Upper Limb Orthotics/Cerebral Palsy with flexion contracture

The patient is a nine year old female who presents with flexion contractures of the wrist. The contracture is only evident on her right side and is a secondary effect of the patients’ unilateral spastic hemiplegic cerebral palsy. At this stage the contracture appears to be limited to the wrist and has not noticeably affected the digits. Measurements taken during the initial appointment stated that the patients resting position of the wrist is thirty five degrees of flexion. This can be corrected to 0 degrees on manipulation. The patient was assessed based on The Manual Ability Classification System and rated as being at level III. Functional assessments of patient performing activities of daily living (i.e. getting dressed, tying shoes, eating etc. indicated that she required help or is very slow when performing these tasks. While current using botulinum toxin injections to manage the contractures, the patient would like to discuss orthotic options. The patients’ goals include independently completing activities of daily living and being able to write and draw more efficiently.

Written information

This piece will consider and explore various treatment options regarding the case study of a nine year old female with spastic cerebral palsy. The focus will be on her right wrist as it has presented with flexion contractures and must be managed. The contracture appears to be due to the prolonged spasticity in her right flexi carpi ulnaris and right flexor carpi radialis muscles. The patient has been assessed according to The Manual Ability Classification System and is considered a level III (Ohrvall, Krumlinde-Sundholm, and Eliasson, 2013). While currently requiring support and environmental adjustments to complete activities of daily living, the patient is highly motivated to become more independent. When deciding on what treatment to provide, it is important to consider the client goals, physiological state and support available.

Evidence

Cerebral Palsy, or ‘the cerebral palsies’, is a non-progressive neurological condition which arises as a result of a legion in an immature or developing brain of a child or foetus and affects both posture and movement (Kerr Graham, and Selber, 2003; Dekker, and Bouter, 2004; Hoare et al. 2010). Cerebral palsy is irreversible and while the legion which causes the condition does not change or progress over time, the symptoms generally do (Koman, Smith, and Shilt, 2004). The patient being studied in this case has spastic hemiplegic cerebral palsy and it has been noted that those in this group, while constituting one of the most common of the groups, are the ones most likely to function and assimilate with other ‘typically developing’ children (Kerr Graham, and Selber, 2003; Russo, Atkins, Haan, and Crotty, 2009). Despite this, spasticity is often present and, when in the upper limb, can lead to issues such as decreased force production, control and range of motion (Burtner et al., 2008). This loss of dexterity can in turn significantly affect an individual’s quality of life by resulting in a loss of independence and a decreased ability to continue with activities of daily living. The spasticity associated with this condition, if ongoing and not stretched, can lead to the formation of contractures (Gracies et al., 1997). Fattal-Valevski, Sagi, and Domenievitz, (2010) state that muscle groups such as the wrist flexors are likely to advance to a state of contracture from spasticity at a faster rate than other upper limb muscles, which is consistent with the patient having contractures in her right flexor carpi ulnaris and flexor carpi radialis. The flexor carpi ulnaris is a muscle of the forearm responsible for flexion of the wrist and, if working in conjunction with the extensor carpi ulnaris, it will also adduct the hand by functioning over the wrist joint. The origins of the flexor carpi ulnaris are the medial epicondyle of the humerus and the olecranon and the insertion sites are the pisiform bone, the hook of hamate and the fifth metacarpal bone. Likewise, the flexor carpi radialis is also responsible for flexion of the wrist and works with the flexor carpi ulnaris to produce this action. The attachment sites for the flexor carpi radialis are the medial epicondyle of the humerus and the base of the second metacarpal bone (Moore, Dalley, & Agur, 2010).

Orthotic treatment options

Upper limb orthoses may be used in this circumstance to aid in the stretching and lengthening of the patients’ wrist flexor tendons to endeavour to manage the flexion contracture. Katalinic, Harvey, and Herbert, (2011) suggest that this could possibly be done by keeping the muscles in a stretched position for a sustained amount of time. Types of non-functional orthoses, such as resting hand orthoses and other removable devices are commonly used to stretch muscles involved in contractures as well as to decrease muscle tone (Jackman, Novak, and Lannin, 2013). The devices used may be either static orthoses or dynamic orthoses. The appropriate materials as well as position of the wrist and digits must be carefully considered before a decision can be finalised. Varying preferences will be seen depending on the clinician or researcher inclination and depending on the different circumstances of the patients involved. In this specific case, the wrist would have to be in slight extension to allow for stretch throughout the wrist flexor muscles in the anterior compartment of the forearm. Louwers, Meester-Delver, Folmer, Nollet, and Beelen, (2011) conclude that if in the correct functional hand position, improvements can be seen through bracing. Gillen, Goldberg, Muller, and Straus, (2008) state that while wrists are conventionally immobilised in thirty degrees of extension when in an orthotic device, the most appropriate degree of extension is dependent on the individual patient and the activities they are most required to perform during the time of treatment. There is little conclusive evidence available indicating with confidence that orthoses are statistically beneficial for managing contractures in cerebral palsy (Exner, and Bonder, 1983; Jackman, Novak, and Lannin, 2013). Katalinic, Harvey, and Herbert, (2011) come to the conclusion that there is no clinically relevant or long term improvements in spasticity or joint mobility when treated with stretch, however it is still a common and widely used form of treatment Exner, and Bonder, (1983) suggests that, in children with spastic hemiplegic cerebral palsy, bilateral hand use and grasp are both improved with the use of a MacKinnon splint. This splint may be appropriate for contracture management as wrist flexion is decreased by placing pressure on the metacarpal heads, leading to a decrease in flexor tone. However Burtner et al. (2008) warn that while static splints may decrease the tone of the flexor muscles present in the forearm, shoulder muscles may be used in activities as compensation when requiring grip and dexterity. Fayer, and Sayed, (2013) recommend dynamic orthoses over static ones. The dynamic splints allow movement of the wrist while holding it in alignment and their study showed that this improved passive and active range of motion compared to the static splints which caused immobilization. An example of a possible dynamic orthoses is the Lycra arm splint which, in a study by Elliott, Raid, Hamer, Alderson, and Elliott, (2011) was shown to improve functional performance and efficiency of movement.

Comparison of orthotic treatment options

Alternative treatment modalities can be used to treat contractures. The use of botulinum toxin injections and surgery are both popular options and are widely used. These options can be used alone, or on conjunction with other methods such as orthotic treatment, occupation therapy or physiotherapy. Botulinum toxin injections are used to decrease the tone of the muscles injected, and lead to improvements in grasp, increased functional scores and increased range of motion. The improvements last an average of seven months before the effects diminish (Hoare et al., 2010; Fattal-Valevski, Sagi, and Domenievitz, 2010). Fattal-Valevski, Sagi, and Domenievitz, (2010) report that the use of these injections resulted in improvements in Manual Ability Classification scores for some of the participants in their study. Surgery is also used successfully to manage wrist contractures. Wenner, and Johnson, (1988) state that post-operative improvements can be seen in grasp and range of motion of the wrist joint. The surgery is performed by transferring the spastic, hypertonic flexor carpi ulnaris muscle from the anterior compartment of the forearm to the posterior compartments’ extensor carpi radialis longus or extensor carpi radialis brevis.

As discussed, multiple treatment options are available when dealing with contractures relating to spastic hemiplegic cerebral palsy. Orthotic interventions appear to be widely used clinically, despite the lack of data supporting its effectiveness. These orthoses range in type, material, and positioning of the wrist and decisions on which device to use are dependent on the clinician preference as well as the patient requirements. Aside from orthoses, surgery and botulinum toxin injections are also used in the management of contractures. While botulinum is a temporary treatment method, its effectiveness is documented. Surgery has the most permanent positive effect and can be used in conjuncture with orthotic treatment to provide maximum benefit to the patient.

Search Strategy

Databases such as Medline, Cinahl, and Cochrane Library were used when researching this case study. Terms such as cerebral, palsy, hemiplegic, contracture, spasticity, muscle tone, orthoses, splint, brace, botox, and wrist flexors were used to complete the search. Once articles were found, the reference lists of those articles were also used as a source of more information.

References

Burtner, P. a, Poole, J. L., Torres, T., Medora, A. M., Abeyta, R., Keene, J., & Qualls, C. (n.d.). Effect of wrist hand splints on grip, pinch, manual dexterity, and muscle activation in children with spastic hemiplegia: a preliminary study. Journal of Hand Therapy : Official Journal of the American Society of Hand Therapists, 21(1), 36–42; quiz 43. doi:10.1197/j.jht.2007.08.018

Exner, C. E., & Bonder, B. R., (1983). Comparative Effects Of Three Hand Splints On Bilateral Hand Use , ...

Dekker, J., & Bouter, L. M. (2004). Occupational therapy for children with cerebral palsy : a systematic review, 1–14.

Elliott, C., Reid, S., Hamer, P., Alderson, J., & Elliott, B. (2011). Lycra ® arm splints improve movement fluency in children with cerebral palsy. Gait and Posture, 33(2), 214–219. doi:10.1016/j.gaitpost.2010.11.008

Fattal-Valevski, a., Sagi, L., & Domenievitz, D. (2011). Botulinum Toxin A Injections to the Upper Limbs in Children With Cerebral Palsy: Duration of Effect. Journal of Child Neurology, 26(2), 166–170. doi:10.1177/0883073810376446

Fayez, E. S., & Sayed, H. M. (2013). Influence of different Types of Hand Splints on Flexor Spasticity in Stroke Patients, 7(1), 65–70.

Gillen, G., Goldberg, R., Muller, S., & Straus, J. (2008). The Effect of Wrist Position on Upper Extremity Function While Wearing a Wrist Immobilizing Splint. JPO Journal of Prosthetics and Orthotics, 20(1), 19–23.

Gracies, J. M., Fitzpatrick, R., Wilson, L., Burke, D., & Gandevia, S. C. (1997). Lycra garments designed for patients with upper limb spasticity: Mechanical effects in normal subjects. Archives of Physical Medicine and Rehabilitation, 78(10), 1066–1071.

Graham, H. K., & Selber, P. (2003). MUSCULOSKELETAL ASPECTS OF CEREBRAL PALSY, 85(2), 157–166.

Hoare, B. J., Wallen, M. a, Imms, C., Villanueva, E., Rawicki, H. B., & Carey, L. (2010). Botulinum toxin A as an adjunct to treatment in the management of the upper limb in children with spastic cerebral palsy (UPDATE). Cochrane Database Syst Rev, (1), CD003469. Retrieved from http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=20091546

Jackman, M., Novak, I., & Lannin, N. (2013). Effectiveness of hand splints in children with cerebral palsy: a systematic review with meta-analysis. Developmental Medicine and Child Neurology, 56(2), 1–10. doi:10.1111/dmcn.12205

Katalinic, O. M., Harvey, L. a, & Herbert, R. D. (2011). Effectiveness of stretch for the treatment and prevention of contractures in people with neurological conditions: a systematic review. Physical Therapy, 91(1), 11–24.

Louwers, A., Meester-Delver, A., Folmer, K., Nollet, F., & Beelen, A. (2011). Immediate effect of a wrist and thumb brace on bimanual activities in children with hemiplegic cerebral palsy. Developmental Medicine and Child Neurology, 53(4), 321–6. doi:10.1111/j.1469-8749.2010.03849.x

Moore, K., Dalley, A., & Agur, A. (2010). Clinically oriented anatomy. Lippincott Williams & Wilkins

Ohrvall, A.-M., Krumlinde-Sundholm, L., & Eliasson, A.-C. (2014). The stability of the Manual Ability Classification System over time. Developmental Medicine and Child Neurology, 56(2), 185–9. doi:10.1111/dmcn.12348

Russo, R. N., Atkins, R., Haan, E., & Crotty, M. (2009). Upper limb orthoses and assistive technology utilization in children with hemiplegic cerebral palsy recruited from a population register. Developmental Neurorehabilitation, 12(2), 92–99. doi:10.1080/17518420902783223

Wenner, S. M., & Johnson, K. a. (1988). Transfer of the flexor carpi ulnaris to the radial wrist extensors in cerebral palsy. The Journal of Hand Surgery, 13(2), 231–233. doi:10.1016/S0363-5023(88)80055-8

Functional Aims and Goals

The goal of the low temperature thermoplastic device is to immobilise the joint involved and to provide a more functional position once the cast has been removed. The patients’ wrist joint will be maintained in fifteen to twenty degrees of extension (depending on what the patient can handle), while preventing any radial deviation, ulnar deviation or flexion. The pollex will be immobilised in an opposed position while all other metacarpal phalangeal joints and inter phalangeal joints will be immobilised in flexion, with no adduction or abduction permitted. The proximal trim line will be 1/3 of the forearm length distal to the cubital crease. The distal trim lines will be just distal to the interphalangeal joints. This position will be used to inhibit any further contracture of the patients’ flexor carpi ulnaris or flexor carpi radialis. It is hoped that this will also work to facilitate the stretching of the flexor carpi ulnaris and flexor carpi radialis tendons to reduce the contracture present. Ultimately, the goal is to reduce contracture and increase the patients’ range of motion. The plaster of paris cast will be used to fabricate a high temperature orthosis. This device maintains the wrist joint in fifteen to twenty degrees of extension, the pollex opposed and all other phalanges flexed as in the low temperature thermoplastic device. The same trim lines will also be applicable. However, this device has the potential to be used as a dynamic splint to further stretch the patients’ flexor carpi ulnaris and flexor carpi radialis tendons. To do this, pullies will be secured to the posterior aspect of the device to further extend the phalanges and wrist. This will require the use of an articulated joint or malleable material at the mobilised joints.

Design

Outline the design of your orthosis, this should include but is not limited to: technical drawings, force system diagrams (3 planes), materials of choice, attachment methods, trimlines and manufacturing procedure. Within this section, if you would choose to make your device from something other than LTT explain why and how this may affect the function of the device you manufacture.

The patients’ wrist joint will be maintained in fifteen to twenty degrees of extension (depending on what the patient can handle), while preventing any radial deviation, ulnar deviation or flexion. The pollex will be immobilised in an opposed position while all other metacarpal phalangeal joints and inter phalangeal joints will be immobilised in flexion, with no adduction or abduction permitted. The proximal trim line will be 1/3 of the forearm length distal to the cubital crease. The distal trim lines will be just distal to the interphalangeal joints. The plaster of Paris and low temperature thermoplastic devices will use the same designs.

Manufacturing process

POP: Materials required • Stockinet • Plaster of Paris bandage (15cm) • Plaster scissors • Water • Indelible pencil

1. Place a well fitting stockinet on patient, covering the hand and forearm 2. With an indelible pencil, carefully mark the previously discussed trim lines 3. Mark all bony landmarks within the trim lines 4. With a fifteen centimetre plaster of Paris bandage, cut out a four layered back slab the length of your device 5. By placing on the patients hand and forearm, mark where the pollex is and cut out a small opening 6. Carefully place the patients upper limb in the desired position and instruct the patient to try and stay in the position 7. Apply the wet plater to the anterior surface of the hand (circumference of the pollex) and forearm and smooth down 8. Correct the position of the patients upper limb and carefully mould the plaster 9. Consistently check the position of the patient until the plaster is dry and ready to be removed 10. Use plaster scissors to cut the device according to the required trim lines 11. Attach straps is required

LTT: Materials required • Padding (if required) • Paper or chux cloth • Low temperature thermoplastic • Scissors • Water

1. Trace the patients hand and forearm onto a piece of paper or chux with the thumb in an extended position 2. Over the tracing, sketch the template shown in the figure 3. Cut out the template and check that is fits on the patients limb 4. Copy the template onto a sheet of low temperature thermoplastic and cut out 5. Carefully place the patients upper limb in the desired position and instruct the patient to try and stay in the position 6. Place the low temperature thermoplastic in hot water and allow to soften 7. Remove the thermoplastic and after checking that it is not too hot, drape over the patients hand and forearm 8. Make sure the patient is in the desired position and mould to his or her anatomy 9. Once cool, remove the device 10. Place the proximal edge into water and flare out slightly to avoid causing pressure points 11. Add straps as shown in figures

Critique of fit

The patient is a nine year old female presenting with a flexion contracture to the right wrist, secondary to her unilateral spastic hemiplegic cerebral palsy. On presentation, the patient reported that she has been undergoing botulinum toxin treatment as contracture management but would like to reduce this and hopefully reply on orthotic treatment options instead. Her goals include independently completing activities of daily living (i.e. getting dressed) and being able to write and draw more efficiently.

A physical assessment was completed and revealed that the resting position of the patients’ wrist is in thirty five degrees of flexion. On manipulation, this can be corrected to approximately fifteen degrees of extension. After further assessment, the patient has been rated as being at level III on the Manual Ability Classification System. Orthotic goals include increasing the patients’ wrist range of motion (specifically, extension) and preventing any further contracture in her flexor carpi ulnaris or flexor carpi radialis. This will aid in the client achieving her desired goals and decrease her need for botulinum toxin injections.

In order to achieve these goals, an orthotic prescription has been developed utilising a custom made wrist hand orthosis. The orthosis has been designs to prevent the patients’ wrist from flexing, as well as stretching her flexor carpi ulnaris and flexor carpi radialis tendons. This is done by immobilising the wrist joint in as much extension as possible. The pollex will be immobilised in an opposed position while all other metacarpal phalangeal joints and inter phalangeal joints will be immobilised in flexion. See functional aims and goals for more information.

The trim lines for the device appear to the appropriate. The position of the straps, final finish and contouring to the patients’ anatomy are the main areas of concern, prompting me to state that the device has not met a high technical standard.

The position of the straps on the proximal surface of the digits could cause difficulty when donning and doffing. While the patient shows no signs of reduced dexterity in her left limb, applying the straps may be cumbersome.

There are pen marks present on the surface of the device and while most are not noticeable, it would be preferable for them to not be present.

(INSERT PIC)

The contouring to the patients anatomy is mostly well done, however there are areas such as the medial-lateral surface of the hand where it could be better.

(INSERT PIC)

The force system acting on the device / patient appears to be appropriate. However, it appears that the low temperature thermoplastic may not be strong enough to resist contracture.

As the high temperature orthosis has not been fabricated, critique is not applicable.

Outcome measures

When treating patients, it is important to ensure that the management being provided is effectively leading to improvements in their condition. To do so, outcome measures are used at various stages of the treatment process, and compared to outline progress.

The outcome measures chosen should address the patients physical symptoms as well as their personal goals.

In this case, goals include increasing wrist range of motion and providing a more functional position with the hope of enabling the patient to complete activities of daily living more efficiently. The patient would also like to improve her writing skills.   By considering these goals, the following outcome measures were implemented.

QuickDASH: this questionnaire focus` on the patients ability to complete activities of daily living and the symptoms felt by the patient in the previous week. While valid, the self reporting nature of the test means that there is an element of bias involved.

Writing: considering writing has been noted as a specific patient goal, her writing skills were tested before and after orthotic use. The patient was asked to write down a sentence before and after orthotic intervention. Both time taken and visual data were recorded for comparison. It is important to note that since the device prescribed is a resting device which immobilises the fingers, this outcome measure was implemented without orthosis wear.

ADL: one of the main activities of daily living that the patient would like to improve on is getting dressed independently. Therefore, she was asked to put on a cardigan before and after orthotic treatment while being timed to record progress.

Outcome measure

Before treatment

After treatment

QuickDASH

59.09

29.55

Writing

74 seconds

45 seconds

ADL (timed)

55 seconds

36 seconds

All three outcome measures were found to be valid and were easily implemented. They were also all easily repeatable. The QuickDASH questionnaire was the least reliable as it relies on self reporting and can therefore introduce bias.

Referral Letter

Addressed to Mr Steve Cucumberbatch, Physiotherapist

Dear Steve Cucumberbatch, Thank you for agreeing to see my patient. I appreciate your contribution in this matter. The patient is a nine year old female with spastic hemiplegic cerebral palsy. She presented to me with a flexion contracture of her right wrist (due to the prolonged spasticity in her right flexor carpi ulnaris and right flexor carpi radialis muscles) and while previously receiving botulinum toxin injections, preferred to have her condition managed with the use of orthotics.

Regarding subjective information, I would say that she is highly motivated and generally compliant. She performs activities of daily living, such as dressing, slowly but is willing to perform exercises to increase her function and independence. The patients’ goals include independently completing activities of daily living and writing more efficiently. On initial presentation, the resting position of the clients’ wrist was in thirty five degrees of flexion, with zero degrees achievable on manipulation. She was assessed as being at level III on The Manual Ability Classification System.

The treatment I provided for the patient consisted of designing, manufacturing and fitting a custom made upper limb orthotic device. This device was made using low temperature thermoplastic and designed to immobilise the patients’ wrist in fifteen degrees of extension. Her pollex was also immobilised in an opposed position while all other metacarpal phalangeal joints and inter phalangeal joints were immobilised in flexion. The device covers the volar surface, with the proximal trim line being 1/3 of the forearm length distal to the patients’ cubital crease and the distal trim line sitting distal to the inter phalangeal joints. Straps were used on the dorsal surface to apply a force on her wrist joint and to hold the device in place.

While this treatment method has been producing improvements (as seen in the functional outcome measures) a more multidisciplinary approach would be beneficial. I believe she would benefit from any manual therapy you think would be appropriate and an exercise program to further increase her range of motion and possibly strengthen her extensors.

Again, I appreciate your contribution in this matter and look forward to us working together for the benefit of this patient.

Kind Regards, Ms. I Peiris Orthotist

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