Orthopedic Care of Patients with Cerebral Palsy: A Clinical Guide to Evaluation and Management across the Lifespan

Many of the existing books focusing on the orthopedic management of patients with cerebral palsy encompass only care for the young patient, but this practical text reviews and delineates orthopedic care for patients with cerebral palsy throughout the lifespan. Readers will find a discussion of both non-operative and operative orthopedic management across all ages and functional levels. 
The text presents a general overview of cerebral palsy, evaluation of patients with cerebral palsy, and procedures commonly used to treat various orthopedic conditions in patients with cerebral palsy. Spasticity management and gait evaluation are likewise highlighted, and surgical chapters cover techniques for the hip, knee, foot and ankle, and spine. It also incorporates chapters focused on issues related to the rehabilitation of patients with cerebral palsy, including bracing, orthotics and other durable medical equipment, physical and occupational therapy, pain management, and adaptive activities and sports, which aim to improve the overall quality of life for patients through the lifespan. Finally, there is a chapter focused on the care transition from childhood to adulthood, an area of importance often neglected in current texts covering patients with cerebral palsy.
Whether in the operating room, multi-specialty clinic or private office, Orthopedic Care of Patients with Cerebral Palsy will be a go-to resource for orthopedists, pediatricians and all medical professionals caring for this population. 

• Language: English
• Publisher: Springer
• Release date: Jun 22, 2020
• ISBN: 9783030465742

Book preview

© Springer Nature Switzerland AG 2020

P. D. Nowicki (ed.)Orthopedic Care of Patients with Cerebral Palsyhttps://doi.org/10.1007/978-3-030-46574-2_1

1. Introduction to the Cerebral Palsies

Henry G. Chambers¹, ²   and Reid C. Chambers³

(1)

University of California San Diego, San Diego, CA, USA

(2)

Southern Family Cerebral Palsy Center, Rady Children’s Hospital San Diego, San Diego, CA, USA

(3)

Nationwide Children’s Hospital, Ohio State University, Columbus, OH, USA

Henry G. Chambers

Keywords

Cerebral palsyGMFCSFMSMACS

What Is Cerebral Palsy?

Cerebral Palsy (CP) is not a disease, but rather a collection of disorders that have a brain malformation or injury as the final common pathway. There are many, including Sir William Osler, who describe this umbrella term cerebral palsy as the cerebral palsies to demonstrate the spectrum of this disorder [1]. Therefore, a strict definition of cerebral palsy is difficult at best. Anyone caring for a patient with CP will appreciate that a child who has mild toe walking is a completely different patient from one who is in a wheelchair, on a respirator and requires total care, yet these children both have cerebral palsy.

In 2005, Bax and the Executive Committee for the Definition of Cerebral Palsy attempted to provide a comprehensive new definition of cerebral palsy [2].

Cerebral palsy (CP) describes a group of permanent disorders of the development of movement and posture, causing activity limitation, that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain. The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, perception, cognition, communication, behavior, by epilepsy and by secondary musculoskeletal problems

Richards and Malouin [3] added some more to the definition with some more clinically relevant considerations in their paper on cerebral palsy:

CP is a disorder of the development of movement and posture, causing activity limitations attributed to nonprogressive disturbances of the fetal or infant brain that may also affect sensation, perception, cognition, communication, and behavior. Motor control during reaching, grasping, and walking are disturbed by spasticity, dyskinesia, hyperreflexia, excessive coactivation of antagonist muscles, retained developmental reactions, and secondary musculoskeletal, malformations together with pareses and defective programing. Weakness and hypoextensibility of the muscles are due not only to inadequate recruitment of motor units, but also to changes in mechanical stresses and hormonal factors.

In none of the recent definitions is there an age requirement, although it is generally accepted that any injury to the developing brain occurring prior to age 3 should be classified as cerebral palsy.

History of Cerebral Palsy

I that am curtailed of this fair proportion,

Cheated of feature by dissembling Nature,

Deform’d, unfinish’d, sent before my time

Into this breathing world, scarce half made up,

And that so lamely and unfashionable,

That dogs bark at me as I halt by them

(Richard III, Act 1, Scene 1 Shakespeare)

William John Little (1810–1984) was one of the first to describe cerebral palsy and ascribed it to a brain injury caused by lack of oxygen at birth. Mr. Little was a sickly child and had many diseases including polio which left him with a clubfoot. He sought out Dr. Georg Friedrich Stromeyer who was a German surgeon. Dr. Strohmeyer performed a tendoachilles lengthening which helped Little immensely. He brought this procedure back to England where he helped establish the field of orthopedic surgery there. He then began his study of children and their deformities. In 1861, he presented his findings to the Obstetrical Society of London where he defined cerebral palsy as an obstetric injury in which abnormal forms of labor in which the child has been partial suffocated injures the nervous system [4]. He essentially defined Spastic Cerebral Palsy (but did not call it by that name) and it was called Little’s Disease for a very long time. There were dozens of other researchers throughout Europe who were describing this disorder with series of case reviews at the same time (Fig. 1.1).

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Fig. 1.1

William John Little (1810–1894)

Sir William Osler (1849–1928) graduated from McGill University in 1872 and trained later in London and in Berlin with Rudolph Ludwig Karl Virchow. He was one of the first to use the term cerebral palsy, in his treatise: The Cerebral Palsies of Children in 1889. He had over 150 patients in this paper and was one of the first to use spastic hemiplegia and diplegia as subgroups. As one of the founders of Johns Hopkins University School of Medicine as well as one the founders of modern medical education, Professor Osler contributed much to the field of cerebral palsy and dozens of other fields. He published over 1200 manuscripts and books in his storied career [5] (Fig. 1.2).

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Fig. 1.2

Sir William Osler (1849–1919)

Sigmund Freud (1856–1939) is, of course, known for his contributions to the field of psychiatry. However, many are unaware of his contributions to the field of cerebral palsy. After his graduation from the University of Vienna in 1881, he spent an additional 3 years working in the field of neuroanatomy. He made several important discoveries which lead to the theory of the neuron. From 1885–1886, he worked with Jean-Marie Charcot and began his interest in clinical neurology. He returned to Berlin where he was appointed to care for children and adults with neurological deficits. Between 1891 and 1897, he published three monographs and several papers about children with cerebral palsy. The first manuscript was over 220 pages and had 180 references and exhaustively studied 35 patients. In 1893, he published a paper that was 168 pages long and had 53 cases. It was in this paper that he described generalized cerebral spasticity: Littles Disease (spastic diplegia), paraplegic spasticity (probably spastic quadriplegia), bilateral spastic hemiplegia, and, for the first time, choreoathetosis. He described ataxic cerebral palsy in a later paper. In 1897, he published a book entitled "Die infantile Cerebrallahmung" (Infantile Cerebral Paralysis) with over 327 pages. This was one of the first books to completely review what was known about cerebral palsy. He was one of the first to describe that, unlike Little who felt that cerebral palsy was secondary to birth trauma, it was some sort of insult to the developing brain and that the cause was unknown in most cases (Fig. 1.3).

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Fig. 1.3

Sigmund Freud (1856–1939) Halberstadt, Max (c. 1921). Sigmund Freud, half-length portrait, facing left, holding cigar in right hand. Library of Congress. Archive

He lost interest in the field of cerebral palsy and began to develop the field of psychoanalysis. In a letter to Wilhelm Fliess, he complained I am fully occupied with the children’s paralysis, in which I am not the least interested. The completely uninteresting work on children’s paralysis has taken all my time. In a subsequent letter he recorded that he felt like Pegasus yoked to the plough! [5].

There was little interest in care of children with cerebral palsy throughout the world in the early twentieth Century. There was a multi-disciplinary clinic at Children’s Hospital in Boston under the neurologist, Bronson Crothers. Winthrop Phelps, an orthopedic surgeon, organized a rehabilitation center in Maryland. The rehabilitation movement that emerged from casualty management after World War II as well as the polio epidemic spurred the National Society for Crippled Children and Adults (Easter Seal Society) to set up a CP Advisory Medical Council which included Earl Carlson (internal medicine), Bronson Crothers (neurology), George Deaver (rehabilitation medicine), Temple Fay (neurosurgery), Meyer Perlstein (pediatrics), and Winthrop Phelps (orthopedic surgery). In 1947, they formed the American Academy for Cerebral Palsy, which was an interdisciplinary group dedicated to the study of children with cerebral palsy. Now the American Academy for Cerebral Palsy and Developmental Medicine is one of the leading voices in the care of children and adults with cerebral palsy and other neurologic disorders (Fig. 1.4).

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Fig. 1.4

American Academy for Cerebral Palsy Founders. Seated: Temple Fay, Winthrop Phelps, Bronson Crothers. Standing: Meyer Perlstein, Earl Carlson, George Dever

Outstanding research is being performed throughout the world in an attempt to discover the etiology of the cerebral palsies as well as the best treatments for those who have this disorder. Patient registries in Scandinavia and Australia will provide more insight into the longitudinal aspects of this disorder. In the past 30 years, management of spasticity and dystonia with oral medications, botulinum toxins, and intrathecal baclofen have greatly altered the treatment and outcomes of patients with cerebral palsy. Motion analysis has helped spur further biomechanical research and new orthopedic surgery techniques have been validated using this technology.

Epidemiology of the Cerebral Palsies

The incidence and prevalence of cerebral palsy has not changed much in the past 50 years. However, the types of cerebral palsy and etiologies have changed over the years. Several long-term registry-based studies have determined that the prevalence ranges from 1.6 per 1000 live births to 3.6 per 1000 live births [6–11]. In other terms, this is 1 in 277 to 1 in 625 live births.

Males are at a higher risk for cerebral palsy, possibly secondary to gender-specific neuronal vulnerabilities [12]. There is a higher incidence of cerebral palsy in very low birthweight and very premature babies (Table 1.1). This has changed over the past 30 years as we have developed neonatal intensive care centers which are very proficient in keeping these children alive. In fact, mechanical ventilation in these premature children may contribute to their cerebral palsy [13]. The risk of cerebral palsy increases four times in twins and 18 times in triplets [14, 15]. There are now more adults with cerebral palsy than there are children.

Table 1.1

Etiologic Risk Factors in Cerebral Palsy, as found in Blair [1]

In most cases the cause of cerebral palsy is not known.

Pathophysiology

The unifying pathology of cerebral palsy is injury to the developing brain. There are several different insults that can lead to cerebral palsy. Most of the injuries occur near the periventricular region (Fig. 1.5). It is in this area where the pyramidal tracts traverse the brain. It is also the area where the basal ganglia are located. Injury to the cortical spinal tracts affects motor signals to the body, and the basal ganglia are responsible for encoding motor patterns. Their main function is to initiate and permit smooth performance of motor movements. Injury to the basal ganglia can lead to dystonia and choreoathetosis.

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Fig. 1.5

Injury to the brain at any level can lead to cerebral palsy (after Gage, Schwartz, Koop, and Novacheck. The Identification and Treatment of Gait Problems in Cerebral Palsy. Clinics in Developmental Medicine 180–181, 2009)

Balance and the integration of motion can be affected by injury to the cerebellum or the tracts which lead to and from the cerebellum. Damage to the cerebellum can lead to the loss of coordination between muscle groups on both the contralateral and ipsilateral sides of the body. The brainstem nuclei can also be involved in cerebral palsy. In patients with more severe cerebral palsy (particularly GMFCS V patients), there can be pseudobulbar palsy. The cardinal features of this disorder are loss of emotional control (with forced crying or laughing), increased drooling, slow eating, choking, and speech abnormalities [16].

As mentioned in the definition of cerebral palsy above, visual disturbances are present in over half of the patients with cerebral palsy. This is secondary to the brain injury itself, involvement of the peripheral visual structures or a combination of both [17]. This can have a huge impact on the treatment of children and adults with cerebral palsy. For example, if someone is unable to see the ground ahead of them, any orthopedic surgery will not improve the patient’s gait. A change in pattern of the floor or of the walls may affect their ability to perceive their position in space. If, for example, they are unable to see their left side secondary to a hemiplegic stroke to the visual cortex, then any upper extremity intervention may not succeed as the patient cannot see their left upper extremity.

This brain insult leads to an upper motor neuron syndrome. In simplistic terms, the main neurotransmitter from the brain (gamma-aminobutyric acid (GABA)) acts as an inhibitor and modulator of the reflex arc whose primary neurotransmitter is acetylcholine (Fig. 1.6). When there is an interruption of the brain’s main neurotransmitter, there are several Positive Signs. These include spasticity, hyperreflexia, dyskinesia, persistent primitive reflexes, and secondary musculoskeletal malformations. Negative Signs reflect the loss of proper sensorimotor control mechanisms leading to poor balance, incoordination, weakness, and impaired walking ability. Other problems that are present in children with cerebral palsy are loss of selective motor control, sensory deficits, delayed growth and development and seizure disorders.

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Fig. 1.6

The reflex arc. The reflex arc is not damaged in cerebral palsy, but the inhibitory and modulating influences of the brain are. This leads to the development of spasticity

Classification Systems

Classification systems can be geographic (part of the body involved) and the listing of the primary motor deficit. For example, Spastic Quadriplegia. As mentioned in the history section above, the classifications of diplegia, hemiplegia, double hemiplegia, and quadriplegia (tetraplegia) were made over a hundred years ago [18]. The intraobserver and interobserver error in the use of this classification scheme is very high.

In 1997, Palisano et al. [19] published one of the key articles in the understanding of cerebral palsy. In this article they described the use of the Gross Motor Function test to delineate the severity of cerebral palsy. When the data of 275 children who were followed longitudinally were plotted, it was noted that the graphs showed five fairly discrete levels of motor function. Wood and Rosenbaum [20] further studied these groups and developed the Gross Motor Functional Classification System (GMFCS). This system has been expanded and revised to include children and adults to age 23.

The GMFCS has become the standard in classifying children and adults with cerebral palsy. It is being validated in other conditions such as spina bifida and muscle disease as well. There are two main points to be made about the GMFCS based on the GMFM. One, is that all children from GMFCS I to V all improve until they are about 6 years old, so any intervention that is done during this time will appear to improve the function of that child. It is really just mimicking natural history. The other aspect is that the GMFCS is not an outcome measure. One is on their level based on the involvement of their brain injury. The goal of surgery is to maintain the child at the level that they were at age 7,8, or 9. So when a teenager loses function and surgery makes them appear to improve a level, it is most likely just returning them to their highest function.

A Practical Guide to the GMFCS Levels

Level I: These are the children with the least amount of involvement. They may have some problems with uneven ground and balance issues but otherwise can walk and run with minimal problems.

Level II: Children who can walk and run but have more problems especially when ascending and descending stairs. In the First World, children who are using AFOs are usually at this level. In the Third World, children who need AFOs are in this group.

Level III: Children can walk on a level surface, they usually are using AFOs as well, but this group does not have the strength or balance to walk without support. These children are using crutches or walkers to walk but for long distances they often use manually powered wheelchairs.

Level IV: These children cannot walk except with maximum support using a walker. They have the upper extremity control to use a powered wheelchair.

Level V: These children have severe physical impairment. They require total care and are dependent on others for transportation (Fig. 1.7).

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Fig. 1.7

The Gross Motor Classification Scale. (Used with the permission of H. Kerr Graham, MD)

Functional Mobility Scale

Graham et al. [21, 22] developed the Functional Mobility Scale as an outcome measure for intervention such as surgery. It is very similar to the GMFCS although Level 6 is the highest functioning level. It assesses the patient to determine what function they have at 5, 50, and 500 meters. We all know of patients who can walk in the clinic without crutches, need crutches to walk 50 meters, but use a wheelchair for long distances (Fig. 1.8):

1.

Uses wheelchair, stroller, or buggy: May stand for transfers and may do some stepping supported by another person or using a walker/frame

2.

Uses Walker: without help from another person

3.

Uses two crutches: without help from another person

4.

Uses one crutch or two canes: without help from another person

5.

Independent on level surfaces: does not use walking aids or need help from another person. If uses furniture, walls, fences, shop fronts for support use 4 above

6.

Independent on all surfaces: does not use any walking aids or need any help from another person when walking, running, climbing slopes and stairs.

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Fig. 1.8

The Functional Mobility Scale. (Used with the permission of H. Kerr Graham, MD)

The Surveillance of Cerebral Palsy in Europe (SCPE) is a group of medical professionals who have used registry-based data to ascertain their population. Through various consensus groups they have determined that they have classified children with cerebral palsy based on the following criteria:

Spastic CP is characterized by at least two of the following:

Abnormal pattern of posture and/or movement

Increased tone (not necessarily constant)

Pathological reflexes (increased reflexes: hyperreflexia and/or pyramidal signs, e.g., Babinski sign

Spastic CP may be either bilateral or unilateral

Spastic bilateral CP is diagnosed if:

Limbs on both sides of the body are involved

Spastic unilateral CP is diagnosed if:

Limbs on one side of the body are involved

Ataxic CP is characterized by both:

Abnormal pattern of posture and/or movement

Loss of orderly muscular control so that movements are performed with abnormal force, rhythm, and accuracy

Dyskinetic CP is dominated by both:

Abnormal pattern of posture and/or movement

Involuntary uncontrolled, recurring, occasionally stereotyped movements

Dyskinetic CP may be either dystonic or choreoathetotic

Dystonic CP is dominated by both

Hypokinesia (reduced activity, i.e., stiff movement)

Hypertonia

Choreoatheototic CP is dominated by both:

Hyperkinesia (increased activity, i.e., stormy movement)

Hypotonia

This is a consensus statement from a group of researchers who have carefully reviewed their registry data [23]. Although this system has been widely adopted, it is clear to many clinicians that the unilateral vs. bilateral classification is too simple. Many children with Unilateral CP have involvement of their opposite sides. There are fewer but possibly more patients with triplegia than is commonly appreciated.

Manual Ability Classification System

While the GMFCS is used almost universally, it is only part of the story as it explicitly evaluates walking ability. The Manual Ability Classification System (MACS) was developed to determine upper extremity function [24].

Level 1: Handles objects easily and successfully

Level 2: Handles objects, but with somewhat reduced quality and/or speed of achievement

Level 3: Handles objects with difficulty; needs help to prepare and/or modify activities

Level 4: Handles a limited selection of easily managed objects in adapted situations

Level 5: Does not handle objects and has severely limited ability to perform even simple tasks

There are other classification systems of which orthopedic surgeons should be aware: The Communication Function Classification System [25] and the Visual Function Classification System [17].

Conclusion

This brief introduction to cerebral palsy will, I hope, set the stage for the remainder of this book. As you read of the different interventions, treatments, and rehabilitation, recall the different classification systems and try to relate that to your patient. Every brain lesion is subtly different, every movement disorder is subtly different, and every deformity is subtly different. Each patient needs to be treated as an individual and cannot be lumped into a single broad category.

References

1.

Blair E. Epidemiology of the cerebral palsies. Orthop Clin North Am. 2010;41(4):441–55. https://​doi.​org/​10.​1016/​j.​ocl.​2010.​06.​004.CrossrefPubMed

2.

Bax M, Goldstein M, Rosenbaum P, Leviton A, Paneth N, Dan B, et al. Proposed definition and classification of cerebral palsy, April 2005. Dev Med Child Neurol. 2005;47(8):571–6. Retrieved from https://​www.​ncbi.​nlm.​nih.​gov/​pubmed/​16108461.Crossref

3.

Richards CL, Malouin F. Cerebral palsy: definition, assessment and rehabilitation. Handb Clin Neurol. 2013;111:183–95. https://​doi.​org/​10.​1016/​B978-0-444-52891-9.​00018-X.CrossrefPubMed

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Schleichkorn J. The sometime physician: William John Little pioneer in treatment of cerebral palsy and orthopedic surgery 1810–1894. Tunbridge Wells: Aquarian Systems; 1999.

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Longo LD, Ashwal S. William Osler, Sigmund Freud and the evolution of ideas concerning cerebral palsy. J Hist Neurosci. 1993;2(4):255–82. https://​doi.​org/​10.​1080/​0964704930952557​6.CrossrefPubMed

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Andersen GL, Irgens LM, Haagaas I, Skranes JS, Meberg AE, Vik T. Cerebral palsy in Norway: prevalence, subtypes and severity. Eur J Paediatr Neurol. 2008;12(1):4–13. https://​doi.​org/​10.​1016/​j.​ejpn.​2007.​05.​001.CrossrefPubMed

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Himmelmann K, Hagberg G, Beckung E, Hagberg B, Uvebrant P. The changing panorama of cerebral palsy in Sweden. IX. Prevalence and origin in the birth-year period 1995–1998. Acta Paediatr. 2005;94(3):287–94. https://​doi.​org/​10.​1111/​j.​1651-2227.​2005.​tb03071.​x.CrossrefPubMed

8.

Smith L, Kelly KD, Prkachin G, Voaklander DC. The prevalence of cerebral palsy in British Columbia, 1991–1995. Can J Neurol Sci. 2008;35(3):342–7. Retrieved from https://​www.​ncbi.​nlm.​nih.​gov/​pubmed/​18714803.Crossref

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Gainsborough M, Surman G, Maestri G, Colver A, Cans C. Validity and reliability of the guidelines of the surveillance of cerebral palsy in Europe for the classification of cerebral palsy. Dev Med Child Neurol. 2008;50(11):828–31. Retrieved from https://​www.​ncbi.​nlm.​nih.​gov/​pubmed/​19058397.Crossref

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Howard J, Soo B, Graham HK, Boyd RN, Reid S, Lanigan A, et al. Cerebral palsy in Victoria: motor types, topography and gross motor function. J Paediatr Child Health. 2005;41(9–10):479–83. https://​doi.​org/​10.​1111/​j.​1440-1754.​2005.​00687.​x.CrossrefPubMed

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Yeargin-Allsopp M, Van Naarden Braun K, Doernberg NS, Benedict RE, Kirby RS, Durkin MS. Prevalence of cerebral palsy in 8-year-old children in three areas of the United States in 2002: a multisite collaboration. Pediatrics. 2008;121(3):547–54. https://​doi.​org/​10.​1542/​peds.​2007-1270.CrossrefPubMed

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Johnston MV, Hagberg H. Sex and the pathogenesis of cerebral palsy. Dev Med Child Neurol. 2007;49(1):74–8. https://​doi.​org/​10.​1111/​j.​1469-8749.​2007.​0199a.​x.CrossrefPubMed

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Aly H. Mechanical ventilation and cerebral palsy. Pediatrics. 2005;115(6):1765–7. https://​doi.​org/​10.​1542/​peds.​2005-0665.CrossrefPubMed

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Scher AI, Petterson B, Blair E, Ellenberg JH, Grether JK, Haan E, et al. The risk of mortality or cerebral palsy in twins: a collaborative population-based study. Pediatr Res. 2002;52(5):671–81. https://​doi.​org/​10.​1203/​00006450-200211000-00011.CrossrefPubMed

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Topp M, Huusom LD, Langhoff-Roos J, Delhumeau C, Hutton JL, Dolk H, SCPE Collaborative Group. Multiple birth and cerebral palsy in Europe: a multicenter study. Acta Obstet Gynecol Scand. 2004;83(6):548–53. https://​doi.​org/​10.​1111/​j.​0001-6349.​2004.​00545.​x.CrossrefPubMed

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Langworthy OR, a. H. F. Syndrome of pseudobulbar palsy. Arch Intern Med (Chic). 1940;65:106–21.Crossref

17.

Baranello G, Signorini S, Tinelli F, Guzzetta A, Pagliano E, Rossi A, et al.. Visual function classification system for children with cerebral palsy: development and validation. Dev Med Child Neurol. 2019. https://​doi.​org/​10.​1111/​dmcn.​14270.

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Minear WL. A classification of cerebral palsy. Pediatrics. 1956;18(5):841–52. Retrieved from https://​www.​ncbi.​nlm.​nih.​gov/​pubmed/​13370256.PubMed

19.

Palisano R, Rosenbaum P, Walter S, Russell D, Wood E, Galuppi B. Development and reliability of a system to classify gross motor function in children with cerebral palsy. Dev Med Child Neurol. 1997;39(4):214–23. Retrieved from https://​www.​ncbi.​nlm.​nih.​gov/​pubmed/​9183258.Crossref

20.

Wood E, Rosenbaum P. The gross motor function classification system for cerebral palsy: a study of reliability and stability over time. Dev Med Child Neurol. 2000;42(5):292–6. Retrieved from https://​www.​ncbi.​nlm.​nih.​gov/​pubmed/​10855648.Crossref

21.

Graham HK, Harvey A, Rodda J, Nattrass GR, Pirpiris M. The functional mobility scale (FMS). J Pediatr Orthop. 2004;24(5):514–20. Retrieved from https://​www.​ncbi.​nlm.​nih.​gov/​pubmed/​15308901.Crossref

22.

Surveillance of Cerebral Palsy in Europe. Surveillance of cerebral palsy in Europe: a collaboration of cerebral palsy surveys and registers. Dev Med Child Neurol. 2000;42:816–24.Crossref

23.

Eliasson AC, Krumlinde-Sundholm L, Rosblad B, Beckung E, Arner M, Ohrvall AM, Rosenbaum P. The Manual Ability Classification System (MACS) for children with cerebral palsy: scale development and evidence of validity and reliability. Dev Med Child Neurol. 2006;48(7):549–54. https://​doi.​org/​10.​1017/​S001216220600116​2.CrossrefPubMed

24.

Barty E, Caynes K, Johnston LM. Development and reliability of the functional communication classification system for children with cerebral palsy. Dev Med Child Neurol. 2016;58(10):1036–41. https://​doi.​org/​10.​1111/​dmcn.​13124.CrossrefPubMed

25.

Harvey A, Graham HK, Morris ME, Baker R, Wolfe R. The functional mobility scale: ability to detect change following single event multilevel surgery. Dev Med Child Neurol. 2007;49(8):603–7. https://​doi.​org/​10.​1111/​j.​1469-8749.​2007.​00603.​x.CrossrefPubMed

© Springer Nature Switzerland AG 2020

P. D. Nowicki (ed.)Orthopedic Care of Patients with Cerebral Palsyhttps://doi.org/10.1007/978-3-030-46574-2_2

2. Principles of Orthotics and Other Durable Medical Equipment

Lisa M. Voss¹  

(1)

Pediatric Physical Medicine and Rehabilitation, Mary Free Bed Hospital, Grand Rapids, MI, USA

Lisa M. Voss

Email: [e-mail]

Keywords

BracingOrthoticsAFOSMOWalkerWheelchairGait trainer

Orthotic Principles

Writing an Orthotic Prescription

Several different things must be taken into account when writing an orthotic prescription. For example, we must first have a goal. Examples include a goal of improved range of motion, minimizing contractures, proper positioning while seated, or improving active ambulation. Goals may change depending on the age of the patient and his/her individual needs at the time, and it is critical to include the patient and their family in the identification of the goal or goals. Time, age, and function will change patient’s goals. For the youngest patient, an improved range of motion with contracture prevention is in the forefront. As the patient ages, their goals often change to become more peer focused as they want to engage in the same activities and sports as their peers, and having adaptations to enable this becomes important. The physician must take into account the patient’s goals and educate the patient and family in regard to appropriate medical goals for their overall health and find the appropriate balance. Once a goal is identified, the next step is deciding how to best achieve that goal. Different equipment and orthoses may be required to help achieve a goal, and for that, different shapes, sizes, styles, material, and even colors/decorations should be considered. Above all else, it should improve their quality of life.

Orthotic Design

The goal of the proper orthotic design is one that will enhance proper movement while minimizing abnormal postures, movements, and tone. This is particularly important in cerebral palsy as patients are at high risk for joint contractures and deformities from spasticity, abnormal posturing/dystonia, and skin breakdown. It is important to have this be our focus while also considering options to make the orthosis lightweight, easily manageable, durable, and personally stylized. The orthotic itself will not be beneficial if the patient is not willing to wear it, and therefore, taking style into account is also an important feature, especially in children and adolescents.

When writing the prescription, it is important to include details such as the general description of the orthosis, the joints involved, and whether there should be motion in the joints. If an articulated joint is requested, the prescriber should note the range of motion desired and if any restrictions or stops should be placed. It is also important to include the amount of stability required for each individual patient. For example, some patients have significantly high tone or strength and may break a brace that is too flimsy. Conversely, a patient who is very weak would not be able to move in a brace that is too heavy and would not require the added force.

A prescription for the orthotic itself should include the design type (custom vs off the shelf), laterality (left, right, bilateral), material, joints involved, and any specifications to tailor to the patient’s needs. Prescription example:

Sig: Custom bilateral articulated thermoplastic AFOs with plantar flexion stop at neutral and free dorsiflexion and flexible forefoot with extra wide and extra depth shoes to accommodate bracing.

Material

The material used should be chosen carefully allowing the brace to be both stable and provide appropriate support but remain lightweight enough to allow the patient to function easily. Currently, braces are made of thermoplastic molding which may be off the shelf or custom. Metal may be used as additional support and for the joints of a brace if articulated or if a patient is >250 lbs. Occasionally a brace may be made of carbon fiber. The goal of the brace must also be considered when determining the material. For example, if the brace is to be worn at night, it will require different material than a brace that must fit into shoes for day use. Similar adjustments will be made for different locations on the body. For example, a patient is less likely to tolerate an exclusively plastic brace on their hand or arm and will require some additional padding or have it made out of neoprene.

Upper Extremity Orthotics

Goal

It is imperative to note the goal of the orthotic when prescribing. For hands and arms, orthotics may be used to increase functionality or may be used for stretching and prevention of contracture. Sometimes, the same brace may provide both functions, but other times they are mutually exclusive. For this reason, it is important to have the discussion on timing and frequency of brace use and specific instruction on use to both the patient and the orthotist.

Brace Types

Thumb Abduction Splint (Fig. 2.1)

Thumb abduction splints are typically used to abduct the thumb for stretching, maintenance of proper posture, and placing the thumb in a functional position. It is typically neoprene and will not overcome a severe cortical thumb. For infants, it may just be a thumb sleeve portion with a wrap-around strap, whereas for older children, the wrist may also be included.

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Fig. 2.1

Standard WHO with thumb abduction (palmar view)

Wrist Hand Orthosis (WHO) (Fig. 2.2a, b)

A wrist hand orthosis crosses over the wrist joint and includes the hand. This may or may not have ability to bend at various angles for wrist and fingers. Some WHOs may be custom, while others are off the shelf. Fingers may be included if the prescriber desires.

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Fig. 2.2

(a, b) Custom wrist hand orthosis

Resting Hand Splint

A resting hand splint is a balance between intrinsic and extrinsic musculature to prevent deformity. These are often used when patients are sleeping or resting as they are not typically functional.

Elbow Extension Splints/Bamboo Braces

These elbow braces are used to increase the extension on the elbow, predominantly used for stretching, and occasionally used in babies to assist with crawling and weight-bearing through arms. Larger extension splints may come with a dynamic hinge which allows for a graduated stretch at specific settings.

Lower Extremity Orthotics

Goal

The identification of the goals of the prescriber and the patient is the first thing that needs to occur. Lower extremity orthotics have multiple purposes such as improved positioning, pain relief, stretching, stability, standing, and/or ambulation. Some orthotics can serve multiple purposes simultaneously, while others are more specific. For example, an ankle foot orthosis (AFO) may provide improved stability and allow independent ambulation, while others may be designed specifically for stretching purposes but not intended to be used for ambulation.

Length

Different lengths provide different forces and therefore provide different degrees of control of the body. It is important to choose an appropriate length that allows adequate but not excessive control as we want the patient to be as functionally mobile as possible while minimizing limitations. The length is twofold in that it is the length up the calf/shin proximally as well as the length distally along the plantar aspect of the foot itself.

Types

Foot Orthosis (FO)

There are multiple different options of foot orthoses used for proper positioning of the foot inside of the shoe, and they may be off the shelf or custom. The focus is on controlling the hindfoot, midfoot, and/or forefoot. A University of California Berkeley Labs (UCBL) orthotic (Fig. 2.3) has the most control that can be provided without extending above the shoe as it provides more firm calcaneal control and arch support. Other options include heel cups for shock absorption, midfoot orthoses for arch support, and metatarsal bars for metatarsalgia.

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Fig. 2.3

Bilateral UCBL foot orthotics

Supramalleolar Orthosis (SMO) (Fig. 2.4a–c)

Supramalleolar orthoses are ankle orthoses that come just above the malleoli. These provide stability in the foot and ankle but not to the same degree as a full AFO. These are typically used in children with very mild and intermittent toe-walking gait or medial-lateral instability at subtalar joint, midfoot, or forefoot. It allows free dorsiflexion and plantar flexion which can be beneficial for a child who is learning to walk and spends a great deal of time crawling on the floor.

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Fig. 2.4

(a) Custom supramalleolar orthoses with full length foot plate. (b) Posterior view of SureStep™ SMOs with free toe to allow for flexion at phalangeal joints. (c) Anterior view of SureStep™ SMOs with free toe

Ankle Foot Orthosis (AFO)

The main goal of this orthosis is to provide stability through the ankle and subtalar joints. This can be accomplished by a variety of methods as noted in more detail below.

Posterior Leaf Spring (PLS)

A PLS style of AFO provides the least amount of support but is the lightest weight and most appropriate for patients who have foot drop as it allows some flexibility in plantar flexion and is typically set in a few degrees of dorsiflexion to assist with toe clearance. These are typically off the shelf and therefore can be obtained quickly. These can be fabricated from different materials such as thermoplastic or carbon fiber. The thermoplastic will flex more easily, while the carbon fiber will retain some of the energy and transfer a portion of the energy when pushing off from the ground.

Solid Ankle (Fig. 2.5a, b)

The amount of support that is applied in a solid ankle AFO varies greatly and must be communicated to your orthotist to achieve the desired goal. The plastic may be semi-flexible or very rigid depending on the needs of the patient. In the case of significant spasticity, the AFO should be made firm. The ankle may be set in different positions to affect the gait and impact the knee. For example, dorsiflexion at the ankle creates knee flexion at heel strike, and plantar flexion at the ankle creates knee extension at heel strike. This must be considered when creating the orthotic and may be used to treat knee instability such as genu recurvatum. These AFOs may also be created with a soft foam or silicone inner liner to provide extra skin protection or support depending on the needs of the patient.

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Fig. 2.5

(a) Anterolateral view of custom solid ankle AFO. (b) Posterior view of custom solid ankle AFO

Articulated (Fig. 2.6a, b)

Articulated AFOs allow for motion at the ankle. It is the decision of the provider as to how much motion and in which direction to allow at the ankle, and this must be specified in the prescription. In a patient with strictly medial/lateral instability, the ankle may be allowed free dorsiflexion and plantar flexion range. A patient who fatigues over the day and whose gait becomes crouched may benefit from a check strap which allows adjustment in the amount of dorsiflexion allowed with continued free plantar flexion. For patients with significant plantar flexion spasticity, it is possible to allow for free dorsiflexion while placing a plantar flexion stop to prevent plantar flexion beyond a specific degree specified by the provider.

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Fig. 2.6

(a) Custom articulated AFO lateral view. (b) Custom articulated AFO anterolateral view

Ground Reaction (GRAFO) (Fig. 2.7)

Ground reaction AFOs work well for patients who ambulate with a crouched gait pattern as the ground reaction pushes up through the anterior cuff and shin of the patient providing an extension torque on the knee. This provides extra stability and supports promoting an upright posture. This does not work well if there is significant hamstring tightness and/or spasticity.

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Fig. 2.7

Ground reaction AFO

Nighttime Stretching (Fig. 2.8)

Night splints may be with Ultraflex™ hinges or Velcro™ with anterior placement depending on the needs of the patient. The Ultraflex™ hinge allows for a progressive stretch (most typically in dorsiflexion) of the ankle where the Velcro™ is less dynamic. Both require the use of a knee immobilizer in order to stretch the gastrocnemius which is most commonly tightened. These are typically worn at night when sleeping to allow a prolonged stretch during times of peak growth. There is typically grip placed on the sole to allow for brief ambulation such as nighttime trips to the bathroom.

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Fig. 2.8

Night brace with hinge

Knee Ankle Foot Orthosis (KAFO) (Fig. 2.9)

KAFOs are often used for ambulation and/or exercise and can assist in the prevention of joint contractures. The knees may be jointed or locked depending on the needs of the patient and the strength of the quadriceps and hamstrings. They require a significant amount of energy expenditure and therefore are not typically used for routine ambulation and very rarely for lengthy distances. Because of the added weight and therefore strength required in an already compromised limb, these can be very tricky to learn to use and typically require the assistance of a physical therapist to train the patient and family on how to use appropriately. The use of a KAFO is excellent for exercise purposes along with improved bone health and growth due to active weight-bearing through the long bone(s).

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Fig. 2.9

Knee ankle foot orthosis

Hip-Knee-Ankle-Foot Orthosis (HKAFO)

An HKAFO has both a hip belt and joint which may be locked or mobile along with the knee. As a patient progresses, more of the joints may become unlocked. Again, this is often cumbersome and used mostly for exercise and bone health purposes but typically not for routine mobility although there are some rare exceptions.

Reciprocating Gait Orthosis (RGO) (Fig. 2.10a, b)

Once a child has obtained good trunk control and coordination while using a standing frame and advanced to ambulation, a reciprocating gait orthosis may be beneficial to them as this provides contralateral hip extension with ipsilateral hip flexion. This is typically used by children aged 3–6 years who have the ability to activate hip flexion but have weak or poor hip extension. Once again, it is not often used for functional ambulation as it is very energy demanding and the patient will fatigue quickly.

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Fig. 2.10

(a) Lateral view of reciprocating gait orthosis (RGO). (b) Anterior view of reciprocating gait orthosis (RGO)

Durable Medical Equipment (DME)

Goal

For DME, like orthoses, the goal must be identified prior to prescription for any durable medical equipment. The key feature of DME is centered first around safety and second around independence. Both of these must be taken into account when writing a prescription. It is not uncommon for the patient to have one type of DME that provides a higher level of safety and stability while working in therapy to progress to the next level with less intervention.

Writing a Prescription

Once the goal is identified, a prescription can be written to either a physical therapist for further evaluation and fitting or to a DME provider if you are knowledgeable of specifics. For items that are not customized, you may write the prescription to the DME provider itself specifying the item that you desire for the patient, the frequency of use you desire, and any specifics about the item that you desire. One way to ensure a good fit, function, and use of a piece of DME is to have the assistance of a physical therapist. They are specially trained to fit the equipment to the patient with full measuring and often have some equipment the patient may try prior to purchase. In addition, the therapist assists after attaining the item for further education and training. Each piece of DME may have specific add-ons, attachments, or other items that can be specially adapted to help your patient function.

Types of DME

Wheelchair

Resembling the diverse needs of the cerebral palsy patients themselves, wheelchairs come in a variety of different types to meet all the various needs of the patient. Wheelchairs can be a standard manual wheelchair (Fig. 2.11) without any electronic components or may be completely motorized power wheelchairs that can even be driven with the slight movement of a head or sip and puff system. Again, it is a delicate balance to