Surgery of the Upper Extremity in Cerebral Palsy

Functional activities of the upper extremity are limited in most individuals with a diagnosis of cerebral palsy (CP). However, surgical interventions are applied in less than 20% of pediatric patients with an upper extremity affected by CP. This article covers the surgical interventions used for the reconstruction of the upper limb in patients with CP. The optimal surgical approach for each deformity type is described. In addition, the various evaluation techniques of the upper extremity, the general principles of an operative treatment plan, and the appropriate postoperative care of these patients is presented.

Functional activities of the upper extremity are limited in most individuals with a diagnosis of cerebral palsy (CP). However, surgical interventions are applied in fewer than 20% of pediatric patients with an upper extremity affected by CP, in marked contrast to the lower extremity in which surgery is more frequently indicated. Apart from improving function, surgical procedures may decrease pain, prevent or fix upper limb deformity, and have a positive impact on the patient’s caregiving, self-esteem, and appearance. Several conservative treatment methods are also available (eg, therapy, casting, electrical stimulation, oral spasmolytic medications, and parenteral neuromuscular blocking agents). These methods are primarily used to preserve joint range of motion (ROM), to delay tendon and muscle contractures, and to prevent upper extremity osseous deformities. In patients without competently functional antagonist muscles, passive stretch for a minimum of 6 out of 24 hours is required to maintain muscle length and to avoid development of a fixed contracture. Occupational therapy and splinting alone do not accomplish long-term reduction of the involuntary spasm. Moreover, pharmacologic agents designed to decrease spasticity have not been validated as a definitive means of providing lasting improvement. In selected cases, surgery following conservative treatment has been reported to give satisfactory results.

This article covers the surgical interventions used for the reconstruction of the upper limb in patients with CP. The optimal surgical approach for each deformity type is described. In addition, the various evaluation techniques of the upper extremity, the general principles of an operative treatment plan, and the appropriate postoperative care of these patients is presented.

Evaluation

History and Physical Examination

A team approach to the management of the patient with CP is important; medical history and physical examination is the basis for the successful assessment of each individual. Input from the patient and his or her caregiver, and any involved health care worker should be provided. Functional classification systems (eg, House scale) as well as standardized testing regimens, such as the Melbourne Assessment of Unilateral Upper Limb Function (Melbourne), are helpful evaluation tools. The Melbourne scale allows objective measurement of the upper extremity function in patients with CP. Both active and passive ROM of the upper extremity joints should be assessed in a reliable and reproducible manner. In the authors’ institution, the Upper Extremity Rating Scale (UERS) is used for this purpose. The UERS provides a composite score of the active and passive ROM of the shoulder, elbow, forearm, and wrist. Degree of spasticity, and absence or presence and magnitude of involuntary movement disorders should also be evaluated and recorded. For patients with mild to moderate involvement, the use of the Melbourne scale and the Jebsen-Taylor Hand Function Test is suggested. A global instrument such as the WeeFIM (Uniform Data System for Medical Rehabilitation, University at Buffalo Foundation Activities, Inc, New York) is also available to measure self-care and functional skills. If possible, the patient is observed during ambulation, standing, and sitting to evaluate certain posturing and motion patterns. Several factors including fatigue level, anxiety, and even time of the day may affect the clinical findings; therefore, serial evaluations are necessary. Preoperatively, sensibility testing including evaluation of proprioception, stereognosis, and 2-point discrimination should also be performed.

Imaging Studies and Ancillary Testing

Evaluation of patients with CP is complex. Several examinations are necessary as part of the surgical planning process. Plain radiographs, computed tomography scans, and magnetic resonance imaging studies are helpful in assessing preoperative joint congruity. An individualized and detailed functional evaluation is also a critical component for developing the appropriate surgical plan. Dynamic electromyography (EMG) provides a qualitative and quantitative assessment of voluntary motor control and the type of motor activity of muscles being considered for transfer. A videotaped evaluation of the upper extremity in children with CP provides an objective assessment of a patient’s motor performance and functional capacity. Carlson and colleagues reported changes to the initial preoperative plan following the study of videotaped evaluations, especially for procedures addressing the wrist, digit, and thumb. Motor blockade produced by injections of botulinum toxin A (BTX-A) or a topical anesthetic agent (eg, bupivacaine) into the muscles identified for surgery may serve as a diagnostic tool to select the proper operative interventions.

General Principles of an Operative Treatment Plan

Diagnostic evaluation is helpful in identifying the suitable candidates for complex reconstructive procedures of the upper extremity. Selection of the appropriate intervention is required to achieve both specific and global outcomes individualized for each patient. However, it must be recognized that although some improvement in function and appearance may be a realistic goal for properly selected patients, normality can rarely, if ever, be achieved. The type of joint deformity, the underlying neuromuscular disorder, the preoperative sensibility and functional capacity of the limb, the patient’s intellectual status and goals, and the surgeon’s preferences are the factors used to devise a treatment plan.

Global surgical goals for best outcomes include (1) improved function, (2) facilitation of care, (3) pain reduction, and (4) enhancement of self-esteem. Specific surgical interventions may be performed at one or more levels (ie, shoulder, elbow, wrist, hand) to release overactive muscle groups, stabilize joints, and augment selective motor control of weak muscles by tendon transfer to achieve each patient’s specific goals. Surgical interventions may be reduced to a checklist of options ( Table 1 ). Proactive planning with multiple team members, including the development of an immediate postoperative regimen (eg, period and type of immobilization) and rehabilitation program, is the key to the success of the operative procedure.

Table 1

Operative interventions of the upper extremity in CP

Joint	Aim	Options
Shoulder	Joint stabilization	Fusion, capsular reconstructions
	Improve external rotation	Lengthen pectoralis major/subscapularis; transfer LD and/or teres major; humeral osteotomy
	Improve internal rotation	Lengthen/release infraspinatus/teres minor
Elbow	Joint stabilization	Fusion
Elbow	Improve extension	Lengthen biceps brachii/brachialis; BR release; flexor-pronator mass release (slide); capsulotomy
Forearm	Improve supination	Reroute, lengthen, or release PT; radius/ulna osteotomy; flexor-pronator release (slide)
Wrist	Stabilization	Fusion
Wrist	Improve extension	Flexor tendon release; proximal row carpectomy; ECU transfer; FCU transfer to ECRB/ECRL/EDC
Thumb	Stabilization	Volar plate arthroplasty; MCP fusion
	Improve extension	Release/lengthen FPL; reinforce EPL
	Improve abduction	Release adductor pollicis; reinforce APL; EPL rerouting
Fingers	Flexion deformity	FDS to EDC transferflexor/pronator release (slide); FDS/FDP lengthening;FDS to FDP transfer
Fingers	Swan-neck deformity	PIP joint tenodesis; central slip tenotomy; intrinsic origin release

Abbreviations: APL, abductor pollicis longus; BR, brachioradialis; ECRB, extensor carpi radialis brevis; ECRL, extensor carpi radialis longus; ECU, extensor carpi ulnaris; EDC, extensor digitorum communis; EPB, extensor pollicis brevis; EPL, extensor pollicis longus; FCU, flexor carpi ulnaris; FDP, flexor digitorum profundus; FDS, flexor digitorum superficialis; FPL, flexor pollicis longus; LD, latissimus dorsi; MCP, metacarpophalangeal; PIP, proximal interphalangeal; PT, pronator teres.

Operative Procedures

Shoulder

Adduction and internal rotation shoulder deformity is common in patients with CP; the deformity is due to unbalanced spasticity of the internal rotators of the arm at the glenohumeral joint (pectoralis major, latissimus dorsi, subscapularis, and teres major). A fixed contracture of the muscles (mentioned earlier) and the joint capsule may contribute to the deformity posture. Surgical interventions may need to address (1) the muscle/tendon and/or capsule contractures, and (2) the subluxation or dislocation of the humeral head in one or more planes of the shoulder joint. Inferior subluxation is the most common form in the hemiplegic shoulder. Moreover, dysplasia of the glenoid or humeral head and/or arthritis of the glenohumeral joint should be encountered in the treatment plan. In dynamic deformities, the glenohumeral articulation is typically stable with congruous articular surface contact. Treatment options include muscle lengthening, tendon transfer, humeral osteotomy, and shoulder joint fusion.

Although surgical treatment is rare, the pectoralis major and subscapularis muscles should be lengthened to correct shoulder adduction and internal rotation deformity; capsular release may also be performed. Transfer of the latissimus dorsi and teres major may augment active external rotation of the arm ( Fig. 1 ). In severe cases, release of the latissimus dorsi and teres major muscles in conjunction with the procedures described above may be required. If tendon/muscle release and/or transfer fail, a proximal or distal osteotomy of the humerus may be used to improve rotation of the arm. Osteotomy is also indicated for patients with dysplastic/subluxed or arthritic shoulder joints. Refractory arthritic pain may be addressed by shoulder fusion in individuals with CP; however, there is no experience with this procedure in the authors’ institution.