8 Upper Extremity

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The upper extremity is frequently affected in children with cerebral palsy (CP). Many children have only mild fine motor difficulties (diplegia) whereas others are more severely affected (hemiplegia and quadriplegia). The classic child with a spastic upper extremity, in whom surgical treatment is considered, has spastic hemiplegia causing posturing of the involved upper extremity with the elbow flexed, forearm pronated, the wrist and fingers flexed, and the thumb adducted and flexed in the palm. Children with movement disorders (athetosis or dystonia) may present with upper extremity involvement however, surgical correction is rarely indicated. The greatest task is to clearly define the functional difficulties (if any), determine optimum goals for a specific child’s developmental stages, and bring together realistic long- term goals between patients and orthopaedic surgeons. This task requires that surgeons understand the concerns of families and children about the cosmesis of the extremity and the specific functional concerns. Often, the concerns of patients, especially adolescents, are different from the concerns of parents. Also, orthopaedists have to understand each component of the global extremity’s impairment and how these impairments evolve with developmental maturation.

Normal Development of Function of Children’s Upper Extremities

Upper extremity spastic deformities start out as a clinched fist position with the thumb in the palm under the flexed fingers. As children grow, the fingers open first, and as more maturity and development occur, the thumb relaxes out of the palm. Often, in children with hemiplegia, the fingers are out of the flexed position by 2 to 3 years of age, and over the next several years the thumb slowly frees up. By 6 to 9 years of age, the thumb may be at the level of maximum abduction, and wrist flexion is becoming the predominant position. There is also significant elbow flexion with forearm pronation from early childhood. As children move through middle childhood and into adolescence, the elbow flexion and pronation often slowly decrease but almost never resolve or become insignificant. By late childhood and early adolescence, the upper extremity deformity has developed the position it will maintain throughout the remainder of individuals’ lives, except some of the contractures such as the contracted finger and wrist flexors may slowly become more fixed and more severe. These progressive contractures seem to be more common in quadriplegia than hemiplegia. Throughout childhood, the evaluation of individual children has to focus on their current function, physical deformity in the context of their age, and cognitive abilities.

8

Upper Extremity

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Evaluation of Patients

Perhaps one of the most difficult tasks is to accurately determine children’s maximum functional abilities to perform tasks of daily living that are age appropriate. This can be done by taking a careful history from the parents, and if possible, questioning the children’s occupational therapists to determine tasks that they are working on and tasks that they have recently achieved. For example, in toddlers, the parents should be asked if these children are able to hold a cup or bottle, pick up finger foods, pass a toy from one hand to the other, or hold a piece of paper or crayon. Another question to ask is if the children tend to ignore one of their extremities or do they vol- untarily use the extremity? In older children, assessing their ability to dress and toilet themselves, comb their hair, button clothing, tie shoes, etc. becomes more important. Older children may be too embarrassed to admit to some functional limitations and questions may best be asked of parents and patients separately. Also ask about the children’s cognitive ability: are they grade appropriate for age, what kind of grades are they making? We have found a parent/patient questionnaire helpful in asking some of these questions (Table 8.1).

Physical Examination

A careful physical examination is done of passive and active range of motion of all joints from the shoulder distally. Evaluation of fixed muscle contractures versus dynamic muscle contractures, as well as recognition of joint contractures and/or joint subluxations and dislocations, are all very important. Even though the Ashworth scale is subjective, it is a good estimate of the tone of the extremity being tested. Particular attention is paid to children’s abilities to abduct and flex the shoulder, fully extend the elbow, supinate the forearm, and extend the wrist. The ability of children to do active finger extension with the wrist held in passive extension provides a means of separat- ing out lack of wrist extensor power from contracture of the finger flexors.

If children can do active finger extension with the wrist extended 20° to 30°, finger motor function is good (Figure 8.1). If the fingers cannot be actively extended but can be passively extended, lack of extensor motor power is the problem. If the fingers cannot be passively extended, the primary problem is lack of contracture of the finger flexors. The thumb is examined for active

Table 8.1. Upper extremity function.

Functional Type:

A Extremity is not functional

B Can use hand as a paperweight, pressure assist, or posting device; is able to swipe a toy and turn a switch on and off

C Hand has mass grasp but poor active control

D Hand has active grasp and release and can place an object with some degree of accuracy

E Hand has fine pinch useful for holding a pen or pencil, has key pinch with the thumb F Normal function, can be used for buttoning and shoestring tying, thumb has fine

tripod opposition

Within each type, also assess level of contractures:

I. No contractures II. Dynamic contractures III. Fixed contractures

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abduction and extension. Particular attention is paid to whether the deformity is a result of a muscle group being spastic with a fixed contracture and/or an opposing muscle group that has excessive weakness. Similar to the finger examination, the thumb should be examined with the wrist in 20° to 30° of dorsiflexion. The inability to passively abduct the thumb means there is a contracture of the thumb adductors. Passive abduction, which cannot even be done in part actively, demonstrates a lack of thumb abduction motor power. The typical thumb-in-palm deformity results from tight adductors of the thumb, tight flexors of the thumb, or weakness of the thumb extensors and/or thumb abductors. Most often a combination of causes of the muscle imbalance exists.

Sensibility must also be carefully evaluated. Two-point discrimination (>15–20 mm) is the ideal to test but it is difficult to accurately ascertain in children under 10 years of age. In younger children, tactile sensitivity, graph- esthesia, stereognosis, and proprioception are easier and more accurate to test. The sensation is a good measure of the overall functional ability of the extremity.

Classification of Upper Extremity Involvement

Classification of patients’ overall function is helpful to not only assess functional limitations, but also to determine realistic long-term goals and possible benefits from surgery. Most classifications that have been devised to describe the cerebral palsied upper extremity focus on a specific deformity or function (e.g., grasp and release at the wrist or thumb function). Few classifications describe overall function, are practical in the busy clinical setting, and have been correlated with parents’ impression of patients’ function.

Some, such as Jebson, have shown strong clinical correlation with function, but children with CP cannot perform many of the timed tests to accurately

Figure 8.1. Upper extremity involvement covers a wide spectrum of muscle contractures and postures. The typical arm has elbow flexion, wrist flexion, and forearm pronation (A). It is important to assess the degree of wrist and finger flexion contracture. For example, this wrist has minimal wrist flexor or joint contracture, as demonstrated by the ease of wrist extension with the fingers flexed (B). However, there are significant finger contractures as demonstrated by the difficulty in extending the fingers with the wrist extended (C). In addition to assessing the contracture, the ability for active motion in each position also needs to be evaluated.

A B

C

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score the test. The use of the tasks of the Jebson test are helpful; however, the timed nature of the scoring often does not reflect the significant pattern changes that a surgical procedure produces (Figure 8.2). A functional classification system based on six categories was developed for use in the clinic (Table 8.2).

Using this classification, we classified 54 pediatric patients with spastic CP into one of the six types. The classification type strongly correlated with the parents/patients questionnaire described earlier. All 14 patients who were surgically treated improved by one type or more indicating that the classification is sensitive enough to show change with surgery. This classification is also easy to perform in a busy clinical setting.

Guidelines for Setting Goals

Parents are concerned about upper extremity function usually after the concern about children’s walking has been addressed. Upper extremity function becomes a major issue as fine motor skills are being considered between 3 and 5 years of age, and become more sharply focused as these children enter school. Goal setting for the treatment and expectations of upper extremity function has to consider the children’s age, cognitive function, physical function, and cosmetic concerns. In general, the guidelines based on young Figure 8.2. Only measuring individual joint

functions can miss the functional use of the limb. The way a helper hand is used also needs to be evaluated with more complex functional measures, such as asking the child to screw a nut onto a bolt. The preoperative pattern shows swan neck deformities with wrist flexion (A); however, after reconstruction of the forearm with a pronator release, thumb adductor release, and transfer of the flexor carpi ulnaris to the extensor carpi radialis brevis, the pattern of use of this helping hand has changed completely. The same task can still be performed but the new pattern of use allows a firmer grip in a position in which the screw is more visible (B).

A B

Table 8.2. Upper extremity functional patterns.

Type 0 No active function in the entire upper extremity

Type 1 Proximal function, none to minimal distal function (uses hand as a paperweight/

posting device)

Type 2 Mass grasp, poor active control and strength, poor fine motor control Type 3 Fair active grasp/release (able to place object with fair accuracy), poor thumb

opposition

Type 4 Good active grasp/release, fair thumb opposition (key pinch only) Type 5 Normal to near-normal function, good thumb opposition, able to perform

sophisticated fine motor tasks (e.g., buttoning clothes)

Each type is further subdivided into A, no contractures; B, dynamic contractures only;

and C, fixed contractures only.

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childhood, middle childhood, and adolescent age groups can be used for setting goals.

Early Childhood: Ages 0 to 6 Years

Children may have considerable spasticity early in their development that usually increases between 9 months and 2 years of age. Relaxation or decrease in the spasticity occurs in early childhood as neurological maturation occurs. In early childhood, gross motor coordination is developing. During this time the focus should be on occupational therapy to help develop these skills. Encouraging children to use two-handed toys so that they can develop the use of their more involved hand, especially as an assist hand, is important in this age group. Splinting at this age should be minimized to night us- age if there are contractures. Some functional daytime splints, such as soft opponens-type thumb splints to keep the thumb out of the palm, may also be helpful. Bulky splints that discourage the use of the involved extremity should be avoided at all costs. Also, splints that cover a significant surface of the palm and palmar surface of the fingers should be avoided because this removes sensory and tactile feedback ability from the hand and will encour- age ignoring the hand, not spontaneous hand use.

Middle Childhood: Ages 6 to 12 Years

Children in this age group generally have maximized gross motor function but are continuing to develop fine motor skills. Also, helping these children to develop skills in activities of daily living, such as getting dressed, self-toileting, and feeding, is extremely important at this age. As these children are going through a rapid growth spurt, muscle contractures are beginning to occur. Dy- namic contractures can be treated with botulinum toxin injections into the affected muscles. Surgery should be considered for fixed contractures in patients who meet the proper surgical prerequisites. Cosmetic concerns about the appearance of the extremity arise in these children during middle childhood.

Adolescence: Ages 12 Years and Older

Individual functional development will focus on activities of daily living and skills, such as recreational activities in younger adolescents and vocational and educational activities in older teenagers. This development will help individuals become independent in school. For some children with more severe involvement, the use of an aide to assist with handwriting and also learning to operate a laptop computer is very helpful. Focusing on what works best for an individual child is most important. Trying to force children into a traditional predetermined mold of the way these children should use the involved extremity can be damaging to their self-esteem. For example, trying to force a child into doing a timed handwritten essay test or penalizing them for poor handwriting is humiliating and fruitless to their overall development, particularly with the technology that is available as an assistive writing device. Surgery can still be performed at this age. It is important to communicate to families and children the realistic functional gains that can be expected with any surgery. On the other hand, the benefits of a cosmetic improvement in the appearance of an involved extremity should not be underestimated.

Of course, overall goals must be individualized after a careful history, physical examination, assessment by therapists and surgeons, and realistic expectations of parents and patients.

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Treatment

Preoperative Evaluation

In addition to a careful history and physical examination by an orthopaedic surgeon, more detailed evaluation by an occupational therapist is extremely helpful. This evaluation generally takes approximately 1 hour and details range of motion, manual muscle testing, assessment of gross and fine motor skills, and assessment of tone and sensation. Occupational therapists will often note details that the surgeon did not for reasons of time limitations. A clear definition of the expected goals of the prescribed treatment is important to help physicians be realistic and for families to hear what they can expect.

For example, an arm is never normal after any treatment, which is clear to physicians but must be stated especially clearly to adolescents. An adolescent may say “Oh yes, I know that” but usually continues to harbor unrealistic expectations. This conversation causes her to verbally acknowledge these expectations. Other diagnostic testing, such as dynamic electromyography (EMG), has been advocated by some clinicians as helpful in the planning of muscle transfers. Muscles found to be in phase with the recipient muscle have been found by some investigators to perform better than those that are non- phasic.¹Hoffer et al. state that flexor carpi ulnaris transfer to the wrist extensors causes excessive wrist extension if it is done out of phase.²We have not used dynamic EMGs in our preoperative planning regimen; however, based on the published reports, it would seem to improve diagnostic workup.

Control of Spasticity

Neuromuscular blocks using botulinum-A toxin can be injected directly into the desired muscle belly to be weakened. This injection can be helpful in pre- dicting the effects of muscle lengthening, although it may be ineffective in the presence of a fixed tendon contracture. The injections can also be used therapeutically; however, they must be repeated every 3 to 4 months because the effects are overcome with neurologic recovery. In the past, ethanol and phenol injections were also used; however, these drugs are very painful to inject and generally require sedation. Botulinum-A toxin, on the other hand, can be injected in the clinic setting without sedation. Also, ethanol and phenol cause pain from sensory neuropathy and muscle fibrosis so they are seldom used today.³Botulinum-A is particularly helpful in younger patients with dynamic spasticity that is interfering with function. In a double-blinded trial, Corry and colleagues found botulinum-A toxin to be effective in improving range of motion and gross motor function in the short term.⁴Long-term benefits are unknown. Aggressive therapy after injection helps strengthen antagonist muscles. Dosages of 20 to 50 units per muscle belly are utilized, limiting the total dosage to 10 to 15 units per kilogram.

During the period of enthusiasm for dorsal rhizotomy, there were many papers extolling the benefits of lumbar rhizotomy on the tone reduction and functional gain in the upper extremities.^{5, 6}However, by using age-matched controls, it was apparent that the effect being recorded was improvement related to maturation and was not related to the rhizotomy.⁷Dorsal lumbar rhizotomy currently has no substantial benefit on upper extremity function.

Likewise with the introduction of intrathecal baclofen, there have been many reports suggesting a reduction of upper extremity tone.^8–11In our experience, this reduction of tone is related to the local placement of the intrathecal catheter. If the catheter tip is placed between C6 and T3, good reduction of upper extremity tone occurs. As the catheter is progressively placed lower,

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there is less upper extremity effect. Intrathecal baclofen to control upper extremity spasticity can be helpful if the catheter is threaded up into the high thoracic or low cervical region. Careful postoperative monitoring is important to look for any signs of respiratory depression.

Orthopaedic Surgery

Several prerequisites have been identified as reliable indicators for functional improvement after upper extremity surgery in children with CP. These include voluntary control of grasp and release, sensibility, intelligence, age of the patient, and type of neurologic involvement. As in the lower extremity, it is best to identify and treat all deformities at one time to prevent secondary deformity and to decrease multiple surgical events. It is very appropriate to consider the cosmetic concerns of families and patients, because the upper extremity, especially the forearm and hand, is the second most publicly visible fully exposed segment of the human body after the face. Improving the appearance of the upper extremity can make a large difference in the self-image of developing adolescents.

Voluntary Control

Voluntary control of grasp and release is the most important criteria to assess children with CP with upper extremity involvement, and it provides the most reliable indicator for functional gains after surgery despite poor indicators in some of the other categories. Zancolli and Zancolli have defined grasp and release patterns according to three patterns.¹²In pattern 1, active finger extension is possible with the wrist in less than 20° of flexion. These patients have difficulty with prehension due to contraction of the wrist flexors during active grasp. In pattern 2, active finger extension is not possible unless the wrist is allowed to flex more than 20°. Subgroup A of pattern 2 has wrist extension with full finger flexion, whereas subgroup B has no wrist extension. In pattern 3, active finger extension is not possible even with maximum wrist flexion. Severe deformity of the fingers and wrist exists. Pat- terns 1 and 2 have the most functional benefits after surgery. Pattern 3 cannot be improved functionally but can have improvement in cosmesis and hygiene. All these patterns are usually associated with some adduction or flexion deformity of the thumb, pronation deformity of the forearm, and flexion contracture of the elbow (Figure 8.3). All patterns must be assessed for the presence or absence of voluntary control.

Sensibility

Sensory deficits are present in most patients with CP with upper extremity involvement. Sensory testing in these patients has been previously described.

Although important, sensibility should not in itself be a contraindication to surgery. Increased severity of sensory deficit is a reflection of an increasing severity of the neurologic impairment. Many children effectively use hand–

eye coordination to compensate for defects in stereognosis and proprioception, particularly if they have good voluntary control. Also, the spastic limb can learn by experience, as shown by tests of fingertip force application based on the material presented.^{13, 14}Even though the uninvolved side of children with hemiplegia is believed to be normal, fine motor testing usually shows deficits in tactile processing.¹³

Intelligence

The ideal patient to consider for surgical reconstruction has an intelligence quotient of greater than 70, adequate behavior, cooperation, and motivation

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before undertaking upper extremity surgery. Postoperative cooperation in therapy is also important. Once again, minor abnormalities in mental status should not contraindicate surgery if children have good voluntary control.

These criteria are most important if the goal is to make functional gains;

however, they are of little importance if the treatment is done to improve cosmesis or improve custodial care problems.

Patient Age

Most orthopaedic surgeons have advocated delaying surgery until age 4 years when adequate maturation of the nervous system has developed and when some degree of patient cooperation can be expected. Traditional teaching is that the ideal age to consider surgery is between 4 and 9 years. We have found children between the ages of 7 and 12 years to be ideal candidates for surgery. This age range gives children enough maturity to cooperate with occupational therapy and enough skeletal growth where recurrence due to increasing muscle tightness secondary to growth is at less risk. These patients are also not too old for retraining of transferred muscles, and they have reached a plateau in their neurologic development.

Neurologic Type

Patients with spasticity benefit most from surgery. It is extremely important to distinguish dystonia from spasticity, which can look very similar. Dystonic patients do poorly with muscle transfers and lengthening as do most patients with movement disorders (including athetosis). In general, tendon surgery should be avoided in patients with movement disorders. Some individuals, especially those with athetosis, may benefit from restraining the nondomi- nant extremity during fine motor skill tasks.¹⁵

Shoulder

Shoulder Contractures

Individuals with quadriplegic pattern involvement often develop shoulder contractures. Typically, these contractures start to become noticeable in Figure 8.3. Specific patterns of spastic hand

deformity, based primarily on the grasp pattern, have been described by Zancolli and Zancolli.¹²Type 1 deformity has full active finger extension with the wrist at 20° of flexion or less. The wrist is in neutral or slight flexion with grasp. In type 2, there is full active finger extension but the wrist requires more than 20° of flexion for this to be possible. Finger grasp occurs with significant wrist flexion only (A). Type 3 pattern has little active finger extension or grasp function (B).

A B

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middle childhood and become more noticeable in adolescence. The most common deformity is protraction and elevation of the shoulder through the scapulothoracic joint, with the clavicle becoming more vertical and anteriorly directed. As severely involved patients become adults, this shoulder position becomes fixed but seldom causes any pain or discomfort. In spastic patients, internal rotation contracture of the shoulder develops as a result of spasticity of the pectoralis major and subscapularis muscle. On rare occasions, extension and external rotation abduction contractures develop, often caused predominantly by the long head of the triceps and teres muscles.

Natural History

The natural history of shoulder contractures is for increasing severity during late childhood and adolescence with minimal change after hormonal and skeletal maturity. Also in middle childhood, primarily in children with quadriplegia, shoulder adduction, internal rotation, and flexion contractures develop. As these contractures become more severe, especially at puberty with the hormonal changes and the growth of axillary hair, the contractures become so severe that proper cleaning and drying of the axilla becomes very difficult. Also, dressing these children, especially placing arms in sleeves, becomes very difficult. For other functional positions, such as seating and different reclining positions, this upper extremity position is good.

During adolescence, there are a small group of children who develop an external rotational abduction contracture of the shoulder. This becomes a functional problem, especially when seated in a wheelchair, as the arms tend to strike walls as these children are being transported. This shoulder position also limits side lying.

For ambulatory children, the most common hemiplegic posturing is with shoulder elevation and protraction combined with adduction, flexion, and internal rotation. This becomes severe enough to cause functional problems only in rare ambulatory children with hemiplegia. There are also a few children who develop shoulder extension and external rotation combined with elbow extension. In ambulatory children this is usually a sign of dystonia, although this may be encountered in individuals with spasticity and contracture (Figure 8.4).

Figure 8.4. Shoulder and elbow extension can be disabling because it causes the arm to be behind and lateral to the individual. This may lead to the arm getting bumped or strik- ing furniture, and it is a significant cosmetic problem (A). After proximal release of the lateral and long head of the triceps, the elbow and shoulder flexion are greatly improved (B). This also allows the arm to hang at the side during ambulation (B). (Case Material contributed by Federico Fernandez- Palazzi and Joaquin Xicoy-Forgas, Caracas, Venezuela.)

A B

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Treatment

In childhood, the primary treatment of shoulder contractures is by passive range-of-motion exercises. Splinting is of no use, especially the attempt to use figure-of-eight straps on the shoulders to counteract the shoulder protraction and elevation. These straps have too little mechanical advantage to make an impact without causing children discomfort.

As children with quadriplegia enter puberty and approach maturity, problems related to dressing and hygiene develop. When the parents or caretakers report problems, treatment is indicated. By this time the contractures are fixed and only surgical lengthening will make a difference. The goal of surgery is to lengthen the shoulder internal rotator and adductors enough so children’s arms can easily be placed in sleeves and the axilla can be cleaned.

Obtaining 90° of shoulder abduction in the operating room is very adequate to accomplish these goals. Usually, this abduction is accomplished with complete release of the pectoral muscles (Case 8.1).

Jessica, a 16-year-old girl who was completely dependent in all activities of daily living, was brought by her caregivers from a facility where she lived. Her mother cared for her at home one weekend a month and was also present. They agreed that the major problem was difficulty in dressing her left upper extremity because her arms were very stiff. The caregivers also complained that it was very difficult to clean her axilla and they could not control her strong body odor because of difficulty with bathing, especially in her axilla and wrist flexion crease. She had had multiple previous surgeries including spinal fusion for scoliosis and hip osteotomies for spastic hip disease.

She had severe mental retardation and seizures. On physical examination, the shoulder demonstrated severe fixed contracture allowing less than 20° of abduction, fixed internal rotation, elbow flexion contractures of 90°, and a fixed wrist flexion contracture with no functional use in the extremity (Table 8.1). She previously had a surgical correction of the right upper extremity. With the goal of improving custodial care, she had release of her pectoralis muscles and subscapularis. In the operating room she could be abducted to 90° at the shoulder and externally rotated almost to neutral. The elbow had a complete release of the biceps, brachialis, and brachioradialis muscles allowing extension to −60°. The wrist had a proximal row carpectomy flexor tendon lengthening and plication of the finger extensors. Postoperatively, she developed a position similar to the right upper extremity with improved ability for bathing and dressing (Figure C8.1.1).

Case 8.1 Jessica

Figure C8.1.1

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For children who have extension and external rotation contractures, usually with a major dynamic component, the triceps can be injected with botulinum toxin. Temporary relief will usually be noted and the definitive treatment is to release the long head of the triceps at the shoulder. This release will allow the limb to hang at the side or stay in the flexed position. If this procedure is done in individuals with dystonia, a severe flexion, adduction, and internal rotation deformity usually develops.

For adolescents with severe contracted abduction and external rotation shoulder contractures, usually bilateral in individuals who use wheelchair mobility, the most reliable procedure is humeral osteotomy. This osteotomy is rarely needed but does address the problem in a definitive and more reliable way than trying to relax all the contracted muscles. Humeral osteotomy is also the best procedure for individuals with predominantly internal rotation contractures who want the arms to be in a more normal position (Case 8.2).

Outcome of Treatment

There are no published reports of the outcome of shoulder adductor lengthenings; however, in our experience, the goals that can be reliably ob- tained are usually limited to improved ability to dress children and provide better personal hygiene. If the procedure is done during puberty there does not seem to be much recurrence of the contracture. Recurrence is the main problem if shoulder adductor lengthenings are done on younger children.

Other Treatment

When these fixed contractures develop, there are no significant options other than surgery. When the problem is noted, there is often an increase in therapy as an attempt to stretch out the contractures. This therapy occasionally seems to help a little, but the passive range of motion is a common cause of proximal metaphyseal humeral fractures as these children are very osteopenic from minimal upper extremity use. Therefore, if passive stretch- ing is instituted, it should be done by an experienced therapist.

Complications of Treatment

Surgical lengthening of the shoulder adductor, triceps release, or humeral osteotomy have minimal complications.

Shoulder Instability

Shoulder joint instability is relatively common in individuals with quadriplegic involvement and children in middle childhood with athetosis. The most common pattern is anterior subluxation as the shoulder becomes pro- tracted and elevated. The humeral head becomes subluxated anteriorly toward the coracoid process. These subluxations cause children no pain but are often accompanied by decreased range of motion, especially external rotation and abduction. The increasing contracture may cause problems with dressing or cleaning the axilla.

Complete acute dislocation of the shoulder, either in the anteroinferior direction or the posterosuperior direction, also occurs. For many children in middle childhood or adolescence, this event causes pain but the shoulder is easy to reduce. In a few children the shoulder becomes dislocated and is not painful. The fixed dislocations occur in individuals with severe involvement and they do not become painful over time.

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Timothy, a 17-year-old boy, complained that his arm always hung in front of him and bothered him, especially when he wanted to walk fast. Also, he was concerned about the appearance of the limb. He was in 11th grade of a regular high school and did age-appropriate aca- demic work. He used the extremity as a helper hand but he was not concerned about improving the function of the extremity. On physical examination he demonstrated the ability to raise the arm to 90° of forward flexion, maximum external rotation to only −45°, and the elbow could be flexed fully and extended actively to −30°. On physical examination the shoulder could be externally rotated to almost neutral passively (Figure C8.2.1). Forward flexion and abduction appeared to be full. The elbow extended to a minimum of 15° with full flexion on passive motion.

He stood and walked with the elbow flexed and the shoul-

der internally rotated. Because this was thought to be a shoulder with useful function, we did not want to do ex- tensive muscle lengthening; therefore, he had a derotation osteotomy of the humerus and a myofascial lengthening of the biceps and brachialis (Figure C8.2.2). Postopera- tively, he had 30° of external rotation of the shoulder, and the elbow came to −20° of extension. He was happy with the result, and although the limb still tended toward internal rotation, it was better than fixed internal rotation.

Case 8.2 Timothy

Figure C8.2.1 Figure C8.2.2

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Natural History

Very few fixed shoulder dislocations become painful. The painful acute dislocations may slowly resolve over time as the shoulder is protected. A small group of individuals, usually late adolescents or young adults, develop severe instability with multiple recurrent dislocations. These dislocations may become progressively more painful as joint degeneration occurs.

Treatment

Fixed anterior subluxations and fixed dislocations of the shoulder with no pain seldom need any treatment. Acute dislocations should be reduced and the position causing the dislocation avoided, which may require the use of a sling but needs to be individualized to each child. Parents and therapists need to be advised to avoid moving the arm into the position where the instability occurs. Caretakers need to be advised to avoid lifting the children by holding the arms and definitely never lift them by pulling the arms into the extended and flexed overhead position. If recurrent dislocations get worse and do not slowly resolve, which almost always happens in childhood and adolescence, surgical reconstruction should be considered using standard shoulder stabilization procedures. If reconstruction fails or if the joint has severe degenerative arthritis, fusion of the shoulder joint is a good option.

Occasionally, athetoid or dystonic patients will have an unstable shoulder. An unstable shoulder is an extremely difficult problem to treat because standard soft-tissue repairs tend to stretch out when patients continue to posture the shoulder in an unstable position. Careful positioning of the shoulder, trying to avoid surgical treatment, is first attempted. Shoulder fusion may be required in resistant cases. We have had one dystonic patient whose shoulder became increasingly unstable as her scoliosis progressed.

After correction of her spinal curvature with a spine fusion, her shoulder reduced and became stable.

Elbow and Forearm

Elbow Flexion Contracture

The elbow flexors have a significant mechanical advantage over the elbow extensors; therefore, when severe spasticity occurs, the flexors tend to shorten, which causes an elbow flexion contracture. The biceps is a two-joint muscle and is the primary cause of the contracture. The brachialis and brachioradialis are one-joint muscles that also develop contractures, especially with severe and long-standing contractures.

Natural History

Fixed contractures tend to develop in late childhood and adolescence. For individuals with severe quadriplegia, the flexion contracture may become so severe that bathing and keeping the elbow flexion crease clean becomes difficult. In individuals with hemiplegia, the position of the flexed elbow causes a significant cosmetic concern. Usually, by young adulthood, the contracture is fixed and not progressive.

Treatment

In young childhood and middle childhood the use of extension splinting may be helpful, although there is no good documentation. Injection of the elbow flexors with botulinum toxin has been reported, but none of these reports

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suggest that any significant lasting benefits occurred.^{4, 16, 17}Botulinum toxin may be useful for a few individuals in whom short-term goals are defined.

As the contracture becomes very severe and causes problems with hygiene, a surgical release of the elbow flexors is indicated. In individuals with severe quadriplegia and severe contractures, the release should include a complete transection of the distal biceps, brachialis, and brachioradialis. Extension splinting then follows this transection. This level of release usually allows elbow extension to be between approximately 60° and 90° of flexion, which is enough to allow for bathing and keeping the elbow clean.

In individuals with hemiplegia, the release of the flexion contracture is indicated because of a cosmetic concern of the elbow always being in a flexed position. If individuals are very functional with their arms and the contractures are mainly dynamic, a Z-lengthening of the biceps tendon is indicated.

If the arms are less functional or a fixed contracture of 10° to 20° is present, a complete release of the biceps tendon is indicated. For children with more severe positioning, especially if their arms are held to almost 90° during ambulation, a myofascial lengthening of the brachialis is added. Elbow flexor lengthening during late childhood and adolescence does seem to provide a permanent improvement in elbow extension.¹⁸

Severe fixed flexion contracture of the elbow is encountered mainly in quadriplegia where there is little indication to treat the contracture beyond a muscle lengthening. In rare occasions, a severe contracture may present in hemiplegia, where there is an indication to gain extension for a specific functional gain or cosmetic concern. Treatment with an extension osteotomy of the distal humerus is a safer and simpler approach than trying to do a complete capsulotomy. Elbow joint resection with a flexor release has been reported as a treatment of severe elbow flexion contractures,¹⁹but we have no experience with this procedure.

Complications of elbow flexor release are rare. The most serious complication is injury to the brachial artery or the medial nerve during flexor lengthening. This complication is best avoided by doing the lengthenings through an open incision and under tourniquet control for optimal visuali- zation of the operative field. Loss of elbow flexor power is only of concern in a few individuals with very heavy use of the extremity. Lack of active flexion has never been encountered and complaints of elbow weakness are almost never reported. Most individuals are happy with the degree of improvement.

Radial Head Dislocation

Radial head dislocation is a relatively common problem in severe quadriplegia with elbow flexion contracture and pronation contracture. Radial head dislocation reportedly occurs in 2% of all children with CP involving the upper extremity when elbow radiographs are carefully evaluated.^{20, 21} In those with severe elbow flexion and forearm pronation contractures, 27%

have radial head dislocation.¹⁸ This association seems to suggest that the flexion contracture combined with the pronation contracture causes the dislocation. Most of these dislocations are posterior, which is also the position into which the radial head would move with elbow flexion.

Natural History

In middle childhood, the radial head starts to migrate posteriorly and later- ally as the pronation and flexion contracture increase. There is a time when

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the elbow may develop pain as the radial head dislocates and then reduces again. Most children develop a fixed dislocation rather quickly and no more pain is noted. For a few children, this pain is increasing and parents may want to have the problem treated. As the radial head becomes dislocated, both the contracture and the radial head dislocation usually limit elbow extension and forearm supination. As children continue to grow, the radial head may become very prominent on the posterolateral aspect of the elbow.

During late adolescence or young adulthood, the prominence of the radial head may lead to skin breakdown from rubbing on wheelchair trays. Also, in individuals who have functional use of the elbow, pain from degenerative arthritis may develop.

Treatment

Most children with radial head dislocations do not need active treatment.

Early recognition and preventive treatment directed at splinting and early contracture release has been recommended as a way to prevent radial head dislocations.²²However, we have not found this to be a useful approach and there are presently no objective data to support early intervention. Although radial head dislocation is a common problem in children with CP, there are only a few reports that mostly focus on reporting that the deformity exists.^20–22This is reflected in practice because few caretakers or children com- plain of pain or disability from this problem. If children do have pain that is persistent and parents wish to pursue intervention, surgical stabilization is the only option. The surgical procedure requires release of the elbow flexors and pronation contracture, followed by stabilizing the radial humeral joint by reconstruction of the annular ligament, usually using the transected biceps tendon. The indication for this procedure in children with passively reducible radial head dislocations is not clear (Case 8.3). The outcome of this procedure is usually a fixed dislocated radial head that is pain free. Fixed dislocations in childhood CP are best left untreated, as they are seldom painful.

Pain, if it develops, occurs in adolescents or young adults. At this time, the best option is radial head excision. Excision may also be occasionally indicated for adolescents with very prominent radial heads that cause skin breakdown (Case 8.4).

Surgical reconstruction in children with severe spasticity has led to a 66%

(four of six) rate of repeat dislocations. These dislocations did not cause pain;

therefore, if the surgery was done for pain relief, it would be successful but maintaining a reduced radial head was not successful. Radial head excisions have been successful in decreasing pain and removing the lateral mass. Ra- dial head excision is not recommended before completion of growth because of fear of radial overgrowth proximally and of proximal migration of the radius causing increased problems at the radioulnar joint at the wrist.

Forearm Pronation

Pronation contracture is a very common deformity in both quadriplegia and hemiplegia (Figure 8.5). The primary deforming muscle is the two-joint pronator teres. At the later stage, the one-joint muscle, pronator quadratus, may become contracted. The pronation contracture is almost always combined with a significant flexion contracture caused by the biceps, which is the strongest supinator. Therefore, the typical release or lengthening of the biceps to treat the flexion contracture also weakens the forearm supination.

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The mother of Shakoor, a 6-year-old boy with severe quadriplegia, complained that he cried when she put his arm in a shirt sleeve. She also reported feeling a click when she moved his arm. Shakoor was a dependent sitter with no functional use of the upper extremity. On physical examination the right radial head could be palpated in an anterolateral dislocated position. With forearm supination and flexion, the radial head reduced easily but seemed to cause him discomfort. When the arm was pronated and extended, the radial head again dislocated, which again caused the child some discomfort. Radio- graphs confirmed the physical examination (Figure C8.3.1). An operative reconstruction was performed, which transected the biceps tendon at the musculo- tendinous junction and used this tendon as the material to form an annulus ligament. The elbow was immobilized for 4 weeks, and 3 months later the radial head was dislocated in a fixed position but without pain. The mother was happy with the result (Figure C8.3.2). Another similar case had a reconstruction with an ulnar osteotomy, be-

cause there was always some bowing deformity in the ulna as part of the deformity (Figure C8.3.3). This, too, went to a fixed dislocation within 3 months postoperatively but again was pain free (Figure C8.3.4). Another example (Figure C8.3.5) was a case in which the radial capetellar joint was fixed with a pin for 6 weeks (Figure C8.3.6);

however, this led to a dislocation of the ulna trochlea joint and severe elbow stiffness (Figure C8.3.7).

As demonstrated in these cases, we do not have a good operative solution for the spastic radial head dislocation, which is the reason we favor decreasing activity and allowing the dislocation to become fixed, then the pain will resolve. Such an example is a 6-year-old boy who presented with almost the same history as above, whose mother was instructed to avoid activities that caused pain and was not to try to splint or otherwise position the arm in a position to prevent the radial head dislocation (Figure C8.3.8).

After a 4-year follow-up, the radial head was in a fixed dislocation with substantial radial head remodeling, and the elbow was pain free (Figures C8.3.9, C8.3.10).

Case 8.3 Shakoor

Figure C8.3.1

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Figure C8.3.4

Figure C8.3.5

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Figure C8.3.6

Figure C8.3.7

Figure C8.3.8

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Figure C8.3.9

Figure C8.3.10

The mother of Corey, a 17-year-old boy with quadriplegia who had some limited use of his upper extremities, complained that he seemed uncomfortable with the extension of the elbows. His mother was especially concerned because he was hitting his elbow against the back of the wheelchair or walls, and getting skin breakdown over the lateral elbow, where he had a prominence from his dislocated radial head (Figure C8.4.1). Physical examination demonstrated mild tenderness with passive elbow extension, which was limited by a 35° fixed flexion contracture. Radiographs of the elbow demonstrated the dislocated radial heads (Figures C8.4.2, C8.4.3). Af- ter radial head excision, his elbow flexion contracture was unchanged, but he no longer had pain and the skin breakdown stopped. Radial head excision gives excellent symptomatic relief, although no change in function should be expected.

Case 8.4 Corey

Figure C8.4.1

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Natural History

The natural history is for the pronation contracture to get worse during growth. For most individuals with hemiplegia, the pronation is a cosmetic deformity that causes functional disability by placing the palm out of sight.

Some degree of pronation contracture is almost always present in children with spasticity of the upper extremity. The deformity aggravates the wrist flexion deformity and when it is severe, patients have a reverse grasp posture. The deformity can also cause difficulty grasping on to handholds of walkers in patients who require assistive devices for walking. Because most hemiplegic limbs have reduced sensation, the use of the hand as a helper requires visual feedback for individuals to know what is in the hand. By decreasing the pronation and allowing the palm to be seen, the hand can be used to hold objects in a more functional manner.

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Treatment

Pronation deformity of the forearm is almost impossible to effectively splint unless a full upper extremity orthosis with the elbow flexed to 90° is used.

This type of orthotic is so large that it has severe functional implications;

therefore, pronation orthotics are seldom used. Usually, the pronation contracture is treated only in the context of an upper extremity reconstruction in which the pronation contracture is recognized as one of many problems.

Correction of the pronation contracture should be performed in combination with procedures to correct the wrist and finger deformities to optimize placement of the hand. The goal of treatment is to reduce the pronation deformity; however, this should not lead to a fixed supination position. A position between neutral and 30° of pronation is best with active supination to 45°. Surgical treatment is indicated when individuals lack the ability to actively supinate at least 20° to 30°.

Many surgical options have been described, and the data on each procedure do not give each a clear advantage. Release of the pronator tendon from the insertion on the radius removes the primary deforming force. This procedure works well over a wide group of individuals, is the simplest procedure, and has the least complications. Transfer of the distal insertion of the pronator from the dorsal insertion on the radius to wrapping the tendon around the volar aspect of the radius and making it a supinator has been reported to increase the amount of active supination compared with a release only.^{23, 24}Another option is to transfer the distal end of the pronator into the extensor carpi radialis to provide wrist extension and decrease pronation.^25–27This procedure probably provides the least pronator weakening and has the advantage of assisting wrist extension. This is a reasonable option if there is a mild supination or pronation deformity with tendency for dynamic wrist flexion; however, there are few children who fit this mild level of involvement and still need surgery. Another option is the proximal pronator flexor slide at the muscle origin. We have found very little indication to do proximal pronator flexor slides in children with good function. We have rarely utilized a proximal pronator flexor slide; it has been used in a child with residual head injury and in a child with severe quadriplegia.

Figure 8.5. The most typical posture of the hemiplegic upper extremity is elbow flexion, forearm pronation, wrist flexion, thumb adduction, and finger extension. Finger flexion contractures usually underlie the extended finger when the wrist is brought to the neutral position. All these individual deformities need to be closely evaluated and each needs to be corrected if the deformity is present when doing a reconstruction of a spastic upper extremity.

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For severe deformities with fixed pronation contractures, a myotomy of the pronator quadratus may need to be added. These children often have a contracture of the interosseous ligament as well, which may need to be stretched out by casting or even cross-fixated with a K-wire.

Outcome of Treatment

Sakellarides and associates found 82% of their patients had good to excellent results with an average of 46° of active supination.²³They did have radius fractures as a complication. Strecker et al. modified this technique by only utilizing a larger unicortical hole with a smaller drillhole to pass suture through and around the radius.²⁴When comparing pronator release with rerouting, they found the latter group to have improved supination.

The worst complication of pronation contracture treatment is overcorrection and development of significant supination deformity. The supination is much more cosmetically objectionable and more functionally debilitating than an equal pronation deformity. If overcorrection occurs and a pronator transfer has been performed, the transferred tendon has to be released. If a transfer of the flexor carpi ulnaris was performed around the lateral ulna and this muscle is tight, causing the supination, the muscle should be released. If the biceps muscle is spastic or contracted and it was not released at the first procedure, it may also be the cause of the overcorrection.

One technique for transferring the tendon around the opposite side of the radius requires drilling a bicortical hole in the distal radius to place a suture to attach the tendon. This drillhole may become the stress riser for a fracture. If a fracture develops, it has to be treated in the standard method, usually with internal fixation using a plate. This complication can be avoided or reduced by using only a small unicortical hole into which a stay suture device is placed, or the tendon can be sutured to the periosteum instead of the bone.

Wrist

Wrist Flexion Deformity

Wrist flexion is a very common deformity in older individuals with spasticity of the upper extremity. In most individuals, the wrist is in flexion and ulnar deviation is caused by overpull and contracture of the wrist flexors. In most wrists, the flexor carpi ulnaris is the primary and most contracted muscle, followed by the flexor carpi radialis, and then the finger flexors. Because the forearm is usually in pronation, gravity also helps to cause the wrist to drop. As the wrist flexes, the extensor tendons of the fingers are put under tension and the finger flexors are relaxed; therefore, the fingers are usually in some extension, although this is variable. The strength and power of grasp in wrist flexion is very weak, so whatever limited hand motor function was present in children tends to be made worse with the poor hand position.

As the wrist flexion deformity increases, the wrist joint tends to collapse and cause subluxation of the intercarpal joints. In some individuals with severe spasticity, the wrist comes to rest against the volar aspect of the forearm and the wrist flexion crease is very hard to keep clean, causing a foul odor to develop.

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Natural History

In young children under 3 years of age, the wrist is most commonly in the fisted position with the thumb in the palm under the flexed fingers. As neurologic development occurs, the wrist drops into flexion, allowing the fingers to open and become more functional. For children who are crawling on the floor, weight bearing on the upper extremity may start with dorsal weight bearing, then for some as the finger flexors relax, palmar weight bearing starts. As children enter middle childhood, the predominant flexed wrist position is established but is usually without fixed flexion contractures. In children with hemiplegia, the wrist flexion remains supple and functional gains may continue to be made into part of middle childhood. As children with hemiplegia enter adolescence, the contractures tend to become more fixed, although many will continue to have a primarily dynamic deformity.

In middle childhood and into adolescence, the functional gains can continue in individuals with quadriplegic involvement. Also, as the severity of the spasticity increases, the rate of fixed contracture development increases.

Diagnostic Evaluations

Hoffer et al. recommend the use of careful physical examination and dynamic EMGs to distinguish two patterns: primary difficulty with grasp and primary difficulty with release.¹Tendon transfer of the wrist flexors is favored over lengthening alone, and these patterns tend to exist. We have relied on the physical examination to separate out the patterns of wrist deformity (see Fig- ure 8.2). These patterns generally follow a pattern of severity of neurologic involvement in the children.

Mild Wrist Flexion Deformity

In a few individuals with hemiplegia, there is a very mild dynamic wrist flexion and forearm pronation present with no fixed contractures. When children are not aware of the extremity position, the wrist tends to be in flexion and when they use the hand, it tends to be predominantly in flexion and pronation. These children have full independent active control of the hand and wrist function. This extremity is classified as a type 5 extremity.

Moderate Wrist Flexion Deformity

The next level of involvement is those individuals with some fixed flexion contracture of the wrist but good active finger extension with the wrist held passively extended. These extremities fall into type 4 on the classification scale.

Severe Wrist Flexion Deformity

At the next level, types 2 and 3 are those individuals who cannot actively extend their fingers with the wrist passively extended to neutral to 20°

of extension. This group has two subgroups. The first subgroup includes those with contracted finger flexors such that the fingers cannot be passively extended with the wrist passively extended. The second subgroup has the ability to get passive finger extension with the wrist held passively in extension. This subgroup has finger extensor deficiency without finger flexor contracture.

Very Severe Wrist Flexion Deformity

The last group is predominantly types 0 and 1, and they have severe fixed wrist flexion contractures getting a maximum of −20° of wrist extension.

These individuals have minimal function in the hand.