Upper extremity surgical treatment of cerebral palsy

Upper Extremity Surgical Treatment of Cerebral Palsy Ann E. Van Heest, MD, James H. House, MD, Minneapolis, MN, Carol Cariello, MD, Valencia, Venezuela

The purpose of this report is to review the surgical treatment of the upper extremity involved with cerebral palsy over a 25-year period and present our results with regard to changes in upper limb function. Surgical results were assessed by comparison of preoperative and postoperative levels of upper extremity functional use using a previously described 9-level scale. The effect of the following cofactors on surgical outcome were examined: type of cerebral palsy, age, voluntary control, mental impairment, sensibility, and type of surgical treatment. One hundred eighty operations representing 718 procedures in 134 patients were reviewed. Surgical treatment was based on the following principles: soft tissue releases of deforming spastic muscles, tendon transfers to augment antagonistic activity, and joint stabilization. Surgical planning was tailored to each child’s particular needs. Comparison of the preoperative and postoperative 9-level functional use scores showed an average improvement of 2.6 functional levels for all patients. Patients with fair and good voluntary control had significantly greater improvement in functional use scores than those with poor voluntary control. No other statistically significant predictive cofactor was found. In selected patients with upper extremity dysfunction secondary to spastic cerebral palsy, surgical intervention improves function, as measured by the upper extremity functional use scale. (J Hand Surg 1999;24A:323–330. Copyright © 1999 by the American Society for Surgery of the Hand.) Key words: Surgical treatment, cerebral palsy.

Cerebral palsy is a nonprogressive central nervous system injury with peripheral manifestations dependent on the area of the brain that is affected. Abnormalities include dysfunction of motor control, sensibility, and mentation. The most common manifestation is motor spasticity causing joint deformity due to muscle imbalance across the joint. In the From Gillette Children’s Speciality Care Hospital, St Paul, MN. Received for publication November 20, 1997; accepted in revised form September 1, 1998. No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. Reprint requests: Ann E. Van Heest, MD, University of MN, Dept of Orthopedic Surgery, Box #492, 420 Delaware St SE, Minneapolis, MN 55455. Copyright © 1999 by the American Society for Surgery of the Hand 0363-5023/99/24A02-0004$3.00/0

upper extremity, the typical pattern of spastic joint deformities includes shoulder internal rotation, elbow flexion, forearm pronation, wrist flexion and ulnar deviation, thumb-in-palm, and finger swan neck or clenched fist deformity. The pattern and severity of joint deformity and limb dysfunction depends on the extent and area of the central nervous system dysfunction. Treatment of upper extremity dysfunction centers on improving muscle balance to maximize hand function. Each child must be individually evaluated for the extent and severity of central nervous system involvement and its resultant peripheral manifestations. The peripheral manifestations in the shoulder, elbow, forearm, wrist, thumb, and fingers must be evaluated for each child. The treatment is initiated through education

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of the parent and child as to the extent and severity of the cerebral palsy. Presently treatment options are not available to change mentation or sensibility deficiencies. The parents and child need to be informed that the deficiencies of mentation and sensibility are permanent parts of the child’s disability. Treatment is thus aimed at the motor system, particularly the spastic imbalance that causes functional impairment. Surgical treatment is indicated in patients with cerebral palsy with spastic contractures and fixed joint contractures, which produce functional impairment and could be improved by better joint positioning. There are various surgical treatment options. Although literature exists regarding the surgical treatment results of specific procedures, there has been no review of a large population of surgically treated patients that describes the surgical treatment of the upper limb as a whole, which may include multiple simultaneous procedures, and the resultant change in upper limb function as a whole. This study was designed to review our results in the surgical treatment of the upper extremity involved with cerebral palsy over a 25-year period and to present our results with regard to changes in upper limb function. The specific questions addressed include: What surgical principles were applied and what surgical procedures were most commonly performed to implement those principles? Does surgical intervention improve upper extremity function in spastic cerebral palsy as measured by the levels of upper extremity functional use? Can any predictive

factors be identified that affect functional improvement?

Materials and Methods All surgical procedures performed by the senior author (J.H.H.) between 1967 and 1992 were retrospectively evaluated by chart review. Patients were categorized by type of cerebral palsy (spastic/athetoid, quadriplegia/hemiplegia/triplegia), age at the time of surgery, degree of mental impairment, sensibility,1 voluntary motor control ability, and type of surgical treatment. Mental impairment and voluntary control were individually assessed by the treating physician. Mental impairment was graded as retarded or normal intelligence. Formal IQ testing was not performed in most patients but was assessed by the treating physician based on patient interaction, parental description, and school performance. Voluntary control was graded as poor, fair, or good during physical examination for each muscle to transfer: poor control demonstrated little selective motor control, fair control demonstrated intermittent selective motor control, and good control demonstrated selective control of the muscle tested. Surgical outcome was assessed using the upper extremity functional use levels previously described by House et al2 these are defined in Table 1. Preoperative and postoperative ratings of functional use levels were recorded and compared for each patient. One hundred eighty operations representing 718 procedures in 134 patients were reviewed. All values not totalling 134 patients are due to missing information. The average age at surgery was 14 years (range, 4 –37 years). Seventy-nine patients were male

Table 1. Classification of Upper Extremity Functional Use According to House et al2 No. of Patients Level

Category

0 1 2 3 4 5 6 7

Does not use Poor passive assist Fair passive assist Good passive assist Poor active assist Fair active assist Good active assist Spontaneous use, partial

8

Spontaneous use, complete

Description Does not use Uses as stabilizing weight only Can hold object placed in hand Can hold object and stabilize it for use by other hand Can actively grasp object and hold it weakly Can actively grasp object and stabilize it well Can actively grasp object and manipulate it Can perform bimanual activities and occasionally uses the hand spontaneously Uses hand completely independently without reference to the other hand

Before Surgery

After Surgery

11 44 27 4 45 0 0 1

0 0 7 30 6 23 48 12

0

4

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and 55 were female. The cerebral palsy patients included 85 spastic hemiplegics, 41 spastic quadriplegics, 5 spastic triplegics, and 3 athetoid. Mental impairment included 55 retarded and 59 normal intelligence patients. Voluntary control was poor in 48 patients, fair in 23 patients, and good in 26 patients. Sensibility was tested by 2-point discrimination and stereognosis function testing with 12 objects.1 Twopoint discrimination was greater than 10 mm in 22 patients, between 6 and 10 mm in 13 patients, and less than 6 mm in 38 patients. Stereognosis testing revealed that 47 patients had severe impairment (0 – 6 objects), 29 patients had moderate impairment (7–9 objects), and 25 patients had mild impairment (10 –12 objects correctly identified). Treatment results were measured by comparison of preoperative and postoperative upper extremity

functional use levels for the 131 spastic patients, with exclusion of the 3 athetoid patients. The 3 athetoid patients were treated with 2 flexor-pronator slides, 1 extensor carpi ulnaris centralization, and 2 metacarpophalangeal fusions. The athetoid patients are included in the study only to show that over a 25-year period, only 3 athetoid patients were treated surgically. Surgical treatment in the athetoid patient was rare and usually involved joint stabilization procedures. Surgeries of the elbow, forearm, wrist, fingers, and thumb are also grouped for comparison (Table 2). Most cerebral palsy patients are treated nonsurgically with passive range of motion and static nighttime splinting for fixed deformities and with coaching in functional adaptation for dynamic deformities. Failure to control joint deformity through nonsurgical

Table 2. Common Cerebral Palsy Deformities and Surgical Procedures Deformity

Procedures Soft tissue releases

Elbow Flexion (n 5 21)

Forearm Pronation (n 5 134)

Biceps lengthenings (11)3 Brachialis lengthenings (10)3

PT releases (80)4 Biceps aponeurosis releases (49) PQ release (1)

Tendon transfers

PT re-routing (1)5

Bone/joint stabilization

Rotational osteotomies (3)

Wrist Flexion/UD (n 5 202)

Finger Deformity (n 5 40)

Thumb-in-Palm (n 5 289)

FCR lengthenings (31)6 Flexor pronator slides (16)7,8 FCU lengthenings (15)6 BR to ECRB/L (50)9,10 ECU to ECRB/L (42) FCU to ECRB/L (28)11,12 FCR to ECRB/L (3) PT to ECRL (1)

FDS lengthenings (14)6

Adductor and/or first DI releases (84)17 First web z-plasties (57) FPL lengthenings (20) FCR to APL (25) PL to APL (17)2 PL to EPB (10) BR to APL (5) BR to EPB (4) PL to EPL (3) EPL re-routings (2) BR to EPL (1) FCR to EPB (1) Accessory muscle of APL to EPB (3) MCP fusions (48)18 MCP capsulodesis (4)19 IP fusions (5)

Wrist fusion with PRC (11) PRC (5)13

FCU to EDC (8) BR to EDC (5) FDS tenodesis (4)15 Lateral band reroutings (3)14 SORL (1)16

Palmar plate capsulodesis (2) PIP fusions (2) DIP fusion (1)

Numbers in parentheses refer to the number of times this procedure was performed in this study population; the superscript numbers refer to the reference number of this surgical technique. UD, ulnar deviation; PT, pronator teres; PQ, pronator quadratus; FCR, flexor carpi radialis; FCU, flexor carpi ulnaris; BR, brachioradialis; ECRB/L, extensor carpi radialis brevis and/or longus; ECU, extensor carpi ulnaris; PRC, proximal row carpectomy; FDS, flexor digitorum superficialis; EDC, extensor digitorum communis; SORL, spiral oblique retinacular ligament reconstruction; DIP, distal interphalangeal joint; PIP, proximal interphalangeal joint; DI, dorsal interosseous; FPL, flexor pollicis longus; PL, palmaris longus; APL, abductor pollicis longus; EPL, extensor pollicis longus; EPB, extensor pollicis brevis; MCP, metacarpophalangeal joint; IP, interphalangeal joint.

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methods, leading to identifiable and improvable functional deficiency, was an indication for surgical intervention. Surgical treatment principles for upper extremity dysfunction due to joint deformity included soft tissue release of deforming spastic muscles, tendon transfers to augment antagonistic activity, and joint stabilization procedures (Table 2). Soft tissue releases were indicated most commonly for spastic contracture, with tendon transfers added when the spastic deformity was more severe and appropriate motors with adequate voluntary control were available. Joint stabilization procedures were indicated for the severe joint contractures (eg, wrist contracture if adequate finger extension was present) or for severe joint instability (eg, thumb metacarpophalangeal joint). Treatment results for the spastic cerebral palsy patients were then analyzed based on the type of cerebral palsy, level of mentation, stereognosis, 2-point discrimination, and voluntary muscle control abilities to determine predictive factors. Treatment results were compared for tendon transfers from the flexor carpi ulnaris, the extensor carpi ulnaris, and the brachioradialis transferred to the extensor carpi radialis and/or brevis. Statistical comparison of results was performed using Kruskal Wallis one-way ANOVA, one-tailed t-test, and chi-squared analysis. A probability value of .05 was considered to be significant.

Results Review of Surgical Procedures The surgical procedures are outlined in Table 2. Thirty-two miscellaneous procedures were performed that are not included in the table. The study

shows that the most common surgical procedures were pronator teres and biceps aponeurosis release for forearm pronation deformity; tendon transfers to extensor carpi radialis longus and/or brevis using flexor carpi ulnaris, brachioradialis, or extensor carpi ulnaris (all with similar results) for wrist flexion/ ulnar deviation deformity; and concomitant first web z-plasty, adductor and first dorsal interosseous release with tendon transfer (most commonly to the abductor pollicis longus using the palmaris longus or flexor carpi radialis) for thumb-in-palm deformities. Most commonly, all deformities were corrected during 1 operation, which averaged 4 procedures per surgery. Most children with cerebral palsy have multiple joint involvement. Maximum upper limb function was achieved through simultaneous correction of the multiple aspects of the limb dysfunction. A case example is presented in Fig. 1.

Surgical Results Using the 9-level scale shown in Table 1, the average functional use score was 2.3 before surgery (range, 0 –7) and 5.0 after surgery (range, 2– 8). The average change in functional use scores, 2.7 levels of improvement, was significant.

Predictive Factors The change in functional use scores from before to after the surgery were then compared based on the type of cerebral palsy, level of mentation, voluntary control ability, stereognosis, and 2-point discrimination (Table 3). The functional use score has a maximum change value of 68 levels. By cerebral palsy type, the average change in functional use scores was 12.7 levels (range, 11 to

™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™™3 Figure 1. A 13-year-old girl with spastic hemiplegia presented with a flexion ulnar deviation deformity (A). The preoperative evaluation revealed a highly motivated individual with normal mentation. The 2-point discrimination was between 6 and 10 mm and the stereognosis examination revealed correct identification of 10 of 12 objects. Grasp was ineffective because of wrist imbalance due to inadequate wrist extension; there was good voluntary control of the brachialis muscle. (B) The digital extensors attempt to substitute for absent active wrist extension. This causes secondary swan neck deformity and ineffective grasp function. A type I thumb-in-palm deformity is seen with adduction of the first metacarpal; there is adequate interphalangeal extension control. (C) Through a radial incision, the brachioradialis tendon was interwoven into the extensor carpi radialis longus tendon to improve wrist positioning and grasp function. The thumb was additionally treated with a first web z-plasty and partial adductor pollicis and first dorsal interosseous release. (D) Wrist extension improved following surgery and the hand assumed a more functional position. Providing the brachioradialis for active wrist extension allows the fingers to grasp. (E) Active wrist flexion has been maintained and the wrist muscles have been effectively balanced. (Figure continues)

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328 Van Heest, House, and Cariello / Surgical Treatment in Cerebral Palsy

Figure 1. (Continued) (F) The thumb exhibits improved abduction and extension, allowing for effective cylindrical pinch.

15) for hemiplegics, 13.0 levels (range, 11 to 15) for triplegics, and 12.7 levels (range, 12 to 14) for quadriplegics; there were no significant differences between groups. By level of mentation, the average change in functional use scores was 12.6 levels (range, 11 to 15) for patients with retarded intelligence and 12.9 levels (range, 21 to 16) for patients with normal intelligence; there were no significant differences between groups. By voluntary motor control, the average change in functional use scores was 12.3 levels (range, 21 to 15) for patients with poor voluntary control, 12.6 levels (range, 11 to 15) for patients with fair voluntary control, and 13.0 levels (range, 11 to 15) for patients with good voluntary control. Patients with fair and good voluntary control had a statistically significant greater improvement in functional use levels than patients with poor voluntary control (p 5 .04). By stereognosis abilities, the average change in functional use scores was 12.5 levels (range, 11 to 15) for patients with severe impairment, 13.2 levels (range, 11 to 15) for patients with moderate impairment, and 12.8 levels (range, 11 to 16) for patients

Table 3. Predictive Factors for Changes in Upper Extremity Functional Use Scores* Average Upper Extremity Functional Use Scores

Cerebral palsy type Hemiplegic Triplegic Quadriplegic Level of mentation Retarded Normal Voluntary motor control Poor Fair Good Stereognosis Severe Moderate Mild 2-Point discrimination . 10 mm 6–10 mm , 6 mm

No. of Patients

Before Surgery

After Surgery

Change (Range)

85 5 41

2.5 2.0 1.9

5.2 5.0 4.6

12.7 (11 to 15) 13.0 (11 to 15) 12.7 (12 to 14)

55 59

1.8 2.7

4.5 5.6

12.6 (11 to 15) 12.9 (21 to 16)

48 23 26

1.8 2.4 3.1

4.2 4.9 6.0

12.3 (21 to 15) 12.6 (11 to 15) 13.0 (11 to 15)

47 29 25

2.3 1.9 2.8

4.8 5.1 5.6

12.5 (11 to 15) 13.2 (11 to 15) 12.8 (11 to 16)

22 13 38

2.0 2.5 2.4

4.7 5.1 5.5

12.7 (11 to 15) 12.7 (21 to 15) 13.1 (11 to 15)

*All values not totaling 131 patients are due to missing information.

The Journal of Hand Surgery / Vol. 24A No. 2 March 1999 329

with mild impairment; there was no statistical significance between groups. By 2-point discrimination function, the average change in functional use scores was 12.7 levels (range, 11 to 15) for patients with discrimination greater than 10 mm, 12.7 levels (range, 21 to 15) for patients with discrimination between 6 and 10 mm, and 13.1 levels (range, 11 to 15) for patients with discrimination less than 6 mm; there were no significant differences between groups. Further analysis of the effect of sensibility on functional use scores is shown in Table 4. A trend may be seen in which patients with more severe stereognosis and 2-point discrimination had slightly lower functional use scores than patients with mild sensibility impairment. Statistical analysis of the groupings listed on Table 4, however, reveals that functional use scores did not correlate with either degree of stereognosis impairment or with 2-point discrimination impairment.

Comparison of Wrist Tendon Transfers The surgical procedure with the numbers and distribution sufficient to allow statistical analysis was tendon transfers for wrist extension. One hundred twenty patients underwent transfers into the extensor carpi radialis and/or brevis with tendon transfers from the brachioradialis (50 patients), the extensor carpi ulnaris (42 patients), and the flexor carpi ulnaris (28 patients). Comparison of these tendon transfers measuring surgical results by change in functional use scores shows an average improvement of 12.5 levels (range, 11 to 15) for the brachioradialis transfer, 12.5 levels (range, 11 to 15) for the

Table 4. Effect of Sensibility on Upper Extremity Functional Use Scores Score

Stereognosis impairment Severe Moderate Mild 2-Point discrimination impairment . 1 cm 6–10 mm , 6 mm

Before Surgery

After Surgery

Change

2.3 1.9 2.8

4.7 5.0 5.5

12.5 13.1 12.7

1.9 2.5 2.4

4.7 5.0 5.9

12.7 12.7 13.1

extensor carpi ulnaris transfer, and 12.5 levels (range, 11 to 15) for the flexor carpi ulnaris transfer; there were no significant differences between transfers.

Discussion This study presents a review of the surgical procedures performed at our institution over a 25-year period to treat cerebral palsy in the upper extremity was carried out. Several important points are highlighted. First, most previous studies6 have reported either diminished surgical results or have recommended nonoperative treatment for severely retarded patients, for those with poor sensibility (stereognosis and 2-point discrimination), and those with poor voluntary motor control. In contradistinction to those recommendations, the children in this series treated surgically represented a full range of mentation abilities, sensibility function, and motor function. Surgical results showed an average 12.6 levels of improvement of functional use patterns for all patients despite the level of mentation, 2-point discrimination, stereognosis function, or type of cerebral palsy. Patients with poor motor control did have less improvement in functional use. Most patients treated surgically were highly motivated. The data presented in this series support the statement that similar functional improvement (2 levels) can be achieved in the highly motivated patient with fair to good motor control regardless of preoperative mentation, sensibility, and type of cerebral palsy. An additional important aspect of this series is that it provides the hand surgeon with data regarding the results of surgical treatment of cerebral palsy upper extremity functional deficiency. Surgical results are presented in terms of an upper extremity functional use 9-level scoring system, which has been previously described2 but not previously validated. This study presents our experience using this 9-level scale. Comparison of preoperative and postoperative upper extremity functional use scores showed a statistically significant improvement. The surgical procedures performed improved upper extremity functional use by an average of 2 levels. This is important information to include as part of the preoperative consultation. For example, patients presenting with a poor passive assist hand could be improved on average to a good passive assist hand provided the

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patient is highly motivated and has good to fair motor control. Slightly diminished results would be seen with poor motor control. The patients treated surgically in this series were primarily low-functioning patients; there was only 1 patient with a preoperative functional use pattern above level 4 (poor active assist). No conclusions can be drawn about treatment for patients presenting with function above this level. The authors thank Melissa Cohen for data analysis and Bonnie Olson for manuscript preparation.

References 1. Van Heest AE, House J, Putnam M. Sensibility deficiencies in the hands of children with spastic hemiplegia. J Hand Surg 1993;18A:278 –281. 2. House JH, Gwathmey FW, Fidler MO. A dynamic approach to the thumb-in-palm deformity in cerebral palsy. J Bone Joint Surg 1981;63A:216 –225. 3. Mital MA. Lengthening of the elbow flexors in cerebral palsy. J Bone Joint Surg 1979;61A:515–522. 4. Strecker WB, Emanuel JP, Dailey L, Manske PR. Comparison of pronator tenotomy and pronator rerouting in children with spastic cerebral palsy. J Hand Surg 1988; 13A:540 –543. 5. Sakellarides HT, Mital MA, Lenzi WD. Treatment of pronation contractures of the forearm in cerebral palsy by changing the insertion of the pronator radii teres. J Bone Joint Surg 1981;63A:645– 652. 6. Zancolli EA. Structural and dynamic bases of hand surgery. 2nd ed. Philadelphia: JB Lippincott, 1968:263–283. 7. Inglis AE, Cooper W. Release of the flexor-pronator origin for flexion deformities of the hand and wrist in spastic paralysis. J Bone Joint Surg 1966;48A:847– 857.

8. White WF. Flexor muscle slide in the spastic hand: the Max Page operation. J Bone Joint Surg 1972;54B:453– 459. 9. House JH, Gwathmey FW. Flexor carpi ulnaris and the brachioradialis as a wrist extension transfer in cerebral palsy. Minn Med 1978;61:481– 484. 10. McCue FC, Honner R, Chapman WC. Transfer of the brachioradialis for hands deformed by cerebral palsy. J Bone Joint Surg 1970;52A:1171–1180. 11. Green WT. Tendon transplantation of the flexor carpi ulnaris for pronation-flexion deformity of the wrist. Surg Gynecol Obstet 1942;75:337–342. 12. Green WT, Banks HH. Flexor carpi ulnaris transplant and its use in cerebral palsy. J Bone Joint Surg 1962;44A: 1343–1352. 13. Omer GE, Capen DA. Proximal row carpectomy with muscle transfers for spastic paralysis. J Hand Surg 1976; 1:197–204. 14. Tonkin MA, Hughes J, Smith KL. Lateral band translocation for swan-neck deformity. J Hand Surg 1992;17A:260 – 267. 15. Swanson AB. Surgery of the hand in cerebral palsy and the swan-neck deformity. J Bone Joint Surg 1960;42A:951– 964. 16. Littler JW. The finger extensor mechanism. Surg Clin North Am 1967;47:415– 432. 17. Matev I. Surgical treatment of spastic “thumb-in-palm” deformity. J Bone Joint Surg 1963;45B:703–708. 18. Goldner JL, Koman LA, Gelberman R, Levin S, Goldner RD. Arthrodesis of the metacarpophalangeal joint of the thumb in children and adults: adjunctive treatment of thumb-in-palm deformity in cerebral palsy. Clin Orthop 1990;253:75– 89. 19. Filler BC, Stark HH, Boyes JH. Capsulodesis of the metacarpophalangeal joint of the thumb in children with cerebral palsy. J Bone Joint Surg 1976;58A:667– 670.