Muscle imbalance, muscle weakness, and soft tissue contractures in the upper limb can create major problems in provision of care for children with neurological involvement. Muscle imbalances caused by spasticity, abnormal tone, or peripheral nerve injury can seriously impair a child’s upper limb motor development and independent function. If left untreated, these muscle imbalances often result in poor postural positioning of the affected limb. Muscle imbalances can cause the development of secondary joint contractures, joint incongruencies, and structural changes within the surrounding soft tissues. Functionally, the result is often an arm that cannot facilitate functional use of the hand. An example of this is the child whose inability to rotate the forearm while reaching impairs the ability to extend the wrist to achieve prehension during a tabletop fine motor task.
Children with obstetric brachial plexus palsy (OBPP) or cerebral palsy (CP) may present with upper limb functional limitations secondary to muscle weakness and imbalance. The incidence of OBPP varies from 1.6 to 2.9 per/1000 births in prospective studies.1,2 The incidence of children with CP is between 2 and 2.5 per/1000 births and is especially prevalent in infants born preterm with low birth weight <1,500 g.3 The United States does not have a nationwide CP registry, and many researchers rely on incidence figures obtained from small independent registries around the country. Children with OBPP and CP often present with poor alignment and motion of the shoulder girdle. This is often caused by muscle imbalance either from peripheral nerve injury as in OBPP or from heightened abnormal tone or spasticity as in CP. Spastic hemiplegia is a relatively common form of CP, which presents with an affected upper limb that assumes a resting position where the shoulder is internally rotated, the elbow is flexed, and the forearm is pronated. In this case, it is the spasticity or increased muscle tone that compromises movement of the upper limb through its full range of motion (ROM) necessary to position and functionally use the hand. Infants with OBPP or hemiplegic CP often require a special splint for their arm or hand, such as the prefabricated McKie splint,4 to position the hand or forearm in biomechanical alignment. Alternatively, the occupational therapist may fabricate a custom hand splint using low temperature thermoplastic material that can easily drape and stretch over the infant’s hand.
However, for the child with OBPP, the neurological recovery will depend on the muscle reinnervation and active ROM of the weak and innervated muscles. Children with OBPP5,6 may present with a shoulder joint that is limited either in internal or in external ROM. This shoulder tightness adversely affects the biomechanical alignment of the humerus, causing it to assume an extremely internally or externally rotated position. Enhanced action of the elbow flexor muscles is a consequence of this abnormal humeral alignment. The forearm assumes either a pronated or a supinated alignment depending on the proximal alignment of the humerus and the influence of the kinetic chain.
Long-standing muscle imbalance around the shoulder leads to progressive instability of the scapula and shoulder. Decreased elbow extension and forearm rotation limit the ability to orient the hand for functional activity. Clinical management is aimed at limiting contractures, improving passive ROM (PROM), and increasing active movement for functional skills. The age of possible surgical intervention for both the child with OBPP and CP hemiplegia depends on the problem and severity. Generally, the child with OBPP may have nerve surgery at an early age, within the first 6 to 9 months of life. However, for both the diagnoses, the procedure for a tendon transfer to support a weakened muscle is optimal when the child can participate in an active therapy program postsurgery.7,8
Serial casting has been successful for improving ROM. A long-arm cast9 encapsulates the elbow, forearm, and wrist. A series of long-arm casts can be applied to the impaired upper limb to gradually increase the ROM at the elbow and forearm. By incorporating the wrist, the forearm can be positioned into either supination or pronation to gently control the forearm contracture. After a period of serial casting, the child can continue with occupational therapy to improve the active range and strength. The serial casting program allows a slow gradual stretch to regain muscle length; however, the child continues to be at risk for return of the contracture and deformity because of the compensatory patterns, poor strength, and muscle imbalance.
Nuismer et al.10 performed a retrospective descriptive study of 20 patients who had a low load prolonged stretch (LLPS) orthotic device applied. The orthosis was worn briefly at the beginning of the treatment and then worn for progressively longer periods of time as the treatment progressed. The use of the LLPS orthosis significantly increased ROM for 17 of the initial 20 patients. The retrospective study supports the effective use of the LLPS orthosis to achieve ROM and functional gains in contracture management. Several recent studies provide support for the use of LLPS in the upper limb to improve ROM and to reduce contractures.11–14
FOREARM ROTATION ELBOW ORTHOSIS
The forearm rotation elbow orthosis (FREO) is formed through the connection of two separate components: a custom wrist-hand orthosis (WHO) and a custom elbow orthosis (EO). To begin fabrication of a FREO, a long-arm cast is taken of the patient in maximum elbow extension and as near to neutral as possible in the forearm. It is important that the patient’s wrist be placed in a neutral or slightly extended alignment to enhance the functional use of the patient’s digits. The base of the thumb is encapsulated by the orthosis to assist in suspension of the orthosis and in distribution of the applied forces. The distal trimlines of the WHO are placed proximal to the metacarpophalangeal joints of the hand to permit full ROM of the digits, which has been subjectively noted to improve patient compliance.
Once the long-arm cast is modified, another cast is taken of just the hand and forearm section, so that a separate positive mold can be formed. This second hand-forearm cast is lined with a compliant material, such as P-Cell™ (Acor Orthopaedic Inc., Cleveland, OH), and then the forearm section is turned into a cylinder by wrapping additional layers of Pelite™ (Southern Prosthetic Supply, Paso Robles, CA) material around its circumference and grinding it to shape. The WHO is then formed by drape molding (under vacuum) the hand-forearm cast with a thin, flexible thermoplastic material such as polyethylene. The last step is to add one or more (depending on the size of the patient) thin strips of 1-in wide Pelite around the circumference of the circular forearm section. This will create “step(s)” in the outer plastic, which not only helps to lock the WHO within the EO but also forms part of the patent pending (#12412358) rotational locking mechanism. The finished WHO is then trimmed, finished, and placed back on the original long-arm cast (Figure 1).
The custom EO is comprised of an upper humeral section, either a bivalved or a anterior shell, and a lower forearm trough that snaps over the aforementioned custom WHO. The humeral section is again lined with a compliant material to improve total contact and to minimize friction between the patient’s skin and the orthosis. The EO is drape molded (under vacuum) over the long-arm cast and custom WHO and then cut off and trimmed to size. The forearm trough needs to encapsulate more than half the circumference of the WHO, so that it holds it in place and permits it to rotate freely in either direction. Once the forearm trough is complete, the strip of Pelite is removed from the inner WHO and it is replaced with a 1-in wide piece of adhesive-backed Velcro™ (Velcro USA Inc., Manchester, NH) pile. This Velcro pile strip enables the WHO to be locked into the desired position by interacting with an overlying locking strap that is attached to the forearm trough whose underneath is lined with Velcro hook.
This patent pending mechanism (#12412358) is unique in that it does not limit the degree of rotational change that can be applied. This design affords clinicians the ability to introduce minute rotational changes in a patient’s forearm position and to maintain these improvements via the simple locking mechanism (Figures 2 and 3). This prolonged static-progressive application of force encourages contracted muscles to lengthen and thereby improves both the active and PROM of the forearm joints. Regular use of the orthosis is also expected to maintain these ROM gains over time for the same reasons as were mentioned previously.
Each subject in this case study was cast and fit with a custom FREO. Each FREO was to be worn during the night to promote growth and lengthening of the contracted muscles surrounding each patient’s elbow and forearm. It was anticipated that once the desired ROM gains were achieved, the orthosis would maintain these gains over time. Each patient was required to return every 3–4 weeks to follow-up with the orthotist. Orthotic follow-ups are extremely important to assess the fit and function of the FREO and to determine the degree of patient and parental compliance with the orthosis.
Both the following case studies incorporated an Ultraflex (Ultraflex Systems Inc., Pottstown, PA) dynamic extension elbow joint in each respective FREO to apply a continuous low-load, long duration application of force to the contracted elbow flexor muscles. It is important to note that with any dynamic application of force it is the time, not the amount of tension, that is key to the improving a joint’s ROM. The initial tension for each patient’s Ultraflex elbow joint was set to “2,” which is the starting level recommended by the manufacturer. Each parent was then instructed to gradually increase the wear time of the orthosis over the first week until 6–8 hours of monitored use was achieved. Regular nighttime use of the FREO was then recommended from that point forward alternating between the static-progressive stretching of the forearm one night (elbow locked at 90°) and dynamic stretching of the elbow muscles the following night (forearm is positioned in a neutral alignment). This alternating schedule reduces the risk of overstretching the patient’s muscles and ensures that the forearm is positioned correctly, so that either the pronator or the supinator muscles are better targeted by the FREO.
Although a dynamic elbow joint was used in these two cases, any design of elbow joint can be incorporated into a FREO (i.e., free motion, dynamic or static-progressive). The type of joint used will be determined by a certified orthotist and will depend largely on the size of the patient’s arm, the desired force application, and the current ROM of the patient’s elbow joint. Free-motion elbow joints are used in cases where elbow ROM is normal but forearm ROM is restricted.
CASE HISTORY: SUBJECT 1
Subject 1 is a 10-year-old boy diagnosed with CP. He presented with right spastic hemiplegia and was referred to occupational therapy to improve his movement and control of his right upper limb (Figure 4). Active, controlled movement of the patient’s right arm was limited because of his increased muscle tone and contractures at both the elbow and forearm joints. Although the subject could actively isolate flexion and extension of the elbow, he could not actively supinate his forearm. When the patient attempted to reach overhead or forward, the humerus internally rotated, the elbow flexed, and the forearm pronated. In addition, the patient lacked wrist stability causing his hand to be maintained in flexion and ulnar deviation. The patient also had difficulty grasping because his thumb was constantly maintained within the palm of his hand. These abnormal arm and hand movements combined with the limitation in elbow and forearm motion inhibited the patient from being able to reach and orient his hand to perform gross or fine motor tasks.
Subject 1 was seen for a series of three long-arm casts over a 1-month period. PROM measurements were taken of both the elbow and forearm joints initially to set a baseline for the subject. Subsequent measurements were taken of both joints after the final serial cast was removed and following FREO use. The FREO was cast and fabricated by the orthotist during the serial casting program, so that it would be finished and ready for delivery when the final long-arm cast was removed. Initially, the subject began wearing the FREO only during the day, so that his family could monitor the fit and function of the orthosis. Once the patient built up a tolerance to 4 hours of continuous use (Figure 5), he wore it only at night and omitted use during the day. The patient then continued with a nightly schedule of FREO use for a period of 10 months. It is important to note that the patient continued with active occupational therapy during this 10-month period to improve the active functional use of his right upper limb.
The results of all of the PROM measurements taken from subject 1 at the elbow and forearm are listed in Table 1. Baseline elbow extension PROM was noted to range from 45°–150° (45° flexion contracture. After the removal of the final serial long-arm cast this PROM increased to 30°–150° (30° flexion contracture). Use of the FREO over a 10-month period continued to improve the PROM of the elbow raising it to 20°–150° (20° flexion contracture). Similar improvements in forearm PROM were also noted after serial cast removal and FREO use. Baseline forearm PROM was noted to be 0°–15° (0° equals full pronation of the forearm). The PROM of the forearm rose an additional 30° (0°–45°) at the end of the serial casting protocol (the forearm could now achieve a neutral position). The FREO again continued to improve this PROM enabling the patient to achieve full forearm PROM (0°–90°) at the end of 10 months. The improvements in elbow and forearm PROM are seen easily when the baseline and 10-month standing photographs are compared (Figures 4 and 6).
CASE HISTORY: SUBJECT 2
Subject 2 is a 6-year-old girl diagnosed with right OBPP. The patient was referred to occupational therapy to treat the contracture of her elbow and forearm. Subject 2 was able to actively flex and abduct her shoulder to 90° but was unable to extend her elbow beyond +40° (40° flexion contracture). Unlike subject 1, subject 2 was unable to pronate her forearm to position her hand for functional grasping (Figure 7). In addition, her wrist was maintained in extension rather than flexion.
Subject 2 was placed in a series of long-arm casts for a period of 1 month to gradually increase the PROM of the elbow and forearm joints (Figure 8). Again, PROM measurements were taken of both the elbow and forearm joints initially to set a baseline for the subject. Subsequent measurements of both joints were taken after the final serial cast was removed and following FREO use. The FREO was cast and fabricated by the orthotist during the serial casting program, so that it would be finished and ready for delivery when the final long-arm cast was removed. Subject 2 also began wearing the FREO during the day until 4 hours of continuous use was tolerated. Once this wear-time was achieved, subject 2 then began wearing the FREO each night for a period of 7 months (Figure 9). Subject 2 also continued with ongoing occupational therapy during this time to improve her active forearm pronation ROM to facilitate the right hand function. She wore a McKie thumb splint with a pronator strap to pronate her hand, so that it could be better positioned for functional grasping (Figure 10).
The results of all of the PROM measurements taken from subject 2 at the elbow and forearm are listed in Table 2. Baseline elbow extension PROM was noted to range from 40° to 150° (40° flexion contracture). After the removal of the final serial long-arm cast, this PROM increased to 25°–150° (25° flexion contracture). Use of the FREO over a 7-month period eliminated the elbow contracture completely 0°–150°. Similar improvements in forearm PROM were also noted after serial cast removal. Baseline forearm PROM was noted to be 20°–90° (lacked the last 20 degrees of forearm pronation). Use of the serial cast eliminated the pronation contracture completely (forearm PROM 0°–90°) and use of the FREO maintained this full ROM over the ensuing 7 months.
For a child with hemiplegic CP or OBPP, weakness and muscle imbalance are often accompanied by tightness of the involved upper limb. This tightness leads to abnormal motion of the involved limb and to poor posturing, resulting in poor positioning of the hand for functional use. Serial casting has been used successfully to encourage tissue lengthening and improvements in joint ROM. Unfortunately, these gains often diminish with time because the underlying muscular imbalances promote the redevelopment of the original muscle contractures.
The two aforementioned case studies support the notion that a FREO could be an effective means not only to maintain but also to improve the PROM of the elbow and forearm joints after serial cast removal. As length is gained in the tight muscles of the forearm and elbow, the over-lengthened antagonist muscles begin to shorten, enabling them to contract more effectively. This improved muscle balance should enhance the outcome of strengthening programs.
These two case studies highlight how the use of a FREO saved both the patients’ and clinicians’ valuable time and monetary resources. Further studies are warranted to determine how successful the FREO is when used in the rehabilitation of numerous patients with central nervous system dysfunction and OPBB.
As with any orthotic intervention, parental and patient compliance is the key to success. It is imperative that the child’s parents understand the purpose and function of the orthosis as they often determine the degree of orthotic compliance. Regular orthotic follow-ups are recommended, typically every 3–4 weeks, throughout the treatment involving a FREO, so that PROM measurements can be taken and to ensure that the fit of the orthosis is maintained as the child grows.
The FREO was shown to improve and maintain the PROM of both the elbow and forearm joint in two test subjects after a 1-month serial long-arm casting protocol. The timeframe of FREO use for each subject was 10 and 7 months. The two subjects also actively participated in regular occupational therapy treatment throughout the testing period to work on strengthening and improving active range of motion. These gains in PROM indicate that additional research is warranted to investigate the potential benefits of the FREO with a large sample size, so that a statistical analysis can be performed and evaluated.
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KEY INDEXING TERMS: upper limb casting; upper limb casting; forearm-elbow orthosis; upper limb orthotics; upper limb orthotics; brachial plexus injury; elbow contracture; forearm contracture