may also have a fixed component. Additionally, dynamic motor imbalance of the muscles about the elbow exacerbates contractures by shortening musculotendinous units. Furthermore, dysplasia and subluxation of the radial head may occur with longstanding hyperpronation deformities. In cases with milder deformities, soft tissue procedures, including lengthening of the elbow flexors, are often successful in correcting the contractures. Flexion contractures between 30 and 60 degrees may be treated in this manner ( Fig. 130-2 ). Contractures greater than 60 degrees may require the additional release of the flexor–pronator origin and/or the elbow capsule. Patients with subluxation of the radial head often present with pain in addition to poor elbow range of motion. These patients may benefit from resection of the radial head, which decreases pain and slightly improves forearm supination/pronation. However, the patient should understand that this procedure rarely improves elbow flexion/extension capabilities. Elbow fusion is reserved for patients who experience intractable pain. Elbow fusion has never been used in our patient population.